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Principles
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Spinal Cord
Medicine
Principles
and Practice
EDITOR-IN-CHIEF
ASSOCIATE EDITORS
New York
Demos Medical Publishing, Inc., 386 Park Avenue South, New York, New York 10016
2003 by Demos Medical Publishing, Inc. All rights reserved. This book is protected by copyright.
No part of it may be reproduced, stored in a retrieval system, or transmitted in any form or by any
means, electronic, mechanical, photocopying, recording, or otherwise, without the prior written
permission of the publisher.
v
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Contents
Preface xiii
Foreword xv
Contributors xvii
I INTRODUCTION
vii
viii CONTENTS
17. The Immune System and Inflammatory Response in Persons with SCI
Fredrick S. Frost, M.D. and Lily C. Pien, M.D. 213
20. Spinal Cord Injury: The Role of Pharmacokinetics in Optimizing Drug Therapy
J. Steve Richardson, Ph.D. and Jack L. Segal, M.D., F.A.C.P. 247
26. Sexual Dysfunction and Infertility in Men with Spinal Cord Disorders
Stacy Elliot, M.D. 349
36. Syringomyelia
James W. Little, M.D., Ph.D. 501
VI MUSCULOSKELETAL CARE
VII REHABILITATION
51. Recreation and Leisure Skills for People with Spinal Cord Disorders
Jo Lemons, T.R. and Nancy C. Cutter, M.D., P.T. 705
Index 993
Preface
S pinal cord medicine (SCM) is best described as the art sections that cover various aspects of medical manage-
and science underlying the diagnosis and treatment of ment, and the management of bladder, bowel, and sex-
patients with spinal cord injury (SCI) and spinal cord ual dysfunctions. A section on the neurologic aspects of
related disorders (SCD). The twentieth century was a spinal cord care considers topics that include the neuro-
landmark era in SCM, highlighted by the development of logic assessment of SCI, electrophysiologic evaluation of
emergency medical services, the establishment of the spe- the spinal tracts, myelopathies, multiple sclerosis, spas-
cialized spinal cord centers, and advances in antibiotic ticity and pain management, autonomic dysfunction, and
use, modern therapies, and technology breakthroughs. concomitant SCI and traumatic brain injury. The mus-
Many patients with SCI and SCD are now living healthy culoskeletal care section addresses overuse injuries, osteo-
lives, with life expectancies approaching that of the gen- porosis and long bone fractures, and interdisciplinary
eral population. Despite recent advances in SCM, con- approaches to upper extremity and pressure ulcer man-
siderable challenges remain and our patients suffer from agement. The rehabilitation section covers wheelchair and
multiorgan failures that require long-term and multidis- seating assessment, orthotics, activity of daily living train-
ciplinary care; and few residency training programs offer ing, vocational and driving training, and functional elec-
exposure or training in comprehensive SCI care. trical stimulation. This is followed by discussions of
This book had its origins in the first review course, recent advances in spinal cord research, including func-
Spinal Cord MedicineAn Intensive Review given at the tional magnetic stimulation, spinal cord regeneration and
annual meetings of the American Paraplegia Society, to repair strategies, and body weight supported ambulation.
prepare physicians to take the subspeciality board in Special topics on aging, womens issues, pediatric care,
spinal cord medicine. Our goal was to develop a com- and SCI prevention are also reviewed. The final section
prehensive text that would cover the breadth and depth considers psychosocial issues, cost of care, SCI systems of
of the field of SCM, covering topics from acute medical care, and various supportive environments for SCI and
and surgical management to cutting-edge research, reha- SCD care.
bilitation, and psychosocial care. This book was developed for all physicians, research
Spinal Cord Medicine consists of 74 chapters in 10 scientists, and other health care professionals involved
sections. It begins with a review of anatomy and physi- in the management of individuals with SCI, multiple scle-
ology, spinal cord imaging, and epidemiology, and is fol- rosis, and other spinal cord disorders. It is a practical
lowed by a comprehensive discussion of acute spinal cord guide, and has been developed to be the ultimate single
injury management that covers prehospital management, source of information on SCM. It will be especially use-
emergency room evaluation and intensive care, and con- ful for physicians who are preparing for the subspecialty
siderations relating to spine surgery. This is followed by certification examination on Spinal Cord Injury Medi-
xiii
xiv PREFACE
cine. We are honored to have chapter contributions from our colleagues in SCM and from all readers in the med-
over 130 authors, selected based upon their training, ical and scientific community.
experience, and clinical leadership, and representing over
20 medical specialties and subspecialties. We welcome Vernon W. Lin, M.D., Ph.D.
comments, suggestions, and constructive criticism from
Foreword
In early 1998, an organizing committee under the lead- In 74 chapters, written by 128 authors, this book
ership of Dr. Vernon Lin began the process of planning covers the normal and abnormal spinal cord from every
an annual review course on Spinal Cord Medicine. This conceivable perspective. Some of the chapters I have had
course has been presented on a yearly basis since. The the opportunity to read are the best I have seen in my 25
committee was unable to find a textbook that it could years of work as a spinal cord specialist (which
recommend to the course attendees that adequately cov- included editing two books on the subject). I have no
ered the course topics. Available texts were either too doubt that they will become the reference whenever
brief and superficial or too large with much content unre- future authors need to cite a review of the subject.
lated to the topic of Spinal Cord Medicine. The closest fit This book is truly a magnum opus. It is unlikely that
was a text by Dr. Robert Young and myself, Diagnosis anything like it will appear again for many years, if ever.
and Management of Disorder of the Spinal Cord, pub-
lished in 1995. However, this book did not cover all top-
ics included in the course. Also, much new information Robert M. Woolsey, M.D.
had become available since 1995 and a second edition Professor of Neurology
of that book was not planned. St. Louis University
Dr. Lin is a problem solver. If a satisfactory book is Director, Spinal Cord Injury Service
not available, he will produce one. In amazingly short St. Louis VA Medical Center
order he assembled the outstanding cadre of editors and
contributors who have created this book.
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Contributors
INTRODUCTION
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1 History of Spinal Cord
Medicine
he history of spinal cord injury is a of spinal cord injury are found in cases 2933 and 48 of
ANCIENT EGYPT Case 33 is very similar. These cases describe the car-
dinal symptoms of complete cervical cord injury sec-
The Edwin Smith papyrus, which dates to 3,000 to 2,500 ondary to fracture of the cervical spine: tetraplegia, com-
years B.C. is the most authentic document in ancient plete sensory loss, urinary incontinence, priapism,
Egypt. It was written by an Egyptian physician, most involuntary ejaculation, and conjunctival congestion
likely Imhotep (Figure 1.1), and later translated by the caused by the loss of vasomotor control.
famous Egyptologist, Dr. James Breasted of Chicago Uni- The Egyptians did not attempt surgical intervention
versity (1,2,3). It contains accurate descriptions of com- for spinal cord injury, although they did reduce fractures
plete injuries of the cervical spinal cord, as well as the and perform surgical decompression following skull frac-
observation that the best treatment for the injured verte- tures. They used bronze catheters for urinary drainage,
brae is rest and support (Figures 1.2, 1.3). Descriptions and described pressure ulcers.
3
4 INTRODUCTION
FIGURE 1.2
FIGURE 1.1
The Edwin Smith papyrus.
Imhotep.
ANCIENT INDIA His travels in Greece and Egypt greatly affected his med-
ical advancements. He created a method called succession
The Sushruta Samhita (4), written in India during the on a ladder, as well as other methods of traction, for the
third or fourth century A.D., describes the treatment of treatment of spinal cord injuries. He also described para-
spinal injury. The care of cervical dislocations involved plegia with bowel and bladder dysfunction, pressure
manipulative reduction, bandages, splints, and bed rest. ulcers, gibbosity at different levels, and paralysis accom-
Fractures of the lower spine were treated by immobiliza- panied by cold abscess (Potts disease). Indicating that
tion; the patient was placed on a board and tied down traumatic gibbosity is not correctable, he stated where-
by ropes to five pegs. The authors of the Sushruta did fore succession on a ladder has never straightened any-
not believe that spinal fractures were curable. body, as far as I know, it is principally practiced by those
Vaidya (400 B.C.) distinguished different types of physicians who seek to astonish the mob.... But the physi-
ulcers using clinical examination and auscultation (5). cians who follow such practice, as far as I have known
them, are all stupid. In Anatomy of the Spine, Hip-
pocrates mentioned details of articulation, nerves and ves-
ANCIENT GREECE sels, sheaths of the spinal marrow (cord), ligaments and
muscles. In regard to spinal injuries, he states:
Fragmentary records describe the contribution of the
In cases of displacement backward along the vertebrae,
ancient Greeks to spinal cord medicine. Aesculapius, god
it does not often happen, in fact, it is rare that one or
of medicine, was believed to be the son of Apollo, god of more vertebrae are torn from one another and dis-
the sun. The religious cult of Aesculapius took the place placed. For such injuries do not readily occur; as a
of scientific medicine. Hippocrates (460370 B.C.) (Fig- spine could not easily be displaced backward but by a
ure 1.4) disassociated medicine from religion, and severe injury on the fore part through the belly (which
brought Greek medicine to its highest achievement (6). would prove fatal) or if a person falls from height and
HISTORY OF SPINAL CORD MEDICINE 5
FIGURE 1.4
Hippocrates.
FIGURE 1.3
lotus, and many other topical applications on decubiti.
Translation of the Edwin Smith papyrus. He recommended wound irrigation with clean water or
boiled river water (7,8). Celsus advised cleaning with
vinegar and suturing small ulcers with womens hair.
should pitch on the nates or the shoulders (and even
Greek medicine, especially from the school of Alexandria,
in this case he would die, but not immediately). The
spinal marrow would suffer, if from displacement of
influenced and was succeeded by Roman medicine.
a vertebra it were to be bent, even to a small extent,
for the displaced vertebra would compress the spinal
marrow, producing insensitivity of many great and ANCIENT ROME
important organs and many other ill consequences of
a serious nature. In the first century A.D., Aulus Cornelius Celsus, in his
treatise De Medicina, included a brief discussion on spinal
He condemned the open reduction of such cases, and cord injuries, especially fractures of the spinous processes.
favored spontaneous callus formation. He mentioned that For incomplete spinal cord lesions, he recommended Hip-
displacement of vertebrae forward is mostly fatal. If it is pocrates method of traction for vertebral dislocations. In
not fatal, the patient loses the power of his legs and arms complete spinal cord lesions, death usually ensued.
(tetraplegia), and has torpor of the body and retention Galen (131201 A.D.) (9), who practiced in second-
of urine. He introduced the extension bench and other century Rome, was a physician to Marcus Aurelius. He
methods for the reduction of deformities, particularly gib- followed Hippocratic methods, adding very few new tech-
bous. Hippocrates did not distinguish between paralysis niques to medical treatments, but as a founder of exper-
caused by trauma or disease and considered treatment to imental physiology, he wrote over 400 separate treatises.
be the same for either of them. Not only did he describe the anatomy of the brain, but
Hippocrates recommended keeping ulcers dry and also that of the spinal cord and the brachial and lum-
exposed to the air, except when wine or a cataplasm is bosacral plexuses. In addition, he anticipated Brown
applied. He used juices, honey, vinegar, oil, lead, alum, Sequards hemisection. He described injuries of the first
6 INTRODUCTION
and second cervical vertebrae, stating that they are fatal, most comprehensive of which is the Canon of Medicine,
and that respiration stops with injuries at the third or in five volumes. This book was translated into Latin and
fourth vertebrae. He also described other lower-level was the main reference used in European schools for
injuries. Galen believed that evacuation of the bladder almost six centuries.
was accomplished by contraction of the abdominal mus- In relation to spinal injuries, Avicenna followed the
cles, and eminent scholars such as Vesalius and Albrecht method of Paul of Aegina. To reduce dislocations of the
von Haller promulgated this doctrine for centuries. thoracolumbar spine, the physician placed the patient in
Oribasius (10) was born in Greece in the fourth cen- the prone position and stood with his heels on the gib-
tury (324400). He later went to Rome at the invitation bosity. To reduce dislocations of the cervical spine, the
of the Emperor Julian, who requested that Oribasius com- patient was placed in the supine position and neck exten-
pile his knowledge of Greek medicine. While in Rome, he sion was followed by splint fixation. The Arab physician
modified the Hippocratic table to correct spinal deformity Moses Maimonides (Figure 1.6) wrote on diet, hygiene,
using forceful and brisk procedures. and toxicology (15). He practiced in Cairo and followed
Paul of Aegina (625690) (11), also a Greco- Avicennas system. His book, published in 1199, men-
Roman, compiled several books. Book VI described Hip- tioned paraplegia as well as some neurologic signs.
pocrates technique for treating spinal injuries and in Another Arab physician, Albucasis, recommended
addition advised postreduction spinal splinting for the removal of bone fragments from the spinal canal.
treatment of dislocations. He is considered the origina- Western medicine at this time was very primitive.
tor of laminectomy for spinal cord decompression and the Medicine was practiced not by physicians, but by monks
removal of the offending bony fragments. who used religious rites, herbs, salves, and brews to cure
These discoveries and advancements influenced later disease. Although there are many references to the med-
civilizations, just as Greek civilization had influenced that ical remedies of the time, there is nothing recorded about
of Rome. spinal cord medicine.
With the gradual infiltration of the Roman Empire by By the thirteenth century, the school of Salerno brought
barbarian tribes, science was replaced by superstition and European medicine out of the Dark Ages. Constantinus
intellectual stagnation. During this time, Jewish medicine Africanus (16) learned the Arabic language and practiced
may have been the only type of medicine practiced (12). Greco-Arabic medicine. Roland of Parma wrote his book,
In the seventh century, with the rise of Arabic and Islamic Chirurgia, (17) in Salerno in 1210. He discarded the use
civilizations, many works were translated from the Greek, of Hippocrates bench and used new methods for spinal
Latin, Persian, and Hindu languages into Arabic. Avi- cord injuries. For cervical dislocations, the patient was
cenna (9801037) (Figure 1.5), a Persian physician, trans- placed in the sitting position and reduction was effected by
lated and made contributions of his own (13,14). Dur- traction applied to the hair or by a cloth sling under the
ing his life, he compiled about one hundred books, the jaw. For thoracic and lumbar dislocations, the patient was
placed in the supine position and the physician exerted
traction on the legs, with countertraction applied to the ery. Almost a decade later in France, Louis removed a
upper half of the body by an assistant. During this time, metal fragment from a paraplegic officer who eventually
several famous authors in France and Italy published recovered. Trephining the lamina was suggested by
whole books on spinal cord medicine. A gradual transition Chopart (20) (17431795) and Desault (20) (17441795).
from the Latin and Greek languages to the French and The latter recommended trephining the laminae for
English languages paralleled the development of science, decompression even in the absence of visible fracture. In
mathematics, and philosophy in Western Europe. 1762 Antrine Louis (21) removed a bullet lodged in the
spine. In 1793, J. Soemmering wrote about dislocations
and fractures of the spine in his book, Bemerkungen ueber
THE LATER RENAISSANCE Verrenkung uns Bruch des Ruckgraths (20). In the second
half of the eighteenth century, Jean Louis Petit (21) wrote
Paul of Aeginas book (18), written in 1465, recom- his treatise, Les Maladies des Os (Diseases of the Bones),
mended a process for the reduction of a spinal fracture. and his method was used almost exclusively during that
While the physician exerts direct pressure at the fracture century. He reduced fractures by hyperflexion of the spine
site, traction is applied to the upper extremities by one in an attempt to disengage the spinous processes. Lorenz
assistant, and to the lower extremities by another. Heister (22) was a leading German surgeon who used
Ambroise Par was a progressive surgeon who wrote Petits method for thoracic and lumbar injuries, but used
the Ten Books of Surgery (Dix Livres de Chirurgie) 1564 extension for cervical injuries. Hunzovsky in Germany
(19). As a barber-surgeon, he joined the army, and hyperextended the neck for cervical injuries by suspend-
advanced to be the chief army surgeon to four successive ing the patient from the ceiling (23).
kings. He adopted and modified the Hippocratic tech-
nique of fracture reduction by placing the patient in the
prone position. However, he cautioned against causing NINETEENTH CENTURY
more damage through manipulation. The spine was
immobilized by lead splints, and the patient remained in The nineteenth century saw advances in anatomy, pathol-
bed in the supine position for a lengthy period of time. ogy, physiology, and surgery. Medicine became a more sci-
Par also went back to the work of Paul of Aegina, reviv- entific discipline. The history of spinal cord medicine in
ing laminectomy for fractures causing cord compression. the nineteenth century was reviewed by Ohry and Ohry-
For fractures of the spinous process, he only recom- Kossoy (24), and in Garrisons History of Neurology (25).
mended the removal of bony fragments if the patient was Sir Astley Cooper (26) described cauda equina lesions and
in pain. If the fragments were still attached by periosteum their management, as well as fractures and dislocations
and the patient was not in pain, he reduced the fracture of the spine resulting in paralysis. In 1860, BrownSequard
and splinted the back until full healing occurred. For cer- described different kinds of spinal paralysis based on vas-
vical dislocation, he had an attendant press hard on the cular changes (27,28,29). Injuries were divided into tho-
shoulders while he pulled up the head by two hands close racolumbar and cervical paraplegia until 1881, when Sir
to the ears. With some turning of the head, the disloca- William Gull coined the term quadriplegia.
tion was reduced and a stabilizing bandage was applied With the adoption of Joseph Listers (18271912)
around the shoulders. France followed Pars technique techniques of antisepsis, surgical morbidity and mortal-
for a long time; in fact, Jean-Franois Calot used the same ity decreased. Hence, surgeons were encouraged to inter-
techniques in the nineteenth century (20). Falicius Hildans vene more frequently, and progress was made in the field.
(15601634) used a clamp to reduce cervical dislocations, In 1815, H. Cline recommended a controversial new pro-
and in some cases, removed bone fragments and repaired cedure: The removal of the fractured spines and laminae
ligaments. (laminectomy) in fracture dislocations. Because laminec-
tomy weakens the spine, Burrell, in 1887, applied a plas-
ter jacket for postsurgical stabilization. This jacket was
SEVENTEENTH AND also used after manipulative reduction without surgery.
EIGHTEENTH CENTURIES In 1895, Sir Victor Horsley presented his results for treat-
ment of tuberculosis of the spine to the British Medical
By the seventeenth and eighteenth centuries, anatomic dis- Association (30).
section was permitted in Europe. Despite this advance- Two famous world leaders were the victims of spinal
ment, the management of spinal injuries was still primi- cord injuries caused by a gunshot wound, and both died.
tive. Pars technique, with some modification, was used Lord Nelson was injured at the battle of Trafalgar, and he
widely in Germany, Holland, Italy, and England. In France, died a few hours after the injury in 1805 (31,32). In 1881,
during the year 1753, Geraud removed a musket ball from James A. Garfield, the twentieth President of the United
the lumbar spine and the patient experienced partial recov- States, was shot, and died in less than three months (33).
8 INTRODUCTION
FIGURE 1.8
FIGURE 1.7
Sir Ludwig Guttman.
Donald Munro, M.D.
EDUCATION
ical Therapy of the Nervous Diseases, probably because
the condition carried a mortality rate of 80 percent dur- The care of SCI was very briefly taught in medical schools
ing World War I. In the 1930s, Donald Munro, a neu- until Donald Munro introduced the concept of compre-
rosurgeon at Boston City Hospital, developed an inter- hensive care for SCI victims. His career heralded a turn-
est in and sympathy for the victims of SCI. He developed ing point in the history of SCI treatment. His ideas on neu-
a small center for the comprehensive care of SCI patients, rotraumatology and rehabilitation were first accepted in
and functioned not only as a neurosurgeon, but also as the military hospitals. For the next 40 years, the Veterans
a rehabilitation specialist, urologist, psychologist, Administration led SCI rehabilitation, and remarkable
socioeconomist, researcher, and teacher. He introduced achievements were produced. Annual conferences were
tidal drainage of the bladder (intermittent bladder irri- held, and the papers presented were recorded in the annual
gation), which reduced the risk of urinary tract infec- proceedings. In the 1970s, Erich Krueger, the national
tions. The U.S. Army initiated a SCI center in Oxford- director, initiated traineeships to prepare physicians qual-
Wingate, Massachusetts, where Munros methods were ified in the care of SCI. In the 1980s, Emanuele Manerino
adopted. Munro was a consultant to those Army hospi- initiated a two-year fellowship program, which graduated
tals having SCI sections in the Bronx, Long Beach, many physicians specialized in the comprehensive care of
Chicago, and elsewhere. Ernest Bors and A. Estin spinal cord injured patients. The Department of Health
Comarr carried the torch of care and research and and Human Services established eighteen SCI centers
opened the way for better care of SCI patients. Advances across the United States, leading to the development of SCI
in rehabilitation were carried out by neurosurgeons act- rehabilitation programs in many major universities. Joseph
ing as chiefs of paraplegia sections (Krueger in the Binard initiated annual courses in different regions and
Bronx, Cramer in Memphis, and Scarffond Pool in established educational courses for non-SCI centers.
Atlantic City). Howard Rusk, Arthur Abramson, and The American Paraplegia Society, founded in 1954
Harry Kessler were important figures in rehabilitation. by A. Estin Comarr, incorporated in 1977 and sponsored
Subspeciality areas began to develop, such as geni- annual educational meetings. The American Paraplegia
tourological rehabilitation, sex therapy, infertility ther- Society also established the Journal of Spinal Cord Med-
apy, drivers training, educational counseling, functional icine, which has become a leading journal in the field. In
independence, and development of assistive devices (47). 1978, the Society attempted to register as the American
10 INTRODUCTION
Board of Spinal Cord Injury, but was not approved by the sected cord (52). At this time, research into genetic engi-
American Board of Medical Specialties. Through the neering and stem cell studies also began (53,54).
efforts of Joel A. De Lisa and other leaders in the field,
in 1995 Spinal Cord Injury Medicine was approved as a
subspecialty under the American Board of Physical Med- SPINAL SHOCK
icine and Rehabilitation. Dr. Margaret Hammond, Chief
Consultant to the VA, headed the task of specifying the In 1874 Goltz and Freusberg in Strasbourg clearly
subject matter of the field. described spinal shock in the dog (55). Sherrington pro-
The American Spinal Injury Association (ASIA), duced spinal shock in the monkey, which he referred to
founded in 1971, has made great contributions to the as a neurogenic condition, by transection of the cervical
diagnosis and comprehensive management of SCI cord. Although he did not use the term autonomic dys-
through annual meetings, teaching sessions, workshops, reflexia, he described a rise in blood pressure elicited by
and research grants. ASIA has sponsored several pro- skin stimulation, which appears after the resolution of
grams overseas and thereby furthered the spread of spinal shock (56).
knowledge in many countries.
In 1961, Sir Ludwig Guttman founded The Inter-
national Medical Society of Paraplegia (IMSOP). This AUTONOMIC DYSREFLEXIA
was a major milestone in the care of SCI worldwide. The
Society holds annual meetings in Stoke Mandeville, U.K., Head and Riddoch first described autonomic dysreflexia
and is affiliated with national societies all over the world, in 1917 (57). Its significance and dangers were described
including the Association Francophone Internationale de by Whitteridge (58); Guttmann and Whitteridge (59),
Groupes dAnimation de la Paraplegie, the American Thompson and Witham (60), Pollock et al., Bors and
Paraplegia Society (APS), the American Spinal Injury French (61,62), Schreibert (63), and Arieff et al. (64).
Association (ASIA), the Australian Branch of IMSOP, the Many other reports followed from Europe, America, and
Deutschprachige Medizinische Gesellschaft fr Paralegie Australia.
(DMPG), the Japan Medical Society of Paraplegia
(JMSOP), the Dutch-Flemish Society of Paraplegia
(NVDP), the National Institute for Care of Paraplegia in HETEROTOPIC OSSIFICATION
Sri Lanka (NIP), the Nordic Medical Society of Paraple-
gia (NMSOP), the Southern African Spinal Cord Associ- Para-articular osteoarthropathy was first described by
ation (SASCA), the Spanish Society of Paraplegia (SEP), Riedel (1883) (65) and Eichart (1895). A detailed descrip-
and the Latin American Society of Paraplegia (SLAP). tion of the condition in paraplegics was elaborated by
IMSOP (now ISCOS) established Spinal Cord as their Madame Klumpke-Dejerine and Cellier (66) and Dejer-
official journal, previously known as Paraplegia. ine et al. (67). They were followed by many others, among
them Soule (68), Abramson (69), Abramson and Kam-
berg (70), Liberson (71), Damanski (72), Hardy and
SPINAL CORD REGENERATION Dickson (73), Paeslack (74) Freehafer and Yurick (75),
and Rossier et al. (76).
The work of Ramon y Cajal (19001920) showed that
although severed central nerve fibers in young and adult
mammals commenced to regenerate, the attempts proved URODYNAMICS
abortive and the process did not functionally benefit the
animal (49). During the next two decades, experiments The first measurement of intravesical pressure was made
on amphibia and reptiles showed that regeneration can by Rudolph Haidenhain (1837897) in Breslau. He and
follow cord injury. Colberg focused on sphincter tone; De Wittich and
From 1950 to 1970, Windle worked with DOCA, Rosenplanter (1867) recorded intravesical pressure, and
ACTH, Promin (bacterial polysaccharide), and millipore Julius Budge (18111888) discovered that the third and
tubules. He concluded that there is little evidence, as yet, fourth sacral anterior roots were the motor nerves of the
that true physiological recovery, either motor or sensory, bladder. Goltz experimentally transected the spinal cord
has been attained in mammals (50). From 1970 to 1980, at the thoracolumbar level in dogs and noted reflex blad-
Kao used microsurgery to transplant autogenous brain der evacuation, which disappeared by ablation of the
tissue, sciatic nerve, and nodose ganglion into the spinal lower cord below this level. Schatz (1841920) initiated
cord, and succeeded in demonstrating axonal growth the use of human subjects for spinal cord medicine
within the grafted tissue (51). Fetal neurons, as solid tis- research. Dubois (18481918) continued his work, mea-
sue and as suspension, were transplanted into the tran- suring bladder pressure and rectal pressure in normal con-
HISTORY OF SPINAL CORD MEDICINE 11
trols and paraplegics. Other names in this field are tified ulcers with five medicines. The bones were forti-
Quinke and Adler in Germany, Angelo Mosso and Paul fied with acid principle, the nerves with stinging princi-
Pellacani in Italy, Fritz Born in Switzerland, and Guyon ple, the pulse with salty principle, and respiration with
and Duchastelet in France (77). bitter principle (104). In ancient Egyptian practice, a wide
In Germany, Adler studied manometry in the neu- variety of topical remedies was offered for decubiti,
rogenic bladder and differentiated conal from supraconal including honey, moldy bread, meat, animal and plant
lesions. In Vienna, Schwartz studied cystometry in gun- extracts, copper sulfate, zinc oxide, and alum. Incanta-
shot wounds of the spinal cord. In the United States, tions and recitals were read before the dressings were
Walker, Dalton, Roussy, and Rossi also made advances. applied (105,106). In Persia, Avicenna applied a variety
In Baltimore, Walker proposed simple devices for cys- of topical treatments (107). Among the Arabs, Mai-
tometry. In St. Louis, Dalton coined the term cystome- monides made nutritional recommendations to promote
try, and described the different types of neurogenic blad- ulcer healing (108).
der. Roussy and Rossi studied cystometry in SCI at During the Renaissance there was no physician of
different levels. In England, Henry Head and George Rid- note until the time of Ambroise Par, who insisted on gen-
doch studied neurogenic bladders and described auto- tle handling and meticulous cleanliness of wounds, and
matic micturition as a part of the mass reflex in supra- made recommendations regarding diet and medication
conal lesions. Sir Gordon Holmes studied neurogenic (109).
bladders in soldiers in France (19151916). In 1933 in During the nineteenth century, the discovery of bac-
London, Denny-Brown and Robertson measured bladder teria by Pasteur and antisepsis by Lister improved the out-
pressure, urethral pressure at the bladder neck, external look of wounds in general. The discovery of X-rays by
sphincter pressure, and rectal pressure. In 1935, Kenneth Roentgen helped diagnose underlying bony pathology. In
Watkins described reflexic and areflexic bladders in cord the nineteenth century, Charcot believed in the trophic
injuries. Donald Munro, who in 1947 introduced tidal theory of pressure ulcers, which holds that ulcers are
drainage, described the atonic cord bladder early in injury, caused by the paralysis itself. However, BrownSequard
the autonomous cord bladder, the hypertonic cord blad- opposed this theory (110).
der, and the inhibited cord bladder. Many others con- The twentieth century brought in the antibiotic era.
tributed to the field in the early years, including Nesbit, Sulfonamide (Prontosil) was developed by Domag K in
Lapides, Tang, Ruch, Bors, Comarr, and Bradley. 1935. Penicillin was discovered by Alexander Fleming in
1929, and clinically applied by Florey and Chain in 1940.
Since then, a tremendous number of antibiotics have been
PHARMACOTHERAPY developed to control wound infection. The role of nutri-
tional factors, such as proteins, vitamins and minerals,
Numerous pharmacotherapeutic agents have been tried in ulcer healing, was recognized in the United States. The
in the treatment of SCI, including methylprednisolone, biomechanics of bedsores has also received a great deal
alone (78) or in combination with the antioxidants alpha- of attention (111).
tocopherol and selenium (79); lidocaine (80); alpha- It is impossible to enumerate the topical medications
methyltyrosine (81); aspirin; dipyridamole (82); naloxone and other forms of treatment that have been tried for pres-
(83); DMSO (84,85,86); immunosuppressive drugs (87); sure ulcers. They include antiseptics and antibiotics
nerve growth factor (88); enzyme therapy such as (112,113); antibacterials, which in some cases were cyto-
hyaluronidinase, trypsin, and elastase (89); calcium-chan- toxic (114,115); debriding agents such as enzymes, mag-
nel antagonists (90); gangliosides (91); thyrotropin releas- gots, and synthetic materials (116); electrostimulation (117);
ing hormone (TRH) (92); neurotrophic factors (53); preg- magnetic stimulation (118,119,120); low-intensity laser
nenolone combined with lipopolysaccarides and (121); ultraviolet light (heliotherapy) (122); topical oxygen
endothacin (93); and nitric acid inhibitors (94). Other therapy (123); hyperbaric oxygen therapy (124); ultrasound
approaches have included genetic strategies (53); cooling (125); ozone (126); dry air (127); carbon dioxide laser (128),
(hypothermia) (95); X-rays (96); electromagnetic stimu- occlusive dressing (129); placenta (130); aloe vera (131);
lation (97,98,99); hyperbaric oxygen therapy (100,101); xenografts (132,133); dermografts (134); growth factors
revascularization (102); and acupuncture (103). (135); topical hyperalimentation (136); and surgical closure
techniques such as flaps (137140) and grafts (141).
PRESSURE ULCERS
CONCLUSION
Pressure ulcers have been recognized since antiquity, and
physicians of different civilizations have proposed vari- Throughout mans history, SCI has been a catastrophic
ous treatments. Chinese ulcer physicians (Young) (97) for- condition, and the prognosis a very gloomy one. Only
12 INTRODUCTION
after World War II did the labor of distinguished clini- 26. Cooper A. Lecture 181 of injuries of the spine. Lancet 1823;
1:303306.
cians and scientists improve the look of SCI victims. In 27. BrownSequard E. Possibility of repair and of return of function
universities all over the world, researchers are working to after a partial or a complete division of the spinal cord in man
erase Imhoteps dictum that spinal cord injury is an ail- and animals. Lancet 1859; 1:9697.
28. BrownSequard CE. Lecture III: Diagnosis and treatment of para-
ment not to be cured. As Professor Max Thoreck has plegia due to myelitis, meningitis or a simple congestion. Lancet
said, La science na pas de patrie (science has no home- 1860; 2:2728.
land). It is hoped that the efforts of researchers around 29. BrownSequard CE. Lecture III, part II. Lancet 1860; 2:7778.
30. Horsley Victor. Chronic spinal meningitis. Brit Med J 1909; 1:513
the world will soon fulfill mans wishes for a cure. 31. Trunkey D. The Admirals wounds. Amer Coll Surg Bull, Feb
1994; Vol 79, 2:1927.
32. Beatty W. Nelsons fatal wound. Lancet, 1906; 6:68.
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14 INTRODUCTION
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2 Anatomy and Function
of the Spinal Cord
segments, and the first lumbar spinous process overlies The spinal cord is not uniform in diameter; it con-
the five sacral segments. The part of the vertebral canal tains two enlargements associated with the innervation of
formed by the last four lumbar vertebrae and the sacrum the upper and lower limbs. The cervical enlargement is
contains a collection of long lumbar and sacral anterior the more prominent of the two and is found at the C5T1
and posterior roots known as the caudal equina (horses levels. These segmental levels are the same levels that give
tail) in addition to a specialization of the pia mater known rise to the nerve roots that form the brachial plexus and
as the filum terminale. thus provide innervation for the upper limb. The lumbar
enlargement gives rise to neurons and fibers that form the
lumbar plexus (L1L4) and the sacral plexus (L4S2),
Surface Anatomy and Enlargements
both of which are involved in the innervation of the lower
The surface of the spinal cord displays a number of lon- limbs. These enlargements are the natural result of the
gitudinally oriented grooves (Figure 2.2). On the poste- necessary increase in neurons and their processes at these
rior surface in the midline is the shallow posterior median levels for the innervation of limb musculature and skin.
sulcus. This sulcus is continuous with the posterior
median septum, a glial partition extending deeply to the
Terminal End of the Spinal Cord
gray matter. The posterolateral sulcus is a shallow groove
that demarcates the entrance of the dorsal roots into the As mentioned earlier, the tapered end of the spinal cord
spinal cord bilaterally. In the cervical and upper six tho- is usually found at the level of the intervertebral disk
racic spinal cord segments, the posterior intermediate sul- between L1 and L2 and is called the conus medullaris
cus and underlying posterior intermediate septum is (Figures 2.1 and 2.3). The conus medullaris consists of
found between the posterior median and posterolateral sacral spinal cord segments. It provides sensory innerva-
sulci on each side of the spinal cord. On the anterior sur- tion to the saddle area, motor innervation for the sphinc-
face, the prominent anterior median fissure penetrates the ters, and parasympathetic innervation for the bladder and
cord for a depth of approximately 3 mm and contains the lower bowel (i.e., from the left splenic flexure to the rec-
sulcal branches of the anterior spinal artery and vein (Fig- tum). Here again an appreciation of the anatomic rela-
ure 2.2). The anterolateral sulcus marks the site at which tionships between the spinal cord and vertebral column
the ventral root fibers exit the cord. However, because the is useful in the diagnosis of some spinal cord disorders
ventral roots exit at less regular intervals and are not as resulting from injury. Trauma in the lower back at the
numerous as dorsal roots, the anterolateral sulcus is not level of the L1 vertebra may result in conus medullaris
as easily seen as the posterolateral sulcus. syndrome, caused by a direct injury of the conus
FIGURE 2.2
medullaris at this level (Figures 2.1 and 2.3). The signs branch of the pudendal nerve (S2S4). As in the bowel,
and symptoms of this syndrome are permanent flaccid the bladder dysfunction of conus medullaris syndrome is
paralysis of the external anal sphincter and fecal inconti- caused by the destruction of neurons at the S24 levels
nence; bladder distension and incontinence; impotence; of the spinal cord. Generally, the bladder is permanently
and perianal or saddle anesthesia. areflexic in conus medullaris syndrome because of the loss
The flaccid paralysis of the external anal sphincter of the sacral spinal neurons that give rise to the bladder
is caused by the destruction of the somatic lower reflexes. Thus, a spastic or automatic bladder does
motoneurons that innervate this voluntary muscle at the not develop as it would when the cord is injured at more
S2S4 levels. These neurons normally project axons to the rostral levels; the sacral bladder reflex circuitry is spared,
muscle via the inferior rectal branch of the pudendal and these neurons become hyperactive after recovery
nerve. Bladder distension and incontinence is caused by from spinal shock.
paralysis of the detrusor muscle, the smooth muscle wall In conus medullaris syndrome, impotence is pri-
of the bladder innervated by the pelvic splanchnic nerves marily caused by a loss of parasympathetic neurons at
arising from preganglionic parasympathetic neurons at S2S4. Erection is achieved normally when parasympa-
S2S4, and also by paralysis of the urethral sphincter, the thetic stimulation causes the smooth muscle of the arter-
striated (voluntary) muscle innervated by the perineal ies associated with the erectile tissue of the penis to relax.
As a result, the arterial lumina enlarge and blood is
allowed to flow into and dilate the cavernous spaces in
the corpora of the penis. The bulbospongiosus and ischio-
cavernosus muscles are innervated by the deep branch of
the perineal nerve, a branch of the pudendal nerve
(S2S4). Normally, during erection, these muscles con-
tract and thus compress the venous plexuses at the periph-
ery of the corpora cavernosa, preventing the return of
venous blood. As a result, the penis becomes erect.
Because it is likely that neurons innervating the bul-
bospongiosus and ischiocavernosus muscles will also be
lost in conus medullaris syndrome, paralysis or paresis
of these muscles contributes to impotence.
Loss of sensory neurons in the dorsal horn at S4 and
S5 causes perianal anesthesia and saddle anesthesia (i.e.,
anesthesia of the posterior thigh). In conus medullaris
syndrome, this anesthesia is caused predominantly by a
loss of sensory neurons at the S2 level. In a pure conus
lesion, normal sensory and motor function is retained in
the lower limb (i.e., assuming only the conus medullaris
is injured and lumbar roots of the cauda equina are
spared). If the S1 level is spared, the ankle jerk is retained.
Finally, if the conus medullaris lesion is incomplete, some
of the signs and symptoms noted above may not occur.
Lesions of the cauda equina (Figure 2.3) below the
L1 vertebral level result in cauda equina syndrome. The
signs and symptoms of this syndrome are similar to those
following conus lesions. However, cauda equina lesions
usually affect not only peripheral nerve fibers from the
sacral segments of the cord, but also a varying number
of the lumbar dorsal and ventral nerve roots. The sensory
and motor losses are, as a rule, more extensive and reach
higher spinal levels (i.e., the lower limbs) following cauda
equina lesions when compared to lesions of the conus
medullaris. Furthermore, the distribution of motor and
sensory loss is usually more irregular because some of the
FIGURE 2.3 nerve roots will be damaged and others will be spared.
It is important to note that because of the anatom-
Posterior view of lower end of the dural sac. ical proximity of the conus medullaris and cauda equina,
ANATOMY AND FUNCTION OF THE SPINAL CORD 19
The pia mater is the innermost of the three tively. The first cervical nerve lacks dorsal roots in 50 per-
meningeal investments and the only one that directly con- cent of people, and the coccygeal nerves may be absent
tacts the surface of the spinal cord (Figure 2.5). The pia entirely (3). It should be noted that the spinal nerves are
also directly covers the roots of the spinal nerves and the relatively short. Almost immediately after exiting the
spinal blood vessels. At the conus medullaris, the pia intervertebral foramen each nerve bifurcates into a dor-
mater continues inferiorly into the lumbar cistern as a sal and ventral ramus (Figure 2.5). The dorsal ramus pro-
slender filament called the filum terminale (Figure 2.3). vides the cutaneous innervation of the back and the motor
Surrounded by the nerve roots of the cauda equina, the innervation of the intrinsic or deep back muscles. All the
filum terminale pierces the arachnoid at the level of the appendicular and remaining trunk muscle innervation
second sacral vertebra and then becomes invested by dura and sensory innervation of the body (excluding the motor
mater to become the coccygeal ligament (Figure 2.4). By and sensory innervation provided by cranial nerves) is
attaching to the periosteum of the coccyx, the coccygeal provided by the ventral ramus of each spinal nerve. It is
ligament centers the spinal cord within the lumbar cistern important to appreciate that whereas the dorsal and ven-
and serves as an anchor to maintain that position to pre- tral roots convey pure sensory and motor fibers respec-
vent cord injury, especially under conditions of sudden tively, the dorsal and ventral rami are mixed nerves and
jarring that could force delicate spinal tissue up against each contains both sensory and motor fibers.
the bony walls of the vertebral canal.
Bilaterally thickened lateral extensions of the pia
mater give rise to the denticulate ligaments (Figure 2.5). NEUROANATOMIC ORGANIZATION OF THE
The two denticulate ligamentsone on the right and one SPINAL GRAY AND WHITE MATTER
on the leftare located between the dorsal and ventral
roots on each side of the spinal cord. From each ligament In cross section, the spinal cord is composed of a central
twenty to twenty-two toothlike triangular processes portion of butterfly-shaped gray matter and peripherally
extend further laterally, pierce the arachnoid, and fuse oriented white matter (Figure 2.5). The gray matter is
with the dura. The attachment of the processes of the lig- comprised predominantly of neurons, their processes, and
ament alternates with the passage of the nerve roots glial cells and has an enriched blood supply. The white
through the dura mater (Figure 2.5). The first attachment matter contains ascending and descending fiber tracts and
is at the level of the foramen magnum, whereas the most glial cells and appears white in unfixed tissue because of
caudal attachment is between the T12 and L1 nerve roots a predominance of myelin. The two halves of the gray
(Figure 2.3). As in the filum terminale and coccygeal lig- matter are connected across the midline by a dorsal and
aments, the attachments of the denticulate ligaments to ventral gray commissure, which is located above and
the dura mater provide an important fixation of the spinal below the central canal respectively (Figure 2.2). The gray
cord, protecting it from sudden displacements. matter is further subdivided into a posterior (dorsal) horn
(column) and an anterior (ventral) horn (column). The
thoracic and upper two lumbar spinal cord segments also
Organization of a Spinal Nerve
display a wedge-shaped, intermediate lateral horn (inter-
Sensory information (i.e., pain, temperature, touch, etc.) mediolateral cell column) (Figure 2.2).
is conveyed from the body into the spinal cord via the dor- The white matter on each side of the spinal cord is
sal roots, which enter the spinal cord posteriorly along organized into three large areas or funiculi (Figure 2.2).
the posterolateral sulcus (Figure 2.5). The neuron cell The posterior funiculus is the area of white matter
bodies responsible for conveying the sensory (afferent) between the posterior median sulcus and the posterolat-
information are located in a distal swelling of the dorsal eral sulcus. The lateral funiculus is defined as the white
root known as the spinal ganglion. The spinal ganglion matter between the posterolateral sulcus and an imagi-
is generally surrounded by bone and is found at the level nary line from the medial border of the anterior horn to
of the intervertebral foramen. The ventral roots of the the anterior surface of the spinal cord. The anterior
spinal cord convey motor (efferent) information from the funiculus is the remaining white matter medial to the
spinal cord to skeletal muscle. Ventral root fibers exit the imaginary line noted above and lateral to the anterior
spinal cord at the anterolateral sulcus and also contain median fissure (Figure 2.2). Each funiculus of the spinal
axons from pregangionic autonomic neurons (Figure 2.5). cord is comprised of bundles of fibers that run together
The dorsal and ventral roots that delineate a spinal cord and subserve the same function. Each bundle is referred
segmental level fuse at the level of the intervertebral fora- to as a tract or fasciculus. For instance, at cervical levels
men just distal to the spinal ganglion to form the spinal and the upper six thoracic levels of the spinal cord, the
nerve associated with that spinal cord segment. The spinal posterior funiculus is subdivided into two major fasci-
nerve is a mixed nerve, in that it contains both sensory culi (gracilis and cuneatus) by the posterior intermediate
and motor fibers from the dorsal and ventral roots respec- sulcus and septum (Figure 2.6). (The functional signifi-
ANATOMY AND FUNCTION OF THE SPINAL CORD 21
FIGURE 2.5
Diagram of the spinal cord showing the somatotopic organi- Diagram of the spinal cord showing the somatotopic organi-
zation of fibers in the posterior funiculus. zation of ventral horn motor neurons.
cance of these fiber tracts is discussed in the section laminae, each containing neurons with specific morpho-
White Matter). logic characteristics. Subsequently, the lamination pattern
was confirmed in several species, including man, and has
been referred to as Rexeds laminae (Figure 2.8). Briefly,
Gray Matter
the first six laminae are found in the posterior horn. Lam-
The posterior (dorsal) horn contains clusters of neurons ina I is equivalent to the nucleus posteromarginalis, lam-
concerned with sensory function. The central processes ina II to the substantia gelatinosa, and laminae III and
of spinal ganglion cells generally synapse on neurons in IV together comprise the nucleus proprius of the older lit-
the dorsal horn, where sensory information is relayed erature. Laminae V and VI comprise the neck and base
either to higher centers in the brain or segmentally within of the posterior horn respectively. Lamina VII is equiva-
the spinal cord. lent to the intermediate gray matter that includes the lat-
The lateral horn is limited to the thoracic and upper eral horn at T1L2 and Clarkes nucleus (nucleus dorsalis)
two lumbar spinal cord segments. It contains pregan- at C8L2 (see posterior spinocerebellar tract, later).
glionic sympathetic neurons whose axons exit the spinal Laminae VIII and IX are in the anterior horn. Lamina VIII
cord via the ventral roots. Preganglionic parasympathetic is composed of interneurons, whereas lamina IX is com-
neurons are located in a comparable region of the gray prised of individual clusters of alpha motoneurons.
matter at the S2S4 levels of the spinal cord. However, Finally, lamina X is comprised of small neurons sur-
well-defined lateral horn is not found at these sacral lev- rounding the central canal. For more detailed informa-
els. Axons of the preganglionic parasympathetic neurons tion regarding Rexeds laminae, consult Carpenter and
distribute to the descending colon, sigmoid colon, rectum, Sutin (4).
and all pelvic viscera via the pelvic splanchnic nerves.
The anterior (ventral) horn contains both interneu-
White Matter
rons and motoneurons. Motoneuron axons innervating
skeletal muscle comprise the major component of the ven- Heavily myelinated nerve fibers in the posterior funicu-
tral root. Alpha motoneurons of the anterior horn are lus are concerned primarily with two general modalities
somatotopically organized such that neurons supplying related to conscious proprioception. These are kinesthe-
flexor muscles are located dorsally in the anterior horn sia (sense of position and movement) and discrimative
and neurons supplying extensor muscles are located ven- touch (discriminating two points and localizing touch sen-
trally. In addition, neurons supplying the trunk muscula- sation). Injury to the spinal cord in the region of the pos-
ture are located medially, whereas neurons innervating terior funiculus may result clinically in a loss or diminu-
the limbs are located laterally within the anterior horn tion of vibratory sense, position sense, two-point tactile
(Figure 2.7). discrimination, and touch and weight perception in the
In 1952, the gray matter of the cat spinal cord was body ipsilateral and caudal to the spinal cord lesion. The
characterized as being comprised of ten distinct zones or central processes of neurons whose cell bodies are located
ANATOMY AND FUNCTION OF THE SPINAL CORD 23
FIGURE 2.9
the dorsal root and then ascend or descend in the fasci- some clinical cases involving preserved sensory function
culus gracilis for one or two segments before synapsing following SCI. In cats, the central processes of spinal gan-
on neurons in the nucleus dorsalis of Clarke (Clarkes glion cells conveying stimuli into the spinal cord from low-
nucleus), located within Rexeds lamina VII. Axons of threshold cutaneous receptors, pressure receptors, and
neurons in the nucleus dorsalis form the posterior spin- impulses following pinching of the skin, synapse on neu-
ocerebellar tract. Since Clarkes nucleus is found only rons in the ipsilateral dorsal horn. These neurons send their
between C8 and L2, the posterior spinocerebellar tract axons into the dorsal aspect of the lateral funiculus, where
is not found caudal to L2 in the spinal cord. The central they ascend to the upper two or three cervical segments
processes of those spinal ganglion cells conveying pro- and synapse on neurons of the lateral cervical nucleus. The
prioceptive information into the spinal cord below L2 lateral cervical nucleus is located lateral to the dorsal horn
ascend to the L2 level in the fasciculus gracilis before in the lateral funiculus of the upper three cervical segments
synapsing on cells in Clarkes nucleus. Similarly, incom- of the cat spinal cord (5). The lateral cervical nucleus gives
ing proprioceptive information entering the spinal cord rise to axons that cross the midline in the anterior white
rostral to C8 are carried by nerve fibers ascending in the commissure (Figure 2.2) at spinal levels C1 and C2 and
fasciculus cuneatus. The cuneate tract nerve fibers ascend with the medial lemniscus to terminate in the con-
synapse on neurons of the accessory cuneate nucleus in tralateral VPL of the thalamus. This spinocervical thala-
the medulla; this is homologous to the nucleus dorsalis. mic tract has a somatotopic organization similar to that of
Both pathways are uncrossed. the posterior funiculus pathways (i.e., sacral fibers are
The posterior spinocerebellar tract conveys to the medially located and cervical fibers are the most laterally
cerebellum information pertaining to muscle contraction, located, [6]). It is important to appreciate that there is evi-
including phase, rate, and strength of contraction. Clini- dence that the pathways in the posterior funiculus (dorsal
cal effects of posterior spinocerebellar tract destruction columnmedial lemniscal system) are functionally linked
in SCI are masked by the effects of destruction of the adja- with the spinocervical thalamic tract, because some spin-
cent lateral corticospinal tract. ocervical collaterals terminate in the dorsal column nuclei
and some cells in the dorsal column nuclei project to the
lateral cervical nucleus (7).
Spinocervical Thalamic Tract
The putative clinical relevance of this information is
Although the spinocervical thalamic tract has not been that the spinocervical thalamic tract accounts for the per-
demonstrated in man, a description of this tract is included sistance of kinesthesia and discriminative touch sensation
here because of its possible relevance to an explanation of following total interruption of the posterior funiculus in
ANATOMY AND FUNCTION OF THE SPINAL CORD 25
experimental animals. According to Afifi and Bergman loss of pain and temperature sensation in the dermatomes
(8), the presence of the spinocervical thalamic tract has corresponding to the affected spinal cord segments. This
been assumed in man because of the similar persistence last pattern of sensory loss is characteristic of
of posterior funiculus sensations after total posterior syringomyelia, a condition caused by a centrally located
funiculus lesions in patients. Thus, the old concept of the cavitation of the spinal cord that destroys the anterior
absolute necessity of the posterior funiculus for all dis- white commissure. Finally, in a BrownSequard lesion
criminatory sensation is evolving into a newer concept (hemisection of the spinal cord), the patient experiences
that attributes to the posterior funiculus a role in the dis- both a contralateral loss of pain and temperature in the
crimination of those sensations that an animal must body (caused by the destruction of the lateral spinothal-
explore actively and to the spinocervical thalamic path- amic pathway in the lateral funiculus) and a bilateral seg-
way a role in the perception of sensations that are mental loss of pain and temperature (caused by a destruc-
impressed passively on the organism (8). tion of the anterior white commissure) that will be slightly
higher than the contralateral loss of pain and temperature
sensation, but still below the level of the lesion.
Lateral Spinothalamic Tract
In the past, the lateral spinothalamic tract was sec-
One of the most clinically important pathways in the tioned surgically to relieve intractable pain. The proce-
spinal cord is the lateral spinothalamic tract, which is con- dure is referred to as cordotomy and may be carried out
cerned with the transmission of pain and temperature sen- unilaterally or bilaterally. If bilateral cordotomy is per-
sations (Figure 2.9). This tract is closely related to the formed, the lesions are made at slightly different levels
anterior spinothalamic tract, and authors often combine in the spinal cord. During surgery, the denticulate liga-
the two pathways and refer to them together as the ment is used as a landmark; lesions are made just ante-
anterolateral system (ALS). In this chapter the pathways rior to the denticulate ligament to locate and transect the
are discussed separately because of their clinical relevance. lateral spinothalamic tract. A unilateral lesion of the tract
Unmyelinated and thinly myelinated dorsal root results in anesthesia of the body wall and limbs, but not
fibers contributing to the lateral spinothalamic tract have the viscera, which are bilaterally represented (9). Fur-
their cell bodies in spinal ganglia. Incoming root fibers thermore, the anogenital region is not markedly affected
synapse on neurons in Rexeds laminae IIV of the dor- with unilateral lesions (9). Clinical results have indicated
sal horn. Neurons in these laminae in turn project axons that after variable periods of time following bilateral cor-
to neurons in laminae VVIII. The laminae VVIII neu- dotomies, there is often a return of pain and temperature
rons, as well as some neurons in lamina I, project axons sensation, thus suggesting that there may be other path-
across the midline in the anterior white commissure to the ways in the spinal cord to convey this modality. These
contralateral lateral funiculus and thus form the lateral pathways may be multisynaptic and pass through the
spinothalamic tract. The crossing fibers of the lateral reticular formation (i.e., spinoreticular, [10]) or involve
spinothalamic pathway ascend from one to two spinal shorter relays (i.e., spinospinal, [9]). In addition, Affifi
segments above their entry level before entering the tract and Bergman (10) have suggested that pain sensation may
in the contralateral lateral funiculus. The fibers of the be mediated through a spinotectal pathway and Carpen-
tract are somatotopically arranged with sacral fibers ter and Sutin (9) have suggested that uncrossed spinothal-
located laterally and cervical fibers located medially amic fibers may be responsible for the return of pain and
within the tract. Note that this arrangement is the reverse temperature sensation following unilateral lesion of the
of the posterior funiculus pathways, in which sacral fibers lateral spinothalamic pathway.
are located medially and cervical fibers are laterally
located. Once formed, the lateral spinothalamic tract
Anterior Spinothalamic Tract
ascends throughout the length of the spinal cord and
brainstem to ultimately terminate on neurons of the ven- The dorsal root fibers conveying light touch sensation and
tral posterolateral nucleus (VPL) of the thalamus. certain types of pain impulses synapse on dorsal horn neu-
Lesions of the lateral spinothalamic tract in the lat- rons in laminae VI to VIII. The axons of these dorsal horn
eral funiculus result in a loss of pain and temperature sen- neurons cross in the anterior white commissure over sev-
sation in the contralateral half of the body, beginning one eral segments and gather in the lateral and anterior funi-
or two segments below the level of the lesion. This pat- culi to form the anterior spinothalamic tract (Figure 2.9).
tern of sensory loss is in marked contrast to the loss that The course and termination of this tract in the spinal cord
occurs following injury to the dorsal roots, in which there and brainstem is similar to the lateral spinothalamic tract.
is segmental or dermatomal loss of sensation ipsilateral Functionally, light touch is defined as the sensa-
to the lesion. Moreover, if pain and temperature fibers are tion provoked by stroking an area of skin devoid of hair
injured as they cross the midline of the spinal cord in the (glabrous skin) with a feather or wisp of cotton (9). This
anterior white commissure, there is a bilateral segmental type of sensation is conveyed to higher CNS centers in
26 INTRODUCTION
addition to the pressure sense and discriminatory tactile lateral funiculus (Figure 2.9). The lateral corticospinal
sensations conveyed by the posterior funiculus. Because tract extends the entire length of the spinal cord and is
tactile sensation is transmitted centrally by the posterior somatotopically organized. As in the lateral spinothala-
funiculi, the anterior spinothalamic tract, and the spin- mic tract, cervical fibers are found medially. They are fol-
ocervical thalamic tract, clinically this particular sensory lowed laterally by thoracic, lumbar, and then sacral fibers.
modality is of little value in localizing injuries to the spinal Once again, it may be instructive to recall that the soma-
cord (9). If the anterior spinothalamic tract is lesioned, totopic organization of both the lateral corticospinal tract
there is little loss of tactile sensibility; however, the affec- and the lateral spinothalamic pathway in the lateral
tive aspect of sensation may be lost. Bilateral destruction funiculus is different from the somatotopic organization
of the anterolateral funiculi may cause a complete loss of the posterior funiculus in that in the latter, sacral fibers
of itching, tickling, and libidinous feeling (11), thus this are mostly medial and cervical fibers are located laterally.
region of the spinal cord has been associated with ones The uncrossed corticospinal fibers descend from the
ability to judge the pleasant or unpleasant character of medulla into the anterior funiculus of the spinal cord as
sensation. In addition to light touch stimuli, the anterior the anterior corticospinal tract (bundle of Trck) (Fig-
spinothalamic tract is thought to convey nondiscrimina- ure 2.9). The anterior corticospinal tract extends only to
tive pain sensations, in contrast to the lateral spinothal- the upper thoracic spinal cord and innervates neurons
amic tract, which is thought to convey the well-localized projecting to the muscles of the upper extremities and
discriminative pain sensations (12). neck. The fibers of this tract generally cross the midline
segmentally within the spinal cord before terminating on
contralateral neurons. In rare cases, fibers do not cross
Other Ascending Tracts
the midline at all and form extremely large anterior cor-
There are several other ascending tracts in the spinal cord ticospinal tracts (16).
lateral and anterior funiculi that are of little clinical sig- Corticospinal fibers terminate mostly on interneu-
nificance. These tracts include the spino-olivary, spino- rons in the spinal cord in laminae IVVII. Evidence also
tectal, spinoreticular, spinocortical, spinovestibular, and exists for a direct projection to alpha and gamma
anterior spinocerebellar. These multisynaptic pathways motoneurons in lamina IX. Because the corticospinal
do not have a well-delineated functional significance, but tracts innervate both alpha and gamma motoneurons,
may play a role in feedback control mechanisms or in the stimulation of corticospinal fibers leads to a co-contrac-
maintenance of the state of consciousness. For more tion of both intrafusal and extrafusal muscle fibers.
detailed information on these pathways consult Carpen- Because of the co-contraction of the two types of muscle
ter and Sutin (13). fibers, there is increased sensitivity of the muscle spindle
(intrafusal fiber) to changes in muscle length even when
the muscle is shortening.
Descending Tracts of the Lateral
It has been estimated that 55 percent of all corti-
and Anterior Funiculi
cospinal fibers end in the cervical cord, 20 percent in the
thoracic, and 25 percent in the lumbosacral segments
Corticospinal Tracts
(17). These data suggest that the corticospinal tracts have
As the name implies, neurons giving rise to the corti- a greater control and influence over the upper extremities
cospinal tracts are found in the cerebral cortex. The axon than over the lower. The corticospinal tracts are essen-
of these neurons projects through the brainstem and ter- tial for skill and precision in movement and also for the
minates in the spinal cord. The corticospinal tracts com- execution of precise movements of the fingers. Interest-
prise the largest, clinically important descending fiber sys- ingly, although the tracts are necessary for speed and
tem in the human neuraxis. The neurons that give rise to agility during precise movements, they are not necessary
the tracts are located in the primary motor cortex (i.e., for the initiation of voluntary movement. They also serve
the precentral gyrus or Brodmanns area 4), the premotor to regulate sensory relay processes and to determine
cortex (area 6), the primary sensory cortex (i.e., the post- which sensory modality reaches the cerebral cortex, as
central gyrus or areas 3, 1, and 2), and adjacent parietal evidenced by terminations on sensory neurons in laminae
cortex (14,15). Although both sensory and motor corti- IV to VII. The proper function of the corticospinal tracts
cal areas contribute to the tracts, the primary motor cor- is dependent on the extent of their myelination. Myeli-
tex and the premotor cortex give rise to 80 percent of the nation of corticospinal fibers begins near birth and is not
tracts. completed until the end of the second year of life.
At the caudal level of the medulla oblongata, the Neurons in the cerebral cortex and their axons that
majority of the corticospinal fibers cross the midline in form the corticospinal tracts have been referred to as
the pyramidal decussation to form the lateral corti- upper motor neurons. The alpha motoneurons in the
cospinal tract, which is located in the dorsal aspect of the spinal cord ventral horns and their axons that directly
ANATOMY AND FUNCTION OF THE SPINAL CORD 27
innervate skeletal muscle are referred to as lower motor Although the rubrospinal tract extends the length of
neurons. Lesion of the lateral corticospinal tract in the the spinal cord in most mammals, it only extends to the
spinal cord lateral funiculus results in an upper motor thoracic segments in man (20), is thinly myelinated, and
neuron syndrome which includes spasticity, hyperactive thought to be rudimentary. Any effect of injury to the
deep tendon reflexes, Babinski sign, clonus, and a loss or rubrospinal tract in patients will likely be masked by the
diminution of superficial reflexes, such as the abdominal severe motor deficits resulting from injury to the adjacent
or cremasteric reflex. In the acute phase of an SCI involv- lateral corticospinal tract.
ing bilateral lesion of the lateral corticospinal tracts, a
patient undergoes spinal shock, in which there is a
Lateral Vestibulospinal Tract
complete shutdown of neuronal activity in the spinal cord
below the level of the injury. The signs of spinal shock The neurons that give rise to this tract are found in the
include flaccid paralysis of muscles, hypotonia, and the lateral vestibular nucleus located at the junction between
absence of myotatic, bowel, and bladder reflexes. the medulla and pons. The axons of the lateral vestibu-
Depending on the level of injury, there may also be brady- lar nucleus descend uncrossed through the medulla and
cardia and significant lowering of blood pressure. Fol- form the lateral vestibulospinal tract in the anterior aspect
lowing a variable period (days to weeks) the patient of the lateral funiculus along the entire length of the spinal
recovers from spinal shock and the upper motor neuron cord (Figure 2.9). Fibers of the tract terminate mostly on
syndrome becomes apparent. The mechanisms underly- interneurons in laminae VII and VIII, but there are some
ing the induction and recovery from spinal shock are direct terminations on alpha motor neuron dendrites in
unknown. Lower motor neuron lesions result in signs the same laminae.
similar to those of a patient in spinal shock. In lower The primary function of the lateral vestibulospinal
motor neuron paralysis there is a loss of all movement, tract is to facilitate extensor muscle tone to maintain an
both reflex and voluntary, as well as a loss of muscle tone upright posture. With eyes closed and feet close together
and subsequent atrophy of the affected muscles. Unlike a normal individual sways slightly from side to side. Bal-
the transient deficits associated with spinal shock, lower ance is maintained because, for example, as the individ-
motor neuron deficits are permanent, assuming that there ual sways to the right impulses from the right semicircu-
is no reinnervation of the denervated structures. lar canals of the inner ear activate neurons in the
The signs associated with an upper motor neuron ipsilateral lateral vestibular nucleus. These neurons, in
syndrome are not always indicative of injury or disease turn, send impulses along the right vestibulospinal tract
of the spinal cord. In older individuals, there is a tendency to extensor muscles, which correct for the sway and move
for the superficial abdominal reflexes to be absent; this the body back to the midline center of gravity. When the
occurs more often in females than in males (18). Although individual sways to the left, the left lateral vestibulospinal
the Babinski sign is commonly associated with injury to tract is activated. Similarly, if a walking individual stum-
the corticospinal system, it can also be elicited in the new- bles, reflex extension of one of the lower extremities may
born infant, a sleeping or intoxicated adult, or following prevent a fall, but if a fall is imminent, extension of the
a generalized seizure. Interestingly, the Babinski sign may upper limbs often prevents severe injury to the face and
be absent in some patients with a known lesion of the cor- head. Under these circumstances, the reflex extension of
ticospinal tract (19). the limbs is also mediated by the lateral vestibulospinal
tracts. If the eighth cranial nerve (vestibulocochlear), lat-
eral vestibular nucleus, or the semicircular canals are
Rubrospinal Tract
injured on one side, a patient will often fall to that side
The neurons that give rise to the axons of the rubrospinal or veer to the side of the injury while walking. The effects
tract are located in the posterior two-thirds of the red of injury to the lateral vestibulospinal tract in the spinal
nucleus in the midbrain. The axons of the tract cross in cord, however, are generally masked by the more severe
the ventral tegmental decussation and descend to spinal deficits in motor capability that result from concomitant
levels, where the tract forms in the lateral funiculus mostly injury to the lateral corticospinal tract.
anterior to (partially overlapping) the lateral corticospinal
tract (Figure 2.9). The fibers of the rubrospinal tract ter-
Medial Vestibulospinal Tract
minate in the same laminae as the lateral corticospinal
tract and function to facilitate flexor motor neuron activ- The neurons that give rise to the medial vestibulospinal
ity. Because the red nucleus receives an input from the cor- tract are located in the medial vestibular nucleus of the
tex (corticorubral) and because of the similar termina- medulla. The fiber of these neurons descends through the
tions of both tracts in the spinal cord, the rubrospinal medulla bilaterally in a composite bundle of several dif-
tract is thought to be functionally related to the lateral ferent fiber systems known as the medial longitudinal fas-
corticospinal tract. ciculus (MLF). The MLF containing the medial vestibu-
28 INTRODUCTION
lospinal tract is located in the posterior aspect of the ante- turbances. If injury occurs at or above the T1 level,
rior funiculus of the cervical spinal cord (Figure 2.9). Horners Syndrome results because of injury to the sym-
Fibers of the medial vestibulospinal tract terminate on pathetic component of the descending autonomic path-
interneurons in laminae VII and VIII and play a role in ways. The signs of this syndrome are seen predominantly
the labyrinthine regulation of head positions. in the eye ipsilateral to injury and consist of miosis caused
by paralysis of the pupillary dilator muscle; slight ptosis
caused by paralysis of the smooth muscle (tarsal plate)
Reticulospinal Tracts
of the upper eyelid; and enophthalmos. The specific cause
The neurons that give rise to these tracts are located in of the enophthalmos is debated. Some suggest that the
the central core of the brainstem known as the reticular slight retraction of the eyeball into the orbit is caused by
formation, at the level of the pons and medulla. Because the paralysis of smooth muscles in the inferior orbital fis-
of the different origins and locations of these tracts in the sure, whereas others suggest that the enophthalmos is
spinal cord, they are often referred to separately as the caused by the interruption of sympathetic innervation to
pontine and medullary reticulospinal tracts. The pontine retro-orbital fat. Brodal (27) has argued that, because the
reticulospinal tract is mostly ipsilateral and descends in inferior orbital smooth muscles are so sparse, it is unlikely
the medial part of the anterior funiculus along the entire that they are responsible and that, because measurements
length of the spinal cord (Figure 2.9). The fibers termi- made with an exophthalmometer have indicated that the
nate in laminae VII and VIII. The medullary reticulospinal difference between the two sides is negligible, the enoph-
tract is also primarily ipsilateral and descends the length thalmos may simply be caused by the ptosis. In addition
of the spinal cord in the anterior part of the lateral funicu- to the signs in the eye, the patient may have anhydrosis
lus (Figure 2.9). The fibers of this pathway terminate in of the face because of the interruption of the sympathetic
laminae VII and VIII in close association with the termi- innervation of the sweat glands of the face ipsilateral to
nation of the fibers of the pontine reticulospinal tract, the the spinal cord injury.
rubrospinal tract, and the corticospinal tracts. When the descending autonomic pathways inner-
Animal studies have shown that stimulation of the vating preganglionic parasympathetic neurons are
brainstem reticular formation can facilitate or inhibit vol- lesioned bilaterally at any level of the spinal cord rostral
untary movement, cortically induced movement and to S2, the result is impotence and a loss of normal bowel
reflex activity; influence muscle tone; affect inspiratory and bladder function. However, after recovery from
phases of respiration; exert pressor or depressor affects spinal shock, spontaneous or reflex erection of the penis
on the circulatory system; and exert inhibitory effects on (or clitoris) may occur and bowel and bladder reflexes
sensory transmission (21). Those areas of the medullary may also return. This is in marked contrast to the deficits
reticular formation giving rise to the medullary reticu- associated with the conus medullaris and cauda equina
lospinal tract correspond closely with the regions from syndromes explained earlier in this chapter. In these lat-
which inspiratory, inhibitory, and depressor effects are ter cases, the preganglionic parasympathetic neurons at
obtained (2224). The areas of the reticular formation S2S4 are destroyed or their axons are severed; this usu-
related to facilitatory effects, expiration, and pressor ally results in a permanently areflexic bowel and bladder
vasomotor responses are rostral to the medulla and and the absence of spontaneous or reflex erections.
extend beyond the regions that give rise to reticulospinal In contrast to erection, ejaculation is controlled by
fibers (25). Thus, the reticulospinal tracts may not be the preganglionic sympathetic neurons located at the L1 and
mediators of some facilitatory effects originating from L2 levels of the spinal cord. During ejaculation, the sem-
neurons in the upper brainstem reticular formation. inal fluid from the seminal vesicles and prostate, as well
as sperm from each epididymis, flows into the prostatic
urethra and is ejected from the penis by rhythmic con-
Descending Autonomic Pathways
tractions of the bulbospongiosus muscle compressing the
Fibers belonging to this important descending system orig- urethra. This muscle is innervated by the somatic alpha
inate primarily from the hypothalamus. Although there is motor neurons located at the S2S4 levels of the spinal
evidence of direct projections from the hypothalamus to the cord via the pudendal nerves. Thus, erection is controlled
spinal cord (26), polysynaptic routes also pass through var- by parasympathetics and ejaculation is mediated by both
ious regions of the reticular formation before reaching the sympathetic and somatic neurons. During ejaculation,
spinal cord. The descending autonomic pathways are discharge of the semen into the bladder is prevented by
located predominantly in the lateral funiculi and terminate the contraction of the sphincter vesicae, which is inner-
on the preganglionic sympathetic and parasympathetic neu- vated by preganglionic sympathetics located at L1 and
rons located in lamina VII of T1L2 and S2S4 respectively. L2. When there is bilateral destruction of the descending
Lesion of the descending autonomic pathways in autonomic pathways in SCI, there is not only a loss of
spinal cord injury often leads to significant autonomic dis- erection, but also a loss of ejaculation. If the SCI is above
ANATOMY AND FUNCTION OF THE SPINAL CORD 29
FIGURE 2.10
FIGURE 2.11
FIGURE 2.13
Diagram showing the extent of the lesion in anterior cord (spinal artery) syndrome and associated neurologic signs.
32 INTRODUCTION
FIGURE 2.14
Collateral circulation of the spinal cord. When posterior intercostals are tied off surgically, blood flows to the spinal cord via
internal thoracic and lateral thoracic (not shown) artery anastomoses with posterior intercostal arteries.
FIGURE 2.15
blocks transmission of sound waves. However, ultra- ter not only by its density (as does X-ray and CT), but
sound is a portable, noninvasive, and relatively inexpen- also by its different T1 and T2 relaxation parameters, is
sive modality that demonstrates good soft tissue contrast. of unparalleled value in differentiating between soft tis-
It is useful when the bony spinal elements are absent, such sues, such as gray and white matter, and normal vs. ede-
as during intraoperative visualization of the spinal cord matous tissue.
after the bony laminae have been surgically removed, or
in early infancy when spinal ossification is limited.
Imaging Speed
It has been little more than 15 years since the intro-
duction of MRI to spinal imaging. Although it is more Imaging speed is another important parameter, in addi-
expensive than some of the techniques listed above, tion to the three listed above. Unlike those prior three
because of its noninvasive nature (no ionizing radiation), parameters of image quality, which can be measured or
multiplanar imaging capabilities, superb soft tissue con- calculated directly from the image itself, the time required
spicuity, and ability to depict the spinal neural contents to produce the image cannot (although it is measurable
directly, MRI has established itself as the imaging modal- by other means). The imaging speed (i.e., the time
ity of choice for the spine and spinal cord (1). In certain required to obtain the image) is one of the most impor-
situations, however, one or more of the other modalities tant benchmarks of an imaging system, and this is where
discussed above may be more useful than MRI. many of the improvements in MRI (and indeed all med-
ical imaging) are being made.
Some portions of the human anatomy impose strin-
Basic Concepts of Imaging
gent requirements on imaging speed, most notably the
Three major parameters that characterize an imaging beating heart or respiratory motion of the thorax, but the
modality are spatial resolution, signal-to-noise (S/N), and spine in a supine patient is relatively motion-free and con-
contrast resolution (1). Any improvement in image qual- siderably less demanding in this regard. However, both in
ity represents some combination of improved spatial res- the interests of patient comfort and to avoid degradation
olution, increased S/N, and improved contrast resolution. by patient motion (especially in those patients with severe
Spatial resolution, essentially the smallest size back pain), it is desirable to minimize imaging time.
detectable by the technique, is determined by slice thick- Moreover, significant increases in imaging speed can yield
ness, field of view (FOV), and the size of the acquisition major improvements in signal-to-noise, as discussed in the
and display matrices. In a digital system such as MRI, section on fast spin-echo (FSE), also known as the rapid
the unit of in-plane spatial resolution (defined as a pixel) acquisition relaxation enhanced (RARE) sequence.
is the FOV divided by the matrix size. Thus, using a field As has historically occurred with all imaging modal-
of view of 48 cm (which is roughly the length of the spinal ities, including CT, improvements in image quality have
cord), and a 512 512 matrix size yields a nominal in- been accompanied by increases in imaging speed. Faster
plane spatial resolution of 0.94 mm, certainly adequate and more powerful computers, better reconstruction
for imaging spinal structures. Ideally, to achieve good spa- algorithms, and hardware improvements have all resulted
tial resolution, thin sections (3 mm) with a large matrix in these gains.
(256 256 or up to 512 512) should be used, but
unfortunately, as discussed in the following paragraph,
Phased-array Surface Coils
there is a trade-off between improving spatial resolution
and worsening signal-to-noise. Improved spatial resolution in spine imaging has long
Signal-to-noise (S/N), as the name implies, is the ratio been achieved with the use of surface coils, with their
of the desired signal to be measured, divided by the higher signal-to-noise ratio, but with a penalty of a
inevitable noise that degrades and contaminates the signal. smaller field of view and a limited penetration into the
Because the MRI signal is proportional to the number of body (2,3). Fortunately, though, the spinal structures lie
protons in the imaged volume element (voxel), increasing close to the posterior skin surface, so that virtually all
the volume sampled (i.e., increasing voxel and pixel size) modern spine MRI uses posterior surface coils to receive
increases the signal (more than the noise). However, the MR signals.
increasing pixel size means decreasing spatial resolution. The recent development of the phased-array surface
However, an intrinsic increase in S/N, such as from coil has solved the problem of limited longitudinal field-
increasing MRI magnet strength, or from improvements of-view by coupling many (4 to 6) such coils in a longi-
in coil technology, is extremely beneficial, not only tudinal array (4), mounted as a single long unit. This
because S/N is increased, but also because it allows for allows nearly the entire spine (48 cm) to be imaged in a
smaller pixel size and hence improved spatial resolution. single acquisition using a large field of view (FOV), with
Contrast resolution is the ability to discriminate good resolution (using a 512 512 or 512 384
between different tissues. MRI, which distinguishes mat- matrix), rather than requiring separate, time-consuming
IMAGING OF THE SPINAL CORD 37
imaging of the cervical, thoracic, and lumbar regions indi- through the levels of abnormality, rather than through the
vidually (Figure 3.1). Various combinations of the phased entire spine. A large FOV fast spin-echo (FSE) (discussed
coils may be electronically activated to view only the cer- below) T2-weighted sagittal image may be obtained in
vical, thoracic, or lumbar regions, or a combination about 3 to 6 minutes to clearly delineate regions of cord
thereof, without physically moving the coil or the patient. impingement (Figure 3.2.) The total imaging time for this
This allows screening studies of the entire spine to study is thus only about 20 minutes, which can generally
be made in one acquisition, rather than treating the spine be tolerated by almost any patient.
as three separate units (cervical, thoracic, and lumbar). Also, screening for intrathecal disease throughout the
It is particularly valuable in screening for metastatic dis- spine can be performed with a single postgadolinium sagit-
ease; a screening 3-mm series of T1-weighted sagittal
images of essentially the entire spine may be performed
in a single 3- to 7-minute sequence, and selected axial
images (T1 or fast spin-echo T2) should be obtained only
FIGURE 3.2
tal sequence in about 5 minutes, with a resolution of 0.9 al. (14), the RARE sequence and variations thereupon, have
mm (48 cm FOV / 512 matrix), using selected axial images been a major development in fast-scanning, particularly
only through the regions of abnormal enhancement. useful in spine MRI. This sequence, also known as fast-
spin-echo (FSE) or turbo-spin-echo, gives good T2-
weighted contrast in a fraction of the time required by true
FAST-SCANNING TECHNIQUES: RATIONALE (conventional) spin-echo, thus resulting in better resolution
and signal-to-noise than previously practically attainable
To improve the signal-to-noise ratio (S/N), one may (Figure 3.2). (FSE generally requires only a few minutes per
increase the signal by increasing the number of acquisitions sequence, rather than about 20 minutes for an equivalent
(i.e., the number of times the image data is acquired; in multiexcitation true T2-weighted acquisition.)
effect, scanning the patient multiple times), but this obvi- This sequence places several (from 3 to 16 or more)
ously significantly lengthens the exam and increases the different phase-encoding gradients followed by 180-
risk of image degradation from patient motion. However, degree radio frequency pulses after each 90-degree pulse,
if an imaging sequence is intrinsically very fast, doubling rather just one phase-encode and one or two 180-degree
or tripling the (very short) imaging time is a feasible option, pulses. This results in a much more efficient use of imag-
resulting in significant improvement in S/N and conse- ing time overall, cutting scanning time by a factor of 2
quently allowing smaller pixels (i.e., better spatial resolu- to 6 (or more).
tion). Hence, recent developments have focused on FSE images demonstrate less magnetic susceptibility
decreasing scan timea technique known as fast-scanning. artifact than true spin-echo images (15,16), which mini-
mizes artifacts from bone spurs, and (to a certain degree)
reduces artifact from surgical hardware (17) (See Figure
Gradient-echo Imaging
3.10). Possible disadvantages of FSE include bright signal
Many fast-scanning methods have been developed, of from fat and decreased sensitivity to hemosiderin (old blood
which gradient-echo imaging (GRE) was one of the first. products), as well as some blurring of images may occa-
This technique relies on gradients to refocus the spins, sionally be significant (14,15,16,18). One major artifact on
rather than 180-degree pulses, as are used in conventional FSE imaging is caused by CSF pulsation in the rostral
spin-echo imaging. Images are produced rapidly because regions of the spine, which yields signal voids that could
of small flip angles and short repetition times (TRs) (5,6). be misinterpreted as arteriovenous fistulas or AVMs in the
Its major advantage, besides speed, is the very thin slices CSF dorsal to the cord, especially on FSE images (15,17)
obtainable (less than 1mm thick), representing multiple
thin partitions of a large three-dimensional (3-D) imag-
ing volume, in contrast to the usual 3-mm minimum slice
thickness attainable with conventional 2-D spin-echo
slices. However, the signal characteristics of gradient-echo
images do not correspond directly to conventional T1- or
T2-weighted spin-echo imaging (7). In particular, GRE
images of the spine are inferior to conventional T2-
weighted or fast spin-echo images, because they consis-
tently overestimate the hydration of the disks, overesti-
mate the size of osteophytes because of susceptibility
artifact, and provide poor visualization of the neural
structures (7,8). Also, because of the lack of the 180-
degree refocusing pulse, gradient-echo scans are more
prone to susceptibility artifacts from tissue and magnetic
field inhomogeneity, and from motion (9).
In the spine, gradient-echo imaging currently has its
most widespread application in 3D-volumetric thin-sec-
tion axial images of the cervical spine, where the relatively
small size of the anatomic structures necessitates con-
tiguous thin-section images (10,11).
FIGURE 3.3
Fast Spin-echo (FSE or RARE) Imaging Consecutive sagittal FSE T2-weighted images of the thoracic
spine show multiple focal signal voids in the CSF posterior to the
Developed in 1990 by Melki and Mulkern et al. (12,13) spinal cord. These are common artifacts on this pulse sequence
as a refinement of a technique first proposed by Hennig et and must not be misinterpreted as a vascular malformation.
IMAGING OF THE SPINAL CORD 39
A B
FIGURE 3.5
Three-dimensional fast spin-echo (3D FSE-T2) of cervical spine. A. 1.2-mm-thick sagittal fast spin-echo T2-weighted image
was obtained as part of a 3-D volume acquisition, with forty-eight slices in 7.5 minutes. Note the clear delineation between the
vertebrae, CSF, and cord in this 36-year-old woman with prior C4-5 fusion. B. 0.9-mm-thick axial T2-weighted image was recon-
structed from the sagittal data acquired and shown in A. No axial images were directly acquired. Hence, both sagittal and axial
views can be obtained in a single 7.5-minute acquisition, with 1.2-mm resolution. Note the clear delineation of the spinal cord
and neural foramina on this reconstructed image (1).
In the lumbar spine, T1 and multiecho FSE T2- nerve roots in the neural foramina, and the surrounding
weighted images should be obtained in the sagittal plane, epidural fat. However, there are two major circumstances
followed by axial T1 and FSE T2-images. in which the presence of bright fat can mask pathology:
As discussed, the best technique for evaluating a) Enhancing structures on post-gadolinium T1-weighted
intrinsic spinal cord lesions includes sagittal FSE proton- sequences may be masked by surrounding T1-bright fat,
density and T2-weighted images, supplemented with and b) Intrinsically T2-bright lesions in the vertebral mar-
sagittal fast-STIR images. T1-weighted images after intra- row, such as metastases, may be masked by T2-bright fat
venous administration of Gd-DTPA are necessary to eval- on FSE images.
uate intradural-extramedullary or intramedullary lesions. In both these situations, suppression of the bright fat
signal by special pulse sequences enables the enhancement
or the T2-bright signal of lesions to be visualized; this may
Fat Saturation
provide better conspicuity of inflammatory and neoplas-
Fat is bright on T1-weighted images and fairly bright on tic lesions in the spine (18,26,27). Short inversiontime
FSE-T2 images. This can be advantageous, providing nat- inversion-recovery (STIR) is another method of fat sup-
ural contrast between the T1-dark CSF in the thecal sac, pression that may be especially useful in visualizing ver-
IMAGING OF THE SPINAL CORD 41
FIGURE 3.6
Sagittal MR images of a 58-year-old man with multiple sclerosis. Left. FSE proton-density image clearly demonstrates a small,
ovoid, bright plaque in the distal conus. Middle. FSE T2-weighted image only subtly demonstrates the lesion. Right. Fast-
STIR image subtly demonstrates the plaque slightly better than FSE-T2, but not as well as FSE proton-density. Note the dark
appearance of all the bony and soft tissue structures on the fast-STIR image, with only CSF and lesion hyperintense.
Summary
There have been many recent developments in the fast-
growing field of spinal MRI. Some (fast imaging, espe-
cially FSE) result in faster intrinsic imaging times, with
the added benefit of improved signal-to-noise and spa-
tial resolution. Others (phased-array coil and variable
bandwidth) yield improved signal-to-noise and secon-
darily result in faster imaging and improved patient com-
fort. Still others (fat saturation, and use of titanium instru-
mentation) improve contrast resolution or otherwise
improve image quality.
All these developments are undergoing continual
refinement, and advances in the future will continue to
improve image quality, imaging speed, and ultimately, the
quality of patient care.
CONGENITAL ABNORMALITIES
FIGURE 3.10
B Titanium hardware minimizes artifact on MRI. Axial fast spin-
echo (FSE) T2-weighted image of vertebra at level of bilat-
eral titanium pedicle screws shows only minimal suscepti-
bility artifact in the vicinity of the screws. The central canal
is still clearly visualized, as are the individual nerve roots.
Stainless steel hardware would have rendered this level unin-
terpretable. Use of fast spin-echo sequence also contributes
to minimizing artifact (1).
Myelomeningocele. Sagittal T1-weighted MR image of 6-year- Chiari II and spinal cord atrophy. Sagittal T1-weighted MR
old child with symptoms of tethered cord and previous image of a 6-year-old child with myelomeningocele shows
surgery for myelomeningocele. The spinal cord extends infe- downward displacement of the cerebellum far down into the
riorly into the meningocele at the level of the sacrum. cervical spinal canal. The cervical spinal cord is atrophied.
lipoma can be demonstrated by CT but is especially well subpial collection of fat, nearly always along the dorsal
shown by MRI (Figure 3.13). Low attenuation on CT and surface of the spinal cord (Figure 3.15).
similar signal intensity to fat elsewhere on MRI are spe- A focal failure of disjunction of the spinal cord from
cific for lipomas in the spinal canal. The position of the dis- the overlying ectoderm leads to the formation of a dor-
tal spinal cord is best demonstrated by MRI. Spina bifida sal dermal sinus tract, lined by epithelial tissue. When a
occulta is seen at the site of communication with subcuta- dimple, patch of hair, or pigmented skin leads to suspi-
neous fat. Lipomyelomeningocele is not associated with the cion of such a tract, MRI can demonstrate the tract. Such
Chiari II malformation, and the incidence of hydrocephalus tracts often extend obliquely through the subcutaneous
in these patients is similar to that of the general population. tissue. They may terminate anywhere from subcutaneous
A lipoma of the filum terminale may be associated tissue to epidural space, to dura, to the spinal cord itself.
with a tethered cord (Figure 3.14). Very small amounts In about half of dorsal dermal sinus tracts, a dermoid or
of fat within the filum terminale are commonly seen and epidermoid cyst is present. MRI shows such tumors or
are of doubtful significance. The lipoma of the spinal cord cysts as round or oval masses in the spinal canal and can
is the least common of the spinal lipomas. It is actually a show their relationship to the spinal cord.
IMAGING OF THE SPINAL CORD 45
A B
FIGURE 3.13
The spinal cord can be tethered from a variety of mentally divided in the sagittal plane, and the dural sac
causes. Myelomeningocele and lipomyelomeningocele may be intact or may also be divided. In about half of
both imply that the spinal cord is tethered. However, these cases there is a bony or cartilaginous septum
errors in a later stage of development relating to the cau- through the spinal canal. Such septa are best demon-
dal cell mass can lead to a tethered cord without the more strated by CT but may be shown by MRI, especially with
obvious abnormalities of the spinal cord and vertebra. gradient-echo techniques. The split spinal cord and the
The tip of the conus medullaris lies at the L2 level or length of the division are best shown by MRI.
above in 98 percent of people, and position of the conus
medullaris at or below the L23 level is generally con-
Trauma
sidered abnormally low (37). Sagittal MR images alone
can sometimes be confusing, because the nerve roots in Imaging plays a key role in the evaluation of spine
the cauda equina may simulate the conus medullaris; trauma, both in the acute situation and in the later imag-
additional coronal or axial images are often necessary to ing of complications. The complementary nature of var-
accurately image the distal spinal cord. T1-weighted ious imaging modalities is especially important in imag-
images are often most reliable to identify the conus ing spine trauma.
medullaris. As mentioned above, sonography is only pos- Plain radiographs are part of the routine evaluation
sible early in life but has the advantage of providing of patients in whom there is a strong suspicion of spine
dynamic information. injury. Radiographic evaluation of the cervical spine is
Diastematomyelia is a rare congenital malformation recommended for all patients with evidence of significant
resulting from a split notochord. The spinal cord is seg- injury above the clavicles. There is no consensus on which
46 INTRODUCTION
FIGURE 3.15
A C
FIGURE 3.17
combination with extension causes pillar or lateral mass Combined fractures of the pedicle and lamina result in a
fractures, which can be especially difficult to demonstrate separation of the lateral mass and risk of rotational insta-
on plain films. We have found that MRI can be helpful bility (Figure 3.20).
with these posterior-element fractures to demonstrate the Injuries resulting primarily from axial loading mech-
extent of accompanying ligamentous injury, which may anisms include the Jefferson burst fracture of C1, burst
be an important factor in determining instability (45). fractures of C3 through C7, burst fractures involving the
More extensive ligamentous injury probably indicates a thoracic or lumbar spine, and oblique sagittal fractures
greater risk of instability in these lateral mass fractures. (type 2) of the C2 body. The Jefferson fracture pattern
IMAGING OF THE SPINAL CORD 49
FIGURE 3.18
FIGURE 3.20
FIGURE 3.22
FIGURE 3.23
A B
FIGURE 3.24
Atlanto-occipital dissociation. MRI was performed on a patient who was in a high-speed motor vehicle accident. Midline sagit-
tal T2-weighted inversion recovery image (A) shows high signal intensity of severe anterior and posterior paraspinal soft tissue
edema at the cervicocranial junction. Heterogeneous signal intensity within the spinal canal reflects some blood present.
Parasagittal image (B) shows subluxation of the atlanto-occipital joint, with high signal intensity fluid between the occipital
condyle and lateral mass of C1 (arrow).
52 INTRODUCTION
get-film distance. The distance from basion perpendicu- very helpful for planning surgical intervention. MRI is the
larly to a line extended rostrally from the posterior cor- best imaging method to evaluate the spinal cord (Figure
tical margin of the body of C2 (basionaxial interval or 3.26). Finally, in any injury in which the surgical
BAI) measured on a lateral radiograph should not exceed approach may be affected by the location of the artery
12 mm in children or adults. MRI can more directly of Adamkiewicz, spinal angiography may be indicated.
demonstrate ligamentous disruption (Figure 3.24). A combination of flexion and distraction results in
Injury patterns in the thoracic and lumbar spine, in a distinctive type of injury, usually in the upper lumbar or
which motion is more restricted, are somewhat more lim- lower thoracic spine. The classic Chance fracture extends
ited than those in the cervical spine. Compression frac- horizontally through the vertebral body and through both
tures are common injuries that result in loss of height of pedicles, and may occur in automobile accidents when the
the vertebral body, anteriorly more so than posteriorly. In patient is wearing a lap belt but not a shoulder harness.
mild cases, radiographic findings include deformity of the It is often best demonstrated on plain radiographs,
cortical margins and end-plates. Axial loading can cause because axial CT is less well suited to identifying axially
a burst fracture, in which the force transmitted through oriented fractures (see Figure 3.16). However, other pat-
the intervertebral disk results in bursting of the centrum terns of flexion-distraction injuries can also occur, still
below. Posterior element fractures are usually present, with an axial orientation and posterior distraction. In
there is nearly always a prominent sagittal component of these injuries, shearing may occur through the disk space,
the vertebral body fractures, and displacement into the with posterior subluxation or dislocation of the facet
spinal canal is variable. CT is usually helpful to define the joints (50,51).
extent of fractures and posterior element involvement In addition to mechanical narrowing of the spinal
(Figure 3.25). Fracture-dislocations involve complex, canal by displaced bone, the spinal cord can be compro-
severe forces, variable amounts of dislocation of one ver-
tebra on another, and usually multiple fractures. Neuro-
logic deficits from spinal cord compression are common.
Multiple imaging modalities are often helpful in man-
agement. The dislocation is usually initially demonstrated
on plain radiographs, which are excellent for demon-
strating alignment. CT of the affected vertebrae can be
Burst fracture of T12 suffered in a parachuting accident. The Thoracic fracture-dislocation. Sagittal T2-weighted inversion
patient had incomplete paraplegia. Axial CT shows numer- recovery image of a young adult who suffered severe T1011
ous fractures in the body of T12, with posterior displacement fracture-dislocation in a logging accident shows dislocation
of fragments into the spinal canal. and spinal cord transection.
IMAGING OF THE SPINAL CORD 53
FIGURE 3.28
A B
FIGURE 3.29
A B
FIGURE 3.30
Sixty-seven-year-old man with multiple thoracic disc protrusions. A. Axial image from postmyelogram CT shows a broadbased
central disc protrusion flattening the ventral thecal sac, compressing the spinal cord (black arrow), which is outlined by white
contrast-laden CSF. B. Sagittal reconstruction from the axial CT data shows two thoracic disc protrusions (white arrows) effac-
ing the ventral CSF; the lower protrusion corresponds to A.,, slightly compressing the spinal cord.
IMAGING OF THE SPINAL CORD 55
A B
FIGURE 3.32
Cervical spinal cord compression and gliosis caused by large disc protrusions in a 62-year-old man. Sagittal (A) T1-weighted and (B)
T2-weighted MR images show large disc protrusions at essentially all cervical levels, with spinal cord compression and flattening at
C34 and C56. A small focus of T2-bright gliosis is present within the spinal cord (B) associated with the compression at C34.
56 INTRODUCTION
Postoperative Complications
Postoperative complications include postoperative
hematomas, pseudomeningocele, infection, recurrent disc
herniation, and arachnoiditis (60). Recurrent disc herni-
ation can be distinguished from postoperative scar tissue
in that, unlike normal scar tissue, recurrent disc hernia-
tions do not enhance with IV gadolinium-DTPA (61).
Arachnoiditis has many causes, but may be associ-
ated with a history of pantopaque myelography or spinal
surgery. On MRI, the nerve roots are thickened and
clumped, and exhibit variable enhancement. They may
adhere to the periphery of the thecal sac. Intrathecal
arachnoid bands or cysts may be present (Figure 3.34).
Another postoperative complication is improperly
positioned spinal hardware. As discussed earlier, titanium
hardware is better visualized on CT and MRI than stain-
less steel (Figures 3.8, 3.9, 3.10). Occasionally, CT
demonstrates metal hardware directly impinging on
spinal nerve roots (Figure 3.35).
FIGURE 3.35
SPINAL INFECTIONS
Sixty-four-year-old man with left S1 radiculopathy after spinal
surgery. Axial image from postmyelogram CT shows the left
pedicle screw incorrectly positioned, within the left S1 neural The incidence of central nervous system (CNS) infections,
canal, obliterating the left S1 nerve root and correlating with including spinal involvement, has increased in recent
the patients symptoms. The right pedicle screw is malposi- decades, largely because of AIDS (67). The most common
tioned medial to the right S1 neural canal. The black arrow site of spinal infection is the vertebral bodies and discs, which
points to the right S1 nerve root in its neural canal. can then exert mass effect on the thecal sac, spinal cord, and
58 INTRODUCTION
FIGURE 3.37
FIGURE 3.40
A B
FIGURE 3.41
FIGURE 3.42
Pyogenic spinal infection in an adult male with L5S1 diskitis, osteomyelitis, and epidural abscess. Left. Sagittal T2-weighted
image shows T2-bright pus within the L5S1 disc, extending posteriorly. T2-bright edema or infection is noted involving the
adjacent endplates. Middle. T1-weighted image better shows the associated epidural abscess compressing the distal thecal
sac. Right. Postcontrast T1-weighted image again shows enhancing phlegmonous tissue compressing the anterior aspect of the
thecal sac. T1-dark pus is noted centrally in the epidural abscess (arrows).
IMAGING OF THE SPINAL CORD 61
Epidural abscesses are often found in association Brucellosis presents with bony lytic lesions of the
with bony osteomyelitis and discitis (Figure 3.42), but vertebral body, usually in the lower lumbar spine (72,73).
also may be an isolated finding (Figure 3.43). Again, MRI Tuberculosis (74) differs from pyogenic bacterial
is the imaging modality of choice, especially with the use infections in that the intervertebral disc is often relatively
of intravenous gadolinium-DTPA. Staphylococcus aureus spared in early disease (possibly because mycobacteria
is the most common causative organism (in about 60 per- lack proteolytic enzymes). It appears without abnormal
cent of cases). Another 13 percent of cases are caused by T2-bright signal, more involvement of posterior aspects
other gram-positive cocci, and 15 percent are caused by of the vertebrae, involvement of more than two vertebral
gram-negative organisms (67,70,71). Most epidural bodies, and larger associated paraspinal masses. CT may
abscesses enhance homogeneously with IV Gd-DTPA, show the small soft-tissue calcifications suggestive of
suggesting that they are largely phlegmonous, but nonen- tuberculosis, which are not reliably shown on MRI (75).
hancing frank pus may also be recognized as a central col- Chronic tuberculosis infection may result in a classic
lection of fluidlike T1-dark, T2-bright signal surrounded kyphotic deformity, known as Potts disease, usually in
by an enhancing soft-tissue rim (Figures 3.42 and 3.43). the mid-thoracic region.
Patients with AIDS may often present with
polyradiculopathy caused by viruses, most commonly
cytomegalovirus (76). Gadolinium-enhanced MRI is by
far the best modality to visualize the enhancing nerve
roots of the cauda equina, which may appear normal on
standard T1- and T2-weighted images; occasionally
enhancement extends diffusely throughout the spinal sub-
arachnoid space (Figure 3.44). Infection of the spinal cord
by the HIV virus has been found to cause vacuolar
myelopathy in AIDS patients. Spinal MRI may be nor-
mal, but usually presents with spinal cord atrophy or
occasionally with T2-bright signal (77). Enhancement
with IV contrast is not generally seen in AIDS-associated
myelopathy (77).
FIGURE 3.44
Thirty-one-year-old man with AIDS, who presented with bilateral leg pain and weakness and loss of bowel and bladder control.
Left. Sagittal T1-weighted MR images of the cervical spine are normal, as are sagittal T2-weighted images, Right. (Patient is
angulated on the T2-weighted images; the lower spinal cord is out of view so that CSF, rather than cord, is imaged; note that
spinous processes are also out of field.) Middle. However, T1-weighted images after IV administration of gadolinium-DTPA
show diffuse marked enhancement of inflammatory tissue throughout the spinal subarachnoid space (white and black arrows).
Herpes simplex II virus was cultured from his CSF, and cytomegalovirus was cultured from his blood.
ance of hemorrhage are not reviewed here. It is important tral nidus in the case of AVMs, or the location of the direct
to consider the effect of MRI technique on the appear- fistulous connection in fistulas (Figure 3.47B); it also
ance of hemorrhage. Fast spin-echo techniques, which are shows the extent and direction of venous drainage.
widely used for spine imaging because of the advantages Depending on the nature and location of the malforma-
in acquisition time and artifact reduction, also reduce the tion, endovascular therapy, such as embolization, can be
susceptibility changes of blood. This can make blood less used either alone or in combination with surgery to treat
conspicuous with fast spin-echo techniques. Gradient- the condition. In some cases of suspected fistula, an
echo techniques, on the other hand, exaggerate magnetic exhaustive search of spinal arteries may be necessary,
susceptibility differences between blood and surrounding from the lowest lumbosacral branches up through the ver-
tissues. Although this can increase some artifacts, it has tebral, cervical, and external carotid arteries.
the potential advantage of making hemorrhage more con- Cavernous malformations can occur in the spinal
spicuous. cord as well in the brain, and the imaging characteristics
The differential diagnosis of hemorrhage within the are similar. Typically, blood products of varying age are
spinal cord includes trauma (which would usually be present with a surrounding rim of dark T2-signal inten-
obvious by history), vascular malformation, and neo- sity from hemosiderin. Larger lesions often have a retic-
plasm. The presence of multiple areas of flow void on ulated appearance because they contain pockets of methe-
MRI provides additional evidence of a high-flow vascu- moglobin, but small lesions may be seen only as
lar malformation. These may be present within the spinal hemosiderin scars. Acute hemorrhage within a cavernous
cord in the case of intramedullary AVMs (Figure 3.46), malformation has a less-specific appearance unless the
or on the surface of the spinal cord or more peripherally surrounding older blood products are visible.
in the case of AV fistulas (Figure 3.47). Tortuous drain-
ing veins are usually more conspicuous than the feeding
arteries. Superficial vessels, especially enlarged draining SPINAL TUMORS
veins, can be shown on myelography.
Angiography plays an important role in the diag- An estimated one in four or five central nervous system
nosis of spinal vascular malformations. It defines the loca- tumors are located in the spine (79,80,81). A study by
tion and number of feeding arteries, the size of the cen- Kurland (82) estimated the incidence of primary spine
IMAGING OF THE SPINAL CORD 63
FIGURE 3.46
FIGURE 3.45
Intramedullary spinal arteriovenous malformation (AVM) in
Spinal cord infarct in a 68-year-old man, sustained during sur- a 29-year-old man presenting with hemiparesis and difficulty
gical repair of abdominal aortic aneurysm. Sagittal T2-weighted breathing. Sagittal T2-weighted MRI image shows diffuse
image shows T2-bright edema (white arrows) within the distal swelling and edema throughout the cervical spinal cord. Ser-
thoracic spinal cord and conus, with small amount of dark piginous linear flow voids of the intramedullary AVM and
intramedullary hemorrhage (black arrowheads) distally. its draining vein extend from the C4 to C7 levels (small
white arrows). The AVM was successfully endovascularly
embolized via a right vertebral artery approach.
A B
FIGURE 3.47
Spinal-dural arteriovenous fistula in a 42-year-old man presenting with paraplegia. A. Sagittal T2-weighted MRI image shows
swelling and edema (white arrows) within the mid-lower thoracic spinal cord and multiple serpiginous flow-voids of an abnor-
mal draining vein immediately dorsal to the spinal cord. B. Spinal angiogram with injection at the T7 level shows an abnormal
fistulous connection (black arrowhead) between the radicular artery (white arrow) and the abnormal tortuous, dilated draining
vein coursing superiorly. The patients symptoms resolved within 2 minutes of endovascular embolization with glue.
available with fast spin-echo, and motion artifacts can between soft tissue or tumor and CSF is poorer than on
be a problem for those inexperienced in reading FSE true-T2 or FSE-T2 images. GRE images are only useful
images. for detecting small amounts of hemosiderin or calcium,
The use of phased-array coils, if available, is which could be missed on FSE images and may be missed
extremely valuable in obtaining better signal-to-noise and on SE T2-weighted images.
obviating the need to move the coil or the patient when Spine tumors are generally classified according to
covering a large portion of the spine (as in screening for anatomic location as extradural; intradural, extramedullary;
epidural cord compression). or intramedullary.
In addition to T1-weighted images, inversion-recov-
ery (IR) (or preferably, FSE IR) sequences also demonstrate
Extradural Tumors
vertebral marrow metastases by nulling the neighboring
marrow fat (28, 29). Inversion-recovery images, like T2- These account for about a third of all spine neoplasms
weighted images, clearly demonstrate intramedullary and generally involve the vertebrae (79). The majority are
edema or tumors as bright signal. metastases to bone. Metastases to the epidural space are
Coronal imaging may be useful for example in imag- less common, and even less common are tumors (benign
ing cervicothoracic neurofibromas. or malignant) arising from the osseous or notochordal
Gradient-echo (GRE) images are generally not use- structures of the spine. A small fraction of neurofibromas
ful in the imaging of spinal tumors. The differentiation and meningiomas are completely extradural.
IMAGING OF THE SPINAL CORD 65
Extradural Malignancy
Metastases to bone are by far the most common malig-
nant tumors involving the spine encountered in every-
day practice. Often the site of disease cannot be accu-
rately localized clinically. Hence, it is important to be able
to quickly and efficiently screen the entire spine in these
patients. It is impractical and unnecessary to obtain sagit-
tal and axial T1- and T2-weighted images, as well as pre-
and postgadolinium images through all levels of the
spine. These patients often suffer excruciating pain and
are unable to lie motionless for even moderate lengths
of time.
Instead, the screening examination should consist of
first, a single T1-weighted sequence using a large field of
view (4850 cm) with a large matrix (512 512 or 512
384) in the sagittal plane. This will cover essentially the
entire spine in a single acquisition of about 6 minutes or
less (Figure 3.48). A fast spin-echo T2-weighted (FSE-T2)
sagittal screening sequence can give a myelogram-effect
scan in another few minutes, pointing out regions of CSF
effacement by tumor. Then, 4-mm axial images (either
T1- or fast spin-echo T2) may be obtained only at the lev-
els of cord compression, to better delineate the degree of B
compression. Gadolinium is generally not needed to diag-
nose and evaluate cord compression from bony vertebral
metastases. Hence, a complete examination can be
obtained in about 20 minutes, which can be tolerated by
almost all patients.
Good analgesia, such as morphine, is important to
aid patient comfort and prevent image degradation by
motion. Anxiolytics such as diazepam (Valium) or mida-
zolam are less valuable; these patients move around in
the scanner more from pain than from claustrophobia
or anxiety.
Phased-array coils, if available, give superb signal-
to-noise and enable complete spinal coverage without
moving the patient or the coil. However, lacking a phased-
array coil, the body coil should be used to screen the entire FIGURE 3.48
spine using the single large FOV T1-weighted sagittal
sequence. Use of a conventional license-plate coil to ini- Metastatic bladder rhabdomyosarcoma in a 6-year-old girl.
A. Sagittal T1-weighted large field-of-view image of the entire
tially separately screen the cervical spine, the thoracic
spine obtained in only 5 minutes shows the regions of verte-
spine, and the lumbar spine, using both T1- and T2- bral tumor involvement (large arrows) and cord compression
weighted sequences in the axial and sagittal planes, fol- (arrowhead). B. Axial T1-image shows the large amount of
lowed by postgadolinium sagittal and axial images of the vertebral and paraspinal tumor, which anteriorly displaces the
cervical, thoracic, and lumbar regions will take hours, aorta and vena cava and extends medially through the neural
result in extreme patient discomfort, and reams of unnec- foramina into the spinal canal and compresses the cord (black
essary and motion-degraded images. arrow) from both sides (83).
66 INTRODUCTION
Multiple Myeloma
Plasma cell neoplasms commonly involve the spine, usu-
ally in the thoracic region and generally in patients
beyond middle age (88). The classic radiographic appear-
ance of small punched out lytic lesions may also be seen
as multiple small T1-dark foci in the vertebral body mar-
row on MRI. MRI is an efficient way to screen the entire
FIGURE 3.50
FIGURE 3.49
B-cell lymphoma in a 23-year-old man. A. Sagittal T1 MR-
Multiple myeloma in a 61-year-old woman. Left. Sagittal T1- image shows patchy increased and decreased signal within
weighted image shows diffuse abnormal low intensity in all the the vertebral marrow, indicating tumor involvement. A large
marrow because of multiple myeloma infiltration. Right. Sagit- posterior epidural tumor mass compresses the cord from
tal fast spin-echo T2-weighted image gives a myelogram effect behind. B. Axial T1-image at this level shows vertebral mar-
and shows good conspicuity between the posteriorly bulging row involvement, a large paravertebral tumor mass, and the
tumor-laden vertebrae and the CSF and neural structures (83). posterior epidural mass (arrow) compressing the cord (83).
IMAGING OF THE SPINAL CORD 67
Hemangioma
These tumors are very common incidental findings on
MRI. They are found in 10 to 15 percent of patients at
autopsy (88), increasing in incidence with age. In our
experience, small bone hemangiomas are seen in almost
every older patient. The most common location is the tho-
racic spine, followed by the lumbar spine (89). Mottled
T1-bright signal caused by interspersed adipose tissue,
and a T2-bright signal of fluid and cells are characteris-
tic (Figure 3.51). They are usually round and small when
found incidentally, but may occupy the entire vertebral FIGURE 3.52
body, expanding it and causing neurologic symptoms.
The bony striations and spicules classically seen on plain Giant cell tumor, with vertebral collapse and posterior
films and CT may be seen on MRI. retropulsion into the spinal canal in a 28-year-old man, shown
on a sagittal T1-weighted MR image (83).
Intradural-Extramedullary Tumors
FIGURE 3.53
FIGURE 3.55
Schwannoma in a 52-year-old man. Consecutive sagittal post-
gadolinium T1 images show an oval, well-circumscribed, Multiple cauda equina schwannomas in NF-2 in an 18-year-
intradural-extramedullary enhancing mass that contains old-woman. Sagittal postgadolinium T1-weighted MR image
darker cystic-appearing components and markedly com- shows multiple nodular enhancing masses adherent to the
presses the mid thoracic spinal cord (83). cauda (83).
IMAGING OF THE SPINAL CORD 69
Paraganglioma
Spinal paragangliomas have the same histology as their
more familiar counterparts elsewhere in the body, such as
the adrenal medulla and carotid body, and have similar
imaging characteristics, including prominent contrast
enhancement. They are found in adults and are generally
attached to the filum terminale or cauda equina (93) (Fig-
ure 3.58).
FIGURE 3.56
FIGURE 3.57
A B
FIGURE 3.59
Intradural extramedullary lipoma in a 20-year-old woman. A, Left. Sagittal T1 MR image shows a very large well-circumscribed
intradural-extramedullary T1-bright mass posterior to and compressing the upper thoracic cord. A, Right. Fat-saturated post-
gadolinium T1 image shows complete suppression of the T1-bright signal, proving the presence of fat rather than T1-bright blood.
A subtle thin rim of enhancement is noted around the tumor capsule. B. Axial T1 image shows the lipoma filling and expand-
ing the thecal sac and spinal canal, markedly flattening the cord (arrow) (83).
IMAGING OF THE SPINAL CORD 71
A A
FIGURE 3.60
FIGURE 3.61
moma tumor is classically associated with neurofibro-
matosis-2. (91) (Figure 3.65). Tethered cord with conus lipomyelomeningocele in an 8-year-
Astrocytomas usually show a fusiform expansion of old girl. A. Sagittal T1 image shows a tethered cord with conus
(white arrow) at the L34 level, with posteriorly adherent T1-
the cord associated with T2-prolongation and (variable)
bright intradural lipoma. The posterior dura (small arrow) is
enhancement (89) (Figures 3.66 and 3.67). Cysts may be
well visualized, outlined by fat. Note the absence of normal
present, either intrinsic to tumor or at the edge of the posterior elements at this level and the wide spina bifida
tumor. defect (large arrow). B. Axial T1-image shows the C-shaped
Spinal cord oligodendrogliomas are rare, compris- T1-bright lipoma wrapped around the right side of the conus.
ing only 0.8 to 4.7 percent of intramedullary tumors of Note also the epidural lipoma posterior to the thecal sac and
the spinal cord and filum (97). They are found in the cer- the abnormal bony posterior elements (83).
72 INTRODUCTION
FIGURE 3.62
A B
FIGURE 3.64
Conus myxopapillary ependymoma in a 43-year-old man. A, Left. Sagittal pre-contrast and Right, postgadolinium T1-weighted
images show a well-circumscribed enhancing mass at the conus. B. Consecutive sagittal T2-weighted images clearly demon-
strate the mass. The small T2-dark focus (arrow) suggests the presence of hemosiderin (blood products), a useful clue favoring
the diagnosis of ependymoma (83).
IMAGING OF THE SPINAL CORD 73
FIGURE 3.65
Hemangioblastoma
These tumors may occur sporadically, but are often mul-
tiple and associated with the cerebellar hemangioblas-
tomas of von HippelLindau syndrome. They may exhibit
the typical cyst with associated mural nodule, which
enhances brightly with gadolinium (Figure 3.69). There
may be associated edema and, as in the brain, they may
have associated prominent vascularity (93).
FIGURE 3.66
Embryonal Tumor
Astrocytoma in a 4-year-old girl. Left. Sagittal postgadolin-
ium T1 MR image shows fusiform expansion of the upper cer- These were previously discussed in the section on
vical cord, with mild partial enhancement, and extensive T1- intradural-extramedullary tumors, but may also occur in
dark edema extending up to the medulla and down to C6. an intramedullary location. As mentioned, they may be
Right. Sagittal T2 image again shows the fusiform cord associated with spinal dysraphism, especially lipomas.
expansion. The T2-bright edema is well demonstrated (83). Dermoids and epidermoids have variable signal intensity,
74 INTRODUCTION
Oligodendroglioma. 64-year-old man presented with left leg Hemangioblastoma in von HippelLindau syndrome in a 28-
atrophy and weakness. Postcontrast sagittal T1-weighted MR year-old woman. Sagittal postgadolinium T1 image shows a
image shows an enhancing intramedullary mass (arrow- small enhancing nodule in the posterior upper cervical cord,
heads), with some cystic components. (Because of patients with an associated cyst that expands the cord slightly. Patient
scoliosis, normal neural foramina are noted between the has had cerebellar hemangiomas resected previously (83).
regions of tumor.)
FIGURE 3.71
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4 Epidemiology of
Spinal Cord Injury
pinal cord injury (SCI) epidemiol- However, the NSCISC Database has several advantages,
States is approximately 40 cases per million population, several trends are apparent. SCI incidence rates are low-
or just over 10,000 new cases per year. Moreover, est for the pediatric age group, highest for persons in their
although there have been changes in the most common late teens and early twenties, and decline consistently there-
underlying causes of SCI during the past 20 years, the after (9,10,12,14,16). The state of Oklahoma is typical,
overall incidence of new cases each year has remained rel- where age-specific SCI incidence rates per million popu-
atively stable (19). lation for 19881990 were 6 for age 014 (3 percent of
This annual incidence rate of forty new cases per cases), 94 for age 1519 (18 percent of cases), 85 for age
million population does not include persons who die at 2024 (15 percent of cases), 71 for age 2529 (14 percent
the scene of the accident. Most population-based state of cases), 47 for age 3044 (27 percent of cases), 32 for
registries do not report deaths that occur prior to hospi- age 4559 (12 percent of cases), and 26 for persons who
tal admission. An exception is the state of Utah, where were at least 60 years of age (11 percent of cases) (10).
the incidence of prehospital SCI deaths from 19891991 A similar pattern emerges from the NSCISC Data-
was reported to be approximately 4 cases per million pop- base, where the mean age at time of injury is 32.7 years
ulation per year, or 9.3 percent of the incidence rate for ( 15.9 years), the median age at injury is 27 years, and
hospitalized cases (11). Nonetheless, incomplete report- the most common age at injury is 19 years (Table 4.2).
ing of these prehospital deaths is possible, and this esti- Overall, 52.7 percent of persons enrolled in the NSCISC
mate of fatal SCI incidence is probably conservative. Database since 1973 were between the ages of 16 and 30
Incidence of SCI elsewhere is consistently lower than at the time of their injury. However, these overall figures
in the United States, and usually does not exceed twenty mask a rather substantial trend toward increasing age at
new cases per million population per year (2026). The injury in recent years. From 19731977, the mean age at
increased incidence in the United States compared to injury for persons enrolled in the NSCISC Database was
other countries is caused in large part by the acts of vio- 28.2 years, whereas among persons injured between 1995
lence that are a common cause of SCI in the United States, and 1999, the mean age at injury was 36.5 years. These
but a rare cause of SCI elsewhere. However, even in the figures mirror the increasing average age of the general
absence of SCI caused by acts of violence, the incidence United States population, which was 28 years in 1970 and
rate is higher in the United States than in other countries. 34.9 years in 1997, although changes in underlying age-
specific incidence rates cannot be ruled out as also con-
tributing to this trend in age at time of SCI (8).
RISK FACTORS
Gender
Age at Injury
SCI occurs predominantly among men. Gender-specific
Comparisons of age-specific SCI incidence rates are diffi-
SCI annual incidence rates have been reported by several
cult among the state registries because of the lack of uni-
state registries, with men ranging from a 4.6 times higher
form categorization of reported age ranges. Nonetheless,
incidence rate in West Virginia (41 new cases per million
men vs. 9 new cases per million women), to only a 2.3
times higher incidence rate in Louisiana (67 new cases per
TABLE 4.1 million men vs. 29 new cases per million women) (12,16).
Annual SCI Incidence Rates Derived from The percentage of SCIs that occur among men is 81.5 per-
Population-based State Registries cent for persons enrolled in the NSCISC Database (Table
4.2); 80 percent for Oklahoma and Arkansas; 78 percent
ANNUAL for Virginia; 76 percent for Utah, Colorado, and Geor-
INCIDENCE gia; and 69 percent for Louisiana (811,14,1618). This
STATE DATES RATE/106 four-to-one ratio of male to female SCIs has remained
very consistent over time despite significant trends in age
Arkansas (9) 198089 27 at injury, ethnicity, and etiology of injury (Table 4.2).
New York (15) 198288 43
West Virginia (12) 198588 25
Oklahoma (10) 198890 40 Race and Ethnicity
Utah (11) 198991 43
Colorado (14) 198996 45 Fewer state registries have reported race and ethnicity
Virginia (17) 199092 30 data than information on age and gender. Nonetheless,
Louisiana (16) 1991 46 some clear patterns have emerged. SCI incidence rates are
Georgia (18) 199192 46 substantially and consistently higher for African Ameri-
Mississippi (13) 199295 59 cans than whites. In Oklahoma, the annual SCI incidence
rate for African Americans was 57 new cases per million
EPIDEMIOLOGY OF SPINAL CORD INJURY 81
TABLE 4.2
Trends in Demographic Characteristics of Persons Enrolled
in the NSCISC Database by Year of Injury
population compared to 40 new cases per million popu- Interestingly, the average age at injury varies by
lation for whites and 29 new cases per million population racial and ethnic group. Among persons enrolled in the
for Native Americans (10). In Louisiana, the difference NSCISC Database, persons of Hispanic descent have the
is even more striking, with African Americans having an youngest average age at injury (27.8 years), followed by
annual SCI incidence rate of 72 new cases per million Native Americans (29.3 years), whites (32.5 years),
population compared to 36 new cases per million popu- African Americans (33.2 years), and Asian Americans
lation for whites (16). In fact, when race and gender were (35.6 years) (8). Moreover, since 1973, the average age at
considered together, the SCI annual incidence rate per mil- time of injury for persons enrolled in the NSCISC Data-
lion population in Louisiana in 1991 was 127 for African base has increased by about 10 years for whites while
American men, 41 for white men, 31 for white women, increasing only marginally for other racial groups (8).
and 26 for African American women (16). Unfortunately,
state registries have not yet published SCI incidence rates
for other racial and ethnic groups, such as Asian Ameri- ETIOLOGY OF INJURY
cans and persons of Hispanic descent.
Since 1995, 62.2 percent of newly injured persons
State Registries
enrolled in the NSCISC Database were white; 23.7 per-
cent were African American; 10.9 percent were Hispanic; Cause-specific incidence rates have been reported by gender
2.2 percent were Asian American; and 0.3 percent were for Arkansas, by race for Oklahoma, and by both gender
Native American (Table 4.2). However, between 1973 and race for Louisiana (9,10,16). Other states often report
and 1977, 76.8 percent of new persons enrolled in the data on causes of SCI only in percentage terms that are heav-
NSCISC Database were white; 14.9 percent were African ily influenced by the racial and ethnic composition of the
American; 6.2 percent were Hispanic; 0.8 percent were general population of each state (11,12,14). Overall, motor
Asian American; and 1.3 percent were Native American. vehicle crashes are the leading cause of SCI in all states. In
This trend toward an increasing percentage of new SCIs most states, such as Colorado, Oklahoma, Utah, West Vir-
occurring among minority populations may be caused by ginia, and Arkansas, falls rank second, followed by sports
periodic changes in the location of the model systems that and acts of violence (912,14). However, in states like
participate in the NSCISC Database and changes in refer- Louisiana and Mississippi, with greater proportions of
ral patterns to model systems. However, some changes African Americans and/or persons of Hispanic descent, acts
in underlying race-specific SCI incidence rates may also of violence are the second leading cause of SCI (13,16).
have occurred. Ultimately, data from the state registries In fact, Oklahoma data reveal that the higher annual
should be examined to address this question. incidence of SCI among African Americans is caused
82 INTRODUCTION
entirely by acts of violence (10). Overall in Oklahoma, participation rates, but have an annual incidence rate of
the annual incidence rate of SCI was 17 new cases per mil- only 1 case per 100,000 athletes (27).
lion population higher for African Americans than whites The overall pattern of causes of SCI varies substan-
(57 vs. 40), whereas the cause-specific annual incidence tially by racial group (Table 4.3). Among whites, auto-
rate of SCI caused by violence was 18 new cases per mil- mobile crashes rank first (38.9 percent), followed by falls
lion population higher for African Americans than whites (24.5 percent), diving mishaps (6.2 percent), motorcycle
(21 vs. 3). There were no other meaningful differences in crashes (5.8 percent), and gunshot wounds (4.6 percent).
cause-specific annual SCI incidence rates among the races Among African Americans, gunshot wounds rank first
in Oklahoma (10). (41.4 percent), followed by automobile crashes (25.2 per-
Although motor vehicle crashes are the overall lead- cent), falls (17.6 percent), person-to-person contact
ing cause of SCI, among African American men, violence (fights) (3.4 percent), and medical or surgical complica-
ranks first. In Louisiana during 1991, the annual cause- tions (2.6 percent). Among persons of Hispanic descent,
specific SCI incidence rate per million population caused the top five causes of SCI are gunshot wounds (37.0 per-
by acts of violence was 71 for African American men, but cent), automobile crashes (27.7 percent), falls (17.8 per-
only 10 for African American women, 5 for white cent), diving mishaps (3.2 percent), and being hit by
women, and 4 for white men (16). By comparison, annual falling or flying objects (2.9 percent). Among Asian Amer-
cause-specific SCI incidence rates per million population icans, the top five causes of SCI are automobile crashes
caused by motor vehicle crashes in Louisiana during 1991 (40.8 percent), gunshot wounds (21.1 percent), falls (16.2
were 26 for African American men, 22 for white men, percent), motorcycle crashes (4.9 percent), and medical
17 for white women, and 13 for African American or surgical complications (2.8 percent).
women (16). As reported by Nobunaga et al., causes of SCI
reported to the NSCISC Database between 1994 and
1998 also varied by gender and age (8). Automobile
NSCISC Database
crashes ranked first among both men and women, but
Specific causes of all new spinal cord injuries reported to caused a much greater share of SCIs among women than
the NSCISC Database between 1995 and 2000 by race men (49.1 vs. 29.7 percent). Gunshot wounds ranked sec-
and ethnicity appear in Table 4.3. Again, because Table ond among men (22.4 percent) but third among women
4.3 reflects percentages rather than actual incidence rates, (11.1 percent), whereas falls ranked third among men
the figures for all races combined are influenced by the (21.7 percent) but second among women (19.8 percent).
locations of the eighteen model systems that contributed Motor vehicle crashes are the leading cause of SCI among
data during this period. However, the race-specific per- persons younger than 45 years of age, whereas falls rank
centages should be reasonably representative of the typ- first among persons older than age 45. The occurrence
ical causes of SCI that occur within each racial group. of SCIs caused by recreational sports activities and acts
Overall, automobile crashes accounted for 34.5 per- of violence declines rapidly with advancing age (8).
cent of SCIs reported to the NSCISC Database, followed
by falls (22.0 percent), gunshot wounds (17.2 percent),
diving mishaps (4.5 percent), and motorcycle crashes (4.4 PREVALENCE
percent). Among recreational sports activities, diving
mishaps rank first, followed by snow skiing, other Although incidence reflects the number of new cases that
unspecified winter sports, and horseback riding. The occur each year, prevalence is defined as the number of per-
occurrence of SCIs while playing football, which once sons with a SCI who are currently alive. There are two ways
ranked second among recreational sporting activities, has to estimate prevalence. One approach is based on the epi-
declined dramatically in recent years because of rules demiologic relationship between prevalence, incidence, and
changes that have banned deliberate spearing and the the duration of the condition. When a condition occurs rel-
use of the top of the helmet as the initial point of contact atively rarely, as is the case with SCI, then the prevalence
in making tackles. rate can be estimated as the product of the incidence rate
Whereas diving mishaps are the most common and average duration. Using this mathematical formula and
recreational sporting activity leading to a SCI, that does the best available data at the time, the prevalence of SCI in
not mean that diving is the most risky of these activities. the United States in 1980 was estimated to be 906 persons
To assess risk, the underlying rate of exposure to that per million population, or just under 200,000 existing cases
activity must be known. For example, male gymnastics (28). However, an underlying assumption when making
causes few SCIs because of its low participation rates, but this calculation is that both incidence and duration (as mea-
has been reported to have an annual incidence rate of 14.3 sured in the case of SCI by life expectancy) have remained
per 100,000 athletes (27). By comparison, high school constant over time. Because life expectancy for persons with
and college football cause more SCIs because of higher SCI continues to increase, and a current estimate of life
EPIDEMIOLOGY OF SPINAL CORD INJURY 83
TABLE 4.3
Causes (%*) of New Spinal Cord Injuries Reported to the NSCISC Database
between 1995 and 2000, by Racial and Ethnic Group
RACE
Vehicular
Automobile 38.9 25.2 27.7 40.8 34.5
Motorcycle 5.8 1.9 2.5 4.9 4.4
Bicycle 1.4 0.7 1.0 2.1 1.2
All Terrain Vehicle 1.1 0.1 0.3 0.7 0.8
Snowmobile 0.5 0.0 0.0 0.0 0.3
Fixed Wing Aircraft 0.3 0.0 0.1 0.0 0.2
Rotating Wing Aircraft 0.1 0.0 0.0 0.7 0.1
Boat 0.1 0.0 0.0 0.0 0.1
Other Vehicle 0.7 0.1 0.4 0.7 0.5
Violence
Gunshot 4.6 41.4 37.0 21.1 17.2
Personal Contact 0.5 3.4 0.7 0.7 1.2
Stab 0.3 0.7 0.6 0.7 0.4
Sports
Diving 6.2 1.1 3.2 1.4 4.5
Snow Skiing 1.0 0.0 0.3 0.7 0.7
Surfing 0.8 0.1 0.1 1.4 0.5
Horseback Riding 0.7 0.0 0.3 0.0 0.4
Wrestling 0.3 0.2 0.0 0.0 0.2
Track and Field 0.3 0.0 0.0 0.0 0.2
Football 0.2 0.5 0.1 0.0 0.2
Trampoline 0.3 0.0 0.1 0.0 0.2
Water Skiing 0.1 0.0 0.0 0.0 0.1
Gymnastics 0.1 0.1 0.0 0.7 0.1
Rodeo 0.2 0.0 0.0 0.0 0.1
Hang Gliding 0.1 0.0 0.0 0.0 0.1
Air Sports 0.2 0.0 0.0 0.0 0.1
Baseball 0.1 0.1 0.0 0.0 0.1
Other Winter Sports 0.7 0.0 0.0 0.0 0.5
All Other Sports 0.6 0.1 0.0 0.0 0.4
Falls 24.5 17.6 17.8 16.2 22.0
Falling Objects 3.0 1.5 2.9 0.7 2.6
Pedestrian 1.5 2.2 2.0 2.1 1.7
Medical/Surgical Complications 3.8 2.6 1.9 2.8 3.3
Other/Unclassified 1.1 0.3 0.9 1.4 0.9
expectancy was used, prevalence was slightly overestimated Eventually, substantial funding was provided by the Par-
by using this formula (29). alyzed Veterans of America to use a sophisticated prob-
Of course, the more straightforward way to estimate ability sampling plan of small geographic areas and insti-
prevalence is simply to count people using standard sam- tutions to estimate conservatively the prevalence of SCI
pling and survey techniques. For many years, this was not in the United States at 721 persons per million popula-
attempted because of the large sample size, time, and tion, or approximately 176,965 persons in 1988 (30).
expense that would be required to produce an accurate More recently, current estimates of agesex-specific
estimate of prevalence for a condition as rare as SCI. incidence and mortality have been combined with the
84 INTRODUCTION
results of the 1988 survey to project the growth in preva- Traumatic SCI in Olmsted County, Minnesota, 19351981. Am
J Epidemiol 1985; 121:884895.
lence of SCI in the United States over time (31). Using new 3. Kalsbeek WD, McLaurin RL, Harris BSH, Miller JD. The national
mathematical models, the prevalence of SCI in the United head and spinal cord injury survey: Major findings. J Neurosurg
States was projected to increase to 246,882 persons by 1980; 53Suppl:1931.
4. Bracken MB, Freeman DH, Hellenbrand K. Incidence of acute
2004, and 276,281 persons by 2014 (31). This projected traumatic hospitalized spinal cord injury in the United States,
growth in prevalence results exclusively from improved 19701977. Am J Epidemiol 1981; 113:615622.
life expectancies rather than any anticipated change in 5. Stover SL, DeVivo MJ, Go BK. History, implementation, and cur-
rent status of the national spinal cord injury database. Arch Phys
SCI incidence rates. Med Rehabil 1999; 80:13651371.
Because of the lifetime duration of SCI as well as dif- 6. DeVivo MJ, Rutt RD, Black KJ, Go BK, Stover SL. Trends in
ferences in life expectancy for persons with different spinal cord injury demographics and treatment outcomes between
1973 and 1986. Arch Phys Med Rehabil 1992; 73:424430.
demographic characteristics and degrees of SCI impair- 7. Go BK, DeVivo MJ, Richards JS. The epidemiology of spinal cord
ment, the profile of persons with SCI who are alive today injury. In: Stover SL, DeLisa JA, Whiteneck GG, (eds.), Spinal
is different from the typical characteristics of persons who Cord Injury: Clinical Outcomes from the Model Systems.
Gaithersburg, MD.: Aspen Publishers, 1995, 2155.
incur these injuries each year. For example, the median 8. Nobunaga AI, Go BK, Karunas RB. Recent demographic and
current age of all persons with SCI who were alive in 1988 injury trends in people served by the model spinal care injury care
was estimated to be 41 years (15 years older than the systems. Arch Phys Med Rehabil 1999; 80:13721382.
9. Acton PA, Farley T, Freni LW, Ilegbodu VA, Sniezek JE, Wohlleb
median age of new cases occurring at that time) (30). JC. Traumatic spinal cord injury in Arkansas, 19801989. Arch Phys
Moreover, because that estimate of median age is now Med Rehabil 1993; 74:10351040.
12 years old, and because the median age of new cases 10. Price C, Makintubee S, Herndon W, Istre GR. Epidemiology of
traumatic spinal cord injury and acute hospitalization and reha-
that occur each year is increasing, the median age of all bilitation charges for spinal cord injuries in Oklahoma,
persons with SCI who are alive today would be somewhat 19881990. Am J Epidemiol 1994; 139:3747.
higher than it was in 1988 (but less than 12 years higher 11. Thurman DJ, Burnett CL, Jeppson L, Beaudoin DE, Sniezek JE.
Surveillance of spinal cord injuries in Utah, USA. Paraplegia 1994;
because of higher mortality rates among older persons). 32:665669.
Similarly, it was estimated in 1988 that only 71 per- 12. Woodruff BA, Baron RC. A description of nonfatal spinal cord
cent of persons with SCI who were alive at that time were injury using a hospital-based registry. Am J Prev Med 1994;
10:1014.
men, compared to 82 percent of new cases enrolled in 13. Surkin J. Spinal Cord Injury: Mississippis Facts and Figures. Jack-
the NSCISC Database (30). Again, the percentage of per- son, MS: Mississippi State Department of Health, 1995.
sons with SCI who are alive today and who are men will 14. Colorado Department of Public Health and Environment, Disease
Control and Environmental Epidemiology Division. 1996 Annual
be slightly lower than 71 percent, because men have Report of the Traumatic Spinal Cord Injury Early Notification
shorter life expectancies than women. However, there System. Denver, CO: Colorado Department of Transportation
have been no new attempts to develop a profile of preva- Printing Office, 1997.
15. Relethford JH, Standfast SJ, Morse DL. Trends in traumatic spinal
lence of persons with SCI since 1988, so one can only cord injuryNew York, 19821988. MMWR 1991; 40:535537,
speculate about current SCI population characteristics 543.
such as age, gender, race, neurologic level of injury, and 16. Bayakly AR, Lawrence DW. Spinal Cord Injury in Louisiana 1991
Annual Report. New Orleans, LA: Louisiana Office of Public
degree of injury completeness. Health, 1992.
17. Virginia Department of Rehabilitation Services. Spinal Cord
Injury in Virginia: A Statistical Fact Sheet. Fishersville, VA: Vir-
CONCLUSION ginia Spinal Cord Injury System, 1993.
18. Johnson SC. Georgia Central Registry: Spinal Cord Disabilities
and Traumatic Brain Injury. Warm Springs, GA: Roosevelt Warm
Thanks to the initiation of population-based SCI registries Springs Institute for Rehabilitation, 1992.
in several states, as well as the continued existence of the 19. Glick T. SCI surveillance: Is there a decrease in incidence?
[abstract] J Spinal Cord Med 2000; 23Suppl:61.
NSCISC Database, much is now known about the 20. Chen CF, Lien IN. Spinal cord injuries in Taipei, Taiwan,
descriptive epidemiology of SCI in the United States. 19781981. Paraplegia 1985; 23:364370.
Future epidemiologic efforts should focus on developing 21. Biering-Sorensen F, Pedersen V, Clausen S. Epidemiology of spinal
cord lesions in Denmark. Paraplegia 1990; 28:105118.
a more accurate profile of persons with SCI who are alive 22. Garcia-Reneses J, Herruzo-Cabrera R, Martinez-Moreno M. Epi-
today, as well as detailed investigations of the exact cir- demiological study of SCI in Spain 19841985. Paraplegia 1991;
cumstances surrounding how these injuries occur. Such 28:180190.
23. Karamehmetoglu SS, Unal S, Karacan I, Yilmaz H, Togay HS,
research might provide important clues to developing Ertekin M, Dosoglu M, Ziyal MI, Kassaroglu D, Hakan T. Trau-
cost-effective primary prevention programs. matic spinal cord injuries in Istanbul, Turkey: An epidemiologi-
cal study. Paraplegia 1995; 33:469471.
24. Schonherr MC, Groothoff JW, Mulder GA, Eisma WH. Rehabil-
References itation of patients with spinal cord lesions in The Netherlands: An
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1. Kraus JF, Franti CE, Riggins RS, Richards D, Borhani NO. Inci- 25. Otom AS, Doughan AM, Kawar JS, Hattar EZ. Traumatic spinal
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2. Griffin MR, Opitz JL, Kurland LT, Ebersold MJ, OFallon WM. 26. Martins F, Freitas F, Martins L, Dartigues JF, Barat M. Spinal cord
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injuriesepidemiology in Portugals central region. Spinal Cord and causes of death among persons with spinal cord injury. Arch
1998; 36:574578. Phys Med Rehabil 1999; 80:14111419.
27. Clark KS. Spinal cord injuries in organized sports. Model Sys- 30. Berkowitz M, Harvey C, Greene CG, Wilson SE. The Economic
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28. DeVivo MJ, Fine PR, Maetz HM, Stover SL. Prevalence of spinal Demos Publishers, 1992.
cord injury: A reestimation employing life table techniques. Arch 31. Lasfargues JE, Custis D, Morrone F, Carswell J, Nguyen T. A
Neurol 1980; 37:707708. model for estimating spinal cord injury prevalence in the United
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This Page Intentionally Left Blank
5 Outcomes Following
Spinal Cord Injury
pinal cord injury (SCI) is one of the resources that will only minimally improve a patients
injury, because the results of an early examination may ery (or deterioration) that has occurred in the intervening
be compromised by pain, confusion, and associated period. By dividing the difference between scores by the
injuries (24). number of days in the interval, the change per day can
The accuracy of predicting recovery based on neu- be calculated. Finally, by multiplying the change per day
rologic examination improves with a longer interval by 365 days, the annualized rate of change can be deter-
between onset of injury and the most recent neurologic mined. The annualized rate of change represents the rate
examination. Whereas Waters and associates studied of change during a particular interval that could be
neurologic recovery in over 500 patients and found long- expected if it were to continue for one year.
term motor recovery could be reliably predicted using the Once completeness of injury and level of injury
1-month neurologic examination to predict recovery (tetraplegia or paraplegia) have been determined, rate of
(58), Marino and colleagues found that a neurologic recovery and individual muscle recovery data can be
examination performed 1 week following injury had reviewed to predict recovery in a specific patient group. For
prognostic value for determining recovery (9). These the following discussion, patients are categorized into one
investigators used the ASIA Impairment Scale (AIS) and of four groups: complete tetraplegia, incomplete tetraple-
the Frankel scale to predict neurologic recovery. Clearly, gia, complete paraplegia, and incomplete paraplegia.
it is the responsibility of the rehabilitation practitioner
to perform serial examinations on a frequent basis dur-
ing the rehabilitation process so that functional goals can MOTOR RECOVERY
be updated and modified appropriately. The authors rec-
Complete Paraplegia (5)
ommend such examinations be performed on a weekly
basis. In the overwhelming majority (96 percent) of patients with
The International Standards for Neurologic and complete paraplegia 1 month following injury, the injury
Functional Classification of SCI have been accepted as the will remain complete. For the few patients, 4 percent, who
most accurate and reliable instrument for documenting undergo late conversion to incomplete status, motor
neurologic status following SCI (10). These standards recovery is small. Late conversion is significant, however,
require determination of both motor and sensory levels for recovery of sacral functions. Approximately half of
bilaterally, as well as determination of completeness of patients who undergo late conversions will regain voli-
injury. Impairment is graded on a five-point scale, which tional bowel and bladder function.
is a modified version of the Frankel scale. Activities of Recovery of motor function is related to level of
daily living are assessed by use of the Functional Inde- injury. No patients with a neurologic level of injury (NLI)
pendence Measure (FIM). above T9 regained motor function 1 year following
To determine motor level of injury, ten key muscles injury. At the more caudal levels of injury, there is a
representing specific spinal levels are tested bilaterally greater recovery of motor functions.
using the standard six-point (zero to five) manual mus- Recovery of functional strength (3/5 or greater) is
cle testing (MMT) scale. An individual with no neurologic minimal in muscles with a grade of 0/5 at 1 month (Table
deficit has an ASIA Motor Score (AMS) of 100 points (50 5.1). Only about 5 percent of these muscles will regain
points each for left and right sides of the body or 50 points functional strength 1 year following injury. In contrast,
each for the bilateral upper extremities and the bilateral 64 percent of muscles with either 1/5 or 2/5 strength at
lower extremities). Sensory level is determined by testing one month will have grade 3/5 strength at 1 year.
key points in each of twenty-eight dermatomes on both Only 5 percent of individuals with complete para-
the right and left sides of the body. Both pinprick and plegia will become community ambulators 1 year after
light-touch sensation are tested in each dermatome. Sen- injury. By definition, community ambulators are able to
sory function is scored on a three-point scale (zero to walk for more than 250 meters (the distance needed to
three). The total possible score for each of the sensory perform routine community activities). Paraplegics lack-
modalities is 112 points. Details of neurological assess- ing sufficient hip flexion to achieve a reciprocal gait pat-
ment of SCI is covered in Chapter 27. tern must utilize an energy intensive swing-through,
Determining completeness of injury by the sacral crutch-assisted gait pattern to walk. This type of gait pat-
sparing definition is the most important factor in deter- tern is generally impractical and unsuitable for routine
mining recovery at neurologic levels distal to the lesion. mobility because of the required severe arm exertion and
A complete injury is one without any sensory or motor high rates of energy expenditure.
function in the lowest sacral segments. In an incomplete
injury, there is partial preservation of motor and/or sen-
Incomplete Paraplegia (6)
sory function in the lowest sacral segments.
The differences in motor and sensory scores between In patients with incomplete paraplegia, motor recovery is
successive neurologic examinations represent the recov- independent of level of injury. The average increase in
OUTCOMES FOLLOWING SPINAL CORD INJURY 89
motor scores was 12 points at 1 year after injury. Among sequentially. As with other categories of injury, the rate
patients with incomplete paraplegia, 76 percent will of motor recovery declines rapidly in the first 6 months
attain community ambulation status at 1 year. Thus, just following injury. Recovery of functional strength in indi-
knowing only whether a paraplegic is complete or incom- vidual muscles is very good for those patients with incom-
plete after injury is a very powerful predictor of future plete tetraplegia. All upper extremity muscles and 97 per-
ambulatory function. cent of lower extremity muscles with initial strength
Recovery of functional strength is also improved in grades of 1/5 or 2/5 will recover to at least 3/5 by 1 year.
incomplete paraplegia in comparison to complete para- In the upper extremity, 20 percent of muscles with an ini-
plegia. For muscles with 0/5 strength at 1 month, 26 per- tial grade of 0/5 will recover functional strength (Tables
cent will recover functional strength at 1 year. Eighty-five 5.1 and 5.2). In the lower extremity, 24 percent of those
percent of muscles with an initial strength of 1/5 or 2/5 with absent strength initially will recover to at least 3/5
will recover to 3/5 strength at 1 year (Table 5.1). by 1 year (Table 5.2).
Forty-six percent of those with incomplete tetraple-
gia become community ambulators at 1 year. The percent
Complete Tetraplegia (7)
of incomplete tetraplegics who attain community ambu-
Only 10 percent of those with complete tetraplegia at lation status is lower than for patients with incomplete
1 month following injury will convert to incomplete sta- paraplegia who have equivalent lower extremity function.
tus. As in patients with complete paraplegia, motor recov- Because the upper extremities may be severely impaired
ery is minimal in those with conversion to incomplete sta- in patients with incomplete tetraplegia, these individuals
tus, but recovery of sacral function is enhanced. may be unable to use their upper extremities for the
Recovery of motor strength is again independent of crutch-assisted gait that may be necessary to compensate
level of injury. Prediction of individual muscle recovery for lower extremity weakness.
to functional strength will help in determining indepen-
dent functioning. For example, 97 percent of wrist exten-
Timing of Motor Recovery
sors with an initial strength of 1/5 will recover to at least
3/5 at 1 year following injury. Therefore, an individual Graphing the annualized rates of change against time
who is initially dependent or needs assistance with table- since injury reveals the course of motor recovery. Regard-
top activities and transfers can anticipate independence less of level or completeness of injury, the majority of
in these activities at 1 year following injury. With the recovery occurs in the first 6 months following injury. The
exception of the triceps, all upper extremity muscles with rate-of-change plateaus at approximately 9 months but it
an initial strength grade of at least 1/5 recovered to at least does not equal zero (Figure 5.1). Although some motor
3/5 by 1 year (Table 5.2). recovery may continue 2 or more years after injury, the
amount is generally small and not likely to significantly
improve function.
Incomplete Tetraplegia (8)
Mange and associates examined motor recovery in
For patients with incomplete tetraplegia, recovery in the the zone of injury and compared recovery of motor com-
upper and lower extremities occurs concurrently, not plete and motor incomplete subjects. Their results suggest
90 INTRODUCTION
TABLE 5.3
Expected Functional Outcomes, LEVEL C1-3
TABLE 5.3
Expected Functional Outcomes, LEVEL C1-3 (continued)
FIM/ASSISTANCE
DATA
EXPECTED FUNCTIONAL OUTCOMES EQUIPMENT EXP MED
LEVEL C4
Functionally relevant muscles innervated: Upper trapezius, diaphragm, cervical paraspinal muscles
Movement possible: Neck flexion, extension, rotation, scapular elevation, inspiration
Patterns of Weakness: Paralysis of trunk, upper extremities, lower extremities; inability to cough, endurance and
respiratory reserve low secondary to paralysis of intercostals
FIM/Assistance Data: Exp Expected FIM Score
Med NSCISC Median
NSCISC Sample Size: FIM 15 Assist 12
FIM/ASSISTANCE
DATA
EXPECTED FUNCTIONAL OUTCOMES EQUIPMENT EXP MED
Respiratory May be able to breathe without If not ventilator free, see C1-3 for
a ventilator equipment requirements
Bowel Total assist Reclining shower/commode chair 1 1
(if roll-in shower available)
Bladder Total assist 1 1
Bed Mobility Total assist Full electric hospital bed with
Trendelenburg feature and side rails
Bed/Wheelchair Total assist Transfer board 1 1
Transfers Power or mechanical lift with sling
Pressure Relief/ Total assist; may be independent Power recline and/or tilt wheelchair
Positioning with equipment Wheelchair pressure-relief cushion
Postural support and head control
devices as indicated
Hand splints may be indicated
Specialty bed or pressure-relief
mattress may be indicated
Eating Total assist 1 1
Dressing Total assist 1 1
Grooming Total assist 1 1
Bathing Total assist Shampoo tray 1 1
Handheld shower
Padded reclining shower/commode
chair (if roll-in shower available)
Wheelchair Power: Independent Power recline and/or tilt wheelchair 6 1
Propulsion Manual: Total assist with head, chin, or breath control and
manual recliner
Vent tray
OUTCOMES FOLLOWING SPINAL CORD INJURY 93
TABLE 5.3
Expected Functional Outcomes, LEVEL C4 (continued)
FIM/ASSISTANCE
DATA
EXPECTED FUNCTIONAL OUTCOMES EQUIPMENT EXP MED
LEVEL C5
Functionally relevant muscles innervated: Deltoid, biceps, brachialis, brachioradialis, rhomboids, serratus anterior
(partially innervated)
Movement possible: Shoulder flexion, abduction, and extension; elbow flexion and supination, scapular adduction and
abduction
Patterns of Weakness: Absence of elbow extension, pronation, all wrist and hand movement. Total paralysis of trunk
and lower extremities
FIM/Assistance Data: Exp Expected FIM Score
Med NSCISC Median
NSCISC Sample Size: FIM 15 Assist 12
FIM/ASSISTANCE
DATA
EXPECTED FUNCTIONAL OUTCOMES EQUIPMENT EXP MED
TABLE 5.3
Expected Functional Outcomes, LEVEL C5 (continued)
FIM/ASSISTANCE
DATA
EXPECTED FUNCTIONAL OUTCOMES EQUIPMENT EXP MED
Pressure Relief/ Independent with equipment Power recline and/or tilt wheelchair
Positioning Wheelchair pressure-relief cushion
Hand splints
Specialty bed or pressure-relief mattress
may be indicated
Postural support devices
Eating Total assist for setup, then independent Long opponens splint 5 5
eating with equipment Adaptive devices ad indicated
Dressing Lower extremity: Total assist Long opponens splint 1 1
Upper extremity: Some assist Adaptive devices as indicated
Grooming Some to total assist Long opponens splints 13 1
Adaptive devices as indicated
Bathing Total assist Padded tub transfer bench or 1 1
shower/commode chair
Handheld shower
Wheelchair Power: Independent Power: Power recline and/or tilt with 6 6
Propulsion Manual: Independent to some assist arm drive control
indoors on noncarpet, level surface; Manual: Lightweight rigid or folding
some to total assist outdoors frame with handrim modifications
Standing/ Total assist Hydraulic standing frame
Ambulation
Communication Independent to some assist after Long opponens splint
setup with equipment Adaptive devices as needed for page
turning, writing, button pushing
Transportation Independent with highly specialized Highly specialized modified van
equipment; some assist with accessible with lift
public transportation; total assist for
attendant-operated vehicle
Homemaking Total assist
Assist Required Personal care: 10 hours/day 16* 23*
Homecare: 6 hours/day
Able to instruct in all aspects of care
OUTCOMES FOLLOWING SPINAL CORD INJURY 95
TABLE 5.3
Expected Functional Outcomes, LEVEL C6
Functionally relevant muscles innervated: Clavicular pectoralis supinator; extensor carpi radialis longus and brevis,
serratus anterior, latissimus dorsi
Movement possible: Scapular protractor, some horizontal adduction, forearm supination, radial wrist extension
Patterns of Weakness: Absence of wrist flexion, elbow extension, hand movement. Total paralysis of trunk and lower
extremities
FIM/Assistance Data: Exp Expected FIM Score
Med NSCISC Median
NSCISC Sample Size: FIM 15 Assist 12
FIM/ASSISTANCE
DATA
EXPECTED FUNCTIONAL OUTCOMES EQUIPMENT EXP MED
TABLE 5.3
Expected Functional Outcomes, LEVEL C6 (continued)
FIM/ASSISTANCE
DATA
EXPECTED FUNCTIONAL OUTCOMES EQUIPMENT EXP MED
LEVEL C78
Functionally relevant muscles innervated: Latissimus dorsi, sternal pectoralis, triceps, pronator quadratus, extensor
carpi ulnaris, flexor carpi radialis, flexor digitorum profundus and superficialis, extensor communis, pronator/flexor/
extensor/abductor pollicis, lumbricals [partially innervated]
Movement possible: Elbow extension; ulnar/wrist extension, wrist flexion, finger flexions and extensions, thumb flexion/
extension/abduction
Patterns of Weakness: Paralysis of trunk and lower extremities, limited grasp release and dexterity secondary to partial
intrinsic muscles of the hand
FIM/Assistance Data: Exp Expected FIM Score
Med NSCISC Median
NSCISC Sample Size: FIM 15 Assist 12
FIM/ASSISTANCE
DATA
EXPECTED FUNCTIONAL OUTCOMES EQUIPMENT EXP MED
TABLE 5.3
Expected Functional Outcomes, LEVEL C78 (continued)
FIM/ASSISTANCE
DATA
EXPECTED FUNCTIONAL OUTCOMES EQUIPMENT EXP MED
LEVEL T19
Functionally relevant muscles innervated: Intrinsics of the hand including thumbs, internal and external intercostals,
erector spinae, lumbricals, flexor/extensor/abductor pollicis
Movement possible: Upper extremities fully intact, limited upper trunk stability. Endurance increased secondary inner-
vation of intercostals.
Patterns of Weakness: Lower trunk paralysis. Total paralysis lower extremities
FIM/Assistance Data: Exp Expected FIM Score
Med NSCISC Median
NSCISC Sample Size: FIM 15 Assist 12
FIM/ASSISTANCE
DATA
EXPECTED FUNCTIONAL OUTCOMES EQUIPMENT EXP MED
TABLE 5.3
Expected Functional Outcomes, LEVEL T19 (continued)
FIM/ASSISTANCE
DATA
EXPECTED FUNCTIONAL OUTCOMES EQUIPMENT EXP MED
Bladder Independent 6 6
Bed Mobility Independent Full to king standard bed
Bed/Wheelchair Independent May or may not require transfer board 67 6
Transfers
Pressure Relief/ Independent Wheelchair pressure-relief cushion
Positioning Postural support devices as indicated
Pressure-relief mattress or overlay may
be indicated
Eating Independent 7 7
Dressing Independent 7 7
Grooming Independent 7 7
Bathing Independent Padded tub transfer bench or 67 6
shower/commode chair
Handheld shower
Wheelchair Independent Manual rigid or folding lightweight 6 6
Propulsion wheelchair
Standing/ Standing: Independent Standing frame
Ambulation Ambulation: Typically not functional
Communication Independent
Transportation Independent in car, including loading Hand controls
and unloading wheelchair
Homemaking Independent with complex meal prep
and light housecleaning; total to
some assist with heavy housekeeping
Assist Required Homemaking: 3 hours/day 3* 3*
LEVEL T10L1
Functionally relevant muscles innervated: Fully intact intercostals, external obliques, rectus abdominis
Movement possible: Good trunk stability
Patterns of Weakness: Paralysis of lower extremities
FIM/Assistance Data: Exp Expected FIM Score
Med NSCISC Median
NSCISC Sample Size: FIM 15 Assist 12
FIM/ASSISTANCE
DATA
EXPECTED FUNCTIONAL OUTCOMES EQUIPMENT EXP MED
TABLE 5.3
Expected Functional Outcomes, LEVEL T10L1 (continued)
FIM/ASSISTANCE
DATA
EXPECTED FUNCTIONAL OUTCOMES EQUIPMENT EXP MED
Bed/Wheelchair Independent 7
Transfers
Pressure Relief/ Independent Wheelchair pressure-relief cushion
Positioning Postural support devices as indicated
Pressure-relief mattress or overlay
may be indicated
Eating Independent 7 7
Dressing Independent 7 7
Grooming Independent 7 7
Bathing Independent Padded tub transfer bench 67 6
Handheld shower
Wheelchair Independent all indoor and Manual rigid or folding lightweight 6 6
Propulsion outdoor surfaces wheelchair
Standing/ Standing: Independent Standing frame
Ambulation Ambulation: Functional, some assist Forearm crutches or walker
to independent Knee, ankle, foot orthosis (KAFO)
Communication Independent
Transportation Independent in car, including loading Hand controls
and unloading wheelchair
Homemaking Independent with complex meal
prep and light housecleaning; some
assist with heavy housekeeping
Assist Required Homemaking: 2 hours/day 2* 2*
LEVEL L2S5
Functionally relevant muscles innervated: Fully intact abdominals and all other trunk muscles; depending on level,
some degree of hip flexors, extensors, abductors, adductors; knee flexors, extensors; ankle dorsi flexors, plantar flexors
Movement possible: Good trunk stability. Partial to full control lower extremities
Patterns of Weakness: Partial paralysis lower extremities, hips, knees, ankle, foot
FIM/Assistance Data: Exp Expected FIM Score
Med NSCISC Median
NSCISC Sample Size: FIM 15 Assist 12
FIM/ASSISTANCE
DATA
EXPECTED FUNCTIONAL OUTCOMES EQUIPMENT EXP MED
TABLE 5.3
Expected Functional Outcomes, LEVEL L2S5 (continued)
FIM/ASSISTANCE
DATA
EXPECTED FUNCTIONAL OUTCOMES EQUIPMENT EXP MED
Patients with a C4 NLI may be able to breathe without munity mobility, at the C78 levels, a manual chair is typ-
a ventilator. They are generally dependent for all other ically used.
functional activities. Individuals with paraplegia have the potential to be
At the C5 NLI, individuals need less assistance for independent in all self-care activities, as well as with
activities of daily living. For some activities, such as eat- bowel and bladder management. The amount of assis-
ing and upper-extremity dressing, they may require assis- tance required for housekeeping declines with a more cau-
tance with setup but then are able to complete tasks. At dal NLI. Finally, ambulation is dependent on the level of
this level, the use of specialized adaptive devices becomes NLI. Patients with NLI above T10 typically do not ambu-
critical in determining the level of assistance required to late in a functional manner. At the T10L2 levels, func-
perform various dressing, eating, and grooming activities. tional ambulation is possible but will probably entail a
The use of a manual chair with handrim projections is a high energy cost if a swing-through gait pattern is
possibility at this level, although some assistance will required. Thus, at these levels, even an individual capa-
likely be required for propulsion outdoors, up inclines, or ble of walking may still elect to use a wheelchair as a more
on a rough surface. efficient means of mobility.
At the lower cervical levels, C6 and C78, individ- Because expected functional outcomes are general-
uals will still likely need assistance with bowel and blad- ized, it is important for providers and patients to be alert
der management, but they have the capability to be inde- to individual circumstances that may limit the realization
pendent with nearly all other functional activities (with of the stated outcomes. Patient motivation and training
appropriate adaptive equipment). Although patients with play a large role in the ability to attain a certain functional
a C6 level may still need a power wheelchair for com- level. Additionally, physical limitations or concomitant
OUTCOMES FOLLOWING SPINAL CORD INJURY 101
conditions may limit function. For example, patients with A reciprocal gait pattern can be utilized when there
a NLI of C6 can typically attain independence in sliding is pelvic control with at least 3/5 strength in the hip flex-
board transfers. If, however, there is an elbow flexion con- ors and in one quadriceps (16). This gait allows for knee
tracture, independence will be delayed until full elbow stability without the use of a knee-ankle-foot orthosis
extension is achieved. Similarly, whereas patients with (KAFO). Although a reciprocal gait pattern requires less
paraplegia can become independent in dressing activities, energy than a swing-through pattern, the rate of energy
they will be limited if they do not have adequate range of expenditure is still higher than that demonstrated by able-
motion in the hamstrings. In addition to conditions that bodied subjects.
may limit range of motion (i.e., contractures, joint defor- When the population of individuals with SCI is stud-
mities, heterotopic bone), other factors that can affect ied as a whole, experts generally agree that a minority of
achievement of optimal function include patient age, level individuals are able to ambulate following SCI. The pro-
of physical conditioning and potential for conditioning, portion of patients who are able to ambulate following a
length of time since injury, family support, spasticity, obe- SCI does, however, vary with the level and completeness
sity, and other medical complications. of injury. Daverat and co-workers reported that 28 per-
cent of 157 patients with SCI were functional walkers
1 year following injury (17). Of those with cervical lesions,
Ambulation
24 percent were functional walkers compared to 38 per-
A primary concern of SCI patients (and their families) is cent in the individuals with paraplegia. Maynard and asso-
whether they will be able to walk following an injury. ciates reported that among patients with tetraplegia, 47
Ambulation, like other functional outcomes, is dependent percent of those with incomplete sensory deficit and 87
on many factors in addition to neurologic function. In percent of those with incomplete motor deficit were able
addition to the basic question of whether a patient will to walk (18). Burns and colleagues studied 105 patients
be able to ambulate or will rely solely on a wheelchair, with ASIA C or D tetraplegia and found that all patients
there is the complicating factor of the degree of ambula- with ASIA D tetraplegia were able to ambulate at discharge
tory function that can be attained. from inpatient rehabilitation. Among individuals with
Stauffer divided ambulatory status into four cate- ASIA C-type injuries, those younger than 50 years had a
gories: community ambulatory, household ambulatory, better prognosis for ambulation than those older than 50
exercise ambulatory, and nonambulatory (14). Commu- years (91 percent versus 42 percent, respectively) (19).
nity ambulators are able to transfer themselves out of bed Crozier estimated that 20 percent of patients who are
or a wheelchair and walk reasonable distances in and out motor complete and sensory incomplete are ambulatory
of the home without assistance from another person. (20). Waters and colleagues determined that only 5 per-
Household ambulators may or may not require assistance cent of patients with complete paraplegia, 76 percent of
with transfers from bed or wheelchair; they are able to those with incomplete paraplegia, and 46 percent of those
ambulate in the home with relative independence, but are with incomplete tetraplegia were able to attain community
unable to ambulate outside of the home for any signifi- ambulation at 1 year following injury (5,6,8). No patients
cant distance. These individuals frequently use a wheel- with complete tetraplegia were able to ambulate.
chair for mobility outside the home. The exercise ambu- Hussey and Stauffer determined that there is a direct
lator attains functional mobility with a wheelchair and relationship between lower extremity strength and the
can ambulate only under closely controlled conditions. ability to ambulate (16). They reported that pelvic con-
Considerable physical assistance is also required to ambu- trol with at least fair hip extensor strength and at least
late. Individuals who are nonambulators rely exclusively fair strength in one knee extensor is required for com-
on a wheelchair. Both community and household ambu- munity ambulation with a reciprocal gait pattern.
lation are considered functional ambulation, whereas Crozier and colleagues reported on two measures
exercise ambulation is considered nonfunctional. that they found to be predictive of ability to ambulate
The type of gait pattern utilized depends on the (20). These investigators performed initial neurologic
degree of neurologic loss. A swing-through gait pattern examinations 72 hours following injury and found that
requires arm and shoulder girdle strength sufficient to lift among individuals with motor complete injuries, those
the weight of the entire body and swing it forward. This who had preserved pinprick as well as light touch sensa-
type of gait pattern requires a much higher rate of energy tion had an excellent prognosis for ambulation com-
expenditure and results in a substantially slower walk- pared to those with preservation of only light touch.
ing velocity (15). Thus, although an individual may uti- In another study, Crozier and coworkers studied
lize a swing-through gait pattern to negotiate architec- individuals with Frankel C (motor useless) injuries
tural barriers or to walk for exercise or psychologic (21). They studied recovery of the quadriceps muscle and
reasons, few employ this manner of ambulation as their reported that all patients who had achieved a quadriceps
primary mode of mobility. strength of at least 3/5 at 2 months following injury pro-
102 INTRODUCTION
Finally, although motor function affects functional plete cervical spinal cord injured patients. Arch Phys Med Reha-
bil 1990; 71:562565.
outcomes following SCI, self-reported quality of life is not 12. Outcomes Following Traumatic Spinal Cord Injury: Clinical Prac-
necessarily affected. Therefore, although prognosis of tice Guidelines for Health-Care Professionals. Consortium for
functional outcomes is a key factor in setting realistic Spinal Cord Medicine. Paralyzed Veterans of America, 1999.
13. Graves DE, Frankiewicz RG, Carter E. Gain in functional ability
goals and implementing effective rehabilitation programs, during medical rehabilitation as related to rehabilitation process
psychosocial needs and expected outcomes must also be indices and neurologic measures. Arch Phys Med Rehabil 1999;
considered to plan any needed interventions to maximize 80:14641470.
14. Stauffer ES. Study of 100 Paraplegics. Rancho Los Amigos Papers,
a patients life satisfaction. 1968.
15. Waters RL, Lunsford BR. The energy cost of paraplegic locomo-
tion. JBJS 1985; 67A:12451250.
References 16. Hussey RW, Stauffer ES. Spinal Cord Injury: Requirements for
ambulation. Arch Phys Med Rehabil 1973; 54:544547.
1. The National SCI Statistical Center. Spinal Cord Injury: Facts and 17. Daverat P, Sibrac MC, Dartigues JF, Mazaux JM, Marit E,
Figures at a Glance. Birmingham: University of Alabama at Birm- Debelleix X, Barat M. Early prognostic factors for walking in
ingham National SCI Center, April 1999. spinal cord injuries. Paraplegia 1988; 26: 255261.
2. Brown PJ, Marino RJ, Herbison GJ, et al. The 72-hour examina- 18. Maynard FM, Reynolds GG, Fountain S, et al. Neurologic prog-
tion as a predictor of recovery in motor complete quadriplgia. nosis after traumatic quadriplegia. J Neurosurg 1979;
Arch Phys Med Rehabil 1991; 2:546548. 50:611616.
3. Herbison GJ, Zerby SA, Cohen ME, et al. Motor power difference 19. Burns SP, Golding DG, Rolle WA, Graziani V, Dittuno JF. Recov-
within the first two weeks post-SCI in cervical spinal cord quad- ery of ambulation in motor incomplete tetraplegia. Arch Phys Med
riplegic subjects, J Neurotrauma 1991; 9:373380. Rehabil 1997; 78:11691172.
4. Mange KC, Ditunno JF, Herbison GJ, et al. Recovery of strength 20. Crozier KS, Graziani V, Ditunno JF, Herbison GJ. Spinal cord
at the zone of injury in motor complete and motor incomplete cer- injury: Prognosis for ambulation based on sensory examination
vical spinal cord injured patients. Arch Phys Med Rehabil 1990; in patients who are initially motor complete. Arch Phys Med
71:562565. Rehabil 1991; 72:119121.
5. Waters RL, Yakura JS, Adkins RH, Sie I. Recovery following com- 21. Crozier, KS. Cheng LL, Graziani V, Zorn G, Herbison G, Ditunno
plete paraplegia. Arch Phys Med Rehabil 1992; 73:784-789. JF. Spinal cord injury: Prognosis for ambulation based on quadri-
6. Waters RL, Yakura JS, Adkins RH, Sie I. Motor and sensory recov- ceps recovery. Paraplegia 1992; 30:762767.
ery following incomplete paraplegia. Arch Phys Med Rehabil 22. Waters RL, Adkins R, Yakura J, Vigil D. Prediction of ambulatory
1994; 75:67-72. performance based on motor scores derived from standards of the
7. Waters RL, Yakura JS, Adkins RH, Sie I. Motor and sensory recov- American Spinal Injury Association. Arch Phys Med Rehabil
ery following complete tetraplegia. Arch Phys Med Rehabil 1993; 1994; 75:756760.
4:242247. 23. Waters RL, Yoshida GM. Prognosis of spinal cord injuries. In:
8. Waters RL, Yakura JS, Adkins RH, Sie I. Motor and sensory recov- Levine AM (ed.), Orthopaedic Knowledge Update, Trauma.
ery following incomplete tetraplegia. Arch Phys Med Rehabil American Academy of Orthopaedic Surgeons, 303310, 1996.
1994; 75:306311. 24. Whiteneck GG, Charlifue SW, Gerhart KA et al. Quantifying
9. Marino RJ, Dittuno JF, Donovan WH, Maynard F. Neurologic handicap: A new measure of long-term rehabilitation outcomes.
recovery after traumatic spinal cord injury: Data from the model Arch Phys Med Rehabil 1992; 73:519526.
spinal cord injury systems. Arch Phys Med Rehabil 1999; 25. Dijkers M. Quality of life after spinal cord injury: A meta-analy-
80:13911396. sis of the effects of diablement components. Spinal Cord 1997;
10. American Spinal Injury Association/International Medical Society 35:829840.
of Paraplegia (ASIA/IMSOP). International Standards for Neu- 26. Tate DG, Stiers W, Daugherty J et al. The effects of insurance ben-
rologic and Functional Classification of Spinal Cord Injury, efits coverage on functional and psychosocial outcomes after
Revised 1996. Chicago, IL: ASIA. spinal cord injury. Arch Phys Med Rehabil 1994; 75:407414.
11. Mange KC, Dittuno JF, Herbison GJ, Jaweed MM. Recovery of 27. Diener E, Emmons RA, Larson RJ, et al. The satisfaction with life
strength at the zone of injury in motor complete and motor incom- scale. J Personality Assessment 1985; 49:7174.
This Page Intentionally Left Blank
II
Log-roll technique. One provider (designated log-roll leader) Log-roll technique. On leaders command, all three providers
at the head of the patient controls the head and cervical spine. roll patient in a single, fluid motion (carefully avoiding any
Meanwhile, two assistants with interlinked arms control trunk twisting motion). The fourth provider evaluates the thoracic
and lower body. and lumbar/sacral spine for tenderness.
nary retention, priapism, or loss of anal sphincter tone stabilization. Many authors have demonstrated that oro-
should be noted. Skin temperature and appearance should tracheal intubation with in-line stabilization (Figure 6.3)
also be evaluated. Warm, flushed skin suggests loss of is a safe method if performed by experienced personnel
sympathetic vascular tone below the injury. Even in the (57). In a cadaver model, Gerling et al. showed that intu-
absence of any of the above findings, the multi-trauma or bation with in-line stabilization did not cause any signif-
major trauma mechanism patient must be placed in a rigid icant vertebral body movement. Intubation attempts with
collar and backboard to be immobilized for transport. cervical collar immobilization, however, did produce a
significant amount of vertebral distraction. Thus, the cer-
vical collar should always be opened prior to attempted
RESUSCITATION intubation. If the airway cannot be secured following
three intubation attempts, the prehospital provider should
Resuscitation begins during the evaluation and primary be prepared to perform a surgical airway.
survey. Airway control is of the highest priority and Prehospital circulation resuscitation consists of
should start with the immediate application of oxygen aggressive intravenous access and proper fluid resuscita-
while the cervical spine is being immobilized. Initial air- tion. The cervical SCI patient may present with either neu-
way management includes the basic maneuvers of chin- rogenic or hemorrhagic shock. Neurogenic shock is the
lift, jaw thrust, and the placement of a nasal or oral air- result of an SCI at or above the fourth thoracic vertebra.
way. Suction should be available to remove blood, These injuries cause a loss of sympathetic peripheral vas-
secretions, and possible foreign bodies. If adequate oxy- cular tone and thus reduce central venous return. There
genation cannot be maintained and the paramedic crew is also an associated bradycardia caused by loss of sym-
is properly trained, the patient should be prepared for pathetic innervation to the heart and unopposed
intubation. Both blind nasotracheal intubation (assum- parasympathetic tone. This clinical picture is in contrast
ing the patient has no evidence of midface trauma) or oro- to the tachycardia associated with hemorrhagic hypoten-
tracheal intubation are considered appropriate, assuming sion. Both hemorrhagic and neurogenic shock may be ini-
that they are performed with proper in-line cervical spine tially treated by placing the patient in a spine-immobi-
PREHOSPITAL MANAGEMENT OF SPINAL CORD INJURED PATIENTS 109
Helmet Removal
lized Trendelenburg position (8). This maneuver helps to The on-site management of a neck-injured patient with
decrease the lower extremity pooling of blood and a helmet is different from that of other traumatic cervi-
increases central return. Bleeding from the neck or spinal cal spine injuries. Although the helmet causes the patients
area should be controlled by direct pressure. Large-bore neck to be slightly flexed (especially in the absence of
peripheral intravenous lines should be placed in antici- shoulder pads) and may be concealing life threatening
pation of saline and blood infusion. hemorrhage, its removal is not required in the field unless
airway problems exist. If the helmet must be removed for
airway access, the two-person technique recommended
IMMOBILIZATION by the American College of Surgeons should be used. One
provider maintains in-line immobilization, while the other
To prevent further SCI, all prehospital personnel must be provider gently removes the helmet (12).
well trained in the techniques of immobilization. All major
trauma victims, patients complaining of neck pain or neu-
Immobilizing the Injured Athlete
rologic symptoms, and any patient with altered mental sta-
tus of uncertain cause must be immobilized. Within these Unique management issues can arise when caring for the
liberal guidelines, cervical spine immobilization has injured athlete. They include on-field evaluation, immo-
become one of the most frequently performed prehospital bilization techniques, and removal of protective equip-
procedures (9). It is estimated that nearly 5 million patients ment. The first step in evaluating the athlete with a poten-
receive spinal immobilization annually, at a cost of $15 per tial cervical spine injury is the on-field evaluation. The
person (10). In addition to the cost, immobilization causes unconscious athlete should be carefully log-rolled into a
patient discomfort and increased paramedic scene time. supine position. The mouthpiece should be removed while
Recently Hauswald et al. (11) examined the effect of emer- the airway, breathing, and circulation are assessed. Pro-
gency out-of-hospital spinal immobilization on neurologic tective equipment (e.g., helmet and shoulder pads) should
injury by comparing trauma patients in Malaysia (no pre- be left in place until adequate immobilization of the head
hospital emergency medical services) with a group of and neck has occurred (13). Multiple studies have demon-
trauma patients in New Mexico (with prehospital spinal strated that immobilizing the neck-injured football player
immobilization.) Suprisingly, the unimmobilized with only the helmet or only the shoulder pads in place
Malaysian patients were found to have a lower rate of neu- causes significant cervical spine malalignment (1416). If
rologic disability. Although this study is intriguing, the protective equipment must be removed in the prehospital
110 ACUTE SPINAL CORD INJURY MANAGEMENT AND SURGICAL CONSIDERATIONS
Technique of Immobilization
Because SCIs are likely to involve multiple levels, it is
imperative to immobilize the entire spine. The best FIGURE 6.5
method is to use a rigid board. The neck is first secured
with a rigid collar and sandbags are placed on each side Properly immobilized patient.
of the head. Alternatively, some paramedics prefer to use
a prefabricated immobilization kit with its own cushions
and sandbag equivalents (Figure 6.4). Tape and Velcro and transportation. The scene commander must take into
straps are placed over the patients forehead to prevent account geography, weather, man-made obstacles (pow-
flexion of the neck. It important to note that cervical col- erlines), distance to the designated trauma center, and other
lars alone are relatively ineffective in minimizing neck unique aspects of the accident scene. Some individual acci-
motion (17). Collars must be combined with taping to dent scenes will be easily accessible by EMS flight crews,
limit both flexion and extension (18,19). Following whereas other sites will be more rapidly accessed by
proper neck immobilization, the chest and abdomen are ground transport. As in all trauma situations, the com-
immobilized with seatbelts tightly fastened, yet allowing mander must aim to quickly stabilize the SCI patient, while
for inspiration (Figure 6.5). arranging for the most rapid transport to a trauma center.
Proper prehospital immobilization, resuscitation, and
transportation not only prevents further SCI, but maxi-
EXTRICATION AND TRANSPORTATION mizes the possibility of neurologic recovery.
Once the patient has been properly immobilized, the pre- References
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ball equipment: A cadaveric cervical spine injury model. Ann
Emerg Med 1998; 32:411417.
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7
Acute Medical
Management of Spinal
Cord Injury
he medical treatment of spinal cord normal rotation, and 65 percent of normal lateral bend-
care should be taken to avoid vertebral column move- necrotic tissue. These two phases of the cellular inflam-
ment. When there is radiologic evidence of vertebral col- matory response are implicated in the axonal demyeli-
umn bony or ligamentous displacement, the goal of man- nation that starts within 24 hours after injury and
agement is the realignment and fixation of the involved increases for several weeks (13).
segments. Indications for surgery include an unstable frac-
ture, a fracture that will not reduce with spinal traction,
gross spinal misalignment, evidence of cord compression CORTICOSTEROID THERAPY
in the presence of an incomplete injury, deteriorating neu-
rologic status, and persistent instability following con- Corticosteroids have been used in neurotrauma based on
servative management (57). in-vitro and animal studies that demonstrated their the-
The timing of surgical stabilization is controversial. oretical ability to stabilize membranes, inhibit lipid per-
Some favor early surgical stabilization because it has been oxydation, suppress vasogenic edema by restoring the
demonstrated that systemic complications in patients with blood-central nervous system (CNS) barrier, enhance the
SCI decrease if surgery is performed within 24 hours of spinal cord blood flow, inhibit pituitary endorphin
injury (8). Advocates of delayed surgery prefer to perform release, and attenuate the inflammatory response
a well-planned procedure on patients who are medically sta- (23,2528). Steroid therapy was chosen for clinical inves-
ble and whose neurologic status has reached a plateau (9). tigation in humans based on the extensive preclinical data
supporting its use (29). Methylprednisolone sodium suc-
cinate (MPSS) has been used in three randomized,
PHARMACOLOGIC MANAGEMENT prospective double-blinded multicenter clinical trials in
OF SPINE INJURIES patients with acute SCI. This agent is less likely to pro-
duce neutropenia and crosses cell membranes more
Aside from the mechanical factors that compromise spinal rapidly and completely than other steroids (26,30).
cord function, local tissue perfusion and oxygenation can In 1979, the first National Acute Spinal Cord Injury
profoundly modulate the extent of injury resulting from Study (NASCIS I) was begun and results were reported in
any given mechanical stress (10). Other processes that 1984. This study compared the efficacy of administering
take place at the site of injury such as edema, hemorrhage, 100 mg versus 1,000 mg of MPSS each day for a total of
axonal degeneration, or demyelination ultimately alter 10 days to patients with any motor or sensory deficit
the cellular bioenergetic mechanisms. Damage of the cord below the level of injury. The study found no difference
vessels produces leakage of plasma protein and leads to in the recovery of motor or sensory function between the
cord edema (11, 12) that will further diminish local spinal two groups at 6 weeks, 6 months, and 1 year after injury
cord blood flow (13). Mechanical injury or systemic coag- (31). However the beneficial or detrimental effects of
ulation abnormalities may lead to petechial hemorrhages steroid therapy could not be determined because of the
in the cord and thus impair spinal perfusion (11). Focal lack of a placebo group in the study protocol. Addition-
ischemia is compounded by systemic hypoxia or hypoten- ally, a trend towards a higher risk of complications was
sion, because autoregulation is lost in the region of injury noted in the high-dose steroid group.
and blood flow changes more directly with alterations in Between 1985 and 1988, the NASCIS II trial was
systemic hemodynamics (1012,14,15). At the cellular conducted, with the final year follow-up examination per-
level, the initial ischemic process is followed by the devel- formed in 1990. In this study, patients were randomized
opment of toxic levels of excitatory amino-acids (EAA) to receive MPSS (30 mg /kg bolus, then 5.4 mg/kg/h infu-
(16,17) an eight-time increase in the glutamate levels sion for 23 hours), naloxone (5.4 mg/kg bolus, then
(18,19), depletion of ATP stores (1820), and sustained 4mg/kg/h infusion for 23 hours), or placebo. Patients who
elevation of cytosolic calcium concentration, the final received a high-dose MPSS infusion within 8 hours of
pathway mediating cell death (16,21,22). Other processes spinal cord injury had better neurologic recovery at 6
that potentiate cell damage include an alteration of the weeks, 6 months, and after 1 year following injury when
ratio between thromboxane and prostacycline produc- compared with placebo or naloxone treated patients (32).
tion, lipid peroxydation and free radical production Those who received MPSS after 8 hours had worse neu-
(23,24), and the production of endogenous opioids and rologic function than the placebo group. This study has
pro-inflammatory cytokines. been criticized for deficiencies in experimental design and
Histopathologically, the region of injury demon- incomplete data reporting. The number of subgroups of
strates signs of inflammation caused by an early neu- patients treated within 8 hours could produce a statisti-
trophil invasion. These neutrophils release lytic enzymes cally significant result by chance alone (33). The initial
that further damage vascular, neuronal, and glial cells resuscitation protocols varied with each center, no func-
(25). This is followed by macrophage penetration, thus tional assessment scale was used as an outcome measure,
precipitating the phagocytosis of hemorrhagic and and no analysis of individual muscle group function to
ACUTE MEDICAL MANAGEMENT OF SPINAL CORD INJURY 115
determine the region of drug-induced improvement was ing wound infections, pneumonia, and gastrointestinal
performed (34). In 1995, two retrospective studies were bleeding. Careful observation for the development of
published and both failed to demonstrate improvement these complications and prompt initiation of treatment
in neurologic function after administration of MPSS in is essential.
SCI patients (35,36). Although all patients involved in
NASCIS II had blunt SCIs, the study results were inap-
21-Aminosteroids (Lazeroids)
propriately extrapolated by practicing clinicians to pen-
etrating injury, radicular, and cauda equina lesions. In The best-studied compound from this group of medica-
1997, a retrospective review of patients with gunshot tion is tirilazad mesylate, a 21-aminosteroid with potent
wounds to the spine treated with intravenous steroids antioxidant effects. In the NASCIS III trial, patients
demonstrated no neurologic benefit (37). The conse- receiving a 48-hour infusion of tirilazad had patterns of
quences of high-dose steroid therapy, particularly the rate motor recovery similar to those receiving MPSS infusion
of infectious complications, were analyzed and the inci- for 24 hours, but with fewer side effects (41). However,
dence of pneumonia was demonstrated to be 4 times because an initial bolus of MPSS was administered in
higher during the acute phase of spinal cord injury and patients who received TM, the role of TM in SCI is not
2.6 times higher overall, although this finding did not well defined (41).The 21-aminosteroids lack mineralo-
reach statistical significance (38). corticoid or glucocorticoid activity, but are more potent
In 1997, the third NASCIS was reported. This study inhibitors of iron-dependent lipid peroxydation than
analyzed the effects of the duration of MPSS therapy. All methylprednosolone (44). Tirilazad mesylate was well tol-
patients received MPSS 30 mg/kg via intravenous bolus, erated during NASCIS III trial. The observed secondary
and then patients were randomized to receive an infu- effects were nausea, abdominal pain, back ache, muscle
sion of MPSS at 5.4 mg/kg/h for 24 hours or 48 hours, twitching, and cramps (43). At present, MPSS and TM
or an infusion of tirilazad mesylate (48 TM), a lipid per- could be considered a reasonable alternative to conven-
oxydase inhibitor, for 48 hours. This trial was prompted tional MPSS treatment.
by the information that tirilazad offered superior free rad-
ical scavenger efficacy with little glucocorticoid activity
GM1 Ganglioside
(39). High doses of glucocorticoids might interfere with
neuronal protection by inhibiting immune cellular activ- Gangliosides are sialic acidcontaining glycolipids in cell
ity, thus exacerbating acute postischemic neuronal necro- membranes. These glycolipids promote neuronal sprout-
sis, and inhibiting axonal sprouting. The investigators ing, outgrowth, and synaptic transmission (13,42). A
acknowledged the fact that the timing of steroid therapy prospective, randomized, placebo-controlled, double-
may be a critical element in its ultimate efficacy. The blinded trial of GM-1 use in eighteen SCI patients showed
mechanism of lipid peroxydation inhibition may be lost that GM-1 enhances recovery of neurologic function at
if MPSS is administered more than 8 hours after the spine 1 year following injury when compared to patients receiv-
injury (29,30). ing placebo. The drug was administered within 48 hours
NASCIS III found that patients who were treated after injury and was continued for approximately 26
within 3 hours of injury had similar recovery at 6 weeks, days. The dose used in this trial was the highest approved
6 months, and after 1 year, whether they were treated for by the FDA for investigation in the United States. The sec-
24 or 48 hours. Patients whose treatment with MPSS ond multicenter GM-1 trial, enrolling hundreds of
started between 3 and 8 hours of injury had better motor patients, has yet to be published (34). Gangliosides can
and functional recovery if the infusion was continued for block some of the neuroprotective effects of MPSS by
48 hours. Patients started on MPSS more than 8 hours modulating the protein-kinase activity that inhibits
after injury did worse, in terms of neurologic outcome, lipocortins. Lipocortins are known for mediating the anti-
than the placebo group in NASCIS II trial, perhaps inflammatory effects of methylprednisolone (27). During
because of the lack of protective effect against lipid per- the early European use of this drug, a link with the devel-
oxidation (40). The conclusions of the NASCIS III trial opment of GuillainBarr syndrome was implicated, but
were that MPSS therapy, when started within 3 hours of further study failed to determine a clear association
spine injury, should be continued for 24 hours, and when (44,45). Other secondary effects include rash and pain
initiated between 3 and 8 hours should be continued for at the injection site (45).
48 hours unless there are complicating medical factors.
This protocol has gained widespread acceptance in clin-
Opiate Antagonists
ical practice, in part from medico-legal reasons and also
because steroids are a reasonable therapeutic idea with It is well documented that the local release of endogenous
treatable adverse effects. It must be appreciated that opioid peptides occurs after spine trauma, and there are
steroid therapy is associated with systemic effects includ- theoretical concerns regarding their contribution to sec-
116 ACUTE SPINAL CORD INJURY MANAGEMENT AND SURGICAL CONSIDERATIONS
ondary injury through an opioid receptormediated should have adequate volume resuscitation, as reflected
mechanism (42). The and opioid receptors have been by their filling pressures and, if needed, vasopressor ther-
found to be the mediators of the pathophysiologic mech- apy. The ideal MAP necessary to maximize spinal cord
anisms implicated in cord injury, but precisely which opi- perfusion after acute injury is not known, but a high nor-
oids and which receptors are involved remains the sub- mal MAP range of 80 to 100 mmHg has been selected
ject of debate (42). Endogenous opioids released in based on clinical experience with patients suffering cere-
response to shock alter the autonomic tone by inhibiting bral ischemia. Intensive care unit monitoring is a priority,
the dopaminergic system and produce depression of the because pulmonary edema from aggressive volume reple-
cardiovascular system. Opioid antagonists, therefore, tion must be avoided.
may prevent this systemic hypotension, thus improving
spinal cord microcirculation (48,49). In the NASCIS II
trial, naloxone initially failed to demonstrate any clini- AIRWAY MANAGEMENT
cal benefit compared with placebo, but subsequent
reanalysis of the data indicated that naloxone improved Airway management in patients with cervical spine (c-
functional recovery when administered within 8 hours spine) injury requires skill and expertise. Alert trauma
of injury (26,30). Further studies are needed to define the patients who report neck pain or tenderness may be eval-
appropriate drug dosing schedule and the optimal tim- uated for SCI. Those who are alert and do not complain
ing of opiate antagonist therapy. of neck pain or tenderness should not have cervical injury
Other pharmacologic agents have been used in and should not require further c-spine evaluation, neck
experimental SCI, but their clinical efficacy is still to be immobilization, or special precautions during airway
proved. Excitatory amino acid receptor antagonists, management. These are very stringent criteria; and if there
specifically n-methyl-d-aspartate (NMDA) GK 11, are is the slightest neck discomfort, depressed consciousness,
currently under evaluation (40). The administration of or other painful injuries, c-spine precautions must be
calcium channel antagonists after SCI is controversial maintained until the absence of injury is demonstrated.
(21). Thyrotropine-releasing hormone (TRH), platelet- SCI patients require establishment of a definitive
activating factor antagonists, free radical scavengers, and endotracheal airway in the following circumstances: a)
4-aminopyridine continue to be investigated as potential diminished or absent airway protective mechanisms
treatments of acute SCI (40,42,45). because of coexistent intracranial injury or other pathol-
ogy; b) evidence of airway obstruction in the context of
multiple trauma; c) acute respiratory failure in patients
AXONAL REGENERATION with injuries at or above C4 who lack diaphragmatic
function and in patients with coexistent thoracoabdom-
Experimental studies in neurobiology are exploring the inal trauma; d) inability to cough, clear secretions, and
potential of an injured spinal cord for regrowth. Embry- participate in bronchial hygiene maneuvers secondary to
onic spinal cord transplantation (50), transplantation of the reductions in forced expiratory volume and forced
Schwann cells (51), monoclonal antibodies against myelin vital capacity that develop with loss of intercostal and
(52), and gene therapy that promotes axonal regenera- abdominal muscle activity. Once a cervical injury is sus-
tion (53) are promising studies that open a new chapter pected, the vertebral column should be immobilized.
in the management of SCI. Options for relieving upper airway obstruction
include chin lift, jaw thrust continuous positive airway
pressure, or nasal or oral airway insertion. The use of chin
MINIMIZING THE SECONDARY INJURY lift and jaw thrust is discouraged in the setting of poten-
tial spinal instability, because this maneuver may cause a
The inciting events that lead to SCI are followed by a more than 5 mm widening of the disk space and C56
series of detrimental hemodynamic and biochemical instability (54). This instability is not diminished by a
processes that can be mitigated by early and aggressive rigid collar. On the other hand, oral or nasopharyngeal
medical management. Below the level of injury, the spinal airway insertion produces minimal cervical spine move-
cord is often hypoperfused because of the associated neu- ment. Bag and mask ventilation provides oxygenation but
rogenic shock, hemorrhage, vasospasm, or thrombosis. is not a long-term airway control method.
The posttraumatic hemorrhage and edema is followed by Endotracheal intubation should be performed while
axonal and neuronal necrosis (14). The goal in treating maintaining an immobile spine. One method to immobi-
post SCI hypotension is to restore the mean arterial pres- lize the neck during laryngoscopy and oral tracheal intu-
sure (MAP) to normal levels. Hypertension should be bation is manual in-line axial stabilization (MIAS). Dur-
avoided because of the theoretic risk of enhancing ing direct laryngoscopy, the cervical movement is
intramedullary hemorrhage and edema (14). All patients concentrated at the occipitoatlantoaxial complex and, in
ACUTE MEDICAL MANAGEMENT OF SPINAL CORD INJURY 117
normal patients and volunteers, there is minimal move- ization, more commonly using the fiberoptic technique,
ment below C3 (5557). Thus, patients with unstable C1 or after administering intravenous anesthetics and mus-
or C2 injuries might be the most vulnerable to neurologic cle relaxants. Before administering intravenous medica-
damage from atlanto-occipital extension. MIAS reduces tions, the physician must make sure that ventilation by
by 60 percent atlanto-occipital extension and flexion in the mask can be adequately performed. If mask ventilation
lower cervical spine. Sudden worsening of the neurologic and direct laryngoscopy are expected to be difficult or
deficits has been reported when traction is applied for spine there is coexistent facial trauma, the practitioner must
stabilization or to expose C7 on radiographs (58). Over be prepared to secure the airway using a surgical tech-
the past decade, there have been five reports of SCI fol- nique (e.g., cricothyroidotomy) (62).
lowing direct laryngoscopy and tracheal intubation (59). When manipulating the airway of an SCI patient,
Difficult or failed tracheal intubation is another dan- the practitioners goal must be to maintain adequate
ger of direct laryngoscopy in patients with potential SCI. spinal cord blood flow and spinal cord perfusion pressure
MIAS prevents complete alignment of the mouth and (SCPP). SCPP depends on MAP, cerebrospinal fluid (CSF)
glottis, and the person stabilizing the neck is in the way pressure, and the pressure in the spinal canal or dural sac.
of the laryngoscopist. Therefore, spine immobilization is The clinician should also remember that the autoregula-
associated with a high incidence of poor glottic visual- tion of blood flow to damaged neurologic tissue might be
ization during conventional laryngoscopy. Facial and absent (63), so SCPP becomes dependent on MAP. All the
neck trauma and the prevertebral swelling associated with intravenous anesthetic agents that are administered before
cervical spine injury can increase the difficulty of laryn- intubation have the potential to decrease the MAP to lev-
goscopy. Rigid fiberoptic devices (e.g., Bullard laryngo- els that compromise the SCPP; thus, judicious dosages
scope) have been successfully used to intubate the trachea adjusted to a patients specific hemodynamic profile
in patients with cervical spine injury; these devices require should be utilized. The CSF pressure can increase with
less neck movement than direct laryngoscopy using the alterations of the spinal cord curvature, thus decreasing
McIntosh or Miller laryngoscope blades (60). The inci- the SCPP by as much as 20 mmHg (64).
dence of grade 3 and 4 laryngeal views ranges from 22 In conclusion, maintaining the head and neck in neu-
to 34 percent, when using MIAS in paralyzed, anes- tral position; performing the intubation technique with
thetized patients and a McIntosh blade, to 64 percent which the practitioner is most comfortable; and assuring
when the patients cervical spine is immobilized using a adequate spinal cord perfusion pressure, proper oxy-
rigid collar, tape, and sand bags. genation, and ventilation are the primary goals to be
Another alternative to direct, rigid laryngoscopy is achieved while managing the airway of a SCI patient.
fiberoptic endoscopy using either the flexible or the rigid
scope. The fiberoptic method provides better views and
is associated with a lower degree of difficulty when com- RESPIRATORY MANAGEMENT
pared with direct rigid laryngoscopy; the rate of success-
ful intubation and the time to intubation is similar Spinal cord disease has a most profound impact on pul-
between groups (61). Fiberoptic tracheal intubation per- monary function. Pulmonary complications are the sin-
formed in an awake patient requires cooperation, a secre- gle most common cause of morbidity and mortality after
tion- and blood-free airway, a pharyngeal space unre- SCI and currently constitute the leading primary cause
stricted by edema, and adequate supra- and infraglottic of death (i.e., pneumonia ) (65). The level of cord injury,
anesthesia. Although this technique does not depend on the age of the patient, and the presence of pre-existing
atlanto-occipital extension, there is no documentation to medical problems all contribute to the mortality associ-
show that it minimizes neck movement. ated with SCI and pulmonary dysfunction.
Cricothyroidotomy is a rapid but invasive method Lumbar cord lesions have little or no effect on ven-
of controlling the airway. The procedure may be more dif- tilation or cough. Lesions of the thoracic cord can impair
ficult when the cervical spine is immobile. There are no cough, but have little effect on normal breathing. Cord
studies of neurologic outcome after cricothyroidotomy in injury at or above C5 can affect diaphragmatic function;
patients with cervical spine injuries (62). Transtracheal complete lesions at C3 or higher produce bilateral
ventilation is a temporary mode of ventilation and oxy- diaphragm paralysis. Obviously, this is incompatible with
genation; it does not protect against aspiration and does life in the absence of artificial ventilation. C45 lesions
not allow adequate hyperventilation. It may be difficult are associated with variable degrees of diaphragmatic dys-
to perform if the neck cannot be extended. Other tech- function. Lesions from C6 to T12 are typically charac-
niques available are blind nasotracheal intubation, light terized by an intact diaphragm, which can provide up to
wands, and retrograde intubation. 90 percent of tidal volume, but nonfunctional intercostals
Securing the airway in SCI patients can be per- that cannot stabilize the rib cage. When the accessory
formed with an awake patient after appropriate topical- muscles are used, the increase in the anteroposterior
118 ACUTE SPINAL CORD INJURY MANAGEMENT AND SURGICAL CONSIDERATIONS
diameter of the upper rib cage pulls the diaphragm cepha- pressure breathing, and therapy with beta agonists may
lad, and induces negative intra-abdominal pressure and be helpful, depending on the clinical situation. SCI
paradoxical breathing. The spontaneously breathing patients with cervical lesions have abnormal pulmonary
tetraplegic patient develops acute restrictive disease with dynamics during cough in the supine position. The most
a drop in total lung capacity (TLC), vital capacity (VC), effective method of producing a cough in this patient pop-
tidal volume (TV), functional residual capacity (FRC), ulation is a combination of positive pressure and assisted
mean inspiratory pressure (MIP), and mean expiratory cough (70,71).
pressure (MEP). There is an accompanying rise in resid- In summary, conscientious pulmonary assessment
ual volume (RV). and aggressive bronchial hygiene are paramount in pre-
The loss of FRC and the decrease in static lung com- venting and detecting pulmonary complications follow-
pliance is followed by the retention of pulmonary secre- ing acute SCI.
tions, increased ventilation perfusion (V/Q) mismatch,
and a poor ability to sigh and cough. Following the acute
SCI, while the patient is in spinal shock, oxygen diffusion CARDIOVASCULAR RESUSCITATION
capacity is impaired. This process can be accentuated by
the aggressive volume resuscitation that is often necessary, The physiologic and anatomic transection of the spinal
and it can result in impaired gas exchange and hypoxemia cord, followed by loss or depression of all or most spinal
(66). reflex activity below the level of the injury, is known as
Patients with lesions above C5 must usually be intu- spinal shock. This term usually implies temporary loss
bated and placed on mechanical ventilation. Even in the or depression of all spinal reflex activity below the level
absence of high cervical injury, the presence of respiratory of injury. Hypotension may be an accompanying sign,
difficulty requires intubation and full ventilatory support. depending on the level of injury, and is caused by a with-
Ventilation with TV of 15 to 20 cc/kg, rather than 5 to drawal of sympathetic tone. With the loss of the sympa-
10 cc/kg has been found to decrease complication rates thetic innervation, the unopposed parasympathetic tone
and shorten the time on the ventilator (66,67). Patients predominates (72). The vasodilatation, hypotension,
who retain diaphragmatic function can use some acces- decreased peripheral vascular resistance (PVR), decreased
sory respiratory muscles to augment respiration, but res- preload, and bradycardia are part of the clinical syndrome
piratory deterioration frequently develops gradually, of neurogenic shock (73,74). Because the sympathetic ner-
often between day 4 and 7 post injury (68). Pulmonary vous system (SNS) extends from T1 to L1, any SCI above
function must be closely monitored through serial VC, L1 is at risk for some degree of neurogenic shock. Patients
negative inspiratory force (NIF), and serial blood gas with injuries above the most cephalad sympathetic out-
analysis, as well. If the VC decreases to less than 15 ml/kg flow at T6 are most likely to exhibit profound autonomic
(in a 70 kg adult
1,000 ml) or the NIF falls below 20 changes. In this setting, a lack of activity in the T1T4
cm H2O, the patient will require intubation for bronchial cardioacceleratory fibers is responsible for bradycardia
hygiene needs. Acute respiratory failure development is (75). Although patients with blunt SCI are at risk for a
indicated by rising PCO2 and falling pH; these patients concomitant hemorrhagic injury, 70 percent of those with
will require intubation for mechanical ventilatory support hypotension have neurogenic shock. The hypotension
(66). Monitoring patients exclusively by pulse oximetry seen in neurogenic shock is rapidly responsive to cate-
is not adequate and capnography or frequent arterial cholamine infusion; in the clinical context of trauma this
blood gas analyses are generally required. Hypoxemia, hypotension should be differentiated from that of hem-
caused by retained bronchial secretions, or atelectasis can orrhagic shock. Compounding this scenario is the poten-
lead to pneumonia and may necessitate intubation for tial risk for the development of pulmonary edema sec-
both bronchial hygiene and mechanical ventilatory sup- ondary to the unopposed catecholamine surge triggered
port. The unopposed vagal tone of the submucosal glands by the spinal cord trauma. The initial sympathetic dis-
in the respiratory tract results in mucus hypersecretion. charge associated with trauma often lasts several minutes
Many patients will require suctioning several times an and produces hypertension; increase in the afterload;
hour. Comprehensive respiratory management has been tachycardia and arrhythmia followed by left ventricular
shown to considerably reduce mortality from respiratory strain; or failure and disruption of the pulmonary capil-
failure (15). The goal is to achieve 60 percent of predicted lary endothelium. Soon after trauma, neurogenic pul-
VC with various therapeutic procedures (66). monary edema may become clinically manifest as left ven-
The greatest incidence of atelectasis and pneumo- tricular failure with a pulmonary artery occlusion
nia occurs in the first 5 to 7 days, and is often focused pri- pressure (PCOP) above 18 mmHg or capillary leak syn-
marily in the left lower lobe (69). Atelectasis is managed drome (PCOP less than 18 mmHg).
primarily with incentive spirometry, but percussion and After trauma the spinal cord suffers a secondary
vibration, frequent position changes, intermittent positive ischemic injury and the autoregulation of the blood flow
ACUTE MEDICAL MANAGEMENT OF SPINAL CORD INJURY 119
is known to be impaired. The degree of post-traumatic sumptiondelivery curve reaches a plateau (82). The bal-
ischemia correlates with the severity of the injury, is pro- ance between volume, pressors, and inotropic agents is
gressive during the first few hours, and persists for at least based on changes in the SVR, PCOP, and stroke index.
24 hours. It should be aggressively treated to prevent If the persistent bradycardia associated with
post-traumatic infarction. In the face of systemic hypoten- hypotension does not respond to anticholinergic therapy
sion, spinal cord blood flow (SCBF) declines even more, (atropine, glycopyrolate, etc.) transcutaneous pacing may
and the severely injured cord cannot maintain its perfu- be required as a temporary step before permanent pace-
sion. Animal studies have shown that elevation of post- maker placement. The actual occurrence of bradycardia
traumatic MAP to more than 160 mmHg did not improve in cervical SCI is > 50 percent, on average occurs 2.4 +
SCBF at the injury site and caused hyperemia at adjacent 4.1 days after injury, and has a first occurrence ranging
sites (14). from day 0 to day 18 after initial injury (Peruzzi, unpub-
The treatment of secondary vascular injury should lished data). The bradycardia noted in SCI can be signif-
be directed toward local circulatory effects in the cord and icant, but serious episodes usually resolve after approxi-
systemic neurogenic shock (14). The treatment goals dur- mately 6 weeks, and permanent cardiac pacemakers are
ing the acute phase of SCI are restoration of cardiac out- rarely required (83).
put (CO) and tissue perfusion. The relative hypovolemia, The degree and extent of organ failure that develops
in relation to the expanded vascular bed, is corrected with from the sudden loss of a functioning nervous system
intravenous crystalloid solutions. An ongoing controversy below the level of injury depends on the severity of neu-
exists over the use crystalloid as opposed to colloid flu- rologic injury and its location. Therefore, clinicians man-
ids. If hypotension persists after reasonable volume resus- aging patients with neurogenic shock require exceptional
citation has occurred, invasive hemodynamic monitoring vigilance and management skills to limit the extent of tis-
should be instituted to assess the adequacy of fluid sue damage from original trauma as well as developing
replacement and to measure the PAOP, CO, and SVR. complications.
The PAOP should not be allowed to rise above 18 mmHg,
because these patients are at risk for developing pul-
monary edema (76). The ideal MAP necessary to maxi- ACUTE FLUID AND ELECTROLYTE
mize spinal cord perfusion after injury is not known with DISTURBANCES
certainty; thus, the practitioner should aim toward nor-
motension (MAP8090mmHg) (77,78). Peripheral per- In the acute SCI patient, fluid and electrolyte balance can
fusion, rather than pressure, should be used as a measure be affected by the respiratory acidosis that results from
of oxygen delivery at the site of SCI (79). The SCPP alveolar hypoventilation; metabolic alkalosis because of
depends on MAP, spinal fluid pressure (SFP), spinal vomiting or gastric suction;and hypochloremia caused by
venous pressure (SVP), and spinal cord interstitial pres- vomiting, gastric suction, or loss into a dilated gut (84).
sure (SCIP). A reduction in the SCPP to less than 40 Within the first week post injury, patients with acute SCI
mmHg in an otherwise normal spinal cord is associated are at risk for developing hyponatremia, especially if
with diminished dorsal cord evoked potentials (80). In the hypotonic fluids are administered. Patients with more
absence of function, it is suggested to maintain the SCPP severe neurologic injuries, as described by the Frankel A
in the 60 to 70 mmHg range. Only if fluid therapy fails classification, are at higher risk of becoming hypona-
should pressor agents be utilized, because increasing cir- tremic than those with less severe neurologic damage (85).
culating volume rather than vascular resistance is of most The mechanisms implicated include excessive antidiuretic
benefit to SCBF (81). hormone (ADH, vasopressin) secretion and a disruption
The vasopressors used are direct the alpha agonists in the normal sodium excretion rhythms post injury (86).
phenylephrine, dopamine, norepinephrine, and phento- Because patients with complete or incomplete injuries
lamine. In addition to raising the SVR, these agents also appear to have the reduced ability to excrete free water
increase the pulmonary vascular resistance (PVR). After and retain sodium, they should be primarily resuscitated
acute SCI; patients exhibit a reduced pressor response to with 0.9 percent NaCl or other balanced electrolyte solu-
conventional volume challenge within the pulmonary vas- tions. Close observation of electrolyte balance is neces-
cular bed. This returns to normal after dopamine infusion sary to avoid serious imbalances during the acute phase
(77). The cardiac index (CI) may drop because of the of SCI.
direct cardiac effect of SCI and may be related to an Numerous experimental and clinical studies indicate
increase in the level of circulating catecholamine (77). The that high plasma glucose adversely influences recovery
administration of inotropic agents is indicated in this cir- from ischemic neurologic injury. Therefore, it is reason-
cumstance and is aimed at improving blood flow and per- able to be concerned about hyperglycemia in the setting
fusion. Although controversial, this intervention is of a traumatized spinal cord. Plasma glucose levels as lit-
believed by some to be adequate when the oxygen con- tle as 40 mg /dL above normal may worsen the injury pro-
120 ACUTE SPINAL CORD INJURY MANAGEMENT AND SURGICAL CONSIDERATIONS
duced by spinal cord ischemia. Thus, glucose-containing the risk of bacterial infections. If the Foley catheter is
fluids should be used judiciously or not at all in victims removed too early, high bladder-filling pressures can
of spinal cord trauma, and efforts should be made to result in detrusor damage and reflux (90).
maintain a normal plasma glucose level (87). The most frequent secondary medical complication
reported during the acute care of SCI patients is urinary
tract infection (UTI). Between 1986 and 1992, 80.4 per-
GASTROINTESTINAL CARE cent of all patients reported by the Model Spinal Cord
Injury System had UTI or bacteriuria. Symptomatic UTI
During spinal shock, gastric dilatation and paralytic ileus with fever, leukocytosis, and pyuria should be treated
may develop. This can worsen the patients already com- with appropriate antibiotics for 7 to 14 days. Asympto-
promised respiratory status by restricting diaphragmatic matic bacteriuria does not need to be treated routinely,
movements. A distended stomach also predisposes to and prophylactic antibiotics, urinary antiseptics, and uri-
vomiting and aspiration. To avoid this complication, the nary acidification drugs have little role in the acute set-
stomach must be decompressed with a nasogastric tube ting. In some instances the presence of urease-producing
(88). In some instances, percutaneous gastrostomy is nec- organisms (i.e., Proteus, Klebsiella, Serratia) that are asso-
essary for protracted management. ciated with stone formation may warrant antibiotic treat-
Stress ulcers may occur in the acute phase follow- ment (91). Prevention of UTI requires meticulous
ing SCI. The incidence of gastrointestinal hemorrhage catheterization technique and the understanding that no
increases with the level and the severity of neurologic artificial drainage method guarantees the maintenance
injury. Another factor contributing to the occurrence of of sterile urine.
gastrointestinal hemorrhage after SCI is the use of steroids
(32). Prophylactic therapy with H2 blockers should be
instituted as soon as possible to decrease the incidence SKIN CARE
of gastrointestinal bleeding. Other acute gastrointestinal
complications after SCI include superior mesenteric artery Pressure ulcers continue to be a frequent complication of
syndrome and pancreatitis (89). Obviously, one must be SCI; therefore, prevention should start from the acute care
alert to the potential for such problems, because symp- phase. The NASCIS III study found a 12.3 percent inci-
toms are often masked by the loss of neurologic function. dence of pressure ulcers in SCI patients. Immediately fol-
Even though altered bowel elimination is a late com- lowing the SCI, patients must be turned every 2 hours to
plication of SCI patients, it should be addressed early, and avoid the development of ulcers (92). The time spent in each
an aggressive bowel management program should be position can be increased gradually as skin tolerance allows.
established once normal bowel sounds and motility are Specially designed foam, water, or air mattresses can reduce
restored. Rectal emptying methods, most commonly a the pressure over bony prominences, but will not obviate
combination of suppository use and finger anorectal stim- the need for turning. Patients with unstable spines may be
ulation, promotes a physiologic pattern of evacuation in placed on rotating beds that rock continuously. The beds
the distal colon. Although gentle oral cathartics may be should be adjusted so that no sliding or shear forces are
used, the use of strong cathartic medications precludes exerted, especially over the sacral prominence.
the development of a normal column of stool in the colon
and interferes with the re-establishment of normal elec-
trolyte levels. Strong cathartics also increase gaseous dis- DEEP VENOUS THROMBOSIS:
tension, which may restrict pulmonary expansions; thus, PREVENTION AND TREATMENT
such agents should be used sparingly.
The Model SCI System reported an incidence of 13.6 per-
cent of deep venous thrombosis (DVT) during acute inpa-
BLADDER CARE tient stay. The incidence of DVT was found to be similar
in patients between ages 16 and 75 years, occurred more
During the period of spinal shock, the urinary bladder commonly in male patients with paraplegia rather than
will not empty spontaneously. If not drained by catheter- tetraplegia, and more often in patients with complete
ization, bladder distension, urinary reflux, and kidney injuries. The factors implicated in the pathogenesis of
failure can result. Upon admission to the hospital, a Foley DVT are immobility, vascular dilatation and stasis,
catheter should be inserted. The return of the micturi- endothelial damage, and an increase in the level of fac-
tion reflex is variable and sometimes the reflex may never tor VIII and fibrinogen (93). The risk of DVT drops
be recovered. Intermittent bladder catheterization is approximately 20 days after the initial SCI (94).
started during the subacute phase of injury, when fluid When there is clinical suspicion, DVT can be diag-
intake and output have stabilized, in an effort to decrease nosed with a venogram. Venograms have high sensitiv-
ACUTE MEDICAL MANAGEMENT OF SPINAL CORD INJURY 121
ity and specificity, and can be performed as the initial 3. Chandler DR, Nemejc C, Adkins RH, Waters RL. Emergency cer-
vical-spine immobilization. Ann Emerg Med 1992;21:11851188.
diagnostic procedure. However, the venogram is an inva- 4. McGuire RA, Green BA, Eismont FJ, Watts C. Comparison of sta-
sive test that is not risk free. Real-time ultrasound and bility provided to the unstable spine by the kinetic therapy table
color Doppler are very sensitive methods of diagnosing and the Stryker frame. Neurosurgery 1988; 22:842845.
5. Meyer P, Lamour O. The performance of scoring systems in pre-
DVT. In 1997, the Consortium for Spinal Cord Medicine dicting outcome of head trauma patients [letter; comment]. J
recommended guidelines for the prevention of DVT based Trauma 1989; 29:1454.
on the level of risk. Among the recommendations were: 6. Aebi M, Mohler J, Zach GA, Morscher E. Indication, surgical
technique, and results of 100 surgically treated fractures and frac-
ture-dislocations of the cervical spine. Clin Orthop1986:244257.
Compression hose or pneumatic devices should be 7. Meyer P, Heim S. Surgical stabilization of the cervical spine. In:
applied to the lower extremities for the first 2 weeks Meyer P, (ed.), Surgery of Spine Trauma. New York: Churchill Liv-
ingstone, 1989:397523.
after the injury; 8. Wilberger JE. Diagnosis and management of spinal cord trauma.
In the event that thromboprophylaxis is delayed for J Neurotrauma 1991; 8 Suppl 1:S218; discussion S2930.
more than 72 hours, the extremities should be tested 9. Bohlman HH, Anderson PA. Anterior decompression and arthrode-
sis of the cervical spine: Long-term motor improvement. Part I
with either noninvasive ultrasound studies or venog- Improvement in incomplete traumatic quadriparesis. J Bone Joint
raphy for thrombi formation prior to application Surg [Am] 1992; 74:671682.
of compression boots; 10. Dolan EJ, Tator CH. The effect of blood transfusion, dopamine,
and gamma hydroxybutyrate on posttraumatic ischemia of the
Anticoagulant prophylaxis with unfractionated spinal cord. J Neurosurg 1982; 56:350358.
heparin or low molecular weight heparin (LMWH) 11. Sandler AN, Tator CH. Effect of acute spinal cord compression
should be initiated within 72 hours and continued injury on regional spinal cord blood flow in primates. J Neuro-
surg 1976; 45:660676.
until discharge in patients with incomplete motor 12. Sandler AN, Tator CH. Review of the effect of spinal cord trauma
injuries and for 8 weeks in patients with uncompli- on the vessels and blood flow in the spinal cord. J Neurosurg 1976;
cated complete motor injuries; 45:638646.
13. Schwab ME, Bartholdi D. Degeneration and regeneration of axons
Patients with complete injuries or other risk factors in the lesioned spinal cord. Physiol Rev 1996; 76:319370.
should receive LMWH for 12 weeks or until dis- 14. Tator CH, Fehlings MG. Review of the secondary injury theory
charge from rehabilitation (95). of acute spinal cord trauma with emphasis on vascular mecha-
nisms [see comments]. J Neurosurg 1991; 75:1526.
15. Chapman JR, Anderson PA. Thoracolumbar spine fractures with
Careful assessment should be done every 8 hours to neurologic deficit. Orthop Clin North Am 1994; 25:595612.
properly assess the lower extremities for swelling or 16. Tymianski M, Tator CH. Normal and abnormal calcium home-
ostasis in neurons: A basis for the pathophysiology of traumatic
edema formation and to ensure proper placement of and ischemic central nervous system injury. Neurosurgery 1996;
pneumatic devices. If the diagnosis of DVT is made, full 38:11761195.
anticoagulation is recommended either with intravenous 17. Wrathall JR, Teng YD, Choiniere D. Amelioration of functional
deficits from spinal cord trauma with systemically administered
heparin or with subcutaneous LMWH. NBQX, an antagonist of non-N-methyl-D- aspartate receptors.
Exp Neurol 1996; 137:119126.
18. Choi DW. Calcium-mediated neurotoxicity: Relationship to spe-
cific channel types and role in ischemic damage. Trends Neurosci
CONCLUSION 1988; 11:465469.
19. Rothman SM, Olney JW. Glutamate and the pathophysiology of
Acute SCI presents a unique and serious challenge to the hypoxicischemic brain damage. Ann Neurol 1986; 19:105111.
20. Greenamyre JT, Porter RH. Anatomy and physiology of glutamate
acute care/critical care medicine practitioner. Physical in the CNS. Neurology 1994; 44:S713.
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22. Lipton SA, Rosenberg PA. Excitatory amino acids as a final com-
agement, and the physiologic perturbations associated mon pathway for neurologic disorders [see comments]. N Engl J
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citation and care of these patients more challenging. 23. Hall ED. The role of oxygen radicals in traumatic injury: Clinical
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Careful and recurrent assessments are necessary to fully 24. Hall ED, Wolf DL. A pharmacological analysis of the patho-
define the pattern and extent of injury. Constant adjust- physiological mechanisms of posttraumatic spinal cord ischemia.
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25. Anderson DK, Means ED, Waters TR, Green ES. Microvascular
necessary for successful and positive outcomes in this perfusion and metabolism in injured spinal cord after methyl-
patient population. prednisolone treatment. J Neurosurg 1982; 56:106113.
26. Bracken MB et al. Methylprednisolone or naloxone treatment
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8 Factors Affecting Surgical
Decision Making
125
126 ACUTE SPINAL CORD INJURY MANAGEMENT AND SURGICAL CONSIDERATIONS
from the skull to the coccyx for areas of localized tender- ographs are an essential part of the evaluation. Although
ness is extremely helpful to localize a spinal injury. Root- 85 percent of significant injuries will be detected with a lat-
specific muscle strength testing of the upper and lower eral cervical spine radiographs that allows visualization
extremities and rectal examination for tone and sensation of C 7, an injury at the cervical thoracic junction may
are extremely important in determining the anatomic level escape detection. Consequently, current spine trauma pro-
of injury. Cranial nerve examination, sensory response to tocol should require complete cervical spine radiographs
pin and light touch, deep tendon, and plantar reflexes com- with full visualization of the body of T1. If this is not pos-
plete the spine injury assessment. Finally, careful docu- sible a swimmers view or CT scan is required.
mentation of the initial examination is essential. MRI is extremely helpful in the evaluation of
patients with SCI or neurologic deficit. It may comple-
ment, but does not replace CT. A thin-section CT scan
GENERAL PRINCIPLES OF TREATMENT of the spine provides greater bone detail and remains the
gold standard for demonstrating fracture pattern and
The important steps of treating patients who have sus- canal compromise in spinal injuries.
tained a spine injury are immobilization, medical stabi- Technetium bone scanning is occasionally helpful in
lization, restoration of spinal alignment, neural decom- ruling out occult injuries of the spine in patients with pro-
pression, and spinal stabilization. longed symptoms and a negative radiographic evaluation.
Immobilization should be performed in the field
using a rigid cervical collar as well as a spinal board and
sandbags. Immobilization should be maintained until the MECHANISM OF INJURY AND
patient has been evaluated and cleared by the treating STABILITY OF THE FRACTURE
physician. If the patient has not been stabilized in the field,
cervical spinal stabilization should be one of the first steps Spine fractures can be classified on the basis of the mech-
taken by the evaluating physician in the emergency room. anism of injury (59). Injuries to the spine may occur sec-
Medical stabilization follows the basic principles of ondary to the forces of flexion, extension, lateral rota-
trauma care. This requires continuous monitoring of the tion, axial loading, or a combination of these forces. The
patients respiratory and cardiovascular status and is gen- forces that most often cause injury to the spinal cord are
erally conducted in the emergency room or intensive care a combination of flexion and axial loading. The specific
unit setting. Patients with spinal cord injuries and neuro- fracture that these forces produce varies according to the
genic shock present with hypotension and bradycardia as age of the patient, pre-existing medical conditions (i.e.,
a result of the traumatic sympathectomy. Spinal shock is osteoporosis, ankylosing spondylolitis, etc.), as well as
different from neurogenic shock and is defined as the the level of injury.
absence of spinal reflex activity below an SCI. The sig- Spine fractures classification, along with personal
nificance of spinal shock is that it limits the ability to both experience and training, enable the surgeon to develop a
classify the extent of paralysis and predict recovery of treatment plan. The degree of stability or instability of an
cord function. Spinal shock should be considered resolved SCI will change with time as the osseous elements heal.
with the return of the bulbocavernosus reflex (BCR) Therefore, an injury that might initially be considered unsta-
within 48 hours after injury. In a small percentage of ble may well be stable 12 weeks after injury. Conversely,
patients, the BCR never returns. an injury that involves primarily ligamentous structures may
Realignment of the injured cervical spine is usually become more unstable with time. Most spine fracture clas-
accomplished with skeletal traction via halo ring or Gard- sification systems are designed to evaluate acute injuries.
ner Wells tongs. Although recently there has been some Holdsworth (8) is credited with developing the first
concern about the necessity of MRI or myelography spine fracture classification that included specific recom-
before the reduction of a cervical spine dislocation, this mendations for treatment. Denis (6) modified this system
is generally not necessary in a setting where the patient by adding the concept of the three columns of the thoracic
is being placed in traction while awake and is subjected and lumbar spine. Allen and Ferguson (5) developed a
to repeat neurologic and radiographic evaluation. mechanistic classification of closed, indirect, fractures and
dislocations of the lower cervical spine. In each system, the
goal of fracture classification is to determine whether the
IMAGING fracture is stable or unstable. The locations of most spine
fractures are most often the transitional zones of the spine,
The open-mouth anteroposterior view of the spine is essen- specifically the cervicothoracic junction and the thora-
tial for evaluation of the upper cervical spine. The stan- columbar junction. The explanation of this phenomenon
dard AP view of the spine allows evaluation of lateral mass is twofold: a change in rigidity associated with the rib cage
and sagittal plane fractures. Lateral cervical spine radi- and thoracic spine, and the change in the saggital align-
FACTORS AFFECTING SURGICAL DECISION MAKING 127
ment that occurs at the thoracic spine (cervical lordosis, in an older patient with a pre-existing cervical spondylo-
thoracic kyphosis, lumbar lordosis). The location of a frac- sis. Clinically, the patient has a greater loss of function
ture can be just as important as the fracture pattern in in the upper extremities than in the lower extremities.
regard to stability. Specifically, fractures in the mid-tho- Perianal sensation is often preserved. Historically, this has
racic region are inherentally more stable in the presence been ascribed to the somatotopic organization of the cor-
of an intact rib cage than similar fractures at the thora- ticospinal tract (CST), because the arm fibers are centrally
columbar junction. Although the specifics of spinal stabi- located. However, more recent investigations show that
lization are beyond the scope of this chapter, in general, a the CST in higher primates is not somatotopically orga-
patient who has been realigned with traction or postural nized and that the clinical syndrome may be secondary to
reduction, and do not have significant canal compromise the disproportionate influence of the CST to hand and
are often treated with posterior stabilization and fusion upper extremity motor function.
alone. In injuries that are treated with an anterior decom- BrownSequard syndrome is produced by a pre-
pressive procedure, anterior bone grafting alone does not dominant injury to the lateral half of the spinal cord.
provide immediate internal fixation and an anterior plate Motor paralysis and proprioception are greater on the
or posterior stabilization is required. side of the injury. Pain and temperature sensory loss are
Fractures producing SCI are, by definition, unsta- greater contralateral to the side of the SCI. This syndrome
ble and are usually best treated with surgical stabilization. has a fair prognosis for functional recovery.
Immediate stabilization allows for early mobilization and In the clinical setting, there often arises some con-
rehabilitation. These benefits must be weighed against the fusion in the labeling of cord injury syndromes. In a
potential risks of surgeryspecifically, infection and inad- partial SCI, the caudal segments of the spinal cord are
equate fracture stabilization. Each fracture has a per- either stronger, weaker, or the same. Caudal loss is con-
sonality of its own, and the method of spinal stabiliza- sistent with an anterior cord syndrome. A patient with
tion depends on the personality of the fracture, as well better caudal motor strength than cranial motor strength
as the ability of the surgeon. If surgery will not provide has a central cord-type lesion. Patients with isolated nerve
immediate improved spinal stability, it should probably root injuries should be classified as having neurologic loss
not be performed. without SCI.
SCI can be broadly classified as complete or incomplete Most spinal cord deficit is attributed to contusion and/or
(3). The diagnosis of complete SCI cannot be made until compression, rather than to complete transection. The ini-
spinal cord shock resolves. If the BCR is present and there tial blunt injury leads to a secondary sequence of molecu-
is no motor or sensory function below the injury, by def- lar and cellular events that results in ischemia, tissue
inition the injury is complete. If, following the return of hypoxia, and secondary tissue degeneration. The extent of
the BCR, the patient has some sensation below the level tissue damage and the resulting neural injury can be related
of injury, he is considered to be sensory incomplete. If to the magnitude of the initial force. Whether the initial
the BCR has returned and the patient has some motor blunt insult or the effect of continued pressure on the dam-
function and sensation below the level of injury, he is con- aged neural elements is greater in causing persistence of
sidered to be sensory and motor incomplete. neural deficit remains a topic of controversy. The microvas-
There are four incomplete SCI syndromes. The ante- culature can be disrupted by mechanical deformation and
rior cord syndrome is characterized by injury to the ante- propagated by edema, thrombosis, or further vasconstric-
rior cord opposite the area of spinal injury, with relative tion induced by local and circulating factors. Although the
sparing of dorsal column sensory pathways. The mecha- initial insult to the spinal cord is currently irreversible,
nism of injury is usually a compression or flexion type maintenance of perfusion at the cellular level of the spinal
injury. Clinically, there is paralysis in portions of the cord, if possible, is important to protect the remaining liv-
upper limbs. In the lower extremities, deep pressure and ing, healthy tissue and to possibly improve recovery.
position sense are preserved, with no motor function
below the area of injury. The prognosis for anterior cord
syndrome is the worst of all the incomplete syndromes. PHARMACOLOGIC TREATMENT OF
Posterior cord syndrome is rare. In this injury, there SPINAL CORD INJURY
is preservation of motor function with loss of sensory
function below the level of injury. The goal of pharmacologic intervention is to decrease sec-
The central cord syndrome is common. The mech- ondary neurologic damage rationale following the initial
anism of injury is usually an extension injury that occurs mechanical injury (1,10,11). To understand the rationale
128 ACUTE SPINAL CORD INJURY MANAGEMENT AND SURGICAL CONSIDERATIONS
of pharmacologic treatment of SCI, a brief discussion of shown a significant benefit for patients with both complete
secondary SCI is necessary. and incomplete quadriplegia. Although the exact timing
Numerous theories exist regarding secondary SCI. of anterior decompressive procedures is not entirely clear,
Overall, the SCI site becomes an area of ischemia, animal studies indicate that the effect of spinal cord com-
hypoxia, and edema. The free-radical theory suggests pression is inversely related to the duration of compres-
that, because of depletion of antioxidants, free radicals sion. It appears that patients with SCI, both complete and
accumulate and attack membrane lipids. Lipid peroxides incomplete, should undergo imaging assessment following
are produced, and the cell membranes fail. In the calcium their initial stabilization and realignment. If significant
theory, there is an influx of extracellular calcium into the anterior cord compression remains following realignment
nerve cell. This, in turn, activates phospholipases, caus- with traction, then an anterior decompressive procedure
ing a resultant interruption of mitochondrial activity and should be considered in both the incomplete and complete
a disruption of the cell membrane. SCI patient to maximize the recovery of cord injury and
The explanation for the observed effects of methyl- root return, respectively.
prednisolone is that it suppresses the breakdown of cell In general, because of the relative size of the upper
membranes by inhibiting lipid peroxidation and hydrol- cervical spine, a decompressive procedure of the upper
ysis at the site of injury. The high doses required are those cervical spine is rarely, if ever, indicated. In the lower cer-
shown to be most effective in inhibiting lipid peroxida- vical spine, the vast majority of patients can be success-
tion and the breakdown of neurofilaments in laboratory- fully stabilized by a posterior procedure alone. If signifi-
induced SCI. cant canal compromise by disc or bony fragments occurs
Because the beneficial effects of methylprednisolone following reduction, an anterior decompression along
on SCI are believed to be unrelated to its glucocorticoid with surgical stabilization is usually performed. The ben-
effects, interest has developed in the utilization of 21- efits of anterior cervical decompression with residual
aminosteroids for SCI. Tirilazad is a lazeroid (synthetic canal compromise are twofold; it provides a biomechan-
21-aminosteroid). It is a potent antioxidant without the ically sound fusion environment and provides an opti-
glucocorticoid effects of methylprednisolone. GM 1 gan- mal environment for neurologic recovery and root return.
gliosides are complex glycolipids of an acid nature that In thoracic and lumbar injuries, realignment and pos-
are present in high concentrations in central nervous sys- terior stabilization provides an indirect decompression of
tem (CNS) cells. Although their functions are not entirely the spinal canal that often eliminates the requirement for
clear, there is experimental evidence that suggests they anterior decompression. If significant canal compromise
augment neurite outgrowth, induce regeneration and (often defined as greater than 50 percent canal compromise)
sprouting of neurons, and restore neurologic function remains following posterior reduction and stabilization, an
after injury. GM-1 may be effective in treating SCI even anterior decompression may be necessary. In most clinical
if administration is delayed for 48 hours. Unfortunately, situations, an anterior thoracic or lumbar decompression
the one major drawback of GM-1 is that it may have an is not urgently or emergently required. The decision to per-
antagonistic effect on the neuroprotective role of methyl- form an anterior decompression in the acute setting, via a
prednisolone. This may become quite cumbersome in the thoracotomy or a thoracolumbar approach, requires care-
design of further clinical trials. ful consideration. The potential benefit of anterior decom-
Current recommendations are a result of the third pression is often outweighed by the deleterious effect that a
National Acute Spinal Cord Injury Study (NASCIS-III). thoracotomy may have on a multitrauma patient.
The recommended guideline for patients who receive
treatment within three hours of injury is the administra-
tion of methylprednisolone as a bolus (30 mg/kg-body TIMING OF SURGERY
weight) followed by an infusion (5.4 mg/kg/hr) for 23
hours. For those patients in which methylprednisolone When a progressive neurologic deficit exists in the pres-
is initiated within three to eight hours of injury, the infu- ence of malalignment and spinal canal compromise, emer-
sion should continue for 48 hours. gency decompression is indicated. In all others with SCI,
the timing of surgery is controversial (1,12,13). Some
authors recommend treatment as soon as the patient is
ROLE OF ANATERIOR DECOMPRESSION medically stable, whereas others advocate a delay of 4 or
more days to allow for posttraumatic swelling to resolve.
Direct anterior spinal cord decompression (12,13) should Delamarter evaluated the effect of the timing of spinal
be considered when there is significant residual anterior cord decompression in a canine SCI model. In his study, a
compression of the spinal cord following realignment. In constriction band was utilized to create a controlled para-
the past, concern existed about further injury and desta- plegia. In the group that was decompressed at 1 hour fol-
bilization by anterior procedures, but recent work has lowing injury, the animals recovered neurologic function.
FACTORS AFFECTING SURGICAL DECISION MAKING 129
In the group that was decompressed at 6 hours following metastatic disease, and ankylosing spondylitis are asso-
injury, there was no neurologic recovery. The results of ciated with spine fractures. Each disease presents its own
his study suggested that not all injury to the spinal cord challenges in the treatment of spine fractures (1418).
occurred at the initial trauma and that early decompres- Osteoporosis is the most prevalent metabolic bone
sion had a significant role in neurologic recovery. disease. Osteoporotic compression fractures often occur
Whether early decompression and reduction of neural in the elderly. Most often, these fractures are stable in
structures enhances neurologic recovery continues to be nature and do not require surgical intervention. Unsta-
debated. Although animal studies suggest a benefit with ble fractures associated with SCI, when they do occur,
early decompression, this hypothesis has not been clinically require careful surgical planning. The usual methods of
proven. Indeed, significant functional recovery has been surgical stabilization often fail to provide adequate sur-
observed following anterior decompression of the spinal gical stabilization in light of this soft bone. The judi-
cord in patients whose initial injury was up to 20 years old. cious use of polymethylmethocroylate is often necessary.
Currently, a reasonable approach would be to treat non- Metastatic spinal lesions are an important consid-
progressive neurologic deficits on a semiurgent basis, when eration in the evaluation of low-impact spine fractures.
the patients systemic condition is medically stable. Metastatic lesions are identified at autopsy in 40 to 80
percent of patients who die of cancer. Spinal cord com-
pression occurs in 20 percent of patients with spinal
GUNSHOT WOUNDS metastases. In treating patients with metastatic spine frac-
tures, the surgeon must consider the overall health of the
Over the last 10 years, a significant increase in the inci- patient, the degree of pain, and the biology of the primary
dence of spinal cord injury is related to gunshot wounds. tumor, as well as the integrity and stability of the spinal
The most recent data is that 25 percent of SCI is related column.
to gunshot wounds. There data, from 1991, reported that Patients with ankylosing spondylitis have a 3.5 times
52 percent of total admissions for SCI at one regional SCI greater incidence of cervical spine injury than the nor-
center were related to gunshot wound trauma. mal population. Seventy-five percent of the spine fractures
These injuries rarely cause sufficient bone or liga- sustained by patients with ankylosing spondylitis occur
mentous injury to warrant surgical treatment. Immobi- in the cervical spine. The diagnosis is often delayed.
lization with a halo vest or rigid cervical orthosis usually Patients typically present with complaints of neck pain
provides adequate stability for healing. The issue of following minimal trauma. The mechanism of injury is
removing a bullet from the spinal canal remains contro- most often a hyperextension injury. Because of the disease
versial. The argument for bullet removal revolves around process, the spine breaks like a solid long bone. These
diminishing the risk of CSF leak, meningitis, lead toxic- fractures are highly unstable. Patients are at a high risk
ity, pain, neurologic decline, and bullet fragment migra- for neurologic complications.
tion. The argument for the nonoperative approach is that Traditional treatment for this type of fracture is rigid
retention of bullet fragments does not routinely lead to immobilization with a halo cast or a vest, paying careful
complications and that the risks of surgical intervention attention to align and maintain the patients preinjury
outweigh the potential benefits of fragment removal. alignment. Recent development in spinal instrumentation
Overall, these cases are never straightforward. They has allowed for rigid internal fixation; in the hands of
must be evaluated on a case-by-case basis. If the bullet some experienced spine surgeons, early stabilization with
fragment is creating compressive pathology, its removal a combined anterior and posterior procedure is now the
may benefit the patient. Likewise, bullet migrations in the treatment of choice for fractures in patients with anky-
spinal canal or progression of neurologic deficit are indi- losing spondylitis.
cations for surgical intervention. Recent studies evaluated
the impact of bullet removal in SCI patients. The con-
clusion of the studies was that removal of the bullet did CONCLUSION
not affect the incidence of pain. Bullet removal from the
canal between T1T11 had no significant affect on motor When treating patients with SCI, the physician must look
recovery, but when bullets were removed from the cauda at the individual needs of the patient. Age, level of injury,
equina region, patients did receive some benefit. fracture pattern, preexisting medical conditions, and asso-
ciated traumatic injuries are factors that influence the
treatment plan. If early mobilization of the patient
PATHOLOGIC BONE through surgical stabilization is realistic, then early sur-
gical intervention should be performed.
Fractures associated with minimal trauma are most
often associated with pathologic bone. Osteoporosis,
130 ACUTE SPINAL CORD INJURY MANAGEMENT AND SURGICAL CONSIDERATIONS
ervical spine injuries frequently unstable injury (9), and this chapter focuses on the for-
traumatic spondylolisthesis (the hangmans fracture), is caused. Mechanical stability refers to the relative
recognition of the mechanism also plays a key role. The motion of vertebral segments under the physiologic loads
majority of these injuries arise from an extension force, of everyday activity. An illustrative case is a ventilator-
although some are the result of flexion-type mechanism dependent patient who sustained a cervical fracture dis-
(7). In contrast to other patterns, flexion-type fracture is location along with a massive nonfatal head injury and
exaggerated by cervical traction and thus necessitates pulmonary contusion. At bed rest, he is undergoing strict
axial compression for adequate reduction. log-roll precautions. The spine injury was reduced with
longitudinal traction at the time of presentation, and the
patient has since been placed on 15 pounds of mainte-
Imaging
nance weight. MRI and CT scans indicated no herniated
High-quality radiographs are crucial to effective preop- disc or significant canal compromise. Neurologically, the
erative planning. Films should include anteroposterior danger of further cord compression is unlikely, given that
and lateral views of the entire cervical spine. Failure to the patient is not being mobilized or seated upright, this
adequately image each spinal segment can lead to missed despite the fact that the vertebral fracture-dislocation is
injuries, especially because the rate of concomitant mechanically unstable. Before further mobilization can be
injuries in the cervical spine is high (7). Open-mouth and contemplated, surgical stabilization must be accom-
swimmers views are helpful in imaging the atlantoaxial plished. This will require both initial intensive care and
and cervicothoracic junctions, respectively. In some further rehabilitative efforts once the patients medical
patients, especially obese individuals, plain film visual- condition has stabilized. Stabilization of the spine facili-
ization is difficult and more advanced imaging modalities tates nursing care, and respiratory, physical, and occu-
must be pursued. pational therapy for these patients.
Computerized tomography (CT) provides highly To assess mechanical stability, the subaxial cervical
detailed axial images of each vertebral segment and is supe- vertebral elements have been divided into three columns
rior to MRI for delineating fractures. CT can demonstrate (13). Effectively, the columns can be considered longi-
nondisplaced fractures not discernible on plain roentgen- tudinal members that span the length of the spine. The
ograms. In addition, CT is an excellent method for evalu- anterior column consists of the ALL, the anterior aspect
ating and quantifying the degree of spinal canal compro- of the intervertebral disc, and the anterior half of the ver-
mise. Although these advantages are clear, CT alone is not tebral body. Likewise, the middle column is made up of
a substitute for plain radiographs, because the latter are the PLL and the respective posterior aspects of the disc
better for determining overall spinal alignment and coro- and vertebral body. The posterior column refers to all
nal or sagittal deformity. Pure dislocations can be easily remaining structures, including the pedicles, laminae,
missed with CT alone and are best diagnosed by plain radi- transverse processes, facet joint, and the PLC (inter-
ograph. CT machines also allow for reconstructed three- spinous, supraspinous ligaments). The junction of the
dimensional (3D) images, which may provide further middle and posterior columns forms the spinal canal.
information. However, these are computer generated and Normally, the spine rotates around an axis within the
limited by the number of axial images obtained and the spinal canal, ensuring as little motion as possible in the
computer program used for reformatting. region of the neural elements (14).
MRI is the modality of choice for evaluating the A column can be disrupted by either fracture or lig-
spinal cord and neural elements. In addition, MRI can amentous disruption. By definition, disruption of two or
reveal edematous soft tissues, in either the anterior or pos- more columns imparts instability (13). Gunshot wounds
terior cervical spine. MRI is especially important in trau- to the cervical spine are an exception to this rule. Because
matic spine injuries because it visualizes the cord. Often, of the mechanism of injury, gunshot wounds can be sta-
diffuse cord edema represents the degree and extent of ble even with two- or three-column injury (9). By collat-
injury. MRI offers information regarding the integrity of ing information from plain radiography, CT scans, and
the ligamentous structures, including the anterior longi- MRI imaging, the integrity of each column can be deduced.
tudinal ligament (ALL) and the posterior ligamentous In some situations, spinal injury occurs without
complex (PLC), and the presence of herniated disc mate- obvious radiographic findings. Spinal cord injury (SCI)
rial in the spinal canal or foramina (11,12). may or may not be present. In the awake, neurologically
intact, cooperative patient, dynamic flexion-extension lat-
eral radiographs can reveal abnormal motion between
SPINAL STABILITY vertebral segments. This is best judged by drawing a lon-
gitudinal line along the anterior and posterior aspects of
Stability can refer to both mechanical and neurologic fac- the vertebral bodies. An abrupt break in the line should
tors. Neurologic stability denotes a state in which, under be measured. If the break is more than 3.5 millimeters,
the stresses that are imposed, no further neural damage or 11 degrees of motion is present, the spine is unstable
CERVICAL INJURIES: INDICATIONS AND OPTIONS AND SURGERY 133
and likely requires operative intervention (15). Some ditionally, this is filled with a tricortical bone graft (3,17).
advocate deferring flexion-extension lateral radiographs The purpose of the graft is two-fold. First, it acts as a strut
until 2 weeks after acute injury, because of the risk of to maintain the height of the anterior column. For this rea-
false-negative studies from motion limitations caused by son, cortical bone must be used. Softer cancellous
pain and muscle spasm (16). (medullary) bone cannot sustain these large forces. Most
commonly, tricortical autograft from the patients anterior
iliac crest is used, although allograft can also be utilized.
SURGICAL TREATMENT OPTIONS The second purpose of the graft is to fuse the vertebrae.
The bone graft is wedged between the endplates.
Goals of Surgery High compressive forces help to maintain its position.
With neck extension, however, these compressive forces
As stated previously, the main objectives in the operative may be diminished. Normally, the ALL acts as a check
treatment of cervical spine injuries are to decompress the rein to these distraction forces. Although postoperative
spinal canal and achieve mechanical stability. Decom- neck extension can be controlled to some extent with a
pression can be effected by anterior or posterior hard cervical collar, many surgeons opt to place anterior
approaches. Anterior decompression consists of the instrumentation (1). Fixed to the uninjured vertebral bod-
removal of the vertebral body and/or intervertebral disc. ies with screws, anterior plates span the fused vertebral
Posterior decompression is accomplished by removing the segments and act as an artificial ALL. In some circum-
posterior neural arch, namely the spinous processes, lam- stances, anterior instrumentation can obviate the need for
inae, and PLC. Stabilization usually consists of a fusion external postoperative orthoses, shorten the length of
procedure. Anteriorly, this is achieved by implanting time they are worn postoperatively, or allow for less rigid
structural bone graft material between adjacent vertebrae. immobilization.
Posteriorly, this is accomplished by on-lay bone grafting Rarely, the C12 junction is approached anteriorly
over decorticated posterior elements. The use of instru- (2). Because the mandible and its associated structures
mentation can protect the fusion mass until healing create an obstruction, a transoral approach has been
occurs, thus preventing graft dislodgment, and obviating developed. By incising the posterior pharyngeal mucosa
the need for postoperative orthoses in some cases. at the approximate level of the hard palate, the surgeon
gains access to the anterior atlantoaxial joint. The bony
elements can then be removed as necessary. Stabilization
Anterior Decompression and Stabilization
procedures are difficult and dangerous to perform
The anterior approach to the cervical spine can be used through this approach, but have been described (2). If sta-
for decompression of C3 to C7 (3,11,17). Palpable sur- bility is compromised, posterior fusion and/or instru-
face landmarks include the cricoid cartilage (C6), thyroid mentation is recommended (2).
cartilage (C4), the hyoid bone (C6), and the carotid tuber-
cle (C6). Dissection is carried medial to the carotid sheath.
Posterior Decompression
In its deepest layers, the interval between the longus colli
and Stabilization
muscle and the anterolateral aspects of the vertebral body
is utilized. Anatomic dangers are the recurrent laryngeal The cervical spine can be approached posteriorly at any
nerve, more at risk on the right side than the left, and the level. Utilizing a midline incision, the paraspinal muscles
sympathetic chain, which is at risk with lateral dissec- are elevated off the bony elements to expose the spinous
tion on top of the longus colli. With retractors in place, processes, laminae, and tiny transverse processes. At the
the vertebral body and anterior disc are easily visualized. C1 level, lateral dissection is limited by the vertebral
The injured disc(s) are incised and removed piece- artery nerve, which lies 1.5 centimeters from the midline.
meal. The posterior limit to discectomy is the PLL. After In the subaxial spine, the medial aspect of the transverse
disc removal, the vertebral body is removed in a similar processes form the vertebral artery foramen; this is the
fashion. With the corpectomy complete, the PLL is lateral border of the safe zone.
inspected. If free fragments of bone or disc were noted Posterior decompressive procedures are used less fre-
on preoperative imaging, the canal must be visualized. If quently after cervical spinal trauma. Following anterior
not already disrupted from the initial injury, the PLL can column injury, a posterior approach is often contraindi-
be carefully incised. At all times, clear visualization of the cated and will cause worsening of stability in an already
neural elements must be maintained when working in the compromised spine. Canal compromise is approached
spinal canal. At each disc level, the exiting nerve roots are from the direction of the offending elements. Retropulsed
inspected and decompressed. fragments of vertebral body or disc are the usual culprit.
Anterior decompression leaves a void between the cra- In some situations, a laminectomy is indicated to retrieve
nial and caudal endplates of the uninjured segments. Tra- posterior fragments of bone or intervertebral disc. Lam-
134 ACUTE SPINAL CORD INJURY MANAGEMENT AND SURGICAL CONSIDERATIONS
inal fractures are associated with dural tears, which SPECIFIC INJURIES
require requiring repair in 50 percent of cases. Decom-
pressive laminectomy begins with removal of the spin- Bony Anatomy
ous processes down to the junction with the laminae.
Briefly, the atlas is a bony ring that rotates around the
Next, the laminae are carefully removed piecemeal until
dens of the axis (C2). Broad inferior articular surfaces
the dural sac is visualized.
enhance the stability of the atlantoaxial relationship. The
In certain cases, the stability of the posterior mid-
atlas articulates with the occiput through two broad artic-
line structures can be preserved with more limited
ular lateral surfaces. The posterior ligaments are loose, to
decompression laminotomies and foraminotomies.
allow greater range of motion. The dens is an upward
However, after traumatic injury, a stabilization or fusion
projection of the C2 body. Transverse and apical liga-
procedure should be performed regardless of limited pos-
ments stabilize the atlantodens articulation. Transverse
terior bone removal, to prevent progressive deformity in
ligaments extend from the posterior aspect of the dens
most situations.
(odontoid process) to the posterior aspect of the anterior
In cases of dislocation or fracture-dislocation,
arch of C1. The apical ligament spans from the tip of the
closed reduction with longitudinal tong traction is usu-
odontoid process (C2) to the anterior aspect of the fora-
ally effective. In some cases, however, the dislocation
men magnum (C0). The axis (C2) intimates with C3
may be irreducible. The most common scenario is of
through lateral articular masses (the lateral masses) and
locked facets in which the inferior articular process of
marks the transition to the lower cervical spine. Because
the cranial vertebra (which normally lies posterior to the
of this unique function, the cranial and caudal articula-
superior articular process of the caudal vertebra) is
tions of the axis are substantially offset anterior and pos-
trapped in front of the superior articular process of the
terior, respectively. This offset puts the region between the
caudal segment (11,18). Using the posterior approach,
upper and lower articular processes at particular risk for
the offending superior articular process is carefully
fracture (hangmans fracture). The subaxial spine is sim-
removed, thus allowing the cranial segment to return to
ilar to vertebral segments at other levels. The anterior ver-
its antomic position. In this case, laminectomy is not nec-
tebral body is connected to the posterior arch through
essary, because reduction and restoration of alignment
two pedicles. At the pediclelamina junction, superior and
has decompressed the canal. If the patient was not
inferior processes project to articulate with adjacent
awake and alert for a full neurologic exam prior to
motion segments.
surgery, a preoperative MRI is necessary (12). Postop-
erative MRI is useful to detect a herniated disc after
reduction, which may necessitate subsequent anterior Atlas (C1)
decompression if further neurologic deficit develops. The majority of atlas injuries can be treated nonsurgically
Postoperative studies can be difficult to interpret fol- using either a halo vest or cervical collar. With a spacious
lowing any type of instrumentation. spinal canal, cord injury is less likely in the C1C2 region
After laminectomy or reduction of facet disloca- than with subaxial trauma. Injuries can be divided into
tions, posterior fusion is advised. In both cases, the PLC two groups: a) posterior ring fractures and lateral mass
has been disrupted either by the injury or iatrogenically. fractures (Jefferson fractures), and b) transverse ligament
Various techniques of fusion exist. Functionally, they disruptions.
consist of on-lay bone graft across vertebral segments
following decortication to stimulate bone formation. In
Posterior Ring Fractures
the upper cervical spine, a bone graft can be wedged
between the C1 and C2 arches, if they are intact. Occip- Most posterior ring fractures of the atlas are stable and
itoatlantal fusion can be achieved by securing large struts heal well using a hard collar. The mechanism of injury is
of cortical graft to the posterior ring of C1 and the occip- usually axial load with hyperextension. These fractures
ital protuberance. are associated with concomitant cervical spinal injuries
As in the anterior spine, methods of posterior instru- in 50 percent of cases (7). The most frequently encoun-
mentation have been developed (4,6). Interspinous tered concomitant lesion is a type II dens fracture, which
wiring, lateral mass plates, and vertebral screw-rod sys- may be displaced or nondisplaced. In this situation, the
tems have all been used successfully. Conceptually, the dens fracture should be stabilized. It is the authors pref-
constructs act to partially replace the missing or injured erence to place an odontoid screw to stabilize the frac-
posterior ligaments and protect the fusion mass until heal- ture. This can preserve motion of the atlantoaxial joint
ing occurs. Again, these techniques may obviate external and obviates a larger fusion procedure posteriorly. With
postsurgical immobilization, although this depends on the odontoid fixed, a halo vest or hard collar can be used
surgeon preference, the quality of fixation, and the pres- postoperatively to protect the fixation in light of a dis-
ence of concomitant injuries. rupted posterior ring.
CERVICAL INJURIES: INDICATIONS AND OPTIONS AND SURGERY 135
Lateral Mass Fractures until healed. If the condition is irreducible, open reduction
through a posterior approach, followed by posterior
The term lateral mass denotes the articular complex
fusion, is indicated.
of the atlas and its supporting structures. The superior
and inferior joint surfaces are situated directly on top of
one another. They act as the communication between the Axis (C2)
anterior and posterior rings. Fractures can develop ante-
riorly, across, or posteriorly to the articular surfaces. The Odontoid (Dens) Fracture
mechanism of injury is usually axial load with varying
Odontoid fractures occur from flexion or extension
degrees of flexion or extension.
mechanisms. Injuries are classified according to anatomic
Fractures can be unilateral or bilateral. Bilateral frac-
location. Type I fractures arise at the tip or apex of the
tures are colloquially referred to as Jefferson fractures.
process and most probably represent a bony avulsion of
Unique to injury at this level, stability is assessed by the
the apical ligament. These can be treated with good
amount of lateral displacement. On an open-mouth
results using a rigid collar.
anteroposterior radiograph, this can be measured as the
Type II fractures are the most problematic. Because
bilateral total millimeters of lateral overhang of the atlanto
the dens embryologically arises from the C1 level and the
articular processes in relation to the occiptal articular
base (i.e., vertebral body) from the C2 level, there is a water-
processes. A total displacement of greater than 7 to 10 mil-
shed area of blood supply at this junction. With greater than
limeters implies disruption of the transverse ligaments and
4 to 5 millimeters of displacement, nonunion is likely (7).
instability (7,20). Stable lateral mass fractures can be
Closed reduction with flexion or extension maneuvers
treated using a hard cervical orthosis. Unstable fractures
using halo stabilization is effective in most cases. However,
are reduced with an initial period of traction followed by
if fracture reduction cannot be maintained, open reduc-
halo vest immobilization. Because the transverse ligament
tion and stabilization must be entertained.
may not heal, flexion-extension views should be obtained
In the absence of an associated posterior ring frac-
after radiographic bony union. If atlantoaxial instability is
ture, a simple posterior C1C2 fusion using wiring is
present, then posterior C1C2 fusion is indicated.
effective in preventing late atlantoaxial instability. As in
all C1C2 fusions, this sacrifices 50 percent of the rota-
Atlantoaxial Dissociation tion of the head. With ring discontinuity, lateral mass plat-
ing or transarticular screw and fusion can be utilized.
Transverse Ligament Disruption Alternatively, an anterior odontoid screw can be inserted
across the fracture site (19). However, this can only be
Pure, isolated transverse ligament injury can occur with- used when anatomic alignment can be achieved. The
out fracture. Clinically, it can result in C1C2 instability. advantage of this method is that it preserves the rotatory
In the awake, neurologically intact patient, careful motion of the atlantodens articulation. In regards to
dynamic lateral radiographs can demonstrate instability. patient mobilization, the transfacet or Magerl technique
Normally, the atlantodens interval is less than 3 millime- provides the greatest immediate and long-term stability.
ters . Greater intervals imply discontinuity of the trans- Type III dens fractures occur through the body of the
verse ligament. Instability is best treated with a posterior ring. Blood supply is good and healing is reliable. Immo-
C1C2 fusion, with or without instrumentation. Spin- bilization in a rigid cervical collar is usually sufficient.
ous process wiring with on-lay autograft is an effective
method of stabilization.
Traumatic Spondylolisthesis (Hangmans Fracture)
This injury is a subluxation of the axis on the C3 verte-
Rotatory Subluxation or Dislocation
bra in association with a fracture. The fracture occurs in
Rotatory subluxation or dislocation occurs most com- the region between the more anterior cranial articular
monly in children and is often the sequela of nontraumatic process and the posteriorly situated caudal articular
processes, such as retropharyngeal infection (i.e., Grisels surface.
syndrome). After trauma, it can occur from abrupt rota- Type I fractures are nondisplaced. This is a stable
tional forces in both adults and children. Presentation can injury and can be treated in a collar. Type II injuries are
be of a cock-robin posture of the neck, in which the head displaced more than 3 millimeters. Reduction is through
is rotated away and tilted toward the side of the injury. the use of cervical tong traction with slight hyperexten-
Dynamic CT scans can be diagnostic, with comparison of sion of the neck. Alignment is maintained in a halo vest
axial images obtained with the head turned fully to the left for 8 to 12 weeks. If greater than 6 millimeters of dis-
and the right. In most cases, gentle traction leads to a com- placement is present, and it cannot be further reduced,
plete reduction, and the patient can be placed in a halo vest then posterior fusion of C2 to C3 is performed.
136 ACUTE SPINAL CORD INJURY MANAGEMENT AND SURGICAL CONSIDERATIONS
FIGURE 9.5
Pedicle Fractures
FIGURE 9.7
Pedicle fractures represent very unstable, high-energy
lesions (see Figures 9.8 through 9.12). In essence, they are Same patient; following staged anteriorposterior fixation, the
a posterior fracture-dislocation, where the upper spine is patient could be mobilized and rehabilitated with almost full
displaced anteriorly and the lower spine posteriorly. The return of function.
Disc Injuries
Traumatic injury to the intervertebral disc usually requires
surgical stabilization. A traumatic injury may represent
part of a complete ligamentous disruption, known as a
Chance fracture. These are persistently unstable injuries
(because of poor healing of the ligamentous structures),
FIGURE 9.6 and thus require anterior discectomy and fusion. In these
settings, anterior instrumentation allows for a more rigid
Same patient; CT scan showing injury to all three columns construct, higher fusion rates, and faster mobilization
of the spine. from the orthosis.
CERVICAL INJURIES: INDICATIONS AND OPTIONS AND SURGERY 139
FIGURE 9.10
FIGURE 9.8
Same patient; CT scan showing C5 pedicle and facet fracture.
Twenty-year-old female s/p MVA vs. pedestrian. AP with C5
facet and pedicle fracture.
FIGURE 9.11
FIGURE 9.9
Same patient; AP c-spine following anterior C56 anterior
Same patient; 2 weeks following injury with progressive sub- instrumentation and fusion. Surgery performed anteriorly
luxation of C5 on C6. because the disc is disrupted as well.
140 ACUTE SPINAL CORD INJURY MANAGEMENT AND SURGICAL CONSIDERATIONS
COMPLICATIONS
local pin care and oral antibiotics. If the drainage does 8. Hamilton A, Webb JK. The role of anterior surgery for vertebral
fractures with and without cord compression. Clin Orthop 1994;
not resolve, or if cellulitis or an abscess develops, the pin 300:7989.
should be removed after a new pin is inserted at a differ- 9. Kupcha PC, An HS, Cotler JM. Gunshot wounds to the cervical
ent site. Incision and drainage may be required, followed spine. Spine 1990; 15:10581063.
10. Allen BL, Ferguson RL, Lehmann TR, OBrient RP. A mechanis-
by intravenous antibiotic therapy. tic classification of closed, indirect fractures and dislocations of
Pressure sores can develop under the halo vest (26). the lower cervical spine. Spine 1982; 7:1982.
This may be the result of insufficient padding, inappro- 11. Stauffer ES. Managment of Spine Fractures: C3 to C7. Orthop
Clin N America 1986; 17:4553.
priate sizing, or poor cast application (for those in halo 12. Eismont FJ, Arena MJ, Green BA. Extrusion of an intervertebral
casts). Even padded two- and four-poster collars can cause disc associated with traumatic subluxation or dislocation of cer-
decubiti on the occiput. Frequent, careful skin inspection vical facets: Case report. J Bone Joint Surg 1991; 73-A:15551560.
13. Cybulski GR, Douglas RA, Meyer PR, Rovin RA. Complications
beneath any orthotic is essential, especially in patients with in three-column cervical spine injuries requiring anterior-posterior
paralysis or poor protective sensibility (24,25). stabilization. Spine 1992; 17:253256.
14. Haher TR, OBrien M, Felmly WT, Welin D, Perrier G, Choueka
J, Devlin V, Vassiliou A, Chow G. Instantaneous axis of rotation
as a function of the three columns of the spine. Spine 1992;
CONCLUSION 17:S149154.
15. White AA, Panjabi MM. Clinical Biomechanics of the Spine, 2nd
edition. Philadephia: Lippincott-Raven, 1990.
The goals of operative cervical fracture care are to decom- 16. Wang JC, Hatch JD, Sandhu HS, Delamarter RB. Cervical flex-
press the canal, stabilize the cervical spine, restore align- ion and extension radiographs in acutely injured patients. Clin
ment, and prevent late deformity. When spinal stability Orthop 1999; 365:111116.
17. Anderson PA, Bohlman HH. Anterior decompression and
is achieved through internal fixation and/or well-fitting arthrodesis of the cervical spine: Long-term motor improvement.
orthoses, early mobilization can help avoid the morbid- J Bone Joint Surg 1992; 74-A:683692.
ity of prolonged recumbence. 18. Rizzolo SJ, Vaccaro AR, Cotler JM. Cervical Spine Trauma. Spine
1994; 19:22882298.
19. Henry AD, Bohly J, Grosse A. Fixation of odontoid fractures by
References an anterior screw. J Bone Joint Surg 1999; 81-B:472477.
20. Spence KF, Decker S, Sell KW. Bursting atlantal fracture associ-
1. Apfelbaum RI. The use of anterior Caspar plate fixation in acute ated with rupture of the transverse ligament. J Bone Joint Surg
cervical spine injury. Surg Neurol 1992; 38:162163. 1970; 52-A:543549.
2. Bohler J. Anterior stabilization for acute fractures and non-unions 21. Weinstein MA, McCabe JP, Cammisa FP. Postoperative spinal
of the dens. J Bone Joint Surg 1982; 64-A:1826. wound infection: A review of 2,391 consecutive index procedures.
3. Bohlman HH, Anderson PA. Anterior decompression and J Spinal Disord 2000; 13:422426.
arthrodesis of the cervical spine: Long-term motor improvement 22. Younger EM, Chapman MW. Morbidity at the bone graft donor
(Part I-improvement in incomplete traumatic quadriparesis). J sites. J Orthop Trauma 1989; 3:192195.
Bone Joint Surg 1992; 74-A:671682. 23. Schnee LL, Freese A, Weil RJ, Marcotte PJ. Analysis of harvest
4. Ebraheim NA, Rupp RE, Savolaine ER, Brown JA. Posterior plat- morbidity and radiographic outcome using autograft for anterior
ing of the cervical spine. J Spinal Disord 1995; 8:111115. cervical fusion. Spine 1997; 22: 22222227.
5. Osti OL, Fraser RD, Griffiths ER. Reduction and stabilization of 24. Garfin SR, Botte MJ, Waters RL, et al. Complications in the use
cervical dislocations. J Bone Joint Surg 1989; 71-B:275282. of the halo fixation device. J Bone Joint Surg 1986; 68-A:320325.
6. Murphy MJ, Daniaux H, Southwick WO. Posterior cervical fusion 25. Lind B, Sihlbom H, Hordwall A. Halo-vest treatment of unstable
with rigid internal fixation. Orthop Clin N America 1986; traumatic cervical spine injuries. Spine 1988; 13:425432.
17:5565. 26. Botte MJ, Byren TP, Abrams RA, Garfin SR. Halo skeletal fixa-
7. Levine AM, Edwards CC. Treatment of injuries in the C1C2 tion: Techniques of application and prevention of complications.
complex. Orthop Clin N America 1986; 17: 3144. J Am Acad Orthop Surg 1996; 4:4453.
This Page Intentionally Left Blank
10
Surgical Management
for Thoracolumbar
Spinal Injuries
he thoracolumbar region of the or more organ systems (3). Intra-abdominal injury can
CT is indicated for further evaluation of thora- protection. This may explain the increased incidence of
columbar injuries. Approximately 25 percent of burst catastrophic neurologic injury associated with thoracic
fractures may be misdiagnosed as stable wedge fractures spine fractures (18).
by plain radiographs (911). The lateral CT scout views The thoracolumbar region is a transitional junction
should not be overlooked; they may indicate vertebral between the stable, less mobile thoracic spine and the
fractures, particularly in the lumbar spine (12). more flexible lumbar spine. In this junctional region, the
MRI can provide prognostic information that may rib cage no longer provides protection and support, thus
help in the treatment of SCI. MRI is indicated in all cases rendering the thoracolumbar spine more vulnerable to
of acute neurologic deficit and in cases of progressive neu- injury and making it the most common location for burst
rological deterioration. fractures (19).
A classification of MRI spinal cord signal patterns
has been developed for T2 weighted images in acute
spinal injury (13). In Type I lesions a central hypo-dense SPINAL STABILITY
signal of hemorrhage surrounded by a hyper-dense sig-
nal of edema is observed. This lesion has the poorest prog- The definitions and classifications of spinal stability are
nosis for functional neurologic recovery. Type II lesions, various and are based on the integrity of both the skele-
are characterized by spinal cord enlargement and an area tal and neural structures of the spinal column.
of hyper-intensity or edema extending superiorly or infe- The evaluation of radiographs and biomechanical
riorly. Type II lesions have the best prognosis for neuro- studies of thoracolumbar injuries has demonstrated that
logic recovery. Type III lesions contain a small central area the spine functionally consists of load-bearing columns
of hypo-intense hemorrhage surrounded by an area of (2028). The anterior spinal column contains the ante-
hyper-intensity or edema, and can indicate the potential rior longitudinal ligament (ALL) and the anterior half of
for intermediate recovery. For the evaluation of late spine the vertebral body and annulus fibrosus. The middle col-
trauma, MRI can detect posttraumatic syringomyelia and umn contains the posterior half of the vertebral body and
myelomalacia. In the evaluation of SCI without radi- annulus fibrosus and the posterior longitudinal ligament
ographic abnormalities, MRI may be useful. This type of (PLL). The posterior column contains all structures pos-
SCI more commonly occurs in children less than 8 years terior to the PLL.
of age, and may be attributable to the characteristic fea- The anterior and middle vertebral columns provide
tures of the spine in this age group (14). the majority of the axial load-bearing capacity of the
spine. The posterior column resists tension and stabilizes
the load shared by the anterior column (22,29,30).
SPINAL ANATOMY Instability may occur after disruption of the middle
column, in addition to disruption of either the anterior or
The anatomic characteristics of the thoracic and lumbar posterior columns. Each column, however, does not con-
spine may contribute to the pattern of spinal injury. tribute equally to spinal stability (26).
The thoracic spinal cord is shielded from injury by The Dennis classification (24,25) of acute thora-
the regional paraspinal musculature and by the rigid tho- columbar injury is probably the most widely accepted.
racic rib cage. The sternum and rib cage significantly In this classification system, thoracolumbar injuries are
restrict thoracic spine motion and stabilize this region grouped into minor and major injuries. Minor injuries
(15). Increased stability of the thoracic spine is also a include fractures of the spinous and transverse processes,
function of facet joint orientation. The more coronal tho- the pars inter-articularis, and the facet joints. Major spinal
racic facets resist flexion and extension and provide min- injuries include compression wedge fractures, burst frac-
imal resistance to torsional loads. The more sagittal lum- tures, shear fractures, and fracture-dislocations.
bar facets permit significant flexion and extension and The criteria for spinal stability (24,25) have been
limit rotation (16). expanded to also include neurologic deficit, degree of
The physiologic kyphosis of the thoracic spine is deformity, and canal compromise (31).
related to the anterior wedging of the vertebral bodies. A mechanistic classification system, based on the
This kyphosis may predispose the thoracic spine to flex- column concept of spinal stability and associated deform-
ion instability, particularly after surgical laminectomy or ing forces, provides insight into the pathogenesis of tho-
traumatic posterior element destruction (17). racolumbar injuries (32).
Although the increased stability of the supporting In compression-flexion injuries, loading of the flexed
structures in the thoracic spine provides relative protec- spine creates compressive stress through the anterior col-
tion for the spinal cord, the narrower thoracic spinal umn and tension strain through the middle and posterior
canal diameter, as compared with the larger lumbar canal columns. Three major patterns of failure in this type of
diameter, leaves little room to spare for neural element mechanism can occur. In one pattern, only the anterior
SURGICAL MANAGEMENT FOR THORACOLUMBAR SPINAL INJURIES 145
column fails in compression: an anterior wedge com- the involved vertebra (37). These fractures are usually asso-
pression fracture results and the middle and posterior ciated with paraplegia. Neurologic decline occurs most
columns remain intact. In a second pattern, the posterior often with this injury mechanism (36,38). If there is no sur-
column is disrupted by tensile forces: an anterior wedge gically stabilization, progressive deformity can occur (39).
fracture develops and the middle column remains intact.
In a third pattern, the middle column fails in conjunction
with the other columns and elements of the middle col- NONOPERATIVE TREATMENT
umn are retropulsed into the spinal canal. Compression-
flexion injuries represent the most common mechanism Medical Management
of injury in thoracolumbar fractures.
Distraction injuries are less common and may occur Early medical management may play a critical role in the
in conjunction with flexion, extension, and lateral bend- treatment of acute thoracolumbar injuries with neuro-
ing deforming forces. Approximately 50 percent of logic deficit. Maintaining optimal spinal profusion
patients with distraction-type injuries have associated through aggressive medical resuscitation can favorably
intra-abdominal injuries and an increased incidence of influence neurologic recovery (40).
concomitant, noncontiguous spine fractures (4,6). Neurologic deficit following traumatic SCI is related
In flexion-distraction injuries, the fracture pattern is to structural damage of the neural elements and to com-
determined by the location of the axis of rotation at the plex molecular and cellular mechanisms. Local edema,
time of injury. When the axis of rotation is in the verte- microvascular disruption, and thrombotic events propa-
bral body, a compression fracture of the anterior verte- gate the release of various neurotoxic and inflammatory
bral body occurs in combination with posterior ligament mediators that may damage the blood-brain barrier and
disruption and facet joint dislocation. All the spinal neural elements (13,41,42).
columns fail under tension when the axis of rotation is Pharmacologic agents can protect the neural tissues
anterior to the vertebral column. from the deleterious effects of neurotoxic mediators
Chance-type fractures (33), or variations thereof, released at the time of spinal cord injury. Although vari-
occur primarily as a result of tensile failure. Tension fail- ous pharmacologic therapies have been suggested, none
ure can propagate through the bony spine only, through has unequivocally demonstrated reversal of SCI.
the ligamentous structures only, or through a combina- Intravenous steroids can contribute to cellular mem-
tion of these two patterns. brane stabilization, reduce edema, and buffer electrolyte
Distraction-extension injuries result from tension imbalances (13,43,44). The use of steroids, however, may
failure of the anterior column and compression failure increase the likelihood of pneumonia and sepsis in
of the posterior column: they are relatively rare (34). patients receiving a 48-hour steroid regimen (43,65).
These injuries may be difficult to detect by plain radi- Other pharmalogic agents and protocols are being
ographs, because they may spontaneously reduce shortly investigated (46), but none has unequivocally demon-
after injury (32,35). strated the reversal of SCI.
Lateral-flexion injuries develop when lateral bend-
ing of the spine causes an asymmetric compression of the
Closed Reduction and Immobilization
vertebral body and of the posterior elements. In one pat-
tern, the anterior and middle vertebral columns fail uni- Most mechanically stable thoracolumbar injuries with-
laterally. In another pattern, failure of the posterior ele- out neurologic deficit can be treated nonoperatively (46).
ments occurs from compression on one side and tension Nonoperative treatment should be considered in stable
on the other. On the side of tension failure, a unilateral injury patterns with limited potential for progressive
facet dislocation may occur. deformity and neurologic compromise.
Translational injuries develop when shear forces dis- Minor spine fractures are considered stable and may
place the vertebral body anteriorly, posteriorly, or later- be treated symptomatically, with or without bracing (48).
ally. With displacements greater than 25 percent, the facet These fractures should not be ignored and may signal
joints and all ligamentous structures, including the ALL, more significant associated injuries. Major spine frac-
are often disrupted. Because few or no stabilizing struc- tures, such as flexion-compression type fractures, with a
tures remain, this injury pattern has the highest risk of loss of vertebral body height of less than 50 percent, and
associated neurologic injury and can result in disabling initial kyphosis of less than 30 degrees, may be treated
chronic deformity (21,36). with closed reduction and immobilization by casting or
In torsion-flexion injuries, the anterior column fails bracing (49,50).
in compression and rotation while the posterior column Nonoperative treatment of mechanically unstable
fails in tension and rotation. The facet joints are usually thoracolumbar injuries without neurologic deficit remains
fractured and dislocated and the ALL may be stripped off controversial. The initial degree of mechanical instability,
146 ACUTE SPINAL CORD INJURY MANAGEMENT AND SURGICAL CONSIDERATIONS
kyphosis, and canal occlusion that can be managed non- can facilitate early rehabilitation and prevent late post-
operatively is not clear. Complication rates appear simi- traumatic deformity.
lar in both nonoperative and operative cases, with the
exception of surgical wound infections. The length of hos-
pital stay is longer in patients treated nonoperatively (51). OPERATIVE TREATMENT
Burst fractures with less than 30 degrees of kypho-
sis, less than 50 percent loss of vertebral body height, and Indications for Surgery
less than 50 percent canal compromise are considered sta-
The indications for surgical intervention of thoracolum-
ble (52,53). For stable thoracolumbar burst fractures
bar injuries depend on the pattern of injury, alignment
without neurologic deficit, no significant difference in
and stability of the vertebral fracture, neurologic status
functional outcome was observed in patients treated non-
of the patient, and overall medical condition of the
operatively when compared with those patients treated
patient.
operatively (54). Of forty-one patients treated nonoper-
atively, 66 percent had good or excellent results and 90
percent returned to full-time work (55). Similar results Timing of Surgery
have been observed by others (56,57). In patients with spinal injury and neurologic deficit, the
In some unstable thoracolumbar burst fractures optimal timing for medical and/or surgical intervention is
without neurologic deficit, functional outcomes appear debated.
favorable when treated nonoperatively (58,59). Although not conclusively demonstrated in human
Nonoperative treatment is less commonly used for clinical studies, a critical window of opportunity may
thoracolumbar flexion-distraction injuries and fracture- exist in which decompression of the spinal elements may
dislocations. Nonoperative treatment may be considered enhance functional neurologic outcome. In animal mod-
for purely bony flexion-distraction injuries. The results of els, early decompression influenced the potential for neu-
nonoperative treatment, however, are relatively poor. In rologic recovery. Decompression performed within 1 to
twenty-eight patients with a flexion-distraction injury, 3 hours of spinal injury led to recovery of electrophysio-
only one of thirteen patients treated surgically had a poor logic function (69). Some clinical studies suggest that early
outcome when compared to four of seven patients who decompression occurring less than 24 hours post injury,
were treated nonoperatively (60). Results were poor if may be optimal (70). In other cases, early surgical inter-
there was facet joint involvement or an initial kyphosis vention has led to an increase in neurologic deterioration
greater than 17 degrees (61). (71). In the only controlled prospective randomized study
Neurologic recovery can occur in patients with tho- to date, no significant difference in functional neural
racolumbar injuries treated nonoperatively. In patients recovery was demonstrated with surgical intervention,
with an incomplete neurologic deficit, improvement of early or late (72).
at least one Frankel grade was observed in as many as
95 percent of patients (62). When treated nonoperatively,
Surgical Decompression
eight of thirteen patients with thoracolumbar fractures
with neurologic deficit demonstrated complete neuro- In thoracolumbar injuries with neurologic deficit, the ben-
logical recovery. Neurologic recovery may in part be efit of surgical decompression is still undetermined
attributed to spontaneous remodeling of impinging bony (36,73,74). In patients with an incomplete neurologic
fragments within the spinal canal (58). deficit, improvement in neurologic function has been
Neurologic decline has been observed in 2 to 21 per- observed after surgical reduction and stabilization
cent of patients with thoracolumbar injuries treated non- (75,76). In patients with a complete neurologic deficit
operatively (6264). This may be related to late kyphotic and/or unstable thoracolumbar fracture, surgical stabi-
deformity and/or late spinal stenosis. Segmental kypho- lization has decreased hospitalization and rehabilitation
sis increased by an average of 6 to 10 degrees in 10 to 36 times and the rate of complication (77,78). Although
percent of patients managed nonoperatively (65,66). approximately 60 percent of patients with neurologic
Canal stenosis and kyphosis may additively cause neural injury below T12 will gain some return of neurologic
compression. Approximately 20 degrees of kyphosis function with nonoperative treatment, neurologic recov-
appears acceptable with less than 35 percent spinal canal ery is more predictable after anterior decompression in
occlusion, whereas 30 degrees of kyphosis, combined certain cases (73,79,80). Late decompression, even years
with more than 70 percent canal occlusion, increases the following injury, may enhance neurologic recovery at the
risk of neurologic symptoms (67). cord level, the conus medullaris, and the cauda equina
In patients with complete neurologic injury, nonop- (8183).
erative treatment with 4 to 6 weeks of bed rest yielded Despite varied opinions, there is no direct correla-
satisfactory results (68). Surgical stabilization, however, tion between the percentage of canal occlusion demon-
SURGICAL MANAGEMENT FOR THORACOLUMBAR SPINAL INJURIES 147
strated radiographically and the severity of neurologic terior instrumentation (91). A laminectomy may be per-
deficit following burst fractures (8486). The initial max- formed in selective cases of thoracolumbar injuries: the
imal amount of contusion of the spinal cord or cauda repair of dural tears caused by posterior-element fractures,
equina may be an underlying cause of neurologic injury. the evacuation of an epidural hematoma, and when direct
In patients with burst fractures, an increased risk of neu- neural compression from displaced posterior vertebral
rologic injury may be observed with spinal canal occlu- fracture fragments occurs (84). Posterolateral approaches
sion greater than 35 percent at T11 and T12, 45 percent for spinal decompression include costotransversectomy,
at L1, and 55 percent at L2 (87). In 148 patients with posterolateral transpedicular decompression, lateral extra-
burst fractures of the thoracolumbar spine, the average cavitary decompression, and lateral extrapleural paras-
percentage of canal compromise was higher in those with capular decompression (9296). Where there is signifi-
neurologic deficit (52 percent) than in those without neu- cantly lateralized or asymmetric anterior canal occlusion,
rologic deficit (35 percent). The degree of neurologic the posterolateral transpedicular and the lateral extra-cav-
impairment was greater in those patients with disruption itary approaches may be useful. These approaches are
of the posterior elements (69.2 percent) than in those with more advantageous in the lower lumbar regions, where
intact posterior elements (29.8 percent). However, the anterior instrumentation and reconstructive techniques
extent of neurologic recovery was greater in the patients pose risks to the neighboring great vessels. The lateral
with disruption of the posterior elements (60.7 percent) extrapleural parascapular decompression, a modification
than in those with no disruption of the posterior elements of the lateral extracavitary approach, may simplify access
(88). to the upper thoracic spine.
The outcomes of anterior versus posterior spinal The anterior approach is the most direct and reliable
surgery for the management of thoracolumbar injuries method for visualizing and decompressing the affected
have been compared. neural elements (83,97).
In a retrospective review of eighty-seven patients
with incomplete neurologic injuries, neurologic recovery
was greater with anterior decompression as compared INDICATIONS AND OPERATIVE TECHNIQUES
with posterior decompression, 88 versus 64 percent FOR THORACOLUMBAR INJURIES
respectively. This was more evident with regard to blad-
der and bowel control, 65 versus 33 percent respectively. Compression Fractures
Patients with inadequate posterior decompression and Surgical stabilization is indicated in compression wedge
residual neurologic deficit improved after a subsequent fractures if there is greater than 30 degrees of initial
anterior decompression (83). kyphosis, and/or greater than 50 percent loss of anterior
In sixty consecutive patients with SCI and greater vertebral body height with posterior element disruption.
than 20 percent encroachment of the spinal canal, no sig- Posterior spinal surgery is the most common method of
nificant change in neurologic improvement was detected the operative management of this injury. It relies on reduc-
if posterior or anterior surgery was performed (85 and 88 tion and stabilization of the fracture with distraction
percent respectively) (85). instrumentation (98).
In a prospective randomized study of forty patients Lateral flexion-compression fractures at risk for pro-
with burst fractures, treated either with indirect posterior gression can be stabilized with a distraction construct on
spinal decompression and stabilization with the AO fix- the injury side and a compression construct on the unin-
ateur, or with anterior decompression and fixation with jured side.
the Kostuik-Harrington device, it was found that anterior
decompression resulted in more effective canal clearance.
Burst Fractures
Correction of kyphosis was similar in both groups. Blood
loss was greater with the anterior surgery (79). The management of thoracolumbar burst fractures is con-
troversial. If surgery is elected, stabilization techniques
should be constructed for the specific fracture pattern. For
Surgical Approaches for Spinal Decompression
unstable burst fractures at the thoracolumbar junction
Decompression procedures may be performed posteriorly, with neurologic deficit, direct anterior decompression of
posterolaterally, or anteriorly. the neural elements followed by spinal stabilization
In thoracolumbar injuries, decompression by a should be considered (79).
laminectomy is usually destabilizing and should be avoided If decompression is elected, an anterior surgical
(89). A laminectomy, in these cases, may lead to post- approach provides more direct, predictable spinal canal
laminectomy kyphosis and progressive neurologic deficit decompression when compared to indirect decompres-
(37,90). The risk of progressive deformity from a laminec- sion by posterior ligamentotaxis (97). In one series, ante-
tomy may be reduced by the application of stabilizing pos- rior decompression improved spinal canal occlusion from
148 ACUTE SPINAL CORD INJURY MANAGEMENT AND SURGICAL CONSIDERATIONS
58 percent preoperatively to 4 percent postoperatively. By struct, spanning one level above and one level below the
posterior indirect reduction, spinal canal occlusion injured level, usually provides adequate stability. If the
improved from 45 percent preoperatively to 16.5 percent middle vertebral column is disrupted, caution should be
postoperatively (79). exercised to avoid retropulsion of bony fragments into
Following decompression, the type of reconstruc- the spinal canal. If the axis of rotation is within the ver-
tive technique selected should be based on the type of tebral body, and anterior column compression failure
fracture pattern and the extent of vertebral deficiency and develops, a distraction construct may be used. The ALL
deformity. serves as a stabilizing tension band in this situation.
If the posterior element tension band remains intact,
anterior and middle column insufficiency at the thora-
Fracture-Dislocations
columbar junction should be reconstructed with an inter-
body implant and reinforced with stand-alone anterior Fracture-dislocations are rotationally and/or translation-
instrumentation (99105). In 150 patients with thora- ally unstable and require surgical stabilization. Postural
columbar burst fractures with associated neurologic reduction is ineffective in bilateral facet dislocations
deficit managed with a one-stage anterior decompression, (122). Standard techniques of hyperextension position-
strut grafting, and anterior instrumentation, 95 percent ing are counterproductive in the reduction of fracture-dis-
of the patients demonstrated functional neurologic locations and may make the reduction more difficult. If
improvement of at least one Frankel grade, and 72 per- posterior reduction is not adequate, or residual neural
cent of the patients experienced complete neurologic compression is detected, additional anterior decompres-
recovery (106). In unstable fractures with posterior col- sion and stabilization may be performed following the
umn disruption, additional posterior stabilization should posterior approach (123).
be considered.
Despite the increased rigidity of posterior pedicle
Distraction-Extension Injuries
screw systems (107110), stand-alone posterior short-seg-
ment pedicle screw fixation of unstable thoracolumbar Distraction-extension injuries are uncommon, unstable
fractures has resulted in high failure rates (111,112). injuries that often are associated with neurologic injury.
These failures appear to be related to the fracture pat- Supine positioning may exacerbate the deformity and
tern and to the inability of the injured vertebral bodies may contribute to neurologic decline. After postural
to load-share. reduction, spinal stability and alignment can be main-
The best burst-fracture candidates for stand-alone tained by posterior segmental fixation. Anterior proce-
posterior short-segment fixation are those with less than dures are reserved for injuries with a significant anterior
30 percent vertebral body comminution on sagittal CT fish-mouth type deformity, which may lead to late spinal
images, less than 2 mm displacement between fracture instability.
fragments, and less than 10 degrees of kyphosis correc-
tion required (113).
In cases with significant anterior column deficiency CONCLUSION
not requiring decompression, stand-alone posterior short-
segment fusion and instrumentation should be avoided The appropriate treatment of thoracolumbar spinal
(111,114,115). A circumferential surgical approach injuries should be guided by a thorough understanding of
should be considered (116118). The sequence of surgi- the interrelationships of spinal anatomy, spinal biome-
cal technique depends on the fracture pattern. Anterior chanics, injury mechanisms, and fracture patterns. Early
decompression, and/or reconstruction, is usually per- medical management and early surgical decompression
formed first. Anterior column reconstruction improves may be beneficial in preserving and improving neurologic
the load-sharing and load-transferring ability of the ante- function. Nonoperative treatment can provide satisfac-
rior spinal column (119,120). If posterior instability is tory outcomes in most stable thoracolumbar injuries. For
present following the anterior procedure, posterior sta- unstable thoracolumbar injuries, with or without neuro-
bilization is recommended (121). logic deficit, surgical treatment should be considered.
Flexion-Distraction Injuries
References
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Flexion-distraction injuries involving predominantly soft ture study. Spine 1992; 17:528539.
tissue disruption should be surgically stabilized, because 2. Stansias MJ, Latham JM, Porter KM. A high-risk group for tho-
the disrupted interspinous ligaments, PLL and disc heal racolumbar fractures. Injury 1998; 29(1):1518.
3. Savitsky E, Votey S. Emergency department approach to acute
slowly and unpredictably in the adult (6). If the middle thoracolumbar spine injury J Emergency Med 1997; 15(1):4960.
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MEDICAL MANAGEMENT
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11 Respiratory Dysfunction in
Spinal Cord Disorders
155
156 MEDICAL MANAGEMENT
versus 86 cm H2O in the control subjects. The PEmax was major for 6 weeks improves the expiratory function in
57 cm H2O versus 126 cm H2O, respectively. these patients (16). Estenne and coworkers (17) demon-
In the study by Sinderby and coworkers (6), approx- strated that the abdominal muscles retain their force-gen-
imately 80 percent of patients with low cervical cord erating ability if they are stimulated by an external source.
injury (C5C8) had diaphragm muscle strength within the The application of paired magnetic stimulation to the
range of that of normal subjects (mean, 120 cm H2O). abdominal muscles achieves an increased intrathoracic
Diaphragm muscle strength was measured as the maxi- pressure that exceeds the pressure required to cause
mum transdiaphragmatic pressure (PDImax). Subjects dynamic airway compression, thus providing the poten-
achieved this maximum pressure using a visual-feedback tial to improve cough and airway clearance. There was a
method in which the subject was encouraged to generate positive correlation between the change in gastric pres-
a target transdiaphragmatic pressure displayed on an sure (Pga) obtained during magnetic stimulation of the
oscilloscope (7). Despite the maintenance of inspiratory abdominal muscles at total lung capacity (TLC) and
muscle and diaphragm muscle strength, both global inspi- abdominal muscle thickness. This confirmed the associ-
ratory and diaphragm muscle endurance is limited in ation between atrophy of the abdominal muscles and the
these patients. When they breathed against an incremen- reduction in abdominal muscle strength. In these patients,
tal threshold load, the ratio of the pressure generated at abdominal muscle strength is 60 percent less than in
the maximum load to the PImax was significantly lower healthy control subjects (14). Abdominal muscles also
than that of the control subjects (49 versus 82 percent), demonstrate phasic electrical activity when ventilatory
suggesting that the inspiratory muscles have limited demand increases; such activity is absent during tidal
capacity to generate pressure against the load (5). These breathing (18). This phasic electromyographic activity is
findings indicate early development of inspiratory mus- believed to be triggered by stretching of the abdominal
cle fatigue (5). During resting breathing, the tensiontime wall on inspiration. Its effect decreases the compliance
index of the diaphragm (TTDI), defined as the product of of the abdominal wall, allowing the diaphragm to gener-
the ratio of mean transdiaphragmatic pressure (PDI) and ate a greater intra-abdominal pressure during inspiration,
PDImax, and the duty cycle (TI/Ttot; the ratio of inspira- and causing improved inflation of the lower rib cage.
tory time to total breath cycle duration, respectively) is With paralysis of both the major inspiratory and
higher than that of normal subjects (0.07 versus 0.04, expiratory muscles, as in patients with high spinal cord
respectively). This provides further evidence of diaphragm injury (C1C2), De Troyer and coworkers (19) demon-
muscle fatigue (6,8). In fact, during arm or leg ergome- strated that these patients can use the sternocleidomas-
ter exercises, seven of the ten patients with low cervical toid muscles, as well as other neck muscles (trapezeii,
cord injury were observed to develop diaphragm muscle platysma, sterno, and mylohyoid) to sustain brief periods
fatigue at low workloads, preceding the development of of spontaneous breathing. Contraction of these muscles
task failure (9). Despite the development of diaphragm expands the upper rib cage anteriorly, decreases pleural
muscle fatigue, those patients were still capable of increas- pressure, and is accompanied by a fall in intra-abdomi-
ing the PDI associated with increasing tidal volume (VT) nal pressure, thus causing inward displacement of the lat-
and respiratory frequency (fr) until task failure. Further- eral walls of the lower rib cage. Thus, the benefit of
more, conditioning of the diaphragm with inspiratory recruiting the accessory muscles to augment spontaneous
resistive training (10) or phrenic nerve stimulations breathing can be negated by the paradoxical movement
appear to improve strength and endurance of the of the lower rib cage.
diaphragm (11) and lung function (12).
In patients with low cervical cord injury, the
Respiratory Mechanics
diaphragm not only functions as the major inspiratory
muscle, but also as a trunk extensor muscle (13). When
Lung Mechanics
performing forward trunk flexion, these patients exhibit
continuous and augmented diaphragm electrical activity During acute injury, the forced vital capacity (FVC) of
and abdominal pressures (14). During posture imbalance, patients with tetraplegia is markedly reduced, with no
the postural needs temporarily override the respiratory expiratory reserve volume detectable (20). The level of
function, but this postural loading to the diaphragm risks injury, however, does not correlate with the FVC (21).
the development of diaphragm muscle fatigue (13). Ledsome and Sharp (20) showed that 1 week after injury,
Paralysis of the abdominal muscles in patients with the FVC of patients with tetraplegia (C5C6) was 30 per-
SCI results in ineffective cough and impaired clearance of cent and the forced expiratory volume in 1 sec (FEV1)
airway secretions. To assist in cough, during forced expi- was 27 percent of predicted normal value (20). Both FVC
ration patients with tetraplegia can recruit the clavicular and FEV1 improve significantly at 5 weeks (45 and 42
portion of the pectorals major to deflate the rib cage (15). percent of predicted, respectively) and continue to
Repetitive isometric contraction training of the pectoralis improve at 5 months after the injury (58 and 59 percent
RESPIRATORY DYSFUNCTION IN SPINAL CORD DISORDERS 157
of predicted, respectively) (20). Gains in ventilatory para- tion of changes in total lung capacity (TLC) and functional
meters are small thereafter (4,22). In the Ledsome and residual capacity (FRC) is similar to that of healthy sub-
Sharp (20) study, five patients with injury to the lowest jects: that is, both TLC and FRC decrease in the supine
functional segment of C4 showed a mean FVC 24 percent posture when compared to the seated posture (26,27). In
of predicted, and all of these patients required mechani- healthy subjects, VC decreases when changing from the
cal ventilatory support for 2 to 3 weeks. Three months upright to the supine posture (28). In contrast, patients
postinjury, their FVC increased to 44 percent of predicted with tetraplegia and high paraplegia exhibit VC increases
(range 18 to 62 percent). when changing from the upright to the supine posture (27)
The impaired function of the diaphragm in the acute (Figure 11.1). Estenne and De Troyer (27) described supine
stage of injury is attributable to the mechanical ineffi- position increases of 16 percent in tetraplegic subjects and
ciencies associated with paradoxical rib cage movement 11 percent in paraplegic subjects. This increase in VC in
and unfavorable changes in thoracoabdominal compli- the supine posture results primarily from the reduction in
ance as a result of inspiratory muscle paralysis. The residual volume (RV) caused by the effect of gravity on the
improvement of lung function over time could be related abdominal contents (Figure 11.2). The application of an
to improved neurologic function in the cord segments at abdominal binder helps to maintain the RV in the seated
and above the injury level. The development of spastic- posture by compressing the abdominal contents into the
ity and reflex contraction of the intercostal and abdomi- diaphragm, thus improving thoracoabdominal compliance
nal muscles may cause the rib cage to become more sta- and placing the diaphragm at a more efficient, stretched,
ble (23). In the acute stage, the degree of lung function resting posture. This effect appears to be independent of
impairment and the level of the injury are not predictive changes in intrathoracic blood volume (27).
of the recovery of lung function (22). The lung compliance (CstL) of patients with acute
In the chronic stage of injury, the level and com- and chronic tetraplegia is markedly reduced, approxi-
pleteness of injury, combined with tobacco smoking his- mately 52 percent of that of control healthy subjects (29).
tory, serve as predictors of lung function impairment This reduction in CstL plateaus 1 month after the injury
(24,25). Almenoff and coworkers (24) studied 165 and does not change significantly over time. When CstL
patients with SCI. Patients with high complete cervical is corrected for lung volume, the mean value falls to near
tetraplegia had lower FVC, FEV1, and peak expiratory 70 percent of predicted value for healthy subjects (29).
flow (PEF) than those with incomplete lesions of the same This suggests that the decrease in CstL is partly attribut-
and lower levels of injury. In the patients with low able to reduced lung volume and partly to the altered
tetraplegia or high and low paraplegia, the extent of the mechanical properties of the lung. De Troyer and Heil-
motor lesion did not affect the degree of lung function porn (30) demonstrated similar findings in their patients
impairment. Tobacco smoking played a significant role. with chronic tetraplegia (CstL of 60 percent of predicted
The FEV1 and PEF in current smokers were markedly value for healthy subjects) in whom intercostal muscle
reduced compared with ex-smokers and those who never activity was completely absent. Conversely, in the two
smoked. patients in whom intercostal muscle electrical activity was
Posture affects lung function in patients with SCI detected, CstL was within normal limits (93 percent of
(25). With the exception of vital capacity (VC), the direc- predicted). The authors concluded that the intercostal
1.0 2.0 3.0 4.0 5.0 3.0 4.0 5.0 6.0 7.0
VC - Seated (L) VC - Seated (L)
158 MEDICAL MANAGEMENT
muscles help to stabilize the chest wall and prevent lung compared to healthy subjects (33). In addition, when
collapse. abdominal contents fall toward the pelvis as an upright
posture is achieved, the diaphragm lacks a fulcrum;
diaphragm contraction results in reduced lower rib cage
Chest Wall Mechanics
expansion and further reduces tidal volume. The use of
The chest wall compliance of patients with tetraplegia is binders while the patient is supine is not recommended,
also reduced. Estenne and De Troyer (31) studied thora- because restriction of the lower rib cage by this practice
coabdominal compliance in twenty patients with chronic reduces tidal volumes.
tetraplegia. The thoracic compliance decreased to an aver-
age of 55 percent of normal, whereas the abdominal com-
Airway Mechanics
pliance increased to 170 percent of normal. Thus, the
decreased chest wall compliance is primarily caused by During cough, healthy subjects develop peak pleural pres-
the abnormally stiff rib cage in the chronic stage of injury sures of greater than 100 cm H2O (34). This induces
(Table 11.1). The stiffened rib cage in these patients is dynamic compression of the intrathoracic airways, which
likely caused by alterations in the elastic properties of the causes the linear velocity and kinetic energy of the flow-
rib cage itself. With decreased physical activity, fewer ing gas to increase markedly (35), thus accelerating mucus
sighs (32), and paralysis of the intercostal muscles, rib secretions mouthward. Because of paralysis of the
cage excursion is diminished. With reduced load on rib abdominal muscles, the cough effort is impaired in
cage structures, a gradual stiffening of rib cage tendons patients with SCI, particularly in patients with tetraple-
and ligaments occurs, which can lead to ankylosis in the gia. Despite paralysis of the expiratory muscles, Estenne
rib cage joints (31). These compliance changes result in and coworkers (36) recently demonstrated evidence of
greater abdominal contribution to tidal volume, when dynamic airway compression in six of the twelve patients
TABLE 11.1
Static Lung and Chest Wall Compliance and its Rib Cage and
Abdominal Components in Patients with Quadriplegia
STATIC LUNG COMPLIANCE STATIC CHEST WALL COMPLIANCE RIB CAGE COMPLIANCE ABDOMINAL COMPLIANCE
0.215 0.010 73.4 3.4 0.132 0.009 72.1 4.5 0.085 0.005 55.1 3.4 0.047 0.004 170.7 15.0
studied. Patients who demonstrated dynamic airway com- larly, the metacholine hyperresponsiveness is also blocked
pression show an obvious flow plateau at lower lung vol- by baclofen, a -aminobutyric acid (GABA) agonist.
umes on the isovolume esophageal pressure flow curves Through interaction with GABA receptors, baclofen
(Figure 11.3). Direct observation with videoendoscopy inhibits the release of acetylcholine from the postgan-
confirms the dynamic compression of the airways in those glionic cholinergic neurons that innervate airway smooth
patients. The results of this study suggest that contraction muscle (41).
of the clavicular portion of the pectoralis major is capa-
ble of producing dynamic compression of the airways.
Control of Breathing
For this reason, strength-training of the pectoralis major
to enhance its pressure generating capacity will poten-
Pattern of Breathing
tially enhance cough effort, particularly when combined
with the application of an abdominal binder (36). The resting breathing pattern in patients with tetraple-
Although patients with tetraplegia demonstrate gia is characterized by a small VT, increased respiratory
dynamic airway collapse, one study of patients with rate, and a VE similar to that of age-matched healthy con-
chronic tetraplegia demonstrated a significant bron- trols (42). The inspiratory time (TI) is the same as in con-
chodilation effect with the administration of inhaled trols, whereas expiratory time (TE) is significantly short-
bronchodilator (37) and airway hyperreactivity to inhaled ened, thus accounting for the increase in respiratory
metacholine (38). The mechanism of this airway hyper- frequency. Mean inspiratory flow rate (VT/Ti) is smaller
responsiveness is thought to be caused by the unopposed than in controls. In the seated position, when VT is par-
cholinergic tone, since the inhaled anticholinergic agent, titioned between the rib cage and abdominal compart-
ipatropium bromide, blocks the response (3840). Simi- ments, expansion of the abdomen is associated with
expansion of the lower rib cage. However, rib cage expan-
sion is greater in the transverse than in the anteroposte-
8 A 70% VC
rior diameter. In addition, many of these patients have a
50% VC reduced anteroposterior diameter of the upper rib cage
30% VC (paradoxical rib cage motion) (43,44) (Figure 11.4). This
Ve xp (L/s )
FIGURE 11.5
Left panel: Ventilatory response to hypercapnia (D E/ D PCO2) in nine patients with quadriplegia (Q) and eight normal (N)
subjects. Middle panel: The ventilatory response to hypercapnia normalized for maximum voluntary ventilation (MVV). Right
panel: The ventilatory response to hypercapnia normalized for vital capacity (VC), respectively. The ventilatory response to
hypercapnia in patients with Q is significantly less than in N subjects (p 0.001), even when normalized for indices of respi-
ratory muscle performance. Closed circles are mean values, bars are standard deviation. (From reference 50 with permission.)
changes in PCO2, whereas perceived work and effort ning and coworkers (58) studied the sensation of air
is associated with changes in ventilation. The sensation hunger in ventilator-dependent patients with C23
of air hunger arises as a result of chemoreceptor affer- quadriplegia. In this study, first VT and the respiratory
ent activity or medullary corollary discharge (56,57). frequency of the ventilator were maintained constant at
Conversely, the sensation of work and effort arises baseline ventilator settings while end-tidal CO2 (PETCO2)
from respiratory muscle mechanoreceptors, awareness of was increased. Then, VT was reduced 40 to 70 percent of
cortical motor drive to respiratory muscles, or both (56). baseline VT, while PETCO2 was maintained constant. The
In patients with SCI, the perception of air hunger to sensation of air hunger correlated significantly with the
increased PCO2 or reduced tidal volume is intact. Man- mean PETCO2. Similarly, there was a correlation between
1.6
(L/min/torr)
0.3
surements of the ventilatory
1.2
0.2 (VE/PCO2) and occlusion pres-
. 0.8 sure (P0.1/PCO2) responses to
0.1 hypercapnia, and respiratory
0.4
muscle function in two patients
0.0 0.0 with acute quadriplegia. Lower
0 100 200 300 0 100 200 300 panel: Improvement in respira-
Time from injury (days) tory muscle function, vital capac-
ity (VC), and maximum inspira-
tory pressure (MIP) was not
5 90 0.75 associated with an increase in the
ventilatory or occlusion pressure
MIP (cm H20)
4
response to hypercapnia. (From
ERV (L)
60 0.50
3
VC (L)
0 0 0.00
0 100 200 300 0 100 200 300 0 100 200 300
VT and the sensation of air hunger, although the sen- amounts of secretions are produced because of unop-
sation of work or effort was not evaluated. Neverthe- posed vagal tone (64). This leads to acute respiratory dis-
less, these findings suggest that the sensation of air tress, hypoxemia, worsening hypercapnia (which may be
hunger in patients with tetraplegia is intact, and its mod- superimposed on chronic alveolar hypoventilation), and
ification is independent of afferent information from the the need for intubation for ventilatory support and air-
chest wall. way suctioning. When the expiratory muscles are assisted
to help eliminate secretions, these events can often be
avoided.
MANAGEMENT OF RESPIRATORY Cough flows will be diminished when a) the patient
COMPLICATIONS cannot inhale at least 1.5 L because of weak inspiratory
muscles; b) inadequate thoracic abdominal pressures can-
Respiratory Complications not be generated because of weak abdominal muscles or
loss of glottic control; c) weakened bulbar muscles or tra-
Respiratory failure following acute SCI may occur cheostomy preclude firm glottic closure or upper airway
because of partial or complete respiratory muscle paral- stability during the cough; or d) irreversible airway
ysis, fatigue of remaining intact muscles, or in association obstruction occurs, such as with tracheal stenosis or
with pleuropulmonary pathology. Jackson and Groomes chronic obstructive pulmonary disease. To prevent pneu-
(59), in their study of 205 patients with high quadriple- monia and respiratory failure, these patients more often
gia (C14), found the most common complications were receive supplemental oxygen and physical therapy to clear
atelectasis in 40 percent and pneumonia in 63 percent of airway secretions, rather than therapies to assist their res-
the patients. These findings are similar to those in Fish- piratory muscles.
burns study (60), which showed a 50 percent incidence
of atelectasis or pneumonia in spinal cord-injured patients
with injury level from C3 to T11. Assisted Cough Techniques
Many patients with high cervical cord injury will As a rule, anyone with a VC below 1.5 L should receive
recover substantial respiratory function following the a maximal insufflation delivered by a manual resuscita-
acute insult. Ledsome and Sharp (20) measured the vital tor or portable volume ventilator to achieve adequate
capacity (VC) sequentially in patients still breathing spon- cough flows. This action alone can more than double
taneously after cervical spinal damage and demonstrated peak cough flows (65). Once an optimal volume is
a progressive improvement from 31 percent to 58 percent reached, the expiratory muscles can either be self-assisted
of predicted VC between the 1st and 20th week. In a ret- or manually assisted by one of several methods that
rospective study, Carter et al. (61) showed that of the require care-provider assistance (66). Manually assisted
twenty-two patients with injury level at and below C4 coughing techniques include the costophrenic assist, the
with unilateral diaphragmatic paralysis, sixteen (73 per- Heimlich-type or abdominal thrust assist, anterior chest
cent) had improvement of VC after a mean interval of compression assist, and the counterrotation assist (65,66).
76 days, the longest duration for recovery being 14 Most individuals with inadequate vital capacities but
months. Even patients with a C2C3 level of injury can good bulbar muscle function can also be taught glos-
expect a significant recovery of diaphragmatic function. sopharyngeal breathing to obtain optimal volume (67).
Oo et al. (62) found that 25 percent of patients with a VC
of 15 ml/kg could be weaned off ventilatory support
between 93 and 430 (mean 246) days after injury; those Mechanically Assisted Coughing
with a VC of 10 ml/kg weaned later, on the average of Mechanical insufflationexsufflation involves the deliv-
290 days. The weaning time is likely to be influenced by ery of a maximal insufflation followed immediately by a
the duration of paralysis, because those recovering after decrease in pressure of about 80 cm H2O. It is indicated
prolonged paralysis have to regain diaphragm bulk. if the individual cannot maintain a spontaneous VC of
at least 1.5 L because of weak oropharyngeal muscles.
Mechanical insufflationexsufflation can generate as
Cough Effort and Secretion Clearance
much as 600 L/min of expiratory flow that exsufflates
Patients with neuromuscular disorders have diminished (sucks out) both the left and right airways. Insuffla-
cough capacity, primarily because of expiratory muscle tionexsufflation can be applied through a full-face mask,
weakness (63). When these patients, including those who a simple mouthpiece, or a translaryngeal or tracheostomy
have SCI, contract an upper respiratory infection, they tube. It is most effective when bulbar muscle function and
often produce airway secretions that they cannot clear airway patency are adequate to permit manually assisted
because of an ineffective cough. The most difficult prob- peak cough flows of 160 L/min or greater. The ability to
lem is in the acute phase of the cord injury, when large generate adequate peak cough flows is more important
RESPIRATORY DYSFUNCTION IN SPINAL CORD DISORDERS 163
T-IPPV are numerous. These include nosocomial pneu- tages as an option for interim or permanent ventilation of
monia, mucus plugging, cardiac arrhythmias, accidental high SCI individuals without severe bulbar dysfunction.
disconnection, chronic purulent bronchitis, granulation Miller et al. (82) described their experience using the pneu-
tissue formation, stomal infections, sinusitis, tracheoma- mobelt in high tetraplegic patients. Twelve subjects were
lacia, tracheal perforation, hemorrhage, stenosis, fistula, able to progress within days to up to 4 hours of continu-
vocal cord paralysis, and hypopharyngeal muscle dys- ous use, and thereafter to 12-hour or all-day use. The
function (2). The tracheostomy tube requires special major advantages included improved appearance (no tra-
attention, including regular bronchial suctioning, site cheostomy), speech, and mobility. Disadvantages included
care, and cannula changes. Swallowing difficulties may pump noise, promotion of stomach gas, and positioning
occur as a result of impediment to the strap muscles, glot- difficulties. By placing the corsets horizontal upper bor-
tic closure and cricopharyngeal sphincter function, and der approximately two finger breadths below the
compression of the esophagus. Tracheal suctioning may costophrenic junction, one can avoid the paradoxical
cause irritation, bleeding, the formation of granulation expansion of the chest caused by enclosure of the lower
tissues, increased secretions, and hypoxia. Suctioning thorax, as described by Hill (83). The pneumobelts major
often misses the left main bronchus (60). limitation is that individuals must be sitting upright to use
it. This device employs the same principle as the rocking
(oscillating) bed in displacing abdominal contents.
Noninvasive Ventilation
Noninvasive (i.e., nontracheostomy assisted) positive pres-
Electrophrenic Pacing
sure ventilation (NPPV) is increasingly used as an alterna-
tive for patients with chronic respiratory insufficiency. Such The main indications for electrophrenic stimulation are ven-
techniques have been described mostly in the management tilatory insufficiency caused by disruption of central control
of patients with muscular dystrophy and poliomyelitis over phrenic nerve motoneurons and malfunction of the res-
(67,78,79). Until the early 1980s, the literature concern- piratory control center (71). Diaphragmatic pacemakers
ing these methods was mostly limited to the application of cannot be used in patients with respiratory muscle paraly-
body (negative pressure, iron lung) ventilators (67). sis resulting from phrenic nerve (cell body) damage,
Upper airway collapse and aspiration are potential com- parenchymal lung disease, or acute respiratory failure. High
plications, and many patients are unable to tolerate this cervical cord injuries are treatable by diaphragmatic pacing,
method of ventilation over an extended period. provided that the lower motoneurons of the phrenic nerves
Respiratory support by intermittent positive pres- (C3C5) are intact (84). In addition, SCI individuals should
sure via a variety of nasal or oral interfaces has been used be neurologically stable for at least 3 months, and long-term
to support individuals with various neuromuscular and access to technological and clinical support services must be
chest wall disorders (67,76,80). Bach and Alba (76) present. Full time ventilatory support can often be achieved
showed that twenty-three of twenty-five ventilated with progressive conditioning of the diaphragm, usually
patients with traumatic tetraplegia could be converted after 3 to 4 months of bilateral pacing (84).
from tracheostomies to noninvasive ventilation through Fodstadt (85) reported the outcomes in forty
a carefully monitored sequence of manual and mechani- patients using diaphragm pacing unilaterally, on alternate
cal coughing techniques, progressive tracheostomy cuff sides, or both sides simultaneously, 8 to 24 hours daily.
deflation, and adjustment of delivered ventilator volumes Five tetraplegics used their pacers at different time inter-
for adequate insufflation and speech. Some patients can vals during sleep and intermittently during wakefulness.
master the use of mouth IPPV, or learn glossopharyngeal Six tetraplegics stopped pacing after intermittent phrenic
breathing to supplement tidal volumes. nerve stimulation for 1 month to 3 years. By lengthening
The biphasic positive airway pressure system the pulse duration, adjusting the threshold ramp, and
(BiPAP) is a simple, mechanical system that provides non- changing the inspiratory time, it was possible to increase
invasive respiratory pressure support via nasal mask. tidal volumes, decrease upper airway resistance, and
Unlike continuous positive airway pressure, it has the achieve smoother diaphragmatic contractions.
ability to provide different levels of inspiratory and expi- A permanent tracheostomy is usually a necessity
ratory positive airway pressure. The latter also provides with full-time pacing to keep the upper airway unob-
positive end-expiratory pressure. Tromans et al. (81) were structed. There is a lack of coordination between the
able to apply BiPAP successfully in twenty-eight patients. paced diaphragm, the upper airway muscles, and the
This technique was shown to be useful in preventing res- accessory respiratory muscles in these patients. Upper air-
piratory failure and as a means of facilitating weaning way resistance is augmented by diaphragmatic contrac-
from full-time ventilation. tion or diminished inspiratory upper airway muscular
Of the ventilator devices available for home use, the activity and may be induced by diaphragmatic pacing
pneumobelt (a cyclically inflated corset) may have advan- during sleep in patients with SCI (86).
RESPIRATORY DYSFUNCTION IN SPINAL CORD DISORDERS 165
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12 Sleep Disorders in
Spinal Cord Injury
leep, a time of rest for most people, describe the information available on sleep disorders in
tive benefits of sleep. Their sleep time may be shortened NORMAL SLEEP
or their sleep fragmented, and they can awaken feeling
as sleepy as when they went to bed. The few available To understand the disorders that occur during sleep, it is
studies on sleep in spinal cord injury (SCI) patients sug- important to have an understanding of normal sleep.
gest high rates of sleep disorders. In one questionnaire Despite its external appearance, sleep is a dynamic state,
study of medical problems in SCI, it was found that 34 progressing through multiple stages in a relatively pre-
percent of 353 individuals reported sleep disturbance in dictable manner. During sleep there are two states that
the week prior to the survey and 30 percent complained are as distinct from each other as each is from wakeful-
of fatigue (1). Because this survey was not given to an ness. Dreaming occurs during rapid eye movement (REM)
able-bodied group, the study did not allow a direct com- sleep and is defined by an activated electroencephalo-
parison with SCI. However, a contrast can be drawn from graphic (EEG) pattern (low-voltage, high-frequency wave
a phone survey that found that only 20 percent of unse- activity), muscle atonia, and episodic bursts of eye move-
lected American adults reported being so sleepy that it ments (5). Non-rapid eye movement (NREM) sleep has
interfered with their daily activities in the week prior to a higher voltage, lower-frequency EEG that reflects the
the survey (2). In one study of individuals living in the synchrony of cortical activity. NREM is typically divided
community, 312 men with SCI were found to spend a sig- into four stages, representing a rough continuum of depth
nificantly greater portion of their day sleeping than a of sleep. Stages 1 and 2 represent lighter sleep. Stages 3
comparison group of 3,617 able-bodied men (3). The and 4 are referred to as slow-wave sleep because of their
data suggest that the high prevalence of fatigue in SCI is characteristic low-frequency, high-voltage EEG (5).
caused by disordered sleep. This is supported by another Although stage 2 is restorative, slow-wave sleep is more
study utilizing the Nordic Sleep Questionnaire, which so, being replaced first after sleep deprivation (6).
found that problems with sleep were reported more in SCI There is a typical progression of sleep stages
than in able-bodied controls (4). In this chapter, we throughout the night. The normal adult spends a short
169
170 MEDICAL MANAGEMENT
constant routine protocol holds these variables constant cervical spinal cord. However, this study does not shed
(21). Subjects participated in a 4-day study in rooms free light on the impact that the disruption of melatonin cir-
from time cues, with temperature constant and lights dim. cadian rhythmicity may have on the sleepwake pattern
After an 8-hour sleep period, the subjects began a 46-hour of individuals with SCI.
period of enforced wakefulness in the semirecumbent
position with liquid and meals given in hourly aliquots.
Serum was sampled three times an hour through an SLEEP DISORDERS IN SCI
indwelling catheter. The tetraplegics had no detectable
Sleep-disordered Breathing
basal melatonin levels and demonstrated no rhythmicity
of melatonin secretion. Both paraplegic subjects demon- Obstructive sleep apnea (OSA), a disorder of breathing
strated normal melatonin rhythmicity (see Figure 12.4). during sleep, is characterized by repetitive collapse of the
In contrast, the rhythmic production of cortisol and TSH upper airway. OSA is common in the able-bodied popu-
was intact in all subjects regardless of level. These find- lation and has significant health consequences. Risk fac-
ings support the hypothesis that the human pineal gland tors for OSA in the able-bodied include increased age,
must be stimulated to produce melatonin by the sympa- obesity, increased neck circumference, sedative or alcohol
thetic nervous system via a neural pathway through the use, and a family history of OSA (2731). Several stud-
ies suggest that the SCI population may be at greater risk
for OSA than the general population.
During inhalation, pressure in the pharyngeal air-
way decreases because of the combination of contraction
of the muscles of inhalation and the flow-resistant prop-
erties of the airway. This negative pressure tends to col-
lapse the airway. Because individuals with OSA typically
have smaller than normal pharyngeal airways (32), flow-
resistive pressure drops are greater and, therefore, their
pharyngeal airways are particularly vulnerable to col-
lapse. During wakefulness, reflex and behavioral mech-
anisms activate dilator muscles and maintain patency of
the upper airway (33). During sleep, these mechanisms
diminish and, in OSA, are inadequate, thus leading to
marked narrowing or even complete collapse of the upper
airway. Complete collapse causes apnea: that is, total ces-
sation of airflow (see Figure 12.5). The event terminates
with a brief arousal from sleep and the reestablishment
of neuromuscular compensation to open the airway.
Hypopnea is the term for a significant but not complete
airway collapse that substantially diminishes airflow and
triggers associated arousal and hypoxemia.
The clinical consequences of OSA are the direct
result of abnormalities occurring during sleep. The repet-
itive arousals cause fragmentation of sleep and loss of its
FIGURE 12.4 restorative function, and are also associated with
increased sympathetic nervous system activity. Hypox-
Melatonin waveforms in a group of able-bodied control sub- emia, hypercapnia, acidemia, marked swings in pul-
jects (average SEM; n24; upper left) and in T4A para- monary and systemic blood pressure, and cardiac dys-
plegic (lower left), T5A paraplegic (middle left), C6/7A rhythmias occur during and immediately following the
tetraplegic (upper right), C6A tetraplegic (middle right), and apneic episodes. The end result is excessive sleepiness, per-
C4A tetraplegic (lower right) patients during a 46-hour con-
formance decrements, systemic and pulmonary hyper-
stant routine. Included in the figure are the time of the mela-
tonin maximum (open triangle), the times of habitual bed
tension, and higher than expected rates of cardiovascu-
(solid line) and wake time (dashed line), and the time of a 2- lar events such as stroke and myocardial infarction (34).
hour episode of brighter light (open box). Reprinted with per- The prevalence of OSA in 30- to 60-year-old able-
mission from Zeitzer JM, Ayas NT, Shea SA, Bown R, Czeisler bodied males, defined by an apneahypopnea index
CA. Absence of detectable melatonin and preservation of cor- (AHI) of 15 events per hour of sleep, is 9 percent (27).
tisol and thyrotropin rhythms in tetraplegia. J Clin Endocrinol Initial attempts to study this in SCI were inadequate
Metab 2000; 85:21892196. because only pulse oximetry was used (3537). Such stud-
SLEEP DISORDERS IN SPINAL CORD INJURY 173
FIGURE 12.5
ies do not provide any information on the nature or archi- studied by Ayas et al. (48). Subjects responding to a ques-
tecture of sleep or the cause of the desaturation. tionnaire on respiratory function in SCI were asked about
Studies of the prevalence of OSA in SCI have found the occurrence and frequency of snoring. In the cohort of
rates ranging from 15 to 45 percent (3844); these rates 197 men with chronic stable SCI, 43 percent reported snor-
are much higher than those found in the able-bodied pop- ing. Obesity, defined as the highest quartile of body mass
ulation. Part of the variation is caused by differing defi- index (BMI), being married, and using antispasticity med-
nitions of OSA and the use of different methods for study- ications were predictors of habitual snoring. The highest
ing sleep and breathing (see Table 12.1). Three studies risk for snoring (odds ratio 7.0, 95 percent C.I. 2.7118.09,
used overnight polysomnography, the most sensitive and p 0.001) occurred in those individuals who were over-
accurate method for monitoring sleep and breathing, and weight (BMI 25.3) and used antispasticity medications,
an AHI 15 as a definition of OSA (40,42,44). The rates specifically baclofen, diazepam, or the combination.
found were 27, 28, and 31 percent respectively, which are To test the hypothesis that antispasticity medications
markedly higher than the 9 percent estimate in the gen- were in part responsible for OSA in SCI, a cohort of forty-
eral able-bodied population. two chronic stable SCI subjects underwent overnight
There are many possible reasons for increased OSA polysomnography to look for the prevalence and predic-
rates in SCI. In the able-bodied, the best independent pre- tors of OSA (44). The subjects were inpatients with no
dictors of OSA are increased BMI and neck circumference acute medical problems (i.e., respite care or decubitus ulcer
(27,28). Sedentary lifestyles might lead to increased body care) and not selected for the presence of sleep-related
fat after injury. An increase in neck size, independent of symptoms. Thirty-one percent of the subjects had an AHI
weight gain, has been demonstrated in tetraplegics (45). 15/hour. Older age was the strongest predictor of OSA.
The level and completeness of injury might influence the Within the older age group ( median age of 52 years),
likelihood of OSA. This is because higher level injuries lead obesity was a significant predictor of OSA. These results
to reduced lung volumes. At lower lung volumes, upper are consistent with results in the able-bodied (27). In the
airway diameter is reduced, thus predisposing to airway younger subjects ( median age of 52 years), only baclofen
collapse (46). Medications used commonly in SCI might use was a predictor of OSA. Young subjects not using anti-
contribute to the high rate of OSA. For example, benzo- spasticity medication were unlikely to have OSA. Baclofen
diazepines promote airway collapse through selective is a GABA agonist used to treat spasticity, but it may also
reduction of upper-airway dilator muscle activity (47). decrease upper-airway muscle tone. These data are con-
Because a cardinal symptom of OSA is snoring, sistent with those of Finimore et al. (49), who found that
caused by the partial collapse of the upper airway, the a nocturnal dose of baclofen increased the severity of OSA
prevalence and predictors of snoring in an SCI cohort were in ten able-bodied subjects, and of Burns et al. (43) who
174 MEDICAL MANAGEMENT
TABLE 12.1
Studies of the Prevalence of Obstructive Sleep Apnea (OSA) in SCI
AUTHOR
(REFERENCE) STUDY TYPE OSA CRITERIA N OSA PREVALENCE
PSG polysomnography
SaO2 oxyhemoglobin saturation
AHI Apnea/Hypopnea Index
ODI Oxygen Desaturation Index = average number of SaO2 drops > 4%/hour of sleep
found that the occurrence of OSA correlated with the use Nocturnal Movement Disorders
of antispasticity medications.
The level of injury, the time since injury and subjec- Spasticity is common after SCI and can present as hyper-
tive reports of sleepiness were not predictive of OSA in active tendon jerks, clonus, increased muscle tone, and
this study, as in others (3844). Larger studies are needed spontaneous muscle spasms. These movements can cause
to detect the true prevalence of OSA in SCI and to clar- the fragmentation of sleep. In one survey, 65 percent of
ify the risk groups. However, based on data available cur- respondents reported that spasms, most commonly exten-
rently, it appears that individuals with SCI are at increased sor spasms, interfered with their sleep (52). The usual
risk for developing OSA. Information is not available on treatment is antispasticity medication, typically baclofen
whether the long-term clinical consequences of OSA are and/or diazepam. In a study using intrathecal baclofen
similar in SCI and the able-bodied. This is also an area to reduce leg spasms, postawakening muscle activity was
that needs more study. reduced. This improved sleep continuity by improving the
Continuous positive airway pressure (CPAP) is the likelihood of returning to sleep after a brief arousal (53).
treatment of choice for OSA (50). CPAP is applied to the It is unclear how much of this benefit was caused by the
upper airway via a mask that covers the nose or nose and antispasticity effects or by the purely sedative properties
mouth. The pressure acts as a pneumatic stent to prevent of the medications.
the narrowing and closure of the upper airway (Figure Periodic limb movements of sleep (PLMS) are stereo-
12.6). The device is highly effective, but limited by toler- typed repetitive movements that occur during sleep. Also
ance and compliance. In one study, only 46 percent of called nocturnal myoclonus (although by definition they
patients had acceptable long-term compliance (51). In are longer than myoclonic movements), they typically
tetraplegics, the use of CPAP may be limited by an inabil- occur in clusters of movements lasting 0.5 to 5 seconds,
ity to manipulate the mask into place, and assistance may with a periodicity of 5 to 90 seconds, and repeat for sev-
be needed. Otherwise, SCI does not limit use of this eral minutes to hours (see Figure 12.7). The movements
device. Other treatment options include oral appliances may occur in one or both lower limbs and typically are
that move either the tongue or mandible forward to open more numerous during the first half of the night. A range
the posterior airspace, or upper airway surgery. There is of movement amplitudes can be seen, from simple dorsi-
currently no information available regarding the efficacy flexion of the foot to a triple flexion response. They are
of the various OSA treatments in the SCI population. most common in stages 1 and 2 sleep and are unusual in
SLEEP DISORDERS IN SPINAL CORD INJURY 175
FIGURE 12.7
A polysomnographic tracing of an
SCI patient with periodic limb
movements of sleep (PLMS). The
top channel of this 2-minute trac-
ing is a central electroencephalo-
gram lead (C3A2). The PLMS can
be seen in the leg electromyogram
channel (LEGS LR), which mon-
itors movements in either leg. Note
the repetitive chain of movements
occurring during NREM sleep.
There is no evidence of sleep-dis-
ordered breathing. (LOC-A2 left
eye lead; ROC-A1 right eye lead;
EMG1 chin electromyogram;
ECG electrocardiogram; THOR
RES chest wall movement; SaO2
oxyhemoglobin saturation.)
176 MEDICAL MANAGEMENT
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37. Cahan, Gothe B, Decker MJ, Arnold JL, Strohl KP. Arterial oxy- man. International classification of sleep disorders: Diagnostic and
gen saturation over time and sleep studies in quadriplegic patients. coding Manual. Rochester, Minnesota: American Sleep Disorders
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38. Bonekat HW, Andersen G, Squires J. Obstructive disordered 55. Montplaisir J, Godbout R, Pelletier G, Warnes H. Restless legs
breathing during sleep in patients with spinal cord injury. Para- syndrome and periodic limb movements during sleep. In: Kryger
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plegia 1995; 33:271273. less legs syndrome and periodic movements during sleep with L-dopa:
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patients over 40 years of age with spinal cord lesions. J Neurol 57. Kavey N, Walters AS, Hening W, Gidro-Frank S. Opiod treatment
Neurosurg Psychiatry 1992; 55:10321036. of periodic movements in sleep in patients witout restless legs.
41. Klefbeck B, Sternhag M, Weinberg J, Levi R, Hultling C, Borg J. Neuropeptides 1988; 11:181184.
Obstructive sleep apneas in relation to severity of spinal cord 58. Yokota T, Hirose K, Tanabe H, Tsukagoshi H. Sleep-related peri-
injury. Spinal Cord 1998; 36:621628. odic leg movements (nocturnal myoclonus) due to spinal cord
42. McEvoy RD, Myketyn I, Sajkov D, Flavell H, Marshall R, Amic lesion. J Neurol Sci 1991; 104:1318.
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rax 1995; 50:612619. ment sleep periodic leg movements in patients with spinal cord
43. Burns SP, Yin KS, Buhrer R, Kapur V. Population-based case-con- injury. Sleep 1994; 17:733738.
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Rehab 2000; In Press. leg movements and of the restless legs syndome during sleep fol-
44. Ayas NT, Epstein LJ, Brown R, Hibbert AM, Garshick E. Baclofen lowing acute physical activity in spinal cord injury subjects. Spinal
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45. Frisbie JH, Brown R. Waist and neck enlargement after quadri- between K complex, periodic leg movements (PLM), and
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13
Cardiovascular
Dysfunction in
Spinal Cord Disorders
ardiovascular function is altered in inverse relationship has been described between the level
effects in patients with SCI (40). Small, frequent meals may Pharmacologic Management
minimize postprandial symptoms (24,37). Patients may
Several drugs to treat OH have been tried (Table 13.4),
have greater functional capacity before a meal than in the
but many of these are only marginally useful
hour following a meal and may be able to adjust their
(4042,4657). The drugs for which there has been the
activities accordingly. If blood pressure is higher later in
most experience in use for SCI-related OH include flu-
the day, physical exertion, such as exercise programs or
drocortisone (4648), ephedrine (49,50), and more
physical therapy, may be better tolerated in the afternoon
recently, midodrine (5356).
rather than the early morning (38). The nocturnal diure-
Fludrocortisone is a potent mineralocorticoid with
sis that sometimes occurs in SCI may lead to inadequate
little glucocorticoid activity. It has been used to manage
blood volume. Elevating the head of the bed (reverse Tren-
the OH related to autonomic dysfunction for more than
delenberg position) may reduce nocturnal diuresis, morn-
40 years (46,47). The pressor action of fludrocortisone is
ing postural hypotension, hypovolemia, and supine hyper-
a result of sodium retention, which occurs over several
tension, although patients may not be able to tolerate more
days (40,46). This delayed action should be clear to the
than a few degrees of head-up tilt during the night (40).
patient as well as provider to avoid expectations of imme-
Rapid changes in position should be avoided, as should
diate benefit. For the same reason, doses should be altered
excessive exertion in hot environments. A review of patient
no faster than on a weekly or biweekly interval. In addi-
medications is mandatory and may need modification. If
tion to enhancing renal sodium conservation, fludrocorti-
vasoactive medications are being administered with meals,
sone increases the sensitivity of arterioles to norepineph-
that may be particularly disabling. Liberalizing salt and
rine (40). Many patients have received benefit with a
water intake may improve blood volume. Abdominal
once-daily regimen. However, it has been suggested that a
binders and compressive stockings can be used in an
twice-daily regimen may be more efficacious, because the
attempt to increase venous pressure and reduce venous
drug has been demonstrated to have a relatively short half-
pooling (43), although donning these may present practi-
life of 2 to 3 hours (40). The usual starting dose is 0.1
cal problems for these patients. The repeated and gradual
mg/day given orally. Doses lower than 0.05 mg daily are
increase in postural challenges, such as with the use of a
rarely of any benefit. The dose can be increased by 0.1 mg
tilt-table, may be useful in the acute stages (25,27). A tilt-
increments at intervals of 1 to 2 weeks. Few patients need
in-space or reclining wheelchair is beneficial for accom-
more than 0.4 mg daily, although there are reports in the
modating to a progressive increase in the sitting angle and
literature of higher doses being used. No significant glu-
also allows reclining in response to symptoms.
cocorticoid effect is seen at these doses, but ACTH sup-
A possible role for functional electrical stimulation
pression, manifested by reduced cortisol level, may occur
(FES) has been suggested in the treatment of OH in SCI,
at higher doses. Fludrocortisone treatment can be associ-
based on studies showing an increase in blood pressure
and a decrease in hypotension-related symptoms (44).
The response appears to be dose dependent and inde-
pendent of the site of stimulation. Further research in this
area is warranted. Biofeedback has also been tried for the TABLE 13.4
management of OH in SCI (45). Pharmacologic Management of
Orthostatic Hypotension
Fludrocortisone*
Sympathomimetic amines
Ephedrine*
Midodrine*
TABLE 13.3
Nonsteroidal anti-inflammatory drugs
Nonpharmacologic Management of Indomethacin
Orthostatic Hypotension in SCI Ibuprofen
Clonidine
Identify and minimize exacerbating factors (Table 13.2) Ergot alkaloids
Increase salt intake Erythropoietin
Compression stockings, abdominal binder Vasopressin analogs
Tilt table, progressive postural challenges Desmopressin
Head-up tilt during sleep Somatostatin analogs
Elevating wheelchair leg rests Somatostatin
Reclining or tilt-in-space wheelchair Octreotide
Functional electrical stimulation
Biofeedback * Drugs most often used in SCI-related OH
182 MEDICAL MANAGEMENT
ated with several side effects (25,27,40). Since fluid reten- midodrine is initiated at a dose of 2.5 mg with breakfast
tion is critical to the beneficial effect, patients often gain and lunch, but the dose is increased at 2.5 mg increments
between 5 to 8 pounds before optimal effect is seen, until a satisfactory response is achieved, to a maximum
although higher weight gain than that should be avoided. of 30 mg a day. Patients are often maintained on a dose
Fludrocortisone should be avoided in patients who are of 5 to 10 mg on arising, a second dose mid-morning, and
unable to tolerate an increased fluid load, such as those a third dose mid-afternoon. The use of midodrine early
with congestive heart failure. An electrolyte imbalance can in the day provides the maximum effect when the patient
also occur with fludrocortisone treatment and periodic most needs it and avoids the side effect of excessive supine
checking of serum electrolytes is advisable. Hypokalemia hypertension at night. Supine hypertension is the poten-
is especially common and may occur in as many as 50 per- tially most serious adverse effect, and it is essential to
cent of patients within 2 weeks. It should be treated with monitor both supine and sitting blood pressure in patients
concomitant potassium supplementation. Hypomagne- maintained on midodrine. Sleeping with the head elevated
semia may occur in 5 percent of patients and usually and taking the last dose at least 4 hours before bedtime
responds secondarily to correction of hypokalemia, though may help minimize supine hypertension. Other side-
some patients may need small doses of magnesium sulfate. effects of midodrine are also predominantly related to its
Headache is another relatively common side effect of flu- 1-adrenoreceptor agonist activity. Stimulation of the
drocortisone, although it may be more of a problem in piloerector muscles results in patients commonly experi-
healthy, young adults than in sick or elderly patients. encing the sensation of gooseflesh, paresthesia of the
Potential drug interactions include patients on warfarin scalp, or pruritis. These symptoms are often mild and may
who may require a larger warfarin dose to maintain pre- even be a welcome sign that the drug is functioning, but
vious protimes after starting fludrocortisone. a few patients find these troublesome enough to discon-
Ephedrine was the first available orally active sym- tinue treatment. Action on the -receptors in the blad-
pathomimetic drug. It has been used for the treatment of der may exacerbate urinary retention.
hypotension for many years (27,50). It acts primarily Other agents have been used to treat OH with vari-
through the release of stored catecholamines and has able success, but there is little published experience of
additional direct action on adrenoreceptors. It is nonse- their use in patients with SCI (40). Nonsteroidal anti-
lective and mimics epinephrine in its spectrum of effects inflammatory drugs such as indomethacin or ibuprofen,
(51). The usual oral dose is 25 to 50 mg repeated every which act through inhibition of prostaglandin-induced
4 hours as needed. Its use is often individualized to max- vasoconstriction, are sometimes used. Clonidine has been
imize efficiencyfor example, administering it 15 to 30 reported to cause a paradoxical beneficial increase in
minutes prior to arising. Side effects include the risk of blood pressure in some patients with OH (57). Ergot alka-
supine hypertension, tremulousness, palpitations, and, loids have been used in the past (46), but have largely been
occasionally, cardiac arrhythmias in susceptible patients replaced by other agents. Relatively recently used phar-
(50,51). The sympathomimetic effects on the bladder macotherapy for the OH related to autonomic dysfunc-
sphincter may increase urinary retention. Insomnia may tion includes recombinant human erythropoietin, the
occur, because it is a CNS stimulant. Tachyphylaxis may vasopressin analog desmopressin (DDAVP), and the
occur with repetitive dosing, especially among patients somatostatin analog octreotide (24,40). The mechanism
taking three or more doses per day over several weeks. of action of recombinant human erythropoietin (epoetin
The stimulant properties of this drug present the possi- ) in OH is not entirely clear, but standing blood pressure
bility of abuse and dependence. increases of about 10 to 20 mm Hg have been observed
Midodrine is an alpha1-adrenoreceptor agonist that with its administration in pilot studies in patients with
has been shown to be effective in randomized controlled OH (40). In addition to the increase in RBC and blood
trials for patients with OH (54,55). It has been used viscosity changes that occur with epoeitin , it may have
recently with some success to treat the OH associated a yet unrecognized effect on the vasculature. Although its
with SCI (56). It is the only drug currently approved by use specifically in SCI-related OH has not been described,
the FDA for treatment of OH. Midodrine directly it may be of value in some instances in view of the asso-
increases blood pressure by arteriolar and venous con- ciated anemia and inadequate endogenous erythropoeitin
striction. It is a prodrug that is metabolized to desglymi- response seen in these patients. One disadvantage to its
dodrine after absorption. It is 93 percent absorbed fol- use is that it must be administered parenterally. The usual
lowing oral administration and its bioavailability is not dose is 25 to 75 U/kg administered intravenously or sub-
affected by food. Its half-life is 30 minutes, and the half- cutaneously three times a week. Hematocrit increase takes
life of its active metabolite, desglymidodrine, is about 3 2 to 6 weeks. Patients with renal failure are prone to side
hours. The use of this drug must be individualized to effects of hypertension and thrombosis, because of the
attain maximum therapeutic benefit, and patient input rapid increase in blood viscosity associated with ery-
is useful in determining dosage scheduling. Typically, thropoeitin treatment. Long- or short-term treatment of
CARDIOVASCULAR DYSFUNCTION IN SPINAL CORD DISORDERS 183
OH with desmopressin minimizes fluid loss (24,40). Sin- 3. If the blood pressure is elevated and the individual
gle intranasal doses at bedtime have been used to prevent is supine, immediately sit the person up.
nocturia and morning postural hypotension. Octreotide 4. Loosen any clothing or constrictive devices.
is administered parenterally and inhibits the release of gut 5. Monitor the blood pressure and pulse frequently.
peptides, thus producing vasodilating and hypotensive 6. Quickly survey the individual for the instigating
effects. Because many of these peptides are released after causes, beginning with the urinary system.
a meal, octreotide may be most effective for managing 7. If an indwelling urinary catheter is not in place,
postprandial hypotension (24,37). catheterize the individual.
8. Prior to inserting the catheter, instill 2 percent lido-
caine jelly (if readily available) into the urethra and
BRADYCARDIA AND CARDIAC ARREST wait several minutes.
9. If the individual has an indwelling urinary catheter,
The imbalance of the autonomic nervous system caused check the system along its entire length for kinks,
by a decrease in sympathetic activity and a relative pre- folds, constrictions, or obstructions and for correct
dominance of parasympathetic activity results in brady- placement of the indwelling catheter. If a problem
cardia (7,58). This is a particular problem with cervical is found, correct it immediately.
injuries and may even be life threatening in the acute 10. If the catheter appears to be blocked, gently irrigate
stage, but usually resolves after the first 2 to 6 weeks after the bladder with a small amount of fluid, such as
injury. The tracheal stimulation that occurs during suc- normal saline at body temperature. Avoid manually
tioning or bronchial toilet can precipitate reflex brady- compressing or tapping on the bladder.
cardia and even cardiac arrest in recently injured patients 11. If the catheter is draining and the blood pressure
by increasing the unopposed vagal stimulation (58,59). remains elevated, proceed with step 16.
Management consists of ensuring adequate oxygenation, 12. If the catheter is not draining and the blood pressure
prompt treatment of factors such as respiratory infections remains elevated, remove and replace the catheter.
that can contribute to hypoxia, and the administration of 13. Prior to replacing the catheter, instill 2 percent lido-
atropine about 10 minutes prior to tracheal suction, if caine jelly (if readily available) into the urethra and
needed (17). Temporary cardiac pacemakers have been wait several minutes.
used in patients with severe recurrent episodes (60). There 14. If the catheter cannot be replaced, consider attempt-
is no evidence of a significant increase in cardiac arrhyth- ing to pass a coude catheter, or consult an urologist.
mias in patients with chronic SCI (61). 15. Monitor the individuals blood pressure during blad-
der drainage.
16. If acute symptoms of autonomic dysreflexia persist,
AUTONOMIC DYSREFLEXIA including a sustained elevated blood pressure, sus-
pect fecal impaction.
SCI at the thoracic level T6 or above (i.e., above the 17. If the elevated blood pressure is at or above 150 mm
major splanchnic outflow) predisposes the patient to Hg systolic, consider pharmacologic management to
autonomic dysreflexia, although occurrence with injuries reduce the systolic blood pressure without causing
as low as T8 is possible (62). The feature of most concern hypotension prior to checking for fecal impaction.
with autonomic dysreflexia is the significant and poten- If the blood pressure remains elevated, but is less
tially life-threatening elevation in blood pressure. The than 150 mm Hg systolic, proceed to step 20.
pathogenesis, clinical features, and management of auto- 18. Use an antihypertensive agent with rapid onset and
nomic dysreflexia are discussed in detail in Chapter 35. short duration while the causes of autonomic dys-
The Consortium for Spinal Cord Medicine has published reflexia are being investigated.
clinical practice guidelines for the acute management of 19. Monitor the individual for symptomatic hypo-
autonomic dysreflexia (62). The summary of these treat- tension.
ment guidelines is as follows1: 20. If fecal impaction is suspected, check the rectum for
stool, using the following procedure: With a gloved
1. Check the individuals blood pressure. hand, instill a topical anesthetic agent such as 2 per-
2 If the blood pressure is not elevated, refer the indi- cent lidocaine jelly generously into the rectum. Wait
vidual to a consultant, if necessary. approximately 5 minutes for sensation in the area to
decrease. Then, with a gloved hand, insert a lubri-
cated finger into the rectum and check for the pres-
1From the Consortium for Spinal Cord Medicine Clinical ence of stool. If present, gently remove, if possible.
Practice Guidelines on Acute Management of Autonomic dys- If autonomic dysreflexia becomes worse, stop the
reflexia. 1997. Paralyzed Veterans of America. manual evacuation. Instill additional topical anes-
184 MEDICAL MANAGEMENT
thetic and recheck the rectum for the presence of by the attainable VO2max is reduced, and exercise intol-
stool after approximately 20 minutes. erance leads to general deconditioning. Neither sympa-
21. Monitor the individuals symptoms and blood pres- thetically mediated vasoconstriction with increased
sure for at least 2 hours after resolution of the auto- venous return nor increased heart rate and myocardial
nomic dysreflexia episode to make sure that it does contractility is available in response to aerobic and anaer-
not recur. obic activities in these patients, although partial tachy-
22. If there is poor response to the treatment specified cardia can occur through a reduction in vagal activity.
above and/or if the cause of the dysreflexia has not Studies have shown that individuals with tetraplegia have
been identified, strongly consider admitting the indi- a significantly lower maximal heart rate compared with
vidual to the hospital to be monitored, to maintain able-bodied controls or those with paraplegia (65). In
pharmacologic control of the blood pressure, and to spite of these limitations, various potentially effective
investigate other causes of the dysreflexia. options for exercise in SCI have been described (6679).
23. Document the episode in the individuals medical Detailed discussion addressing the issue of exercise in SCI
record. This record should include the presenting is included in Chapter 45.
signs and symptoms and their course, treatment
instituted, recordings of blood pressure and pulse,
and response to treatment. The effectiveness of the CORONARY HEART DISEASE
treatment may be evaluated according to the level of
outcome criteria reached: It has been suggested that the prevalence of coronary
The cause of the autonomic dysreflexia episode heart disease (CHD) is increased after SCI (8082),
has been identified. although there are other reports that show no significant
The blood pressure has been restored to normal difference in its prevalence between patients with SCI and
limits for the individual (usually 90 to 110 sys- otherwise comparable untrained able-bodied adults (83).
tolic mm Hg for a tetraplegic person in the sit- The relatively small number of subjects and differences in
ting position). patient populations studied may account for the discrep-
The pulse rate has been restored to normal ancy in results among these studies. In any case, heart dis-
limits. ease is now a major cause of mortality and morbidity in
The individual is comfortable, with no signs or patients with SCI, and with the aging of the population
symptoms of autonomic dysreflexia, of increased with SCI, CHD-related issues are becoming increasingly
intracranial pressure, or of heart failure. important in the care of these patients (8487). Informa-
24. Once the individual with SCI has been stabilized, tion gleaned from the SCI model system data suggests that
review the precipitating causes with the individual, heart disease has become the second leading cause of mor-
members of the individuals family, significant others, tality in patients with spinal cord injury (84,85). In the
and caregivers. This process entails adjusting the most recent 5-year data, among patients with SCI beyond
treatment plan to ensure that future episodes are rec- the first year after injury, heart disease was the reported
ognized and treated to prevent a medical crisis or, ide- cause of death in more than 20 percent (85), and even
ally, are avoided altogether. The process also entails higher figures have been reported in other studies.
discussion of autonomic dysreflexia in the SCI indi- Although CHD is not separated out in this group because
viduals education program, so that he or she will be of lack of autopsy data, it likely comprises the major pro-
able to recognize early onset and obtain help as portion of deaths. Data from other developed countries
quickly as possible. It is recommended that an indi- also report ischemic heart disease as a leading cause of
vidual with an SCI be given a written description of death in people with SCI (86). It is therefore important
treatment for autonomic dysreflexia at the time of for SCI care providers to be aware of the risk factors and
discharge that can be referred to in an emergency. prevention strategies for CHD, and to be familiar with
special issues in the diagnosis and management of CHD
in patients with SCI.
REDUCED CARDIOVASCULAR FITNESS
AND ALTERED EXERCISE CAPACITY
Risk Factors and Prevention Strategies
The available muscle mass under voluntary control, is Risk factors for atherosclerosis and CHD have been
reduced after SCI (63). In addition, reduced sympathetic extensively studied and well described in the general pop-
efferent output in people with high complete SCI results ulation. Long-term epidemiologic studies, such as the
in a loss of the normal mechanisms that compensate for Framingham study, have provided important information
the cardiovascular stresses induced by active physical on the establishment of these factors (88,89). There may
exercise (7,6365). Thus, exercise capacity as measured be an increased prevalence of some of these risk factors
CARDIOVASCULAR DYSFUNCTION IN SPINAL CORD DISORDERS 185
There is excellent evidence that treatment of hypertension Elevated LDL cholesterol level is a proven risk factor for
decreases morbidity and mortality from cardiac disease CHD. There is convincing evidence, including angio-
(88,89,96). In patients with SCI, a low rather than high graphic trials, that significant reduction of LDL choles-
baseline blood pressure is typical (7,27). However there terol reduces the rate of CHD progression (89,107). Peo-
are reports that suggest that there is a significant preva- ple with SCI have levels of LDL cholesterol similar to
those of the general population (80,81). A value of LDL
cholesterol over 160 mg/dl is considered high and a value
below 130 mg/dl is desirable. For secondary prevention
in those with known CHD, an LDL level of less than 100
TABLE 13.6
mg/dl is the target. The dietary approach to primary pre-
Key Prevention Targets for CHD vention of CHD follows the rule of 3s (fat intake 30
percent of calories, with 1/3 each saturated fat, mono-
Smoking cessation
saturated fat, and polyunsaturated fat, and daily choles-
Lipid management
Decrease in LDL cholesterol levels terol intake 300 mg) (96). Fat intake recommendations
Modification of HDL cholesterol and trigylcerides are more stringent in those with known CHD. There are
Blood pressure control excellent concise reviews of drug treatment for lipid dis-
Weight management orders available (168).
Physical activity at least three times a week HDL cholesterol is a strong protective factor, and
with high HDL levels are inversely related to the risk of
186 MEDICAL MANAGEMENT
CHD (96). There is generally accepted evidence that HDL appropriate, as is regular exercise. Depending on the
levels are lower in the population with chronic SCI when level of injury, basal energy requirements should be
compared to the general population (80,81). About 24 to reduced from those calculated for able-bodied individ-
40 percent of people with SCI have been shown to have uals by a factor ranging from 10 percent for those with
HDL cholesterol of less than 35 mg/dl, compared to only low paraplegia to 25 percent for those with high tetraple-
10 percent of the general population (81). The relation- gia (112). Because of decreased lean muscle mass, ideal
ship, if any, to completeness or level of injury is not estab- body weights may be 10 to 20 pounds lower than those
lished. At this time, there is no conclusive evidence that recommended in the general population, depending on
isolated low HDL cholesterol levels require drug treat- the level of SCI (113).
ment, and effective lifestyle modifications are the key for
HDL cholesterol intervention (96). These include smok-
Diabetes, Impaired Glucose Tolerance and
ing cessation, weight reduction for overweight patients,
Hyperinsulinemia
and increased activity for sedentary patients. Although
alcohol intake in moderation may increase HDL choles- The risk for CHD is especially high for individuals with
terol levels, initiation of alcohol for this purpose is not diabetes (97,114). Much of this risk is caused by lipid
warranted, considering the other medical, cognitive, and abnormalities, but factors such as insulin levels and blood
drug-interaction risks of alcohol use in these patients. glucose also appear to have an independent role
(115,116). A recent meta-analysis demonstrated a posi-
tive association between insulin levels and the subsequent
Physical Activity
risk for cardiovascular disease (115). The prevalence of
There is strong evidence that a sedentary lifestyle is an impaired glucose tolerance, insulin resistance, and hyper-
independent risk factor for CHD (96,109). Several pos- insulinemia is reportedly increased in individuals with
sible mechanisms for the beneficial effects of physical chronic SCI (92,93). Suggested determinants of increased
activity have been suggested. These include an antiathero- insulin resistance in SCI include changes in insulin sensi-
genic effect through increased HDL cholesterol and tivity after denervation of skeletal muscle, prolonged inac-
decreased LDL cholesterol and triglycerides, favorable tivity, and increased adiposity. Dietary modifications,
effects on platelet adhesiveness and blood viscosity, physical exercise, and blood glucose control are impor-
increased insulin sensitivity, more effective cardiac use of tant interventions in these patients.
oxygen with conditioning, and reduction of blood pres-
sure. It is now recommended that physical activity be per-
Psychosocial Factors
formed for 30 to 60 minutes 3 times a week or more. Peo-
ple with SCI often lead a sedentary lifestyle as a result of There is evidence that psychosocial factors contribute to
their impaired mobility and have an altered physiologic the risk of CHD (117120). Some of the identified fac-
response to exercise, as mentioned in the prior section tors for which there is convincing evidence include
(63,74). Physical exertion from activities of daily living depression, social isolation, and chronic life stresses. Cer-
and everyday mobility with SCI is not adequate for car- tain personality types were thought to be at greater risk
diovascular fitness. Individuals with SCI should be for CHD but that theory is controversial (119). Epi-
encouraged to use some of the several available exercise demiological studies have demonstrated a graded rela-
options discussed in the Chapter 45 entitled Cardiovas- tionship between the degree of depression and the pre-
cular Fitness in Spinal Cord Injury, including electrically dictability of coronary events (120). The presumed
stimulated exercise, arm endurance exercises, and arm mechanisms involve evidence of impaired platelet func-
resistance exercise. tion in depression and the promotion of atherogenesis
through hormonal changes such as an increased cortisol
level. Social isolation is measured by the lack of family,
Obesity and Excessive Weight
friends, or group activities as a regular part of an indi-
Once the acute phase is over, patients with SCI gener- viduals life. Such social isolation or a low level of emo-
ally have a decreased energy expenditure than normal tional support has been associated with a three-fold risk
(110). Rates for those with chronic tetraplegia are gen- for subsequent cardiac events after myocardial infarction
erally lower than in those with paraplegia. As a result, (118). Although estimates of prevalence vary, there is evi-
excessive weight gain is not uncommon. Patients with dence that depression and social isolation are more com-
chronic quadriplegia have a decreased lean body mass mon in people with SCI than in the general population
and an increased percentage of body fat (110). Excess (121123). Assessment of depressive symptomology and
body fat contributes to insulin resistance and increases the social support system should be a routine part of the
the risk for CHD (111). Because energy needs decrease evaluation of these patients, followed by appropriate
with chronic SCI, a reduction in calories consumed is medical and psychosocial interventions (123).
CARDIOVASCULAR DYSFUNCTION IN SPINAL CORD DISORDERS 187
by using oxygen-sensitive electrodes. This measurement effects. The vulnerability of people with SCI to the
is also useful in determining the adequacy of skin perfu- hypotensive side effects of medications was mentioned
sion for healing at a given amputation site. Values above in the section on OH (27). This may be a significant prob-
40 mmHg are generally adequate, whereas those below lem with medications used to treat problems such as spas-
20 mmHg are not. Imaging modalities such as 2-D real- ticity, pain, or depression in SCI, and may affect the
time ultrasound, computed tomography (CT), and mag- choice of drugs used to treat these conditions. As previ-
netic resonance angiography are being increasingly used ously mentioned, the use of sildenafil to treat SCI-related
instead of invasive angiography. erectile dysfunction has recently increased (144,145).
Most patients can tolerate the drug without significant
problems, but hypotension may necessitate a lower start-
Management
ing dose and caution in upgrading the dosage in some.
Minimizing risk factors such as smoking, diabetes, and The big concern with sildenafil use in SCI is its interac-
hyperlipedemia is a key component of management tion with nitrates. Concomitant use of the two drugs can
(149152). Because CHD is a common comorbidity in cause catastrophic, even fatal, hypotension (144,145).
those with peripheral vascular disease and a frequent Because topical nitroglycerin ointment is often used to
cause of death in these patients, primary and secondary treat autonomic dysreflexia, patients with cervical and
prevention measures for CHD are indicated. Limb revas- thoracic injuries above T6 should be strongly cautioned
cularization for the relief of claudication may not be an against the concurrent use of these medications. Providers
issue because of the lack of this symptom in patients with should inquire about sildenafil use prior to initiating top-
SCI. Arterial reconstruction for occlusive disease may be ical nitrates or using intravenous nitroglycerin for emer-
difficult because of small and atrophic arteries in SCI gency treatment of high blood pressure in dysreflexia.
(155). Consideration of amputation for patients with In the past few years, cisapride was increasingly used
advanced disease should be a team decision that should to manage SCI-associated gastrointestinal motility dys-
include the patient and SCI physician in addition to the function (158). A significant risk of significant cardiac con-
surgeons. The effect of amputation on balance and trans- duction abnormalities is associated with the use of this
fers, weight redistribution with new pressure areas, and medication, including QT prolongation and potentially
skin breakdown of the insensate stump are some factors fatal ventricular arrhythmias (159,160). This prompted a
that should be included in the deliberations (152,156). review by the FDA (161), and the manufacturer withdrew
After amputation, patients should be evaluated by the the drug in July 2000, although the company may continue
rehabilitation team. They often need a new wheelchair to make the drug available to patients who meet specific
and retraining for transfers and wheelchair mobility, in clinical eligibility criteria for a limited-access protocol.
addition to education for stump and skin care.
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State of the Art Reviews 1987; 1:3, 475488. cardiac arrhythmias among cisapride users in the United King-
137. Blocker WP, Merrill JM, Krebs MA, et al. An electrocardio- dom and Canada. Am J Med 1999; 107: 352362.
graphic survey of patients with spinal cord injury. Am Correct 161. Miller JL. FDA, Janssen bolster cardiac risk warnings for cis-
Ther J 1983; 37:101. apride. Am J Health Syst Pharm 2000; 57(5):414.
14 Thromboembolism in
Spinal Cord Disorders
hromboembolism, which includes erature reveals varying incidence figures from a number
lynen et al. similarly reported a 100 percent incidence of injury. This coincides with the peak incidence of venous
DVT when using fibrinogen scanning (8). Other investi- thrombosis in the group studied (18). In addition to raised
gators have also reported on the high incidence of DVT levels of clotting factors, changes in the inhibitors of coag-
when utilizing fibrinogen scanning as the diagnostic cri- ulation and fibrinolysis after acute SCI also increase the
teria, whereas others have recently reported similar higher risk of thrombus development. Decreases in antithrom-
incidences utilizing venography, which many believe to bin III have been found in persons following trauma or
be the gold standard in diagnosing DVT. In summariz- surgery, and a reduction in fibrinolytic activity has also
ing these more recent reports that utilize diagnostic tech- been shown after acute SCI (15,19.) Diminished fibri-
niques that are theoretically more accurate, the incidence nolysis may occur through three mechanisms: a decrease
of DVT in SCI individuals ranges from 23 to 100 per- in the release of tissue plasminogen activator caused by
cent (912). the lack of muscle contraction in the paralyzed limb (20,
Although the incidence of clinically significant 21); inhibition of plasminogen binding to fibrin by high
thromboembolism appears to be generally less than concentrations of fibrinogen (22); and increases in plasma
recently reported incidence rates that utilize more sensi- levels of plasminogen activator-1 (19).
tive diagnostic techniques, it is still imperative that our A number of clinical factors have been suggested to
understanding of those risk factors which predispose this be associated with the development of thromboembolism
patient population to development of DVTs and PEs in persons with SCI. In a review of 2,186 persons with
improve so that effective prophylactic measures may be acute SCI, Ragnarsson et al. found a higher rate of occur-
utilized, given the potentially devastating consequences rence of DVT among those with motor complete injuries,
of this condition. a greater risk for DVT in paraplegics than tetraplegics,
and a higher rate of occurrence in males (23). It was fur-
ther noted that the incidence of PE was not influenced
PATHOPHYSIOLOGY AND RISK FACTORS by degree or level of injury. Age was not a factor in the
development of DVT, but PE was noted to be higher in
In examining the risk for developing thromboembolism persons aged 61 to 75 years.
in any medical condition, it is often helpful to recall Vir-
chows Triad (13), which describes three factors that may
contribute to the development of thrombogenesis. These CLINICAL PRESENTATION AND
three factors are the blood vessel wall, blood flow, and DIAGNOSTIC MODALITIES
blood constituents.
The blood vessel wall, more specifically the venous
Deep Vein Thrombosis
endothelium, may be injured by extrinsic pressure on the
paralyzed extremity in the neurologically impaired In the able-bodied population, the early diagnosis of DVT
patient. In addition, the acute course of a spinal cord is often delayed by the nonspecific nature of the clinical
injured person is frequently complicated by sepsis or other signs and symptoms usually associated with DVT
infections, which results in the creation of an inflamma- (swelling, warmth, discoloration). The fact that many of
tory state. This often results in the production and expo- these signs and symptoms occur in other disorders asso-
sure of the vessel walls to free oxygen radicals or endo- ciated with SCI further complicates diagnosis in these
toxins, which may damage the endothelium and provoke patients. For example, HO in persons with SCI is often
thrombosis. heralded by the development of swelling, erythema and
Altered blood flow results in a venous stasis that pre- increased warmth in the affected extremity. In fact, it is
disposes the individual to the development of thrombus. well recognized that the clinical diagnosis of DVT in the
Contributions to the reduced venous flow include fail- SCI population is low in accuracy and specificity (24). Of
ure of the venous muscle pump in the paralyzed limb, even greater concern are those individuals who were
increased blood viscosity secondary to dehydration, tran- found to have DVT by objective tests, yet presented no
sudation of fluid into the interstitial spaces of the para- specific clinical signs or symptoms, or had very general,
lyzed limb, and hyperfibrinogenemia (14,15). nonspecific signs such as fever (25). This fact emphasizes
Alterations in blood constituents may result in alter- that the clinical suspicion for DVT must be high in this
ations in hemostasis and a transient hypercoagulable state patient population, and underscores the importance of
in persons with SCI (16). Several investigators have objective testing for making the proper diagnosis.
shown that there are increased levels and activity of fac- Impedence plethysmography (IPG) measures the
tor VIII and vonWillebrand factor in acute SCI (17,18). rapid decrease in electrical resistance of a limb when an
It has also been shown that increases of these factor lev- occlusive tourniquet is deflated, after taking into account
els begin soon after injury and reach maximum values the venous capacitance. Occlusion of a proximal vein, for
often two to three times normalabout a week after example by a new DVT, significantly reduces the rate of
THROMBOEMBOLISM IN SPINAL CORD DISORDERS 195
artifacts, and a more reliable degree of contrast enhance- itoring (4245). In addition, LMWHs appear to have
ment. This procedure also has the advantage of requir- fewer serious complications such as bleeding, osteoporo-
ing only about 20 minutes to complete (30). sis, and heparin-induced thrombocytopenia when com-
pared to unfractionated heparin (4648).
After initiation of treatment with either unfraction-
TREATMENT AND MANAGEMENT OF ated or low molecular weight heparin, oral anticoagula-
THROMBOEMBOLISM tion with warfarin follows and should be continued long
term. The purpose of long-term anticoagulant therapy is
Until recently, the standard treatment for established to prevent recurrent thromboembolic disease. It is gen-
venous thromboembolism has been anticoagulation with erally recommended that heparin and warfarin treatment
unfractionated heparin, given by constant intravenous should overlap by 4 to 5 days.
infusion, followed immediately by oral warfarin for a Warfarin is administered at an initial dosage of 5
specified number of months. The objective of this initial to 10 mg/day for the first 2 days. The daily dose is then
treatment is to prevent new thrombus formation and the adjusted according to the International Normalized
extension of existing thrombus. Standard anticoagulation Ratio (INR), which was developed by the World Health
will not prevent embolization from existing thrombus or Organization and has become the accepted standard-
induce dissolution of the thrombus. ization of the prothrombin time (PT) for monitoring
Treatment is generally initiated by the administra- oral anticoagulant therapy. Heparin therapy is discon-
tion of an intravenous bolus of heparin. Various proto- tinued on the fourth or fifth day following initiation of
cols or regimens have been studied and recommended warfarin therapy, provided the INR is in the recom-
for the administration of intravenous heparin in patients mended therapeutic range of 2 to 3 (49). It should be
with venous thromboembolism. Hull et al. suggest an emphasized that individuals metabolize warfarin at dif-
initial intravenous heparin bolus of 5000 U (31), ferent rates, so that selection of the correct dosage of
whereas Raschke et al. recommend an initial bolus dose warfarin must be individualized. Therefore, frequent
based on body weight, 80 IU/kg (32). Both wellrecog- INR determinations are often required early on to estab-
nized regimens then include the constant infusion of lish therapeutic anticoagulation.
intravenous heparin, with the goal of achieving an acti- Once the anticoagulant effect and warfarin dosage
vated partial thromboplastin time (aPTT) of at least 1.5 are stable, the INR should be monitored every 1 to 3
times the mean normal control value. Both protocols weeks at regular intervals. If any factors exist that may
involve periodically obtaining the aPTT and adjusting produce an unpredictable response to warfarin or the pos-
the heparin dose. It is important that an aPTT of at least sibility of an alteration in dosage (e.g., concomitant drug
1.5 times control be achieved within 24 to 72 hours of therapy such as antibiotics), the INR should be monitored
initiating treatment, otherwise effectiveness is reduced more frequently (49).
and the risk of recurrent thrombosis is increased Although the optimal duration of treatment for
(31,33,34). thromboembolism is not entirely clear, it is generally rec-
Recently, an increasing number of low molecular ommended that treatment of a first episode with warfarin
weight heparins (LMWH) have become available and in the general population should be at least 3 months, and
have been studied as an initial anticoagulant treatment possibly as long as 6 months. This regimen probably
for acute thromboembolism. A number of clinical trials holds true for persons with reversible or time-limited risk
have examined the use of subcutaneous unmonitored factors, however, in persons with chronic conditions, such
LMWH compared with continuous intravenous heparin as SCI, the optimal duration is not known (5052).
for the treatment of proximal venous thrombosis and
have shown that LMWH is at least as effective and safe
(less major bleeding and mortality) as unfractionated PREVENTIVE MANAGEMENT OF
heparin (3540). Several studies have also shown the THROMBOEMBOLISM
equivalent effectiveness of LMWH when compared to
unfractionated heparin in the treatment of acute pul- Given the significant risk, morbidity, and mortality of
monary embolism (40,41). thromboembolism in the SCI population, it is clear that
LMWHs differ from unfractionated heparins in a methods to prevent the development of this medical com-
number of advantageous ways: a) increased bioavailabil- plication should be strongly considered and implemented.
ity ( 90 percent after subcutaneous injection); b) pro- Various nonpharmacologic modalities and pharma-
longed half-life and predictable clearance, which enables cologic agents are available and frequently utilized in try-
once or twice daily injection, and c) a predictable ing to prevent the development of DVTs. These are dis-
antithrombotic response based on body weight, which cussed be low in greater detail, including their
permits treatment without the need for laboratory mon- effectiveness in the SCI population.
THROMBOEMBOLISM IN SPINAL CORD DISORDERS 197
Nonpharmacologic Modalities bination with aspirin and dipyridamole (57). Merli et al.
also reported a reduction in DVT in persons with SCI uti-
Early Mobilization lizing a combination therapy consisting of PCS, compres-
Minimizing immobility and initiating out-of-bed activity sion stockings, and low-dose unfractionated heparin (58).
at the earliest possible time is desirable and believed to Although they provide some benefit, it appears that
be beneficial in preventing thromboembolism in the hos- they are not sufficient alone in preventing DVT in the SCI
pitalized patient. It has been shown that early ambulation population. In addition, drawbacks with these devices
of patients following acute myocardial infarction signif- include the need to don and doff the sleeves to allow the
icantly reduced the incidence of DVT (53). patient in the rehabilitation setting to participate in ther-
However, for a number of obvious reasons, rang- apeutic activities. Occasionally, an inability to tolerate
ing from the presence of other associated injuries to res- the sleeves occurs in individuals with extreme sensory
piratory complications to instability of the spine and the hypersensitivity and dysesthetic pain caused by their neu-
neurologic deficits themselves, early mobilization and rologic injuries.
ambulation are frequently not possible or practical in the
acute SCI patient. For these individuals, passive range-of- Vena Cava Filter
motion (ROM) exercises are often initiated by physical
and occupational therapists in the intensive care setting. Placement of vena cava filters is frequently performed in
In addition to preventing joint contractures and skin com- certain high-risk patient populations to prevent the devel-
plications, there have been suggestions that this activity opment of PE; however, it should be emphasized that the
may also contribute to the prevention of DVT. Unfortu- presence of a filter itself does not prevent the development
nately, there are no studies that have examined the effec- of venous thrombosis. In general, vena cava filter place-
tiveness of passive or active exercises in preventing DVTs. ment is usually reserved for those patients in whom anti-
coagulant prophylaxis is contraindicated or in those with
established DVT in whom anticoagulation is contraindi-
Compression Stockings
cated (e.g., active or potential bleeding complications).
Graduated elastic or compression stockings are frequently Although no large-scale studies have examined the use
used in hospitalized persons who are immobilized for pro- of filters specifically in SCI patients, examination of a small
longed periods of time. These commercially available series of SCI individuals and several studies of high-risk
items, which may be calf- or thigh-length, apply firm pres- trauma patients (which have included persons with SCI)
sure to the extremity in a graduated fashion (greater dis- have reported the significant effectiveness of vena cava fil-
tally than proximally) and reduce the venous capacitance ters in reducing the incidence of symptomatic PE (5961).
of the extremity. Compression stockings have been shown The effectiveness of vena cava filters in preventing
to be effective in reducing the incidence of DVT in low- PE must be weighed against any potential adverse effects
risk surgical patients; however, their effectiveness in SCI when considering their use. These effects include cava
patients is not known (54). thrombosis, filter migration, and vena cava perforation.
Filter migration and malposition are usually the result of
poor insertion technique, or anatomic anomalies of the
Pneumatic Compression Sleeves
vena cava or renal veins, and can be avoided by the use
Pneumatic compression sleeves (PCSs) are commercially of preinsertion inferior vena cavagrams and insertion of
available devices that are applied to the lower limbs to the filter under fluoroscopic guidance. After placement of
exert rhythmic, sequential compression of the calves and the filter, a follow-up abdominal film should be per-
thighs. These devices are readily available and frequently formed to confirm filter placement and position.
used in the general hospital population for DVT pro- It bears repeating that the use of vena cava filters
phylaxis, and have been found to be effective in persons does not prevent the development of DVT and its poten-
at moderate risk for DVT, such as persons with malignant tial comorbidities. In fact, it has been suggested that even
disease or those undergoing general surgery (55). Their with the concomitant use of low-dose heparin, the risk
effectiveness may be caused by their better ability to aug- of recurrent DVT is higher in persons with filters than in
ment lower extremity venous return, improve flow veloc- those without filters (62).
ity and volume flow rate, and stimulate fibrinolysis (56).
In the SCI population, PCS have been found in clin-
Pharmacologic Agents
ical studies to be variably effective in preventing DVT.
Green et al. demonstrated a reduction in DVT in persons
Dextran
with new motor-complete SCI with PCS alone compared
to persons in which no prophylaxis was used, and an even Although infrequently used in clinical settings, intra-
greater reduction in persons where PCS were used in com- venous dextran has been shown to be an effective
198 MEDICAL MANAGEMENT
antithrombotic agent in persons undergoing general (68). Currently, there are a number of LMWH prepara-
surgery (63). However, in recent years, the availability of tions commercially available; these have been approved
unfractionated (UFH) and low molecular weight heparins by the U.S. Food and Drug Administration for use in the
has largely eliminated the need for the use of dextran in prophylaxis of DVT in patients undergoing abdominal,
most patient populations. These agents have been shown pelvic, hip, and knee surgical procedures.
to be as effective as dextran, if not more so, in certain Although no LMWH preparations are currently
patient populations (55). The major disadvantages of dex- FDA approved for prophylaxis specifically in the acute
tran use include high cost, the need for intravenous SCI population, there are a number of published studies
administration, and the potential for anaphylactic reac- and reports of the increased effectiveness of LMWH in
tions and renal failure. decreasing the incidence of DVT in this patient group
(6972), and it has been suggested and recommended by
the Consortium for Spinal Cord Medicine as one of the
Warfarin
more effective forms of DVT prophylaxis in persons with
As a means of prophylaxis, the use of warfarin has the acute SCI (67).
advantage of being orally effective. However, it has the
disadvantage of requiring close monitoring of the pro- Duration of Preventive Management
thrombin time or INR, and the increased risk of bleed-
ing complications. The close monitoring of the coagula- The duration of DVT prophylaxis in persons with acute
tion times and need for frequent dose adjustment is SCI has been and remains controversial. Prolonging the
commonly necessary in the acute SCI patient, who may administration of any anticoagulant agent unnecessarily
be receiving other medications that may potentiate or exposes the individual to the increased risk of bleeding
inhibit the anticoagulant effects of warfarin; whose nutri- complications and adds to the cost of health care, yet dis-
tional status may be poor, thus potentially enhancing the continuing any treatment prematurely may expose the
effects of the anticoagulant agent; or, in those undergo- patient to the increased risk of developing a throm-
ing surgery, often will require the temporary withhold- boembolus. It has been recommended that the duration
ing and restarting of warfarin. of prophylaxis be individualized, depending on the risk
When used as a prophylactic agent, warfarin is com- factors, need, medical condition, functional status, and
monly used in smaller dosages with the goal of achieving care and support services of the individual (67). Recom-
minimal prolongation of the prothrombin time (4 seconds mended guidelines for the prevention of thromboem-
above control; INR of 1.52.0). Using this approach, an bolism in persons with SCI have been developed by the
effective prophylactic benefit may be attained while min- Consortium for Spinal Cord Medicine and are shown in
imizing the potential bleeding risks (64). Table 14.1.
TABLE 14.1
Guidelines for the Prevention of Thromboembolism in SCI
Intensity of Prophylaxis
Low Compression hose Compression hose Compression hose
Compression boots Compression boots Compression boots
and and and
Intermediate UH+:5000 U q12h; UH+: Dose adjusted UH+: Dose adjusted to
or LMWH+++ to high normal high normal aPTT++; or
aPTT++; or LMWH+++
LMWH+++
and
High ___ ___ Inferior vena cava filter in
certain situations
Duration of Prophylaxis
Compression boots: Compression boots: Compression boots:
2 weeks; 2 weeks; 2 weeks;
Anticoagulants: Anticoagulants: Anticoagulants:
While in hospital at least 8 weeks 12 weeks or until
for ASIA class D discharge from
and up to 8 weeks rehabilitation
for ASIA class C
* Other risk factors: lower limb fracture, previous thrombosis, cancer, heart failure, obesity, age over 70
+UH Unfractionated heparin
++activated partial thromboplastin time
+++LMWH Low molecular weight heparin, prophylactic dose as recommended by the manufacturers
(Reproduced with permission from Consortium for Spinal Cord Medicine. Prevention of Thromboembolism in Spinal Cord Injury, 2nd
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low molecular weight and standard heparin on calcium loss from prophylaxis in spinal injury. Am J Phys Med Rehabil 1996;
fetal rat calvaria. Blood 1995; 86: 13681373. 75:13.
48. Warkentin TE, Levine MN, Hirsh J, et al. Heparin induced throm- 72. Geerts WH, Jay RM, Code KI, et al. A comparison of low-dose
bocytopenia in patients treated with low molecular weight heparin heparin with low-molecular-weight heparin as prophylaxis against
or unfractionated heparin. N Engl J Med 1995; 332:13301335. venous thromboembolism after major trauma. N Engl J Med
49. Hirsh J, Dalen JE, Anderson DR, et al. Oral anticoagulants: Mech- 1996; 335:701707.
15 Infection and
Spinal Cord Injury
atients with spinal cord injury (SCI) URINARY TRACT INFECTION (UTI)
201
202 MEDICAL MANAGEMENT
of specific urinary symptoms that results from sensory complications, such as during pregnancy or perioperative
deficits constitutes the single most important obstacle in periods.
the diagnosis of UTI in this population. The above-described limitations in using systemic
Another potential problem in making a diagnosis antibiotics for prevention of symptomatic UTI in patients
of UTI in patients with SCI exists because bacteriuria, with SCI have encouraged the examination of the inno-
which constitutes the cornerstone for diagnosing this vative preventive approach of bacterial interference (11).
infection, is ubiquitous in this population. Bacteriuria is This approach is based on the principal that nonpatho-
most frequent in patients who have chronic indwelling genic organisms may inhibit the presence of pathogenic
bladder catheters: cultures of randomly obtained urine organisms in the urinary tract. A preliminary nonran-
samples yielded bacterial growth in about 98 percent of domized, open-label clinical trial indicated that inten-
instances (5). Even patients who undergo intermittent tional colonization of the neurogenic bladder with a non-
bladder catheterization have a 70 percent likelihood rate pathogenic strain of E. coli 83972 reduced the rate of
of being bacteriuric (6). Most cases of bacteriuria in symptomatic infection in patients with SCI who had suf-
patients with SCI represent asymptomatic bladder colo- fered from frequent episodes of UTI (12,13). The efficacy
nization. Although asymptomatic bladder colonization and safety of this approach is currently being investigated
may progress to symptomatic infection, often it does not. in a prospective, randomized clinical trial.
The nonspecificity of the finding of pyuria, like bac- The vast majority of episodes of UTI in patients with
teriuria, can also hamper the diagnosis of UTI. Pyuria SCI are caused by commensal bowel flora, primarily
reflects an inflammation of the uromucosal lining and Gram-negative bacilli and enterococci. The microbiology
may signal the transition from bladder colonization to of organisms residing in the bladder can be affected by
symptomatic UTI. However, pyuria can also be caused by many factors, including patients gender and level of SCI.
a variety of noninfectious conditions, including renal For instance, Klebsiella pneumoniae is a very common
stones, recent urologic procedures, catheter-induced cause of urinary tract infection in hospitalized patients
trauma, and interstitial nephritis. (7,14), although Escherichia coli and Enterococcus
Because of these potential problems in establishing species cause more than two-thirds of cases of UTI in
a diagnosis, there exists no universally accepted diagno- female patients undergoing intermittent bladder catheter-
sis of symptomatic UTI in patients with SCI. A useful def- ization (15). Urine cultures that grow multiple flora pre-
inition of symptomatic UTI in these patients requires the sent a particular therapeutic dilemma in this group. Iso-
presence of significant bacteriuria (105 CFU/ml); pyuria lation of more than one bacterial species in the general
( 104 WBC/ml of uncentrifuged urine or 10 WBC/hpf population is usually considered to indicate contamina-
for spun urine); and fever (100F) plus more than one tion and such a finding in patients with SCI may not be
of the following signs and symptoms: suprapubic or flank disregarded. Almost half of positive urine cultures in
discomfort, bladder spasm, change in voiding habits, patients with SCI yield polymicrobial flora (16), and this
increased spasticity, and worsening dysreflexia, provided finding is particularly prevalent in patients who have
that no other potential etiologies for these clinical mani- chronic indwelling urethral catheters (5). Even in patients
festations are identified (7). who have pyelonephritis in association with polymicro-
Because asymptomatic bacteriuria can progress to bial bacteriuria, growth of only one organism from blood
symptomatic infection, it is theoretically possible that pre- cultures may not negate the contribution of other organ-
vention or eradication of asymptomatic bacteriuria can isms to UTI. Because there is no clinically available
decrease the rate of symptomatic UTI (7). However, stud- method to accurately discern which isolated organisms
ies that addressed this issue in patients with SCI have are pathogenic and which do not contribute to clinical
yielded conflicting results (7,8). This is the case in the infection, it is not unreasonable to treat all organisms
study of prophylactic trimethoprim-sulfamethoxazole, grown from urine cultures.
which has been shown in some studies (9) but not others Another dilemma centers around the value of moni-
(10) to significantly reduce the overall rate of asympto- toring pyuria levels after completion of antibiotic therapy
matic bacteriuria and symptomatic UTI. The use of for UTI. Although pyuria generally regresses to normal val-
trimethoprim-sulfamethoxazole for prevention is associ- ues after completion of successful treatment of UTI in
ated with multiple adverse effects (9) and antibiotic resis- patients with SCI undergoing intermittent bladder
tance (10). Therefore, chronic use of systemic antimicro- catheterization, above-normal levels of pyuria may per-
bial drugs is usually discouraged for the prevention of sist post-therapy in patients with indwelling bladder
symptomatic UTI in patients with SCI. Exceptions include catheters (17). Monitoring of pyuria levels after establish-
patients with struvite urinary stones associated with urea- ing a clinical response is generally not warranted. Because
splitting organisms, such as Proteus mirabilis and Provi- the urinary tract will usually become colonized with other
dentia stewartii, as well as instances in which asympto- organisms during treatment for a specific urinary
matic bacteriuria might be associated with significant pathogen, follow-up urine cultures should not be routinely
INFECTION AND SPINAL CORD INJURY 203
obtained after observing adequate clinical response to and the history of ulcer treatment commonly involves
antibiotic therapy. In patients with persistent or recurrent multiple treatments by multiple previous providers.
UTI, the urinary tract should be investigated for anatomic The diagnostic dilemma caused by the inadequate or
abnormalities (abscess, stone, excessive sediment, obstruc- incomplete history provided by SCI patients can be height-
tion, stricture) and functional alterations (vesicoureteral ened by the presence of neurogenic or referred pain that
reflux, high residual volume of urine in bladder). may have no relation to the infection. Physical findings,
The duration of therapy for symptomatic UTI is usu- including fever and local inflammatory changes (purulent
ally based on the localization of the site of infection. The drainage, erythema, swelling, and warmth) are usually
laboratory techniques that help distinguish between upper relied upon to diagnose the infection of pressure sores.
and lower UTI entail evaluation of urine samples obtained Because of the universal colonization of pressure
via ureteral catheterization, sequential analysis of urine sores by bacteria, swab cultures of open ulcers should not
specimens after irrigation of the bladder (bladder washout), be obtained unless infection is clinically evident. Cultures
and examination for antibody coating of urinary bacteria. of the sinus tract are also generally unreliable. Needle aspi-
These techniques are cumbersome and expensive, and are ration is sometimes attempted, but cultures of obtained
rarely employed in the clinical setting (18,19). Most health- material tend to overestimate the number of bacterial iso-
care providers localize the site of urinary tract infection on lates (22). Biopsy of deep soft tissue is probably the most
the basis of clinical findings. For example, the presence of reliable means for determining the cause of infection.
high fever, chills, systemic toxicity, and/or leukocyte casts Although cellulitis adjacent to a pressure sore can theo-
in urinary sediment generally points to infection of the retically be caused by organisms present in the pressure
upper urinary tract. Pyelonephritis is particularly likely to sore, a skin biopsy in able-bodied patients usually yields
occur in patients with vesicoureteral reflux and is usually mostly skin organisms including staphylococci and strep-
treated with a 2-week course of antibiotics. A longer dura- tococci, and clinical response is usually achieved by treat-
tion of antibiotic therapy (4 to 6 weeks) is advocated in ing only these skin organisms (23). Although the study of
patients with persistent infection, documented relapse of cellulitis adjacent to pressure ulcers in patients with SCI
infection, or prostatitis. Although studies in otherwise has not been reported, it is likely to yield similar results.
healthy individuals indicate that short courses of oral Another diagnostic problem in patients with SCI
antibiotics (one large dose or a 3-day course) are efficacious centers around the ability to delineate the extent and
in eradicating uncomplicated lower UTI (20), these results depth of infection in association with pressure sores. Even
may not be extrapolated to the SCI population. Although in patients with apparently healed pressure sores, deep
there have been no prospective, randomized clinical trials soft tissue abscesses may exist. In these instances, the only
that examined different durations of antibiotic therapy, clinical manifestations could be fever or bacteremia (21).
catheter-related infections of the neurogenic bladder in Although gallium scan is usually highly sensitive for
patients with SCI are generally treated with a 7- to 10-day detecting soft tissue abscesses, this nuclear medicine test
course of antibiotics. can yield false positive results in SCI patients who have
an infected pressure sore without an associated abscess
(24). Soft tissue abscesses in association with an infected
INFECTIONS OF SOFT TISSUE AND pressure sore are more accurately diagnosed by computed
UNDERLYING BONE tomography (CT) (24). Because pressure necrosis affects
subcutaneous and muscular tissues more so than skin, the
Factors that contribute to skin and soft tissue infection visualized opening of a sinus tract onto the skin may seem
in the vicinity of pressure ulcers include breaks in skin deceptively small. Probing of the sinus tract, although
integrity and bacterial contamination caused by the soil- generally helpful, may still not reveal the full depth of
ing of the ulcer by stools or urine. The former factor pre- the sinus tract. Sinography constitutes the gold-standard
disposes to infection by skin organisms including staphy- technique for delineating the full depth of the sinus tract
lococci and streptococci, whereas the latter factor and any potential communication with bone, joint, vis-
predisposes to infection by gram-negative bacilli and ceral organs, or deep-seated abscesses. In patients with
anaerobic bacteria (21). nonhealing moist pressure sores, injection of dye into the
The majority of pressure sores in patients with SCI intestines or bladder may help establish the presence of
develop in areas adjacent to the ischium, sacrum, and fistulous communications.
greater trochanter. These patients usually have absent or Diagnosis of bone infection beneath pressure ulcers,
altered sensation in the area of the pressure ulcer and can- the most common form of osteomyelitis in SCI patients,
not directly visualize the affected body area. Because of remains very problematic. In patients with deep non-
these factors, the history provided by patients is usually healing pressure sores, clinical evaluation poorly predicts
incomplete and infection is advanced at the time of pre- the existence of underlying osteomyelitis. Neither clinical
sentation. Many pressure ulcers are chronic or recurrent, evaluation (duration of ulcer, bone exposure, purulent
204 MEDICAL MANAGEMENT
drainage, fever, peripheral WBC count and erythrocyte beneath the other pressure sores. Furthermore, even if
sedimentation rate) nor radiologic examination (plain histopathologic findings from various sites are similar,
roentgenogram and technetium bone scan) correlates well bone cultures may grow different organisms.
with the likelihood of finding histopathologic evidence The optimal management of infected pressure sores
for bone infection (2527). Bone scan, a commonly used usually requires a combination of antibiotic therapy and
test that yields often confusing results, is very sensitive surgery. A lack of response to seemingly adequate treat-
(almost 100 percent) but poorly specific (33 percent) ment may be due to inadequate antibiotic therapy, unrec-
for diagnosing osteomyelitis beneath pressure sores (25). ognized soft tissue abscess, undiagnosed osteomyelitis
This low specificity is caused by the tendency of tech- beneath the pressure sore, or fistulous communication
netium molecules to concentrate in areas of bone that are with the urinary or gastrointestinal tract. Although the
affected by pressure-induced changes and in foci of het- penetration of antibiotics into paralyzed soft tissue was
erotopic bone ossification. Therefore, bone scan should demonstrated to be adequate in a rat model (29), few data
be used primarily for its high negative predictive value (to exist on the levels of antibiotics achieved in bone beneath
rule out osteomyelitis and obviate the need for perform- pressure sores in patients with SCI. Often, organisms that
ing bone biopsy) rather than its low positive predictive were not discovered in initial cultures appear in the pres-
value (to diagnose osteomyelitis). sure sore after initiating antibiotic therapy. In patients
Definitive diagnosis of osteomyelitis beneath pres- with established clinical response, this phenomenon usu-
sure sores requires the histopathologic examination of ally represents colonization of the pressure sore by new
bone tissue (25,27). Although the failure of pressure sores organisms, and these organisms can be ignored. A 2-week
to heal can result from underlying osteomyelitis (21,26), course of antibiotics is usually adequate for treatment of
nonhealing of pressure sores is more likely caused by non- infected soft tissue in patients without underlying
infectious conditions such as pressure-related changes, osteomyelitis. Although the ideal duration and route of
malnutrition, heterotopic bone ossification, and spastic- antibiotic therapy for osteomyelitis beneath pressure sores
ity. This may help to explain why percutaneous needle has not been demonstrated, most patients receive 4 to 6
bone biopsy yields histopathologic evidence for infection weeks of intravenous antibiotics; in cases of chronic
of bone beneath nonhealing pressure sores in only about osteomyelitis, this is often followed by few months of oral
one-fifth to one-third of cases (2527). Because osteomye- antibiotic therapy. If all infected bone is surgically
litis is likely to be a focal process, percutaneous bone removed, a shorter course of antibiotics may be adequate.
biopsy may fail to sample the truly infected focus. There- Surgical debridement of necrotic tissue and the
fore, intraoperative bone biopsy may be more sensitive drainage of collections of pus are essential for establish-
than percutaneous bone biopsy in diagnosing osteomye- ing a cure of the infection. Musculocutaneous flap surgery
litis beneath pressure sores (2628). is superior to debridement alone because the transposi-
Not only is it difficult to determine whether bone tion of a well-vascularized muscle allows for more exten-
beneath a pressure sore is infected, but it may also be dif- sive removal of devitalized tissue, provides better vascu-
ficult to discern which organisms are responsible for bone lar supply to facilitate healing, and enhances host defense
infection. Swab cultures of overlying ulcers usually do not against infection. Extreme care should be provided to pre-
predict the organisms causing osteomyelitis (26). Because vent postoperative flap wound infections that can impair
fibrotic tissue adherent to bone is usually colonized with the vitality of the musculcutaneous flap and require fur-
bacteria, bone cultures are positive in at least two-thirds ther surgical intervention (30). In afebrile, clinically sta-
of patients in whom histopathologic examination of bone ble patients, the initiation of IV antibiotics should take
tissue is incompatible with osteomyelitis (26). Therefore, place in consideration of the timing of planned surgical
in patients with histopathologic evidence of osteomyelitis, procedures. Potent antibiotics that are dispensed several
it may be reasonable to treat all organisms that grow from weeks prior to surgical intervention will change wound
bone cultures, except those that are usual colonizers such flora, making surgical wound management even more
as Staphylococcus epidermidis. Most cases of osteomye- difficult because of the appearance of yeast and antibi-
litis beneath pressure sores are caused by two or more otic-resistant bacteria. In cases of recurrent infection
bacterial species, including gram-positive cocci (particu- or very extensive disease, extremity amputation may be
larly S. aureus and Streptococcus species), gram-negative considered (31).
bacilli (including Pseudomonas aeruginosa and organisms Antibiotic therapy for infections of soft tissue and
belonging to the Enterobacteriaceae group), and anaer- underlying bone, like other types of infection in SCI
obic bacteria (mainly Bacteroides species) (26). patients, poses particular pharmacokinetic challenges that
Diagnostic limitations are particularly prominent in involve the estimation of renal function and antibiotic dis-
patients with multiple pressure sores. In these patients, tribution. The application of various equations that have
the histopathologic evaluation of a bone specimen from been established in able-bodied patients to predict crea-
one site may not accurately reflect the status of bone tinine clearance from serum creatinine has yielded pre-
INFECTION AND SPINAL CORD INJURY 205
scription methods that overestimate creatinine clearance fever, tachypnea, tachycardia, etc.) and abnormal test
in patients with SCI (32). This prompted the evaluation results (leukocytosis, hypoxemia, and infiltrates on chest
of simpler and SCI-specific methods to properly admin- radiographs).
ister vancomycin and other antibiotics in this population.
The changes in gross body composition that occur after
Problematic Diagnosis
SCI can alter the disposition of antibiotics that are pri-
marily distributed within the extracellular fluid, such as A number of noninfectious pulmonary complications,
vancomycin (33) and aminoglycosides (34). As extracel- including atelectasis, pulmonary embolism, fat embolism,
lular water replaces eroded skeletal muscle mass in and chemical pneumonitis can be clinically confused with
patients with SCI, the extracellular volume expands, thus pneumonia. For instance, atelectasis, like pneumonia,
leading to a larger weight-adjusted volume of distribution commonly occurs in patients with cervical or high tho-
for aminoglycosides (34). Therefore, patients with SCI racic SCI who retain pulmonary secretions. Low-grade
may require larger weight-adjusted loading and mainte- fever can be caused by either pneumonia or atelectasis.
nance doses to achieve similar antibiotic concentrations Futhermore, the site of pulmonary involvement may not
than able-bodied counterparts. help differentiate atelectasis from pneumonia, because
both conditions predominantly affect the left lung.
Because of ineffective cough, patients with cervical or
RESPIRATORY TRACT INFECTIONS high thoracic lesions may not be able to provide adequate
sputum samples. If tracheal secretions cannot be ade-
Although pneumonia is much less common than UTI in quately suctioned, bronchoscopy may be required both
SCI patients, the high mortality associated with pneumo- for diagnostic and therapeutic purposes.
nia makes it the leading cause of death by infection in this Pulmonary embolism (PE) can also be clinically con-
population (2). Pneumonia is the most common pul- fused with pneumonia (36). This is particularly true
monary complication in the immediate post-injury period because the majority of SCI patients disclose no throm-
(35). It is particularly likely to occur in the first few months botic source for PE (38). Equally problematic is the obser-
after cervical or high thoracic injury and among quadri- vation that SCI patients commonly have baseline
plegics and persons older than 55 years (2). In patients with roentgenographic abnormalities in the lungs as a result of
cervical or high thoracic SCI, weakness of the diaphrag- atelectasis or other causes; this makes it difficult to inter-
matic and intercostal muscles impairs the capacity to clear pret any defects observed by ventilation-perfusion lung
respiratory secretions, thereby predisposing to pneumo- scanning. Often, lateral chest radiographs are not per-
nia. The presence of an endotracheal or tracheostomy tube formed, making X-ray diagnosis of LLL pneumonia loss
predisposes both to pneumonia and tracheitis, commonly specific. A definitive diagnosis of PE in these cases may
caused by S. aureus and Pseudomonas aeruginosa. require a pulmonary angiogram.
Aspiration pneumonia is likely to occur in SCI Fat embolism to the lungs may also mimic pneu-
patients who have an abnormal state of consciousness monia. It can occur acutely after SCI in association with
caused by associated head injury or illicit drug ingestion fractures of the long bones. The findings of petechiae and
(36). Additionally, paralytic ileus often occurs soon after cerebral dysfunction may help differentiate fat embolism
SCI and predisposes to the aspiration of gastric contents. from pneumonia.
Aspiration pneumonia is usually caused by gram-negative Chemical pneumonitis can also mimic bacterial
and anaerobic bacteria. In patients without respiratory pneumonia. The distinction between these two entities is
tubes or aspiration, community-acquired respiratory tract particularly problematic in patients who aspirate gastric
infections are commonly caused by Streptococcus pneu- contents secondary to paralytic ileus and ineffective cough
moniae, Haemophilus influenzae, and Branhamella reflex. Microbiologic examination of adequate samples
catarrhalis. of respiratory secretions may help distinguish between
these two clinical entities by showing a plethora of organ-
isms (along with WBCs) in samples obtained from
Unusual Clinical Manifestations
patients with bacterial pneumonia.
Patients with cervical or thoracic SCI can have absent or The use of systemic antibiotics for the prevention
altered sensations of chest pain and dyspnea. Because of of pneumonia in high-risk SCI patients is not advocated.
weakness in the diaphragmatic and intercostal muscles, For hospitalized SCI patients, aggressive respiratory care
cough may also be absent or minimal. Because of unop- and pulmonary toilet should be provided. For those with
posed vagal tone, mucous hypersecretion is common, tracheal tubes, suctioning with curved-tip catheters offers
especially in the first few weeks after injury (37). In such the best chance at clearing secretions from the left lung
patients, the only clinical manifestations of pneumonia (39). Because pneumonia can either occur more fre-
may consist of physical findings (distressed appearance, quently or result in more serious complications in patients
206 MEDICAL MANAGEMENT
with SCI than in the general population, eligible SCI 10. Sandock DS, Gothe BG, Bodner DR. Trimethoprim-sul-
patients should be immunized against potentially pre- famethoxazole prophylaxis against urinary tract infection in the
chronic spinal cord injury patient. Paraplegia 1995; 33:156160.
ventable causes of pneumonia. Almost two-thirds of 11. Darouiche RO, Hull RA. Bacterial interference for prevention of
patients with SCI are eligible for vaccination against S. urinary tract infection: an overview. J Spinal Cord Med 2000;
pneumoniae and influenza virus by virtue of old age, 23:136141.
chronic respiratory disease, and/or residence in chronic- 12. Hull RA, Rudy DC, Donovan WH, Wieser IE, Stewart C,
Darouiche RO. Virulence properties of Escherichia coli 83972, a
care facilities. The antibody response to the pneumococ-
prototype strain associated with asymptomatic bacteriuria. Infect
cal (40) and influenza vaccination of SCI patients appears Immun 1999; 67:429432.
adequate. Although there have been no prospective stud- 13. Hull RA, Rudy DC, Donovan WH, Wieser IE, Stewart C, Svan-
ies that examined the clinical benefit of these vaccinations borg C, Darouiche RO. Clinical outcome of intentional bladder
in patients with SCI, it is generally recommended that colonization with E.coli 83972. J Urol 163:872-877.
14. Kil KS, Darouiche RO, Hull RA, Mansouri MD, Musher DM.
patients at risk receive influenza vaccine every year and Identification of a Klebsiella pneumoniae strain associated with
pneumococcal vaccine every 5 years. nosocomial urinary tract infection. J Clin Microbiol 1997;
Because there have been no prospective, randomized 35:23702374.
clinical trials that assessed the optimal duration of ther- 15. Bennett CJ, Young MN, Darrington H. Differences in urinary tract
infection in male and female spinal cord injury patients on inter-
apy and type of antibiotics for the treatment of respira-
mittent catheterization. Paraplegia 1995; 33:6972.
tory tract infections in patients with SCI, management 16. Darouiche RO, Priebe M, Clarridge JE. Limited vs full microbio-
guidelines that have been established in the general pop- logical investigation for the management of symptomatic polymi-
ulation are usually utilized. In general, pneumonia and crobial urinary tract infection in adult spinal cord-injured patients.
tracheitis in SCI patients are usually treated with a 10- Spinal Cord 1997; 35:534539.
17. Joshi A, Darouiche RO. Regression of pyuria during the treatment
to 14-day course of antibiotics (preferably guided by of symptomatic urinary tract infection in patients with spinal cord
antimicrobial susceptibilities). The specific choice of injury. Spinal Cord 1996; 34:742744.
antibiotics can be affected by the condition predisposing 18. Kuhlemeier KV, Lloyd LK, Stover SL. Failure of antibody-coated
to respiratory tract infection. For example, coverage for bacteria and bladder washout tests to localize infection in spinal
anaerobes is required for therapy of aspiration pneumo- cord injury patients. J Urol 1983; 130:729731.
19. Hooton TM, OShaughnessy EJ, Clowers D, et al. Localization
nia, whereas agents effective against methicillin-resistant of urinary tract infection in patients with spinal cord injury. J
S. aureus and multidrug resistant P. aeruginosa may be Infect Dis 1984; 150:8591.
empirically initiated (pending the results of susceptibility 20. Kunin CM. Duration of treatment of urinary tract infections. Am
studies) for treatment of pneumonia or tracheitis in asso- J Med 1981; 71:849854.
21. Sugarman B. Infection and pressure sores. Arch Phys Med Reha-
ciation with respiratory tubes.
bil 1985; 66:177179.
22. Rudensky B, Lipschits M, Isaacsohn M, et al. Infected pressure
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(NIDRR) Consensus Statement. The prevention and management uation of cutaneous cellulitis in adults. Arch Intern Med 1986;
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J Am Paraplegia Soc 1992; 15:194207. 24. Firooznia H, Rafii M, Golimbu C, et al. Computerized tomogra-
2. DeVivo MJ, Kartus PL, Stover SL, et al. Cause of death for patients phy of pelvic osteomyelitis in patients with spinal cord injuries.
with spinal cord injuries. Arch Intern Med 1989; 149:17611766. Clin Orthop 1983; 126131.
3. Sugarman B, Brown D, Musher D. Fever and infection in spinal 25. Sugarman B. Pressure sores and underlying bone infection. Arch
cord injury patients. JAMA 1982; 248:6670. Intern Med 1987; 147:553555.
4. Stover SL, Lloyd LK, Waites KB, et al. Urinary tract infection in 26. Thornhill-Joynes M, Gonzales F, Stewart CA, et al. Osteomyelitis
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29. Darouiche R, Musher D, Hamill R, Ou C, Rognerud C.
Musher D. Progression from asymptomatic to symptomatic uri-
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Am Paraplegia Soc 1993: 16:221226. 30. Garg M, Rubayi S, Montgomerie JZ. Postoperative wound infec-
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33. Griver AR, Prince RA, Darouiche RO. A simple method for 37. Bahskar, KR, Brown R Osullivan DD et al. Bronchial mucus
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16 Thermoregulation
in the SCI Patient
MECHANICS OF TEMPERATURE signals from the preoptic area in the anterior hypothala-
REGULATION IN NORMAL INDIVIDUALS mus, midbrain, and spinal cord. The hypothalamus inte-
grates these signals and mounts the appropriate response.
emperature regulation is a process In addition, endocrine factors may participate in thermal
209
210 MEDICAL MANAGEMENT
These regulatory mechanisms are so effective that, in the FEVER IN SPINAL CORD DISEASE
normal persons body, temperatures can be maintained
for extended periods, even when ambient temperatures Based on these considerations of thermal instability in SCI
range from 13C to 60C. patients, one might anticipate that the presence of fever
Fever is usually mediated through the release of is not necessarily as reliable an indicator of infection as
endogenous pyrogens. These are secreted by a number in the normal population. Theoretically, the febrile
of different cells in the body, but especially by macro- response should be limited in patients who are able to
phages. Endogenous pyrogens include interleukin-1 beta, achieve shivering and vasoconstriction only in those seg-
interleukin-6, tumor necrosis factor (TNF), and inter- ments above their spinal cord interruption. However, clin-
ferons and . The secretion of these endogenous pyro- ical practice indicates that most patients may retain the
gens can occur when the appropriate cells (usually ability to mount a significant fever. One group of inves-
macrophages) interact with various substances including tigators reported sixty of seventy-one patients with acute
microbes, antigen antibody complexes and complement, SCI had fever at some point during their initial hospital-
and certain complement components. Endogenous pyro- ization (15). Other investigators studied seventy-two
gens, in turn, travel to the hypothalamus via the blood patients hospitalized on a SCI service after the initial
stream to change the set point for the body temperature. injury (beginning 4 to 6 weeks after injury) and reported
The brain recognizes endogenous pyrogens in the cir- that most patients with fever were found to have an infec-
cumventricular organs, which lack the bloodbrain bar- tious cause (16). Indeed, only eight of seventy-one
rier. Changing the set point is accomplished in the hypo- prospectively surveyed patients and two of 106 retro-
thalamus through the secretion of a number of spectively surveyed patients had unexplained fever. These
substances, particularly prostaglandins. The final effec- findings were corroborated by other researchers (17).
tor pathways for fever, however, travel via neural path- Therefore, despite the abnormal thermoregulatory abili-
ways to the cutaneous vasculature to affect shivering ties of SCI patients, an elevation of temperature (38C
mechanisms. or more) should always necessitate a careful search for a
cause, especially one of an infectious nature. Only when
other potential etiologies of fever are carefully excluded
TEMPERATURE REGULATION IN in SCI patients should thermoregulatory disturbances be
SPINAL CORD DISEASE assigned as the cause of fever (1821).
Most febrile episodes caused by thermoregulatory
Because neural pathways are important in the modula- disturbances are usually self-limited and resolve sponta-
tion of fever, the question of temperature regulation in the neously within hours to days (16). However, a unique syn-
patient with spinal cord injury (SCI) is a relevant one and drome, called quadriplegia fever, can occur in recently
the subject of a number of prior studies (513). Patients injured tetraplegic patients. This fever has no identifiable
with complete SCI above T6 usually have difficulty in focus of infection and lasts weeks to months (22). This
maintaining normal body temperature and exhibit par- syndrome is particularly problematic because it may incite
tial poikilothermia, with lower core temperatures in cold repeated evaluations for infection and several courses of
environments and higher core temperatures in warm envi- unnecessary antibiotics.
ronments. This inability to maintain a constant core tem- Rarely, fever may occur in the context of autonomic
perature is caused by the lack of effective afferent path- dysreflexia, a paroxysmal syndrome characterized mainly
ways from skin receptors and inability to regulate by hypertension, sweating, facial flushing, and headache
vasoconstriction, vasodilation, and sweating in the insen- (23). This syndrome is seen primarily in patients with SCI
sate portion of the body. Some evidence indicates patients above T6 and is usually triggered by distention of viscerae
with SCI may be able to alter the thermal set point depen- (rectum or bladder), cutaneous stimulation (ingrown toe-
dent on the ambient temperature (5). Thermoregulatory nails), or even infection. Bradycardia is a finding that is
vasomotor tone may, in part, be adjusted through resid- too inconsistent to be reliable; it occurs mostly in text-
ual spinal cord reflexes, and some investigators have books.
described sweating in persons with quadriplegia (14). Many causes of fever in patients with SCI include
Clearly, wider swings in body temperature may occur in both infectious and noninfectious agents. More detailed
patients with SCI and, thus, these patients should avoid information on the causes of fever in these patients may
extremes in ambient temperature. Because tetraplegics are be found in other chapters in this textbook. Up to 47 per-
particularly liable to environmental poikilothermia, it is cent of SCI patients have concurrent head trauma, there-
important to have an adjustable environmental temper- fore many in the quad fever group may have fever
ature for these patients. caused by traumatic brain injury (TBI) (24).
THERMOREGULATION IN THE SCI PATIENT 211
hrough thousands of years of This response is carried out through the interaction of
TABLE 17.1
Differentiation of hematopoietic stem cells: Pleuripotent stem cells differentiate into lymphoid stem cells or
myeloid stem cells. T lymphocytes, natural killer (NK) cells, and B lymphocytes are derived from lymphoid
stem cells. Myeloid stem cells differentiate into lineage-specific precursors or colony forming units (CFUs).
Examples of cell functions are given.
GENERATION 1 2 3 4 5 FUNCTIONS
face markers have been transformed to signal distress. NK of physiologic roles. The complement proteins, albumin,
cells do not require previous exposure to the pathogen fibrinogen, haptoglobin, and other coagulation proteins
or foreign material. These cells form a powerful line of fall into this group and have important clinical implica-
defense against infection, malignancy, and toxic debris, tions in the development and propagation of septic,
recognizing their targets by their disordered expression of thrombotic, and autoimmune responses. The activation
several cell surface receptor subgroups. These cells are of the circulating complement cascade in response to the
capable of differentiating between self and non-self mate- presence of foreign molecules brings about a number of
rial and receive inhibitory signals from host-specific major important responses to infection. The C3B complement
histocompatability complex (MHC) molecules on poten- molecule binds to the cell surface of invading microor-
tial target cells. Infected, distressed, or malignant cells lose ganisms, thus enhancing phagocytosis. Other comple-
their MHC surface molecules, marking them for destruc- ment proteins have the ability to attract neutrophils.
tion by NK cells. Through interaction and cross linkage, complement pro-
The production of a variety of soluble proteins that teins can form a membrane attack complex, perforating
modulate immunologic activity is carried out, mainly in the membrane of targeted cells. Other complement pro-
the liver, in response to circulating cell breakdown prod- teins trigger mast cells to release histamine.
ucts, foreign carbohydrates and proteins, and antibodies Cytokines, a group of soluble molecules produced
in the bloodstream. These molecules, collectively termed by macrophages, lymphocytes, NK cells, and cells infected
acute phase proteins (APPs), modulate response to infec- by viruses, are immunologically active. They convey sig-
tion and inflammation. These proteins play a wide range nals between cells of the immune system. This group
IMMUNE SYSTEM AND INFLAMMATORY RESPONSE IN PERSONS WITH SCI 215
(Table 17.2) includes the interferons, the interleukins, and B2 cells. Initially expressing low-affinity IgM and IgD on
tumor necrosis factors (TNFs). The interaction between their surfaces, these cells switch to the use of high-affin-
cytokines, APPs, hormones, and neurotransmitters is ity IgG, IgA, and IgE receptors after exposure to an anti-
complex and allows for an integrated network aimed at gen. These long-lived lymphocytes are activated to clone
regulating the response to infection and injury. Better themselves and produce an accelerated response, or mem-
understanding of the manner by which these substances ory, to a previously encountered antigen with the help of
govern inflammation and the response to infection has cytokines and complement. Finally, in the secondary lym-
allowed for improved methods of measuring clinical dis- phoid tissues (spleen, lymph nodes, and mucosa), some
ease processes, and for the development of novel thera- B2 cells differentiate into plasma cells, which secrete sol-
pies for a variety of disease conditions, including athero- uble antibodies into the blood stream that bind to
sclerosis, rheumatoid arthritis, multiple sclerosis, and pathogens and facilitate removal by macrophages, neu-
sepsis. trophils, monocytes, and T-cells.
poor indicator of impairment in immune function. In presence of chronic disease, becomes persistent, self-per-
many cases, recurrent infections in this population are petuating, and detrimental.
precipitated by impairment in integument and mucosal The impairments in barrier defense against infection
barrier defenses associated with neurologic injury. Some precipitated by SCI are well recognized. Lung, bladder, and
special circumstances may exist that raise the clinical sus- gastrointestinal mucosal barriers are rendered vulnerable
picion that impairment in immunity may exist and war- by the withdrawal of normal neurologic innervation. In
rant further testing (6). Recurrent fungal infections, the lungs, mucous hypersecretion accompanies acute SCI,
although usually related to quinolone antibiotic use, may probably because of the effects of unopposed vagal inner-
also herald a granulocyte or T-cell defect. Persistent vation on the airway submucosal glands (11). Impaired
sinopulmonary infections are seen in those with impaired bladder emptying and the need for artificial drainage meth-
B-cell immunity. Although many patients with SCI exhibit ods result in chronic inflammation and erosive changes in
chronic urinary colonization with pseudomonas and the bladder mucosa, thus reducing the powerful antimi-
staphylococci bacteria, the presence of a granulocyte crobial effect of mucosal enzymes that usually prevent bac-
defect might be considered in patients with clinical infec- terial adherence to urinary tract structures. Hemorrhoids
tions that are life threatening, infections that persist for and rectal fissures are common after SCI and provide
long periods, or in those with multiple recurrences that another route of invasion for colonic pathogens (12).
dont respond to standard therapy. Multiple changes in skin morphology have been
Forty years ago, clinicians treating persons with SCI documented after SCI. Similarities in skin biopsies
had an intimate awareness of the cumulative, long-term between SCI and scleroderma patients provide evidence
health effects of recurrent infections and inflammation. that these changes may be autoimmune in nature (13).
Secondary renal amyloidosis was a frequent cause of renal Primary neurologic factors are certainly important as
failure and death in this population. The danger associ- well. Pressure ulcers afflict over 60 percent of SCI patients
ated with chronically high circulating levels of the APP in the community setting (8), but are very rare in patients
serum amyloid A have been well described (10). Thank- with amyotrophic lateral sclerosis (ALS), thus implicat-
fully, amyloidosis and renal failure are now rarely seen. ing the absence of normal autonomic and sensory nerve
Over the last three decades, advances in the general med- function in the SCI population (14).
ical care of persons with SCI, most notably in the field The pathogens that have breached the ectodermal
of antibacterial therapy and bladder care, have resulted and endodermal obstacles made vulnerable by SCI are
in longer life expectancies. Despite this, virtually all of the met with two additional levels of defense, the innate and
systemic phenomena known to be associated with chronic acquired immune responses. With the advancement of
inflammation are seen in persons with longstanding SCI laboratory techniques aimed at the study of the immune
(Table 17.3). Although infections are the most notable system and the inflammatory response, researchers have
precursors to an inflammatory response in these patients, been able to delineate the mechanisms of these responses,
other processes (subsequent trauma, burns, stress, stren- and data are available to detail changes that occur after
uous exercise, bone fractures, childbirth, autoimmune dis- SCI (Table 17.4).
ease) may also trigger an acute phase response that, in the Deficits in immune function and the presence of a
chronic inflammatory state have been documented in the
acute and chronic phases of spinal cord injury (1522).
TABLE 17.3
Cytokine responses and autoimmune phenomena may
underlie much of the secondary damage to the spinal cord
Systemic Effects of Chronic Inflammation
that occurs within hours of the initial trauma and pro-
Metabolic changes: Loss of muscle and negative nitro-
vides a fertile field of research for those investigating
gen balance, decreased gluconeogenesis, osteoporosis, strategies for spinal cord injury repair (23,24). Cruise
increased hepatic lipogenesis, increased lipolysis in adi- (25,26) noted an initial decline in NK and T-cell func-
pose tissue, decreased lipoprotein lipase activity in mus- tion shortly after injury. These abnormalities improved
cle and adipose tissue, cachexia towards normal levels as the patients were studied at 6
Hematopoetic changes: Anemia of chronic disease, months post-injury. Whether these changes came about
leukocytosis, thrombocytosis as a result of rehabilitation exercise treatment, as opposed
to a time-dependent recovery after an initial trauma, is
Neuroendocrine changes: Fever; somnolence;
anorexia; increased secretion of corticotropin-releasing
difficult to ascertain.
hormone, corticotropin, and cortisol; increased secretion Other investigations support the presence of a more
of arginine vasopressin; decreased production of insulin- profound suppression of immune function in patients with
like growth factor 1; increased adrenal secretion of tetraplegia and complete spinal cord transection, in con-
cathecholamines; impaired growth; reduced testosterone trast to that found in those with less severe SCI. (2729).
These findings support the theory that alterations in
218 MEDICAL MANAGEMENT
ties is unknown. It is clear that these complex responses 8. Fleisher TA, Tomar RH. Introduction to diagnostic laboratory
immunology. JAMA 1997; 278,22:18231834.
to injury, insult, and infection may vary widely with age, 9. Puck JM. Primary immunodeficiency diseases. JAMA 1997;
gender, and comorbid conditions. The measurement of 278:18351841.
acute phase proteins is a good predictor of the extent and 10. Urieli-Shoval S, Linke RP, Matznery. Expression and function of
serum amyloid A, a major acute phase protein in normal and dis-
severity of inflammation in some diseases (e.g., rheuma- ease states. Current Opinion in Hematology 2000; 7(10: 6469.
toid arthritis) but not in others (e.g., lupus) (6). Power- 11. Bhaskar KR, Brown R, OSullivan DD, et al. Bronchial mucus
ful new therapies have been developed that alter the hypersecretion in acute quadriplegia. Am Rev Respir Dis 1991;
143:630638.
serum levels of soluble mediators and affect changes in 12. Stone JM, Nino-Murcia M, Wolfe UA. Chronic gastrointestinal
immunologic response. Two drugs, etanercept and inflix- problems in spinal cord injury patients: A prospective analysis.
imab, which lower the levels of the pro-inflammatory Am J Gastroenterol 1990; 85: 11141119.
13. Stover SL, Gay RE, Koopman W, Sahgal V, Gale LL. Dermal
cytokine TNF, are now in common use for the treatment fibrosis in spinal cord injury patients: A scleroderma variant?
of rheumatoid arthritis. Another drug, recombinant acti- Arthritis & Rheumatism 1980; 23(11):13121317.
vated protein C, has been shown to reduce proinflam- 14. Watanabe S, Yamada K, Ono S, et al. Skin changes in patients with
amyotrophic lateral sclerosis: Light and electron microscopic
matory cytokine levels and reduce mortality from sepsis observations. J Am Acad Dermatol 1987; 17:10061012.
in humans (40). The judicious clinical use of therapies 15. Segal JL, Brunnemann SR. Circulating levels of soluble interleukin 2
that alter the cytokine and immune response demands receptors are elevated in the sera of humans with spinal cord injury.
J Am Paraplegia Soc 1993; 16:3033.
that the acute phase response to a variety of disease states, 16. Frost FS, Schreiber P, Kushner I. Assessment of chronic inflam-
in a variety of different hosts, be clearly defined. It is con- mation after spinal cord injury using measurements of C-reactive
venient to assume that the laboratory abnormalities seen protein and cytokines. Arch Phys Med Rehabil 2003; In press.
17. Nash MS. Known and plausible modulators of depressed immune
in SCI patients represent a loss of homeostasis that might functions following spinal cord injuries. J Spinal Cord Med 2000;
benefit from these therapies. Experience has shown, how- 23:2,111120.
ever, that many of these laboratory abnormalities docu- 18. Rebhun J, Madorsky JGB, Glovsky M. Proteins of the comple-
ment system and acute phase reactants in sera of patients with
ment a protective, adaptive response to tissue injury or spinal cord injury. Ann Allergy 1991; 66:335338.
invasion. For example, the use of anti-inflammatory med- 19. Cruse JM, Lewis RE, Bishop GR, Lampton JA, Mallory MD,
ication appears to promote immune suppression and to Bryant ML, Keith JC. Adhesion molecules and wound healing in
spinal cord injury. Pathobiology 1996; 64(4):193197.
prolong symptoms of the common cold (17,41). Like 20. Kleisch WF, Cruse JM, Lewis RE, Bishop GR, et al. Restoration
renal failure, AIDS, and rheumatoid arthritis, chronic of depressed immune function in spinal cord injury patients receiv-
human SCI presents another experimental model for the ing rehabilitation therapy. Paraplegia 1996; 34(2):8290.
21. Nash MS. Immune responses to nervous system decentralization
study of the mechanisms and effects of inflammation and and exercise in quadriplegia. Med Sci Sports Exerc 1994;
recurrent infections in long-standing, chronic illness. 26(2):164171.
Patients with SCI are likely to benefit from research into 22. Cruse JM, Lewis RE, Bishop GR, et al. Decreased immune reac-
tivity and neuroendocrine alterations related to chronic stress in
the disease mechanisms and treatment of these other spinal cord injury and stroke patients. Pathobiology 1993;
chronic illnesses. Treatments now used to counteract the 61(34):183192.
inflammatory and immunologic effects (e.g., anemia, 23. Segal JL, Gonzales E, Yousefi S, et al. Circulating levels of IL-
2R, ICAM-1 and IL6 in spinal cord injuries. Arch Phys Med Reha-
nutritional wasting, osteoporosis) of these diseases rep- bil 1997; 78(1):4447.
resent promising therapeutic opportunities for physicians 24. McTigue DM, Popovich PG, Jakeman LB, Stokes BT. Strategies
treating patients with chronic SCI. for spinal cord injury repair. Prog Brain Res 2000; 128:38.
25. Cruse JM, Lewis RE, Bishop GR, et al. Neuroendocrine immune
interactions associated with loss and restoration of immune sys-
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18
Endocrine and Metabolic
Consequences of
Spinal Cord Injuries
pinal cord injury (SCI) has an impact Sympathetic activity inhibits the juxtaglomerular
PITUITARY DISORDERS
FIGURE 18.2
Case Study
A 30-year-old man who had been tetraplegic for 10 years FIGURE 18.3
following a knife wound was evaluated for pressure sores,
chronic osteomyelitis of the hips, secondary amyloido- Hypothalamic-pituitary-adrenal axis. High levels of serum
sis, and renal failure. Because of lethargy, the diagnosis of cortisol suppress ACTH and CRH. With low levels of cortisol,
CRH and ACTH become elevated.
hypothyroidism was considered. Thyroid function test-
ing revealed serum T4 1.5 g/ml (normal 4.5 to 10.5),
RT3U 45 percent (N 25 to 35 percent). Serum TSH was
4 l/ml (normal less than 7 l/ml). Free thyroxine index stimulates ACTH secretion from the pituitary. ACTH in
was normal. turn stimulates cortisol release from the adrenal gland.
Cortisol stimulates gluconeogenosis and is necessary for
the bodys response to stress. Without cortisol, SCI
Discussion
patients having other medical conditions such as sepsis
The combination of low T4 and high RT3U suggests that and myocardial infarction face the likelihood of irre-
the abnormality in thyroid function testing is caused by versible shock.
a decrease in thyroid hormone binding proteins. The nor- Aldosterone, the major mineralocorticoid of the
mal TSH confirms that the patient is euthyroid. The body, stimulates sodium reabsorption and potassium
serum albumin was found to be 2.3 gm/dl (normal 3.5 excretion by the kidney. Aldosterone is primarily under
to 5.5 gm/dl), and 24-hour urinary protein excretion was control of the renin-angiotensin system. Hypovolemia
13 gm. The abnormality in thyroid function tests was and hyperkalemia stimulate renin release by the juta-
caused by nephrotic syndrome secondary to systemic glomerular apparatus of the kidney. Renin secretion is
amyloidosis. inhibited by the sympathetic nervous system. As a result
of disordered sympathetic inhibition after SCI, renin,
angiotensin II, and aldosterone can be elevated in
ADRENOCORTICAL HORMONES tetraplegic patients. Dehydroepiandrosterone, the major
adrenal androgen, can also be elevated.
The major hormones secreted by the adrenal cortex are Cushings syndrome, characterized by increased glu-
cortisol, aldosterone and dehydroepandrosterone (13). cocorticoid levels, can occur with pituitary tumors,
Cortisol is under control of the hypothalamic-pituitary adrenal adenomas, and in the setting of exogenous glu-
axis and is secreted in a negative feedback mode (see Fig- cocorticoid production. Patients with Cushings syndrome
ure 18.3). Stress results in the release of corticotropin present with hypertension, truncal obesity, carbohydrate
releasing hormone (CRH) from the hypothalamus, which intolerance, and peripheral wasting. The obesity that
ENDOCRINE AND METABOLIC CONSEQUENCES OF SPINAL CORD INJURIES 225
occurs in some sedentary SCI patients can mimic Cush- with 25 mg of cortisone acetate, given in the morning,
ings syndrome. The initial screening test for Cushings and 12.5 mg of cortisone acetate, given in the evening,
syndrome is an overnight dexamethasone suppression or 20 mg of hydrocortisone in the morning and 10 mg in
test: one milligram of dexamethasone (a synthetic steroid the evening. Patients with Addisons disease lack aldos-
that is not detected by the cortisol assay) is administered terone as well as cortisol. Mineralcorticoid may be
at 11 PM. At 8 the next morning, a serum cortisol level is replaced with 0.1 mg of flurocortisone per day. This dose
drawn. In normal subjects the morning cortisol is sup- of flurocortisone is often used to treat postural hypoten-
pressed below 5 micrograms per ml. If the morning cor- sion in tetraplegic patients.
tisol level is not suppressed, further testing, including for
24-hour cortisol secretion, is done. In normal subjects,
the 24-hour urinary cortisol secretion is below 100 micro- ADRENAL MEDULLA
grams. In almost all patients with Cushings syndrome,
the 24-hour urine cortisol is elevated. Pheochromocytomas are catecholamine secreting tumors
Patients with Cushings syndrome caused by an of the adrenal cortex (15,16). Patients with pheochro-
adrenal adenoma will have a depressed ACTH level, mocytomas have manifestations of increased cate-
whereas patients with a pituitary cause will have inap- cholamine levels, including hypertension, tachycardia,
propriately elevated ACTH levels. Cushings syndrome, headache, and sweating. Autonomic dysreflexia also pro-
depending on the cause, is treated by the removal of the duces these symptoms, so it is important to distinguish
pituitary tumor or adrenal tumor. the two disorders. Table 18.1 compares the clinical char-
Primary adrenal insufficiency occurs with damage acteristics of autonomic dysreflexia and pheochromocy-
to the adrenal cortex from causes such as autoantibodies toma. Basal catecholamine secretion in pheochromocy-
or infiltrative diseases such tuberculosis, cancer, and amy- tomas is elevated, however serum and urinary
loidosis (which occurs with increased frequency in catecholamine levels in tetraplegics are less than those in
patients with SCI). Secondary adrenal insufficiency is normal subjects. Although serum catecholamine levels
caused by lack of ACTH. Some causes of secondary increase during paroxysms of autonomic dysreflexia, they
adrenal insufficiency are pituitary tumors, radiation of do not exceed the baseline levels found in able-bodied per-
the pituitary gland, and chronic suppression with exoge- sons. If the catecholamine levels are obtained during a
nous glucocorticoids. Secondary adrenal insufficiency period when the paroxysms of autonomic dysreflexia are
may also occur from brain trauma associated with SCI not occurring, they will be normal. If the diagnosis is still
(14). Patients with primary adrenal insufficiency or Addi- uncertain, clonidine can be administered. Clonidine will
sons disease will lack cortisol and aldosterone. As a not decrease the blood pressure or catecholamine levels
result, these patients will be hypotensive, hyperkalemic, in pheochromocytoma, but will in autonomic dysreflexia.
and hyponatremic. It may be difficult to differentiate the Autonomic dysreflexia is common in SCI patients, occur-
orthostatic hypotension of tetraplegia from that of ring in 66 to 85 percent of tetraplegic patients. However,
adrenal insufficiency. Patients with Addisons disease pheochromcytoma is rare, occurring in fewer than 1 per
often are hyperpigmented from increased melanophore 1000 of hypertensive patients.
stimulating hormone levels. The major concern with
adrenal insufficiency is the possibility that these patients
will go into shock when stressed. SCI patients may be DIABETES MELLITUS
treated with glucocorticoids for a variety of conditions,
including COPD. Patients who have been treated with Diabetes mellitus (1,2,17) is defined as a fasting serum
glucocorticoids for 2 weeks or more within the preced- glucose of 126 mg/dl on a random glucose over 200
ing year may not be at risk for adrenal insufficiency. The mg/dl. There are two major forms of diabetes mellitus,
most convenient way of evaluating adrenal function is the Type I and Type II. About 10 percent of diabetics are Type
cortrosyn (synthetic ACTH) test: At 8 AM, a baseline I (insulin dependent, IDDM). Type I diabetics have essen-
serum cortisol is obtained. Then 0.25 milligrams of tially no insulin secretion. Therefore hyperglycemia,
cortrosyn are administered subcutaneously or intra- ketoacidosis, and death will result unless insulin is given.
venously. Serum cortisol levels are obtained at 30, 45, and IDDM is probably an autoimmune disorder. It is theo-
60 minutes. A normal test is a basal serum cortisol above rized that an acute event, such as a viral infection, leads
7 micrograms per ml, which increases to above 18 micro- to the release of antigenic proteins. Certain individuals
grams per ml during the test. If acute adrenal insufficiency (persons with HLA antigens Dw3 and Dw4) are especially
is suspected (e.g., hypotension is present) patients should susceptible. Autoimmune destruction of the islet cells
be treated with intravenous saline and 300 mg of hydro- occurs, eventually resulting in decreased insulin secretion.
cortisone per day, given intravenously in divided doses. Chronic pancreatitis is another cause of IDDM. Repeated
Patients with chronic adrenal insufficiency are treated insults to the pancreas from alcohol and other factors
226 MEDICAL MANAGEMENT
TABLE 18.1
Clinical Characteristics of Autonomic Dysreflexia and Pheochromocytoma
(Modified from Schmitt J, Adler R (1)
AUTONOMIC
DYSREFLEXIA PHEOCHROMOCYTOMA
results in the destruction of insulin secreting cells. Unlike In the treatment of patients with IDDM, patients
autoimmune diabetes, which destroys only the beta cells, require both a basal level of insulin and rapidly acting
diabetes mellitus secondary to pancreatitis also destroys insulin before meals to prevent postprandial hyper-
the alpha cells. Lack of glucagon secretion by the alpha glycemia. There are several ways in which this may be
cells results in a form of diabetes mellitus that is extremely accomplished. Two injections per day of a 2/1 mixture
sensitive to insulin. Patients with this disorder are pre-
disposed to hypoglycemia. Such hypoglycemia is
extremely dangerous in tetraplegic patients, who may lack
the usual adrenergic signs and symptoms of hypoglycemia
(sweating, anxiety and tachycardia).
The most common form of diabetes mellitus is Type
II (non-insulindependent diabetes mellitus, NIDDM).
Type II diabetes mellitus usually occurs in middle-aged,
overweight people. Basal and stimulated insulin levels in
patients with NIDDM are usually elevated or normal,
although the first phase of insulin secretion is diminished.
In spite of this, blood glucose is elevated. These patients
are usually insulin resistant. The cause of insulin resis-
tance is multifactorial. Decreased insulin binding to recep-
tors in insulin-sensitive tissues has been demonstrated in
these patients. In diabetics with fasting serum glucose lev-
els above 160 to 180 mg/ dl, insulin resistance is caused
by a cellular glucose uptake abnormality beyond the level
of the receptor (postreceptor abnormality). Thus, Type
II diabetes mellitus is a disease of insulin resistance and
impaired insulin secretion.
Duckworth et al. (18) found that twenty-three of
forty-one SCI patients had abnormal glucose tolerance
(see Figure 18.4). Even in SCI patients with normal glu- FIGURE 18.4
cose tolerance, serum glucose levels exceeded levels in
Glucose values after 100 gm of oral glucose in glucose-toler-
controls. In these same patients, insulin levels exceeded ant and glucose-intolerant SCI patients versus control sub-
levels in controls (Figure 18.5). Thus, the diabetes melli- jects. *P.05, p.01:p.001. (From Duckworth WL, et
tus of SCI is caused by insulin resistance. Multiple al.) Glucose intolerance due to insulin resistance in patients
characteristics of the SCI patient can predispose to insulin with spinal cord injuries. Diabetes 1980; 24:906910. (Repro-
resistance, including muscle wasting, adiposity, and inac- duced with permission from the American Diabetes Associa-
tivity (2). tion, Inc.)
ENDOCRINE AND METABOLIC CONSEQUENCES OF SPINAL CORD INJURIES 227
FIGURE 18.6
TABLE 18.2
Commonly Used Sulfonylureas for Treatment of Diabetes Mellitus
(Modified from Lebovitz, 22)
trol blood glucose, medication is initiated. Sulfonylureas nephropathy. Diabetics are also at increased risk for the
increase the insulin response to meals and increase insulin macrovascular complications of coronary disease and
sensitivity. strokes.
Biguanides, such as metformin, decrease serum glu- The Diabetes Control and Complications Trial
cose by decreasing gluconeogenesis and glucose absorp- (DCCT) investigated whether intensive diabetic control
tion. They are especially useful in obese, Type II diabetics. could prevent complications (23). One thousand four-
However they are contraindicated in patents who have hundred forty-four Type I diabetics were randomly
liver failure or renal failure (serum creatinine above 1.5), assigned to either intense insulin (3 or more injections
as in these patients, they may precipitate lactic acidosis. per day or an insulin infusion pump) or standard treat-
The starting dose of metformin is 500 mg, taken twice a ment ( one or two shots per day). Hemoglobin AIC lev-
day. This may be increased to as much as 1,000 mg bid. els and serum glucose levels were 1.5 to 2 percent lower
Acarbose is an alpha glucosidase inhibitor that lowers the and 60 to 80 mg/dl lower in those receiving intensive
blood glucose by decreasing glucose absorption. The start- therapy than in those receiving conventional care. There
ing dose is 50 mg po, three times a day. This may be grad- was a significant reduction in retinopathy (76 percent),
ually increased to a maximum dose of 200 mg, 3 times proteinuria (54 percent), and clinical neuropathy (60
daily. Thiazolidines (e.g., rosiglitazole) lower the serum percent) in patients receiving intensive therapy. Although
glucose by decreasing sensitivity to insulin. However, some this study was conducted on Type I diabetics, it seems
thiazolidines (e.g., troglitazone) have been found to cause likely that the conclusions are valid for type II diabetics
liver damage. Commonly, a combination of several agents as well.
is required to control Type II diabetes mellitus. The complications of diabetes mellitus may be dif-
The goals of diabetic control are to achieve a fast- ficult to diagnose in SCI patients. Myocardial ischemia
ing serum glucose below 126, a postprandial glucose may be silent because of the destruction of sensory path-
below 140, and a hemoglobin AIc (an index of long-term ways. Peripheral neuropathy may likewise be silent or dif-
diabetic control) below 7 percent. If oral agents fail to ficult to distinguish from the effects of the cord lesion.
control serum glucose, insulin should be initiated. Adding The only sign that peripheral neuropathy is present may
a nighttime dose of an intermediate-acting insulin, such be a decrease in the magnitude of reflexes in the lower
as NPH, to the oral medications will decrease the fasting extremity. It may be difficult to distinguish the autonomic
blood sugar, thereby reducing the integrated serum glu- neuropathy of diabetes mellitus from the effects of a high
cose during the day. In more severe Type II diabetics, two cord lesion. In situations where the cord lesion is par-
daily shots of insulin may be needed. tial, the effects of autonomic neuropathy add to those of
the cord lesion. Likewise, it may be difficult to distinguish
the effects of diabetic nephropathy from renal failure
Diabetic Control and Complications
from other causes, such as amyloidosis or chronic
Diabetes mellitus results in both microvascular and pyelonephritis. A combination of diabetic nephropathy
macrovascular complications. Microvascular complica- and renal amyloidosis is especially problematic, because
tions include diabetic retinopathy, neuropathy, and both conditions cause proteinuria. Angiotensin-convert-
ENDOCRINE AND METABOLIC CONSEQUENCES OF SPINAL CORD INJURIES 229
HYPOGLYCEMIA
Treatment of Hypoglycemia
Mild hypoglycemia can be treated with oral glucose solu-
tions or juice. In more severe hypoglycemia, or in situa-
tions where oral administration of glucose is not possi-
ble, parenteral therapy must be given. High tetraplegics
may need assistance in the treatment of hypoglycemia.
Glucagon 1mg is available in kits. The powder can be
quickly reconstituted in diluent and administered IV, IM
or SQ. For glucagon to be effective, there must be ade-
quate liver stores of glycogen. The standard parenteral
therapy of hypoglycemia is intravenous glucose. Fifty cc
of 50 percent glucose is administered IV over 5 minutes.
Then an infusion of 5 or 10 percent glucose may be
started. The duration of parenteral glucose treatment
depends on the cause of hypoglycemia. Hypoglycemia
caused by regular insulin and a missed meal may require
only a few hours therapy. If the hypoglycemia is caused
by a long-acting medication, such as chlorpropamide,
especially in an SCI patient with impaired renal function,
parenteral treatment may be required for days.
Wide swings in serum glucose and hypoglycemia
FIGURE 18.8
unawareness can result in impaired mental status and
Plasma glucose and glucagon response (mean SEM) to confusion, placing the paralyzed patient at special risk.
intravenous insulin in five tetraplegic patients (----) and six An insulin infusion pump, which normalizes serum glu-
controls (). (From Palmer JP, et al. Glucagon response to cose (Figure 18.9) delivers a basal amount of insulin to
hypoglycemia in sympathectomized man. J Clin Invest 1976; control glycemia in the fasting state. Before meals, the rate
57:522525. Reproduced by copyright permission of the of infusion in increased. Such pumps improve diabetic
American Society for Clinical Investigation.) control and can prevent wide swings in glucose (32).
REACTIVE HYPOGLYCEMIA
LIPID DISORDERS
Calcium Disorders
In healthy individuals, serum calcium is maintained within
narrow physiologic limits (37,38). The major hormone
involved in calcium homeostasis, parathyroid hormone,
is secreted by the parathyroid glands in response to a
decrease in ionized serum calcium. Vitamin D2 (7-dehy-
drocholesterol) is converted to vitamin D3 in the skin under
the influence of sunlight (Figure 18.11). Vitamin D3 is
hydroxylated to 25 OH D3 in the liver. The best index of
stored vitamin D is the 25-hydroxy vitamin D3 level. Bau-
man et al. (39) found that 25-hydroxy vitamin D3 levels
were decreased in SCI patients and that there was an
inverse correlation between vitamin D levels and PTH lev-
els. This finding of secondary hyperparathyroidism is likely
to result in the development of progressive osteoporosis
in SCI patients. Parathyroid hormone causes osteoclasts to
release calcium from bone and increases renal phosphate
excretion. Parathyroid hormone also stimulates the con-
version of 25-hydroxy vitamin D to 1,25-hydroxy vitamin
D3 in the kidney (see Figure 18.11). 1,25-hydroxy vita-
min D3 causes calcium absorption by the renal tubules.
Patients with SCI have been found to have suppressed PTH
and 1,25 OH D3 levels and increased serum phosphate and
prolactin in the setting of normal Ca++ levels (40). The
degree of PTH suppression correlates with the degree of
neurologic impairment.
Primary hyperparathyroidism is an inappropriate ele-
vation of parathyroid hormone caused by a parathyroid
adenoma, carcinoma, or hyperplasia of the parathyroid
glands. These conditions produce elevated ionized serum
calcium and low serum phosphate levels. A critical element
in the diagnosis of primary hyperparathyroidism is an inap- FIGURE 18.11
propriate elevation of the PTH level. In other forms of Metabolism of vitamin D.
hypercalcemia, the PTH level is suppressed. Primary hyper-
parathyroidism is treated by resection of the adenoma or
subtotal resection of the hyperplastic parathyroid glands.
Secondary hyperaparathyroidism is most commonly caused with renal amyloidosis and nephrotic syndrome, will
by renal failure, low calcium intake, or reduced exposure exhibit decreased serum calcium. In these patients, an ion-
to sunlightcommon scenarios in SCI patients. Impaired ized serum calcium level should be obtained. Hypocal-
renal function results in phosphate retention. Phosphate cemia produces the physical findings of muscular irri-
binds ionized calcium and also decreases the renal pro- tability, such as tetany. The physical findings of
duction of 1,25-hydroxy vitamin D. Secondary hyper- hypocalcemia may be difficult to elicit in SCI patients.
parathyroidism results in the worsening of osteoporosis, Hypercalcemia occurs in the setting of hyper-
which further decreases the low bone density of SCI parathyroidism, hypervitaminosis D, immobilization, and
patients and results in an even greater fracture risk. other causes. Immobilization increases osteoclast activity
Hypoparathyroidism is manifest by low serum cal- and decreases oseoblast activity, thus resulting in hyper-
cium and high serum phosphate levels. The most common calcemia, especially in young people, where the bones are
causes of hypoparathyroidism are thyroid surgery and already in a high turnover state. Signs and symptoms of
autoimmune destruction of the parathyroid glands. hypercalcemia include mental confusion, frequent urina-
Because 50 percent of calcium is bound to protein, patients tion, and thirst. The treatment of hypercalcemia is hydra-
with low serum albumin, such as may occur in SCI patients tion with normal saline, which increases renal calcium
ENDOCRINE AND METABOLIC CONSEQUENCES OF SPINAL CORD INJURIES 233
REPRODUCTIVE FUNCTION IN
FIGURE 18.12 SPINAL CORD INJURY PATIENTS
Correlation between 25(OH)-vitamin D level and parathyroid The pulsatile secretion of gonadotropin releasing hormone
hormone level in spinal cord injury patients. (From Bauman (GnRH) from the hypothalamus stimulates the release of
et al. Vitamin D deficiency in veterans with chronic spinal
luteinizing hormone (LH) and follicle stimulating hormone
cord injury. Metabolism 1995; 44:1612.
(FSH) from the pituitary gland (44,45). In males, LH stim-
ulates the differentiation of the Leydig cells of the testis and
excretion. Various drugs decrease osteoclast activity, promotes the conversion of cholesterol to testosterone and
including calcitonin, mithramycin, and biphosphonates. the secretion of testosterone. Testosterone is largely bound
The use of pamidronate, a biphosphonate, has been advo- to sex hormone binding globulin (SHBG). FSH stimulates
cated for the treatment of immobilization hypercalcemia sperm production by the seminiferous tubules. The semi-
(41). Sixty to ninety milligrams are administered intra- niferous tubules secrete inhibin, a peptide that exists in a
venously. Bisphosphonates cannot be used in children closed-loop feedback with FSH. When seminiferous
because of the risks of precipitating a rachitis syndrome. tubules are damaged, inhibin decreases and FSH increases.
Hypocalcemia is treated by the infusion of intra- In the female, FSH stimulates the growth of ovarian folli-
venous calcium. Ninety-four milligrams (one ampule) of cles, whereas LH stimulates the ovarian production of both
calcium gluconate is administered intravenously, followed androgens and estrogens.
by an infusion of calcium. Six hundred milligrams of cal- Sex hormones in the female are also largely bound
cium gluconate can be added to a liter of D5W (5 per- to circulating plasma proteins. Sex hormones exist in a
cent glucose in water) and administered over 6 to 8 hours. closed-loop feedback with the hypothalamus. Therefore,
In situations in which the serum phosphate is elevated, it in patients with impaired gonadal function, FSH and LH
is important that the serum phosphate be lowered prior increase. Nance et al. (46) studied FSH and LH levels in
to the administration of calcium to prevent precipitation SCI males having varying degrees in injury. Those patients
of calcium phosphate. The administration of glucose stim- having the most severe neurologic deficits had elevated
ulates insulin release. Insulin-mediated glucose uptake gonadotropin levels and an exaggerated response to
into cells lowers serum phosphate. GnRH. It is theorized that trauma and impaired ther-
Hypoparathyroidism may result in chronic hypocal- mogulation (which is most pronounced in the high cord
cemia and hyperphosphatemia. Hypoparathyroidism is lesions) results in damage to the testes.
treated with 1 to 2 grams of elemental calcium per day Semen analysis in SCI patients shows a decreased
and vitamin D. Fifty to one hundred thousand units of number and motility of spermatozoa and abnormal mor-
Vitamin D3 are administered per day. Alternatively, a phology (47). The carnitine level of spermatozoa, which
more active metabolite such as 1,25 (OH) D3 (calcitriol) are an index of epididymal function, is decreased in SCI
may be administered. The standard dose is 0.25 micro- patients. Biopsies of the testes of patients with paraple-
grams per day, which is increased at 4- to 8-week inter- gia and tetraplegia show a lack of spermatogenesis and
vals to 1 microgram per day. The target serum calcium atrophy (Figure 18.13). However, by using electroejac-
in the treatment of hypoparathyroidism is 8 to 9 mg/dl. ultion, viable sperm can be retrieved for artificial insem-
Patients with hypoparathyroidism lack the hypocalciuric ination, and pregnancy may occur.
effect of PTH. Therefore, higher levels of serum calcium Ovarian function is less affected in the female SCI
may result in hypercalciuria and renal stones. patient than is testicular function in the male. The inter-
The high bone turnover state that occurs in the first nal location of the ovaries, compared to the external loca-
several months after spinal injury reduces bone density by tion of the testes, may be protective, because the effects
234 MEDICAL MANAGEMENT
CONCLUSION
FIGURE 18.13
SCI affects the endocrine system, primarily through
Seminiferous tubule atrophy in a tetrapelgic man (400) unpredictable sympathetic nervous system function and
(autopsy specimen). (Courtesy of Dr. B. Kipreos. Department the effects of inactivity. The usual nocturnal increase in
of Pathology, Hunter Holmes McGuire Veterans Affairs Med- ADH secretion does not occur in tetraplegic patients, thus
ical Center, Richmond, Virginia.) resulting in nocturia. Abnormalities in thyroid function
testing and decreased serum T3 occur with increased fre-
quency in SCI. Both hypothyroidism and hyperthy-
of temperature extremes can be reduced. In addition, tes- roidism are more difficult to diagnose in SCI patients than
ticular infections, seeded from the contiguous urinary in ambulatory patients. Type II diabetes mellitus, caused
structures, likely bring about chronic damage to the by insulin resistance, occurs with increased frequency in
reproductive structures. SCI patients. The usual adrenergic signs of hypoglycemia,
Reflex erectile function occurs in as many as 80 per- such as anxiety and sweating, may not be present in these
cent of SCI patients, especially in those with incomplete patients. Autonomic dysreflexia may mimic pheochro-
lesions. Fewer patients experience ejaculation and orgasm. mocytoma. Immobilization hypercalcemia may occur,
With sexual counseling and newer techniques for aug- especially in acutely injured teenagers with tetraplegia.
menting erectile dysfunction, many male patients enjoy a Although some SCI patients may have depressed PTH
satisfactory sex life. Although significant differences have levels, SCI patients are at increased risk for hypovita-
been found between able-bodied women and women with minosis D, which increases osteoporosis risk. Ambula-
disabilities, no significant differences in sexual desire have tion works synergistically with biphosphonates to
been found between the groups. The severity of disability decrease osteoporosis. Hypogonadism, especially in
was not significantly related to level of sexual activity and males, is more common in SCI patients than in ambula-
orgasm in SCI women with intact sacral reflexes (48). tory patients.
he medical care of an individual can The primary care provider for an SCI patient varies.
237
238 MEDICAL MANAGEMENT
PRIMARY CARE OF SCI PATIENTS VS. Sir Ludwig Guttman stated that of the many forms
AMBULATORY PATIENTS of disability which can beset mankind, a severe injury or
disease of the SCI undoubtedly constitutes one of the most
SCI often has a devastating impact on the physical, psy- devastating calamities of human life. SCI patients are
chological and social well being of its victims. SCI commonly depressed, have chronic pain, and are unem-
increases many of the risk factors for disease, and its vic- ployed. Sometimes these problems can be managed by the
tims have a narrower margin of health than the general primary care provider. However, often psychiatrists, psy-
population (4,5). chologists, social workers, and other professionals may
Impaired ventilation and cough in tetraplegics pre- be required. The diagnosis and treatment of the many
disposes these patients to pneumonia, mucus plugging, conditions seen by the primary care provider is described
and ventilatory failure. Patients with complete lesions in other chapters of this book.
above C3 have bilateral diaphragmatic paralysis that One of the primary duties of the primary care
requires artificial ventilation or diaphragmatic pacing. provider is encouragement of behavior that prevents dis-
Injuries to the thoracic cord may result in impaired cough ease. The SCI patient and his provider may falsely assume
and a predisposition to pneumonia in a paraplegic that the patient is already too infirm for preventive med-
patient. icine to make a difference. The most important health
The loss of mobility results in pressure sores, promoting behaviors are proper nutrition, weight con-
osteomyelitis, osteoporosis, and heterotopic ossification. trol, smoking cessation, stress management, physical fit-
Increased reliance on the upper extremities may result in ness, elimination of drug and alcohol abuse, disease and
the upper extremity overuse syndrome (5) especially in injury prevention, development of social support, and
the shoulders and hands. Inactivity predisposes to obe- surveillance (11).
sity, which results in low HDL cholesterol and diabetes
mellitus (6).
Impaired sensation results in a propensity to injury PERIODIC EVALUATION OF THE
from a variety of sources including foreign bodies, SPINAL CORD INJURY PATIENT
trauma, and burns. Silent myocardial ischemia in the
tetraplegic may delay the diagnosis of coronary disease At the time of the annual evaluation, a complete physi-
(7). The first manifestation of coronary disease may be cal examination should be performed, including a dental
congestive heart failure. exam and tonometry to screen for glaucoma. The neuro-
The disruption of the autonomic nervous system is logic examination of SCI is described in Chapter 27.
close to being unique to spinal cord injury. Autonomic
dysreflexia results in paroxysms of hypertension, which
Immunizations
may be life threatening. Autonomic dysreflexia may
mimic conditions ranging from essential hypertension to Primarily because of respiratory compromise and
pheochromocytoma (8). impaired cough, SCI patients are at increased risk for pul-
Impaired micturition results in infection and vesi- monary infections (12). When pneumonia occurs, it is
coureteral reflux, which in turn results in chronic renal more likely to be fatal. Immunization is probably the most
failure (9). Chronic indwelling Foley catheters predispose cost-effective health measure provided for SCI patients.
to bladder stones and malignancy. Impaired sexual per- The standard recommendation for pneumococal vacci-
formance and fertility are common complaints in the SCI nation is that it be given to adults with cardiovascular dis-
population. ease, pulmonary disease, diabetes mellitus, alcoholism,
Prolonged recumbency, obesity, delayed gastric emp- cirrhosis, asplenia, chronic renal failure, HIV, hemato-
tying, and use of medications such as diazepam and anti- logic malignancies, and to high-risk populations such as
cholinergics results in gastroesophageal reflux disease. some native Americans, institutionalized patients, and all
Cholelithiasis occurs more commonly in patients with adults aged 65 or over.
SCI, as may gastrointestinal pseudo-obstruction. SCI patients often fall into one or more of the above
SCI patients are on more medications (10) than categories, and it is the authors opinion that pneumoco-
ambulatory patients, which results in an increased risk for cal vaccination should be performed in the majority of SCI
drug interactions. SCI patients have a relative increase in patients. A second dose of pneumococal vaccine should be
body water, resulting in an increased volume of distribu- considered 6 or more years later for adults at high risk of
tion for water-soluble drugs such as gentamycin. The disease and in patients who lose antibody rapidly
increased volume of distribution means that larger load- (nephrotic syndrome, renal failure, transplant recipients).
ing doses are required, and clearance may be delayed in Influenza vaccination should be given annually to
patients who may already have impaired clearance from patients at high risk for complications such as pneumonia,
chronic renal failure. and to institutionalized patients and healthcare workers.
PRIMARY CARE FOR PERSONS WITH SPINAL CORD INJURY 239
High-risk patients include patients with chronic car- mon in the SCI patient and require vigilant examination.
diopulmonary disorders and patients with chronic diseases, Condom catheters may cause contact dermatitis, ero-
such as diabetes mellitus and chronic renal disease. Hepati- sions, and maceration.
tis B immunization is achieved through 3 doses given over Malignancies such as melanoma and squamous cell
a 6-month period. Hepatitis A immunization should be and basal cell carcinoma should be searched for, especially
administered to high-risk populations such as international in sun-exposed areas.
travelers, intravenous drug users, and homosexuals.
TB skin testing should be performed at least annu-
ally in high-risk patients. These include residents and CARDIOVASCULAR DISORDERS
staffs of long-term care facilities, individuals with chronic
diseases such as renal failure and diabetes mellitus, The risk factors for coronary artery disease including
patients taking steroids or immunosuppressive agents, smoking, male gender over 45, hypertension, diabetes
alcoholics and users of intravenous drugs, and the con- mellitus, low HDL cholesterol, and family history of coro-
tacts of infectious TB cases. nary events should be evaluated (2,3). Diabetes mellitus
and low HDL are more common in SCI patients than in
the general population. A normal blood pressure for an
PULMONARY EVALUATION SCI patient is 140/90 or below. A fasting lipid profile
should be obtained yearly. The target LDL cholesterol is
Routine chest x-rays are not recommended. Only if a 160 mg/dl if one or fewer risk factors is present. With two
patient has symptoms of pneumonia, congestive heart or more risk factors, the target LDL cholesterol is below
failure, or other pulmonary problems, is chest X-ray indi- 130 mg/dl. If the patient has known coronary disease or
cated. Screening chest X-rays for lung cancer in smokers diabetes mellitus, the target LDL cholesterol is 100mg/dl.
have not been shown to significantly increase survival and In view of the potential for multiple risk factors for coro-
are not recommended. Likewise, routine spirometry is not nary disease, the determination of the lipid profile is a key
recommended. In patients with COPD or other chronic part of annual screening. If diet fails, drugs such as HMG-
pulmonary diseases, spirometry should be done to follow COA reductase and inhibitors should be used to achieve
the course of the disease (4). cholesterol goals.
An ECG should be done annually to provide a base-
line. ECG screening is more important in the SCI patient
Dermatological Examination
than in the ambulatory patient because the SCI patient
The pressure sore is the most common cutaneous prob- may have silent myocardial ischemia. If the ECG suggests
lem encountered in the SCI patient. As the longevity of coronary disease, or the patient has symptoms such as
SCI patients has increased, the incidence of pressure ulcers chest pain or dyspnea, a full cardiac evaluation should
has increased (13,14). be done. A chest X-ray may show cardiomegly and pul-
Lindan and co-workers have found that the highest monary congestion secondary to silent infarction. Exer-
pressures in the supine and prone patient are over the sacrum, cise tolerance testing can be done in paraplegics with arm
ischial tuberosities, heels, and knees (15). When the patient crank ergometry. However, nuclear studies such as per-
is lying on one side, pressure over the trochanteric area is santin-thallium scans can be used to assess risk for
higher than that exerted on the sacrum when the patient is ischemia in tetraplegics and paraplegics. Catheterization
supine. Being seated increases pressure in the ischial area. is the gold standard for assessing coronary artery patency.
From these considerations, the skin overlying the
sacrum, ischial tuberosities, trochanters, and heels is most
Case Study
at risk for the development of pressure sores.
Pressure sores are classified as: A 58-year-old C6 tetraplegic was noted to have pedal edema
and bibasilar rales at his periodic evaluation. He denied chest
Stage I. Nonblanchable erythema of intact skin pain but he noted that he was a little more dyspneic than usual.
Stage II. Partial-thickness skin loss resulting in an An ECG showed Q-waves in V1V4 consistent with an old ante-
rior wall myocardial infarction. A chest X-ray showed car-
abrasion or shallow crater
diomegly and pulmonary congestion. A cardiac catheterization
Stage III. Full-thickness skin loss and extension into was performed and showed extensive four-vessel coronary dis-
subcutaneous fat ease and an ejection fraction of 15 percent. Percutaneous
Stage IV. Extension into muscle and bone transcoronary angioplasty (PTCA) was performed. A fasting
lipid profile showed a total cholesterol of 240 mg/dl, with an
Pressure ulcers are prevented by relieving pressure LDL cholesterol of 180 mg, an HDL cholesterol of 20 mg/dl,
by appropriate cushioning and by turning the patient. and a VLDL cholesterol of 40 mg/dl. He was placed on
Skin infections, such as cellulitis and intertrigo, are com- furosemide 40 mg and lisinopril 10 mg to control his congestive
240 MEDICAL MANAGEMENT
heart failure and lovastatin 10 mg/per day to control serum and seen at 3-month intervals. A patient with a blood
lipids. His symptoms improved and his serum cholesterol pressure of 188/110 mmHg may need to be started on
declined to 110 mg/dl. medication immediately and reevaluated in 1 to 2 weeks.
Paraplegics whose sympathetic nervous systems are
largely spared are most likely to be hypertensive. The
Discussion algorithm for treatment of hypertension in these patients
This mans sensory deficit prevented chest pain, thus is therefore similar to that in ambulatory patients (16).
resulting in a silent myocardial infarction with subsequent Before treatment of hypertension is initiated, secondary
congestive heart failure. The high LDL cholesterol and causes, including autonomic dysreflexia, renal artery
low HDL cholesterol put him at high risk for coronary stenosis, chronic renal failure, hyperaldosteronism, and
disease. pheochromocytoma should be ruled out. Reduction of
caffeine and alcohol intake, and cessation of tobacco use
may decrease blood pressure.
HYPERTENSION Treatment of hypertension should be initiated with
restriction of sodium, weight reduction in overweight
Hypertension is usually treated by the primary care prac- individuals, and if possible, exercise. Remember that the
titioner rather than by a subspecialist (14,16). The pres- ideal weight of a paraplegic is 80 percent that of an ambu-
ence of hypertension compounds the damage done by latory patient of the same height, and that of a tetraplegic
other cardiovascular risk factors. Tetraplegics are some- is only 60 percent of his ambulatory counterpart. Arm
what protected from the development of hypertension by exercise can be performed in paraplegic and low
sympathetic underactivity. However, obesity and inactiv- tetraplegic persons. In persons with complete cord lesions
ity result in an increase in the incidence of hypertension above C5, functional electrical stimulation may be used
in paraplegics. Yekutiel and others (17) found that 34 per- to facilitate exercise. Moderate sodium restriction (120
cent of patients with SCI had hypertension, as compared meq/day) for 6 months lowers systolic blood pressure in
to 18.6 percent of ambulatory control subjects. Hyper- ambulatory persons by an average of 8 mmHg and dias-
tension in patients with an intact neuroaxis may be tolic blood pressure by an average of 5 mmHg (18).
defined as a diastolic blood pressure of greater that 85 If several months of nonpharmacologic therapy fail
to 90 mmHg or systolic blood pressure greater than 140 to control blood pressure, medication should be initiated.
mmHg. It seems reasonable to accept this value as the Available data prove that lowering blood pressure with
upper limit of normal blood pressure for a paraplegic sub- diuretics, beta blockers, and calcium channel blockers
ject. The baseline blood pressure in tetraplegic persons is reduces overall mortality and morbidity for strokes and
90 to 110/56 to 70. However, there is no evidence that heart attack (16). Furthermore, diuretics and beta block-
the level of blood pressure requiring treatment in a ers are relatively inexpensive. Therefore, if there is no
tetraplegic should be lower than that in a paraplegic or indication for other agents, these agents should be used
ambulatory person. The goal of treatment in most initially to control hypertension in SCI patients.
patients is to bring the blood pressure below 140/85 to Hydrochlorthiazide or chlorthalidone dosed at 12.5
90 mmHg. However, in certain patients (diabetic patients, to 50 mg per day may be begun.
patients with renal insufficiency, and patients with con- Alternatively, a beta blocker may be started, such
gestive heart failure), the goal blood pressure is 130/85 as metoprolol (50 mg per day; maximum dose 300
mmHg. These conditions often occur in SCI patients. mg/day) or propranolol (40 mg per day maximum dose
Hypertension is divided into stages (16). Patients 480 mg/day). Thiazide diuretics may worsen diabetes
with Stage 1 hypertension have diastolic blood pressure mellitus. SCI patients are at risk for hyponatremia, espe-
of 90 to 99 mmHg. Patients with Stage 2 hypertension cially when placed on thiazide diuretics (19). Beta block-
have a diastolic blood pressure of 100 to 109 mmHg, and ers are contraindicated in asthma and COPD, because
patients with diastolic blood pressures above 109 have they may cause bronchospasm.
Stage 3 hypertension. However, there is increasing evi- Certain types of patients may have compelling indi-
dence that an elevated systolic blood pressure imparts a cations for specific classes of drugs. Diabetics, especially
greater cardiovascular risk than elevated diastolic blood those with proteinuria, require ACE inhibitors for renal
pressure. The pulse pressure, which is the difference protection, which may be even more important in the SCI
between systolic and diastolic blood pressure, seems to be patient whose renal function may be compromised by
a major predictor of risk for conditions such as conges- conditions such as chronic pyelonephritis. Patients with
tive heart failure. Obviously, the more severe the hyper- myocardial infarction require beta blockers. However,
tension, the more aggressive the treatment must be. For beta blockers may exacerbate bradycardia and impair
example, a patient with a blood pressure of 145/89 recognition of and recovery from hypoglycemia in insulin-
mmHg could be initially treated with diet and exercise treated diabetics. Peripheral alpha blockers, such as pra-
PRIMARY CARE FOR PERSONS WITH SPINAL CORD INJURY 241
zosin, have the advantage of reducing urethral sphincter intolerance (8). A fasting serum glucose should be obtained
tone, but may also cause postural hypotension. yearly. Diabetes mellitus is defined by fasting serum glu-
Calcium channel blockers, which were initially cose of 126 mg/dl or higher on two or more occasions.
introduced as antianginal agents, may, in theory, provide
special benefits to SCI patients (16). Dihydropyridine cal-
cium channel blockers are predominantly vasodilators. DIABETES MELLITUS
The first-generation agents, such as nifedipine, have a
modest effect on cardiovascular contractility. Second- In diabetic patients, the hemoglobin A1C level should be
generation agents, such as amlodipine, have essentially no measured at each clinic visit. Ideally, the hemoglobin A1C
effect on myocardial contractility. SCI patients may have should be below 7 mg/dl. Fundoscopic examination by
decreased cardiac output secondary to myocardial an ophthalmologist, optometrist, or retinal photos should
ischemia, which is often silent. Dihydropyridine calcium be performed annually. A complete foot exam should be
channel blockers have the advantage of improving car- performed and referral to a podiatrist made when indi-
diac output while lowering blood pressure. Nondihy- cated. In ambulatory diabetics, foot sensation is evaluated
dropyridine calcium channel blockers, such as diltiazem, with a special filament to detect neuropathy. This is often
decrease myocardial contractility. By impairing cardiac not possible in SCI patients.
contractility and inhibiting atrioventricular conduction, For a detailed analysis of diabetes mellitus in the SCI
they may be cardioprotective. Short-acting calcium chan- patient, please see Chapter 18.
nel blockers (such as nifedipine) increase coronary events
when used to treat hypertension (20). However, long-act-
ing calcium channel blockers (such as amlodipine and ver- DEPRESSION
apamil) do not share these possible dangers.
If there is no response to the medication, or trou- Depression occurs with increased frequency in SCI
blesome side effects occur, another medication should be patients. Depressed people are more likely to develop
substituted. If there is an inadequate response to med- SCI from mechanisms such as suicide attempts, and SCI
ication, but the medication is well tolerated, a second often results in depression. Depressed mood, despon-
agent should be added. If the initial drug is not a diuretic, dency, and grief are common reactions to serious illness.
a diuretic should be added. Diuretics work synergistically Grief includes depressed mood, anger, guilt, anxiety,
with other agents. For example, diuretics decrease plasma hopelessness, and despair. Grief may last two years or
volume, thereby making the patient more sensitive to the more.
effects of ACE inhibitors. The criteria for major affective disorders, as defined
When hypertension is noted in a tetraplegic patient, in the American Psychiatric Association Diagnostic and
autonomic dysreflexia should be considered before a Statistical Manual of Disorders are (22):
commitment is made to chronic pharmacologic treat-
ment. Loss of sympathetic tone results in increased A. Dysphoric mood in all or almost all usual activities
reliance on the renin-angiotensin system to maintain is relatively prominent but not necessarily the most
blood pressure, especially when hypovolemia has been dominant symptom
induced by a diuretic. Angiotensin-converting enzyme B. At least four of the following symptoms have been
inhibitors may therefore induce profound hypotension in present every day for at least two weeks (symptoms
tetraplegic subjects and should be avoided or used with are not due to medical condition):
extreme caution in these patients (22). 1. Poor appetite or hyperphagia
2. Insomnia or hypersomnia
3. Psychomotor agitation or retardation
LABORATORY VALUES 4. Loss of interest in usual activities or decrease in
sexual drive
A complete blood count (CBC) should be ordered to 5. Loss of energy
detect processes such as anemia, infection, and drug- 6. Feelings of worthlessness or inappropriate guilt
related thrombocytopenia. Electrolytes, blood urea nitro- 7. Difficulty thinking or concentrating
gen (BUN), and creatinine should be measured and a fast- 8. Recurrent thoughts of death or suicide
ing lipid profile should be determined. Blood levels of C. Neither of the following dominates the clinical
medications such as digoxin and phenytoin should be picture
determined when indicated, and liver function tests 1. Mood-incongruent delusion or hallucination
should be performed with use of medications such as the 2. Bizarre behavior
statins. D. Not superimposed on schizophrenia, schizophreni-
SCI patients are at increased risk for carbohydrate form disorder, or paranoid disorder
242 MEDICAL MANAGEMENT
in SCI patients than in ambulatory patients, but is the important counseling topic. The health risk of tobacco
most common cancer in older men (29). smoking is greater in SCI patients, especially tetraplegics,
A digital rectal exam should be performed annually than in the general population. Impaired ventilation and
in males over 40. At the time of the digital rectal exam, cough predispose to pneumonia, and the risk is made even
the prostate should be examined for nodularity and size. greater by smoking. Obstructive lung disease super-
The utility of prostate specific antigen (PSA) screen- imposed on the restrictive disease in tetraplegics acceler-
ing for the diagnosis of prostate cancer is uncertain. The ates the progression to ventilator dependence and death.
presence of prosthetic devices or indwelling catheters, Second-hand smoke is probably a greater risk to SCI
which are common in SCI patients, increases the PSA. The patients than to ambulatory patients.
determination of PSA has not been shown to decrease Smoking cessation should be discussed at each visit
mortality from prostate cancer. Many elderly patients that the patient makes. If the patient wishes to quit, nico-
who have subclinical prostate cancer die of something tine patches and gum may be prescribed and referral to
else. The surgical treatment of prostate cancer has a high a smoking cessation clinic made.
incidence of the complications of impotence and incon- The substance abuse history begins with questions
tinence, although these complications may be less of an about nonthreatening subjects. Then the subject moves
issue in SCI patients. The patient should be counseled to questions about legal and illegal substances, such as
about prostate cancer yearly at age 50 and PSA testing the number of drinks taken per day. There are several
offered. An elevated PSA and/or a nodule on digital rec- questionnaires available to aid in this process. The
tal exam must prompt a GU referral. briefest of the instruments is the CAGE Questionnaire,
which has 85 percent sensitivity and 89 percent specificity
(31):
Case Study
A 53-year-old T4 paraplegic was found to have a 1. Have you ever felt you ought to Cut down on drink-
decreased creatinine clearance of 40 cc/min at annual ing?
screening (the prior creatinine clearance had been 90 cc 2. Have people Annoyed by you criticized your drink-
per min). The patient emptied his bladder by reflex void- ing?
ing. Urinalysis showed many WBCs, RBCs, protein, and 3. Have you ever felt bad or Guilty about your drink-
RBC casts. Renal ultrasound showed kidneys increased ing?
in size with no evidence of reflux. Post-void residual urine 4. Have you ever had a drink first thing in the morn-
volume was 20 cc. ing to steady your nerves or get rid of a hangover
Antistreptolysin O titer was elevated and the diag- (Eye-opener)?
nosis of post-streptococcal glomerulonephritis was made.
The patient then recalled that several months earlier he One yes response should raise the suspicion of alco-
had had a throat infection. He was placed on prednisone hol abuse. More than one yes response should be con-
and renal function rapidly improved. sidered a strong indicator that alcohol abuse exists. Other
screening questionnaires include the CAGE AID Ques-
tionnaire, the Alcohol Use Disorders Identification Test,
Discussion
the Michigan Alcoholism Screening Test (MAST), and the
The relatively sudden decline in creatinine clearance sig- Drug Abuse Screening Test (DAST). If screening for alco-
naled a problem. Postvoiding residual volume was nor- hol or other drug abuse is positive, referral for rehabili-
mal, making vesicoureteral reflux unlikely. Enlarged kid- tation should be considered.
neys on ultrasound and red cell casts in the urine sediment
suggested intrinsic renal disease. For a detailed discussion
Case Study
of the genitourinary problems of SCI patients, see Chap-
ters 22 and 23. A 38-year-old C7 complete tetraplegic was noted to be
tremulous and tachycardic to 120 beats per minute at
his primary care visit. Blood pressure was 160/80. The
SUBSTANCE ABUSE diagnosis of autonomic dysreflexia was considered. How-
ever, upon questioning, the patient reported that he con-
SCI patients are probably at increased risk for substance sumed two six-packs of beer per day for the past several
abuse (30,31). Indeed, substance abuse, such as alco- months and had stopped drinking only two days before
holism, may cause the injury through events such as his clinic visit.
drunk driving. The diagnosis of alcohol withdrawal syndrome was
Smoking is the leading cause of preventable death in made. He was placed on diazepam and referred for alco-
the United States. Smoking cessation is the single most hol rehab.
244 MEDICAL MANAGEMENT
tive in nature. Diffuse burning or aching pain below the of function and pain reduction. Reduction of work
level of injury, which is the most common type of pain in through the use of devices, such as electric wheelchairs,
patients with SCI, is neuropathic. Radiating pain, which and the use of nonsteroidial anti-inflammatory drugs
extends from the site of SCI into the distribution of adja- (NSAIDs) and steroid injections can reduce inflammation
cent dermatomes, is neuropathic. Nociceptive pain relief and prevent functional loss.
may be achieved by peripherally acting analgesics (aspirin)
and centrally acting analgesics, such as codeine. Combi- Case Study
nations of agents are superior to individual agents. Periph-
erally acting analgesics and centrally acting analgesics are A 56-year-old man with C6C7 tetraplegia from a fall 1 year
helpful in treating radicular pain; however, they are not earlier presented for his annual evaluation. He complained
useful in treating segmental pain. Diffuse pain below the of swelling and pain in his hands during the prior 2 months.
level of spinal disease or central pain is neuropathic in Radiographs of the hands and wrists revealed cystic
origin and is difficult to treat. Opioids and serotonin reup- changes, and a bone scan demonstrated increased uptake
take inhibitors may be helpful. For a detailed discussion of in both wrists and hands. The diagnosis of reflex sympa-
pain in SCI patients, see Chapter 33. thetic dystrophy was made. Upon further questioning, the
patient informed his provider that physical therapy follow-
ing his injury had been delayed because of pneumonia.
SPASTICITY Reflex sympathetic dystrophy presents as pain and
swelling in an extremity, trophic skin changes, and the
More than 75 percent of SCI patients have spasticity one signs and symptoms of vasomotor instability. It may be
year after discharge (5). An increase in spasticity may sig- prevented by early physical therapy.
nal the presence of an exacerbating factor such as
syringomyelia, infection, or decubitus ulcer. Spasticity
may be treated with stretching exercises and medication PREVENTION OF ACCIDENTS AND VIOLENCE
including baclofen, benzodiazepines, and dantroline. For
more information on treating spasticity in the SCI patient, Accidents and violence cause most spinal cord injuries. At
see Chapter 34. their periodic evaluation, SCI patients should be ques-
tioned and counseled on the prevention of accidents. The
wearing of seatbelts and the safe handling of firearms are
MUSCULOSKETAL DISORDERS important subjects for such discussions.
3. DeLisa JA, Kirshblum S. A review: Frustrations and needs in clin- dial infarction associated with antihypertensive drug therapy.
ical care of SCI patients. J Spinal Cord Med 1997; 20:384390. JAMA 1995; 274:620625.
4. Schmitt JK, Midha M, McKenzie N. Medical complications of 21. Schmitt JK, Koch KS, Midha M. Profound hypotension in a
spinal cord disease. In: Young R, Woolsey R (eds.), Diagnosis and tetraplegic patient following antiotensin-converting enzyme
Management of Disorders of the Spinal Cord. Philadelphia: WB inhibitor, lisinopril. Paraplegia 1994; 32:871874.
Saunders, 1995; 297316. 22. McKenzie ND, Julius DA, Macdonald RE. Psychiatric aspects of
5. Ditunno JF, Formal CS. Chronic spinal cord injury. NEJM 1994; spinal cord injury. Phys Med Rehabil 1987; 1:499514.
330:550556. 23. American Cancer Society. Summary of American Cancer Society
6. Brenes G, Dearwater MS, Shapero R. et al. High density lipopro- Recommendations for early detection of cancer in asymptomatic
tein cholesterol concentrations in physically active and sedentary people. Atlanta, GA.: American Cancer Society, 1992.
spinal cord injured patients. Arch Phys Med Rehabil 1986; 24. Lieberman DA, Weiss DG, Bond JH, et al. Use of colonscopy to
76:445. screen asymptomatic adults for colorectal cancer. N Engl J Med
7. Walker WC, Khokar MS. Silent cardiac ischemia in cervical spinal 2001; 343:162168.
cord injury. Case study. Arch Phys Med Rehabil 1992; 73:91. 25. Podolsky D. Going the distance. The case for true colorectal cancer
8. Schmitt JK, Adler RA. Endocrine-metabolic consequences of screening. N Engl J Med 2000; 343:207208.
spinal cord injury. Arch Phys Med 1987; 1:425441. 26. Cockroft DW, Gault MH. Prediction of creatinine clearance from
9. Mayo ME, Bradley WE. The urinary bladder in spinal cord dis- serum creatinine. Nephron 1976; 16:31.
ease. In: Young R, Woolsey R (eds.), Diagnosis and Management 27. Navon JD, Soliman H, Khonsari F, et al. Screening Cystoscopy
of Disorders of the Spinal Cord. Philadelphia: WB Saunders, 1995; and survival in spinal cord injury patients with squamous cell can-
211243. cer of the bladder. J Urol 1997; 157:21092111.
10. Segal JL. Clinical pharmacology of spinal cord injury. In: Young 28. Stonehil WH, Goldman HB, Dmochouski RR. The use of urine
R, Woolsey R (eds.), Diagnosis and Management of Disorders of cytology for diagnosing bladder cancer in spinal cord injury
the Spinal Cord. Philadelphia: WB Saunders, 1995; 441438. patients. J Urol 1997; 157:21122114.
11. Bockenek WI, Mann N, Lanig I, Dejong G, Beatty L, Delisa JA, 29. Steinberg G, Brendler C. Diseases of the prostate. In:
Gaus BM (eds.), Primary care for persons with disability. Philadel- Bennett JC, Plum F (eds.), Cecil Textbook of Medicine 29th ed.
phia: Lippincott, 1998; 905928. Philadelphia: WB Saunders, 1996; 13411345.
12. Orenstein WA. Vaccination/immunization. In: Bennett JC, Plum 30. Centers for Disease Control, Office of Smoking Cessation. A
F (eds.), Cecil Textbook of Medicine 20th ed. Philadelphia: WB report of the Surgeon General, 1990, at a glance. Rockville, MD:
Saunders, 1996; 4046. Centers for Disease Control, 1990; USDHHS Publication CDC
13. Garrett AB, MacDonald RE. Dermatological problems in the 90-8419.
spinal cord injury patient. Phys Med Rehab 1987; 1:489497. 31. Alcohol and Other Drug Abuse. In: Clinicians Handbook of Pre-
14. Woolsey RM, McGarry JD. Cause, prevention and treatment of ventive Services. U.S. Department of Health and Human Services.
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15. Lindan O, Gerbenway RM, Piazza JM. Pressure distribution on Washington, D.C.: U.S. Government Priority Office, 1994;
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16. Kaplan NM. Treatment of hypertension drug therapy. In: Clini- 33. Bistrian B. Nutritional assessment. In: Bennett JC, Plum F (eds.),
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17. Yekutiel M, Brooks ME, Ohry A, et al. The prevalence of hyper- 34. Woolsey RM. Pain in spinal cord injury disorders. In: Young R,
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18. Omvik P, Myking OL. Unchanged central dynamics after six 35. Garland DE, Stewart CA, Adkins RA (eds.), Osteoporosis after
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Spinal Cord Injury:
20 The Role of
Pharmacokinetics in
Optimizing Drug Therapy
mong the many factors contributing patients bloodstream to replace those that the patients
ment are usually rather straightforward: administer the bioavailability. All of the molecules of oral doses of
drug of choice at the recommended dose and frequency. diazepam, valproic acid, sulfamethoxazole, and trimetho-
However, the decisions involved in identifying the drug prim end up in the systemic blood (i.e., 100 percent
of choice, and in establishing the recommended dose bioavailability), and so do almost all of the molecules of
and frequency, reflect extensive research on the phar- oral doses of amoxicillin, cephalexin, clonidine, enalapril,
macodynamic and pharmacokinetic properties of the indomethacin, theophylline, and warfarin (bioavailabili-
drug. Most of the time, following standard procedures ties of 90 to 100 percent). These drugs are both lipophilic
results in maintaining an appropriate concentration of the and hydrophilic, are mostly nonionized at the pHs found
drug in the target organ for a sufficient length of time to in the gastrointestinal (GI) tract, and are not metabolized
bring about the successful treatment of the patients con- by enzymes in the gut or on their first pass through the
dition. However, for some patients, standard dosing is not liver in the liver portal blood flow. Among the drugs with
enough (2,3), and so they do not improve. For other low oral bioavailability are propranolol (26 percent),
patients, standard dosing is too much, and they suffer the morphine (24 percent), acyclovir (23 percent),
consequences of toxic overdose. These opposite reactions cyclosporine (23 percent), terbutaline (14 percent), and
of different individuals to identical drug doses are due to the aminoglycoside antibiotics (essentially 0 percent).
pharmacokinetic differences among people (4,5). Drugs with low bioavailabilities tend to be drugs that are
either highly ionized in the gut (morphine), or highly
hydrophilic and poorly lipophilic (the aminoglycosides),
Pharmacokinetics
or highly metabolized in the gut or on first pass through
When a patient is given a drug, the drug molecules are the liver (propranolol). The majority of clinically relevant
absorbed into the bloodstream, distributed by the blood drugs have oral bioavailabilities ranging from 40 to 80
to the target organ (and throughout the rest of the body), percent.
metabolized, and excreted. Hence, the pharmacokinetic
parameters are: absorption, distribution, metabolism, and
Distribution
excretionA, D, M, E.
Drug distribution is the movement of a drug from one
location in the body to another. Drug distribution, like
Absorption
drug absorption, is generally by passive diffusion down
Most drugs are fat soluble, and whether they are given the concentration gradient. As long as blood levels are
orally, transcutaneously, subcutaneously, or intramuscu- higher than tissue levels, the drug moves out of the blood
larly, they are absorbed into the bloodstream by passive and into the tissues. When blood levels fall below tissue
diffusion down a concentration gradient. The rate of dif- levels, the drug leaves the tissues and returns to the blood.
fusion/absorption is determined by the molecular size, While in the blood, drugs bind to plasma proteins: acidic
lipophilicity, and ionization of the drug; the permeability, drugs bind to several different sites on albumin, basic
thickness, and surface area of the absorptive surface; and drugs bind to lipoproteins or to alpha-1 acid glycopro-
the concentration difference between the site of adminis- tein. The degree of plasma protein binding of different
tration and the blood. The rate of absorption is fastest for drugs varies from none (acetaminophen and lithium) to
a small, highly fat-soluble, nonionized drug, being 99 percent (diazepam, furosemide, and warfarin); many
absorbed across a highly permeable, thin, large-area drugs are in the 80 to 98 percent range. For any particu-
membrane that has a high rate of blood flow to carry off lar drug, there is a constant equilibrium between the per-
drug molecules as soon as they enter the blood thereby centage of the drug that is bound, and the percentage that
maintaining the concentration gradient. All drugs given is free in the plasma. Drug molecules that are bound to
orally are absorbed better from the small intestine than plasma proteins are not able to leave the blood. Only the
from the stomach because of the greater surface area and unbound molecules are free to diffuse out of the blood
the greater blood flow of the small intestine. Some drugs, to provide the therapeutic concentration in the target
such as weak acids or very weak bases, which would be organ or to be removed from the body. The plasma pro-
mostly nonionized in the acidic stomach fluid, can be tein binding sites may become saturated at high drug con-
absorbed from the stomach, but the bulk of drug absorp- centrations, and competition occurs between drugs that
tion occurs in the small intestine. bind to the same site on plasma proteins.
patients blood. To emphasize the theoretic nature of this Many different isoforms of cytochrome P450 have
term, it is also known as the apparent volume of distrib- been identified (7). The individual members of the
ution. Comparing the volume of distribution (Vd) of a cytochrome P450 superfamily are referred to using the
drug to the physiologic volumes of the various water com- nomenclative CYP (for the superfamily) plus a number
partments of the human body gives some indication of identifying a family grouping of isozymes with a more
how extensively the drug is distributed throughout the than 40 percent homology of amino acid sequences (e.g.,
body and how much of it is bound to storage sites in the CYP2). An additional letter identifies a subfamily group-
tissues. Furosemide (Vd about 8 L) is stored mostly in ing with more than 55 percent homology with each other
the blood (about 5 L for a 70-kg person), amoxicillin (Vd (CYP2D), and another number identifies one specific
15 L) is stored mostly in the extracellular fluid (14 L), pra- enzyme (CYP2D6). Of the twenty-six subfamilies of CYP
zosin (Vd 42 L) is stored in total body water (42 L), and enzymes that have been found in mammals, most clini-
diltiazem (Vd 220 L) is stored in tissues. cally relevant drugs are metabolized by members of the
CYP1A, CYP2C, CYP2D, or CYP3A subfamilies.
Although most drugs are metabolized by more than one
Metabolism
CYP enzyme, the metabolism of a particular drug may be
Drug metabolism, perhaps more correctly referred to as done more efficiently by a particular isozyme. The simul-
drug biotransformation, is an enzymatic process in taneous or sequential administration of two or more com-
which the drug molecule is usually made more polar, and pounds that are preferred substrates for the same isozyme
therefore less lipophilic. A molecule of the lipophilic par- will alter the metabolism of both drugs. Thus, unless dos-
ent drug may be filtered or excreted by the kidney into ing is adjusted, this results in undesired blood concen-
the lumen of the nephron, but, because the drug mole- trations of the drugs. If one of the drugs induces the
cule is lipophilic, it diffuses back into the bloodstream shared isozyme, metabolism of the second drug will be
and does not leave the body in the urine. The less increased and its blood levels will be reduced, possibly
lipophilic polar metabolite on the other hand, may be to subtherapeutic concentrations. Conversely, if one of
excreted in the urine, or it may be acted on by other the drugs inhibits the shared isozyme, metabolism of the
enzymes that make it even more polar and easier to second drug will be reduced and its blood levels will be
excrete. Thus, drug metabolism is a crucial step in elim- increased, possibly to toxic or lethal concentrations. The
inating lipophilic compounds from the body. In general, disruption of CYP isozyme drug metabolism is not
the metabolites of a drug lack its pharmacodynamic restricted just to other drugs.
properties, and drug metabolism usually terminates the For example, a series of flavonoids and fura-
desired therapeutic action. However, this is not always nocoumarins found in high concentrations in the peel, but
the case. The metabolites of some drugs share the phar- not the pulp, of grapefruit, are potent inhibitors of
macologic actions of the parent drug; other medications CYP3A4. While little, if any, of these compounds are
are given as inactive prodrugs that require metabolism ingested when eating grapefruit sections or drinking hand
into an active form to provide the therapeutic benefit. squeezed grapefruit juice, the commercial process of
Consequently, drug metabolism should not be consid- extracting the juice by crushing the entire grapefruit can
ered to be drug detoxification. transfer considerable amounts of the flavonoids and fura-
nocoumarins into the juice and subsequently into the con-
sumer. There is a great variability from brand to brand,
Cytochrome P450 Isozymes
and from lot to lot, as to the amount of CYP3A4 inhibit-
Although liver cells perform the bulk of drug metabolism, ing substances present in commercial grapefruit juice, but
cells in other organs can do so as well. The actual meta- the clinical relevance of this fooddrug interaction has
bolic transformation of drugs, and a wide variety of other been demonstrated in the enhanced hemodynamic action
endogenous and food-derived lipophilic compounds, is of felodipine and nifedipine (8) and in excessive sedation
carried out by a large number of mixed-function oxidase and ataxia of anxiolytics and hypnotics (9). Undoubtedly,
enzymes, collectively referred to as the cytochrome P450 many other compounds in our environment can also
enzymes. These enzymes are located in the membranes change CYP isozyme activity but have not yet been
of the smooth endoplasmic reticulum of cells in the liver reported.
and other tissues. The cytochrome P450s are a super- Because preliminary investigations suggest that SCI
family of iron-containing enzymes that add a functional reduces liver drug metabolism (10) and gene expression
group, usually a hydroxyl, to the structure of lipophilic (11), the consequences of drugdrug and fooddrug inter-
endogenous molecules and lipophilic drugs and environ- action at the level of cytochrome P450 metabolism may
mental chemicals that have entered the body. This facili- be particularly problematic for patients with SCI. The
tates the excretion of these compounds by making them CYP-related interactions of some common drugs are
more polar and less lipophilic. listed in Table 20.1 (12).
250 MEDICAL MANAGEMENT
TABLE 20.1
Interaction of selected drugs and cytochrome P450 isozymes.
Based on information taken from Herman (1999)
CYP1A2
estrogens fluvoxamine insulin
caffeine, theophylline ciprofloxacine cigarette smoke
diazepam, other benzodiazepines cimetidine erythromycin, clarithromycin
propranolol, local anesthetics oral contraceptives phenobarbital, phenytoin, carbamazepine
haloperidol, olanzapine omeprazole, lansoprazole
TCAs ritonavir
CYP2C9
activates losartan fluvoxamine phenobarbital
warfarin, dicumarol amiodarone phenytoin
ASA and most NSAIDs omeprazole carbamazepine
phenobarbital, phenytoin tolbutamide
fluoxetine, moclobemide HMG-CoA reductase inhibitors
diazepam, temazepam ritonavir
CYP2C19
TCAs fluoxetine prednisone, norethindrone
diazepam, temazepam fluvoxamine phenobarbital, phenytoin, carbamazepine
omeprazole, lansoprazole paroxetine
propranolol omeprazole, lansoprazole
phenytoin, barbiturates, valproic acid ritonavir
CYP2D6
codeine, other opioids quinidine, amiodarone
dextromethorphan fluoxetine, paroxetine, sertraline
several beta blockers TCAs, venlafaxine, nefazodone
TCAs, SSRIs, venlafaxine chlorpheniramine
haloperidol cimetidine
omeprazole phenothiazines, haloperidol
halothane ketoconazole
selegiline HMG-CoA reductase inhibitors
CYP3A4
halothane ketoconazole, itraconazole, fluconazole dexamethasone
TCAs, SSRIs erythromycin, clarithromycin sex steroids
erythromycin TCAs, venlafaxine, nefazodone omeprazole, lansoprazole
HIV protease inhibitors fluvoxamine, fluoxetine, sertraline erythromycin
calcium channel blockers cyclosporin phenytoin, barbiturates
(except diltiazem) omeprazole, lansoprazole
cyclosporin calcium channel blockers (diltiazem strong)
terfenadine midazolam
midazolam, alprazolam, triazolam corticosteroids
lovastatin, simvastatin, atorvastatin commercial grapefruit juice (from the peel)
NOTE:
TCAs = tricyclic antidepressants, e.g., amitriptyline, imipramine, desipramine, etc.
SSRIs = serotonin selective reuptake inhibitor antidepressants, e.g., fluoxetine, fluvoxamine, paroxetine, etc.
HMG-CoA = hydroxymethylglutaryl-coenzyme A, reductase inhibitors are lovastatin, simvastatin, atorvastatin, etc.
SPINAL CORD INJURY: THE ROLE OF PHARMACOKINETICS IN OPTIMIZING DRUG THERAPY 251
cern with the fluoroquinolones is that they are weak withdrawal syndrome that includes hallucinations and
inhibitors of CYP1A2, with the exception of ciprofloxacin, seizures. The neurochemical basis for this involves both
which is a strong inhibitor of CYP1A2. Theophylline is receptor changes induced by chronic baclofen and
metabolized by CYP1A2. If ciprofloxacin, or another baclofens short half-life of 3 to 4 hours (38). Being an
quinolone, is given to a patient taking theophylline for res- agonist, chronic baclofen downregulates (i.e., makes sub-
piratory problems, the metabolism of theophylline will be sensitive) GABA-B receptors. By inhibiting the release of
reduced. Unless the dose of theophylline is reduced, theo- numerous neurotransmitters in the brain, chronic
phylline blood levels can rise to the toxic range, and the baclofen upregulates (i.e., makes supersensitive) the
patient may develop seizures or other toxic side effects. receptors for dopamine, norepinephrine, serotonin, glu-
The fluoroquinolone antibiotics also reduce neuronal inhi- tamate, and other neurotransmitters. When baclofen is
bition in the central nervous system, mediated by the discontinued, it is completely cleared from the body in 3
inhibitory neurotransmitter gamma aminobutyric acid to 5 half-lives, or about 9 to 20 hours. However, it takes
(GABA). This not only lowers the seizure threshold and the supersensitive receptors 2 to 3 weeks to return to their
increases the risk of theophylline-induced seizures, but also pre-baclofen level of sensitivity. As soon as the baclofen
may induce or exacerbate spasticity in SCI patients. These is gone, neurotransmitter release returns to normal, but
pharmacodynamic effects suggest caution with the use of the released transmitters are now acting on supersensitive
fluoroquinolone antibiotics in people with SCI. receptors that overdrive their respective neural pathways.
Over-driving dopamine produces a paranoid state
similar to that seen in amphetamine overdose; overdriv-
Spasticity Reduction
ing glutamate produces seizures. These brain-related com-
Spasticity, a motor syndrome characterized by increased plications of baclofen withdrawal can be avoided by grad-
muscle tone, exaggerated tendon jerks, and flexor spasms, ually reducing the dose of baclofen over a 4- to 6-week
is a common result of lesions to the upper motor neurons. period, or by administering baclofen intrathecally directly
Baclofen, diazepam, tizanidine, gabapentin, clonidine, and into the spinal cord via implantable programmable infu-
dantrolene are the pharmacologic agents currently used to sion pumps. The intrathecal administration of baclofen
reduce the disruptive impact of spasticity on the lives of SCI is, however, an invasive, complex procedure that has met
patients (36,37). The equivocal results of antispasticity with variable success in selected patients (39).
drugs administered to SCI patients may reflect an altered An alternative to baclofen is the orally active agent
pharmacokinetic profile caused by the physiologic changes tizanidine. Tizanidine is a structural analog of clonidine
induced by the SCI (6). This results in some patients hav- and, like clonidine, is an agonist at norepinephrine alpha-
ing subtherapeutic blood levels, while other patients, are 2 receptors and at imidazoline receptorsa class of
exposed to excessively high toxic levels. recently discovered receptors in the CNS. Also, like cloni-
Baclofen is a structural analog of the inhibitory neu- dine, tizanidine reduces spasticity, but, unlike clonidine,
rotransmitter GABA. It acts as an agonist at GABA-B recep- does so at doses having little effect on blood pressure.
tors on the presynaptic nerve terminals of neurons and Tizanidine facilitates both presynaptic and postsynaptic
releases a variety of neurotransmitters in the brain and in inhibition in the spinal cord and also inhibits transmis-
the spinal cord. Stimulation of the GABA-B receptors hyper- sion in pain pathways in the spinal cord. Whether these
polarizes the presynaptic nerve membrane and prevents the actions are mediated by alpha-2 receptors or imidazoline
release of the neurotransmitter from that nerve terminal. receptors remains to be established. A procedure to
Diazepam facilitates the GABA-mediated opening of the switching patients from baclofen to tizanidine has been
chloride channels linked to GABA-A receptors. This also described by Brenner et al. (40). Although there do not
hyperpolarizes neural membranes, blocks action potentials, appear to be any adverse interactions between baclofen
and reduces the release of neurotransmitters from their nerve and tizanidine (41), existing studies have been done with
terminals. It seems that the antispastic action of both healthy, able-bodied subjects. Comparable studies with
baclofen and diazepam is caused by their inhibition of the SCI patients remain to be performed.
release of excitatory neurotransmitters in the motor horn of Other compounds, such as gabapentin (42) and 4-
the spinal cord. In the able-bodied, baclofen produces less aminopyridine (43,44) also show promise in reducing
sedation than diazepam and does not reduce muscle spasticity in SCI patients, but, as with so many other
strength, as may be the case with dantrolene. aspects of spinal cord medicine, more research is needed.
However, the exaggerated enterohepatic recircula-
tion and prolonged elimination of lorazepam suggests
that other benzodiazepines should be investigated for CONCLUSION
unexpected pharmacokinetics in patients with SCI. In
addition to having unpredictable antispasticity effects, As a consequence of the disruption of central neural path-
oral baclofen can have an unusual, albeit uncommon, ways, spinal cord-injured humans have a wide range of
SPINAL CORD INJURY: THE ROLE OF PHARMACOKINETICS IN OPTIMIZING DRUG THERAPY 255
altered or absent physiologic processes. These changes 15. Seifert J, Lob G, Stoephasius E, Probst J, Brendal W. Blood flow
in muscles of paraplegic patients under various conditions mea-
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in treating their medical problems. Unfortunately, at the 10:185191.
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clearance of lorazepam in humans with spinal cord injury. J Clin
logic changes and the resulting alterations in pharmaco- Pharmacol 1991; 31:651656.
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79(3):260265.
population pharmacokinetic data. A comprehensive for- 18. Lipetz JS, Kirshblum SC, OConner KC, Voorman SJ, Johnson
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19. Nuhlicek DN, Spurr GB, Barboriak JJ, Rooney CB, el Ghatit AZ,
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256 MEDICAL MANAGEMENT
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IV
MANAGEMENT OF THE
BLADDER, BOWEL, AND
SEXUAL DYSFUNCTION
This Page Intentionally Left Blank
21 Normal and
Abnormal Micturition
259
260 MANAGEMENT OF THE BLADDER, BOWEL, AND SEXUAL DYSFUNCTION
great vessels, and synapse at the inferior mesenteric and Knowledge of this neuroanatomy enables one to
hypogastric plexuses. The postganglionic hypogastric understand the bladder function associated with certain
nerve supplies the bladder neck and proximal urethra, lesions (1). For example, a stroke in the frontal cortex
including the prostate gland (in the male); this nerve indi- may cause a loss of cortical inhibition to the pons, result-
rectly affects the parasympathetic function. This nerve ing in uninhibited detrusor activity (detrusor hyper-
system primarily mediates bladder storage. The somatic reflexia), but coordination between bladder and sphinc-
nerve originates from the S2 to S4 pudendal nucleus (in ter is preserved because of the intact pontine sacral axis.
the ventral horn of the spinal cord) and courses along the A lesion below the pons (i.e., spinal cord injury) results
autonomic nerves to innervate the external striated mus- in the lack of coordinated bladder sphincter activity (blad-
cle sphincter. The somatic nerve mediates bladder storage der sphincter dyssynergia) owing to the disrupted pontine
by increasing urethral resistance. sacral axis.
Neurotransmitters, released at the neuromuscular A summary of normal voluntary micturition includes
junction, are received by appropriate neuroreceptors (2). bladder filling, storage, and emptying of the bladder (1).
Cholinergic receptors are located in the bladder base and During bladder filling, pressure in the bladder rises slowly
body and mediate bladder contraction, which is activated with a compliant bladder wall and minimal cholinergic
by the acetylcholine released by the parasympathetic activity. Urethral resistance also increases at this time. At
nerves. The adrenergic receptors are activated by a critical pressure and volume (the micturition threshold),
norephinephrine released by the sympathetic nerves. Beta- sympathetic nerve activity increases, causing contraction
adrenergic receptors are located in the bladder body and of the internal sphincter via alpha reception and promot-
mediate bladder inhibition, thus promoting bladder stor- ing continence. Also, pudendal nerve activity increases
age and continence. Alpha-adrenergic receptors are located involuntarily (and voluntarily) to contract the external ure-
in the bladder base and proximal urethra (and the prostate) thral sphincter and promote continence. Bladder filling
at the internal sphincter; these receptors mediate bladder occurs at low intravesical pressure, with normal sensation
neck contraction, thus promoting continence, by increas- and no involuntary bladder activity.
ing urethral resistance. Acetycholine is also released at the Eventually, bladder distension leads to coordinated
pudendal neuromuscular junction and mediates external micturition. Voluntary inhibition of somatic discharge to
sphincter contraction, which also promotes continence. the external sphincter decreases urinary outlet resistance.
Other potential neurotransmitters such as adenosine Sympathetic activity decreases, causing bladder neck
triphosphate, vasoactive peptides, enkephalins, substance relaxation and facilitation of parasympathetic nerve activ-
P, and nitric oxide, have been identified in the lower uri- ity, thus promoting a bladder contraction. The pons,
nary tract and may be deemed useful in future bladder modulated by the frontal cortex, coordinates this activ-
management if they can be appropriately manipulated (2). ity to allow normal voiding with decreased urethral
In addition, estrogens help maintain urethral tone in sphincter resistance, no urinary outlet obstruction, and
females via local estrogen receptors located in and around a well-functioning bladder.
the vessels of the spongy urethral tissue. Estrogen also
helps maintain pelvic muscle tone.
Sensory innervation occurs through the pelvic nerve, NEUROUROLOGIC EVALUATION
which mediates bladder wall stretch reception during the
filling of the bladder. The hypogastric nerve is responsi- To assess the function of the bladder and/or urethra, an
ble for mediating bladder mucosal pain and temperature. appropriate evaluation must be made. This evaluation
The CNS normally controls and coordinates mic- includes a basic patient history, including a questionnaire
turition. During micturition, afferent sensory nerve acti- and voiding diary to document the micturition pattern,
vation results from bladder filling and pressure, travels to and a physical examination, especially to evaluate the
the sacral cord, and ascends from the sacral cord to the pelvic anatomy and neurologic system. Pelvic anatomy
rostral pontine reticular formationthe micturition cen- is assessed by examining for pelvic prolapse in the female
ter. This center coordinates urethral sphincter relaxation and prostate enlargement in the male. The focus of the
just before a normal detrusor contraction and initiates neurourologic examination includes an assessment of the
and sustains detrusor contraction during voiding. Vol- pelvic and lower extremity sensation, tone, and reflexes,
untary control of the detrusor is from the frontal cortex especially the bulbosynaptic reflex. The bulbosynaptic
to the pons and down the reticulospinal tract of the spinal reflex is a monosynaptic reflex of the sacral cord. It is per-
cord to the detrusor nucleus. This control is net inhibitory. formed by providing afferent stimulis (penile, clitoral, or
Without this control, the bladder would have involuntary, pubic stimulation) simultaneous with the examiners fin-
uninhibited contractile activity. The frontal cortex also ger in the rectum to sense a reflex pelvic muscle contrac-
has direct corticospinal tract connections to the pudendal tion caused by the stimulus. The reflex is present in most
nucleus to help voluntarily control the external sphincter. patients with an intact sacral cord.
NORMAL AND ABNORMAL MICTURITION 261
A urinalysis and urine culture are done to rule out interaction. In addition, decreased EMG activity may be
any obvious pathologic processes, such as urinary tract indicative of pudendal neuropathy and decreased urethral
infection (UTI) and tumor. A cystoscopy should be done resistance.
in such cases. Next, an assessment of urinary function is Assessment of the proximal urethral sphincter can
done. Micturition is best defined by the urodynamic eval- be done using voiding cystourethrography; the patient
uation, which includes a urinary flow rate, bladder cys- voids radiographic contrast under radiographic moni-
tometrogram (CMG), sphincter electromyography toring. It also can be assessed by the UPP, which measures
(EMG), leak point pressure (LPP) measurement, and ure- urethral pressure at points along the urethral length using
thral pressure profile (UPP). The flow rate evaluation is a small catheter that is slowly withdrawn along the ure-
a noninvasive quantification of urinary flow that depends thra and connected to a pressure transducer. A decreased
on volume and speed of the voided stream. A poor flow urethral length or pressure may indicate weak pelvic floor
rate may indicate urinary outlet obstruction or ineffective muscles and correlate with stress urinary incontinence.
detrusor activity. After determining the flow rate, a LPP measurements are the most recent parameters to
catheter is passed into the bladder and a postvoid resid- be popularized (4). The CMG leak point pressure is the
ual urine amount is recorded. A large volume (100 ml) measured detrusor pressure at which the bladder, usually
is also usually secondary to an obstruction or to an inef- an unstable or poorly compliant bladder, leaks. Generally,
fective detrusor. CMG assesses the efficacy of the detru- a CMG pressure over 40 cm H2O places the upper urinary
sor and further differentiates these problems. tract at risk for hydronephrosis, reflux, infection, and other
The CMG monitors bladder pressure during blad- problems (5). The stress (valsalva, cough) LPP is the blad-
der filling and emptying by use of a catheter in the blad- der pressure at which point the stressed bladder leaks dur-
der that is connected to a pressure transducer. Gas (car- ing filling CMG (usually at 150 to 200 cm filling volume).
bon dioxide) or water is infused at a controlled rate, often It is also equivalent to the bladder pressure needed to drive
30 to 60 ml/min, which is usually faster than physiologic urine across the urethra (4). Basically, it is a quantifica-
filling. The CMG assesses bladder compliance, sensation, tion of the Marshall test that is performed with a full blad-
volume, and presence or absence of normal or uninhib- der, under stress, during the pelvic examination.
ited bladder activity (detrusor instability). Total CMG The normal CMG and EMG start with low blad-
includes both bladder and abdominal pressure measure- der pressure, at zero volume. As bladder filling increases
ments. The true detrusor CMG can be determined by sub- to 300 to 400 ml, with the bladder pressure increasing
tracting the abdominal pressure (measured by a rectal slightly, the patient feels a sensation of fullness. The EMG
pressure transducer) from the total CMG pressures. The increases as the patient feels the sensation to void (the
latter requires at least a four-channel CMG. guarding reflex). At the micturition threshold (the
The pressure-flow study more accurately indicates patients bladder volume limit and the patient is ready to
the presence or absence of urinary outlet obstruction (3). void), the EMG silences, urethral resistance diminishes,
This is the recorded bladder pressure at the point of max- and then there is a rapid bladder contraction, as noted
imal urinary flow. The recorded values can be plotted by a rapid increase in detrusor pressure, with sphincteric
against a standard nomogram to assess the relative value relaxation. Here, the patient has a normal, well-con-
of the obstruction. This is important, because a low uri- trolled, and coordinated void, as recorded by flow rate.
nary flow rate does not necessarily indicate urinary The above basic urodynamics generally provide a
obstruction, unless it is associated with a high bladder urologic diagnosis as to the etiology of the voiding dys-
pressure. Also, a high urinary flow rate does not rule out function and help classify it (6). If the patient has a more
urinary obstruction unless it is associated with a low blad- complex history or has failed previous treatments, more
der pressure. sophisticated urodynamic studies are needed. Combina-
The EMG is an assessment of pudendal nerve func- tion voiding cystourethrography with concomitant uro-
tion using a recording electrode on the perineum or the dynamic pressure measurements provides a more exact
rectal or vaginal wall, or using needle electrodes placed diagnosis, if needed. However, although this method of
into the external sphincter via the perineum. Nerve depo- lower urinary tract monitoring is state of the art, it is not
larization of the muscle membrane results in electrical universally available.
potentials that are received by the electrodes and The first pattern in the classification of bladder dys-
recorded. The total EMG reflects activity of the urinary function is detrusor instability, which is involuntary,
outlet by recording pelvic floor striated muscle and exter- insuppressable detrusor activity with bladder pressure
nal sphincter activity. Increased EMG activity usually changes over 15 cm of water during bladder filling. Usu-
indicates increased urethral resistance, and decreased ally at low volumes, there is a short latency between the
EMG activity usually indicates decreased urethral resis- urge to void and the detrusor contraction. The patient can
tance. Furthermore, EMG recorded simultaneously with have irritative voiding symptoms, frequency, urgency,
detrusor activity (CMG) assesses the detrusor sphincter nocturia, or obstructive symptoms, such as decreased
262 MANAGEMENT OF THE BLADDER, BOWEL, AND SEXUAL DYSFUNCTION
force of stream, hesitancy, and straining, if an obstruction by defects in the bladders ability to empty urine by poor
exists. If the etiology of the instability is neurologic in bladder contraction or in the urethras ability to block
nature, then the problem is termed detrusor hyperreflexia urine flow by urethral obstruction (1).
(6). Detrusor hyperreflexia can occur with coordinated To improve the storage of urine, bladder overactiv-
urethral sphincter relaxation, typical of suprapontine ity must be suppressed either by medication (primarily
lesions, such as a cerebral vascular accident. Detrusor anticholinergics), electrical stimulation (via feedback inhi-
hyperreflexia also can occur with discoordinated sphinc- bition), or surgical augmentation of the bladder (1). Fur-
ter activity (vesicosphincter dyssynergia), in which the thermore, urethral resistance must be improved to
sphincter contracts abnormally during the bladder con- improve incontinence via medication (alpha adrenergics),
traction, causing increased bladder pressure. This pattern electrical stimulation, Kegel exercises, or by surgical
is typical of patients with SCI. In patients without neu- means that increase resistance (urethropexy, artificial
rogenic cause, the detrusor instability can be caused by sphincter, urethral bulking agents) (1).
urinary tract inflammations, urinary obstruction, or blad- To improve emptying of urine, the bladder can be
der lesions, or it can be idiopathic or functional. treated with medication (cholinergics, which usually dont
The second pattern is the acontractile detrusor, which work) or, usually, by intermittent catheterization (1).
is a lack of detrusor contraction during CMG. It can be a Additionally, urethral resistance can be lowered by either
normal finding, probably owing to test inhibition or func- medication (primarily alpha-adrenergic blockers) or
tional causes, or it can be neurogenic in origin. If so, it is surgery (transurethral resection of the obstruction) (1).
termed detrusor areflexia (6). To differentiate neurogenic
from nonneurogenic detrusor areflexia, a bethanechol test
can be done (7). The bethanechol test is based on Cannons CONCLUSION
law, which states that a denervated organ is supersensi-
tive to its neurotransmitter (7). A supersensitive response In summary, knowledge of the neuroanatomy and phys-
to bethanechol (urecholine) during CMG (bladder pres- iology of the normal and abnormal lower urinary tract
sure change over 15 cm H2O at 100cc volume) usually can be enhanced and better understood by urodynamics.
confirms a neuropathic bladder (7). Urodynamic tests define the problem and then help guide
appropriate therapy, especially in patients who have void-
ing dysfunction and a complex neurologic history, such
NEUROUROLOGIC TREATMENT as SCI.
If voiding function results from a normal micturition
reflex of bladder filling and urinary storage and empty-
ing, then the voiding dysfunction can result from either References
a failure of urinary storage or a failure of urinary emp- 1. Wein AJ. Classification of voiding dysfunction: A simple
tying (1). Urinary retention is an extreme consequence approach. In: Barrett DM, Wein AJ (eds.), Controversies in Neuro-
Urology, New York, Churchill Livingstone, 1984; 239.
of failure to empty the bladder, whereas urinary inconti- 2. deGroat WC, Kawatani M. Neuro control of the urinary blad-
nence is an extreme consequence of failure to store urine der. J Neurol Urodyn 1985; 4:285300.
(1). Therefore, treatment of urinary incontinence must 3. Griffiths DJ. Pressure flow studies of micturition. Urology Clin
1996; 23:(31)279.
focus on the improvement of urinary storage capabilities 4. McGuire EJ, Cespedes RD, OConnell H. Leak point pressures,
(1). Failure to store urine can be caused either by defects Urology Clin, N Am 1996; 23(2)253.
in the bladders ability to hold urine (i.e., bladder insta- 5. McGuire EJ, Woodside JR, Border TA, Weiss RM. Prognastic
value of urodynamic testing in myelodysplastic patients. J Urol
bility or poor compliance), or by defects in the urethras 1981; 126: 205209.
ability to withhold bladder pressure, such as poor ure- 6. Krane RT, Siroky MB. Classification of neurourologic disorders.
thral resistance. Also, treatment of urinary retention must In: Krane RT, Siroky MB (eds.), Clinical Neurourology. Boston:
Little, Brown, 1979, 9.
focus on the improvement of urinary emptying capabili- 7. Lapides J, Frend CR, Ajemian EP, Reus WF. A new test for neu-
ties (1). Failure to empty the bladder can be caused either rogenic bladder. J Urol 1962; 88:245.
22
Renal Insufficiency
in Patients with
Spinal Cord Injury
ARF related to renal artery thrombosis can occur as a con- blood volume has been substantially reduced (i.e., heart
sequence of blunt trauma, penetrating trauma, or in asso- failure or third spacing of fluid), there is baroreceptor-
ciation with severe deceleration injuries (13,14). Addi- mediated activation of both the sympathetic nervous sys-
tional causes of ARF in the setting of major trauma include tem and reninangiotensinaldosterone system, as well as
myoglobinuria (as a consequence of muscle injury and stimulation of antidiuretic hormone secretion.
rhabdomyolysis) and iatrogenic causes (e.g., exposure to These neurohumoral counterregulatory systems act
nephrotoxic antibiotics, nonsteroidal anti-inflammatory in concert to maintain the blood pressure and perfusion
drugs, or intravenously administered radiographic contrast of critical organs (the heart and central nervous system)
material) (1521). Regarding clinical assessment, it is use- by increasing both cardiac output and systemic vascular
ful for purposes of diagnosis and management to consider resistance (the latter being achieved through selective
that all cases of ARF can be separated into three basic cat- vasoconstriction involving less-critical vascular beds). In
egories based on the underlying pathophysiology: a) pre- addition, avid renal conservation of sodium, chloride, and
renal failure; b) postrenal failure or obstructive nephropa- water function to minimize further volume losses, while
thy; and c) intrinsic renal failure. In addition, both prerenal the stimulation of thirst and salt appetite promotes
failure and acute tubular necrosis (the most common cause restoration of volume deficits. However, the increased
of intrinsic renal failure) are at opposite ends of a spectrum synthesis and release of potent vasoconstrictors such as
of diseases caused by renal ischemia. Moreover, in aggre- norepinephrine and angiotensin II can also cause severe
gate, ischemic forms of renal injury play a causal role in intrarenal vasoconstriction and mesangial cell contrac-
75 to 85 percent of all ARF cases (2224). The various tion. Moreover, the combination of decreased systemic
causes of renal ischemia, as well as the related type and blood pressure and intrarenal vasoconstriction can cause
severity of renal injury, is discussed under the heading substantial renal hypoperfusion and a reduction in
Ischemic Acute Tubular Necrosis. glomerular filtration rate (GFR). However, severe
glomerular capillary hypoperfusion is usually ameliorated
through the effects of renal autoregulation, a process that
Prerenal Azotemia
functions to preserve renal perfusion by facilitating a
In the general population, prerenal azotemia is one of the combination of afferent arteriolar dilatation and effer-
most common forms of acute renal insufficiency; it occurs ent arteriolar constriction in response to reductions in
in a variety of conditions that cause renal hypoperfusion renal perfusion pressure. Afferent arteriolar dilatation is
(23,24). These typically include intravascular volume primarily mediated by an increase in the intrarenal
depletion (i.e., from hemorrhaging, fluid losses, and or biosynthesis of vasodilatory prostaglandins and kinins.
fluid shifts); low cardiac output states (i.e., severe car- Furthermore, a reduction in preglomerular perfusion
diomyopathy or tamponade); or alterations in intrarenal pressure (detected by stretch receptors located in the walls
hemodynamics (Table 22.1). As a result of either true vol- of afferent arterioles) may also stimulate afferent arteri-
ume depletion, or in conditions where effective arterial olar dilatation in a process mediated through enhanced
production of endothelial-derived relaxing factor or nitric
oxide (NO). Consequently, efferent arteriolar constric-
tion, which is mediated through an increase in angiotensin
TABLE 22.1 II, acts in combination with afferent arteriolar dilatation
Causes of Prerenal Failure to help maintain adequate glomerular capillary pressure
and GFR during periods of renal hypoperfusion. How-
Reduction of Intravascular Volume ever, when the mean systemic arterial pressure falls below
Excessive gastrointestinal losses 80 mmHg, the autoregulatory mechanisms are over-
Hemorrhage whelmed and GFR declines precipitously.
Excessive urinary losses Clinically, prerenal azotemia is characterized by
Sequestration of fluid in third spaces (e.g.,
both a reduction in GFR and a marked increase in the
bowel wall and peritoneal cavity)
Cardiovascular disorders causing renal hypoperfusion
tubular reabsorption of filtrate (the latter being an appro-
Severe heart failure priate renal response to perceived volume depletion).
Pericardial tamponade This, in turn, causes a reduction in urine output (i.e.,
Massive pulmonary embolism with reduction in 400500 ml/d) or oliguria and retention of nitrogen
cardiac output waste (resulting in azotemia). However, patients with
Renal vasoconstriction underlying tubular dysfunction are generally incapable of
Early sepsis syndrome achieving such substantial reductions in urine output and
Severe liver disease volume (i.e., 500 ml/day) even in the presence of pre-
Drug induced (i.e., NSAIDs and cyclosporine) renal failure. Therefore, the absence of oliguria does not
necessarily rule out this diagnosis.
RENAL INSUFFICIENCY IN PATIENTS WITH SPINAL CORD INJURY 265
Diagnosis and Management fluid challenges can be repeated until blood pressure and
hemodynamic status has stabilized. Most patients with
In addition to oliguria, which is usually a manifestation
hypovolemic shock will tolerate an infusion of at least 2
of prerenal azotemia, other characteristic features include
liters of isotonic saline at a rate of 6 ml/min/kg. Follow-
both a low urinary sodium concentration (less than 20
ing a favorable response, the saline infusion should be
mEq/L) and a fractional excretion of sodium (less than 1
continued until the following common endpoints of vol-
percent) caused by the enhanced tubular reabsorption of
ume resuscitation are accomplished (26,27):
this cation. Fractional excretion of sodium (FENa) is cal-
culated from simultaneous measurements of sodium (Na)
1. Central venous pressure of 15mmHg
and creatinine concentrations in serum and spot urine
2. Pulmonary wedge pressure of 1012 mmHg
samples using the following formula:
3. Cardiac index 3L/min/m2
UNa/SNa 4. Oxygen uptake (VO2) 100 ml/min/m2
FENa 5. Blood lactate 4 mmol/L
U Creat/S Creat 100
6. Base deficit 3 to 3 mmol/L
where UNa and SNa represent urinary and serum sodium
concentrations and U Creat and S Creat represent urinary Of note, it may be both difficult and dangerous to
and serum creatinine concentrations, respectively. Once place a central venous pressure line in a patient who is
again, a low urinary sodium concentration and FENa will extremely volume depleted and hypotensive. In such
not be attained in patients with underlying sodium-wast- cases, it is more prudent to begin volume resuscitation via
ing conditions (e.g., diuretic administration, tubuloin- any suitable intravenous route followed by frequent clin-
terstitial nephropathies, or mineralocorticoid deficiency) ical assessment of hemodynamic parameters (e.g., blood
despite renal hypoperfusion. This is often the case in pressure, pulse rate, and jugular venous pulse), mental
patients with long-standing SCI, where tubular dysfunc- status, and urine output.
tion may be related to neuropathic bladder dysfunction, If a favorable hemodynamic response to isotonic
chronic pyelonephritis, and/or urolithiasis. Prerenal saline infusion does not occur, then colloid fluids or blood
azotemia is further characterized by a disproportional products (in the case of hemorrhagic shock) may be added
increase in blood urea nitrogen (BUN) compared to serum to the resuscitation regimen (2830). The administration
creatinine (i.e., the ratio of BUN/Cr typically exceeds 20). of vasopressors (e.g., dopamine, phenylephrine, epi-
However, this ratio may also be increased in patients with nephrine, and/or norepinephrine) is indicated in patients
chronic SCI because of low serum creatinine values who are refractory to volume resuscitation (31,32). In
related to reduced muscle mass. most cases, hypotension will respond to fluid-volume
Careful attention to the history and physical find- resuscitation; however, because SCI patients are more sus-
ings remains an essential part of the assessment in patients ceptible to pulmonary edema, massive infusions of vol-
presenting with ARF. For example the presence of thirst, ume should generally be avoided (16,32). Therefore, in
dry mouth, lightheadedness, reduced skin turgor, and cases where shock (particularly spinal shock) is refractory
orthostatic symptoms are indicative of intravascular vol- to 2 to 3 liters of volume replacement, vasopressor ther-
ume depletion and would suggest a diagnosis of prerenal apy should be initiated (11,32).
failure. In some critically ill patients, measurement of cen- During the acute-care period following spinal injury,
tral venous (CVP) or pulmonary wedge pressure (PAWP) an indwelling bladder catheter should be placed to facil-
may be necessary in the diagnosis and management of itate urine drainage, because most patients (especially
prerenal failure. Prompt recognition and correction of those with cord lesions above S2) initially develop flacid
prerenal azotemia is important, because renal hypo- bladders subject to overdistention. Hypovolemia should
perfusion predisposes the patient to ischemic parenchy- be prevented or promptly treated, as there is evidence
mal injury and acute tubular necrosis. Additionally, that, in addition to its well-established adverse affects,
hypoperfusion markedly increases the risk of renal injury ischemia may also play an important role in aggravating
associated with nephrotoxic antibiotics, radiocontrast SCI (33,34). Hypoxia, hyperpyrexia, and anemia should
material, septicemia, and rhabdomyolysis (1525). also be treated (O2 saturation and hematocrit should be
Prerenal azotemia is readily reversible with the maintained above 95 percent and 30 percent, respec-
restoration of normal renal perfusion, which is usually tively). A hematocrit in the range of 30 to 34 percent
accomplished with the rapid infusion of isotonic saline. probably provides the best balance of oxygen-carrying
In hypotensive patients, boluses of 250 to 500 ml (0.9 per- capacity and reduced viscosity. Associated electrolyte and
cent saline or Ringers lactate) should be administered acidbase disturbances should be treated with the prior-
under careful observation, with frequent monitoring of ity for correcting multiple concomitant abnormalities as
blood pressure, pulse, cardiopulmonary status, and urine follows: first, volume and perfusion deficits; second, pH;
output. In the absence of congestive heart failure, saline third, potassium, calcium, and magnesium abnormalities;
266 MANAGEMENT OF THE BLADDER, BOWEL, AND SEXUAL DYSFUNCTION
and fourth, sodium and chloride abnormalities (35). It tion of an indwelling bladder catheter should always be
should be noted that restoration of tissue perfusion often ruled out as a possible cause of renal insufficiency. In
results in the improvement of electrolyte and acidbase chronic SCI, the high prevalence of acute and chronic
disturbances. pyelonephritis predisposes patients to papillary necrosis
and subsequent ureteral obstruction caused by sloughed
tissue and purulent debris (8). Another potential cause
Postrenal Azotemia
of obstruction is urolithiasis, which is also quite preva-
Partial or complete urinary tract obstruction should always lent in chronic SCI patients (3739). Factors predispos-
be ruled out in the evaluation of ARF, especially in patients ing to urinary tract stone disease (in SCI) include hyper-
with underlying acute or chronic SCI. The major causes calciuria, hyperuricosuria, and hyperoxaluria (37,
of urinary tract obstruction are listed in Table 22.2. SCI 4042). In addition, urinary tract infection (UTI) (with
patients are particularly prone to urinary tract obstruction, urease-producing bacteria) results in the formation of
with the most important predisposing factor being neuro- urine that is highly alkaline (pH 7.5) and supersatu-
pathic bladder dysfunction (16,36). Essentially, three types rated with NH4, PO43, and CO32. This, in turn, leads
of neuropathic bladder dysfunction occur as a result of to struvite stone formation. Infection stones or struvite
spinal cord injury: a) a hyperreflexic or spastic bladder; stones are very difficult to manage and are often compli-
b) an areflexic or flacid bladder; and c) mixed profiles of cated by pyonephrosis, sepsis, and renal failure (38,43).
bladder dysfunction. For the first several weeks following Other factors that may predispose SCI patients to urinary
SCI, the bladder is usually flaccid and subject to hyper- tract calculous disease include an abnormal urinary flow
distention, which is caused by the temporary loss of all rate and reduced urinary excretion of citrate, pyrophos-
reflex activity below the level of injury. In patients with phate, and orthophosphate, which are major inhibitors
upper motor neuron lesions (above S2), after several weeks of stone formation (37,42,44,45).
of flaccidity, the bladder becomes a low volume, hyper- For obstruction to cause significant azotemia, both
reflexic spastic organ that is very inefficient in emptying kidneys must be affected (assuming the presence of two
because of detrusor dyssynergia and sphincter spasm. In normally functioning kidneys). Therefore, a single lesion
contrast, a permanent state of bladder flaccidity and are- at the level of the urethra or bladder neck can cause ARF,
flexia occurs following injury to the reflex-voiding center whereas, above the level of the bladder, the obstructing
(S2S4), which constitutes a lower motor neuron lesion. lesions must be bilateral to cause ARF. Complete obstruc-
As a consequence of incomplete SCI, or with injury involv- tion of both kidneys is manifested by anuria; however,
ing the conuscauda equina junctions, mixed bladder incomplete obstruction may present with polyuria or with
lesions may occur. For example such combinations as flac- widely fluctuating urinary volumes. It should be noted
cid bladder/spastic internal sphincter, spastic bladder/lax that, because of the associated sensory deficits, acute blad-
external sphincter, or a sensory bladder with no con- der distention is usually asymptomatic in SCI patients;
trol over voiding have been described (16). patients with urinary tract obstruction however, may
Less commonly, obstructive nephropathy may be exhibit hypertension. Moreover, acute bladder distention
caused by traumatic disruption of the ureters, bladder, may lead to autonomic dysreflexia in patients with SCI
or urethra, or related to massive retroperitoneal bleeding. above T7, which characteristically presents as a constel-
In both the acute and chronic phases of SCI, the obstruc- lation of signs and symptoms including severe hyperten-
sion, flushing, diaphoresis, pounding headache, and car-
diac dysrhythmias. In addition, subarachnoid bleeding,
convulsions, coma, and even death may complicate this
TABLE 22.2 syndrome (4648).
Causes of Urinary Tract Obstruction The clinical and laboratory features of obstructive
nephropathy often reflect associated abnormalities in
Intrarenal obstruction renal tubular function (4953). Consequently, the urine
Due to uric acid crystals sodium concentration and fractional excretion of sodium
Due to poorly soluble drugs or their metabolites (FENa) are generally increased ( 20 mEq/L and 1 per-
(e.g., sulfa, acyclovir, and methotrexate) cent, respectively). However, early in acute obstruction,
Extrarenal
the FENa may be quite low ( 1 percent of the filtered
Intraluminal (calculi, purulent debris, sloughed
load), indicative of the normal tubular function that may
papillae, fungal balls, blood clots, tumors, and
strictures) be present during the initial phase of obstructive
Extraluminal (tumors, retroperitoneal hematoma, nephropathy, thus mimicking prerenal disease. Partial
surgical disruption, retroperitoneal or pelvic obstruction may also cause a form of hyperkalemic dis-
abscess, and retroperitoneal fibrosis) tal renal tubular acidosis, which appears to result from
several tubular abnormalities beginning with a defect in
RENAL INSUFFICIENCY IN PATIENTS WITH SPINAL CORD INJURY 267
distal tubular sodium reabsorption (54,55). This, in turn, the anatomy and patency of renal arteries and veins (23).
reduces the ability of the distal tubule and collecting duct In some cases however, antegrade or retrograde pyelo-
to generate sufficient luminal electronegativity, which is graphy may be required to define the site and cause of
necessary for normal tubular potassium and hydrogen ion obstruction. Once the diagnosis of obstruction is estab-
secretion. Hydrogen ion secretion may be further lished, prompt measures should be taken to restore uri-
impaired by diminished collecting duct hydrogen-ATPase nary flow to minimize the occurrence of irrevocable renal
activity, whereas the problem of hyperkalemia may be parenchymal damage. For patients in whom a urethral
compounded by increased collecting duct potassium reab- catheter cannot be placed, suprapubic cystostomy may be
sorption related to enhanced activity of H-K-ATPase (an necessary, whereas lesions obstructing the ureters gener-
enzyme that promotes potassium reabsorption as well as ally require cystoscopy and stent placement. In situations
hydrogen ion secretion) (54). Another consequence of where a stent cannot be passed beyond the ureteral
obstructive uropathy is a vasopressin-resistant urinary obstruction, percutaneous nephrostomy tube placement
concentration defect. This abnormality is primarily and antegrade stent placement can be performed. In addi-
responsible for the polyuria associated with partial tion, appropriate antibiotic therapy should be instituted
obstruction and may also be a contributing factor in post- if UTI is present. Supportive measures, including dialysis,
obstructive diuresis. may be necessary to control severe azotemia, volume
overload, and electrolyte and acidbase disturbances.
A brisk diuresis usually accompanies the relief of
Diagnosis and Management
bilateral obstruction, which is often caused by a combi-
Bladder dysfunction, as well as some degree of urinary nation of factors including volume expansion, increased
obstruction, is present in virtually all SCI patients. Dur- osmotic load, defective tubular transport of salt and
ing the acute phase, placement of an indwelling catheter water, and vasopressin resistance. In the majority of cases,
is usually required to facilitate urinary drainage and to postobstructive diuresis is self-limited (generally lasting
closely monitor urine output. The use of either intermit- several days) and appropriate for the degree of volume
tent bladder catheterization or percutaneous fine-bore expansion and azotemia. However, a severe persistent
suprapubic cystostomy is recommended in the subacute diuresis can occur as a result of tubular dysfunction and
setting (usually lasting 1 to 2 weeks post-injury), because the impaired reabsorption of sodium and water. Conse-
the use of either technique is shown to reduce the risk of quently, these patients are at risk for volume and elec-
UTI (5658). Urinary tract obstruction should be ruled trolyte depletion, including hypokalemia and hypomag-
out in all azotemic patients. On physical examination, a nesemia. The treatment of postobstructive diuresis usually
suprapubic mass may be apparent in patients with blad- necessitates the administration of intravenous fluids. The
der distention. Moreover, the placement of a bladder replacement of volume and electrolyte losses is generally
catheter may be useful in the diagnosis and initial man- initiated by infusing matching volumes of half normal
agement of obstruction (at or below the level of the blad- saline (0.45 percent sodium chloride), to which appro-
der); patients with an indwelling catheter in place should priate quantities of KCl, NaHCO3, and, if necessary,
have the catheter tested for patency. MgSO4 have been added. The volume and composition
Renal ultrasonography is generally the preferred of replacement fluids are subsequently determined by
diagnostic imaging procedure used in evaluating acute monitoring the rate of urine output and from measure-
obstruction, because it is reliable, safe, and readily avail- ments of serum and urine electrolyte concentrations (i.e.,
able (5962). If additional diagnostic information is Na, K, Cl, and Mg). Should hyponatremia develop, fluid
required, other imaging modalities such as intravenous replacement should be changed to normal saline alter-
pyelography, computed tomography (CT), or magnetic nating with half normal saline. However, if hypernatremia
resonance imaging (MRI) can be utilized (63). It should occurs, replacement fluid should be changed to quarter
be noted, however, that administration of intravenous normal saline alternating with half normal saline. With
radiographic contrast can cause ARF in patients with massive diuresis, the measurement of electrolytes should
underlying renal insufficiency. The use of unenhanced be performed at least every 6 hours. Blood pressure,
helical (spiral) CT is a highly sensitive technique that can weight, pulse rate, and cardiopulmonary status should
be used to image the urinary tract from the top of the kid- also be frequently assessed. In addition, serum calcium,
neys to the base of the bladder without the use of radi- phosphorous, and bicarbonate should be monitored,
ographic contrast (63). Moreover, this technique is shown because hypophosphatemia and metabolic acidosis can
to be more sensitive than the combined use of ultrasound complicate postobstructive diuresis.
and plain X-ray (KUB) in identifying the cause of obstruc- At times, it is difficult to differentiate persistent pos-
tion. In addition, spiral CT, as well as Doppler ultra- tobstructive diuresis from that driven by volume expan-
sonography and magnetic resonance angiography sion (i.e., related to excessive fluid administration). In
(MRA), appear to be promising techniques for assessing such cases, it is appropriate to decrease the rate of fluid
268 MANAGEMENT OF THE BLADDER, BOWEL, AND SEXUAL DYSFUNCTION
administration while closely monitoring pulse rate, blood giopathies, and glomerulonephritities)have not been
pressure, urine output, body weight, BUN, creatinine, and included, because they fall outside the scope of this
(if available) central venous pressure (CVP) or pulmonary review. For a comprehensive discussion of ARF, the reader
artery wedge pressure (PAWP). The continuation of is referred to references 23 and 6467).
polyuria in the presence of hypotension, tachycardia, or Typically, a combination of toxic and ischemic
a fall in central pressure (CVP or PAWP) is indicative of insults are operative in the genesis of acute intrinsic renal
persistent postobstructive diuresis, and appropriate vol- failure in any given patient; an example of this would be
ume replacement should be continued in such instances. a septic patient receiving aminoglycosides. Acute renal
In contrast, a reduction in urine output with associated failure caused by ischemia or nephrotoxin exposure is
hemodynamic stability (following a reduction in the rate characteristically associated with histologically demon-
of fluid administration) is indicative of iatrogenic strable tubular epithelial cell necrosis; hence the term,
polyuria. In this latter situation, parenteral fluid replace- ATN. However, other terms, including acute vasomotor
ment should be reduced or discontinued. nephropathy (AVN) and lower nephron nephrosis, have
been used interchangeably with ATN.
Acute Intrinsic Renal Failure
Ischemic Acute Tubular Necrosis (ATN)
Unlike prerenal and postrenal azotemia, acute intrinsic
renal failure is always accompanied by renal parenchy- Although acute tubular necrosis (ATN) and prerenal fail-
mal injury and consequently, the renal failure is not imme- ure are usually described as distinct pathophysiologic
diately reversible upon withdrawal of the offending fac- entities, they can also be viewed as representing opposite
tor or conditions. However, complete or partial recovery extremes of a spectrum of conditions caused by various
may occur following correction or with resolution of the degrees of renal ischemia. For example, mild to moderate
underlying pathophysiologic process causing injury. renal hypoperfusion usually causes prerenal azotemia, a
Acute intrinsic renal failure with acute tubular necrosis condition devoid of any associated renal parenchymal
(ATN) is usually caused by ischemic or nephrotoxic damage; it is also immediately reversible with restora-
injury, whereas acute interstitial nephritis (AIN) is tion of adequate renal perfusion. With more severe or
thought to be the result of an idiosyncratic or hypersen- prolonged renal hypoperfusion, however, incipient ATN
sitivity reaction involving cellular and possibly humeral can occur, where the ability to conserve water is lost. This
mechanisms. A classification of the major causes of acute condition is characterized by nonoliguric renal failure
intrinsic renal failure is provided in Table 22.3. However, (urine output 400 to 500 ml/day) and a low urine
less-common causes of ARFnot specifically relevant to osmolality ( 500 mOsm/kg). Although incipient ATN is
SCI (e.g., vasculitic syndromes, thrombotic microan- not immediately reversible, renal function usually
improves within 2 to 3 days following restoration of renal
perfusion. A further reduction in renal blood flow may
result in established ATN, which is characterized by a
TABLE 22.3 severe reduction in GFR with associated oliguria (urine
Classification of Acute Intrinsic Renal Failure output 400 to 500 ml/day) and marked tubular dys-
function, with an inability to conserve sodium (FENa
Ischemic acute tubular necrosis (ATN) 3 percent). With established ATN, recovery of renal func-
Severe volume depletion: hemorrhage, gastroin- tion generally requires a longer time and is often incom-
testinal losses, urinary losses, cardiovascular plete. The GFR rarely returns to normal (or back to its
collapse, shock baseline level), and tubular function likewise remains to
Sepsis syndrome some extent compromised. The overall prognosisfor
Trauma, rhabdomyolysis
functional recoverydepends on the severity and dura-
Intravascular hemolysis
tion of the ischemic or toxic insult. Finally, extreme states
Nephrotoxic acute tubular necrosis (ATN)
Nephrotoxic antibiotics: aminoglycosides, ampho- of renal hypoperfusion can result in partial or complete
tericin, vancomycin, cephaloridine cortical necrosis. This latter condition generally occurs in
Iodinated radiographic contrast media association with profound shock, overwhelming sepsis,
Anesthetic agents: methoxyfluorane, enflurane and disseminated intravascular coagulation and usually
Acute interstitial nephritis results in irreversible renal failure (23,25). Thus, depend-
Drug-induced ing on severity, renal ischemia can cause a spectrum of
Infections disorders ranging from rapidly reversible prerenal fail-
Autoimmune ure to irreversible cortical necrosis. Furthermore, under-
Idiopathic lying renal hypoperfusion and ischemia increases both the
frequency and severity of toxin-induced renal injury.
RENAL INSUFFICIENCY IN PATIENTS WITH SPINAL CORD INJURY 269
A number of mechanisms have been implicated in ety of granular casts and other tubular debris. However,
the genesis of acute renal failure associated with ischemic in some patients, the urine sediment is relatively inactive;
or toxic insults (23,25,6467). These include: a) tubular therefore, the absence of a dirty sediment does not rule
obstruction by swollen epithelial cells or sloughed tubu- out ATN. The presence of mild proteinuria and microhe-
lar debris; b) back-leak of the filtrate through damaged maturia is also consistent with a diagnosis of ATN. In con-
tubular epithelium, thereby negating glomerular filtra- trast, urine demonstrating heavy proteinuria, hematuria,
tion; c) persistent afferent arteriolar constriction and/or and red blood cell casts is indicative of glomerulonephri-
efferent arteriolar dilatation leading to a fall in glomeru- tis, whereas the presence of pyuria with eosinophiluria is
lar capillary pressure and net filtration (hence the term presumptively diagnostic for acute interstitial nephritis.
acute vasomotor nephropathy); and d) a reduction in Urine that is red, reddish brown, or dark brown suggests
glomerular capillary wall permeability. Several of these the presence of blood, hemoglobin, or myoglobin. How-
mechanisms are generally operative in most cases of ATN. ever, because all three test positive for blood (by orthoto-
The associated histopathologic changes, however, are usu- lidine impregnated strips), further differentiation requires
ally mild and include patchy tubular cell necrosis along microscopic examination of the urine (which will identify
with areas of regeneration, proximal tubule brush border red blood cells). Conversely, the absence of proportional
loss, dilatation of Bowmans space, the presence of tubu- hematuria provides strong presumptive evidence for either
lar casts, interstitial edema, and inflammation. In general, myoglobinuria or hemoglobinuria, whereas more specific
these changes poorly correlate with both the incidence laboratory testing is required to differentiate between
and severity of ATN; consequently renal biopsy is rarely hemoglobin and myoglobin.
used in the diagnosis or prognosis of this disorder. The diagnosis of ATN, therefore, is usually made on
A variety of factors predispose SCI patients to ATN the basis of clinical and laboratory evidence and requires
(5,16,6870). First, there is an increased risk for ischemia the exclusion of other causes of ARF such as prerenal
during the initial post-traumatic period (because of spinal azotemia, postrenal failure, acute interstitial nephritis, renal
shock, hemorrhagic shock, or shock related to third spac- vascular disorders, and rapidly progressive glomeru-
ing of fluid into traumatized tissue). Second, there is an lonephritis (23). Close attention should also be given to the
increased risk for sepsis and septic shock in SCI patients, sequence of events leading to renal failure as well as the clin-
with the major sources for infection being the lungs, uri- ical setting. For example, ARF occurring in association with
nary tract, surgical wounds, decubitus ulcers and sepsis or other forms of shock, nephrotoxin exposure,
osteomyelitis. Third, SCI patients are frequently exposed severe trauma, or surgery is most likely caused by ATN.
to various endogenous and exogenous nephrotoxins. An
example of the former would be myoglobin, released
Drug-Induced Renal Failure
from muscle as a result of traumatic or ischemic muscle
injury, whereas examples of the latter would include Despite their relatively small size, the kidneys receive a
exposure to nephrotoxic antibiotics (e.g., aminoglyco- large portion (approximately 20 to 25 percent) of the car-
sides and amphotericin B) and intravenous radiographic diac output and consequently, the rate of blood flow per
contrast material. gram mass of renal tissue is among the highest of any
body organ. Therefore, not surprisingly, the kidneys play
an important role in the metabolism and excretion of a
Diagnosis and Management
variety of drugs and toxins. However, the kidneys are at
Laboratory tests useful in the diagnosis of ATN are gen- increased risk for toxic injury because of their ability to
erally those indicative of tubular dysfunction that specif- concentrate toxins (in tubular epithelial cells as well as
ically underscore the kidneys inability to appropriately in the medullary interstitium), and their capacity for
reabsorb sodium and water and concentrate urine. There- xenobiotic metabolism (wherein relatively harmless par-
fore (with established oliguric ATN), urine osmolality is ent compounds are transformed into toxic metabolites)
low ( 350 mOsm/kg H2O), urine sodium concentration (23,71,72). The major mechanisms by which pharma-
is increased ( 20 mEq/L), and the fractional excretion ceutical agents cause acute renal failure are listed in Table
of sodium is greater than 1 percent (FENa 1 percent). 22.4. These include a) a reduction in glomerular capillary
However, patients with incipient or mild forms of ATN perfusion pressure, causing prerenal failure or, in extreme
are typically nonoliguric and may or may not demonstrate cases, ischemic ATN; b) a direct toxic effect resulting in
abnormalities in sodium reabsorption. Consequently, tubular epithelial cell injury (i.e., toxin-induced ATN);
measurement of urine osmolality and FENa (in nonolig- c) the triggering of the development of acute interstitial
uric ATN) is of dubious diagnostic value. nephritis; d) through intrarenal vascular injury (i.e.,
The urinalysis in ATN characteristically reveals a caused by drug-induced vasculitis or thrombotic microan-
muddy, brown, so-called dirty sediment, consisting of giopathy); and e) precipitation of intrarenal tubular
tubular epithelial cells, tubular cell casts, as well as a vari- obstruction.
270 MANAGEMENT OF THE BLADDER, BOWEL, AND SEXUAL DYSFUNCTION
AMINOGLYCOSIDE-INDUCED ACUTE TUBULAR and Gault formula, which utilize the serum creatinine
NECROSIS. Aminoglycoside antibiotics are commonly concentration to estimate renal function, require appro-
used in the treatment of serious infections caused by gram- priate modification for use in SCI patients (98,99). This
negative organisms, particularly Pseudomonas. Amino- concept is discussed in more detail under the heading
glycosides are polycations that are only minimally bound Clinical and Laboratory Features.
to serum proteins; consequently, they are freely filtered at Following the administration of aminoglycosides,
the glomeruli. However, because of their attraction and demonstrable increases in serum creatinine concentration
interaction with negatively charged phospholipid residues and corresponding reductions in the glomerular filtration
located on brush border membranes, aminoglycoside rate generally do not occur until after 7 to 10 days of ther-
polycations are favorably positioned to be taken up by apy, unless drug dosage is excessive or risk factors are pre-
proximal tubular cells via pinocytosis (25,72). Once inside sent. It is important to emphasize that patients with amino-
the cell, aminoglycosides bind to subcellular organelles or glycoside-induced ATN are usually nonoliguric, with daily
are taken up by lysosomes, where their accumulation urine output ranging between 500 and 2,000 ml. Conse-
causes lysosomal dysfunction and cellular injury. Clinically, quently, the presence of a normal urine output should
mild to moderate renal insufficiency occurs with the not be regarded as evidence of normal renal function.
parenteral use of these drugs in about 10 percent of treated Instead, daily measurement of BUN and serum creatinine
patients. However, the incidence of severe renal failure should be used to monitor renal function. Patients should
(requiring dialytic therapy) is considerably less. Factors also be monitored for manifestations of aminoglycoside
affecting the risk for aminoglycoside nephrotoxicity tubular toxicity, including renal potassium and magnesium
include (72,9094). wasting, which can result in moderate to severe
hypokalemia and hypomagnesemia (91,93,100).
The daily drug dosage, dosing interval, and duration Those measures that reduce the risk for aminogly-
of treatment (i.e., the risk for nephrotoxicity directly coside nephrotoxicity include:
correlates with the quantity of drug administered,
the frequency of dosing, and duration of treatment). Limiting the use of aminoglycosides to specific indi-
Sequential courses of aminoglycoside therapy cations (i.e., serious gram-negative infections such
(within days or weeks of each other) also increase as P. aeruginosa) and at the first reasonable oppor-
the risk for nephrotoxicity. tunity, changing to a less-nephrotoxic regimen.
The specific aminoglycoside agent used (i.e., based Other antibiotics shown to be effective in the treat-
on clinical trial data, the rank order for nephrotox- ment of serious gram-negative infections include
icity appears to be gentamicin tobramycin third-generation cephalosporins, aztreonam, and
amikacin netilmicin) (9397). imipenem.
Concurrent drug therapy (i.e., the risk for nephro- When forced to use an aminoglycoside, select the
toxicity is increased when aminoglycosides are appropriate agent with the least nephrotoxicity.
administered in conjunction with amphotericin B, Because clinical efficacy is directly related to high
vancomycin, cephalothin, clindamycin, cisplatin, peak serum levels, while toxicity is shown to corre-
furosemide, and intravenous radiocontrast agents). late with high trough levels, we do not recommend
The presence of a coexisting fluid, electrolyte, or dosing more frequently than once a day. Recent
acidbase disturbance (e.g., volume depletion, studies (in patients with normal renal function)
hypokalemia, hypomagnesemia, and/or metabolic show that single daily injections of gentamicin (4 to
acidosis) has been shown to potentiate aminogly- 5 mg/kg/day), netilmicin (5mg/kg/day), and
coside nephrotoxicity. amikacin (15 mg/kg/day) have resulted in a 30 to 50
Additional factors that potentiate risk for amino- percent reduction in the renal cortical concentration
glycoside toxicity are advanced age, female gender, of each respective drug when compared with the
obesity, pre-existing liver disease, and pre-existing administration of the same daily dose by continuous
renal insufficiency. infusion (101,102). For most aminoglycosides, reg-
imens using single (once-a-day) injections have been
Furthermore, it is important to note that estimations associated with a lower incidence of nephrotoxicity
of renal function based on serum creatinine measure- when compared with regimens where the same daily
ments (commonly used in able-bodied patients) usually dose was administered by continuous infusion
overestimate renal function in SCI patients. This is (103105). Moreover, a recent randomized study
because serum creatinine values are often substantially has shown that once-daily dosing of gentamicin (4
lower than expected (for any given level of renal function) mg/kg in patients with normal renal function) was
in SCI patients because of associated reductions in mus- less nephrotoxic and more efficacious than three
cle mass. Consequently, formulas such as the Cockroft daily doses of 1.33 mg/kg (106). Once-daily amino-
272 MANAGEMENT OF THE BLADDER, BOWEL, AND SEXUAL DYSFUNCTION
glycoside regimens are also more cost effective than administration. Examples of amphotericin-induced
divided-dose regimens. In theory, the same loading tubular dysfunction include metabolic acidosis (secondary
dose can be administered regardless of renal func- to distal renal tubular acidosis), hypokalemia and
tion; however, with respect to aminoglycosides, hypomagnesemia (resulting from renal potassium and
smaller loading doses are generally recommended. magnesium wasting), and the loss of urinary concentrating
For example typical loading doses for some of the ability (secondary to nephrogenic diabetes insipidus)
commonly used aminoglycosides are: gentamicin (109,110). An examination of the urinary sediment
(1.7 mg/kg); tobramicin (1.7 mg/kg); amikacin (7.5 typically reveals the presence of microhematuria, pyuria,
mg/kg); and netilmicin (2mg/kg), whereas mainte- and cylindruria. Amphotericin-induced ARF is generally
nance dosing (which can range from every 8 to 24 dependent on the dosage and duration of therapy, being
hours) should be determined by serum trough lev- uncommon when the cumulative dosage is less than 600
els. Monitoring serum peak and trough levels should mg, whereas up to 80 percent of patients exhibit
begin within 48 hours after starting therapy. Peak nephrotoxicity when the cumulative dosage is between 2
aminoglycoside levels, which measure the adequacy and 3 grams (72,93,111113). However, in some patients,
of dosing, should be obtained 30 minutes after the ARF occurs early in the course of therapy as a
completion of IV-infusion or 60 minutes following consequence of severe intrarenal vasoconstriction; this is
IM injection. Trough levels, which gauge drug accu- usually reversible with either dose reduction or
mulation and potential toxicity, should be measured discontinuation of amphotericin. With prolonged
at the end of the dosing interval just prior to the next treatment, renal insufficiency appears to be a consequence
dose. Recommended serum trough levels for the of both vascular dysfunction, and tubular injury, and
commonly used aminoglycosides are: gentamicin (1 recovery is often delayed for weeks or even months.
to 2mg/L); tobramycin (1 to 2mg/L); amikacin (5 to Progressive chronic renal failure, however, is unusual but
10ml/L); and netilmicin (0.5 to 2 mg/L). may occur as a consequence of prolonged therapy or with
It is important to correct any pre-existing or devel- multiple courses of therapy.
oping electrolyte abnormalities (e.g., hypokalemia Additional risk factors for amphotericin nephrotox-
and hypomagnesemia) and maintain an adequate icity include volume depletion, underlying renal insuffi-
state of volume repletion. ciency, and concomitant exposure to other potential
If, possible, avoid exposure to other nephrotoxic nephrotoxins (e.g., diuretics, aminoglycosides, cisplatin,
agents. cyclosporine, radiocontrast agents, and deoxycholate).
Because nephrotoxicity is also a function of accu- Ironically, deoxycholate is the standard vehicle in which
mulative dose, attempts should be made to limit the amphotericin is suspended; consequently, the administra-
duration of therapy with the substitution of less tion of amphotericin in liposomes or with other lipid vehi-
nephrotoxic drugs as soon as it is clinically appro- cles, has been shown to reduce nephrotoxicity without
priate. Although acute renal failure associated with compromising efficacy (114116). Additional measures
aminoglycosides is typically nonoliguric and shown to diminish the risk for amphotericin nephrotoxi-
reversible (following dose reduction or discontinu- city include the maintenance of an adequate state of vol-
ation), the serum creatinine will often continue to ume repletion along with the avoidance of diuretics and/or
increase for several days (up to a week) before reach- intravenous radiocontrast material or nephrotoxic drugs,
ing a plateau and returning toward the baseline such as aminoglycosides. Because of the high potential risk
value. for nephrotoxicity, all patients receiving amphotericin
should undergo frequent monitoring of serum creatinine
AMPHOTERICIN NEPHROTOXICITY. Amphotericin and serum electrolytes, including sodium, chloride, potas-
(a polyene antibiotic frequently used in the treatment of sium, bicarbonate, and magnesium.
systemic fungal infections) is also commonly associated
with nephrotoxicity (72,91,93,107,108). Both the VANCOMYCIN NEPHROTOXICITY. Vancomycin is a
antifungal and nephrotoxic actions of amphotericin are glycopeptide antibiotic that is very effective in treating a
related to its interaction with the lipid components of cell variety of gram-positive infections, especially those
membranes, wherein cholesterolamphotericin complexes resistant to penicillins (i.e., methicillin-resistant
formed in cell membranes serve as aqueous channels that Staphylococcus aureus). Vancomycin is also frequently
allow easy passage of small molecules and ions such as used in the management of pseudomembranous colitis
water, potassium, hydrogen ion, and various other solutes. caused by Clostridium difficile. When it is used alone, the
The resulting nephrotoxicity, which can manifest itself in incidence of vancomycin-induced ARF is about 5 percent
the form of tubular dysfunction or as ARF, is a function (72,117). However, when it is used in combination with
of the daily dosage of amphotericin, the accumulative aminoglycosides, the reported incidence of nephrotoxicity
amount administered, as well as the modality of is between 5 and 35 percent (118). The risk for
RENAL INSUFFICIENCY IN PATIENTS WITH SPINAL CORD INJURY 273
the presence of allergic manifestations is characteristic of It should also be noted that conditions associated
AIN caused by agents other than NSAIDs. There is also with tissue destruction or increased cell turnover (e.g.,
occasional overlap between drug-induced AIN and ATN, rhabdomyolysis or tumor lysis syndrome) can cause
as well as between AIN and hypersensitivity vasculitis. severe hyperuricemia with ensuing crystalluria and ARF
For example, in some cases of drug-induced AIN, infil- (129).
trating inflammatory cells have been shown to inflict Measures shown to reduce the risk for crystalluria-
tubular injury, apparently triggering superimposed ATN induced tubular obstruction include avoiding unneces-
(60,119). In addition, a number of drugs, including sul- sarily high doses of the drug or agent in question; main-
fonamides, thiazides, and allopurinol, have been shown taining an adequate state of hydration and volume
to cause AIN with an associated multiple-organ small- repletion; avoiding uricosuric agents in the presence of
vessel vasculitis that involves the skin, liver, heart, and marked hyperuricemia as well as the use of allopurinol
lungs (90,91,127). (in treatment of hyperuricemia or used as prophylaxis);
Although a definitive diagnosis of AIN generally and alkalinization of the urine if the solubility of the drug
requires a kidney biopsy, the presence of eosinophiluria or agent in question is increased by this measure (i.e., sul-
in a patient with ARF provides strong presumptive evi- fadiazine, methotrexate, and uric acid).
dence for this diagnosis. In either case, the underlying
inciting agent or condition must be identified and
Acute Tubular Necrosis Associated with
removed or effectively treated (i.e., the offending drug dis-
Radiographic Contrast Agents
continued or the infection treated). Following such mea-
sures, improvement in renal function should occur, but ARF is now a well-recognized complication of intravas-
this may be delayed for several weeks. In patients with cularly administered radiographic contrast agents
drug-induced AIN, where renal function has not (72,136139). The majority of contrast agents currently
improved within 3 to 4 weeks (following discontinua- available for intravascular administration consist of var-
tion of the offending drug), a 7- to 14-day course of pred- ious salts of triiodinated benzoic acid. Moreover, because
nisone therapy (1mg/kg/d) may be beneficial in hasten- of their low PK values, these salts maintain their anionic
ing restoration of renal function. Steroid therapy, form in the circulation and, as such, are exclusively dis-
however, is not recommended in the management of tributed within the extracellular fluid compartment. Con-
infection-related AIN. sequently, they are readily excreted by the kidney, thus
generating a substantial tubular osmolar load that may
be a causal factor in the genesis of nephrotoxicity.
CRYSTALLURIA, TUBULAR Although the newer nonionic contrast agents contribute
OBSTRUCTION, AND ARF substantially lower osmolar loads compared to the ionic
agents, it remains unclear whether or not nonionic agents
A final mechanism to be discussed with regard to ARF significantly reduce the risk for radiographic contrast
involves renal tubular obstruction related to crystalluria. nephropathy (140,141).
This occurs when high concentrations of a relatively insol- Although, the risk for ARF (from radiographic con-
uble substance (e.g., uric acid, calcium oxalate, or certain trast material) is very low in the general population
drugs and their metabolites) precipitate out, causing (about 0.1 percent), this risk may be substantially
intrarenal microtubular obstruction (25,12830). Drugs increased in patients with underlying renal failure, dia-
and chemicals reported to cause crystalluria-induced ARF betes mellitus, advanced age, hyperuricemia, multiple
include: myeloma (with associated volume depletion), and in low
cardiac output states (137144). Additional factors
high doses of certain sulfonamides (e.g., sulfadiazine shown to increase ARF risk include the use of large
and sulfamethoxazole) (131,132); amounts of intravenous contrast material, repeated or
high doses of methotrexate (133); multiple exposures to radiocontrast, concomitant expo-
acyclovir (when given in large doses intravenously) sure with other nephrotoxins (e.g., aminoglycosides,
(134); amphotericin, and NSAIDs), as well as a previous history
therapeutic doses of indinavir (a protease inhibitor for radiocontrast nephrotoxicity (137145). Preexisting
used in the treatment of HIV infection) (25); renal disease, however, is probably the most important
uricosuric agents (e.g., probenecid and radiographic risk factor for radiocontrast nephropathy. Furthermore,
contrast agents) when administered in the presence the severity of the underlying renal insufficiency generally
of hyperuricemia; and correlates with the frequency and severity of the super-
massive doses of vitamin C or ethylene glycol inges- imposed component of ARF.
tion, both of which cause oxalate crystalluria (135). Diabetes mellitus (with associated diabetic
nephropathy) and advanced age (accompanied by dimin-
RENAL INSUFFICIENCY IN PATIENTS WITH SPINAL CORD INJURY 275
ished nephron mass) have also been reported to increase The co-administration of other potentially nephro-
the risk for radiocontrast nephropathy (142144). How- toxic agents should be avoided (e.g., NSAIDs and
ever, other studies involving either diabetic or elderly nephrotoxic antibiotics should be discontinued or
patients with normal renal function have failed to demon- avoided, if possible).
strate any predisposition to radiocontrast-induced ARF Dehydrating preparatory measures such as the use
(144146). These conditions per se (i.e., diabetes and old of cathartics and overnight fasting should be
age), therefore, may not represent independent risk fac- avoided, especially in high-risk patients.
tors for contrast nephropathy. Although volume deple- Patients should be maintained in a volume-repleted
tion and low cardiac output states reportedly increase the state prior to, during, and following the adminis-
risk for radiocontrast-induced ARF, in a similar fashion tration of radiographic contrast material.
this risk appears to be only minimally increased in Hyperuricemia should be corrected prior to the
patients with normal renal function, which again empha- administration of radiographic contrast material
sizes the importance of renal function in determining risk. (these agents are uricosuric and ARF can result
However, volume depletion in the presence of renal dis- from intratubular obstruction caused by uric acid
ease, diabetes, or multiple myeloma markedly increases sludging).
the risk for radiocontrast-induced ARF. The quantity of contrast material being administered
The severity of radiocontrast-induced renal impair- should be kept to a minimum and multiple sequen-
ment may vary from that which is mild, transient, and tial studies within the same 2- to 3-day period
nonoliguric to severe oliguric ARF requiring dialysis. Most should be avoided in high-risk patients.
episodes of ARF, however, are reversible and characterized Additional measures reported to reduce the risk for
by nonoliguria. Furthermore, elevations in serum creati- radiocontrast-induced ARF include the use of non-
nine and BUN typically occur within 24 hours of radi- ionic contrast material and prophylactic treatment
ographic contrast exposure, reaching a peak in several days with either a calcium channel blocker or a selective
and returning to baseline within 1 to 2 weeks. In addition, dopamine receptor agonist (e.g., fenoldopam)
an exceedingly high (nonphysiologic) urine specific grav- (72,139141,147,148).
ity ( 1.032), caused by the presence of iodinated radio-
contrast, is often apparent on urinalysis.
Acute Tubular Necrosis Associated with
Although the pathogenesis of radiocontrast
Rhabdomyolysis
nephropathy is not completely understood, the following
mechanisms may be involved: Rhabdomyolysis and myoglobinuria may occur in asso-
ciation with acute SCI as a result of either direct muscle
direct tubular epithelial damage as evidenced by vac- trauma (crush injury) or muscle ischemia (1618). In
uolization, degeneration, and necrosis of tubular addition, a variety of other conditions that may be pre-
cells, which is thought to be mediated through sent in this setting including metabolic disorders, toxic
hyperosmolar-induced oxygen free-radical produc- insults, pressure necrosis, seizures, and hyperpyrexia may
tion (19); cause rhabdomyolysis (17,149152). Moreover, approx-
intrarenal obstruction caused by sloughed tubular imately 33 percent of patients with rhabdomyolysis
cells, proteinaceous casts (i.e., TammHorsfall gly- develop ARF.
coprotein), and uric acid (augmented by the urico- Patients with rhabdomyolysis characteristically pre-
suric effect of radiocontrast agents); and sent with reddish-brown urine that tests markedly posi-
renal vasoconstriction, which generally follows tive for blood (using orthotolidine test strips, which are
an initial period of vasodilation. Although the sensitive to both myoglobin and hemoglobin), whereas
asoconstrictive response is usually transient, lasting microscopic examination of the urinary sediment dis-
only a few minutes, at times it may be persistent closes disproportionately few red blood cells. In addition,
(particularly in the presence of an activated the affected muscles may be painful, tender, and swollen.
reninangiotensin system). Patients with rhabdomyolysis and ARF are also at risk for
pronounced hyperkalemia, hyperuricemia, and hyper-
Radiographic contrast nephrotoxicity can usually be phosphatemia caused by the increased release of potas-
prevented by observing the following points: sium, phosphorus, and nucleoproteins from damaged
muscle. Marked initial hypocalcemia with subsequent
Risk factors should be identified and based on a risk hypercalcemia, the latter occurring during the recovery
versus benefit analysis; alternative diagnostic proce- phase of ATN, has also been reported (153). The hypocal-
dures should be considered and used in those patients cemia may be related to the development of hyperphos-
at increased risk, when deemed appropriate (e.g., phatemia, which promotes precipitation of calcium into
ultrasonography, MRI, and radionuclide scanning). soft tissues. Other putative mechanisms for hypocalcemia
276 MANAGEMENT OF THE BLADDER, BOWEL, AND SEXUAL DYSFUNCTION
include skeletal resistance to the calcemic action of SCI), chronic renal failure was found to be the principal
parathyroid hormone or in some cases, the development cause of death in 45 percent of patients. An additional
of transient parathyroid gland dysfunction per se; remo- report by Borges and Hackler (involving a group of Viet-
bilization of calcium from damaged muscle appears to nam War veterans with SCI) demonstrated a further
be the major mechanism responsible for hypercalcemia reduction in the percentage of deaths from renal causes
(153). The proportional increase in serum creatinine is (20 percent) (158), while in 1982 Price reported a renal-
generally greater in ATN associated with rhabdomyoly- related mortality rate of only 14 percent in a long-term
sis caused by the release of both creatinine and creatine follow-up study (involving a large group of civilian SCI
from the injured muscle. Serum levels of creatine phos- patients) (159). More recent studies continue to demon-
phokinase (CPK), aldolase, aspartate aminotransferase strate this trend. DeVivo et al., through a process of merg-
(AST), and lactate dehydrogenase (LDH) rise dramati- ing survival data (from the National Database, available
cally in patients with rhabdomyolysis as a result of mus- collaborative studies, and the Social Security Adminis-
cle injury. Therefore, the demonstration of high serum lev- tration), have shown that kidney and urinary tract dis-
els of one or more muscle enzymes (i.e., CPK, LDH, AST, eases account for only a small percentage (3.5 percent)
or aldolase) is essential in the diagnosis of rhabdomyol- of all deaths in SCI patients (5,160). However, diseases of
ysis. If positive, the presence of myoglobin in the urine is the urinary system were found to be the most frequent
also diagnostically useful. secondary contributing cause of death in SCI patients.
There are a number of mechanisms by which Much of the progress in the prevention of renal insuffi-
myoglobin has been postulated to evoke ATN ciency and the reduction of urinary tract diseaserelated
(15,25,151,153,154). These include the formation of mortality in SCI patients has been achieved through a
obstructing tubular casts (a process enhanced by acidic combination of intensive patient education, the preven-
urine); by causing intrarenal vasoconstriction (possibly tion and control of infection, the prevention of reflux, and
resulting from the quelching and inactivation of nitric most importantly, special attention to the maintenance of
oxide); and oxidative tubular injury related to iron urine flow and bladder drainage (2,5,160162).
released by the heme protein. Furthermore, other factors
such as intravascular volume depletion (from extensive
Pathogenesis
third spacing of fluid into damaged tissue) and/or hype-
ruricemia may also contribute to ARF in patients with Tubulointerstitial diseases, characterized by progressive
rhabdomyolysis. Regarding the management of rhab- renal insufficiency (with associated pyuria but only mini-
domyolysis, ARF is often preventable with timely imple- mal proteinuria) have been generally described as the pre-
mentation of a forced saline diuresis (25,154,155). There- dominant type of kidney disease occurring in SCI patients.
fore, as soon as possible prior to the development of ATN, However, it has been our experience that moderate to
infusion of normal saline should be started (at 1 to 2 liters heavy proteinuria, indicative of glomerular disease, is fre-
per hour) to attain a slightly expanded intravascular vol- quently present in SCI patients with moderate to advanced
ume and to maintain an increased urine output of 100 renal insufficiency. It would therefore appear that both
to 200 ml/hour. Occasionally, even higher rates of saline tubulointerstitial and glomerular injury are involved in the
infusion are required to compensate for the extensive development of chronic renal failure in patients with long-
third spacing of fluid that can occur in this setting. In crit- standing SCI. This concept is consistent with a growing
ically ill patients, close monitoring of central pressures body of evidence indicating that multiple predisposing fac-
(i.e., CVP and/or PAWP) is extremely useful in fluid man- tors and at least several pathophysiologic processes are
agement. In addition, the infusion of sodium bicarbon- likely involved in the genesis of chronic renal insufficiency
ate to maintain urinary pH at > 7 has been shown to in patients with longstanding SCI. These include chronic
reduce the incidence of ARF (154,155). pyelonephritis, nephrolithiasis, obstructive nephropathy,
reflux nephropathy, amyloidosis, and hypertensive nephro-
sclerosis (2,36,162165).
CHRONIC RENAL INSUFFICIENCY In a study by our group, chronic UTI with pyelo-
nephritis contributed to renal failure in 100 percent of
Chronic renal insufficiency is a serious complication of forty-three SCI patients with end-stage renal disease
longstanding SCI, and it causes substantial morbidity and (166), which is similar to the experience reported by other
mortality in this setting. In a large necropsy series investigators (156). In SCI patients, bacteria may gain
reported by Tribe and Silver in 1969, renal insufficiency easy access to the bladder via an indwelling catheter or
was the primary cause of death in the great majority (75 as a complication of intermittent catheterization. Fecal
percent) of cases studied (156). In a subsequent report contamination, as well as the dampness associated with
published in 1977 by Hackler (157) (involving a cohort absorbent garments, may also increase the frequency and
of World War II and Korean War veterans with traumatic rate of bacterial colonization and subsequent infection.
RENAL INSUFFICIENCY IN PATIENTS WITH SPINAL CORD INJURY 277
Also, in patients with decubitus ulcers, there is often cross by causing urinary obstruction and/or by providing a
infection between infected pressure sores and the urinary nidus for infection, thus prolonging the duration and
tract (166). Moreover, in SCI patients, UTIs are frequently complicating the management of UTI. Sequential studies
perpetuated by impaired urinary drainage, the presence have also revealed significant reductions in renal plasma
of urinary calculi, or the use of indwelling catheters flow in SCI patients following the development of
(2,45,162,165,166). Furthermore, the combination of nephrolithiasis (170). Moreover, infection stones sub-
active infection and functional obstruction often leads to stantially increase the risk for complications such as
vesicoureteral reflux, along with a progressive destructive pyonephrosis and urosepsis in SCI patients.
pyelonephritis. In addition to causing tubulointerstitial The development of amyloidosis is an important
injury and predisposing to pyelonephritis, reflux cause of renal insufficiency in patients with long-stand-
nephropathy has been associated with the development ing SCI (4,163,171). In addition, amyloidosis is a major
of focal glomerulosclerosis (167). This condition gener- cause of heavy proteinuria in this population. According
ally presents with proteinuria and hypertension and may to the results of autopsy studies reported by Tribe and Sil-
cause or contribute to progressive renal failure in SCI ver, 50 percent of patients studied had evidence for renal
patients. The mechanism by which vesicoureteral reflux amyloidosis (156). In a more recent study involving forty-
leads to glomerulosclerosis is thought to involve the devel- three spinal cord injured patients with end-stage renal dis-
opment of glomerular capillary hypertension with sub- ease, we confirmed the presence of renal amyloidosis in
sequent hyperfiltration injury. It should be noted, how- 81 percent (6). The higher incidence of renal amyloido-
ever, that the development of glomerular capillary sis in our patients was thought to be a function of greater
hypertension (in the remaining functioning nephrons) is longevity made possible by the availability of mainte-
nonspecific and appears to be part of a maladaptive nance hemodialysis. Infected pressure sores, osteo-
response to progressive nephron loss irrespective of cause myelitis, and UTIs complicated by pyelonephritis appear
(i.e., reflux, infection, or other causes). to be the major causal factors in the genesis of secondary
Nephrolithiasis is another frequent finding in amyloidosis.
patients with longstanding SCI, occurring most often in As a final consideration, hypertensive nephroscle-
patients with persistent or recurrent UTI; however, a num- rosis may contribute to the progression of renal failure
ber of factors may be involved in the genesis of this abnor- in SCI patients (particularly in those patients with under-
mality (3739,168). Although hypercalciuria caused by lying renal insufficiency) (4,161). This observation is
immobilization is common during the early phase of SCI, based on clinical experience as well as autopsy data and
it is usually absent or less pronounced during the more underscores the importance of maintaining good blood
chronic phases of this condition (169). In fact, urinary cal- pressure control.
cium excretion may be reduced in those patients with
chronic renal impairment because of an associated
Prevention
impairment in vitamin D metabolism or by the develop-
ment of secondary hyperparathyroidism. In addition, we Chronic renal insufficiency is a potentially preventable
have noted substantial hyperoxaluria in a group of spinal complication of SCI. This is evidenced by the steady
cord injured patients with varying degrees of renal insuf- decline in mortality from renal causes, which parallels
ficiency that may be caused by increased intestinal our better understanding of the causal mechanisms
absorption of oxalate occasioned by the associated involved in renal injury as well as improvements in tech-
impairment of bowel motility (41). We have also noted nology and education. Specifically, improvements in the
reductions in urinary citrate in SCI patients, and other diagnosis and management of urinary tract obstruction
investigators have confirmed this observation (42,44). and infection have played a pivotal role in reducing the
Consequently, a reduction in the urinary content of cit- incidence of renal disease and the related morbidity and
rate, which is a potent inhibitor of stone formation, may mortality. This begins with good bladder management,
be a causal factor in the genesis of urolithiasis in this pop- which includes the maintenance of a low voiding pres-
ulation. However, UTI caused by urease-producing sure; the provision of a relatively large-capacity low-pres-
organisms is probably the most important predisposing sure storage system; and prevention of infection (162).
factor with regard to urolithiasis (43,45). Such infections Ideally, good bladder management will be achievable via
can facilitate the formation of struvite stones by provid- reflex voiding. If this is successfully accomplished, post-
ing an abundance of ammonium while also greatly void residual volumes should be less that 100 ml, and
increasing the urinary pH. Urinary stasis, related to func- bladder pressures (during storage and while voiding)
tional obstruction, and the increased excretion of inflam- should be below 35 to 40 cm H2O. In some patients, high
matory debris associated with infection, also facilitate voiding pressures can be ameliorated with the use of
stone formation in SCI patients. The development of either alpha-adrenergic blocking drugs or antispasticity
urolithiasis, in turn, can contribute to renal deterioration agents, whereas the administration of musculotropic
278 MANAGEMENT OF THE BLADDER, BOWEL, AND SEXUAL DYSFUNCTION
relaxants, tricyclic antidepressants, or anticholinergic infected and may be further complicated by the develop-
agents can improve bladder storage capacity (162). When ment of cellulitis or osteomyelitis. Respiratory complica-
reflex voiding is not feasible, the utilization of intermit- tions, however, are the leading cause of mortality in SCI
tent bladder catheterization provides a viable option. A patients, with atelectasis and pneumonia being particu-
clean technique should be used (regarding catheteriza- larly problematic in patients who have high cervical
tion), and bladder overdistention must be avoided lesions (5,6,160). With further progress in the area of
through the use of frequent or timely catheterization. Fur- infectious disease management, both mortality and mor-
thermore, the adjunctive use of medication may be effec- bidity rates should continue to improve in SCI patients.
tive in both increasing bladder capacity and reducing
intravesical pressure (2,162). Placement of a chronic
Clinical and Laboratory Features
indwelling catheter, however, is less desirable because of
the increased risk for UTI, calculous disease, dysreflexia, Progression of renal disease causes not only a reduction
and bladder cancer (2,162,165). In male patients, exter- in excretory function but also results in renal-related meta-
nal sphincterotomy may be useful in lowering the intrav- bolic and endocrine dysfunction. Diminished excretory
esicular pressure and in ameliorating vesicoureteral reflux function is manifested by a reduction in the glomerular fil-
as well as allowing a catheter-free status in patients with tration rate with associated elevations in BUN and serum
detrusorsphincter dyssynergia (162). It is recommended creatinine levels. As noted, because of reduced muscle
that assessment of the urinary tract be performed annu- mass, the magnitude of serum creatinine elevation in SCI
ally to screen for bladder changes, vesicoureteral reflux, patients may be considerably less than in able-bodied indi-
and calculous disease (2,162). This can usually be accom- viduals with comparable renal insufficiency. For the same
plished with a cystogram and ultrasonography or with reason, the serum concentration and urinary excretion of
excretory urography (if renal function is normal). creatinine are proportionately lower in quadriplegics when
Prevention or effective management of urolithiasis compared with paraplegics having comparable levels of
is also very important in SCI patients. Measures that effec- renal function (99). However, it should be noted that the
tively prevent or reduce the frequency and severity of UTIs, measurement of creatinine clearance can be reliably used
as well as treatment designed to minimize obstruction and to assess glomerular filtration rate in SCI patients. This is
maintain satisfactory urine flow, are essential in reducing because the clearance of a substance measures the effi-
urolithiasis risk (2,45,162,168). Such measures are par- ciency of excretion (i.e., the greater the excretion of crea-
ticularly important in preventing the formation of stru- tinine per unit concentration in plasma, the higher the
vite stones, which are the most common type of renal cal- clearance). Consequently, the clearance of creatinine would
culi seen in SCI patients and the most problematic. In not be affected by differences in muscle mass or creatinine
addition, the use of urease inhibitors may prove to be use- production. Calculation of creatinine clearance however,
ful in the management of struvite stones. Patients who requires a timed urine collection (usually for a period of
form calcium oxalate stones may benefit from a reduction 12 or 24 hours) with concurrent measurements of the uri-
in dietary oxalate, improved bowel care, and oral pyri- nary creatinine concentration (Ucr), plasma creatinine con-
doxine supplementation, which decreases endogenous centration (Pcr), and urinary flow rate (V); as well as the
oxalate production. In addition, increasing oral calcium use of the following clearance formula:
intake can reduce oxalate absorption by chelating dietary
oxalate in the intestine. Also, patients should be advised Creatinine clearance UcrV / Pcr
against consuming large quantities of vitamin C, which
acts as an oxalate precursor and increases the production where Ucr and Pcr are generally measured in mg/dl, while
of endogenous oxalate. As a final consideration, the V is measured in ml per minute. However, in SCI patients,
administration of inhibitors of stone formation (e.g., mag- one should avoid using standard formulas or nomograms
nesium supplements and/or potassium citrate) may prove that estimate (rather than measure) creatinine clearance
useful in SCI patients, although, to our knowledge, such and are based on the assumed presence of a normal mus-
therapy has not been evaluated in this setting. cle mass. We compared creatinine clearance measure-
Because secondary amyloidosis is a consequence of ments, obtained from urine and serum collections, with
chronic infection and suppuration, its prevention is pred- those calculated from the serum creatinine concentration
icated on either the prevention or effective management using an equation popularized by Cockroft and Gault
of infection. SCI patients are at high risk for a number (98,99):
of infections, including UTIs with pyelonephritis, that (140 age) wt(kg)
may be further complicated by abscess formation (renal Ccr
72 Scr(mg / dl)
and perinephric) and sepsis. Decubitus ulcers are also
common, having an annual incidence of approximately
23 percent (6,161). Moreover, these lesions often become for men and
RENAL INSUFFICIENCY IN PATIENTS WITH SPINAL CORD INJURY 279
Hematologic Complications ease can occur in patients who are receiving aluminum-
containing compounds (usually in the form of antacids or
One of the most constant and disabling consequences of phosphate binders) (185). Aluminum overload has also
end-stage renal disease is anemia, which is primarily been reported in patients inadvertently dialyzed with alu-
related to erythropoietin deficiency (174). However, minum-contaminated water (186). Aluminum toxicity
impaired iron utilization, a shortened erythrocyte life (which can cause bone disease, dementia, myopathy, and
span, nutritional deficiencies, and blood loss are also anemia) has been substantially curtailed with the use of
involved in the genesis of anemia in chronic renal failure better water purification techniques in dialysis and by the
(175). In a study involving the hematopoietic system in diminished use of aluminum-containing phosphate
end-stage renal disease (ESRD) we found anemia to be binders. The latter has been accomplished by the substi-
of greater severity in SCI patients treated with hemodial- tution of effective nonaluminum containing phosphate
ysis compared to able-bodied hemodialysis patients (176). binders (e.g., calcium carbonate, calcium acetate, and
In addition, we noted a high incidence of amyloid depo- Renagel).
sition in the bone marrow of SCI patients with ESRD, Finally, a peculiar type of amyloidosis has been
which may also be a contributing factor in the develop- described in long-term dialysis patients, where there is
ment of anemia. deposition of amyloid fibrils (containing beta 2-
Another complication of severe renal insufficiency microglobulin) in collagen tissue, particularly in the syn-
is platelet dysfunction, which can predispose to bleeding ovia of joints and tendons, as well as in subchondral bone
problems. In our studies involving coagulation and fibri- (187). The most common clinical manifestations of this
nolytic pathways, patients with longstanding SCI and disease are carpal tunnel syndrome and a destructive
ESRD have revealed numerous abnormalities in both the spondyloarthropathy typically involving the shoulders,
intrinsic and extrinsic pathways (177180). There was knees, hips, and axial skeleton. Characteristic radi-
also an increased prevalence of antithrombin III defi- ographic findings include erosions and defects involving
ciency, along with decreased protein C anticoagulant the margins of affected bone, particularly at tendon inser-
activity (in the presence of increased protein C antigen tion sites, as well as the presence of radiolucent cystic
concentration) (180). In addition, significant alterations lesions within subchondral bone. The pathophysiology of
were noted in the fibrinolytic system, including a marked this dialysis-related amyloidosis involves tissue accumu-
reduction in tissue plasminogen activator activity (180). lation of beta2-microglobulin (a low-molecular weight
Overall the coagulation and fibrinolytic abnormalities protein expressed by nucleated cells), which is normally
observed in this population suggest a predisposition for filtered and degraded by the kidneys. Although beta2-
thrombophilic diathesis. microglobulin is removed with dialysis (up to 400 to 600
mg/wk with high-flux dialysis), accumulation continues
because of the high rate of production (approximately
Disorders of Bone and Mineral Metabolism
1500 mg/wk). Consequently, the only known effective
The kidneys are the principal producer of 1,25(OH)2D3 treatment for this condition is successful kidney
(the biologically active metabolite of vitamin D), which transplantation.
is essential in calcium absorption and normal bone metab-
olism. Advanced renal insufficiency causes 1,25(OH)2D3
Neurologic Consequences
deficiency, which in turn can result in a negative calcium
balance and osteomalacia. Furthermore, the combination Both the central nervous system (CNS) and peripheral
of 1,25(OH)2D3 deficiency, hypocalcemia, and hyper- nervous system (PNS) are affected by uremia. Some of the
phosphatemia (the latter a consequence of reduced renal major CNS manifestations include the reversal of nor-
phosphate excretion) stimulates parathyroid activity, mal sleep patterns, reduction in cognitive function, aster-
which often results in secondary hyperparathyroidism. ixis, confusion, obtundation, and coma. Uremic periph-
Moreover, the complications of secondary hyper- eral neuropathy can involve both sensory and motor
parathyroidism, including high-turnover bone disease, modalities. CNS manifestations readily improve with the
metastatic calcifications, and osteitis fibrosa cystica, sub- institution of adequate dialysis therapy. However, once
stantially contribute to morbidity and mortality in ESRD significant uremic neuropathy develops, the response (if
(181183). Recent studies have shown that risk for any) to dialysis therapy is slow and incomplete. For this
metastatic soft tissue calcification is significantly reason, dialytic therapy should be initiated prior to the
increased when the serum calcium phosphate (Ca P) development of clinically significant peripheral neuropa-
product exceeds 60 to 70 mg2/dL2 (181,184). Bone dis- thy. Because the superimposed neurologic manifestations
ease (in SCI-ESRD) may be further complicated by dem- of uremia can compound and greatly magnify disability
ineralization, related to chronic acidosis and reduced in SCI patients, the early institution of renal replacement
physical activity. In addition, aluminum-related bone dis- therapy should be considered in this setting.
RENAL INSUFFICIENCY IN PATIENTS WITH SPINAL CORD INJURY 281
Cardiovascular and Pulmonary Complications effective in lowering serum homocysteine levels in ESRD
patients (198).
Severe renal insufficiency predisposes patients to fluid Additional measures that may be beneficial in reduc-
overload, congestive heart failure, pulmonary edema, and ing cardiac risk in CRF patients include: early treatment
hypertension (188192). However, a combination of of anemia with erythropoietin targeted to maintain the
dietary fluid and sodium restriction, dialysis, and the use hematocrit between 33 and 36 percent; prevention of
of antihypertensive agents can effectively control these hyperphosphatemia; aggressive treatment of secondary
problems. Nevertheless, the most common cause of death hyperparathyroidism; the timely initiation and mainte-
in CRF patients is cardiovascular disease (i.e., myocardial nance of adequate dialysis; and the maintenance of ade-
infarction, stroke, cardiomyopathy, and congestive heart quate nutrition (192,197199).
failure) (189,193,194). Moreover, in ESRD the relative Pericarditis is generally a late manifestation of
risk for cardiovascular death is approximately 17 times untreated uremia, which can be complicated by cardiac
that of the general population (194). Although not fully tamponade (188,192,199,200). The presence of uremic
understood, the increased risk for cardiovascular disease pericarditis signifies the need for the prompt institution
in CRF patients appears to be multifactoral, involving of dialytic therapy. Patients already receiving adequate
conventional risk factors (e.g., hypertension, dyslipi- dialysis may occasionally develop dialysis-associated peri-
demia, hypercoagulability, sedentary lifestyle, obesity, and carditis that does not appear to be uremic-related. How-
smoking), as well as other causes or risk factors more ever, an assessment of dialysis adequacy is indicated in all
specifically related to CRF (e.g., abnormal calcium and dialysis patients presenting with pericarditis, because an
phosphorous, metabolism, hyperparathyroidism, vascu- inadequate dialysis regimen may be an underlying factor
lar calcification, oxalate retention, hyperhomocystinemia, in the genesis of this condition (197,199,200). Patients
increased carbamylation and glycation of macromole- should also be evaluated for other potential triggering fac-
cules, increased oxidative stress, and endothelial dys- tors or causes of pericarditis including intercurrent infec-
function) (189192). Furthermore, ESRD patients are tion, hyperparathyroidism, hyperuricemia, chronic volume
shown to have a high prevalence of left ventricular hyper- overload, and malnutrition. Additionally, the occurrence
trophy as well as high serum levels of inflammatory medi- of pericarditis may be a manifestation of an underlying sys-
ators (i.e., C-reactive protein), which are also considered temic disease (e.g., systemic lupus erythematosus) or
markers for cardiovascular risk and mortality. Therefore, related to the use of a drug (e.g., minoxidil). Acute peri-
given the very high cardiovascular event rate associated carditis should be suspected in patients who present with
with renal failure, SCI patients with CRF should be placed chest pain and fever; the diagnosis is confirmed by the pres-
in the highest risk stratum with respect to both the man- ence of a pericardial friction rub. Echocardiography is also
agement of risk factors and decisions regarding treatment. useful in detecting and monitoring the progress of peri-
This would include the early detection and control of cardial effusions. It should be noted, however, that small
hypertension, dyslipidemia, and obesity, the avoidance of asymptomatic pericardial effusions (< 100ml) are routinely
tobacco, and, if possible, the implementation of an aero- present on ultrasonography in dialysis patients and prob-
bic exercise program. Because of the increased risk of gas- ably have no clinical relevance. Patients with acute peri-
trointestinal bleeding in patients with CRF, aspirin ther- carditis should be hospitalized and closely monitored for
apy is generally not recommended as primary prevention; arrhythmias, as well as for signs of impending tampon-
however, as secondary prevention (i.e., in patients with ade (e.g., pulsus paradoxus or unexplained dialysis-asso-
established cardiovascular disease) we recommend low- ciated hypotension) (197,200). The management of dial-
dose aspirin (81 to 150 mg daily) unless otherwise con- ysis-associated pericarditis includes:
traindicated. In addition, the use of antioxidants (e.g., vit-
amin E, ascorbic acid, or probucol) may be beneficial in Intensifying the dialysis regimen by increasing the
reducing cardiovascular risk, as evidenced by a recent frequency of dialysis, usually to five treatments per
report showing vitamin E to be effective in reducing LDL- week for a period of 2 to 4 weeks.
oxidation in dialysis patients (195). Again, it should be Avoidance of anticoagulation (to reduce the risk of
noted that when ascorbic acid is administered to CRF tamponade, heparin-free dialysis should be used).
patients, the daily dose should not exceed 100 mg because Avoidance of volume depletion; and patients should
of the risk for increased oxalate production. With regard not be excessively ultrafiltrated below their targeted
to hyperhomocystinemia, there is evidence that treatment dry weight.
with folic acid (1 to 15 mg/d), pyridoxine (50 to 100 Attainment of adequate pain control using either
mg/d), and vitamin B12 (1 mg/d) can lower serum homo- acetaminophen, codeine, hydrocodone, or, in
cysteine levels in CRF patients (196,197). In addition, extreme cases, morphine sulfate. It should be noted
there are recent data indicating that a combination of that neither the use of NSAIDs, systemic steroids,
intravenous folinic acid and pyridoxine may be even more nor intrapericardial steroids has been shown to be
282 MANAGEMENT OF THE BLADDER, BOWEL, AND SEXUAL DYSFUNCTION
tract (212). Also reported in dialysis patients is an patients) may be further aggravated with the development
increased incidence of GI angiodysplasia, a condition that of ESRD as a result of factors such as the implementa-
can cause life-threatening, difficult-to-manage recurrent tion of a renal diet and the related fluid restriction, or
episodes of GI bleeding (213,214). Other conditions that from the use of medications (e.g., calcium-containing
increase the risk for GI bleeding in ESRD include esophagi- phosphate binders, oral iron preparations, and calcium
tis, gastritis, duodenitis, and colitis, as well as the occur- antagonists). To minimize constipation and prevent fecal
rence of single discrete ulcerations, usually located in the impaction, patients should be maintained on a good
cecum or ascending colon (209,215,216). In addition, the bowel management program that ensures regular and
use of medications such as aspirin, NSAIDs, prednisone, complete evacuation. Meals should be given at regular
and oral iron has been associated with upper GI bleeding times, the intake of high-fiber foods encouraged, physi-
in dialysis patients. Furthermore, underlying platelet dys- cal activity should be encouraged, and fluid intake should
function (commonly present in uncontrolled uremia) can be as liberal as possible, considering the severity of renal
reoccur as a consequence of inadequate dialysis and insufficiency and the related volume of urine output. The
should, therefore, be ruled out as a causal factor with use of bulk-forming laxatives and stool softeners (emol-
regard to GI bleeding in ESRD patients. If present, quali- lient laxatives) is also helpful in establishing and regulat-
tative platelet dysfunction (as evidenced by a prolonged ing bowel programs, while the intermittent use of a stim-
Ivy bleeding time) should improve following the restora- ulant laxative may be useful in moving stool that has
tion of adequate dialysis therapy. When indicated, a more impacted proximal to the rectum.
rapid improvement in platelet function (within 1 to 2 An idiopathic form of ascites is associated with renal
hours) can be accomplished with desmopressin (DDAVP) failure and dialysis. However, in patients who develop
administration (0.3 g/kg IV or SC); however, this effect ascites, underlying causes such as liver disease, abdomi-
is of short duration (lasting only 6 to 8 hours) and repeat nal malignancies, heart failure, and constrictive pericardi-
dosing may be ineffective (217). The intravenous admin- tis should be ruled out, because hemodialysis-associated
istration of estrogen (0.6 mg/kg/day for 5 days) may also ascites is a diagnosis of exclusion (223). Although the
be effective in improving abnormal bleeding times (its pathogenesis of this condition is not fully understood, it
onset of action is approximately 6 hours, whereas dura- is thought to result from a combination of chronic fluid-
tion of effect is approximately 30 days) (218). Moreover, volume overload and increased capillary permeability. Sup-
in patients who are at increased risk for bleeding, port for this comes from the observation that most patients
hemodialysis should be performed using a heparin-free diagnosed with hemodialysis-associated ascites have a his-
protocol (219). tory of chronic volume overload and large interdialytic
Of additional concern in able-bodied hemodialysis weight gains as a consequence of dietary indiscretion.
patients is the disproportionately high incidence of acute Because anorexia, progressive malnutrition, and life-
abdominal disease including acute pancreatitis, divertic- threatening cachexia often complicate this condition,
ulitis, spontaneous colonic perforation, bowel infarction, aggressive management is warranted. This includes a com-
and strangulated abdominal wall hernia (211,220222). bination of achieving strict patient compliance regarding
Because SCI is also associated with increased risk for dietary salt and fluid restriction and increasing fluid
acute abdominal disease, it would be logical to assume removal during dialysis with an aggressive regimen of
that SCI-ESRD patients have at least a similar, if not ultrafiltration.
greater, risk for life-threatening acute abdominal disease. Liver disease in dialysis patients may result from
Moreover, because visceral sensation is generally impaired infection with either hepatitis B (HBV) or C (HCV) virus
in SCI patients, many of the signs and symptoms that (224,225). However, because of a combination of factors,
would normally facilitate an early diagnosis of acute including better serologic techniques used in screening
abdominal disease are obscured. For example, after bowel blood products, reduced blood transfusion requirements
perforation, abdominal discomfort may not be apparent in dialysis patients (related to the routine use of erythro-
until inflammation has reached the upper abdominal peri- poietin), and the implementation of vaccination pro-
toneum, which receives innervation from high cervical grams, the incidence of HBV infection has progressively
cord segments. Because early diagnosis of life-threatening declined. Consequently, HCV has now emerged as the
acute abdominal disease is often difficult in SCI patients, most common type of viral hepatitis in hemodialysis
even vague or apparently mild symptomatology must be patients, having a reported prevalence rate (for anti-HCV
taken seriously. positivity) ranging from 1 to 20 percent (225227).
Other GI-related problems reported to occur with Dialysis patients are also at increased risk for drug-
increased frequency in chronic hemodialysis patients induced or toxic hepatitis, which can occur in association
include constipation, ascites, liver disease, and rarely, GI with a variety of different types of drug exposures includ-
disease related to beta2-microglobulin amyloid infiltration ing allopurinol, methyldopa, fluoxymesterone, diazepam,
(208,223226). Constipation (already problematic in SCI ampicillin, indomethacin, and iron dextran (228,229). In
284 MANAGEMENT OF THE BLADDER, BOWEL, AND SEXUAL DYSFUNCTION
patients suspected of having drug-induced hepatitis, dis- pathophysiologic shift in water content from the extracel-
continuation of the offending agent is generally followed lular fluid into the cells is caused by a transient reduction
by improvement in liver function. Iron overload with in extracellular fluid osmolality of sufficient magnitude
hemosiderosis can occur in patients who have received to allow the rapid movement of water into cells along an
large amounts of iron (usually in the form of parenter- osmolar gradient. Apparently, the combination of an
ally administered iron or as a result of multiple blood underlying uremic-related increase in brain osmolality,
transfusions). A presumptive diagnosis of hepatic sidero- caused by an increase in idiogenic osmoles, and a dialy-
sis can be made in patients exhibiting a combination of sis-related rapid reduction in plasma solute content, caused
elevated transaminases (ALT and AST), high serum fer- by the removal of urea and other readily dialyzable solutes,
ritin levels (1000 g/L), and a history compatible with results in the development of a transient, but substantial,
iron overload. Treatment should include the elimination osmolar gradient between the brain and the extracellular
of any exogenous source of iron as well as mobilization fluid compartment. Because the increased brain osmolal-
of iron from tissue stores. The latter can be accomplished ity is related to an increase in idiogenic osmoles and not
with the administration of erythropoietin, wherein the to urea per se, the rapid reduction in plasma urea (with
resulting increase in red blood cell formation and iron uti- high-efficiency dialysis) disproportionately lowers plasma
lization facilitates the mobilization of iron from tissue and extracellular fluid osmolality despite the fact that urea
stores. In severe cases, where serum ferritin levels exceed freely crosses cell membranes and is cleared from both
2000 g/L, the combination of phlebotomy and erythro- brain and plasma at essentially the same rate. DDS can be
poietin administration is shown to be both safe and effi- avoided by prescribing an initial series of low-efficiency
cacious in the management of hepatic siderosis (228). dialysis treatments. When initiating hemodialysis, the fol-
Another consideration is that GI symptomatology lowing is recommended:
may be caused by or related to some aspect of the dialysis
procedure per se (i.e., cytokine release, an electrolyte Avoid exceeding a 30 percent reduction in BUN dur-
imbalance, or possibly a dialysate contaminant). More- ing the initial two-dialysis treatments. This can be
over, the development of GI symptomatology during dial- accomplished by using a small surface area dialyzer,
ysis may be indicative of a potentially serious dialysis- maintaining a relatively low blood flow rate (not to
related complication. For example, the occurrence of exceed 250 ml/min.), and limiting the duration of
nausea or vomiting during hemodialysis is usually an early dialysis to 2 hours.
manifestation of volume depletion (related to excessive Use dialysate with a sodium concentration approx-
ultrafiltration). Consequently, these symptoms often her- imately equal to the patients serum sodium con-
ald the onset of hypotension and are also frequently centration and containing at least 200 ml/dL of glu-
accompanied by weakness, light-headedness, and muscle cose. It is noteworthy that DDS has not been
cramps (230). On the other hand, nausea and vomiting described with peritoneal dialysis (PD). This is prob-
occurring towards the end of a dialysis treatment, or ably a function of the relative inefficiency of PD in
shortly thereafter, may be an early manifestation of the clearing small-molecular weight solutes.
dialysis disequilibrium syndrome (DDS) (231,232). How-
ever, this syndrome primarily occurs in severely azotemic
Nutritional Disorders
patients following the initiation of a hemodialysis regimen
that is overly efficient with regard to the rate and magni- Many patients with ESRD have evidence of protein-
tude of solute removal. DDS is characterized by a con- energy malnutrition as defined by various biochemical
stellation of signs and symptoms ranging from nausea, and anthropometric parameters (233235). Moreover,
vomiting, agitation, and headache in mild cases to stupor, both the prevalence and severity of malnutrition appear
seizures, and coma in severe cases. Although most cases to be even greater in SCI patients with chronic renal fail-
of DDS occur within the first 6 months of hemodialysis ure. This was demonstrated in a study involving dialysis
therapy, this syndrome can occur virtually any time in asso- patients with and without spinal acord injury. The SCI
ciation with a highly efficient dialysis, especially in patients group exhibited a higher incidence of suboptimal dry
who have been previously underdialyzed; for example, body weight along with reduced values (regarding mea-
when a long-duration, high-efficiency hemodialysis treat- surements of mid-arm muscle circumference, triceps skin
ment is performed as compensation for one or more missed fold thickness, serum albumin concentration, serum
treatments. In addition, DDS is more likely to occur in transferrin level, and total lymphocyte counts) when com-
pediatric and elderly dialysis patients as well as in patients pared with the group of able-bodied dialysis patients
undergoing twice-weekly dialysis as opposed to three- (236). A variety of factors are thought to be operative in
times-a-week dialysis. Although its pathogenesis remains the genesis of proteinenergy malnutrition in this popu-
controversial, DDS is thought to result from a rapid lation, including chronic infection, amyloidosis, depres-
increase in brain cell water, which causes brain edema. This sion, anorexia, and prescribed or self-imposed protein
RENAL INSUFFICIENCY IN PATIENTS WITH SPINAL CORD INJURY 285
restriction. In addition, factors related to the dialysis pro- water. Consequently, they are at increased risk for dehy-
cedure per se may adversely affect nutrition. These dration and volume depletion, which can occur as the
include the release of proinflammatory cytokines, an result of increased gastrointestinal fluid losses, increased
increased catabolic effect, and the loss of nutrients that insensible losses, or enhanced renal losses. Conversely,
can occur in association with either hemodialysis or peri- other SCI patients, particularly those with more advanced
toneal dialysis (substantial losses of protein can also occur renal insufficiency, also develop an inability to normally
with peritoneal dialysis). In turn, malnutrition has been excrete sodium and volume, which accordingly places
shown to be an important risk factor for increased mor- them at risk for volume overload and related complica-
bidity and mortality in ESRD patients (184). Conse- tions (e.g., hypertension and pulmonary edema). There-
quently, every effort should be made to maintain a good fore, close attention should be given to the fluid-volume
state of nutrition in SCI patients. and electrolyte status in SCI patients with renal insuffi-
ciency, especially in the event of an intervening illness.
Moreover, when glomerular filtration falls irreversibly
Management
(below 8 to 10 ml/min.), renal replacement therapy
The management of SCI patients with chronic renal dis- should be instituted with hemodialysis, peritoneal dialy-
ease should begin with the identification and correction sis, or possibly renal transplantation. Published data
of any potentially reversible components of renal insuffi- regarding the use of dialysis in SCI patients are still very
ciency, such as urinary obstruction (functional or mechan- limited, whereas information concerning renal trans-
ical), active infection, renal hypoperfusion, and/or severe plantation in this setting is practically nonexistent.
hypertension. In CRF patients with significant residual
renal function (i.e., glomerular filtration rates greater than
Hemodiaylsis
10 ml/min.), every effort should be made to delay further
progression of renal disease. This can be achieved by main- With the development and implementation of dialysis, the
taining satisfactory urinary flow, along with effective treat- mortality associated with acute renal failure has been
ment of urolithiasis and control of infection (e.g., urinary reduced, and death as a direct consequence of ESRD has
tract, pressure wounds, and osteomyelitis). Additional been virtually eliminated. Moreover, the noted beneficial
renal protective measures include the maintenance of nor- effects of dialysis (reported in able-bodied ESRD patients)
mal blood pressure and the avoidance of nephrotoxin are for the most part achievable in SCI-ESRD patients.
exposure. Furthermore, the administration of an However, because of the increased prevalence of pneumo-
angiotensin-converting enzyme inhibitor (ACE-I) or nia, pulmonary embolism, nonischemic heart disease, sep-
angiotensin-II receptor blocking drug (ARB) may be ben- ticemia, amyloidosis, and malnutrition, the mortality rate
eficial in reducing the rate of progression of renal failure in SCI patients on maintenance dialysis is expectedly higher
in selected patients (e.g., those with hypertension and/or than that observed in able-bodied patients (242244).
proteinuria, with mild to moderate renal insufficiency). As in all extracorporeal systems, hemodialysis
These agents can prevent the development of glomerular requires the establishment of an adequate vascular access:
capillary hypertension and hyperfiltration, which is a mal- selection of the specific type and anatomical location of
adaptive process shown to accelerate the progression of the access is generally determined both by the patients
nephron loss in patients with underlying renal disease needs as well as by practicality. For example, a short-term
(237,238). Mild protein restriction (approximately 0.8 or temporary access is appropriate for use in the treat-
g/kg/day with high biologic value protein) has also been ment of potentially reversible acute renal failure or for
shown to reduce glomerular capillary pressure and slow temporary use in an ESRD patient who has a failed per-
the rate of progression of renal disease (239). Although manent access. A number of double-lumen catheters are
both of the aforementioned treatments effectuate renal currently available for use in either the femoral, subcla-
protection by the same putative mechanism (a reduction vian, or jugular vein; however, the internal jugular vein
in glomerular capillary pressure), this is accomplished via is the preferred site for catheter placement. This is because
different actions on the glomerular microcirculation. In the subclavian catheters are associated with an increased risk
case of ACE-Is and ARBs, the reduction in glomerular cap- for subclavian vein stenosis, whereas femoral catheters
illary pressure is the result of efferent arteriolar dilatation, both restrict lower extremity movement and have a high
caused by inhibition of angiotensin II-mediated vasocon- infection rate when left in place for more than 2 days
striction. In protein restriction, however, glomerular cap- (245247). Additional complications shared by all tran-
illary pressure reduction is caused by afferent arteriolar scutaneous catheters include hemorrhage, thrombosis,
vasoconstriction, apparently related to the inhibition of and infection. Furthermore, hemothorax or pneumotho-
vasodilatory prostaglandins (240,241). rax can immediately complicate placement of internal
As previously noted, SCI patients with renal insuf- jugular and subclavian vein catheters; therefore, a post-
ficiency often exhibit an inability to conserve sodium and procedure chest radiograph must be obtained (245,248).
286 MANAGEMENT OF THE BLADDER, BOWEL, AND SEXUAL DYSFUNCTION
A long-term or permanent blood access should be ment, and maintenance of vascular accesses and who are
placed in patients requiring chronic hemodialysis. Cur- experienced in managing the related complications. When
rently, the most commonly used permanent vascular a patient is initially diagnosed with chronic renal failure,
accesses are the arteriovenous (AV) fistula and the arte- every effort should be made to protect the cephalic veins,
riovenous (AV) graft (245,248). These devices are sub- especially in the nondominant arm. This vein should not
cutaneous and may be created or placed in either an upper be subjected to venipuncture or the placement of an
or lower extremity. The preferred site for an AV fistula is indwelling catheter; however, veins in the dorsum of the
the distal (nondominant) forearm, where the anastomo- hand may be used for this purpose.
sis is usually created between the radial artery and
cephalic vein; however, if this is not feasible, upper arm
Hemodialysis Treatment in Spinal Cord Injury
vessels can be used (e.g., an elbow brachiocephalic anas-
tomosis). If it is not possible to create an AV fistula, an Although limited, the clinical experience would indicate
adequate blood access can almost always be established that standard dialysis procedures are generally well tol-
with either an AV graft or a transposed brachial-basilic erated in SCI patients (242244,252,253). However, one
vein fistula (245). The most widely used AV grafts are potentially problematic area regarding hemodialysis in
composed of synthetic material (polytetrafluoroethylene SCI patients involves the type of buffer used (the two
[PTFE]). The currently recommended sites and types of buffers currently in use are bicarbonate and acetate).
AV grafts in order of preference are: a) a forearm curved Acetate, which is a congener of bicarbonate, is metabo-
looped brachial cephalic graft, b) an upper arm straight lized to bicarbonate primarily by skeletal muscle. Con-
graft, c) a forearm straight radial cephalic graft, and d) a sequently, patients with reduced muscle mass (i.e., SCI
looped thigh graft (245,248,249). A final option (for vas- patients) are more susceptible to acetate accumulation
cular access) is the placement of a subcutaneously tun- and toxicity (254). The adverse effects of acetate accu-
neled, cuffed central venous catheter. The use of this type mulation include arterial hypoxemia (related to associ-
of catheter should be reserved for patients who are unsuit- ated hypocapnia and hypoventilation), peripheral vasodi-
able for peritoneal dialysis, and where either the lack of lation, decreased myocardial contractility, metabolic
adequate blood vessels or the presence of complications, acidosis, vasomotor instability, nausea, vomiting,
such as distal limb ischemia, precludes the use of an AV headache, and fatigue. Therefore, in view of the increased
vascular access. potential for acetate accumulation in patients with
Important complications of vascular access devices reduced muscle mass, bicarbonate buffered dialysate
include infection with or without sepsis, access stenosis should be preferentially used in SCI patients (255). For-
or thrombosis, formation of an aneurism or pseudoa- tunately, the vast majority of dialysis facilities currently
neurysm, access rupture with hemorrhage, and limb utilize or have the capability of utilizing bicarbonate
ischemia distal to the access (245,248250). Overall, the buffered dialysate.
preferred type of vascular access is the AV fistula, which A second problematic area concerns the relative bio-
has proven to be the safest and longest lasting type of per- compatibility of the artificial membrane used in hemodial-
manent access. AV fistulas, however, must be created at ysis. Of the four membrane types currently in use (cellu-
least 4 to 6 weeks (preferably 2 to 3 months) before they lose, substituted cellulose, cellulose synthetic, and
are suitable for use in hemodialysis. Furthermore, the size synthetic) the least biocompatible is cellulose (232). Arti-
and condition of the patients peripheral blood vessels can ficial membranes made from unsubstituted cellulose on
limit their application. Although AV (PTFE) grafts have contact with blood can both activate the complement sys-
certain advantages (e.g., the variety of potential place- tem and stimulate the activation of phagocytic cells. This,
ment sites, the ability to bridge distant vessels, and an in turn, can result in numerous adverse effects including
excellent capacity for high blood-flow rates) they are the activation of proinflammatory cytokines, the genera-
overall less desirable than AV fistulas because of a higher tion of reactive oxygen species, cell lysis, hypoxemia, and
incidence of complications, particularly infection, steno- tissue injury (256258). In contrast, dialyzers utilizing
sis, and thrombosis (mainly attributable to the foreign membranes made from substituted cellulose (e.g., cellulose
nature of the AV conduit) (245,248). The subcutaneously acetate) or synthetics (e.g., polyacrylonitrile, polysulfone,
tunneled, cuffed central venous catheter, however, is the polycarbonate, and polyamine) are associated with con-
type of access most frequently complicated by infection; siderably less complement activation. Furthermore, in
the relative risk of bacteremia is reported to be 7.6 per- experimental animal models of ARF, an inverse relation-
cent, compared with AV fistulae (251). ship between the rate of recovery from ARF and the com-
Dialysis centers should have in place a team of plement-activating potential of the dialysis membrane has
trained personnel, including a nephrologist, vascular sur- been recently demonstrated (259,260). Furthermore, stud-
geon, and interventional radiologist, as well as nurses and ies in humans with ARF have demonstrated both a reduc-
technicians who are responsible for the planning, place- tion in mortality, as well as an increased rate in the recov-
RENAL INSUFFICIENCY IN PATIENTS WITH SPINAL CORD INJURY 287
ery of renal function, when dialysis was performed using the practicality and efficacy of peritoneal dialysis in SCI-
synthetic biocompatible membranes compared with dial- ESRD patients are very limited. It has been our experience,
ysis using nonbiocompatible membranes (261,262). however, that long-term IPD is as effective as hemodialy-
Therefore, in the management of ARF, compelling data sis in SCI patients in regard to controlling azotemia, vol-
support the preferential use of biocompatible dialyzers. ume, serum electrolytes, and acidbase balance (253).
Data in chronic hemodialysis patients also indicate that the Moreover, a study of SCI patients treated with IPD exhib-
use of nonbiocompatible dialyzers may adversely affect ited greater hemodynamic stability compared to those
patients by increasing lipid peroxidation and the genera- receiving hemodialysis; however, serum albumin levels
tion of reactive oxygen species (256258). In addition, the were substantially lower in IPD-treated patients, because
use of nonbiocompatible membranes has been shown to of losses from the peritoneal cavity (253). This recognized
cause or aggravate hypoxemia in patients undergoing complication of peritoneal dialysis might compound the
hemodialysis (232,250). One of the proposed mechanisms problem of proteinenergy malnutrition that is frequently
for this involves increased leukocyte sequestration in pul- present in SCI-ESRD patients. In our experience, the inci-
monary capillaries, producing leukocyte microthrom- dence of peritonitis is not increased in SCI-ESRD patients,
bic, which is thought to be mediated via enhanced expres- compared to able-bodied ESRD patients. However, in
sion of leukocyte adhesion molecules. However, it should patients who develop peritonitis, the infecting organism
be noted that dialysis-associated hypoxemia appears to is often concomitantly present in the urine and/or a decu-
be multifactorial, involving a number of mechanisms that bitus ulcer, indicative of cross-contamination.
include: With improvements in peritoneal catheter design,
method of placement, and maintenance, as well as the
hypocapnia-induced compensatory alveolar hypo- implementation of more effective aseptic procedures and
ventilation (observed with acetate-buffered improved catheter connection technology, the overall inci-
dialysate), as a consequence of intradialytic losses of dence of peritonitis has markedly decreased in patients
CO2 ; treated with peritoneal dialysis (263). It is, therefore, our
compensatory hypoventilation caused by alkalemia opinion that CAPD, CCPD, or NIPD can provide a viable
(observed with bicarbonate-buffered dialysate), alternative form of renal replacement therapy in selected
when high concentrations of bicarbonate (35 SCI-ESRD patients. However, there are a number of
mEq/L) are used; and important issues that require careful consideration
histamine-mediated abnormalities that affect venti- regarding the use of peritoneal dialysis in SCI-ESRD
lation and perfusion (resulting from complement- patients.
induced mast cell degranulation) (232,250).
Because protein loss (associated with peritoneal dial-
ysis) may compound pre-existing proteincalorie
Peritoneal Dialysis
malnutrition, the patients nutritional status should
Before the advent of indwelling peritoneal catheters, peri- be carefully evaluated and frequently monitored.
toneal dialysis was used only as a temporary modality in Furthermore, in our opinion, preexisting hypoalbu-
the treatment of acute renal failure. However, during the minemia should be considered a relative con-
past two decades peritoneal dialysis has emerged as one traindication to peritoneal dialysis.
of the major therapeutic modalities for the treatment of The observation that the bacteria infecting or colo-
ESRD (263). Furthermore, several variations of peritoneal nizing pressure sores or the urinary tract are likely
dialysis are now available for use in the management of to contaminate the peritoneal cavity via the PD
renal failure. These include intermittent peritoneal dialy- catheter underscores the importance of meticulous
sis (IPD), continuous cycler-assisted peritoneal dialysis observance of aseptic technique during catheter dis-
(CCPD), and continuous ambulatory peritoneal dialysis connectreconnect procedures (e.g., necessitated by
(CAPD). Because IPD is usually performed at night while dialysate bag exchanges or during hook up with an
the patient is asleep (via an automated delivery system); automated system). In our opinion, the presence of
it is often referred to as nocturnal intermittent peritoneal infected pressure sores is another relative con-
dialysis (NIPD). CCPD is identical to NIPD except that traindication to peritoneal dialysis.
in the former, the patient performs at least one additional A further consideration involves the increased fre-
long-dwell daytime exchange. With CAPD, usually four quency of respiratory diseases, which are currently
intermediate-dwell exchanges (approximately 6 hours per the leading cause of mortality in patients with SCI
exchange) are performed daily, on a continuous basis. (5,160). We have also observed a high incidence of
Although each of these modalities (CAPD, CCPD, acute and chronic pulmonary disorders in SCI
NIPD) has been proved to be effective as renal replacement patients with advanced renal disease (203). Conse-
therapy in able-bodied patients, published data regarding quently, there is understandable concern that instil-
288 MANAGEMENT OF THE BLADDER, BOWEL, AND SEXUAL DYSFUNCTION
lation of large volumes of fluid into the peritoneal lation of dialysate can cause sensations of fullness and
cavity may further compromise both respiratory satiety, with resulting anorexia (263,265). Side effects of
function and the resistance to pulmonary infection. hemodialysis such as nausea, vomiting, fatigue, and
In such circumstances, the use of multiple small-vol- malaise can also contribute to malnutrition (250,265).
ume exchanges (approximately 1 liter) with shorter These symptoms may be related to excessive ultrafiltra-
dwell times (1 to 2 hours), which can be easily tion or to dialysis-associated cytokine release. Limiting
accomplished with a programmable automated interdialytic weight gain to approximately 1 liter (by strict
cycler, may substantially diminish the risk for pul- adherence to dietary salt and fluid restriction) obviates
monary compromise. However, the use of hemodial- the necessity for large-volume or excessive ultrafiltration
ysis (with biocompatible dialyzers) may be more with dialysis. For example, in anuric patients, recom-
appropriate in pulmonary-compromised patients. mended sodium and water restriction is 2 grams and 1
High concentrations of glucose are required to pro- liter per day, respectively. If the patient is being dialyzed
vide the osmotic force for ultrafiltration in peri- with a poorly biocompatible cellulosic membrane dia-
toneal dialysis. Consequently, the absorption of glu- lyzer, changing to a more biocompatible dialyzer may also
cose from the peritoneal cavity can cause improve symptoms such as nausea and anorexia. Some
hyperglycemia during the procedure and can also losses of nutrients invariably occur during dialysis, includ-
occasionally result in delayed reactive hypoglycemia. ing water-soluble vitamins, amino acids, and intact pro-
This issue is of further concern in view of the teins. Using peritoneal dialysis, protein losses average 9
reported high incidence of glucose intolerance grams/day. Protein losses during hemodialysis are negli-
among spinal cord injured patients (264). gible; however, polypeptide and amino acid losses can
Abdominal muscle weakness, impaired intestinal average 10 to 13 grams per treatment (263,265).
motility, and related constipation, which are often In the management of malnutrition, it is important
present in SCI patients, can occasionally interfere to recognize and treat comorbid conditions (e.g., chronic
with peritoneal dialysis efficiency (particularly infection or depression) that are often present in this set-
dialysate drainage). In turn, incomplete drainage of ting. Gastroparesis, which is often related to ANS dys-
dialysate from the peritoneal cavity can cause function in SCI patients, usually responds to metoclo-
abdominal distention and bloating and can also pramide (administered 15 to 30 minutes prior to meals
result in suboptimal solute and water clearances. and at bedtime). The altered sensation of taste in ESRD
Therefore, patients undergoing peritoneal dialysis patients has been attributed to zinc deficiency; conse-
require proper bowel care as well as appropriate quently, this condition may improve with oral zinc sup-
positional changes to optimize dialysate drainage. plementation (20 mg/day). Medications most likely to
cause anorexia, nausea, and dyspepsia in dialysis patients
are the aluminum- or calcium-containing phosphate
Dietary Considerations
binders and the oral iron preparations. The substitution
The primary objective of dietary regimens in SCI patients of Renagel as a phosphate binder or the use of an intra-
receiving dialysis is to provide adequate nutrition while venous iron dextran preparation in place of oral iron are
preventing fluid overload, hypertension, hyperkalemia, effective in minimizing these side effects.
and hyperphosphatemia. Proteinenergy malnutrition is It is also important to recognize that ESRD is asso-
a common complication of CRF, and in turn, a poor nutri- ciated with numerous hormonal and metabolic distur-
tional state predisposes ESRD patients to impaired bances, including: insulin resistance, decreased biologic
wound healing, increased susceptibility to infection, dif- effects of insulin-like growth factors, increased plasma lev-
ficulties with rehabilitation, and increased mortality els of catabolic hormones (e.g., cortisol and glucagon); a
(184,233,236). The cause of malnutrition in CRF is often high prevalence of secondary hyperparathyroidism, defec-
multifactorial, involving medical, psychosocial, economic tive erythropoietin production, chronic metabolic acido-
and, possibly, cultural factors (265). sis, and abnormal carbohydrate, fat, and protein metabo-
Anorexia, which is commonly present in patients lism. Individually, or in combination, these abnormalities
with CRF, may be a manifestation of uremia per se, or are no doubt responsible for a wide variety of uremic man-
caused by other factors associated with ESRD (e.g., an ifestations. In aggregate, they also adversely affect nutri-
intercurrent illness, infection, depression, medications, tional status by inducing a state of hypercatabolism
dialysis, or an altered sensation of taste) (233,234,265). (234,250,265). Moreover, therapeutic modalities that
Anorexia, nausea, and vomiting are well known compli- ameliorate uremic-induced hormonal and metabolic dys-
cations of uremia that should resolve with adequate dial- function also improve nutritional status in ESRD patients.
ysis. However, in certain patients, the dialysis procedure These include adequate dialysis therapy, effective treatment
itself may cause or contribute to anorexia. With peri- of anemia (using erythropoietin), correction of metabolic
toneal dialysis, abdominal distention related to the instil- acidosis, and effective control of hyperparathyroidism
RENAL INSUFFICIENCY IN PATIENTS WITH SPINAL CORD INJURY 289
(265267). Furthermore, there is evidence that the admin- bances in calcium and bone metabolism. For exam-
istration of recombinant human growth hormone and the ple, hyperphosphatemia inhibits the 1-hydroxyla-
use of intradialytic parenteral nutrition can increase pro- tion of vitamin D (exacerbating 1,25 dihydroxy vit-
tein anabolism and improve overall nutritional status in amin D deficiency). Hyperphosphatemia also
malnourished dialysis patients (268,269). stimulates parathyroid hormone (PTH) secretion,
Dietary recommendations for stable chronic dialy- which often leads to the development of secondary
sis patients include the following: hyperparathyroidism. Furthermore, a reduction in
serum ionized calcium, usually resulting from a com-
Protein intake should be approximately 1.2 bination of hyperphosphatemia and vitamin D defi-
g/kg/day, with at least 50 percent being of high bio- ciency, is an additional stimulus for PTH secretion.
logic value. Hyperphosphatemia is also commonly associated
Because dialysis patients have impaired cell energy with metastatic soft tissue calcifications, which is
metabolism, caloric intake for sedentary, nonobese usually seen in patients with high Ca P products
patients should be approximately 35 kcal/kg/day, (i.e., 60 to 70 mg2/dL2). Moreover, mortality rates
with carbohydrate providing 40 to 50 percent of the are shown to be higher in ESRD patients (when Ca
total calories. Higher caloric intakes are required in P products exceed 72 mg2/dL2, or serum phos-
patients who are underweight, in patients who rou- phate levels are 6.6 mg/dL) (181). The control of
tinely engage in strenuous activity, and in hyper- serum phosphate, to levels of 6.5 mg/dL or lower but
catabolic patients (i.e., related to trauma, surgery, or ideally in the range of 4.5 to 5.5 mg/dL, usually
infection). requires a combination of dietary phosphate restric-
Protein and caloric intake should be based not on tion (approximately 800 mg/day), adequate dialy-
the patients actual body weight, but instead on the sis therapy, and the administration of phosphate
average body weight for healthy subjects of the same binders with meals (e.g., calcium acetate, calcium
age, sex, height, and body frame as that of the carbonate, and/or Renagel).
patient. Dialysis patients are also at greater risk for develop-
Sodium and water restriction (in anuric patients ing deficiencies involving water-soluble vitamins and
on hemodialysis) should be 1 to 2 g/day (40 to certain minerals, which may occur as a consequence
80mEq/day) and 1 liter/day, respectively. Compli- of either reduced dietary intake, diminished absorp-
ance with sodium restriction is necessary to control tion, altered metabolism, or from losses related to
excessive thirst, hypervolemia, and hypertension. In dialysis per se. Therefore, the following daily vita-
anuric patients receiving peritoneal dialysis, sodium min/mineral prescription is recommended: folic acid
and water intake can usually be liberalized to 3 to (1 mg); pyridoxine (20 mg); thiamin (30 mg); and
4 g/day (130 to 170mEq/d) and 2 to 2.5 liters/day, zinc (20 mg), plus the usual daily allowance of other
respectively. B vitamins (265,270). Although plasma levels of sele-
Hyperkalemia is a common and potentially life- nium are reported to be low in dialysis patients, the
threatening problem in ESRD patients. Therefore, in effects or possible benefits of administering this
most patients, dietary potassium intake should not important antioxidant have not been studied in this
exceed 2 to 3 g/ day (50 to 75 mEq/day). However, setting. Likewise, the potentially beneficial effects of
in patients with significant residual renal function, vitamin E have not been studied in dialysis patients;
only mild potassium restriction may be necessary. however, there are preliminary data indicating that
Because of the presence of renal failurerelated vit- vitamin E may ameliorate the oxidative damage asso-
amin D deficiency, dietary calcium requirements in ciated with both lipid peroxidation and iron therapy
CRF patients generally exceed the recommended 1 (195). As previously discussed, the administration of
g/day for healthy nonuremic adults. To help achieve vitamin C should be limited to 150 mg/day because
positive calcium balance, a higher dialysate calcium higher doses can cause hyperoxalemia (265). Finally,
concentration (3.5 mEq/L) or calcitriol (vitamin D) the administration of vitamin A should be avoided,
supplementation may be required in some patients. as serum vitamin A levels are generally already ele-
However, it should be noted that the majority of vated in CRF because of the combination of
ESRD patients receive moderate to large quantities decreased renal catabolism and increased serum lev-
of calcium carbonate or calcium acetate as phos- els of retinal A binding protein (265).
phate binders and consequently, hypercalcemia may
be problematic.
Medication and Pharmacologic Considerations
In ESRD, dietary phosphate should be restricted to
0.8 g/day to reduce the risk for hyperphosphatemia, In addition to vitamins, trace elements, and phosphate
which plays a major role in the associated distur- binders, the majority of ESRD patients also require med-
290 MANAGEMENT OF THE BLADDER, BOWEL, AND SEXUAL DYSFUNCTION
ication for the management of anemia and hypertension. treated with iron dextran) a test dose of 0.5 ml of iron
The recommended range of 33 to 36 percent for target dextran (25 mg/ml) is recommended (274). Delayed
hematocrits can be achieved with the use of erythropoi- milder reactions with fever, myalgia, and lymph-
etin, which is generally administered intravenously (thrice adenopathy have also been reported in association with
weekly) with hemodialysis or subcutaneously adminis- iron dextran administration. Furthermore, these reactions
tered (twice weekly) in peritoneal dialysis patients are more common when individual doses exceed 250 mg
(271,272). However, for erythropoietin to be effective, (274). The therapeutic efficacy of iron therapy can be
folate, vitamin B12, and iron stores must be replete. Low evaluated by the hemoglobin/hematocrit response that
serum levels of iron and ferritin and/or a low transferrin usually occurs within 3 to 4 weeks, whereas the serum
saturation value ( 20 percent) are indicative of iron defi- iron, ferritin, and transferrin saturation percentage are
ciency. Iron-deficient patients should be evaluated for useful in monitoring iron stores. However, a period of
blood loss, including occult losses from the GI tract. approximately 3 to 4 weeks is also required (post-iron
However, it should be recognized that hemodialysis therapy) for these tests to accurately reflect iron stores.
patients have an increased risk for iron-deficiency anemia Hypertension in patients with ESRD often has a vol-
because of unavoidable losses of small quantities of blood ume-related component, which may respond to a combi-
with dialysis (retained in the extracorporal circulation). nation of dietary sodium restriction and fluid removal with
In addition to iron deficiency and chronic blood loss, dialysis. However, many ESRD patients with hypertension
other causes of erythropoietin-resistant anemia include also require medication for blood pressure control, and
hemolysis, aluminum intoxication, underlying infection most antihypertensive agents can be safely and effectively
or inflammation, secondary hyperparathyroidism, and used in the management of hypertension in SCI-ESRD
bone marrow disease. In the treatment of iron-deficiency patients. These include calcium channel antagonists,
anemia, oral iron preparations (e.g., ferrous sulfite, fer- angiotensin converting enzyme inhibitors (ACE-Is),
rous fumarate, or ferrous sulfate) may be used in doses angiotensin II receptor blockers (ARBs), alpha-blockers,
providing 100 to 200 mg daily of elemental iron. In beta-blockers, central sympatholytics, and vasodilators.
patients intolerant or refractory to oral iron, intravenous Diuretics, however, are generally ineffective in patients
preparations (e.g., iron dextran, ferric gluconate, or fer- with ESRD, although large doses of loop diuretics (e.g.,
ric hydroxysaccharate) may be used. Intravenous iron furosemide) have been used with some success in the man-
administration is generally required for correction of ane- agement of hypertension and volume overload in selected
mia in hemodialysis patients (caused by the associated ESRD patients (i.e., those with a GFR 5 to 10 ml/min).
increased iron losses), whereas with peritoneal dialysis, SCI patients with advanced renal disease are usually
oral iron therapy is usually effective in correcting anemia. exposed to a wide variety of medications. However,
Currently there are three preparations of intravenous iron because of the pathophysiologic effects of both SCI and
(iron dextran, iron gluconate, and iron sucrose); however, renal insufficiency, the bioavailability, distribution, and
only iron dextran and iron gluconate are approved for use metabolism of pharmacologic agents are often altered.
in the United States. Although both iron gluconate and For example, drug bioavailability can be affected by the
iron sucrose have been successfully used in Europe for a alterations in GI motility and absorption commonly pre-
number of years, only recently has iron gluconate been sent in SCI-ESRD patients. Furthermore, increased gas-
available for use in the United States. In iron-deficient tric alkalinization in uremic patients (resulting from the
patients, a total dose of approximately 1,000 mg of intra- action of gastric urease on urea) may reduce the bioavail-
venous iron will usually replete iron stores. When using ability of agents dependent on acid hydrolysis for absorp-
iron dextran in hemodialysis patients, it is our preference tion (275). Uremia is also associated with alterations in
to administer this in 100-mg infusions (diluted in 50 ml first-pass hepatic metabolism that can have opposing
of normal saline), which is given incrementally over 10 effects on drug bioavailability (276). For example, ure-
consecutive dialysis treatments. When using iron glu- mic-associated inhibition of hepatic biotransformation
conate, we follow the protocol recommended by Nis- could function to increase drug bioavailability, whereas
senson, et al., wherein 125 mg of iron gluconate, diluted uremic-associated reduction in protein binding could
in 200 ml of normal saline, is infused over 2 hours dur- decrease drug bioavailability by increasing the availabil-
ing 8 consecutive hemodialysis treatments (273). When ity of unbound drug for hepatic metabolism.
peritoneal dialysis patients require parenteral iron ther- The apparent volume of drug distribution (a math-
apy for practical reasons, we administer larger individ- ematical construct useful in estimating the drug dose nec-
ual doses of iron dextran (i.e., 250 to 500 mg diluted in essary to achieve therapeutic plasma levels) is also affected
250 to 500 ml of normal saline infused over 30 to 60 min- in SCI-ESRD patients (276,277). For example, drugs that
utes); consequently 1000 mg of iron can be given in 2 to are water soluble or highly protein bound tend to be
4 sessions. Because of the risk for anaphylactic reactions restricted to the extracellular fluid compartment and, con-
(occurring in approximately 0.7 percent of patients sequently, have relatively small volumes of distribution.
RENAL INSUFFICIENCY IN PATIENTS WITH SPINAL CORD INJURY 291
In contrast, lipid-soluble drugs that can readily enter cells Clp Clr Clnr
and tissue have comparatively large volumes of distribu-
tion. Therefore, conditions that result in decreased where Clr and CLnr represent renal and nonrenal clear-
drugprotein binding (e.g., uremia and hypoproteinemia) ances, respectively.
cause the apparent volume of distribution of protein- The renal clearance of a drug is determined not only
bound drugs to increase. The relationship between the by its rate of glomerular filtration but also by the net effect
apparent volume of distribution of a drug and its degree of tubular modulation (i.e., tubular secretion and/or reab-
of binding is demonstrated by the following: sorption of the drug). In general, the kidneys do not read-
ily clear drugs that have a large volume of distribution or
Vd VB VT (FB/FT) are extensively bound in tissue. Likewise, drugs that are
highly protein bound are not cleared very well by glomeru-
where Vd is the apparent volume distribution, VB and VT lar filtration because of their poor filterability; however;
are the actual volumes of blood and water in tissues, their renal excretion may be substantially increased by
respectively, and FB and FT are the fractions of free drug proximal tubular secretion. Obviously, with progressive
in blood and tissue, respectively. In addition, the presence renal failure, the capacity for filtration and tubular secre-
of edema or ascites increases the Vd of water-soluble and tion is lost. Furthermore, because the plasma clearance of
protein-bound drugs, whereas muscle wasting (which is such a wide variety of pharmaceutic agents is affected by
highly prevalent in SCI-ESRD) decreases Vd (277,278). renal failure, the physician must have access to a compre-
Because Vd, after completion of drug absorption, is equal hensive source regarding drug dosing guidelines in renal
to the fractional absorption of the dose (fxD) divided by failure. Fortunately, there are a number of excellent pub-
the plasma concentration (c) of the drug, Vd can also be lications available for this purpose (277280). For drug-
calculated using the following: dose adjustments in SCI patients with renal insufficiency
(assuming the serum creatinine is stable), the GFR can be
fxD
Vd estimated, preferably from measurement of creatinine
c clearance or from the clearance of an exogenously admin-
istered radionuclide (281,282). Alternatively, the GFR can
The most important consideration, however, is that be estimated using the empiric formula developed by
the metabolism and excretion of a wide variety of drugs Cockcroft and Gault, along with the appropriate correc-
are profoundly affected by renal insufficiency per se. tion factors for paraplegia and quadriplegia (98,99):
Although certain drugs are excreted in the urine essen-
tially unaltered, most drugs undergo biotransformation (In males) GFR (140-age in years) (wgt in kg)
prior to their excretion. Hepatic biotransformation, in 72 (serum Cr in mg/dL)
turn, involves a number of reactions including oxidation,
reduction, and hydrolysis, as well as synthetic processes (In females) GFR value for males 0.85
(i.e., conjugation and acetylation) by which the parent
drug or agent is transformed into various metabolites. In (In paraplegia) GFR value for males 0.60
the presence of renal failure, the reaction rates for both
hydrolysis and reduction are reduced, whereas the oxi- (In quadriplegia) GFR value for males 0.40
dation rate of various drugs is accelerated (277,278).
Thus, in addition to its profound influence on drug excre- Regarding the administration of drug loading doses
tion, renal insufficiency can affect drug metabolism via in patients with renal insufficiency, in general, a similar
alterations in hepatic biotransformation. loading-dose equivalent as that administered to patients
Of further consideration is that total body clear- with normal renal function can be safely given in renal fail-
ance or elimination of a drug is determined by the sum- ure in situations that require the rapid achievement of ther-
mation clearance of all involved organ systems (i.e., apeutic drug levels. If the loading dose of the drug is not
renal, hepatic, pulmonary, and others). Also, for conve- known, it can be calculated using the following formula:
nience and practicality, most drug assays are obtained
from plasma, and accordingly, reflect the plasma con- Loading dose Vd IBW Cp
centration of the respective drug rather than total body
concentration. Therefore, both the terminology and the where Vd is the apparent volume of distribution of the
concept of plasma drug clearance should be used in place drug (in liters/kg), IBW refers to the patients ideal body
of total body drug clearance; nevertheless, both terms weight (in kg), and Cp represents the desired steady-state
are frequently used interchangeably. The concept of plasma concentration of the drug. However, in SCI
plasma clearance (Clp) is described by the following patients with reduced muscle mass, we recommend using
expression: the measured body weight (in kg) rather than IBW.
292 MANAGEMENT OF THE BLADDER, BOWEL, AND SEXUAL DYSFUNCTION
There are basically two methods by which mainte- necessitating further dosing adjustments. In fact, sub-
nance drug dosing can be accomplished in renal failure stantial amounts of certain drugs can be removed from
patients. The first is to administer a smaller dose at stan- plasma, especially with hemodialysis. This occurs mainly
dard dose intervals, while the second method is to admin- by diffusion, wherein the drug or its metabolites cross the
ister the standard dose at prolonged intervals. In the first dialysis membrane, from plasma to dialysate, along a
method, the appropriate dose reduction (or fractional concentration gradient. However, a number of factors
dose) can be calculated using the following formula: function to minimize drug removal by dialysis. These
include the molecular size of the drug/metabolite, the
Df t 1/2 normal t 1/2 renal failure degree of protein binding, the apparent volume of drug
distribution, the rate at which the drug/metabolite is
where Df is the fractional dose; t 1/2 normal is the elimi- transported between plasma and other fluid compart-
nation half-life of the drug with normal renal function, ments, and the extent to which the drug/metabolite is
and t 1/2 renal failure is the elimination half-life of the drug eliminated from the body by nonrenal mechanisms. Con-
with renal insufficiency. If it is deemed advantageous to sequently, the removal from plasma of a drug/metabo-
maintain normal dosing intervals (e.g., when administer- lite by hemodialysis is not very efficient when: a) the mol-
ing a drug with a narrow therapeutic range and short ecular mass (i.e., drug/metabolite) exceeds 500 daltons;
plasma half-life), the amount of drug per dose can be esti- b) when protein binding exceeds 90 percent; c) when the
mated by the following expression: apparent volume of distribution is large (i.e., Vd of
drug/metabolite 1 liter/kg body weight); d) when the
Dose in Renal Impairment Normal Dose Df distribution of the drug from plasma to other body com-
partments occurs rapidly; or e) when extensive tissue
However, when prolonged dose intervals are more binding occurs.
advantageous, either for convenience or in cases where The hemodialysis clearance of a drug/metabolite can
the drug has a broad therapeutic range and long plasma be estimated by the following formula:
half-life, the dosing interval can be estimated using the 60
following expression: Cl HD Cl urea
Mwdrug
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1991; 31(12):15841590.
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inferentially basing recommendations regarding the use associated nephrotoxicity. Curr Opin Nephrol Hypertens 1996;
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extrapolated from studies in able-bodied ESRD patients anti-inflammatory drugs. N Engl J Med 1984; 310:563569.
and supported only by limited clinical experience is ques- 21. Kaloyanides GJ. Antibiotic-related nephrotoxicity. Nephrol Dial
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22. Liano F, Pascual J. Epidemiology of acute renal failure: A prospec-
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23 Urologic Management in
Spinal Cord Injury
he annual incidence of spinal cord team, and life-long routine urologic follow-up is essential
the lateral columns of the spinal cord. Efferent fibers to the spinal cord to initiate voiding. A voluntary relax-
from the micturition center connect to the detrusor ation of the external striated urinary sphincter is followed
motor nuclei, located in the gray matter of the second by the opening of the bladder neck, and then reflex con-
through fourth spinal cord roots (6). The S3 and S4 seg- tractrion of the detrusor. The bladder empties with neg-
ments have the primary parasympathetic enervation to ligible residual. Once voiding is complete, bladder pres-
the bladder via the pelvic splanchnic nerve. When acti- sure decreases, urethral pressure increases, and the
vated, the primary role of the parasympathetic enerva- bladder neck closes. External sphincter electromyo-
tion to the bladder is detrusor contraction and bladder graphic activity increases with bladder filling. Because of
emptying. The S2 segment, via the pudendal nerve, pro- the vasoelastic properties of the smooth muscle of the
vides the main enervation for voluntary control of the bladder, large volumes of urine can be accommodated at
external striated urinary sphincter. Sympathetic enerva- low pressure. There should be no leakage of urine dur-
tion to the bladder and sphincter mechanism originates ing the storage phase, and the bladder pressure should
from the thoracolumbar (T12L2) segments of the spinal remain low, without bladder contractions occurring. At
cord and travels via the hypogastric ganglia to synapse a certain point, when it is again appropriate to void, the
in the bladder neck and bladder body. Stimulation of cycle repeats itself.
alpha fibers at the bladder neck and proximal urethra
(smooth muscle urinary sphincter) causes the bladder
neck and proximal urethra to contract, whereas stimu- CHANGES WITH
lation of the beta fibers in the bladder body causes it to SPINAL CORD INJURY
relax. The net effect of sympathetic stimulation is urine
storage (Figure 23.1). SCI can result in significant changes to the function of the
Intracranial lesions above the pons result in blad- urinary bladder and the bladder outlet. Initially, after SCI,
der hyperreflexia, with preserved coordination of the there is often a period of spinal shock when the bladder
bladder and urinary sphincter. Lesions below the pons, is areflexic. During this period, the patient is first man-
but above the conus, will often produce bladder sphinc- aged with an indwelling catheter until medically stable
ter dyssynergia. Lesions at the conus itself can render the and then intermittent catheterization is instituted when
bladder areflexic. possible. The bladders function can be anticipated by the
For micturition to occur in a neurologically intact level of the injury. Lower thoracic and lumbar injuries
individual, signals from the micturition center are sent that involve the conus may injure the parasympathetic
nerve roots to the bladder and render the bladder are-
flexic. Injuries to the spinal cord above the conus fre-
quently result in a bladder that will contract sponta-
neously and result in incontinence. Injuries below the
pons and above the conus are prone to bladder sphincter
dyssynergia. When this occurs, the outlet (external stri-
ated sphincter and or bladder neck) becomes spastic and
closes as the bladder contracts to empty. The net effect is
increased bladder pressure, incomplete bladder emptying,
and, over time, this can lead to vesicoureteral reflux,
hydronephrosis, and renal deterioration. Chronically sus-
tained pressures in the bladder can lead to upper tract
deterioration over time. Identifying and treating blad-
dersphincter dyssynergia, and maintaining low resting
bladder pressures, is an important part of the urologic
management of SCI patients.
Individuals with neurogenic bladder are prone to
bacterial colonization of the urinary tract, especially when
indwelling catheters are used, but even with intermittent
catheterization or reflex voiding. These individuals are
FIGURE 23.1 more susceptible to symptomatic UTIs. Basic urologic
Schematic showing nerve enervation to the bladder for mic- management requires an understanding of when it is
turition and storage. (From Bodner DR. Urologic management appropriate to treat bacteria in the urinary tract and when
of the spinal cord injured patient. In: Resnick MI (ed.), Cur- urinary tract imaging and urodynamics re-evaluation is
rent Trends in Urology, Vol. 4. Baltimore: Williams and necessary to treat recurrent infections or unexplained
Wilkins, 31 [with permission].) autonomic dysreflexia.
UROLOGIC MANAGEMENT IN SPINAL CORD INJURY 301
ied with a multichannel catheter. Profilometry with a pull- formed at a frequency that keeps bladder residual below
through catheter provides a graphic representation of the 400 cc. Individuals are generally started at a 6-hour fre-
lateral pressure along the length of the urethra. The blad- quency of catheterization. A sterile catheterization tech-
der is generally empty for these studies (7). For static ure- nique is performed by nursing personnel, but once an
thral pressure profiles, a triple-lumen catheter is used, with individual is able to perform self-catheterization at
one of the holes meant for sensing pressure positioned in home, a clean technique is employed. Urine should be
the posterior urethra. (The first hole in the catheter is gen- routinely cultured monthly. The bladders of patients on
erally 1 cm from the tip of the catheter, to measure blad- intermittent catheterization, as well as those using
der pressure, and the second hole is placed laterally 10 cm chronic indwelling catheters, will be colonized and cul-
distally, to measure pressure in the urethra.) In patients tures will show bactiuria. Individuals should not rou-
with SCI, increased urethral pressure, along with increased tinely be treated with antibiotics for asymptomatic bac-
EMG activity during a rise in bladder pressure, is consis- tiuria, because overtreating leads to the emergence of
tent with bladder sphincter dyssynergia. resistant bacteria. Those symptoms requiring treatment
include an elevation in temperature, along with consti-
tutional symptoms. An increased number of white blood
Transrectal Linear Ultrasound
cells in the urine (usually greater than 5 to 10 WBC/HPF)
Bladder neck obstruction is easily visualized via tran- is associated with tissue penetration. If an individual has
srectal linear sonography. Simultaneous cystometrics a symptomatic UTI, antibiotic therapy should be insti-
along with transrectal linear ultrasonography, have the tuted based on prior culture and susceptibility testing
advantage over cine radiography of avoiding radiation. while awaiting new cultures (9,10).
Secondary bladder neck obstruction, secondary to a ledge
in patients with SCI has been reported (8). The recogni-
Chronic Indwelling Catheters
tion of this obstruction may be important to explain dif-
ficulties in intermittent catheterization. A coude tip Chronic indwelling catheters have the convenience of
catheter is most effective in this setting. draining the bladder without requiring good hand func-
tion or an attendant to perform intermittent catheteriza-
tion. An indwelling catheter does not have the risk of
BLADDER MANAGEMENT incontinence, as occurs when external condom catheter
falls off. However, indwelling catheters are associated with
Every attempt must be made to achieve a balanced blad- bladder and kidney stones and UTIs. Concretions and cal-
der. In this setting, the bladder contracts at relatively low cification can form on the balloon and break off into the
pressures (less than 60 to 70 cm of water) and voiding bladder, forming a nidus for UTI and bladder stone for-
results in low residuals (approximately 100 cc or less). mation. The urine is colonized with bacteria from chronic
For men, external condom catheters can be considered. catheters. Urethral trauma from pulling on the catheter,
Care in using these devices must be taught to patients to urethral erosion, strictures, bladder fibrosis, and a higher
ensure that the catheters are not applied too tightly and incidence of bladder cancer occur with the use of chronic
are sized properly. Problems can occur with external con- indwelling catheters. Epididymitis and orchitis occur more
dom catheters. If the condom catheter falls off and cloth- frequently in male patients whose neurogenic bladder is
ing becomes wet, it may lead to skin breakdown and pres- managed using a chronic indwelling catheter. Trauma to
sure sores. Catheters that are applied too tightly may lead the urethra from the catheter can result in erosion of the
to strangulation injuries. Individual sensitivity to adhe- urethra, thus leading to traumatic hypospadius formation.
sives used in some of the appliances may cause skin irri- Patients should be encouraged to seek alternative methods
tation and abrasions. of bladder management in an attempt to remain catheter
No external urinary collection devices for women are free. For those individuals with indwelling catheters for
ideal, and alternative therapies include chronic catheters, more than 10 years, yearly cystoscopy should be per-
anticholinergic medication with intermittent catheteriza- formed to rule out bladder tumor occurrence.
tion, bladder augmentation, and posterior sacral rhizo-
tomies. These alternatives are considered when inconti-
nence remains a problem for these individuals. PHARMACOLOGIC MANAGEMENT OF THE
NEUROGENIC BLADDER
Intermittent Catheterization Bladder Sphincter Dyssynergia
Intermittent catheterization is a preferred bladder man- Bladder sphincter dyssynergia is a potential cause of renal
agement technique after acute injury, once the patient is failure in patients with SCI below the level of the mic-
medically stabilized. Catheterization should be per- turition center in the pons and above the conus medularis.
UROLOGIC MANAGEMENT IN SPINAL CORD INJURY 303
The striated sphincter becomes spastic and, instead of pressure (oxybutynin, Ditropan XL, Detrol and Detrol
remaining open during micturition, it may close and block LA, propanthelene) in combination with alpha blockers
off the urinary stream (Figure 23.2). Bladder sphincter (phenoxybenzamine, prazosin, terazosin, dexazosin, tam-
dyssynergia results in high pressure voiding, inefficient sulosin) are used to lower outlet resistance by blocking
bladder emptying, and increased post void residuals. smooth muscle alpha receptors at the bladder neck and
Unrecognized, this can lead to bladder hypertrophy, vesi- proximal urethra. Depending on the clinical setting and
coureteral reflux, and upper tract deterioration. Urody- urodynamic findings, pharmacologic management may
namics should be performed shortly after SCI once the be used in combination with reflex voiding or intermit-
patient is voiding spontaneously or within 3 months of tent catheterization. When intermittent catheterization
injury to identify those at risk for bladder sphincter is used, anticholinergics are added in an attempt to keep
dyssynergia. Silent hydronephrosis has been reported as the individual dry between catheterization.
early as 6 months after SCI in patients who were not yet
voiding and thought to still be in spinal shock.
Surgical Management of
Bladder Sphincter Dyssynergia
Pharmacologic Management of
When pharmacologic management is insufficient or not
Bladder Sphincter Dyssnergia
tolerated, and in patients with limited hand function,
The primary goal in the urologic management of the SCI transurethral sphincterotomy is considered (11). The
patient is to maintain renal function. It has been shown sphincter can be cut with electrocautery at the 12 oclock
that sustained high pressures in the bladder leads to upper position to make the sphincter incompetent. Complica-
tract damage. Bladder-relaxant drugs (anticholinergic, tions of the procedure include bleeding, incomplete
antimuscarinic, antisposmidic) are used to decrease blad- destruction of the sphincter, and stricture formation.
der voiding pressure, reduce or eliminate reflex inconti- Impotence was observed more frequently when the
nence, reduce the frequency of intermittent catheteriza- sphincter was cut at the 5 and 7 oclock position. Today,
tion, keep an individual dry between catheterizations, and impotence following transurethral sphincterotomy occurs
decrease uninhibited contractions in individuals with only in a small percentage of patients. Following sphinc-
chronic indwelling catheters. terotomy, the patient uses an external condom catheter,
The main line in medical treatment of bladder and the risk for upper tract deterioration is lessened.
sphincter dyssynergia are medications to lower detursor Excellent long-term results have been reported using con-
tact laser surgery for sphincteromy, with few long-term
complications and a low reoperation rate (12).
As an alternative to sphincterotomy, the UroLume
prosthesis, a braided wire mesh stent, can be implanted in
the urethra to hold the external sphincter open (Figure
23.3). The surgery required to place the stent is minimally
FIGURE 23.2
FIGURE 23.3
Voiding cystourethrogram consistent with detrusorexternal
sphincter dyssynergia. Note the closed external sphincter and UroLume stent. This is placed by cystoscopy to keep the
dilated prostatic urethra. external urinary sphincter open.
304 MANAGEMENT OF THE BLADDER, BOWEL, AND SEXUAL DYSFUNCTION
invasive and reversible. Migration of the stent is possible implanted in Europe and around the world. The first
until it is well epithelialized. Complications of stricture, Finetech-Brindley anterior sacral root stimulator was
significant bleeding, and impotence are avoided (13). implanted in the United States at the Cleveland VA in
1992. Since that time, more than 50 patients in the United
States with SCI have had the device implanted. UTIs have
Anterior Sacral Root Stimulation
decreased and upper tracts have been preserved using this
for Bladder Emptying
technique. A lamenectomy is required. Vesicoureteral
Electrical stimulation of the anterior sacral roots is an reflex is improved or eliminated in nearly all patients
effective alternative for producing micturition on demand implanted with the Vocare device.
with low residuals in individuals with suprasacral spinal For small fibrotic bladders, augmentation cysto-
cord injuries (14). Posterior sacral rhizotomy abolishes plasty can be performed to increase bladder capacity and
reflex incontinence and increases bladder capacity and decrease bladder storage pressure. Individuals who are
compliance. Bladder sphincter dyssynergia and auto- considered for this procedure should have adequate hand
nomic dysreflexia originating from a bladder etiology are function to perform intermittent catheterization of the
eliminated. Posterior sacral rhizotomy abolishes reflex augmented bladder, if required.
erection and ejaculation in the male, along with any pre-
served sensation, and potential candidates should be
counseled in this regard. Urination is achieved with post AUTONOMIC DYSREFLEXIA
stimulus voiding (Figure 23.4). The bladder smooth mus-
cle and striated urethral sphincter contract with electrical A chapter on the urologic management of SCI patients is
stimulation. When the stimulation is turned off, the exter- not complete without a discussion of autonomic dysre-
nal sphincter relaxes more quickly than the bladder and flexia. Patients with SCI at T6 and above are prone to
post stimulus voiding is achieved. By fine tuning the stim- autonomic dysreflexia, which is characterized by unop-
ulating parameters of bursts and gaps, the controller is posed sympathic discharge that results in severe hyper-
individualized to optimize voiding for each patient. tension, sweating, piloerection, and bradycardia. Lesions
Ideal candidates for anterior sacral stimulation are above T5 lead to sympathetic denervation of the splanch-
female SCI patients who are incontinent despite medica- nic bed, which is supplied by the greater splanchnic nerve.
tion and can self-transfer because they have to the poten- Visceral activity, such as a distended bladder or full rec-
tial to be continent and without a loss of erections or ejac- tum, produces peripheral vasoconstriction that is nor-
ulation. The posterior sacral rhizotomy renders them mally countered by vasodilation in the splanchnic bed
continent, and the bladder is then emptied with electri- through the greater splanchnic nerve. The baroreceptors
cal stimulation. The Vocare (Finetech-Brindley) anterior in the carotid body sense the hypertenison and attempt to
sacral root stimulator (Figure 23.5) has been extensively
FIGURE 23.4
FIGURE 23.5
Vocare anterior sacral root stimulation producing post stim-
ulus voiding. The voiding pattern is interrupted, with urina- The componets of the Vocare anterior sacral root stimulator,
tion occurring during the gaps in stimulation. showing the implanted receiver and extradural electordes.
UROLOGIC MANAGEMENT IN SPINAL CORD INJURY 305
compensate by stimulating the vagus nerve, which leads care. Finding a bladder management that is best accepted
to bradycardia. Treatment consists of elevating the bed and least offensive to an individual will have the greatest
and eliminating the noxious stimulus. If the bladder is dis- likelihood of success.
tended, draining the bladder or irrigating a plugged
catheter is often sufficient to stop the reflex. Sublingual References
niphedipine helps to reduce the blood pressure when
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reflexia occurs during procedures in the operating room, nam paraplegic. J Urol 1972; 108:558.
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of Alabama Press, 2000.
up every 6 to 12 months helps to ensure that renal func- 6. Bradley WE, Teague CT. Spinal cord organization of micturition
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such as recurrent bladder infections, autonomic dysre- for antibiotic resistant organisms in patients with spinal cord
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11. Perkash I. Detrusor-sphincter dyssynergia and dyssynergia
sidered. Bladder management should be individualized to responses: Recognition and rationale for early modified
optimize the patients personal preference and well-being. transurethral sphincterotomy in complete spinal cord injury
Reflex voiding with an external collection device may not lesions. J Urol 1978; 120:469.
12. Perkash I. Contact laser sphincterotomy: Further experiences and
be the best choice for a young paraplegic male who wants longer follow-up. Spinal Cord 1996; 34:227.
to wear short pants during the summer and have no col- 13. Chancellor MB, et al. Sphincteric stent versus external sphinc-
lection device on his leg. In this setting, intermittent terotomy in spinal cord injured med: Prospective randomized mul-
ticenter trial. J Urol 1999; 161:1893.
catheterization and anticholinergics may be preferred. 14. Creasey G. Restoration of bladder, bowel and sexual function.
Preserving renal function is the primary goal of urologic Topics in Spinal Cord Injury Rehabilitation 1999; 5(1):2132.
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24 Urolithiasis in
Spinal Cord Disorders
he spinal cord injured (SCI) patient including urosepsis, renal failure, and stone disease
allowed prompt and accurate diagnosis (68). These stone disease. The dramatic decrease in incidence can be
advancements improved the care of both SCI and ambu- attributed to earlier detection, better radiographic tools,
latory patients with stones. The impact of these advances preventive medical measures, improved antibiotics, and
can be seen by a shift in the presentation of SCI patients the development of new and less-invasive surgical tech-
with advanced stone disease with renal deterioration, niques for emergent drainage and removal of stones (2).
renal failure, and high mortality to patients with survival
rates approaching that of the ambulatory population (2).
RISK FACTORS
NATURAL HISTORY AND EPIDEMIOLOGY The high cost of managing SCI patients with stones has
led to an emphasis on early identification and elimination
The estimated annual incidence of SCIs in the United of risk factors leading to stone disease. Risk factors
States is 40 per million, which is extrapolated to 10,000 include impaired bladder function, alkaline urine, the
SCIs per year (9). Approximately 200,000 people in level and completeness of injury, chronic indwelling
America are living with SCI. The average age of injury is catheterization, and chronic UTIs (see Table 24.1).
31 years (9). Although the total number of patients is low, Impaired bladder function is a risk for the develop-
the number of years of impairment is high. The male to ment of both bladder and kidney stones. Neuropathic
female ratio is 4:1 (9). Motor vehicle accidents account bladders can lead to incomplete emptying, high-pressure
for the majority of post-war SCI, followed by acts of vio- bladder storage and emptying, vesicoureteral reflux, and
lence, falls, and injuries from recreational sports. The hydronephrosis (11). Poor bladder emptying causes uri-
surge in SCI caused by violence over the past two decades nary stasis, bacterial colonization, and infection. Urody-
has primarily been caused by the wide availability of guns namics can identify those patients at risk for bladder dys-
and the increased prevalence of inner-city violence. function and allow the initiation of early bladder
The incidence of stone formation in the general pop- management. Chronic indwelling catheter management
ulation is 0.4 percent, with an incidence of 0.6 and 0.18 of the bladder carries an increased risk of stone forma-
percent in men and women, respectively (10). The preva- tion and renal deterioration (17). Patients with indwelling
lence is 10.1 percent among males and 5.8 percent among catheters and condom catheters are at a greater risk for
females (10). The average recurrence rate is 30 to 40 per- clinically significant UTIs than those using clean inter-
cent, with 30 percent of stone formers having more than mittent catheterization.
three recurrences (10). The first data available for the SCI Alkaline urine predisposes to the formation of mag-
population were obtained from longitudinal follow-up data nesium ammonium phosphate and calcium phosphate
from the post-war veterans. This included information stones. Infections produced by urease-producing organ-
from World War I, World War II, the Korean War, and the isms create alkaline urine. Magnesium ammonium phos-
Vietnam War (11). The survival rate of SCI patients after phate and calcium phosphate stones form in alkaline
World War I was 10 percent and increased to 80 percent urine because of their high pKa value (18).
after World War II (11). The incidence of stones in World The level and the completeness of the SCI affect
War II and Korean War veterans, at 20- and 25-year fol- stone development. The higher and the greater the degree
low-up, was 22 percent, and the incidence of stones in SCI of completeness of the lesion, the more likely is stone for-
Vietnam War veterans was 5 percent at 5-year follow-up. mation (15). The use of chronic indwelling catheters (i.e.,
Today, the incidence of stone disease in SCI patients urethral Foley and/or suprapubic tube urinary diversion)
ranges from 5 to 10 percent, with an incidence of 1.5 per- is not uncommon in those patients who do not have use
cent at 1 year and 1.9 percent at 2 years following the of their hands and fingers. However, these catheters may
injury (12). There is a 53 percent chance of SCI patients potentiate stone development. It is not surprising that a
developing urinary stones 10 years after injury, and a 64 patient with a suprasacral cord lesion has a 67 percent
percent recurrence rate (11). Struvite stones (magnesium chance of developing a stone (15).
ammonium phosphate) comprise 90 percent of these
stones, and 50 percent of these form staghorn calculi
(13,14). Staghorn calculi are of greatest concern because,
TABLE 24.1
by historical reports, 50 percent of these patients will lose
Risk Factors for Stones
the involved kidney (15). Bilateral staghorn calculi have
been associated with a reported mortality of 40 to 60
Alkaline urine
percent (15,16). Recurrent urinary tract infections
Although the incidence of urolithiasis in the SCI Neurogenic bladder
population has decreased over the past 50 years, there is Level and extent of injury
still significant morbidity and mortality associated with
UROLITHIASIS IN SPINAL CORD DISORDERS 309
Hyperoxaluria
TABLE 24.3
Stone Classifications Hyperoxaluria leads to calcium oxalate formation by
increasing the urinary saturation of calcium oxalate (21).
COMPOSITION MORPHOLOGY LOCATION The primary hyperoxaluria is caused by an autosomal
recessive disorder in oxalate biosynthesis that causes an
Calcium Partial staghorn Kidney increase in the hepatic production of oxalate. Secondary
Uric Acid Complete staghorn Bladder hyperoxaluria, enteric oxaluria, is caused by intestinal
Cystine Calyceal hyperabsorption of oxalate (21). Only this condition is
Struvite Ureteropelvic clinically significant in the SCI population. It is usually
Matrix junction
accounted for by a metabolic defect that is coincidental
Other
with the SCI injury and is caused by intestinal malab-
sorption of bile acids and fats. The bile acids and fats
within the lumen of the bowel bind and reduce the
Calcium Stones amount of free calcium, leaving an increased amount of
oxalate to be absorbed from the intestine and excreted
Calcium stones are heterogenous in composition and
in the urine. Oxalate complexes with calcium within the
pathophysiology (21). Calcium oxalate and calcium phos-
renal tubules, precipitates, and aggregates to form stones.
phate are the most frequently encountered compounds.
Hypercalciuria, hypercalcemia, and hyperoxaluria are
three common metabolic disorders that lead to calcium Uric Acid Stones
stone formation.
Uric acid stones are produced in acidic urine (i.e., pH below
5.5). The pH is less than the dissociation constant of uric
Hypercalciuria acid in acidic urine, thus causing an increase in undissoci-
ated uric acid and leading to uric acid stone formation.
This common metabolic abnormality is encountered in 60
Gout, myeloproliferative disorders, and chemotherapy are
percent of stone formers in the ambulatory population (21)
common causes of these stones. They are radiolucent on
and also occurs in the SCI population. There are several
plain X-ray, but can be visualized on CT scan. Once again,
metabolic causes of hypercalciuria that lead to stone for-
this metabolic abnormality is usually coincidental with SCI,
mation. Absorptive hypercalciuria is caused by an increased
but may be of clinical significance.
intestinal calcium absorption. This causes an increase in
renal filtered calcium and produces hypercalciuria. Another
problem often encountered is that renal calcium can pro- Cystine Stones
duce a hypocalcemiac state, which causes a secondary
An autosomal recessive disorder causes the abnormal
hyperparathyroidism. Parathyroid hormone causes intesti-
transport of cystine, ornithine, lysine, and arginine. This
nal absorption and bone resorption of calcium, which leads
defect in cystine transport causes cystinuria. Cystine crys-
to further calcium wasting and hypercalciuria. Resorptive
tals are usually hexagonal in shape. They are generally
hypercalciuria is caused by excessive bone resorption of cal-
present in young children and appear as multiple stones
cium, primarily from hyperparathyroidism. A renal phos-
or a staghorn calculus on radiography. They appear as a
phate leak creates hyperphosphaturia, which can produce
coincidental abnormality with the SCI patient.
the excess 1,25-(OH)2-vitamin D that produces hypercal-
ciuria(21). Citrate is the most important inhibitor of cal-
cium binding to oxalate and phosphate, because citrate Struvite Stones
complexes with calcium ions in the urine. Metabolic aci-
Struvite stones are infectious stones composed of mag-
dosis and hypokalemia, not uncommon in clinical presen-
nesium ammonium phosphate. Struvite stones are the
tations, can cause hypocitriuria (21).
most common stones seen in the SCI population, and they
Hypercalcemia produces a 33 percent chance of
are caused by urease-producing bacteria, of which the
developing a renal stone (22). Hypercalcemia is caused
most common are Proteus, Pseudomonas, Klebsiella,
by hyperparathyroidism, sarcoidosis, steroids, malig-
Providentia, E. coli, Staphylococcus epidermis, and Ure-
nancy, idiopathic causes, and immobilization (23). Immo-
aplasm (18). Urease catalyzes the breakdown of urea and
bilization is the second most common cause of stone dis-
water to ammonium and bicarbonate, thus leading to
ease in the SCI population. Immobilization causes the
very alkaline urine:
increased bone resorption of calcium which, in turn,
increases renal filtration. This creates hypercalciuria and urease
the precipitation of calcium oxalate and calcium phos-
phate stones. NH2-CO-NH2 H2O 2NH4 HCO3
UROLITHIASIS IN SPINAL CORD DISORDERS 311
calyces, the ureteropelvic junction, at the ureter where it blood cells (WBC), red blood cells (RBC), or bacteria in
crosses the iliac vessels, and at the ureterovesical junction. the urine are also important in prioritizing intervention
Each of these location have a typical referred pain such and/or diagnostics, because infection and obstruction
as flank, side, lower quadrant, and testicle or labia respec- place the SCI patient at risk for urosepsis and mandate
tively. The ambulatory patient usually complains of the immediate drainage of the obstruction. Hematuria also
inability to find a comfortable position. Gross hematuria suggests that a stone is present.
and/or UTI may also coexist.
However, patients with sensory deficits from SCI
Imaging
have subtle symptoms (2). These patients may or may not
have pain, or present with nausea and vomiting, which can The goal of imaging is to identify pathology (i.e., stone),
be mistaken for gastrointestinal (GIT) disease (2). They evaluate the size and location of the stone, and evaluate
may have bactiuria and UTIs that are refractory to antibi- the presence and degree of obstruction (18). Several radi-
otics (2). The refractory UTI is the most common presen- ographic tools are commonly used to diagnose and treat
tation (11) and symptoms may include fever, tachycardia, urolithiasis (Table 24.4).
hypotension, tachypnea, and abdominal and/or flank ten-
derness. A palpable flank mass, indicating pyonephrosis
X-Rays
and possible xanthogranulomatous pyelonephritis, may
occur (23). The clinical presentation is quite variable and The KUB film or flat plate is an X-ray that includes the
subtle. An SCI patient may only perceive fatigue and kidneys, ureters, and bladder. It is excellent for identify-
anorexia without any localizing symptoms. A high index ing calcific densities that may overlie the urinary tract.
of suspicion is frequently required to make the diagnosis However, this is a two-dimensional picture, and another
by radiologic assessment. type of imaging is required to localize the calcification to
the urinary tract. Also, radiolucent stones made up of uric
acid, matrix, or xanthine may not be visualized. Struvite
DIAGNOSIS and cystine stones are also less dense and may be obscured
by GI material within the colon.
An organized and systematic work-up is the key to a
timely and proper diagnosis. This begins with a thorough
Intravenous Pyelogram (IVP)
history and physical examination, including vital signs.
The classic signs of upper urinary tract obstruction are The IVP has traditionally been the imaging test of choice
rarely present in the SCI population and only vague sys- for the diagnosis of stones. It provides a gross assessment
temic signs and symptoms may be present. Complaints of renal function and includes a vascular, nephrographic,
that may be voiced include not feeling quite right, loss and excretory phase. It provides confirmation of a calci-
of appetite, or a UTI that does not clear with antibiotics. fied stone within the collecting system, with the addition
The appropriate laboratory and radiographic studies will of oblique films or the identification of a radiolucent fill-
substantiate clinical suspicions. ing defect that may represent a uric acid stone. This does
require the intravenous infusion of a contrast agent with
potential nephrotoxicity. Contraindications include renal
Laboratory Tests
insufficiency and allergy to the contrast. Patients at risk
Laboratory testing should include serum electrolytes, can undergo chemoprevention for anaphylaxis with oral
blood urea nitrogen (BUN), creatinine (Cr), a complete steroids and Benadryl, with close monitoring for allergy.
blood count (CBC), and urinalysis (UA) with dipstick (See Figure 24.2).
analysis and a microscopic assessment of a spun sedi-
ment. An elevated white blood count with a shift to the
left indicates the possibility of a serious infection. The
UA may identify a very alkaline urine (i.e., pH 8), which
could indicate an infection with a urea-splitting organ- TABLE 24.4
ism. An elevated BUN and Cr indicates the possibility
Radiographic Imaging
of renal insufficiency from post-renal obstruction. This
laboratory assessment is an important first step in select- Flat plate (KUB) of abdomen
ing the indicated imaging test to confirm the diagnosis Intravenous pyelogram
of stone. Helical CT scan
Electrolyte abnormalities must be identified and cor- Ultrasound
rected. These can occur in association with vomiting, Retrograde pyelogram
diarrhea, and fluid overhydration. The presence of white
UROLITHIASIS IN SPINAL CORD DISORDERS 313
FIGURE 24.2
An IVP with a stone at the right UPJ and stone in the left kidney on the scout film. There is obstruction to flow with hydro-
nephrosis on the left on the 10 minute film.
Ultrasonography
Ultrasound is a good screening tool that is noninvasive,
although it is limited by its low specificity. It can demon- FIGURE 24.3
strate renal and bladder stones, but is less effective with CAT scan demonstrates a xanthogranulomatous pyelonephri-
ureteral stones. It has minimal toxicity, and it is useful in tis of the left kidney. This kidney shows chronic inflammatory
identifying hydronephrosis and perinephric fluid collection, changes with a stone in the center of the inflammatory
as found in perirenal abscesses (see Figures 24.5 and 24.6). process.
314 MANAGEMENT OF THE BLADDER, BOWEL, AND SEXUAL DYSFUNCTION
FIGURE 24.4
Helical CT scan without contrast demonstrating hydronephrosis with stranding around left kidney.
anatomy. This approach offers the advantage of confir- response, and an inability to clear airway secretions,
mation of the stone and its location and simultaneous which, in turn, may result in atelectasis or pneumonia.
relief of obstruction or removal of stone with endouro- The reported incidence of deep venous thrombosis ranges
logical techniques. from 47 to 72 percent. The death rate from pulmonary
emboli is noted to be as high as 37 percent in the presence
of an existing deep vein thrombosis (DVT). Pressure sores
TREATMENT or ulcers can also occur in the acute setting of SCI.
Impaired response to infection and wound healing are
SCI is a devastating event that results in multisystem also uniformly present in the SCI patient. It is important
impairment, and the resultant morbidity dramatically that the clinician have a strong index of suspicion and
affects the patient, the family, and the community. The promptly treats any comorbid conditions. These prob-
clinical complexity of the SCI patient is apparent when lems may be subtle in their clinical presentation. How-
one considers the high prevalence of comorbidities. ever, the very low reserve strength of this patient popu-
Impaired primary and accessory muscles of respiration lation can cause a patient to quickly slide into a critical
can cause respiratory insufficiency, a weakened cough
FIGURE 24.5
Ultrasound represents a right kidney with two stones. There FIGURE 24.6
is a hyperechoic area in the superior and middle pole of the
kidney with shadowing behind each stone. Ultrasound shows stone in the upper pole of the kidney.
UROLITHIASIS IN SPINAL CORD DISORDERS 315
Pain Endourology
Recurrent infections
Increasing size Urinary Diversion
Recurrent hematuria
Deterioration of renal function
The identification of infection in the presence of a stone
Failure of medical management with obstruction is an emergency and requires emergent
surgical intervention to relieve this obstruction. Cys-
toscopy and the retrograde placement of a ureteral
catheter or a JJ stent can accomplish this. This technique
prognosis. An associated occurrence of chronic indolent diverts the infected urine around the obstructing calcu-
infection can also sap the patients reserve. lus, whether it is located in the ureter or the renal pelvis.
The stone patient can have either medical or sur- If retrograde techniques are unsuccessful, the urologist
gical stone disease. Medical stones are calculi without and/or radiologist must be prepared to provide percuta-
evidence of infection, obstruction, or deteriorating renal neous antegrade drainage with ultrasound-directed, CT
function, and are nonenlarging on serial x-rays (2). Sur- scan-guided, and/or fluoroscopic-visualized placement of
gical stone disease refers to calculi that are associated with a percutaneous nephrostomy tube. The presence of perire-
an increase in size on serial x-rays, the presence of nal fluid collection (i.e., abscess), necessitates the percu-
obstruction, gross hematuria, recurrent UTIs, pain, and taneous drainage as well. These techniques are frequently
progressive renal insufficiency (2). Surgical treatment may life saving. Once drainage is obtained and the infection
require emergent drainage, urinary diversion or surgical is treated with appropriate antibiotic therapy, subsequent
removal (see Table 24.5). surgical intervention is elective.
TABLE 24.6
Surgical Treatment of Stone Disease
*Preferred
316 MANAGEMENT OF THE BLADDER, BOWEL, AND SEXUAL DYSFUNCTION
rigid and flexible instrumentation with fiberoptic scopes grasped with forceps. However, if the stone is greater than
have greatly widened the indications for this approach. 1 cm, the stone will need fragmentation with an energy
However, ureteroscopy is an invasive procedure, source, electrohydraulic lithotripsy, ultrasonic lithotripsy,
requiring a general anesthetic or conscious sedation, and or pulsed laser in order to be extracted (Figure 24.8).
may require an energy source (Figure 24.7). Multiple access sites may be necessary to remove all
Laser and electrohydrolic lithotripsy may risk stones. Nephrostograms and nephrotomograms should
ureteral injury. Ureteroscopy has a reported ureteral be done in 24 to 48 hours to determine if there are any
injury rate of 10 percent (27). Furthermore, if a ureteral residual stones. PCNL is recommended for upper and
stent is placed at the time of surgery, a second procedure middle pole stones 2 cm, cystine stones 1.5 cm,
is required for removal. Ureteroscopy offers an advantage lower pole kidney stones 1 cm; and staghorn calculi.
in that it allows immediate treatment of a stone with min- It is also indicated for stones 1 cm; if there is a nar-
imal hospitalization and can be performed as an outpa- row infundibulum, acute infundibular pelvic angle ( 90
tient procedure. degrees) or a stone of hard composition. Furthermore,
This approach provides significant cost savings and PCNL can be used as a salvage procedure on ureteral
can provide a stone-free status without the inconvenience stones, if ESWL or ureteroscopy has failed (24,28,29).
of spontaneous passage of fragments. Finally, ureteroscopy Finally, PCNL is indicated for densely impacted stones,
offers excellent stone-free rates of greater than 90 percent stones proximal to a ureteral stent, or an obstructed
in appropriately selected patients. The criteria for selection ureter without proximal hydronephrosis. Antegrade
include the location and size of the ureteral stone. The best ureteroscopy can be used in conjunction with PNCL.
candidates are those whose stones are located in the dis- After access is gained, a flexible ureteroscope or cysto-
tal ureter, although with the availability of the Holmium scope can be manipulated into the ureter, thus allowing
laser and smaller flexible instrumentation, the indications stones to be extracted or ablated with an energy source,
for proximal ureteral, renal pelvic, and calyceal stones are EHL, or Holmium laser. Visualization of the ureter down
widening. This remains an area of investigation. to the bladder can performed. Overall, percutaneous
nephrostolithotomy has an 80 to 85 percent stone-free
rate and approaches 95 to 100 percent with repeat pro-
Percutaneous Nephrostolithotomy
cedures. In the SCI population, stone free rates are sim-
Prior to starting the percutaneous nephrostolithotomy ilar. The success of PCNL is limited by dense fascia scars,
(PCNL), the urine must be sterilized. The patient under- immobile kidneys, distorted intrarenal architecture,
goes general or local anesthesia. The patient is placed in bulky stones, scoliosis, kidney malrotation, ectopia, and
the prone position. Localization of entry is through a pos- obesity (24).
terior calyx with biplanar C-arm fluoroscopy. Visualiza-
tion can be assisted by injection of contrast through an
external ureteral catheter (2). Sequential dilation is per-
formed through a nephrostomy tract to 34 French (2).
The renal collecting system is inspected with a nephro-
scope. If a stone is less than 1 cm, then the stone can be
A flexible ureteroscope adjacent to a lower pole stone. Percutaneous nephrostomy tube drainage.
UROLITHIASIS IN SPINAL CORD DISORDERS 317
FIGURE 24.10
Extracorporeal Shock Wave Lithotripsy
The advent of extracorporeal shock wave lithotripsy HM3 Dornier Lithotripter: Large bath fixed biplaner fluoro-
(ESWL) in the early 1980s provided a noninvasive mode scopy and large space.
of therapy for renal and upper ureteral calculi (32). ESWL
utilizes a high-energy spark that causes an explosive
vaporization of water. Recent developments include a in ambulatory patients and 73 percent in the SCI popula-
reduction in the size of the equipment, less space require- tion (29). The stone clearance rate is between 80 and 92
ment, portability, and a reduced requirement for anes- percent (33). The retreatment rate is between 5 and 10 per-
thesia. Shock waves are created and transmitted to a sec- cent. SCI patients have poor clearance with ESWL because
ond focal pointthe stonewhich is blasted and of a large stone burden and poor mobility (Figure 24.11).
fragmented. Biplane C-arm fluoroscopy is used to local- The advantages of ESWL are that it has minimum
ize the stone. Repeat procedures might be necessary. morbidity and mortality; local anesthetic can be used with
Stones that are fragmented either pass spontaneously or intravenous sedation; and it offers some advantage over
are extracted (Figure 24.10).
SCI patients can have large stones with large frag-
ments. ESWL has a stone fragmentation rate of 89 percent
Set up for percutaneous access and intrarenal surgry for stone Lithotron small water balloon transportable and minimal
disease. anesthesia.
318 MANAGEMENT OF THE BLADDER, BOWEL, AND SEXUAL DYSFUNCTION
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der management techniques on struvite calculi associated with ders, 1990.
spinal cord injury. BJU Int 1999; 84(3): 280285. 38. Williams JJ, Rodman JS, et al. A randomized double-blind study
12. McKinley WO, Jackson AB, et al. Long-term medical complica- of acetohydroxamic acid in struvite nephrolithiasis. N Engl J. Med
tions after traumatic spinal cord injury: A regional model systems 1984; 311(12):760764.
analysis. Arch Phys Med Rehabil 1999; 80(11):14021410. 39. Dretler SP, Pfister RC. Primary dissolution therapy of struvite cal-
13. Safani MM, Tatro DS. Acetohydroxamic acid-dissolution of uri- culi. J Urol 1984; 131(5):861863.
nary stones? Drug Intell Clin Pharm 1981; 15(11):886. 40. Puppo P, Germinale F, et al. Propionhydroxamic acid in the man-
14. Nikakhtar B, Vaziri ND, et al. Urolithiasis in patients with spinal agement of struvite urinary stones. Contrib Nephrol 1987;
cord injury. Paraplegia 1981; 19(6):363366. 58:201206.
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25
The Gastrointestinal
System after Spinal
Cord Injury
pinal cord injury (SCI) interrupts toms. Specifically, neurogenic bowel dysfunction pro-
321
322 MANAGEMENT OF THE BLADDER, BOWEL, AND SEXUAL DYSFUNCTION
have been hypothesized as contributory. SCI patients, espe- (i.e., heartburn) may be absent or less reliable in
cially tetraplegics, spend a great proportion of their time tetraplegics. However, those whose lesions are below T7
in the supine or semi-upright position. In lower thoracic can expect to present complaints similar to those of the
SCI, abdominal muscles are frequently used to increase spinally intact. This effect on symptoms may be the rea-
intraabdominal pressure to relieve chronic constipation son that fewer SCI patients are seen and evaluated for
(the Valsalva maneuver), which could lead to transient esophageal disorders. These patients may be empirically
relaxation of the lower esophageal sphincter and subse- treated with proton pump inhibitors (PPIs) or histamine-
quent reflux of gastric contents in the esophageal lumen. 2 receptor antagonists (H2RAs) and, if symptoms persist,
There is evidence that esophageal dysmotility may endoscopic or manometric studies should be considered.
increase the risk of reflux esophagitis (32). It has been However, persons who have lesions at higher levels (above
shown that low amplitude contractions are the charac- T7) may only experience waterbrush (an acidic after-
teristic motility disorder commonly found among spinal taste), and fail to experience typical heartburn. Unfortu-
intact patients who have GERD (33). These contractions nately, the recognition of esophageal disease may only
have also been demonstrated by manometric studies to be take place after the patient complains of dysphagia or
very prevalent among SCI patients (32). High-grade develops GI bleeding; it is only then that a procedure is
esophagitis is more frequent among SCI patients as com- done to diagnose the disease, and response to therapy is
pared to the spinally intact. Unfortunately, fewer endo- monitored (Figure 25.1). Because the prevalence of high-
scopic procedures are carried out to evaluate their GERD grade esophagitis is higher after SCI, it could be argued
symptoms and follow them for pathology (31). that patients with SCI, especially those who have higher
The higher prevalence of high-grade esophagitis lesions, should be screened for esophageal disease.
among persons with SCI could be caused by delayed diag- Once GERD is identified, therapy in individuals
nosis (31). The characteristic symptomatology of GERD with SCI is similar to that in other patients and can be
FIGURE 25.1
either medical or surgical. Medical management includes to H2RAs and are the most effective agents in the ther-
nonpharmacologic and pharmacologic approaches. Non- apy of GERD because they provide more complete acid
pharmacologic therapies include lifestyle modifications, control (37).
such as the reduction of caffeine, chocolate, peppermint, Medications promoting GI motility include bethane-
alcohol intake, and cessation of smoking. Staying upright chol, metoclopramide, and cisapride. Cisapride has been
after meals and avoiding meals immediately before shown to be safe and well tolerated in a population of
bedtime may also improve symptoms (34). Treatment is patients with mild-to-moderate GERD (38), but the pos-
generally directed towards acid suppression and the sibility of fatal arrhythmias (39) has resulted in its with-
improvement in motility within the limits of available drawal from the marketplace. The use of metoclopramide
medications. Antacids, H2RAs, and PPIs are the agents and bethanechol is also limited because of their prominent
most commonly used to control acid secretion. Antacids side-effects, especially extrapyramidal symptoms (40,41).
and H2RAs are usually the first line of treatment because Medical therapy GERD fails for a number of rea-
of their availability as nonprescription medications; see sons: noncompliance, recurrent symptoms when medica-
Table 25.2 (35,36). PPIs have been shown to be superior tions are stopped or reduced, intractable symptoms
TABLE 25.2
Medications for Treatment of Gastroesophageal Reflux Symptoms
ANTACIDS
H2 RECEPTOR ANTAGONISTS
STOMACH AND DUODENUM: INCOMPLETE nal ulceration, and perforation. These risks have been
EMPTYING AND PEPTIC ULCER DISEASE attenuated with the empiric use of H2RAs, PPIs, and stool
guaiac surveillance for GI bleeding.
During the first few weeks after SCI, impairment in gas- The risk for ulcer after SCI is being evaluated in ani-
tric emptying is common, especially in patients with mal models. Animal studies have shown that there is a
lesions above T1. The prolongation of gastric emptying direct relationship between the onset of the injury and the
persists into chronic SCI (42). Treatment with intravenous degree of ulceration (43). A single cause for the patho-
metoclopramide at 5 to 10 mg per dose or bethanechol genesis of gastroduodenal ulceration and hemorrhage has
at 25 mg 30 minutes before meals is effective. not been substantiated. Several mechanisms have been pro-
During the first few weeks after acute SCI, there is posed, including acute ischemia of the gastric mucosa and
also an increased risk for gastric erosions, gastroduode- an imbalance between the parasympathetic and sympa-
thetic activity governing digestive function (30). In rats,
cervical cord transection does not increase gastric acid out-
put or plasma gastrin levels. Conversely, vagal stimulation
enhances gastric acid output in rats with cervical cord tran-
section. However, the characteristics of the gastric ulcera-
tion are not similar to those seen in the acute stages of SCI
in humans (44). Further research is necessary.
injuries, spinal fractures with casting); dietary disorders ing should include flat and upright abdominal films, elec-
(anorexia nervosa, malabsorption syndrome); and the trolytes, complete blood count, and serum amylase testing.
postoperative state (48). Treatment starts with conservative measures, which
The diagnosis is confirmed with a barium upper GI almost always improve symptoms while the causes can be
series (48). Findings include dilatation of the proximal duo- eliminated. Sitting the patient up reduces the forces on the
denum and a cut off of the transverse duodenum that duodenum and facilitates gravity flow in the gut. All meals
blocks barium flow. The superior mesenteric artery (SMA) should be taken sitting upright. Soon after, the person should
branches off the aorta at the L1 level, and traverses the root lie down in the right lateral recumbent position (right side
of the mesentery of the small intestine over the duodenum down) for 1 hour (48). A recommended surgical option is
in a ventral and caudal direction (see Figure 25.4). The angle duodenojejunostomy from the second portion of the duo-
between the small bowel mesentery and the aorta is most denum to the jejunum (49). Other surgeries include gastro-
acute when a patient is supine. The closure of this angle over jejunostomy and Roux-en-Y duodenojejunostomy (50).
the duodenum has been termed the nutcracker effect.
Despite the risk, the actual clinical incidence of SMAS
among patient with SCI is rather low, estimated to be 0.53 THE PANCREAS: INCREASED RISK
percent in a cohort of 567 (25). Other conditions that may FOR PANCREATITIS
mimic SMAS include the poor gastric emptying seen fre-
quently in tetraplegics, peptic ulcer disease, gastroe- The pancreas is affected in the acute phase of the spinal
sophageal reflux, ileus, and colonic impaction. Initial screen- injury as well (25). It has been hypothesized that because
A B
FIGURE 25.4
cholecystitis or advanced biliary disease versus biliary rons that interconnect them. Together they comprise an
colic than those with low lesions. SCI patients were less independent nervous system for the gut that orchestrates
likely to present with cholecystitis or advanced biliary dis- inherent automaticity. This aspect of colon physiology
ease than general patients. Further, comparisons of oper- remains largely intact after SCI. However, propulsive con-
ative times, conversions from laparoscopic to open pro- traction as well as the act of defecation function best with
cedures, estimated blood loss, morbidity, and mortality neural input from outside the colon. These extrinsic influ-
did not substantially differ between the SCI and general ences involve sacral parasympathetic nerves as well as
populations (63). somatic nerves. Not unexpectedly, these latter pathways
are often altered after both upper and lower neuron
injuries to the spinal cord.
THE ILEUM AND JEJUNUM: The colon receives parasympathetic and sympathetic
ILEUS ADYNAMIC innervation (see Figure 25.5.) The vagus nerve (parasym-
pathetic) descends from the brainstem to innervate the
During the first few days to weeks after SCI, abdominal distal two-thirds of the esophagus and end at the splenic
distention is common. This is often attributed to nonme- flexure of the colon. Sympathetic supply comes from the
chanical intestinal obstruction or paralytic adynamic mesenteric (T5T12) and the hypogastric (T12L3)
ileus. People with SCI have many risk factors for this dis- nerves. The pelvic floor is innervated by the mixed
tention including, major trauma, surgery, anesthesia, and somatic pudendal nerve (S2S4).
severe medical illness (64). An evaluation requires a care- With neural circuitry continuously intact, normal
ful check for the presence of bowel sounds. Confirmation colonic peristalsis and defecation occur under combined
comes from an abdominal radiograph that reveals dilated autonomic and voluntary somatic control (66). Colonic
loops of intestine. An upright and lateral decubitus posi-
tion film may reveal air fluid levels and perforation. Man-
agement includes a review of amylase levels, electrolytes,
and CBC. Hyponatremia and hypochloremia should be
treated with intravenous electrolyte therapy. The
intestines can be decompressed by placing a nasogastric
tube and running it to suction. Intravenous metoclo-
pramide can be helpful as well. Symptoms typically
improve in days.
peristalsis consists of mixing segmental contractions on in the anal canal at rest, which averages 50 to 100 mm
the right and more propulsive patterns on the left. Giant Hg and is not altered by SCI (72,73). The external anal
migratory contractions (GMCs) of the colon actively pro- sphincter (EAS) is somatic musculature having continu-
pel stool toward the rectum. Cecum to anus colonic trans- ous contractions that increase in response to mechanical
port typically takes 12 to 20 hours (67). GMCs occur stimulation or challenges to continence (74). The EAS
more frequently in the morning and with the gastro- contracts to retain stool if the rectum is rapidly dilated.
colonic response. This is referred to as the holding reflex.
The gastrocolonic response is an increase in colonic Voluntary defecation begins with the sensation of
motility induced by feeding. The motility starts in min- rectal dilatation from stool advancement (Table 25.5).
utes and peaks in less than an hour and may last for few This stretches the puborectalis muscle (Figure 25.6) and
hours. The term is used because the mechanism is not con- the rectal wall, which signals the urge to defecate (75).
clusively defined and may involve vagal function, intrin- Stool approaches the internal sphincter, which automat-
sic neural pathways in the colon, or hormonal effects via ically relaxes (recto-anal inhibitory reflex) and the exter-
gastrin (68), motilin (69), or cholecystokinin. Fatty foods nal anal sphincter is voluntarily contracted to maintain
and proteins provide the best stimulus. SCI may interfere continence.
with the strength of the response (70,71). When the situation is socially appropriate, volun-
Continence is maintained by the anal sphincters. The tary defecation occurs (Figure 25.7). Sitting straightens
internal anal sphincter (IAS) (smooth muscle), the exter- the rectum and makes the anorectal angle less acute. To
nal anal sphincter (somatic muscle) and the puborectalis initiate defecation, the external anal sphincter and the
muscles act together to keep the rectum closed (72). puborectalis muscle are relaxed. Then the glottis is closed,
Together, they make up the anal sphincter mechanism (see the diaphragm descends, and abdominal muscles contract
Figure 25.6). The IAS is the major contributor of pressure to raise intraabdominal pressure via the Valsalva maneu-
ver. Sigmoid peristalsis via longitudinal muscle layer con-
traction and gravity then propel the stool out.
TABLE 25.5
The Sequence of Events in Normal Defecation
Involuntary activity:
a. Giant migratory contractions (GMCs) advance stool
through the colon to the rectum.
b. Stool distends the rectum and stretches the puborec-
talis muscle as the internal sphincter relaxesrecto-
FIGURE 25.6 anal inhibitory reflex. This triggers a conscious urge
to defecate.
Sagittal and transverse views of the rectum and sphincters.
c. External anal sphincter and puborectalis muscle con-
Sagittal view of the rectum, anal canal, and surrounding mus-
traction retains stoolholding reflex.
cles. The puborectalis muscle forms a sling posteriorly around
the rectum at the anorectal junction. The external anal Voluntary activity:
sphincter (skeletal muscle) surrounds the anal canal and is a. Relaxation of the external anal sphincter and the
closely associated with the puborectalis muscle. The inter- puborectalis.
nal anal sphincter (smooth muscle) lies within the ring of b. Contraction of the levator ani, external abdominals,
external sphincter muscles and is a continuation of the inner and diaphragm combined with glottis closure ele-
circular layer of the smooth muscles of the rectal wall. (From vates intra-abdominal pressure and aids peristalsis in
Madoff RD, Williams JG, Caushaj PF. Fecal incontinence. propelling stool out.
N Engl J Med 1992; 326:10021007. With permission.)
THE GASTROINTESTINAL SYSTEM AFTER SPINAL CORD INJURY 331
FIGURE 25.7
Sphincter relaxation and increase in intraabdominal pressure during defecation. A. Defecation is prevented by a statically
increased tone of the internal anal sphincter and puborectalis, as well as by the mechanical effects of the acute anorectal angle.
Dynamic responses of the external anal sphincter and puborectalis to rectal distention reflexes or increased intraabdominal pres-
sures further impede defecation. B. To initiate defecation, the puborectalis muscle and external anal sphincter relax while intraab-
dominal pressure is increased by a Valsalva maneuver, which is facilitated by squatting. The levator ani helps reduce the acute
anorectal angle to open the distal anal canal to receive the stool bolus. C. Intrarectal reflexes result in continued internal anal
sphincter relaxation and rectal propulsive contractions, which help expel the bolus through the open canal. (Modified from
Shiller LR. Fecal incontinence. In: Sleisenger MH, Fordtran JS (eds.), Gastrointestinal Disease: Pathophysiology, Diagnosis,
Management, 4th ed. Philadelphia: WB Saunders, 1989; 322. With permission.)
increased transit time, constipation, incontinence, and the ally intact after spinal cord damage. Microscopic findings
discoordination of fecal elimination. All of these limit the of the ganglion cell loss and Schwann cell proliferation
human task of socially appropriate and efficient defeca- within the colon wall have been attributed to the transsy-
tion and produce significant activity limitation. Consti- naptic effects of central and peripheral nerve lesions
pation is difficult to define in a population that often (79,80). Gut wall neuroplastic changes, such as branch-
requires assistance for bowel care. However, less than ing and neural circuit remodeling, are expected to occur
three bowel movements per week, incomplete evacuation, in response to spinal lesions, but the actual effects on
and chronic abdominal distention are frequently used cri- enteric nervous control of gut function are yet to be elu-
teria (76). A quantitative study of a heterogeneous groups cidated. There are two basic patterns of colonic and pelvic
of patients with SCI estimated that 58 percent had con- floor dysfunction: lower motor neuron and upper motor
stipation and that 33 percent had abdominal discomfort neuron.
(77). Neurogenic bowel as a term is quite general and
includes a variety of types of dysfunction that are related
Lower Motor Neuron Neurogenic Bowel
to neural lesions located along the spinal cord, peripheral
nerves, and within the enteric nervous system of the The lower motor neuron (LMN) bowel presents a pattern
colon. The response patterns associated with these vari- of colonic dysfunction that results from a lesion affect-
ous lesions can be reviewed by starting at the muscle lay- ing parasympathetic cell bodies within the spinal cord at
ers of the colon and working toward the spinal cord and the conus, their axons in the cauda equina, parasympa-
supraspinal centers (78). thetic axons in the inferior splanchnic nerve, and somatic
Autonomic nervous system lesions that denervate motor axons in the pudendal nerve (78,81). This pattern
the gut have little sustained effect on function. Fortu- of colonic and pelvic floor dysfunction is common in
nately, the gut enteric nervous system remains function- patients with SCI lesions at vertebral level T10 and below.
332 MANAGEMENT OF THE BLADDER, BOWEL, AND SEXUAL DYSFUNCTION
After lesions to the inferior splanchnic nerves, cauda with lower-than-normal resting anal sphincter pressures
equina, or conus, the descending colon wall has a lower tended to have more severe bowel symptoms (89). This
resting tone and no spinal cord-mediated reflex peristal- may be caused by chronic overdistention of the rectum.
sis occurs (78). Slow stool propulsion, with segmental Colonic mucosal surface electromyographic studies
colonic peristalsis is coordinated by the myenteric plexus have demonstrated that persons with UMN SCI have a
alone while water absorption continues. The predomi- higher basal colonic activity than so normal subjects
nant mixing peristaltic pattern is segmental and causes (70,80). This may contribute to an imbalance of innerva-
excess absorption, producing a drier and rounder stool tion, overactive segmental peristalsis, underactive propul-
shape referred to as scybalous (78). The external anal sive peristalsis, and a hyperactive holding reflex with spas-
sphincter (EAS) is frequently denervated because of cauda tic EAS constriction, thus producing megacolon and rectal
equina, sacral root, or pudendal nerve damage, prevent- impaction (76,78). Cervicothoracic SCI interrupts the cor-
ing reflex EAS sphincter contraction and increasing the tical inhibitory pathways and creates excessive sympathetic
risk for incontinence. In addition, the pelvic floor laxity stimulation and increased smooth muscle tone throughout
and decreased puborectalis tone contributes to a loss of the colon (71). This constellation of problems responds
anorectal angle and the absence of a holding reflex. to an upper motor neuron bowel program and bowel care
Unfortunately, the rectalanal inhibitory reflex is retained technique with mechanical (90), chemical, or electrical
even in lesions of the conus medullaris or cauda equina stimulus to trigger and sustain reflex defecation (78,91).
(79,82). This further contributes to the risk for inconti- Intracolonic pressures have been investigated with
nence by reflex relaxation of the internal sphincter with pressure monitors and gradual distention from continu-
rectal distention as stool advances. Furthermore, the lev- ous infusion. Initial studies of colonic compliance of the
ator ani muscles and external anal sphincter lack tone, UMN colon were reported in the 1940s using water
thus allowing the sigmoid and rectum to descend into the manometry (92). Steep rises in intracolonic pressures of
pelvis and the perineum to sag, further reducing and 40 mmHg (normal 5 to 15 mmHg) were observed in sub-
opening the rectal angle and the rectal lumen (83). There- jects with UMN SCI, occurring at infusion volumes as low
fore, stool is more apt to slip out during pressure releases as 400 to 600ml (71). Some of these studies (90,93) have
or contraction of innervated abdominal musculature. proposed a hyperreflexic response of the left colon that
is analogous to that of the UMN bladder. Other investi-
gators (94) have utilized colometrograms, in which water
Upper Motor Neuron Neurogenic Bowel
was infused into the rectum at a slower rate of 100 ml/min
The second pattern, that of the upper motor neuron with continuous intracolonic pressure measurement.
(UMN) bowel, is produced by a spinal cord lesion any- These and other subsequent studies, using gradual or
where above the conus medullaris (78). The spinal cord intermittent rectal infusion, have demonstrated gradual
below the lesion usually retains perfusion that spares the intracolonic pressure increases and an adaptation to dis-
grey matter conal reflex function at levels S1 through S4. tention with colonic compliances that have approached
Stool transit times through the colon are slower after SCI. normal (61,95). Single pressure measurements using a
Transit time remains a very significant clinical measure of hydraulic system may not reliably reflect actual pressures
colonic function (84). However, findings must be inter- throughout the colon because segmental constriction may
preted with consideration of the frequency of bowel care. artifactually raise pressures close to the monitor while
Stool transit times can be increased with standardized more proximal pressures are lower.
bowel programs that include an increase in bowel care A variety of other methodologies have been utilized
frequency (85). Excessive rectosigmoid distention can in the study of colonic pressure transport after SCI
occur with infrequent or ineffective evacuation. Disten- (67,79,82,96). A review of methods by Wald has sug-
tion of the rectum alone can slow intestinal transit (86). gested that these techniques could be useful for the clin-
Cervical spinal cord lesions are associated with prolonged ical evaluation of colonic and anorectal motility (97).
mouth-to-cecum transit time (MCTT). Many people with However, studies of colonic motility that involve pressure
prolonged gastric emptying (87). and paraplegia exhibit sensing catheters placed in the colon are short term and
MCTTs that are comparable to normal. In vivo studies of are prone to catheter displacement during the recording
the UMN colon have demonstrated excessive colonic wall period. To overcome these limitations, a technique has
and external anal sphincter tone (88). been described wherein the motility catheter is tethered
The external anal sphincter (EAS) is included in the to the bowel wall using endoscopically deployed clips
striated muscle that makes up the pelvic floor. This mus- (98). This new approach is employed to study the effect
culature becomes spastic after UMN lesions and can of UMN SCI on colon motility. As compared to controls
obstruct defecation (88). Recent perfusion manometry with intact spinal cords, UMN SCI was found to signifi-
studies show resting anal sphincter pressures that are com- cantly reduce the motility index (% activity mmHg)
parable to controls without SCI. However, SCI patients after a defined standardized meal (Figure 25.7). Despite
THE GASTROINTESTINAL SYSTEM AFTER SPINAL CORD INJURY 333
the reduction in overall motility, the percentage of each ron (LMN) pattern. The rehabilitation database must
type of wave (i.e., propagating, simultaneous, retrograde, include a comprehensive, accurate neurologic examina-
and isolated) was unchanged. This indicates that, at least tion and diagnoses that could impact colonic function or
in part, the slow transit times noted after SCI may be methods for compensation. Impairments of colonic func-
caused by an absolute decrease in the number of propa- tion must be derived from history, examination, and
gating waves after food intake. Previously, investigators appropriate laboratory and functional studies (78). The
have also suggested that the gastrocolonic response after findings from the history and physical examination are
complete SCI is less robust or absent (70,71). compared, and diagnoses and treatment plans are devel-
Colonic transit time can also be measured with oped. Specific difficulties such as constipation, difficulty
sequentially swallowed markers that are followed with evacuation, and fecal incontinence must be under-
through the colon with serial radiographs (99,100). Inves- stood and quantified (78,81,102). The full variety of
tigations applying this technique in UMN SCI have illus- pathology, impairments, and activity limitations that
trated markedly delayed left colon and rectal transit times interact to prevent the ability to maintain continence and
in persons with UMN SCI, as compared with controls willfully defecate must be assessed from the perspective
(67,82). A recent study demonstrated prolonged ascend- of the entire person. Next, limitations in functional mobil-
ing, transverse, and descending transit times with supra- ity, as well as assets that can be utilized in bowel care pro-
conal UMN SCI and prolonged rectosigmoid transit times cedures, such as strength for transfers, sitting balance, and
in conal LMN/cauda equina lesions (100). Technetium residual colonic reflex function, must be discovered.
(101) and Indium-111 Amberlite scintigraphy have also Finally, barriers to full life participation must be
been utilized to investigate colonic transport in subjects addressed, with careful consideration of the persons life
with UMN SCI (96). An isotope, Amberlite IR-120-P, was goals and role expectationsparticularly cultural, sex-
encapsulated in gelatin and coated with methacrylate to ual, and vocational roles.
allow release at the higher pH in the colon. In scinti- The goal of the entire rehabilitation process is to
graphic imaging of the colon after oral ingestion of the overcome disability. Assessing an individuals problems
isotope capsule, the ascending colon emptying half time with neurogenic bowel therefore requires knowledge of
was 29 27 h for subjects with SCI, as compared to 6.81 the person and her limitations (78) through eliciting her
3.03 h for controls, p 0.01; ascending and transverse: strengths for adaptation and her person-centered goals
42 12 hr for subjects with SCI, 15.3 7.16 controls (78,81). Typically, a particular interdisciplinary team
p 0.01. These and other transit studies suggest that member coordinates this effort. Usually this member is
abnormalities of colonic function after UMN SCI can the primary nurse working in close association with the
involve the entire colon and may require oral agents in physiatrist, occupational therapist, and social worker. In
addition to rectal medications and reflex stimulation to the inpatient setting, scheduling can be especially difficult
get effective defecation (78). because of the time-consuming nature of bowel care, as
In conclusion, the lower motor neuron pattern of well as the number of individuals on the rehabilitation
dysfunction results from lesions to the inferior splanch- unit who require bowel care assistance (14). Evening
nic nerve to the descending colon and the pelvic nerve to bowel care often allows for more predictable attention
the pelvic floor. This results in sluggish propulsion; dry, to daily therapies (78). Incontinence episodes seem to be
rounder stool shape; and a high risk for incontinence more common during the few hours after bowel care.
because of the low tone of the external anal sphincter. The Evening bowel care also allows for positioning and drap-
upper motor neuron pattern of dysfunction results from ing in bed to accommodate the possibility of stool incon-
a spinal cord lesion above the conus which results in over- tinence (78).
active segmental peristalsis, less propulsion, and stool The goals and needs projected by the individual must
retention by a tight (spastic) external sphincter. be considered in designing a bowel program that will be
compatible with her post-rehabilitation lifestyle (102). The
scheduling and technique of the new bowel care procedure
Interdisciplinary Rehabilitation Interventions to
is particularly important. The demands of work or school,
Minimize Disablement Associated with
the duration of bowel care, and the needs of other mem-
Neurogenic Bowel Dysfunction
bers of the household must all be considered in individu-
Interventions directed toward problems associated with alizing this scheduling (78,103). Should attendant care be
the neurogenic bowel are managed similarily to other needed on an intermittent basis, early morning bowel care
issues that confront the patient in the rehabilitation and evening bowel care can often fit into part-time, inter-
process (78). Neurogenic bowel is treated the same as mittent schedules. The cost of attendant care and equip-
other impairments. The problem should be stated as suc- ment must be figured into the life care plan (103).
cinctly as possible, but should include a designation of A patient-centered approach is essential for the
either upper motor neuron (UMN) or lower motor neu- proper design of each bowel program. Individual bowel
334 MANAGEMENT OF THE BLADDER, BOWEL, AND SEXUAL DYSFUNCTION
programs are derived from the patterns of bowel function dispose the individual to life-threatening complications,
in the patients past and are practically adapted to the such as toxic megacolon if the bowel program is ineffec-
patients role performance and resources (81,102). The tive (78). SCI individuals must be very compliant with
challenge to the interdisciplinary team is to educate the bowel programs to overcome these obstacles to regular-
patient about altered physiology after SCI and to ity (78). For example, potentially constipating drugs for
empower the patient to design a bowel program and a depression and detrusor hyperreflexia were used by 39.5
bowel care technique that is effective and supportive of percent of sampled patients with SCI (104). Patients need
her lifestyle (78). to understand the relationship between their previous gas-
trointestinal problems and their post SCI bowel dysfunc-
tion. The history and physical examination is an oppor-
Patient History: A Survey of the Past
tunity for the clinician to demonstrate these effects and
to Design a New Bowel Program
educate the patient and caregivers.
The patient history should start with a classic gastroin-
testinal review of systems that includes the identification
Physical Examination: Survey for Pathology,
of pertinent related diagnoses that predate the SCI. Key
Impairments, Activity Limitations, and Complications
questions to ask in the initial interview are summarized
in Table 25.6. Previous bowel conditions and habits must A comprehensive perspective is necessary because the
be related to current function, support resources, and life capabilities of many body systems interact to compensate
demands. for impairments in stool storage and elimination. The
The patterns of premorbid bowel function lay the purpose of the examination is to identify pathology and
foundation for the post-injury bowel program (78). Fac- functional deficits, as well as functional capabilities and
tors to consider include timing of bowel movement fre- methods for compensation. To accomplish this, an exam-
quency, volume, and consistency of bowel movements, as ination that includes the nervous, gastrointestinal, mus-
well as the amount of time needed to complete bowel care. culoskeletal, and integumentary systems is most likely to
Premorbid symptoms, especially constipation, may be yield the information required to plan a successful bowel
modified. Previous habits should be replicated as closely program (102).
as possible to take advantage of well-established patterns. The abdominal examination is a practical place to
Scheduling elimination shortly after a meal takes advan- start (78), beginning with an inspection for asymmetry and
tage of the gastrocolonic response, although as noted distention. Auscultation for bowel sounds and bruits in all
above, this may be somewhat blunted after SCI (78). four quadrants precedes palpation. Abdominal muscle
Any gastrointestinal conditions that were present spasticity can be minimized by supporting the flexed knees
before SCI could complicate the bowel program after SCI. with a pillow. The colon should be palpated in a clockwise
Conditions such as laxative dependency, diabetes, irrita- direction starting at the right lower quadrant, following its
ble bowel syndrome, or inflammatory bowel disease may course as it encircles the abdomen (102). The ascending
prolong or accelerate transit time, affect the responsive- and transverse colon have varied positions in the abdomen.
ness of the gut to bowel care medications, and even pre- The descending colon reliably follows the left lateral
abdomen and becomes retroperitoneal as the colon
descends toward the pelvis (78). Masses, organomegaly,
or high colonic impaction can be detected with superfi-
TABLE 25.6 cial and deep palpation (105). Should inflammation or
Baseline Medical History For Neurogenic Bowel organ distention be present, palpation may not trigger pain
perception, spastic rigidity or local tenderness.
A. Premorbid History: daily fluid intake, diet (fiber, The rectal examination can provide much useful
meal frequency, spice preferences, amounts) bowel information. Anal contraction with cutaneous contact
movements (frequency, duration, difficulties), stool demonstrates anocutaneous reflex function. To start the
(consistency, color, mucus, blood), medications. rectal examination, the tip of the lubricated examining
B. Current status: injury level, daily fluid intake, diet, finger should be held gently against the anal verge, thus
medications, patients understanding of SCIs effect allowing gradual relaxation of anal tone. Sphincter tone
on elimination, bowel care (frequency, duration,
and its response to gradual dilatation reveals innervation
digital stimulation frequency/technique) bowel
incontinence (time of day, frequency, relationships
and the presence of spasticity. Voluntary contraction
to eating). demonstrates motor incomplete SCI. As the sphincter
C. Lifestyle goals: schedules for work or school, opens, the examining finger should be directed toward
availability of assistance if needed, amount of time the umbilicus to follow the anal canal and approach the
needed to complete bowel care regime. rectal angle at the puborectalis muscle. At the puborec-
talis, gentle pressure toward the sacrum is applied to
THE GASTROINTESTINAL SYSTEM AFTER SPINAL CORD INJURY 335
FIGURE 25.9
ing acute rehabilitation and early community reentry to contribute to loose stools. Dietary patterns before and
pursue a long-term bowel management strategy that can after SCI are reviewed for frequency and amount of food
be used to prevent both the short- and long-term seque- in meals, and for a discussion of the various food groups.
lae of neurogenic bowel dysfunction (76,81). In an effort Refined carbohydrates and dairy products tend to pro-
to derive a consensus on knowledge related to neurogenic duce hard dry stools. Spicy foods such as certain chili pep-
bowel dysfunction and rehabilitative interventions, a pers, cayenne pepper, paprika, and excessive garlic can
panel was convened by the Consortium for Spinal Cord promote transit and lead to excess fluids in stools. These
Medicine to research the literature and write clinical prac- spices can cause diarrhea and bowel accidents. Raw or
tice guidelines (102). Subsequently, the guidelines were partially cooked vegetables soften stools and decrease
utilized to write a consumers guide (Neurogenic Bowel: transit time.
What You Should Know) (Figure 25.10) on neurogenic A review of the types and amounts of insoluble fiber
bowel management that presents the pathophysiology is essential for planning a diet. Soluble fiber from fruits
and patient-centered interventions in clear language, out- and vegetables, in combination with adequate water
lined in sequential steps (91). The bowel program is bro- intake, contributes to softer, moist stools. Adequate fiber
ken down into essential components for consideration in intake in the form of whole grains, fruits, and vegetables
history taking and intervention (78). These consist of diet, allows the stool to form sufficient bulk and retain water
exercise, equipment, oral medications, rectal medications, (14). The hydrated fiber maintains a plastic consistency
and scheduled bowel care (91,102). that promotes successful propulsion through the colon
The diet component of the bowel program is mod- (78). Intracolonic material in a viscus fluid phase can be
ulated to control stool consistency (Figure 25.11) and to propelled 32 to 100 times faster than a solid (66). Dairy
prevent gut irritation. The diet is reviewed to ensure that products and high-fat meals tend to work in the oppo-
foods do not trigger incontinence, cause constipation, or site way, slowing stool transit (110).
Stool consistency modulation preserves continence
by keeping stools formed and eases propulsion and evac-
uation with moist soft stools (Figure 25.11). Maintaining
a pliable stool with a balance of fiber and adequate fluid
facilitates its transit and prevents impaction (105). Fluid
intake therefore, is an important part of the diet as well.
FIGURE 25.11
Sufficient fluid intake to produce urinary outputs of 2,000 and lead to more stool output during the bowel care
to 3,000 cc per day is optimal. Caffeinated beverages process. Nonetheless, dietary changes are preferable to
often produce unpredictable results and may have a lax- the prescription of laxatives and should be the first step.
ative as well as a diuretic effect. Fruit juices such as prune Growing evidence indicates that foods high in dietary
juice or apricot nectar provide fluid but also may stimu- fiber may also enhance health by lowering blood choles-
late stool transit. terol (111,113), increasing feelings of satiety resulting in
Vegetable matter or roughage has varied effects on weight loss, and promoting transit of stool through the
bowel function. Dietary fiber includes of a variety of indi- colon (111).
gestible plant-derived material that is primarily composed More research must be directed toward under-
of non-starch polysaccharides. A rough separation of standing the effects of dietary fiber in SCI. The findings
these substances, based on capacity for suspension in of studies completed thus far suggest there may be dif-
water, can be made. Soluble fiber consists of multi- ferences in the physiologic effect of fiber in GI function
branched hydrophilic substances such as pectins, guar, after SCI. Two significant differences are decreased and
and ispaghula that form viscous gels and colloids that varied peristalsis patterns and the problem of the spastic
delay gastric emptying and nutrient absorption. They alle- anal sphincter after SCI, which retains stool and sup-
viate constipation by keeping stool pliable and propul- presses defecation (88). A study of SCI subjects who were
sive. Two types of insoluble fiber are cellulose and lignin, supplemented with 40 gm bran per day for a 3-week
which markedly accelerate colonic transit time in subjects period revealed either no change or a prolongation of
without SCI (78). This effect is proportional to dose and colonic transit times. The increased fiber, in itself, did not
increased basal stool weight (111). produce changes in evacuation frequency, time required
Various types of fiber produce different effects for bowel care, or stool weight per defecation (114). It is
depending on the particular segments they traverse along clear that the addition of fiber to the diet of a person with
the gastrointestinal tract. These effects depend on fiber SCI makes stools softer by retaining fluid. However, the
volume, the degree of water solubility, and the location expected increase in stool bulk may require an increase in
within the GI tract. Inside the stomach, dietary fiber tends the frequency and/or duration of bowel care to fully elim-
to produces early satiety and prolongs gastric emptying inate the excess stool and prevent colonic over-distention
time. In the small intestine absorption may be delayed by (78,85,102).
fiber, whereas transit time is decreased or increased (112). Exercise is the next component of a complete bowel
In the colon, fiber provides bulk to stimulate peristalsis program. It is well known that mechanical movement of
and to retain fluid. Fiber softens and expands stool and the intestines generates peristalsis. Patients report that
eases the transit through the lower bowel, increasing the range of motion exercises before bowel care seem to
frequency of bowel movements and the volume of stools. increase the speed of the fecal elimination and result in
Coarsely ground fiber tends to be more effective than larger stool volumes. Regular activity during each day is
finely ground fiber (111). recommended on a case-by-case basis depending on inde-
Food sources rich in fiber include whole grain breads pendent movement capability. Movement that lifts the
and cereals, particularly bran. There are a variety of ways perineum up off of supporting surfaces reduces pressure
to boost fiber intake. Common additions to the diet on the anus and allows flatus to expel, thus preventing
include whole grain breads, cereals, wheat germ, legumes, overdistention. At a minimum, regular turns in bed,
and salad vegetables. Changing to a breakfast cereal that transfers up to a power wheelchair, and regular tilt or
has more whole grain content is a good first step. To fur- recline pressure releases are expected to provide some gut
ther add fiber to the breakfast, bran muffins and fresh stimulation.
fruit are good choices. Switching to whole grain breads Appropriate adaptive equipment for bowel care
increases fiber intake throughout the day. If flatus is not should be prescribed based on the individuals functional
a problem, increasing the content of beans and legumes needs and discharge environment (102). Seating for
in the diet offers fiber and protein. Dietary supplementa- upright bowel care utilizes gravity and places the abdom-
tion with fiber must be carried out gradually. The inal muscles at maximum mechanical advantage to facil-
expected impact will be a larger volume of softer stools, itate the efficient passage of stool (78). The trial and
a requirement for bowel movements with greater fre- proper identification of the most effective commode chair
quency, and an improvement in bowel care efficiency. that provides ease of transfer, stable seating, and skin pro-
Therefore, changes in the frequency of bowel care will tection is important. Nelson and her co-investigators
likely be required. studied patient satisfaction with commode chairs and
A variety of potential complications that could result found that 37 percent of 147 respondents did not feel safe
from fiber additions to the diet. The increase in indi- in their shower commode chairs (115). Transfers in and
gestible material will certainly increase stool bulk. This out can be problematic. Many commode chairs have fixed
may induce the need for more frequent bowel movements non-removable armrests, and wheel locks that are not
THE GASTROINTESTINAL SYSTEM AFTER SPINAL CORD INJURY 339
effective. Falls were reported during transfers in 35 per- cation before reflex-mediated incontinence occurs. As a
cent of respondents, and 23 percent said their injuries result, bowel care is done as the procedure for initiating
were severe enough to require hospitalization. and assisting defection to avoid incontinence and colonic
Careful measures should be taken to avoid pressure overdistention. The goals of bowel care are to facilitate
ulcers related to fecal incontinence transfers or seating predictable defecation of the maximal stool volume in the
during bowel care (102). Pressure ulcer development dur- least amount of time, with avoidance of stool inconti-
ing commode chair use (116) is a significant risk, espe- nence between bowel care sessions (78).
cially because the typical bowel care session may last Immediately after SCI, there is a period of spinal
longer than an hour and may be followed by a shower. shock, which is operationally defined as the period when
Regular pressure releases every 15 minutes, which allow reflex responses are absent below the level of the injury.
for a shift of the patients position on the seat, can be help- Typically, gastric emptying and intestinal peristalsis may
ful. This can be accomplished with a tip back of the com- slow. Bowel care is done in a side-lying position with the
mode chair or assisted leans forward and to the side. The left side down. Stool is expelled by hooking it with the
seating of the patient on the commode chair should be gloved finger and pulling it out, a maneuver termed man-
reviewed to ensure that excessive pressure and shear does ual evacuation. Excessive, constantly oozing, liquid stool
not threaten the skin over the sacrum and ischia. Focal can be managed by placement of a rectal tube or an adhe-
pressure along the edges of the toilet seat is often a prob- sive fecal collection bag. After colonic function stabilizes,
lem. Foot support should be carefully fitted and padding one of the two basic bowel care procedures is utilized.
utilized as needed to maintain knee and hip flexion just These techniques use residual motor function after UMN
beyond 90 degrees and protect skin from focal pressure and LMN injuries to meet the goals of bowel care.
and shear.
The frequency of bowel care and the time of day are BOWEL CARE WITH LOWER MOTOR NEURON BOWEL:
planned based on the patients life schedule before the FREQUENT MANUAL EVACUATION. Patients with lower
SCI. The clinical guidelines for SCI medicine have rec- levels of SCI (T12 spinal cord level and below) typically
ommended that bowel care be completed no less than have cauda equina or conal syndromes and therefore
three times per week (102). Adhering to a regular sched- LMN injuries. Persons with SCI who have LMN injuries
ule of bowel care is essential. Neglected bowel care ses- often have more difficulty with their bowel care because
sions can contribute to excessive accumulation of stool, of the absence of spinal cord-mediated reflex peristalsis;
which may become increasingly dry, less plastic, and more this is termed areflexic bowel. The rectum must be
difficult to eliminate (78). This can stretch the colon, cleared of stool more frequently, usually one or more
reduce the effectiveness of peristalsis (85), and result in times per day, to prevent the unplanned release of stool
extended bowel care with poor results. The establishment that cannot be retained by the patulous external sphincter
of rehabilitation and spinal cord unit-wide standards for (78,83). Bowel care is often timed to follow a meal to
bowel management can result in uniformity of proce- take advantage of the gastrocolonic response that may
dures, greater patient satisfaction, and improved conti- trigger advancement of stool into the rectum. Between
nence of stool (102,117). bowel care sessions, patients should be taught to avoid
Valsalva maneuvers during transfers to avoid increased
intraabdominal pressure and inadvertent expulsion of
Bowel Care: The Procedure for
stool. Some persons with SCI wear tight underwear or
Facilitated Defecation
bicycle pants to retain stool by supporting the pelvic floor
Scheduled bowel care is a subcomponent of the total and anal sphincter through gluteal adduction (78). When
bowel program. Bowel movements are essential life events any elastic clothing is used, care should be taken to
that have ramifications for health, personal assistance prevent skin breakdown by checking for skin tolerance
needs, modesty, self-esteem, and the role function of the along seams (81). An air or gel cushion can be cleaned
person with SCI. Normal or effective defecation can be easily and is also helpful to evenly distribute pressure
operationally defined as the easy, volitional passage of across the perineum. Because of the lack of gluteal muscle
enough soft, formed stool on a regular basis to prevent mass, these patients are at particular risk for shear and
the sensation of incomplete evacuation and overdisten- pressure ulcers. Padded toilet seats and frequent
tion of the colon (78). The achievement of assisted defe- repositioning can prevent breakdown.
cation that meets this definition with efficiency, conve- The procedure for bowel care for SCI persons with
nience, and privacy is imperative. After SCI, the LMN injuries usually consists of removing stool with the
sensations of need for a bowel movement, control of the finger (manual evacuation). This may include digital rec-
anal sphincter, and the capability for positioning and tal stimulation which may trigger proximal segmental
clean up after a bowel movement are often absent. There- peristasis to help propel stool out. The procedure has been
fore, a preemptive strategy must be utilized to trigger defe- broken down into steps and published in an illustrated
340 MANAGEMENT OF THE BLADDER, BOWEL, AND SEXUAL DYSFUNCTION
consumer guide (Tables 25.6 and 25.7) (91). Bowel care a tendency to produce and accumulate stool and overfill
starts with hand washing, set up, and careful transfer and the colon. Stool is retained too long and desiccates,
positioning on the commode. Digital rectal stimulation is resulting in proximal colon impaction with dry hard stool.
done by sliding the gloved and lubricated finger into the A person with SCI experiences significant disability that
rectum to relax the internal sphincter with circular fin- has a pervasive effect on defecation process. Recumbency
ger movement and proximal rectal dilatation. Deep impairs motility, the gastrocolonic response is blunted or
inhalation, Valsalva maneuver, abdominal wall contrac- absent (71), rectal sensation is impaired, and chest and
tion, and transabdominal colonic massage in a clockwise abdominal musculature are paralyzed.
manner helps trigger peristalsis and propel the stool. At Bowel care procedures for the UMN bowel are
the rectum, stool is hooked with the finger and guided designed to overcome these impairments. Persons with
past the sphincter with the help of gravity. Manual evac- UMN must do scheduled bowel care based on time rather
uation is repeated alternating with digital stimulation than the urge for defecation. This prevents uncontrollable
until there is no further palpable stool. The absence of reflex defecation (118) in response to rectal distention.
palpable stool, the passage of mucus, and tightening of Filling of the colon can trigger a giant migratory colonic
the internal anal sphincter are all signals that defecation contraction with the advancement of stool into the rec-
(bowel care) is complete. tum (78). Therefore, elimination of stool before sponta-
neous reflex defecation extrudes it requires a scheduled
BOWEL CARE WITH UPPER MOTOR NEURON BOWEL: trigger to start bowel care and the completion of defeca-
REFLEX FACILITATION. Upper motor neuron SCI results tion every 1 to 3 days (102). The patient or attendant
from a lesion above the level of the conus medullaris. This stimulates reflex defecation manually with a finger (or
produces a variety of impairments that create problems assistive device) inserted in the rectum (digital rectum
with defecation. The production of effective stimulation) and/or with appropriate chemical or electri-
intraabdominal pressure in patients with lesions above cal stimulus. The initiating stimulus should be of the min-
T12 is limited. No conscious contraction or relaxation of imal intensity required to start and sustain the defeca-
the external anal sphincter occurs, and the sensation of tion process. Typical initiating stimuli in order of intensity
rectal contents is impaired. However, many people with include abdominal massage, digital perianal stimulation,
clinically complete SCI have demonstrated the ability to digital rectal stimulation, glycerin suppository, Theravac
perceive rectal distention (93). The external anal sphincter mini-enema, and bisacodyl suppository (1,85).
is typically spastic and retains stool and flatus. The The steps for bowel care for persons with reflexic
external anal sphincter may persistently constrict, thus (UMN) bowel have been outlined in a consumer guide
impeding defecation during rectal contraction. This is (Table 25.8) (91). The chemical trigger stimulus for defe-
termed anorectal dyssynergia (84). Defecation may be cation is typically a suppository, enema, or mini-enema,
spontaneous because of the reflex relaxation of the which provides a mucosal contact stimulus that is trans-
external anal sphincter, and the poor accommodation of mitted to the conus to trigger reflex-mediated peristalsis
the rectum to stretch (118). Without bowel care, there is (78). The ideal placement of the chemical stimulant is
FIGURE 25.12
Bowel care outcome. Events and intervals of bowel care. Bowel care events separate the bowel care total period into discrete
intervals on a time line. Pharmacologically or mechanically initiated bowel care begins with insertion of rectal medication or
digital-rectal stimulation, which acts as an initializing stimulus. First Flatus ends the interval time to flatus (initializing stimulus
until first gas is passed). Begin stool flow ends the second interval, termed flatus to stool flow, and begins the defecation. The
event transfer off toilet ends the wait until transfer period, which represents the time spent to ensure that defecation is complete.
The time of transfer off toilet ends the bowel care procedure. (From Stiens SA, Biener Bergman S, Goetz LL. Neurogenic bowel
dysfunction after SCI: Clinical evaluation and rehabilitative management (focused review). Arch Phys Med Rehabil 1997; 78:S-
86S-102. With permission.)
THE GASTROINTESTINAL SYSTEM AFTER SPINAL CORD INJURY 341
lation because defecation is not frequently willful and the SCI commonly have diarrhea as a result of urinary tract
bowel care procedure varies between patients. Fecal infection (UTI) treatment with broad-spectrum antibi-
impaction, chronic constipation, and fecal incontinence otics. This is one of many reasons why treatment of UTI
are common, affecting more than 80 percent of persons should include cultures and antibiotics that are targeted
with SCI (5). Unfortunately, colonic function deteriorates specifically at the pathogen. Initial management includes
with time after SCI. Constipation-related symptoms are performing bowel care to clear as much of the liquid stool
less frequent during the 5-year period immediately after from the colon as possible. The application of an occlu-
SCI (25,136). Progressive dysfunction has been attributed sive cream to the perineum after clean up offers some pro-
to colonic over-distention over time. Symptoms include tection from stool irritation. The patient should remain
prolonged and difficult evacuation during bowel care, on regularly scheduled bowel care to prevent spontaneous
abdominal distention, and abdominal pain. liquid incontinence episodes (91).
A recent radiologic study of the volume of colon con- Patient-driven dietary interventions to prevent and
tents in persons with SCI admitted for annual evaluations manage loose stools are essential. The inclusion of a mod-
of non-bowel related medical problems sought to quantify erate amount of fiber, such as psyllium, in the diet nor-
the prevalence of radiologic constipation and megacolon malizes stool consistency by absorbing excess liquid to
(76). Radiologic constipation was defined as severe to mod- maintain formed stools. (Figure 25.11) Ongoing patient
erate stool retention in all segments, and megacolon as a experience with gut tolerance for various foods, especially
colon diameter greater than 6 centimeters. Abdominal radi- spicy or pepper-seasoned dishes, leads to the recommen-
ographs revealed 74 percent of subjects with megacolon, dation for moderation. Preemptive management of gut
and 55 percent of subjects with radiologic constipation. irritation from such indulgences includes the use of cal-
Subjects with radiologic constipation frequently com- cium antacids for symptoms of heartburn and doing
plained of constipation, particularly abdominal distention. bowel care 6 to 12 hours after such food to clear soft
More than one-third of the subjects reported spending stools before a spontaneous defecation occurs. Stool con-
longer than 1 hour on bowel care; stool retention was most sistency should be modulated by dietary choices based on
common in the right colon. Factors significantly associated regular bowel care results (91). Attendants who do bowel
with megacolon were older age, longer duration of injury, care should report consistency to their clients. Foods that
abdominal distention symptoms, radiologic constipation, firm the stool are starches and dairy products.
urinary outlet surgery, laxative drug use, anticholinergic The first step in treatment is to restore the balance
medication, and the use of calcium-containing laxatives in the gut ecology. Lactobacillus is a friendly symbiotic
(76). Hypotheses for this progression of constipation resident that may need to be reintroduced to the gut
include less-frequent or irregular bowel care, less-effective microbe population with Lactinex capsules and active
bowel care, and deficits in colonic peristalsis. Interventions cultured yogurt in the diet. If loose stools persist, abdom-
have included osmotic laxatives to soften stool for propul- inal and rectal examination should be done to rule out
sion, irritant laxatives to increase peristalsis, prokinetic partial obstruction from an inspissated fecal mass caus-
agents such as cisapride (now withdrawn from market), ing a ball valve effect and intermittent obstruction of
and increased frequency and effort with bowel care. liquid fecal flow (138).
Osmotic agents to soften stool do not produce chronic irri- Carcinoma of the colon and rectum remains a sig-
tation to the colon and, used moderately, do not lead to nificant health threat. Reduction in mortality is a conse-
incontinence. Sorbitol has been found to be as effective as quence of earlier detection and surgical removal of ade-
lactulose for chronic treatment (137). Bowel care can be nomatous polyps (139) and malignant colorectal tumors.
modified to include upright position, stronger triggering The presence of neurogenic bowel can complicate screen-
medications, small enemas, more frequent and prolonged ing and diagnosis. The myriad of chronic GI symptoms
digital stimulation, and abdominal massage. Changes in after SCI may mask a change in pattern or function that
bowel care should be followed with monitoring of bowel would trigger evaluation. The question of the contribu-
care parameters for at least 3 weeks (91). Should bowel care tion of SCI to the relative risk for colorectal carcinoma
become prolonged or associated with frequent autonomic is not fully answered. Some have suggested an increased
dysreflexia, or produce insufficient results in spite of a risk (140), whereas others have reported similar incidence
period of adaptive modification of the entire bowel pro- in controlled retrospective studies (141). Screening is
gram, surgical intervention should be considered. problematic. Hemorrhoids are highly prevalent. The
process of bowel care with digital stimulation can cause
some mucosal irritation and bleeding. Consequently, false
Diarrhea: Liquid Stool with
positive stool guaiac studies are common (142). However,
Risk For Incontinence
annual or biannual fecal occult-blood screening reduces
The World Health Organization (WHO) defines diarrhea the incidence of colorectal cancer (143). Annual digital
as stool that takes the shape of the container. Patients with rectal examination as screening is recommended for
344 MANAGEMENT OF THE BLADDER, BOWEL, AND SEXUAL DYSFUNCTION
patients over 40 and after age 35 in patients with a first- Colostomy: An Accessible Colonic Stoma with a
degree relative with a history of colorectal carcinoma or Collection Bag for Continence
polyps. Endoscopic screening and polypectomy is rec-
Colostomy is a reasonable management option for persons
ommended every 3 to 5 years after age 50 (139). Colonic
with neurogenic bowel if a sustained effort has failed to
cleansing in preparation for an examination may require
create a bowel program design that meets the patients per-
assistance at home or hospitalization, because prepara-
son-centered goals and life demands (102,150,151). Fail-
tion is prolonged and can be incomplete. Future studies
ure of bowel care to fully evacuate the colon may call for
of endoscopic screening frequency and methods for effi-
more attention to stool consistency. A common indication
ciency and yield in colon preparation are needed.
is an extremely long bowel care time with insufficient evac-
uation (152). Bowel care with tap water irrigation of the
Surgical Options: Anatomic Revisions to colon as an enema three to five times per week has been
Enhance Function, Hygiene, and Quality of Life effective in patients without SCI who have stool continence
problems. Many patients receive colostomies as a means
Appendicocecostomy: A Catheterizable Stoma for to divert the fecal stream away from high-grade buttock
Antegrade Enemas to Trigger Bowel Care pressure ulcers (136). However, pressure ulcers alone may
not be the best indication, because these often large
An alternative method of enema delivery is through a sur-
wounds have multifocal etiologies and may heal poorly
gically created continent catheterizable appendicocecos-
in the chronic state. This single intervention to prevent
tomy stoma (144). The Malone procedure has been gain-
fecal contamination may not have sufficient impact to pro-
ing in popularity since the early 1990s (144). This
mote healing (153). The best candidates for the procedure
procedure requires a right lower quadrant laparotomy
report significant difficulty or complications with typical
with mobilization of the cecum to bring the appendix
bowel care and demonstrate the capacity to manage and
through the abdominal wall. A stoma is fabricated by
benefit from a colostomy (151). Colostomy requires major
amputating the tip to expose the lumen. This opening into
abdominal surgery and includes complication risks. A vari-
the appendix is sewn into a perforation in the abdomi-
ety of conditions can result from surgical stomas: diver-
nal wall where it is most convenient for independent
sion colitis (154), bowel obstruction, stomal ischemia,
catheterization by the patient. Functional use of the stoma
retraction, prolapse, peristomal hernia fistula, and variceal
is best if the location has been identified previously with
bleeding (102,155). Death during the post-operative
the help of an occupational therapist and rehabilitation
course after colostomy with concurrent large pressure
nurse. This technique has been in widespread use for per-
ulcers has been reported as high as 15 percent of patients
sons with spinal cord dysfunction caused by spinal bifida
in a small series (153).
(144146).
Colostomies in use by people with SCI effectively
Recently, there have been a few case reports of the
reduce the burden of care and can contribute to quality
Malone procedure used on adults after traumatic SCI
of life (151,156). Total bowel care time is reduced, as is
(147,148). In a single subject design, it was demonstrated
incontinence episodes, when compared with these aspects
that the use of the antegrade continence enema to trigger
of the bowel program outcomes before colostomy
bowel care can reduce the time required by 50 percent (2
(136,140,151,153). The best outcomes are achieved with
hours to 1 hour). The bowel care procedure with the ante-
careful patient selection and an individualization of the
grade enema requires the infusion of 200 to 500 cc of nor-
procedure to meet person-centered needs and capabilities.
mal saline into the stoma through a catheter. Peristalsis
Deliberations over diversion of the fecal stream, there-
begins minutes thereafter and bowel care is then carried
fore, require include a broad assessment of the bowel pro-
out with the steps described for the upper motor neuron
gram, bowel care, personal assistance, body image, and
bowel (Table 25.7). Once the technique is established, a
lifestyle issues (1,91). Should the decision for fecal diver-
variety of laxative and stimulant medications previously
sion is made, the surgical procedure and the location of
used to trigger bowel care can be removed from the bowel
the stoma require interdisciplinary assessment including
program (147). Long-term results in adults with trau-
primary rehabilitation nursing and occupational therapy
matic SCI after the Malone procedure have not been as
(102). In our experience, a higher placement of the stoma
favorable as in children with spina bifida. As many as 95
allows for better visualization and self management (78).
percent of children continue to use their stoma success-
fully for bowel care at 6 years, whereas only 77.5 per-
cent of adults continue to use the antegrade enema tech- Acknowledgments
nique after a stoma is fashioned (149). This may be The authors would like to recognize and thank some of our col-
caused by chronic overdistention of the colon and poor laborators who have contributed to the current knowledge and
response to the irrigation and/or spastic external anal methods for treatment of patients with gastrointestinal dys-
sphincter contraction, which retains stool (149). function, summarized in this chapter: Susan Beiner-Bergman
THE GASTROINTESTINAL SYSTEM AFTER SPINAL CORD INJURY 345
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28. Colachis S, Murray K. Esophageal perforation: a delayed com-
plication following traumatic spinal cord injury. Case report.
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26
Sexual Dysfunction and
Infertility in Men with
Spinal Cord Disorders
he majority of traumatic spinal cord to injury will dictate their readiness to explore and adapt
TABLE 26.1
Sexual Functioning Self-reported in Men with Spinal Cord Injuries
According to Level and Completeness of Injury (1)
SUCCESSFUL CHILDREN
ERECTION INTERCOURSE EJACULATION SIRED
lower motor neuron (LMN) injuries. However, self- ery (noradrenaline, for example) or vice versa. Such com-
reports do not always accurately reflect the physiologic plexity helps explain the wide variation of sexual effects
responsiveness to erotic stimulation. For example, penile of medications and emotions on sexual functioning. The
tumescence was demonstrated in male SCI patients who role of the various nervous systems will be explained in
believed they could not get erections when they were greater detail under each specific sexual function.
exposed to erotic stimulation via film, text, and fantasy Sexual triggers from the brain (auditory, visual, gus-
(2). There is no evidence to suggest that there is any dif- tatory, and olfactory inputs, and imaginative or erotic
ference in mental arousal capacity pre- and post-SCI. stimuli), modulated by appropriate hormonal influences,
However, interpretation of, and acting on, sexual signals are integrated with sensory afferents from skin and vis-
post-injury may be different. cera. A generated neuronal signal is coordinated in the
Since ejaculation is generally reported in less than 10 midbrain and carried distally through the brainstem and
percent of men with SCI, fertility is very affected. Fortu- spinal tracts. Although the brain has a regulatory role (it
nately, with the advent of vibrostimulation and electroe- exerts both excitatory and inhibitory control on the spinal
jaculation, semen retrieval is virtually always possible. reflexes for sexual function), it is practical to think of the
Unfortunately the semen quality is often poor (good sperm efferent outflow as primarily inhibitory. It is the removal
count but high abnormal forms and poor motility), and of this inhibition that allows for downgoing signals to be
assisted insemination is necessary in most cases. relayed to the various spinal nuclei, and in some cases,
Before embarking on describing the changes fol- release specific spinal reflexes. However, these spinal
lowing SCI, it is important to understand what is known nuclei can also receive afferent information from the gen-
to date about sexual functioning in those men without italia and can also initiate motor output by activating
SCI. This knowledge is still limited, and may be altered spinal cord systems directly (3). Those men with SCI and
in the future to accommodate new findings of signal concomitant brain injury may not have the same degree
transmission. of cerebral modulation, thus causing some sexual func-
tions, such as erection and ejaculation, to occur inap-
propriately. In addition, particular brain injuries may
cause sexual dysfunctions additive to the SCI, including
SEXUAL NEUROPHYSIOLOGY OF
sexual disinterest or hypersexuality.
THE ABLE-BODIED MALE
and smooth muscle relaxation (Ach, NO, VIP, PGE1, cal- lesions between the two pathways would seem to be
citonin gene-related peptide [CGRP] ). Available (and spared both psychogenic and reflex erections; however,
future) pharmacotherapy utilizes these known modula- the synergism is lost between the two pathways and erec-
tors by increasing the erectogenic ones and/or inhibiting tions are not clinically as reliable as one might expect.
the erectolytic ones, alone or in combination. For exam- Those men with conus terminalis lesions may be spared
ple, PGE1 (alprostadil), delivered via intracavernosal psychogenic erections because of intact thoracolumbar
injection or intraurethral micro-pellet, relaxes corporal fibers. The lower motor lesions seen in cauda equina
smooth muscle, promoting erection directly. Oral silde- injuries result in variable loss of function, from complete
nafil is a selective inhibitor of type 5 (cGMP-specific) (T12L1 injury results in loss of psychogenic and reflex
phosphodiesterase, a dominant isoenzyme that destroys erections because of inclusive damage below the level of
cGMP. Sildenafil (Viagra) works indirectly by extending injury) to partial (S4S5 injury retains psychogenic erec-
the duration of available cGMP in the penile tissues, thus tion and even partial reflexogenic capacity) (12).
enhancing the erection. However, unlike PGE1, sildenafil Although we can expect men with complete injuries
requires sexual arousal to liberate NO from its non- above neurological level T10 to have reflexogenic erec-
cholinergic, nonadrenergic nerve endings (NANC). Dis- tions, and those with sacral reflex arc damage to be capa-
ease states and aging generally influence the balance ble of psychogenic erections, there are numerous clinical
towards flaccidity because of such things as decreased exceptions to these predictions, as noted in the literature
smooth muscle/connective tissue balance from lack of review. This may be because of inaccurate assessment of
oxygenation, decreased cell-to-cell contacts, sinusoidal the level of injury or completeness, unrecognized
endothelial changes (endothelium is a source of NO), and syringomyelia, or because of unknown alternate erection
degenerative changes in nerve fibers (10). It is under- pathways. Obviously, more study must be done in this
standable how hypertension, hyperlipidemias, smoking, area. It is also important to remember that there may be
diabetes, and nerve disorders are common risk factors for other reasons (depression, use of antispasmodic medica-
erection dysfunction (ED). tions, concomitant diabetes, etc.) for ED in men with SCI
Androgens are necessary in the development (orga- in addition to neurologic changes.
nization) and maintenance (activation) of sexual function. In addition, the definition of erection is not consis-
Androgens precise role in erection is not well delineated tent in the literature. In clinical practice, many men with
other than androgens are involved in the role of nitric SCI who are able to obtain an erection do not have
oxide synthase (NOS) and therefore, NO production usable erections. Most have either difficulty maintain-
(11). Absolute required levels of androgens are not ing their erections or cannot attain a second erection if
known, because some men retain erectile capacity despite the first is lost. Some may have detumescence triggered
low levels of serum testosterone. Physiologic erections by uncontrolled seminal emission or may not have a rigid
that occur during REM sleep decline rapidly when andro- or sustainable enough erection for penetration. Many
gen levels drop, but there is a slower decline in the loss men chose to use erection enhancement to avoid the frus-
of erotic or daytime erections. The most consistent sex- tration of unpredictability.
ual symptoms of hypoandrogenemia are lack of sexual Traditional methods of ED investigation may also
interest, followed by delayed ejaculation or orgasm, not be applicable to the SCI population. For example,
smaller ejaculate volumes, loss of nocturnal erections, and despite some controversy, in the able bodied, abnormal
eventually, ED. Endocrinologic ED per se accounts for NPT findings have often been used to differentiate
only 2 to 5 percent of all ED. Hypogonadal men, how- organic from psychologic causes of ED, assuming the
ever, require androgen replacement. psychologic causes of ED when awake and sexual cause
supraspinal inhibition and would be bypassed in sleep,
thus resulting in normal NPT tracings. Organic causes
Changes to Erection Following SCI
(nerve, vascular, or other end organ problems) would
Because both spinal and supraspinal inputs act synergis- result in abnormal tracings. However, one author and col-
tically in sexual functioning, it follows that one system leagues (12), using SCI subjects, proposed and confirmed
can also compensate for the other with variable success the hypothesis that nocturnal erections were primarily of
(11). The SCI population particularly evidences this. Men reflexogenic origin transmitted via the sacral pathway.
with complete SCI above the thoracolumbar outflow (T6) They showed that positive NPT recordings were primar-
who cannot transmit their psychogenic input from the ily among those SCI subjects who maintained reflexogenic
brain to the sacral levels, should have their reflex erec- erection capacity. Moreover, subjects with impaired
tions preserved, or even enhanced (no cortical inhibition) reflexogenic erections generally lost nocturnal erections
(11). Those with sacral damage have abolished reflexo- even if they maintained psychogenic induced erections.
genic response but intact thoracolumbar pathways in They went on to suggest that NPT recordings should,
which to mediate a psychogenic erection. Men with therefore, be used with caution in the SCI population.
354 MANAGEMENT OF THE BLADDER, BOWEL, AND SEXUAL DYSFUNCTION
satile ejaculation if the parasympathetics or somatics are injury that spares some or all of these tracts necessary for
disrupted. In the latter case, semen may slowly be released genital orgasm increases the chance of that person expe-
with gravity over time or go unnoticed in a urinary col- riencing a familiar pelvic-centered orgasm, even if the
lection device. Because the majority of men with SCI do required stimulus may have to be strong or for a pro-
not report seeing antegrade ejaculate or being orgasmic, tracted period of time. Interestingly, in one study (16) 38
they are usually considered anejaculatory. However, some percent of men with complete SCI reported they retained
of these men may have retrograde ejaculation. the ability to perceive orgasm. The topic of orgasmic
In those men with UMN lesions above T10 and loss potential and modulating factors requires further study.
of supraspinal control, the potential for complete ejacu- Clinicians working in the area are aware of those
lation remains because of an intact T10S4 cord. Sperm men with SCI who are able to experience altered orgas-
retrieval methods are able to use this potential and are mic sensations, whether the afferent input is from the gen-
frequently successful at obtaining an ejaculate. However, italia, non-genital areas of sensitivity, or the brain itself.
those men with complete lower injuries involving the It is this authors experience with sperm retrieval meth-
sacral cord, although maintaining a higher potential for ods that about 5 to 10 percent of men undergoing these
ejaculation with sexual practices, may also experience procedures may experience some pleasurable sensations
unwanted seminal emission and resultant detumescence for the first time, even if this was not predicted by tradi-
from stimulation of the thoracolumbar pathways tional neurologic understanding.
involved with psychogenic erections. Unfortunately, they
are, in effect, unable to use the sacral reflex pathways for
either erection or pulsatile ejaculation, although seminal PRESENTATION AND EVALUATION OF
emission may be automatically stimulated as long as SEXUAL CONCERNS
erotic thoughts, required to attain the erection, prevail.
The incompleteness of injury likely spares some Doctors and other healthcare professionals must take
downgoing messages and the coordination of the vari- responsibility for actively addressing sexual concerns in
ous nervous systems. As noted in the literature review, men with SCI. Sexual health should be addressed with the
those men with incomplete LMN were most likely to ejac- same priority as issues about mobility, or bladder or
ulate, because the thoracolumbar and/or sacral reflexes bowel control. Although a man with SCI may ask directly
may be partially spared. However, although those men about sexual functioning and fertility, he will more often
with complete UMN lesions are the least likely to ejacu- wait for questions to be directed to him. Unspoken or
late through private sexual practices, they are the most unattended queries about sexual concerns will present
likely to ejaculate with specific stimulation of the glans themselves in numerous ways in a rehabilitation setting.
penis by vibrostimulation (which requires intact lum- He may turn to the nurse or occupational therapist for
bosacral reflexes). Those with high incomplete lesions some indirect but encouraging word about sexual attrac-
may have better luck than their complete counterparts tiveness or functioning. A physiotherapist may face
in private sexual experiences, but the cerebral interference queries about the ability to manage certain positions that
in a clinical, asexual, and potentially embarrassing situ- simulate sexual acts. These are the male patients efforts
ation may inhibit the reflex. Electroejaculation on the to have this area addressed and these questions may be
other hand, electrically stimulates only the peripheral interpreted as inappropriate, or be rejected or even pun-
nerves eliciting seminal emission, and therefore does not ished. It is more helpful to determine what provoked the
require coordination within the spinal reflex centers. question in the first place, so that respectful information
Men with incomplete lesions of the spinal cord may and reassurance can be delivered.
have the potential of orgasm if the pathways described Because sexuality and fertility are legitimate areas of
above for genital orgasm remain open. Self-report stud- rehabilitation, it is far more effective to have these areas
ies of men with SCI state that 42 to 47 percent report addressed up front. After an assessment of patient readi-
orgasms of a similar, weaker, or different quality from ness and interest, a discussion of various treatments for
those pre-SCI (14,15). Although it is assumed that those erectile and ejaculatory dysfunction should occur early on
men with complete lesions cannot experience genital in the rehabilitation process, even if they are not an imme-
orgasm (since both the upgoing lateral spinothalamic diate concern. The knowledge that it is possible to be sex-
tract and the downgoing corticospinal tract are inter- ually functional and to potentially father a child will pre-
rupted), 38 percent of men with complete SCI reported vent damaging secondary consequences of ignorance and
they retained the ability to achieve orgasm (14). There misinformation. More detailed discussions can then be
may be several explanations for this, including an inac- undertaken when appropriate.
curate assessment of completeness of injury via tradi- A thorough sexual history and a physical examina-
tional methods of testing and definitions as it applies to tion are essential in the evaluation of sexual and fertility
deep internal genitalia responses. An incomplete spinal concerns. The reader is referred to articles for a full
356 MANAGEMENT OF THE BLADDER, BOWEL, AND SEXUAL DYSFUNCTION
most benefit to those men with SCI who have retained some men still prefer to use intracavernosal injections
their reflex erections (generally UMN lesions) and who because maximal rigidity is attained. Because of its phys-
do not also have other risk factors. Three studies have iologic activity, sildenafil has a very low risk of priapism.
been published stemming from the approval process of However, it should not be taken in combination with
the drug (1921). In these studies, those men who were other pharmaceutical methods of erection enhancement
able to get a reflex erection in response to vibratory stim- until clinical studies have been done.
ulation responded to sildenafil (80 percent compared to Sildenafil has been on the market since March 1998
placebo of 10 percent). Even though men without reflex in the United States and was approved a year later in
erections did not fare as well, they still noted an improve- Canada. Viagra is marketed in 25, 50 and 100 mg
ment in their erections. The most common side effects (15 tablets: in the SCI population, 25 mg should be tried first
percent and below) were headaches, vasodilatation (flush- because of sildenafils hypotensive effects. Higher doses
ing and rhinitis), dyspepsia, and blue-tinged vision at are required in those men with more compromised NO
higher dosage levels, similar to those seen in able-bodied sources or more severe ED. Sildenafil should be taken 12
subjects. Sildenafil drops the blood pressure on average to 1 hour prior to planned sexual activity. Sexual arousal
by 8.0 mmHg systolic and 5.5 mmH in the clinical trials is required, but men with SCI may also require direct gen-
in able-bodied men, but this was thought not to be of sig- ital stimulation to encourage the peripheral release of
nificance. In the trials of men with SCI, there was no evi- NO. Sildenafil (or any erection enhancement method)
dence that the alterations in blood pressure were clinically should not be used in those men in whom sexual activity
relevant, but it should be noted that those men with itself is deemed unsafe (a caution that applies particularly
chronic hypotension or with pressures of 80/50 mmHg to cardiovascular patients), and cannot be taken with
were excluded from the trials (Pfizer data on file). Until nitrates in any form (as mentioned above). Sildenafil has
further studies are conducted, this medication should be not been found to add to the risk of cardiac events or
prescribed with caution for men with injuries at T6 or stroke in able-bodied men who do not take nitrates.
above for several reasons. The low blood pressure that The use of other oral medications such as yohimbine
the majority of these men have could be exacerbated with and trazadone is basically ineffective in this population,
the hypotensive effects of sildenafil. Additionally, because with the exception that trazadone may cause priapism.
of their level of SCI, they are also at risk of autonomic However, future oral medications hold some promise, as
dysreflexia, and symptoms of autonomic dysreflexia are shown in Table 26.2.
similar to the side effects of sildenafil (headache and flush-
ing). Because nitrates are sometimes used to treat auto-
nomic dysreflexia, one must be sure that a person is expe- Local Penile Delivery Systems
riencing autonomic dysreflexia and not a side effect of
sildenafil. If the person has ingested sildenafil within the PENILE INJECTIONS. Intracavernosal injections
past 24 hours, then nitrate administration is contraindi- have been the gold standard in treating men with SCI
cated, because nitrates can potentiate the hypotensive because of the reliability and rigidity of the erection
effects of sildenafil and cause severe hypotension. produced by the injection. Alprostadil or PGE1 (or
Overall, it is generally found that sildenafil improves sometimes papaverine, phentolamine, or combinations
erections in men with reflex erections (attainability, rigid- thereof) itself is relatively safe in the correct dose.
ity, and maintenance) but that it has a lesser effect on Alprostadil is commercially available in kits, such as
those men without reflex erections. Some men are unable Caverjet (see Table 26.2). The invasiveness of the
to take it because of the side effects, including dizziness. technique and the potential for intracavernosal calcium
Others find the dizziness transient and acceptable. deposition and fibrosis (especially with papaverine) or
Sildenafil only influences the erectile ability and tunical scarring (penile curvature) from the injection
overall sexual satisfaction, and does not increase libido method is a drawback. Penile pain or aching associated
or lead to ejaculatory or orgasmic changes. However, the with alprostadil is not usually an issue in SCI population,
data from the clinical trials of men with SCI suggested although sensate men with neurogenic ED are
that some men experienced an improvement in their ejac- particularly sensitive. Dose titration should be done
ulation and orgasmic ability, although this was not sta- carefully and slowly, starting with no more than 2 g (0.1
tistically significant. It could be speculated that these cc of a 20 g/ml solution), because of the higher risk of
improvements were probably caused by increased psy- prolonged erection and priapism in this neurogenic
chogenic input that may have maximized their potential population. The latter is likely caused by denervation
functioning. Not all men with SCI prefer sildenafil, even hypersensitivity (22). The medication must be accurately
if effective, because cost is a factor for some, as is the pref- delivered to one corpusa (it will diffuse unaided to the
erence for other methods. For example, although erec- other corpus) by a short, fine (2830 gauge) insulin
tions with sildenafil are usually adequate for intercourse, needle. The technique must be carefully shown to the
358 MANAGEMENT OF THE BLADDER, BOWEL, AND SEXUAL DYSFUNCTION
TABLE 26.2
Current and Future Products for Erection Enhancement
CURRENT
FUTURE
patient and demonstrated by the patient before a pre-Viagra era, it was reported that after 2 years of use,
prescription is given. Alternatively, the method can be approximately 50 percent of SCI patients would drop
taught to a partner if limited hand function or poor out of the injection method (23). This authors survey
visibility makes this method impractical for the client. shows a smaller percentage ( 20 percent). This find-
In addition to the technical difficulties and the risk ing probably reflects the nursing time involved in patient
factors associated with injections, the quality of erection training, troubleshooting, and watching the patient
makes it an attractive method, especially in the younger demonstrate the actual injection. The use of autoinjec-
male SCI population who wish a maximally rigid erec- tors can be helpful for those men with needle phobias
tion, or who are reluctant to try more cumbersome or poor hand function.
mechanical methods. In terms of efficacy, only those men
with significant arterial input difficulties or who have a INTRAURETHRAL MICROPELLET (MUSE). Alpro-
large venous leak dont respond well to this method. stadil or PGE1 can also be administered as a tiny pellet
Slowly increasing the dose (up to 20 g), or the use of into a straightened urethra that is lubricated with urine.
combination mixtures, may improve the efficacy (i.e., The absorption of the medication is encouraged by
Trimix, which consists of alprostadil, papaverine, and rolling of the penis for at least 1 minute and standing
phentolamine, or Quad mixes that add other alpha- or sitting (not lying down) after insertion. Occasionally,
antagonists to the mixtures). Injections should not be the stimulus of the applicator into the urethra may cause
done more than once in 24 hours and not more than 3 reflex voiding, and the medication will be washed out.
times per week to reduce the complication risk. In the In general, this method is easier for most quadriplegics
SEXUAL DYSFUNCTION AND INFERTILITY IN MEN WITH SPINAL CORD DISORDERS 359
who are able to self-catheterize than is the injection terity is required, although there are battery-operated
method, which requires better hand function. Because of models for those with poor hand function. Because the
the potential long-term side effects of using an invasive erection is external only, the penis tends to swivel at the
method such as injection, intraurethral alprostadil has base. The constriction ring may also cause pain or block
a role in men with SCI. However, one study (24) noted ejaculation, but this is not usually a concern in the SCI
that the erection quality, especially when compared to population. Vacuum devices have the advantage of
intracavernosal injection, was poor and that the highest being able to be used more than once in a 24-hour
dose was required to obtain any tumescence. Because this period, and can also be used as a back-up method when
method is reliant on vascular connections between the other enhancement methods have failed. They can also
corpus spongiosum and cavernosal bodies and the be used in men with penile implants and in those who
production of a pressure gradient that encourages have had implants removed.
retrograde venous flow, it is likely that this transference
of medication from the spongiosum to the cavernosum
Surgical Solutions: Penile Prosthesis and Sacral
is less in the wheelchair population. Furthermore, the
Anterior Root Stimulator Implant (SARSI)
urethral mucosa itself may be less likely to absorb the
medication when it has been repeatedly catheterized. Penile prosthesis, especially in a population with sensory
Because of the route of administration, doses are loss, should only be done in those patients in whom the
several-fold that of intracavernosal injections (250, 500, reversible methods have failed or who require penile
and 1000 g). There is also a higher risk of the medica- straightening procedures or reconstructive genitourinary
tions becoming systemic because of venous absorption surgery. Men with SCI experience a much higher infec-
and outflow, causing hypotension. A constrictor band tion and erosion rate with these devices when compared
placed at the base of the penis prior to the MUSE admin- to nonneurologic patients (26), although surgical tech-
istration can minimize this risk and may be essential for niques and the devices themselves have improved con-
efficacy when using this method in the SCI population siderably. Because these devices are placed in the spongy
(24). It is this authors experience that symptomatic tissue of the corpora cavernosal bodies, much of this tis-
hypotension has been less of a clinical issue (hypotension sue is permanently destroyed. This precludes the use of
is also reported in the able-bodied population) than effi- other erection enhancement techniques, except the vac-
cacy. In summary, MUSE has not been well evaluated uum device.
in this population. The addition of erectogenic drugs such Devices are either malleable, semi-rigid, or multi-
as prazosin to alprostadil may well improve the efficacy. component (two- or three-piece) inflatable prostheses.
MUSE can be used up to twice in a 24-hour period. Although the former is less esthetically appealing, the lat-
ter is more prone to malfunction. Although the proce-
dures are primarily done as a permanent solution to sex-
Mechanical Methods
ual difficulties, they have been helpful for bladder
management as well (maintaining condom drainage sta-
CONSTRICTION BANDS AND VACUUM DEVICES. bility) and can be very acceptable.
Many men with SCI can attain a good erection but not The Brindley Finetech SARSI is successful for con-
maintain it for the act of intercourse. A simple, proper trol of micturition with continence in more than 80 per-
medical constriction band at the base of the penis may cent of one series of men who have had the device
assist with this. The ring should not be left on for more implanted. Because the implantation requires the division
than 30 minutes because of venous stasis. Falling asleep of the sacral posterior roots, there is a consequential loss
with the ring on may not provoke typical pain responses of reflex erections: only 60 percent of patients were able
in men with SCI, although serious corporal damage will to use implant-driven erection (27). A similar device,
likely not be experienced unless the penile tissues are VOCare, had received FDA approval and was available
anoxic for several hours. The removal of the ring is aided in the United States.
by wings or pull-tabs attached to the sides, but still
requires some strength and dexterity of the hands.
Future Therapies
Vacuum devices (VD), consisting of a cylinder, a
pump to create a vacuum, and a constriction ring, are Future oral medications include other PDE5 inhibitors
a noninvasive, nonpharmaceutical, and successful that facilitate erections with fewer side effects (or are
method of attaining (via the vacuum) and maintaining more convenient to take), and centrally acting drugs that
(via constriction band) an erection. In one study of SCI initiate erections via dopamine receptor agonists (apo-
men with ED, 41 percent were satisfied with the use of morphine) or adrenergic receptor antagonists (phento-
a VD and 60 percent reported improvement of their sex- lamine). Local erectogenic neurotransmitters (or the sup-
ual relationships (25). A fair amount of manual dex- pression of erectolytic neurotransmitters alone or in
360 MANAGEMENT OF THE BLADDER, BOWEL, AND SEXUAL DYSFUNCTION
combination),will be administered in injection, topical, The most important point to communicate to the
or urethral forms and even via gene therapy, as shown in man or couple is with todays technology, poor semen
Table 26.2. To date, there is no specific information on quality does not preclude biologic fatherhood. However,
these therapies in the SCI population. discussions with couples planning a family should
include, up front, alternatives such as donor insemina-
tion, adoption, finances, and topics such as philosophies
FERTILITY FOLLOWING on medical intervention.
SPINAL CORD INJURY The most common methods of obtaining ejaculates
from men with SCI make use of the neurophysiology
It is well recognized that fertility in men is affected fol- explained earlier. Pharmaceutical methods enhance the
lowing SCI. The majority of ejaculatory disorders are neu- ejaculatory reflex at either an efferent or supraspinal
rogenic in etiology, and result in anejaculation or retro- level. Methods such as vibrostimulation (VS) apply a
grade ejaculation. Rarely, post-injury epididymitis can strong afferent stimulation to cause a triggering of the
result in an obstructive cause of infertility. Furthermore, efferent reflex via spinal cord intermedularies. Electroe-
semen quality invariably deteriorates after SCI, likely jaculation (EE) jump starts the distal efferent fibers of
within the first few weeks after injury, with sperm motil- the reflex arc (and of some surrounding musculature),
ity most affected. The exact cause of this is unknown, but or at least results in a contraction of the seminal vesi-
it has been suggested that stasis of prostatic fluid, alter- cles and terminal vasa (34), causing an emission. It is
ations of the seminal fluid composition, higher free rad- accepted practice that, in those men with injuries above
ical composition, higher testicular temperature, recurrent neurologic level T12 and should be tried if any genital
urinary tract infections, exposure of sperm to urine in ret- reflexes or sensation is present, because it is simpler, less
rograde ejaculates, abnormal testicular histology, altered invasive, and can be easily repeated at low cost. Occa-
hypothalamic-pituitary-testicular antisperm antibodies, sionally, VS may work on men with incomplete injures
and the long-term use of various medications could be below T12 and should be tried if any genital reflexes are
causative (2830). Bladder management also appears present or if some sensation is present. On the very rare
important to semen quality, with intermittent self- occasion that both VS and EE fail, operative sperm
catheterization resulting in better semen quality from elec- retrieval, which is independent of nerve pathways or
troejaculated samples (31). Excepting the first few weeks intact structures, can be used. Ease of semen retrieval,
after injury (30,32), semen quality does not seem to cor- semen quality, female fertility potential, and type of
relate with duration of injury. Semen parameters have assisted reproductive measures required (and their
been shown to improve with repeated ejaculations fol- affordability) will determine pregnancy rates.
lowing 3 to 6 months of weekly vibrostimulation (33). Other nerve stimulation techniques (such as
Regardless of these issues, biologic fatherhood is a real transperineal electroejaculation and hypogastric plexus
possibility in men with SCI because of successful treat- stimulators) and implanted capsules connected to the vas
ments for ejaculatory dysfunction and the use of assisted deferens for direct sperm aspiration, have not been as
reproductive technology (ART). successful. For men with SCI, between the techniques
of EE and VS, semen collection is virtually assured. VS
should always be tried first before EE. To date, EE has
Treatment of Ejaculatory Dysfunction
not been routinely done at home, nor is it as readily
There are two main reasons men with SCI seek assistance available or affordable as VS, and it requires the use of
with ejaculatory dysfunction: they want to know if and ART in some form. Pregnancy rates generally reflect
what it would take to be able to ejaculate (either for a that.
personal self-esteem, or sexual or reproductive reason), Sperm retrieval methods in those with injuries at or
or what their realistic fertility potential is. Knowing their above T6 should only be attempted by a medical team
fertility potential removes the question that will well trained in treating autonomic dysreflexia (AD). AD
undoubtedly be asked by a partner in a future relation- usually occurs near or at an ejaculation, so that with the
shipcan you ever father a child? If men want to use of VS and EE (unless the latter is used with spinal or
actively pursue biologic fatherhood, assessment deter- general anesthesia), precautionary methods must be prac-
mines whether there is live spermatozoa in the ejacu- ticed. Preprocedural antihypertensive medication, such as
late, and if so, what would be expected in terms of tech- nifedipine (10 to 20 mg taken 20 minutes prior to the pro-
nical assistance to have a partner conceive. Except for cedure), or prazosin (1 mg taken 12 hours and 1 hour
those men who require fertility investigations for before the procedure, respectively), can be used for pro-
medicolegal purposes, most men will pursue sperm phylaxis if anesthesia is not used. AD is controlled by
retrieval options when they feel ready or hear of an spinal or general anesthesia by interfering with the uncon-
appropriate center to investigate this area. trolled sympathetic discharge. During VS and EE proce-
SEXUAL DYSFUNCTION AND INFERTILITY IN MEN WITH SPINAL CORD DISORDERS 361
dures, autonomic dysreflexia should be watched for (and after a 1- to 2-minute rest interval. Four to six cycles
can often be minimized) by continuous blood pressure should be tried before abandoning the procedure: how-
monitoring and observing and listening to the patient, and ever, most positive responders do so within 1 to 2 cycles.
decreasing or stopping either the vibratory stimulus or Vibrostimulation is both an art and a science. Spe-
stimulating current when the man begins to exhibit signs cific vibrator speeds and amplitudes are more likely to
of AD. induce the reflex. Sonksen et al. (37) demonstrated that
the optimal peak-to-peak amplitude of a vibrator was 2.5
mm at a speed of 100 Hz, and this has been confirmed
Pharmaceutical Methods
by several other investigators (38). Snksen and col-
Unlike ED, there is not much in the way of neurotrans- leagues were instrumental in the development of a Food
mitter replacement or pharmaceutical treatment to com- and Drug Administration (FDA) registered device (FERTI
pensate for the neurologic deficits seen with ejaculatory CARE) that can reliably deliver this but can also be
disorders in men with SCI. Sympathomimetic drugs such adjusted to vary the amplitude and Hz to meet particu-
as pseudoephedrine can potentiate remaining efferent lar patient needs (see Figure 26.4). Store-bought vibrators
fibers to perform their role in seminal emission and clo- may be powerful enough, but most require rewiring to
sure of the bladder neck. If retrograde ejaculation is deliver higher amplitude. The art consists of the effective
occurring because of nerve damage or bladder neck placement and pressure of the vibrator on the glans, with
surgery, the use of sympathomimetics may result in ante- appropriate rest periods to avoid exhausting the reflex,
grade ejaculation. Because of the action of sympathomi- and reading the patient for signs of impending ejaculation
netics on the sympathetic nervous system, blood pressure while monitoring for autonomic dysreflexia. Most inves-
will invariably be increased and could cause urinary reten- tigators agree that trigger spots on the glans can be used
tion in some patients (30). The use of sympathomimetics to reliably elicit the reflex: these may be at the frenulum,
to promote antegrade ejaculation is fairly successful in the but are often found on the dorsum of the glans also. Some
testicular cancer population who have undergone surgi- men may require the use of one vibrator on the dorsal and
cal sympathectomies during their retroperitoneal lymph another on the ventral surface of the glans. Positive signs
node dissections. However, the use of sympathomimetic include abdominal and leg spasms, testicular elevation,
drugs for enhanced sperm retrieval should be approached and generalized piloerection. Tonic spasms, periurethral
with caution in the SCI population until AD is ruled out contractions, and an acute distension of the glans (with
or prophylactically controlled. or without an erection) just prior to ejaculation (37,38)
Two methods of inducing ejaculation, intrathecal are premonitory to ejaculation. Sometimes multiple ejac-
neostigmine (35) and subcutaneous physostigmine (36), ulations can be safely done at one sitting. The VS proce-
have been less popular because of invasiveness and/or the dure is more fully explained in other readings (34,3739).
severity of side effects, including a death from cerebral The efficacy of VS has been reported in the range
hemorrhage in the former. The reader is referred to a good of 24 to 96 percent (38), but higher rates are no doubt
review of the use of pharmacologic agents that can be related to proper vibrator amplitudes and clinician expe-
used to induce an ejaculation (30). rience. In general, it is the authors opinion that clinics
using proper VS today should operate at an average of 75
percent success rate for those men with lesions above neu-
Vibrostimulation
rologic level T10, with the higher cervical lesions faring
Vibrostimulation (VS) is the first line of treatment for ane- the best. Patient positioning, degree of bladder fullness,
jaculatory men with SCI. VS involves the application of and concurrent bedsores or bladder infections may affect
a high-speed, high-amplitude vibrator to the glans penis the efficacy of VS. VS is noninvasive and simple, with the
to induce ejaculation via an intact ejaculatory r eflex (see major side effect being AD. Occasionally self-limited
Figure 26.3). The lumbosacral autonomic and motor minor chafing of the glans or distal penile edema may be
nerves must be relatively intact, even if there is sensory experienced with prolonged VS.
loss. With other etiologies, if the man can reach orgasm Semen retrieval with VS can be done at home dur-
on his own, it can be predicted that VS will not increase ing ovulation (timed with ovulation predictor kits, for
the chances of ejaculation. An erection may or may not example) if semen quality is adequate and AD is con-
be present prior to ejaculation, particularly if the reflex trolled. If AD is a risk, or if the patient requires better
is fast. In men with SCI, an accompanying orgasm is equipment or clinician experience, VS is done in a med-
unlikely (see the section on orgasm). Although the vibra- ical clinic setting. Intrauterine insemination (with or with-
tor may induce an erection before ejaculation, it is also out ovulation induction) or other assisted reproductive
not unusual to lose a reflex erection with the stimulus. techniques, such as in vitro fertilization (IVF) and intra-
In any event, ejaculation is commonly triggered within 1 cytoplasmic sperm injection (ICSI), can be employed
to 5 minutes of the vibrator being applied or reapplied depending on semen quality and practicalities such as
362 MANAGEMENT OF THE BLADDER, BOWEL, AND SEXUAL DYSFUNCTION
Electroejaculation
VS failure leads to the need for rectal probe electroejac-
ulation to induce seminal emission. There is likely direct
FIGURE 26.3 sympathetic stimulation to the prostate gland and semi-
nal vesicles, with indirect sympathetic stimulation to the
The inexpensive, store-purchased WAHL vibrator has been cauda epididymis (see Figure 26.6). Whereas VS evokes
rewired to deliver high amplitude. The bell shaped cup dis- physiologic neurotransmitter release via the spinal cord
tributes the stimulation over most of the glans simultaneously, ejaculatory reflex and turnover of the seminal contents,
and does not diminish intensity applied to the penis. (WAHL
EE would appear to artificially stimulate this release, pro-
Model 4196, Div Swenson Canada, Inc., 80 Orfus Road,
Toronto Ontario, M6A 1M1).
ducing a resultant contraction of the internal ejaculatory
structures and the expulsion of the stored semen in their
possession. This may partially explain the recognized bet-
ter semen quality with VS than EE (42).
A polyvinyl chloride probe with three stainless steel
electrodes is inserted into an emptied rectum. The current
is administered through the electrodes that are placed
firmly against the prostate. AC current generation is con-
trolled by the operator, and probe temperature regulation
FIGURE 26.4
FIGURE 26.5
The FERTI CARE Clinic vibrator is commercially available
for use in sperm retrieval clinics, and is adjustable in speed The Seager Electroejaculation Stimulation Equipment and
and amplitude. The vibrator is applied to the frenulum until Rectal Probes (Distributor: NeuroControl Corporation, 8333
ejaculation occurs. (Multicept APS, Rungsted, Denmark). Rockside Road, 1st floor, Valley View, Ohio 441256104.)
SEXUAL DYSFUNCTION AND INFERTILITY IN MEN WITH SPINAL CORD DISORDERS 363
neous epididymal sperm aspiration (PESA), or testicular 11. Giuliano F, Rampin O, McKenna KE. Animal models used in the
study of erectile dysfunction. In: Carson C, Kirby R, Goldstein I (eds.),
sperm extraction (TESE). Erectile Dysfunction. Oxford: ISIS Medical Media, 1999; 4349.
12. Coutrois FJ, Charvier KF, Leriche A et al. Clinical approach to
erectile dysfunction in spinal cord injured men: A review of the
CONCLUSION clinical and experimental data. Paraplegia 1995; 33:628635.
13. Shaban SF. Treatment of abnormalities of ejaculation. In: Lipshultz
L, Howards S (eds.), Infertility in the Male. St. Louis: Mosby Year
The availability of choices open to men with SCI and their Book, 1991; 409427.
partners in the field of sexual rehabilitation and fertility 14. Alexander CJ, Sipski ML, Findley TW. Sexual Activities, desire,
and satisfaction in males pre- and post-spinal cord injury. Arch
has improved immensely over the last 10 years and will Sex Behav 1993; 22(3):217228.
continue to do so with the exponential growth in knowl- 15. Phelps G, Brown M, Chen J et al. Sexual experience and plasma
edge of male sexual physiology. Furthermore, the avail- testosterone levels in male veterans after spinal cord injury. Arch
Phys Med Rehabil 1983; 64:4752.
able fertility opportunities are also rapidly expanding as 16. Alexander CJ, Sipski ML, Findley TW. Sexual activities, desire and
technology improves. satisfaction in mates pre- and post-spinal cord injury. Arch Sex
Sexual health care is an integral part of any reha- Behav 1993; 22(3); 217228.
17. Szasz, George: Sexual health care. In: Zejdlik C (ed.), Manage-
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ity issues. Healthcare professionals working with men 18. Sipski ML. Spinal cord injury and sexual function: An Educational
Model. In: Sipski M, Alexander C (eds.), Sexual Function in Peo-
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gle dose, two way cross-over study using Rigiscan. J Spinal Cord
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V
NEUROLOGIC ASPECTS OF
SPINAL CORD CARE
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27 Neurologic Assessment of
Spinal Cord Dysfunction
his chapter reviews the assessment occurs in 10 to 15 percent of traumatic spinal injuries
tant injuries, such as head injury, may make it difficult Testing for light touch sensation is conducted using
to be certain that an injury is truly complete (7). Com- a cotton swab or a cotton strand pulled out from a cot-
plications and medical and surgical interventions over the ton applicator stick. Sometimes, for clinical purposes,
first few days after injury may adversely or favorably testing is performed by lightly touching the patient with
influence spinal cord recovery. a gloved finger. The sensation is graded as normal,
impaired, or absent. Normal sensation is established by
comparison with sensation on the face, or another area
ELEMENTS OF NEUROLOGIC ASSESSMENT with normal sensation, if sensation of the face is affected.
Impaired sensation is any sensation that differs from that
Neurologic assessment is based on the Standards of the on the normal area. Both hyperesthesia and diminished
American Spinal Injury Association (ASIA). The 2000 Stan- sensation are considered abnormal.
dards are described in this chapter. Further details can be Testing for pinprick sensation is performed using a
found in the booklet describing the Standards (1) and the disposable safety pin. Normal pinprick sensation is estab-
reference manual (8), available from ASIA. Elements of the lished by lightly touching the sharp end of the safety pin
examination include the determination of light touch and to the face, or another area determined to be normal, if
pinprick sensation, strength of key muscles, and reflexes. the face is affected. Normal pinprick sensation means that
the sharpness sensation in the area being tested is identi-
cal to that in the normal area. Abnormal sensation means
Sensory Examination
that the sensation is somewhat sharp, but not as sharp
The sensory examination consists of testing a key point as the normal area. Absent means that there is no feeling
in each of twenty-eight dermatomes on the right and left of sharpness. Note that the patient may feel a touch or a
sides of the body (Figure 27.1). These dermatomes are pressure sensation from the pin, but no sensation of
from C2 through S5, with S4S5 taken as one level. The sharpness. In this case pinprick sensation should be
key points are tested for appreciation of light touch and graded as absent. If the area feels sharper than the face
pinprick sensation. Additionally, the external anal sphinc- this should be graded as impaired. Also, in areas of hyper-
ter is examined for the presence of any form of sensa- esthesia, all sensation may be perceived as sharp. If the
tion, including deep anal pressure. patient is unable to differentiate the sensation of the sharp
from the blunt end of the pin, then pinprick sensation
should be graded as absent.
In addition to testing the perianal area for light touch
and pinprick sensation, the external anal sphincter should
be examined with a gloved finger. The patient should be
asked if he/she feels any sensation to the gloved finger or
any sensation of pressure as the finger is moved gently
against the anal sphincter. This is important for determin-
ing whether the injury is complete or incomplete in a
severely injured individual. Any reliable sensory percep-
tion in the anal area means that the injury is incomplete.
Clinically, it is important to test for deep pressure sen-
sation and position sense in the extremities. These modal-
ities have functional significance, but are not used in the
classification of spinal cord dysfunction. Deep pressure
sensation may be present in individuals who have no light
touch or pinprick sensation, but does not designate an
incomplete injury unless it is present in the perianal area.
If not, pinprick is graded as absent at that point. If so, be tested because of inhibiting factors, or if it is unable
then the degree of sharpness is compared to that on the to be tested because of associated injuries, then it should
face to determine whether the pinprick sensation is be graded as not testable (NT).
impaired or normal. The anal sphincter should be examined for any vol-
The T3 dermatome is probably the most difficult to untary contraction. This is done by inserting a gloved fin-
evaluate, because there is considerable variation in the ger into the rectum and asking the patient to squeeze (not
distance that the C4 dermatome extends down the ante- bear down) as if he is holding in a bowel movement. The
rior chest wall. In cervical injuries, if testing for T3 sen- anal sphincter should be graded as present or absent. If
sation occurs too high on the chest, it may seem to be pre- voluntary contraction of the anal sphincter is present,
sent because of C4 innervation. As described in the then the injury is incomplete.
Reference Manual for the International Standards, if sen- Clinically it is important to examine other muscles
sation is absent in T1 and T2, seems to be present in T3, in addition to the ten key muscles needed for classifica-
and absent in T4, it is recommended that the T3 der- tion. Grading additional muscles at the level of injury is
matome be scored as absent (8, p16). important in monitoring the progression of deficits and
recovery. Other clinically significant muscles include the
diaphragm, the deltoids, the abdominals (evaluation of
Motor Examination
Beevors sign), the hamstrings, and the hip adductors.
The motor examination consists of testing ten impaired
myotomes consisting of five muscles in each extremity
Pitfalls in Motor Examination
(see Figure 27.1). The muscles are graded by manual mus-
cle testing (MMT) using a six-point scale (Table 27.1). It Accurate MMT requires a proper positioning of the
is important to score each muscle based on the identifi- extremity being tested and an awareness of substitution
cation of neurologic weakness. Only muscles that are patterns. Perhaps the most difficult muscle to examine
thought to be neurologicly weak should be scored less in the upper extremity is the triceps. When testing for
than Grade 5. If a muscle is felt to be slightly weak grade 2 triceps, care must be taken to maintain the fore-
because of disuse rather than neurologic weakness, then arm in a horizontal position over the chest. Frequently
it should be graded as normal (Grade 5*). Similarly, if the the patient will externally rotate the shoulder, whereupon
patient is inhibited by pain or hypertonicity, but gives a momentum and gravity will allow the elbow to extend.
forceful contraction (perhaps briefly), it may be graded Careful positioning, as well as palpation and visual
normal if the examiner feels that the muscle would test inspection of the triceps muscle, will minimize the chance
normal were it not for these factors. If a muscle cannot of being fooled by this substitution pattern.
Testing the flexor profundus muscle requires stabi-
lization of the wrist and proximal phalanx of the finger
TABLE 27.1 to isolate the muscle. Finger flexion can occur through
tenodesis effect (passive pull of the flexor tendon) when
Manual Muscle Test Grades
the wrist is extended. Although this effect often is clini-
GRADE DEFINITION cally useful, it can confound attempts to grade finger
flexor strength. Finally, in the upper extremities, care
0 Total paralysis (no visible or palpable must be taken when testing the finger abductors. In sub-
contraction) jects with active finger extension, this motion (extension)
1 Palpable or visible muscle contraction results in some abduction of the fingers. The fingers
2 Active movement, full ROM with gravity should be positioned in extension, and the abductors
eliminated observed and palpated when the abductor digiti minimi
3 Active movement, full ROM against gravity is being tested.
In the lower extremities, care should be taken when
4 Active movement, full ROM, against moder-
ate resistance testing the ankle dorsiflexors and plantarflexors in
patients with proximal leg movement. When the heel is
5 (Normal) active movement, full ROM,
against full resistance in contact with the bed, hip movement may cause pas-
sive ankle movement that can be confused with active
5* Sufficient resistance to be considered nor-
mal if identifiable inhibiting factors were movement. When in doubt, the examiner should lift the
not present (see text) leg and repeat the examination with the foot off the bed.
NT Not testable Reflex withdrawal and hypertonicity can also be
confused with voluntary movement. The act of touching
Adapted from ASIA 2000, 1314. With permission. or grabbing the limb may set off a flexion withdrawal
response. Some patients are able to trigger a lower
372 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
extremity spasm when asked to move the limb. Careful the body. When the lesion is asymmetric, a single neuro-
testing is required to distinguish these involuntary move- logic level should not be used to characterize the lesion.
ments from volitional muscle contractions. Instead, the individual sensory and motor levels should
be used; for example, right C5 sensory-C6 motor, left C6
sensory-C7 motor level.
Reflex Testing
The determination of the sensory level is generally
The third component of the neurologic examination is determined reliably (9). There is often difficulty even for
testing of reflexes. Deep tendon reflexes should be eval- experienced examiners in determining the motor level
uated for biceps, wrist extensors, triceps, and finger flex- (10). The Standards have been revised to clarify the def-
ors in the upper extremities. In the lower extremities, the initions and criteria for determining motor level. The
patellar and Achilles reflexes should be assessed. Finally, accuracy and reliability of motor level determination
the anal wink and bulbocavernosus reflexes should be using the current definitions has not been reported.
assessed. These reflexes have significance for bowel and Although the motor level is the most caudal segment
bladder functioning, and their presence indicates the of the spinal cord with normal function, this does not
emergence from spinal shock. mean that the key muscle has normal strength. The key
muscles have been selected because they are innervated
predominantly by two myotomes (Figure 27.2). By con-
CLASSIFICATION OF IMPAIRMENT: vention, if the muscle has at least a grade of 3, it is con-
THE 2000 STANDARDS sidered to have intact innervation by the more rostral seg-
ment. Therefore, operationally, the motor level is defined
The Standards were designed for describing the level and by the lowest key muscle that has a grade of at least 3,
severity of injury in an acute traumatic SCI. The classifi- providing that the key muscles above that level are judged
cation may not be as meaningful if used to describe non- to be normal (grade 5).
traumatic spinal cord disorders. In addition, the Stan- If there is no muscle to test at a given segment, such
dards are difficult to apply when the spinal cord as in the thoracic segments and the C2C4 segments, then
dysfunction is multifocal, as occurs in some traumatic the motor level is determined by the sensory level.
injuries and with diseases such as multiple sclerosis. There
are four components to classification: determination of
Completeness of Injury
levels of injury, completeness of injury, ASIA Impairment
Scale grade, and zone of partial preservation (ZPP) for The criterion for an incomplete injury is based on the
complete injuries. detection of sacral sparing, after work by Waters et al.
(11). Earlier versions of the Standards defined a lesion as
incomplete if there was some preservation of motor or
Levels of Injury
Definitions of neurologic, sensory, and motor levels are
found in Table 27.2. It is recommended that sensory and
motor levels be determined for the left and right sides of
TABLE 27.2
Definitions of Levels of Injury
sensory function more than three levels below the neu- A. Next one determines whether or not an incomplete
rologic level (12). Waters et al. (11) compared this defin- lesion is motor incomplete. To be motor incomplete there
ition to a sacral sparing definition of incomplete; must be either voluntary anal sphincter contraction or
namely, the preservation of some sensory and/or motor some sparing of motor function more than three levels
function in the sacral segments. They found the sacral caudal to the motor level. For example, an individual with
sparing definition to be more stable than the ASIA defi- an L2 sensory and motor level, perianal sensation, no vol-
nition. Subsequently, the sacral sparing definition was untary anal sphincter contraction, grade 2 ankle dorsi-
incorporated into the Standards. Thus, an injury is con- flexor strength (L4), and no movement in more distal
sidered complete if neither motor nor sensory function muscles would be AIS grade B. If, in addition, the same
below the level of injury extends to the lowest sacral seg- individual had grade 1 plantarflexor strength (S1), he
ments. Conversely, an injury is considered incomplete if would receive an AIS grade of C. Note that motor spar-
there is partial preservation of sensory or motor function ing need not be in a key muscle. An individual with sacral
below the neurologic level that includes the lower sacral sparing and a C7 motor level with voluntary movement
segments. This sacral sensation can be light touch or pin- of the hip adductors would be motor incomplete.
prick sensation at the anal mucocutaneous junction, or The final distinction is between AIS grades C and D.
any anal sensation felt during a digital rectal examina- In a motor incomplete lesion, count the number of key mus-
tion. Motor function in the lowest sacral segment is deter- cles below the neurologic level for each side of the body.
mined by the presence of voluntary contraction of the Determine the number of these key muscles with a MMT
external anal sphincter. Note that reflex contraction as grade of at least 3. If less than half of the muscles are at least
obtained with the bulbocavernosus reflex or anal wink grade 3, then the AIS grade is C. If at least half of the mus-
reflex is not voluntary. cles are grade 3 or better, then the AIS grade is D.
Perform detailed examination of zone of injury. 4. Vaccaro AR, An HS, Lin S, Sun S, Balderston RA, Cotler JM. Non-
contiguous injuries of the spine. J Spinal Disord 1992; 5:320329.
Conduct a detailed assessment of sensation and 5. Cohen ME, Sheehan TP, Herbison GJ. Content validity and reli-
strength at the zone of injury. This should include ability of the International Standards for Neurologic Classifica-
muscles and dermatomes one to two levels above tion of spinal cord injury. Top Spinal Cord Inj Rehabil 1996;
1(4):1531.
and below the injury level. 6. Brown PJ, Marino RJ, Herbison GJ, Ditunno JF, Jr. The 72-hour
examination as a predictor of recovery in motor complete quad-
riplegia. Arch Phys Med Rehabil 1991; 72:546548.
7. Maynard FM, Reynolds GG, Fountain S, Wilmot CB, Hamilton
CONCLUSION R. Neurologic prognosis after traumatic quadriplegia: Three-year
experience of California regional spinal cord injury care system.
Accurate neurologic classification after SCI requires care- J Neurosurg 1979; 50:611616.
8. American Spinal Injury Association. Reference Manual for the
ful neurologic examination and a thorough understand- International Standards for Neurologic and Functional Classifi-
ing of the ASIA Standards. Although there will always cation of Spinal Cord Injury. Chicago, IL: American Spinal Injury
be some patients with atypical injuries that are difficult Association, 1994.
9. Cohen ME, Ditunno JFJ, Donovan WH, Maynard FMJ. A test
to classify, this chapter should assist the reader in classi- of the 1992 International Standards for Neurologic and Func-
fying the majority of patients with traumatic SCI. tional Classification of Spinal Cord Injury. Spinal Cord 1998;
36:554560.
10. Donovan WH, Wilkerson MA, Rossi D, Mechoulam F,
References Frankowski RF. A test of ASIA guidelines for classification of
spinal cord injuries. J Neuro Rehab 1990; 4(1):3953.
1. American Spinal Injury Association. International Standards for 11. Waters RL, Adkins RH, Yakura JS. Definition of complete spinal
Neurologic Classification of SCI, revised 2000. Chicago, IL: cord injury. Paraplegia 1991; 29:573581.
American Spinal Injury Association, 2000. 12. American Spinal Injury Association. Standards for Neurologic
2. Bracken MB, Holford TR. Effects of timing of methylprednisolone Classification of Spinal Injury Patients. Chicago, IL: American
or naloxone administration on recovery of segmental and long- Spinal Injury Association, 1989.
tract neurologic function in NASCIA 2. J Neurosurg 1993; 13. Frankel HL, Hancock DO, Hyslop G, Melzak J, Michaelis LS,
79:500507. Ungar GH, Vernon JDS, Walsh JJ. The value of postural reduc-
3. Henderson RL, Reid DC, Saboe LA. Multiple noncontiguous tion in the initial management of closed injuries of the spine with
spine fractures. Spine 1991; 16:128131. paraplegia and tetraplegia, Part I. Paraplegia 1969; 7:179192.
28
The Electrodiagnostic
Examination in Spinal
Cord Disorders
he spinal cord is the long, cylindri- age male, 2 to 3 cm shorter in the female. Similar to the
EDX evaluation of patients with suspected SCDs, mixed axons capable of being stimulated and of conducting
NCS are not mentioned further. impulses between the stimulating and recording points. It
Every time a NCS is performed, the response that is also (along with duration) a reflection of the relative
results contains several measurable components, each one rate of conduction along those axons. Moreover, it is also
of which providing information regarding the anatomic a function of a number of physical factors (e.g., the type
and physiologic status of the nerve fibers being assessed. and size of recording electrodes used, the amount of inter-
For motor NCS, which usually involve stimulation of the vening tissues between the nerve or muscle fibers gener-
nerve at two points along its course, these components are: ating the response and the surface recording electrode).
amplitude, duration, latency, and conduction velocity Finally, in regard to motor NCS, it also conveys infor-
(CV). For each of these, measurements can be determined mation regarding the status of neuromuscular transmis-
and reported separately for the proximal and the distal sion and of the muscle fibers of the recorded muscle. As
response; that is, the responses elicited on proximal and already noted, the amplitude of the CMAP is reported in
distal stimulations. Generally, the durations, although mV whereas that of the SNAP is reported in V (6).
noted, are not formally reported, and the proximal latency
is not reported directly, as is the distal latency. Instead, it
Duration
is used to calculate a CV (see below). Thus, characteristi-
cally, the distal amplitude, distal latency, and the CV are This is the time period between the onset and termination
reported for each motor NCS. During sensory NCS, typ- of the response, measured in milliseconds (ms). It is an indi-
ically the nerve is stimulated at only one point along its cator primarily of the relative rates of conduction along
course, so that the measurable components include the those fibers transmitting impulses between the stimulation
amplitude, duration, and distal or peak latency. Only the and recording points. Duration is obviously related to the
amplitude and latency are reported (Figure 28.2). These amplitude: as the duration increases, the amplitude must
components will now be discussed individually. decrease, if the area under the curve is to remain constant.
As the distance between the stimulating and recording elec-
trodes increases, slight differences in the rate of conduc-
Amplitude
tion along each individual axon become accentuated. Con-
This is the height of the response, measured from base- sequently, the responses on proximal stimulation are
line to peak, for CMAPs and for most SNAPs, and from somewhat increased in duration and lower in amplitude
negative to positive peak for the remaining SNAPs. Its pri- when compared to those on distal stimulation. This phe-
mary importance is as an indicator of the number of nomenon, termed temporal dispersion, has relatively lit-
FIGURE 28.2
The various components of motor and sensory nerve conduction responses are shown. (From Isley MR, Drass GL, Levin KH, Litt
B, Shields RW, Wilbourn AJ. Electromyography/Electroencephalography. Redford, WA: Spacelabs Medical, 1993, with permission.
THE ELECTRODIAGNOSTIC EXAMINATION IN SPINAL CORD DISORDERS 379
tle effect in motor fibers. Generally, the amplitude on prox- unit from the AHCs to the muscle fibers peripherally.
imal stimulation usually decreases less than 15 percent or However, it does so in a different fashion. Consequently,
so, compared to that obtained on distal stimulation. In the motor NCS and the NEE complement, rather than
contrast, whenever sensory fibers are stimulated at two duplicate, each other (6).
points along their course, very often a substantial decrease The NEE customarily is described as having three
in amplitude occurs, because of in-phase cancellation. This phases: insertion, rest, and motor unit action potential
is because the duration of the SNAP is so much shorter (MUAP) activation.
than the CMAPs. Thus, stimulation of the sensory axons
in the normal median nerve at the elbow and wrist, while
Insertion Phase
recording from the digital nerves of the index or ring fin-
ger, can result in an amplitude drop of more than 50 per- The term insertion applies not only to the single instance
cent. This is the principal reason why conduction blocks in which the needle recording electrode is thrust through
(discussed below) are difficult to detect using sensory NCS, the skin into a particular muscle, but also to each of the sev-
compared to motor NCS (13). eral times it is advanced within that muscle while the lat-
ter is being assessed. Whenever a needle recording electrode
is advanced in any normally innervated, healthy muscle, a
Distal or Peak Latency
brief burst of electrical activity is generated, which persists
This is a rate measurement, determined from the moment for approximately one-third of a second. This is labeled
of stimulation to either the onset of the response (distal normal insertional activity. During the insertion phase, the
latency) or to the peak of the response (peak latency). For electrical activity generated by needle advancement is
motor NCS, distal latencies are always employed. For sen- assessed in regard to its presence or absence and, if pre-
sory NCS, some electrodiagnosticians use distal latencies, sent, whether it is accompanied or followed by abnormal
whereas others prefer peak latencies. Of particular note discharges. Under various circumstances, different types
is that the distal latency provides almost no information of electrical activity triggered by the needle movement fol-
regarding the number of axons that are conducting low the normal insertional activity; these vary in their per-
impulses, beyond the fact that at least a few must be doing sistence and significance. Snap, crackle, and pop is one type
so, or it could not be determined (6). of abnormal insertional activity. It most commonly is seen
in young, mesomorphic males, and is considered a normal
variant. Insertional positive sharp waves are another abnor-
Conduction Velocity
mal type. These unsustained potentials can be seen as a nor-
This, also, is a rate measurement. Most often it is obtained mal variant, but also when very recently denervated mus-
by: stimulating the nerve at two points along its course; cles are sampled, at a time before spontaneous fibrillation
subtracting the latency elicited on distal stimulation (i.e., potentials (see below) have had sufficient time to appear.
distal latency) from that obtained on proximal stimulation Needle insertions also can initiate runs of myotonic dis-
(i.e., proximal latency); and then dividing the difference (in charges, a type of insertional and spontaneous activity most
ms) into the distance (in mm) between the two stimulation often seen with certain myopathies. (6,13).
points, as determined with surface recording electrodes.
The result is a CV measured in meters per second (m/s).
At-Rest Phase
Determining the rate of impulse transmission in this man-
ner allows direct comparison to be made between assess- During this segment of the NEE, the needle is held fixed
ments performed on identical nerves of different limbs, in a muscle that is not being contracted (i.e., one in which
even in patients with different limb lengths; for example, muscle fibers are not firing). During this stage, various
the forearm for median and ulnar motor CVs, and the leg types of abnormal potentials can be observed, which col-
for the routine peroneal and tibial motor CVs. Although lectively are known as spontaneous activity. The most
the proximal latency can serve the same purpose in a spe- important of these by far, and the most commonly seen,
cific limb, comparisons would not be possible unless all are fibrillation potentials. These are quite nonspecific in
limb segments were of the same length, or unless compli- nature, in that they can occur with both lower motor neu-
cated normograms were constructed for different limb ron (LMN) and myopathic disorders, as well as with
lengths. The use of CVs eliminates these problems (6). some abnormalities in neuromuscular transmission. (As
will be noted subsequently, they also have been reported
with upper motor neuron [UMN] lesions.) In regard to
Needle Electrode Examination
LMN disorders, these regularly firing potentials are gen-
The second major component of the EDX examination, erated by otherwise healthy single muscle fibers that lost
the NEE, is sometimes also called needle EMG. Similar their nerve supply at least 2 to 3 weeks previously. Ini-
to the motor NCS, it essentially assesses the entire motor tially, the denervated muscle fibers individually begin to
380 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
produce fibrillation potentials only in an unsustained mentioned below, normally MUPs fire no faster than 30
fashion, and after being injured by the needle recording to 40Hz.) Cramp potentials often are seen in patients who
electrode. These potentials (already described), are manifest fasciculations because, as noted, both are signs
referred to as insertional positive sharp waves. Within sev- of abnormal motor unit irritability (i.e., a lowered thresh-
eral days, however, the denervated muscle fibers start to old for activation) (6).
produce such potentials spontaneously, and in a sustained Although many other types of spontaneous activity
manner. Depending on whether the tip of the needle can be observed during this phase of the NEE, they are
recording electrode has actually injured the muscle fibers of little importance in regard to SCDs and, therefore, are
that are responsible for these potentials, or is simply near not discussed.
them, this spontaneous activity has either a positive sharp
wave or a biphasic spike appearance; both types are
Motor Unit Potential Activation Phase
labeled fibrillation potentials (5,6,13).
Fasciculation potentials are another type of sponta- During this portion of the NEE, the patient voluntarily
neous activity. In contrast to fibrillation potentials, which contracts the muscle in which the needle recording elec-
are produced by single denervated muscle fibers, fascicu- trode has been inserted, and the MUAPs that are gener-
lation potentials are generated by motor units or portions ated are assessed in regard to their firing frequency, sta-
of motor units. Thus, they are considerably larger than the bility on repeated firing, external configuration (e.g.,
typical fibrillation potential. Moreover, because the motor amplitude, duration); and internal configuration (e.g., ser-
unit generating them must be intact to do so, they are evi- rations, polyphasicity). Two MUAP firing patterns are
dence of motor unit irritation, rather than motor unit dis- of importance with spinal cord disorders.
integration, as are fibrillation potentials. Finally, from the With reduced MUAP recruitment, a significant num-
viewpoint of recognition, fasciculation potentials typically ber of motor units in the muscle being assessed by NEE
fire irregularly, whereas fibrillation potentials character- do not fire on attempted AHC activation, either because
istically fire with metronomic regularity. Although fascic- the AHC or motor axon of the motor unit has degener-
ulation potentials theoretically can be seen with any PNS ated, or the AHC is unable to initiate an impulse, or con-
disorder, in fact, they are relatively uncommon and most duction is blocked along the axon. As a result, the MUAPs
often limited to those that are causing focal demyelina- that can fire are observed to do so not only in decreased
tion and are chronic in nature (e.g., ulnar neuropathy at numbers, but also faster than their basal firing rate of
the elbow, radiation plexopathy, multifocal motor neu- approximately 10 Hz. There is nothing abnormal about
ropathy). In regard to intraspinal canal disorders, fascic- MUAPs firing faster than 10 Hz; in fact, it is a normal
ulation potentials may occur with almost any one of them, component of the MUAP recruitment process. Thus,
including intramedullary neoplasms and radiculopathies, whenever progressively stronger muscle contractions are
although they rarely have any diagnostic significance in made, progressively more MUAP activation occurs, first
these instances. The sole exception is one of the AHC dis- by spatial recruitment, and then via temporal recruitment.
orders, ALS, in which their presence is nearly mandatory During the latter, to increase the force of contraction,
for diagnosis. The most common situation in which fasci- motor units that were firing at 5 Hz and 10 Hz shift their
culation potentials appear, however, is the benign fascicu- firing frequency to 30 Hz or greater. By this process, the
lation syndrome. As its name implies, this disorder (if it same number of motor units can produce three times or
can be designated as such) consists solely of an otherwise more the amount of force generated by spatial recruit-
healthy persons experiencing widespread, persistent fas- ment alone. However, if there is a normal complement
ciculations, and often cramps as well. (Because both fas- of motor units firing, then the fact that they begin to fire
ciculations and cramps are caused by muscle membrane at faster rates on maximal activation goes undetected,
irritability, they often are seen together) (5,9,13). because too many of them are firing simultaneously for
Complex repetitive discharges are another type of the characteristics of individual ones to be discerned.
spontaneous activity. They are caused by the repetitive fir- Only if a substantial number of MUAPs are not being
ing of a group of muscle fibers, two of which serve as activated when they should; that is, they have dropped-
primary and secondary pacemakers, respectively They are out, can the firing rates of individual ones be recognized.
nonspecific in nature, in that they can be seen with both Reduced MUAP recruitment is unequivocal evidence of
neurogenic and myopathic disorders. Nonetheless, their a LMN lesion, and its severity has a high correlation with
presence is not needed for diagnosis in any instance. How- the clinical weakness of the recorded muscle. Thus, if the
ever, they do demonstrate that the abnormal process is MUAPs are firing in substantially decreased numbers at
chronic, because they rarely are seen with any disorder of 25 to 35 Hz, the muscle being assessed will appear weak
less than 6 months duration (6). on clinical examination; if only one or two MUAPs fire
Cramp potentials consist of involuntary sustained on maximal activation at faster than 10 Hz, the muscle
firing of motor units at high rates (200 to 300Hz). (As is essentially will be paralyzed. Although by far the most
THE ELECTRODIAGNOSTIC EXAMINATION IN SPINAL CORD DISORDERS 381
common cause for reduced MUAP recruitment seen in the cross the baseline). Conversely, when MUAPs are
EDX laboratory is axon loss (from the destruction of polyphasic, they contain more than four phases (compo-
either the AHC or its axon), exactly the same MUAP fir- nents above or below the baseline). Many electrodiag-
ing abnormality is produced when demyelinating con- nosticians consider serrated MUAPs to be simply the ini-
duction block affects the axon. tial or early stages of a polyphasic MUAP. There are a
Incomplete MUAP activation is seen whenever the number of situations in which polyphasic MUAPs cus-
patient is requested to vigorously contract the muscle and tomarily are found. These include disorders of the PNS,
the MUAPs fire in reduced numbers, but at a slow-to- the neuromuscular junctions, and the muscle fibers them-
moderate rate (i.e., less than 10 Hz). Unlike reduced selves. Moreover, a certain proportion of the MUAPs
MUAP recruitment, incomplete MUAP activation is not (approximately 10 percent) in any muscle normally may
a sign of a LMN lesion. Its cause varies: pain on muscle be polyphasic. Thus, polyphasic MUAPs are nonspecific
contraction, conversion reaction and malingering, and, in in nature and not necessarily abnormal. In regard to neu-
the context of this chapter, UMN lesions. rogenic lesions, they are an impressive component of early
Another aspect of MUAP firing, besides recruitment reinnervational MUAPs, and they almost always are a
or activation, is the constancy of the appearance of the component of chronic neurogenic MUAPs (i.e., with
MUAPs on consecutive firings. Under normal circum- chronic neurogenic MUAP changes, the MUAPs not only
stances, this changes little from one firing to the next, are of increased duration and sometimes amplitude, but
thereby producing MUAP firing stability. However, in the are also polyphasic as well). The key point in these cir-
presence of early reinnervation and of primary neuro- cumstances is that the MUAPs are of increased duration,
muscular transmission disorders, a variable number of the not that they are polyphasic. Thus, the often-used term
endplates cannot conduct consecutive impulses, thus to describe these chronic neurogenic MUAP changes,
resulting in MUAP firing instability (6,13). polyphasic MUAPs of long duration, emphasizes the less
The internal and external configurations of the important constituent of the MUAP alteration. Whether
MUAPs are altered by a great number of neurogenic and PNS lesions can solely cause polyphasia, without alter-
myopathic processes. With the former, if entire motor units ations in the external configuration of the MUAP, has
had been lost and first a few, and then progressively more, been much debated by electrodiagnosticians, and there
of the denervated muscle fibers are reinnervated, then the remains no general agreement on this point. Most often,
MUAPs that initially fire are very low in amplitude, quite this controversy arises in regard to chronic radicu-
prolonged in duration, and highly polyphasic in internal lopathiescan radiculopathies be reliably diagnosed by
configuration. As time passes, however, these early rein- the detection of polyphasic MUAPs of normal duration
nervational MUAPs undergo remodeling, so that ulti- in a myotome distribution (5,13)?
mately they may resume a normal appearance or, if much What appear to be MUAPs of increased duration
of the reinnervation was through the process of collateral and polyphasic in configuration are commonly seen
sprouting, they may be permanently abnormal in appear- whenever tremor is present, caused either by a UMN dis-
ance, have increased duration, and sometimes increased order or by conversion reaction or malingering. The
amplitude as well. Such chronic neurogenic MUAP pseudopolyphasic MUAPs in these instances are caused
changes generally indicate that the responsible nerve dam- by the partial superimposition of two or more MUAPs,
age is of at least several months duration. A great num- which are firing in poorly synchronized groups.
ber of denervating processes (e.g., axon loss polyneu-
ropathies and focal nerve trunk injuries) produce chronic
Special Studies
neurogenic MUAP changes in which the MUAPs, although
of increased duration, are of normal amplitude. However, Although a number of specialized EDX tests are grouped
in rather severe axon loss lesions that have affected the under this title, only two are of concern in this chapter,
proximal portions of the motor system (i.e., the AHCs, the so-called late responses: F-waves and H-reflexes. The
roots, or very proximal portions of the plexuses), partic- first of these, F-waves, are caused by stimulation-induced
ularly those that are static and quite remote or very chronic impulses traveling antidromically along motor axons,
in nature, the MUAPs frequently are not only of substan- causing the AHCs to backfire, and thereby sending
tially increased duration, but also of increased amplitude impulses back down the motor axons to the recorded mus-
as well. Such prominent chronic neurogenic MUAP cle. Thus, F-waves assess solely motor axons and their par-
changes typically require years to develop (6). ent AHC within the spinal cord; they do not evaluate any
In regard to the alterations of the internal configu- of the sensory portion of the PNS. In contrast to H-reflexes
ration of MUAPs, these consist of their being either ser- (discussed below), F-waves are elicited by supramaximal
rated or polyphasic. Serrated MUAPs are those that con- stimulation, and can be recorded after stimulation of most
tain so-called turns (i.e., abrupt changes in direction or of the major limb nerves. However, they are more easily
notches in the various peaks of the MUAP that do not detected when obtained from distal limb muscles, since
382 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
often they are obscured by the direct M-response when standard EDX examination, have only two reactions:
more proximal muscles are used for recording purposes. axon degeneration (also known as axon loss), and focal
Only a fraction of the nerve impulses that travel antidromi- demyelination. With the former, which is by far the most
cally up motor fibers each time the nerve is stimulated common of the two, the nerve fibers are killed at the
cause recurrent activation of the AHC and, therefore, pro- lesion site, and the entire portion of them distal to that
duce F-waves. The percent of supramaximal nerve stimu- point undergoes Wallerian degeneration. This affects not
lations to an individual axon that result in F-wave gener- only the axons, but also the structures to which they are
ation varies from one peripheral nerve to another, and is connected, directly or indirectlysensory receptors, neu-
altered by several different physiologic variables (5,13). romuscular junctions, and muscle fibers. Thus, with axon
The second of these, the H-reflex, is a spinal mono- loss, the ultimate effects are never limited to the site of
synaptic reflex initiated by nerve stimulation, as opposed injury. In contrast, with focal demyelination, the only
to tendon percussion, such as deep tendon reflex (DTR) alteration to the nerve fiber is that the myelin at the lesion
assessment. H-reflexes are elicited by submaximal stimu- site is damaged to varying degrees, which can result in
lation of a mixed nerve. As the stimulus strength is several different types of abnormalities. Focal distur-
increased, a direct response (M-wave) also appears and bances in conduction are critical for the electrodiagnos-
increases in amplitude. As this occurs, there is a corre- tician. However, in contradistinction to axon loss lesions,
sponding decrease in the H-wave amplitude. Eventually, in focal demyelination the axons are not killed at the
when near-supramaximal stimulation is delivered to the lesion site, and the portions of nerves distal to that point
nerve, the H-wave often becomes unelicitable. In adults are not disturbed in any manner: the axon and myelin are
with spasticity and in young children, H-waves can be intact and conduction is normal in all respects distal to a
elicited by stimulating many peripheral nerves. However, focal demyelinating injury (6).
in healthy adults, usually the stimulation of only one upper Although only two fundamental types of nerve
and one lower limb nervethe median at the elbow and pathology result from focal nerve damage, the patho-
particularly the posterior tibial at the popliteal fossa physiologic responses they cause, as detected on EDX
results in H-waves being recorded from the pronator teres- studies, are more numerous. Two different pathophysio-
flexor carpi radialis muscles and the gastrocnemius-soleus logic reactions can be seen following an axon loss lesion,
muscles, respectively. In most EDX laboratories, H-reflexes whereas three may result from a focal demyelinating one.
usually are elicited only in the lower limb. Thus, only the Moreover, various combinations are common. These five
S1 root and the S1 segment of the spinal cord are assessed. processes will now be reviewed, beginning with the two
H-reflexes are very sensitive (in that they are often abnor- caused by axon loss.
mal) in two PNS disorders: S1 radiculopathies and gener-
alized polyneuropathies. Often overlooked is the fact that
Axon Loss
whenever an H-reflex is being elicited by recording from
the gastrocnemius-soleous muscles, a direct M-response If an EDX examination is performed within the first sev-
(i.e., a tibial CMAP) can be recorded from those same mus- eral days after a mixed nerve has sustained a focal, sub-
cles as well. The amplitude of this CMAP, recorded on stantial axon loss lesion, a characteristic combination of
supramaximal stimulation, can be very helpful in deter- NCS and NEE changes is seen. On NCS, if the nerve is
mining the amount of axon loss sustained by these mus- stimulated proximal and distal to the site of injury, while
cles. The CMAP amplitude recorded from the anterior and recording further distally, a conduction block pattern is
lateral compartment muscles of the leg on supramaximal observed: the evoked response on stimulating distal to the
stimulation of the common peroneal nerve at the fibular lesion is normal, or low in amplitude, while that on stim-
head is also very useful (5,6,13). ulating proximal to it is even lower in amplitude or unelic-
itable, depending on whether the lesion is partial or com-
plete. This type of conduction block, termed an
PATHOPHYSIOLOGY axon-discontinuity conduction block, is seen because those
portions of the axons comprising the distal stump,
Virtually all the changes seen on the EDX examination although in the early stages of degeneration, are still capa-
are caused by alterations in physiology through the course ble of conducting impulses. This contrasts with the seg-
of various pathologic processes. In regard to the PNS, ments of the axons at the injury site, which have not been
although axons can be damaged by a great number of capable of impulse transmission since the instant of injury.
injurious processescompression, traction, excess cold Axon-discontinuity conduction blocks are short-lived phe-
or heat, chemicals, radiation, etc.their ability to nomena because, by 10 to 11 days after injury, all the dis-
respond to such injuries is extremely limited pathologi- tal stump fibers have degenerated sufficiently that they are
cally. Thus, the large myelinated axons, which are the incapable of transmitting impulses. Daily assessments of
only size of nerve fibers assessed by any component of the conduction along such injured fibers have revealed that the
THE ELECTRODIAGNOSTIC EXAMINATION IN SPINAL CORD DISORDERS 383
amplitudes of the CMAPs on distal stimulation remain lating. For this reason, fibrillation potentials tend to be
normal for 2 to 3 days after injury, then drop steeply. By seen in meager numbers, if at all, with remote, static axon
7 days after injury, if the lesion is complete, CMAPs can- loss lesions (6).
not be elicited on stimulation either proximal or distal to In regard to the CMAP and SNAP amplitude
the lesion site. The time course of changes is somewhat dif- changes seen with axon loss lesions, an important fact
ferent for sensory NCS. The SNAP amplitudes usually do must be appreciated. For uncertain reasons, whenever
not begin to drop until approximately 5 days after injury, focal incomplete axon loss involves mixed nerves, the
and then reach their nadir at 10 to 11 days. Although it SNAP amplitudes usually ultimately are more affected
may superficially appear that the motor and sensory axons than the CMAP amplitudes, even though very early after
distal to a lesion are degenerating at different rates, in fact, injury the latter are the first to decrease in amplitude.
the differences in their conducting properties are caused Thus, when the focal nerve injury apparently has killed
by two other factors: a) dissimilarities in their recording approximately 30 percent of the axons, on NCS per-
methods and b) the degeneration of nerve fibers is always formed 11 days or more after onset, the CMAP ampli-
the most advanced along their most distal segments. With tudes uniformly will have fallen approximately 30 per-
motor NCS, the impulses must traverse the most distal por- cent whereas the SNAP amplitudes will be substantially
tion of the axons to reach the neuromuscular junctions. more decreased, by approximately 60 percent. With even
Hence, the CMAP amplitudes invariably are affected by more severe axon loss, whenever the CMAP amplitudes
the advanced degeneration along those distal portions. In are only 40 to 50 percent of normal, the SNAP responses
contrast, during sensory NCS, the most distal portion of usually are unelicitable. Thus, the CMAP amplitudes cus-
the sensory axons is not assessed. For these reasons, pro- tomarily are more accurate indicators of the actual
gressive changes seen with the SNAP amplitudes always amount of axon loss that has been sustained, whereas the
lag a few days behind those of the CMAP amplitudes. On SNAP amplitudes are more reactive to axon loss. The rea-
NEE, at these very early stages of axon loss, typically the son for the SNAP amplitudes being so hypersensitive
only finding is that of reduced MUAP recruitment. The to axon loss is unknown. Nonetheless, in very practical
duration of the lesion is such that fibrillation potentials, or terms, this means that the SNAP amplitudes are the most
even insertional positive sharp waves, have not had time sensitive NCS indicator of axon loss (6).
to develop (6,13).
With substantial axon loss lesions, by 11 days after
Focal Demyelination
onset, the CMAPs and SNAPs elicited by stimulations
applied at any point along the nerveproximal, distal, Nerve lesions that cause focal demyelination manifest con-
or at the lesion siteare always equally affected. Thus, duction abnormalities at the lesion site, consisting of either
with incomplete axon loss injuries, the amplitudes of the slowing or blocking. These can be detected by NCS, if the
CMAPs are uniformly low, whereas with complete nerve is stimulated proximal and distal to the point of
lesions, they are uniformly unelicitable. This combination injury. Two types of conduction slowing can be recognized:
of NCS changes is designated the conduction failure pat- a) that in which the nerve impulses traversing all the large
tern. It is by far the most common pattern of NCS change myelinated nerve fibers are slowed to the same degree, a
seen following focal nerve injuries, because the majority process called focal or synchronized slowing; and b) that
of the latter are axon loss in type, and nearly all are in which the fastest conducting fibers are not affected, but
assessed 10 or more days after onset, at a time when the conduction along all the others is slowed, and to various
axon-discontinuity conduction block pattern has con- degrees, resulting in desynchronized or differential slow-
verted to the conduction failure pattern. In these lesions, ing. With demyelinating conduction block, a process iden-
reduced MUAP recruitment will be seen whenever the tical in its physiologic aspects to that of axon-discontinu-
weak muscles are assessed with NEE. However, fibrilla- ity conduction block, nerve impulses are stopped at the
tion potentials usually will not appear until some 2.5 to lesion site and cannot progress beyond it, even though the
5 weeks (average 3 weeks) after lesion onset. When the conduction properties of the segments of nerve distal to
lesion is of several months duration, depending on its that point are normal. In contradistinction to lesions caus-
severity and the relative efficiency of the reinnervating ing axon loss, those causing focal demyelination produce
process, chronic neurogenic MUAP changes will be pre- very little change on NEE. With conduction slowing, the
sent. Once denervated muscle fibers are reinnervated, they NEE of the recorded muscle is normal; it is inconsequen-
cease to fibrillate. Consequently, fibrillation potentials tial that all or some of the nerve impulses activating the
usually are no longer present, or at least they are markedly MUAPs in the recorded muscle are slowed transiently as
reduced in numbers, whenever static lesions of more than they traverse the lesion site. With demyelinating conduc-
1 to 1.5 years duration are assessed. By 2 years after tion block, no abnormalities are seen on NEE of the
injury, any muscles which were not reinnervated usually recorded muscle until the process is substantial. At that
have degenerated and, once this occurs, they stop fibril- point, reduced MUAP recruitment is observed, identical to
384 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
that resulting from axon loss, if the same number of motor aspects of the EDX examination, pertains to motor and
axons are involved. Also, although theoretically fibrilla- sensory axons, not to their cell bodies within the
tion potentials are not seen with pure demyelinating intraspinal canal. This is pertinent, because the literature
conduction block injuries, because no motor axons have suggests that certain rules concerning how the various
been killed and, therefore, no muscle fibers denervated, EDX components are affected by lesions at various sites
in fact, they often are observed in these circumstances. Pre- along the plexuses and peripheral nerves may not apply
sumably, this is because at least a minimal amount of in regard to spinal cord involvement. Consequently, the
motor axon loss customarily coexists with substantial EDX manifestation seen under three different circum-
demyelinating conduction block lesions (6). stances will now be described; specifically, in regard to a
Two facts should be appreciated regarding nerve focal SCD, when spinal cord segments are assessed which
pathophysiology, in regard to their EDX and clinical man- a) are cephalad to it, b) are affected by it, or c) are cau-
ifestations: dal to it. The latter includes changes that are also seen
with UMN lesions caused by spinal cord abnormalities.
Only those pathophysiologic processes that affect
the amplitudes of the CMAPs and SNAPs have any
Superior to the Level of the Lesion
relationship to clinical changes; more specifically,
only those that affect the amplitudes without caus- As would be expected, generally all aspects of the EDX
ing increased duration. Thus, axon-discontinuity examination are normal when spinal cord segments
conduction block, conduction failure, and demyeli- cephalad to a focal SCD are assessed. Thus, the ampli-
nating conduction block cause weakness or paraly- tudes and latencies of the CMAPs and SNAPs are nor-
sis whenever they involve a substantial number or mal whereas the NEE reveals no spontaneous activity and
all of the motor axons, and they cause sensory the MUAPs demonstrate a normal firing pattern and are
deficits whenever they affect a substantial number of normal configuration. Consequently, in a patient hav-
of sensory axons. Concerning the latter, however, ing a focal lower thoracic myelopathy, EDX examination
there is some discordance between those processes of the upper limbs typically reveals no abnormalities. One
causing axon loss and those causing demyelination. important note is that the actual rostral extent of a focal
Axon loss produces clinical deficits involving all sen- SCD may be higher than assumed, based on neuroimag-
sory modalities, including pain and temperature. ing studies (14).
Focal demyelination, because it affects only large
myelinated fibers, produces sensory changes limited
At the Level of the Lesion
to position, vibration, and light touch.
In contrast to the above, demyelinating conduc- The somatic motor axons that traverse the various
tion slowing, either focal or differential in type, is plexuses and peripheral nerves to innervate the muscles
responsible for essentially no clinical changes. The of the limbs and trunks have their cell bodies of origin
one exception is that differential slowing is manifest the alpha motor neurons or AHCslocated in the ven-
on those portions of the formal neurologic exami- tral gray matter (the so-called anterior horn) of the spinal
nation that require nerve impulses to pass along the cord. Damage to the AHCs in the spinal cord, or to the
sensory nerves in compact volleys. Thus, it alters the motor nerve fibers within the ventral portion of the spinal
DTRs as well as vibration and two-point discrimi- cord that link them to the primary motor roots, alters
nation testing. the CMAP amplitudes recorded from muscles that they
Whereas the motor NCS, the NEE, or both, may be innervate. Specifically, the more AHCs destroyed, the
abnormal with PNS disorders causing both negative more denervated muscle fibers in the recorded muscle that
(e.g., weakness, sensory loss) and positive (e.g., cannot contribute to the CMAP and, therefore, the lower
cramps, fasciculations) phenomena, sensory NCS the CMAP amplitude. It must be remembered, however,
are abnormal only with those lesions that are pro- that nearly all muscles receive innervation from more than
ducing negative manifestations (i.e., causing sensory one spinal cord segment (4). Consequently, unless both
deficits); those injuries of large myelinated sensory the spinal cord segments supplying the recorded muscle
axons causing only positive phenomenon (e.g., are involved, and all the AHCs within them are destroyed,
paresthesias) do not register on the sensory NCS (6). the CMAPs are more likely to be of low amplitude than
to be unelicitable. Also, the CMAP amplitudes in these
instances are affected by the duration of the lesion, par-
Electrodiagnostic Changes with
ticularly if the latter is incomplete. This is because a vari-
Spinal Cord Disorders
able number of the denervated muscle fibers in the
Much of the previous discussion, regarding the various recorded muscle will be reinnervated by sprouts arising
types of pathophysiology and their effects on different from intramuscular axons whose cell bodies in the spinal
THE ELECTRODIAGNOSTIC EXAMINATION IN SPINAL CORD DISORDERS 385
cord were not injured. Under these circumstances, myelopathies such as cervical spondylosis, cervical
although the number of motor axons supplying the syrinxes, and arteriovenous malformations (17).
recording muscle remains severely reduced, the number Despite the conflicting reports, in our experience the
of innervated muscle fibers in the recorded muscle that SNAP amplitudes characteristically are within the range
can respond to nerve stimuli increases. As a result, the of normal (i.e., neither too high nor abnormally low) in
CMAP responses, which for the first few months after patients with AHC disorders and most other SCDs when
onset of symptoms were quite low, will slowly begin to sensory axons derived from the affected levels are
increase in amplitude. Ultimately, they often reach the assessed. There are two exceptions, however. First, co-
normal range. Consequently, when motor NCS are per- existing or age-related problems often are present. Thus,
formed many months following a focal SCD, the amount many patients have abnormally low amplitude median
of denervation sustained by the recorded muscle may be sensory SNAPs because of superimposed carpal tunnel
seriously underestimated. This will be obvious when NEE syndrome (CTS), whereas many patients over the age of
is performed on the recorded muscle: reduced MUAP 60 years have unelicitable lower extremity SNAPs (sural,
recruitment will be evident, and the MUAPs that can be superficial peroneal sensory) because of age factors. Sec-
activated will show prominent chronic neurogenic ond, one type of SCD, Kennedys disease, although often
changes, indicating that the innervation ratio of the considered to be principally an AHC disease, character-
recorded muscle has been substantially altered (6,13). istically manifests abnormalities on the sensory NCS (e.g.,
The cell bodies of origin of the peripheral sensory low amplitude or unelicitable responses), as well as the
axons, assessed by sensory NCS, are not within the spinal NEE, and often the motor NCS also (6,9).
cord; rather, as noted, they are located in the DRGs, which The late responses are affected, as in all other com-
are situated along the very distal portion of the primary sen- ponents of the EDX examination, when the involved
sory roots. A sensory NCS that assesses axons derived from spinal cord segments are assessed. F-waves, which are
a DRG that resides in the intraspinal canal region that con- generated by AHCs, may not be elicitable. This typically
tains a SCD may be abnormal. Theoretically at least, this is seen when the CMAPs recorded from the same muscles
depends on whether the DRG, or the proximal postgan- are low in amplitude, because of severe denervation. With
glionic sensory axons contiguous to it, has been damaged, lesser involvement of the AHCs, resulting in partial den-
along with the spinal cord itself. Thus, it is certainly plau- ervation of the recorded muscles, F-waves typically are
sible that neoplastic invasion or a traumatic injury result- more difficult to elicit and sometimes are prolonged in
ing from a vertebral fracture with displacement, could latency. The latter presumably is caused by loss of the
destroy not only a localized portion of the spinal cord, but largest motor neurons whose axons are the fastest con-
also the intraspinal sensory structures linked to it. Under ducting. The H-reflex not only assesses sensory axons,
these circumstances, the SNAPs, as well as the CMAPs that including their preganglionic components, but also cer-
assess axons derived from the involved level(s), should be tain portions of the spinal cord as well. Consequently, it
of low amplitude or unelicitable. Conversely, there appears characteristically is unelicitable when the S1 spinal cord
to be little justification for assuming that those situations segment is involved, even in those instances in which the
in which SCDs occur in isolation, as when simultaneous direct M-response recorded from the gastrocnemi-soleus
involvement of the DRG or the post-ganglionic sensory muscle is only modestly low in amplitude. Presumably,
axons does not happen, would alter the SNAPs in any man- the H abnormalities in these instances are caused by
ner. The most obvious examples in which the SNAPs should involvement of the intramedullary pathways, specifically
remain normal are those SCDs that purportedly involve the sensory component (11,14).
solely the AHCs and not the sensory system at all.
Yet, rather surprisingly, there have been several
Inferior to the Level of the Lesion
reports describing reduction in the SNAP amplitudes
occurring with at least one AHC disorder: ALS It seems logical to contend, at least in regard to the NCS
(11,15,16). Usually, however, demonstrating these SNAP and NEE, that the EDX findings on assessing the portion
abnormalities has required the use of specialized tech- of the spinal cord distal to a lesion should be predictable.
niques such as near-nerve recording electrodes. Moreover, Thus, the motor NCS should be normal, unless a super-
the findings typically have been labeled subtle. Gener- imposed lesion of plexuses or peripheral nerves coexists.
ally, this means that even though the SNAP amplitudes Also, it is conceivable that, because of marked disuse atro-
(and latencies) in patients with ALS may be altered sta- phy of the recorded muscle, a CMAP amplitude could be
tistically when compared to those obtained from a nor- low. In our experience, however, this situation is restricted
mal patient population, in the individual case they would to small muscles. We have never seen large muscles, such
be within normal limits. Nonetheless, to add to the as the deltoid, quadriceps, or tibialis anterior, generate
debate, at least one report has described SNAP amplitudes low amplitude CMAPs for this reason, regardless of the
as being abnormally increased in amplitude with certain degree of atrophy present. The sensory NCS, of course,
386 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
should be normal as well, unless there is some superim- fibrillation potentials in them as well as in the limb mus-
posed PNS lesion. Finally, on NEE, the only abnormality cles, whereas Krueger et al. did not (30,32).
present should be one of MUAP activation; either no Not all investigators agree that fibrillation poten-
MUAPs at all should be observed on voluntary effort, or tials are present in muscles affected by UMN lesions.
a substantially decreased number, firing at a slow rate Thus, Alpert and co-workers, in two separate studies
(i.e., showing incomplete MUAP activation.) Any MUAPs dealing with a total of sixty-five patients, reported that
seen should be normal in appearance. A pertinent point fibrillation potentials seldom occur under these circum-
is that fibrillation potentials should not be observed, stances and, when they do, they are not the result of
because the muscles being sampled are paretic or para- UMN disorders (36,37). Others share this view (38,39).
lyzed because of a UMN, rather than an LMN, disorder. Moreover, even among the investigators who generally
Despite the seeming reasonableness of all these pro- agree that fibrillation potentials are seen, there are sig-
nouncements, as Dumitru has observed regarding this nificant differences on many specific points. Most of these
topic: This apparent logical conclusion, however, is not relate to the cause for their presence, and to the timing
the case (13). In fact, nearly all of the above statements of the NEE examination in regard to the duration of the
have been challenged by various investigators. In regard disability. Thus, are these fibrillation potentials essentially
to the motor NCS, several authors have reported finding found only relatively early after onset, during the period
CMAPs that are low in amplitude and often slow in CV of spinal shock? Or are they found with equal incidence
when assessing nerves whose AHCs of origin were situ- later, during the spasticity stage? When are they first
ated caudal to a focal SCD. Similarly, under the same cir- likely to appear, when do they reach their maximum den-
cumstances, the SNAP responses have been reported to sity, and when have they usually resolved? Concerning
be prolonged in latency, and both low in amplitude and those found in the lower limbs with cervical spinal cord
abnormally high in amplitude. Certainly, the most intrigu- injuries, are they as prominent (i.e., have the same den-
ing finding has concerned one aspect of the NEEspecif- sity) as those seen in muscles innervated by the spinal
ically, the presence of fibrillation potentials in muscles cord segments at the level of the lesion? Does their pres-
affected by UMN lesions. In 1967, Goldkamp reported ence, or density, have any relationship to the results of
finding fibrillation potentials in paretic or paralyzed mus- NCS, particularly motor NCS, performed on the same
cles in a large number of patients who had suffered cere- limb? Moreover, the consensus regarding the presence of
bral strokes. Of the 116 patients evaluated, he detected these fibrillation potentials does not at all extend to the
fibrillation potentials in 66 (56.8 percent) in one or both cause for their presence. Instead, a number of different
hemiparetic limbs. He detected them in both limbs in 32 mechanisms have been proposed, including traction on
percent of patients, and in the upper limbs alone in 28 the root and plexus fibers; traction or compression of var-
percent (18). Since that early report, many others (more ious peripheral nerves; dysfunction of AHCs due to spinal
than two dozen) have appeared, describing essentially the shock; absence of a specific antifibrillation factor (AFF);
same findings, not only in limb muscles paretic because transsynaptic degeneration of the alpha motor neuron;
of cerebral disorders, but also in those affected by focal and AHC dysfunction because of transsynaptic influ-
SCDs situated cephalad to the spinal cord segments being ences, which cause alterations in axoplasmic flow, pro-
assessed by the NEE. Some investigators have reported ducing a dying back phenomenon in the peripheral
finding fibrillations in virtually all muscles sampled in nerves (7,18,19,2232,38,40,41). Some version of the
these instances (1925). Most, however, have found them last two explanations appears to be the most popular.
in the majoritya sizable majorityof muscles sampled, The controversy regarding the presence of fibrilla-
ranging from 50 percent to more than 90 percent (2634). tion potentials in muscles of hemiparetic, paraplegic, and
Noteworthy is that the number of muscles assessed in quadriplegic limbs can be summarized as follows:
each limb by the various investigators varied consider-
ably, ranging from just one to many. Probably the most 1. A few investigators contend that they are not seen
common scenario was for four muscles to be sampled in under these circumstances, or their incidence is so
the affected limb, with each muscle receiving its innerva- low as to be insignificant.
tion from a different segment (root) level. Several inves- 2. A few investigators believe that they are observed,
tigators also noted that, in hemiplegic limbs, fibrillation but are caused by superimposed LMN problems,
potentials were found more often, or in greater density, involving the plexuses or peripheral nerves (39).
in upper limb muscles than in lower limb muscles, and 3. The majority believe that they are seen, and in some
that, in a given limb, they often were of higher density in manner the UMN lesion is responsible for their pres-
the more distal, compared to the more proximal, mus- ence.
cles (20,22,35). Most reports did not concern NEE of the
paraspinal muscles, and among the relatively few that did, A resolution of this controversy is unlikely to occur
the results reported were mixed. Thus, Nyboer detected in the foreseeable future, because the topic appears to be
THE ELECTRODIAGNOSTIC EXAMINATION IN SPINAL CORD DISORDERS 387
of little interest to any current investigators. Most of the orders, the reported findings have been rather diverse.
articles on this subject were published in the 1960s and, Concerning motor CVs, several authors have reported
particularly, the 1970s. To the authors knowledge, none either normal results, or only slight slowing in a few of
has appeared over the past decade. An important point the subjects assessed (18,20,30,4244), and sometimes
regarding this topic is that many electrodiagnosticians involving only the lower limb motor NCS. Conversely, a
rarely, if ever, perform NEE on these types of patients few investigators have described differences in side-to-side
because generally they are unnecessary for diagnostic pur- assessments of patients, with those performed on the
poses. Most who do so, as would be expected, specialize hemiparetic limbs being slower. Nonetheless, in these
in rehabilitation medicine; that is, they are physiatrists, reports typically the slowing has been so slight, averag-
and not neurologists. This is undoubtedly the reason why ing just a few m/s, or has involved other than the maxi-
most of the articles on the topic can be found in the phys- mal CV, that it is of significance only when groups of
ical medicine literature. At an international EMG meet- patients, rather than individuals, are considered
ing held in the 1970s, the author attended a session at (39,4548). Proposed causes for this slowing have
which two very eminent early electrodiagnosticians were included reduced limb temperature and decreased diam-
present: Edward Lambert and Fritz Buchthal. Although eter of nerve fibers (39,48). A more significant point
the author cannot recall the specific details of the subject regarding motor NCS changes has been the fact that the
under discussion, he vividly remembers both of them CMAP amplitudes often decrease. Most of the reports
being directly asked if they had ever seen fibrillation have been concerned with the lower limb CMAPs, record-
potentials under these circumstances, and both replied, ing intrinsic foot muscles; that is, the tibial, and particu-
No. Unfortunately, the obvious follow-up question was larly the peroneal, CMAPs have either been low in ampli-
omitted: How often do you perform NEEs on hemi- tude or unelicitable (24,32,33,38,41,49,50). Brown and
paretic, paraplegic, or quadriplegic limbs? If their expe- Snow, however, performed motor NCS while recording
rience is similar to that of most neurologists, the predicted from distal, mid, and proximal muscles in both the upper
answers to that would have been Very infrequently. and lower limbs, and demonstrated that all the motor
The author has encountered what may be examples of NCS responses decreased somewhat in amplitude; these
this phenomena in only a very few patients, but this is to occurred earlier than the third to fourth weeks after onset,
be expected, considering that he rarely performs EDX and persisted through the seventh week (20). Spaans and
examinations on limbs that could manifest these findings. coworkers observed that, in two patients, the peroneal
However, one such patient merits describing: CMAPs were unelicitable over several assessments, being
first detected 7 to 9 months after onset (24). In contrast,
CASE REPORT. A young woman sustained multiple Taylor and coworkers noted that the peroneal CMAP
injuries, principally to the head and the right lower limb, amplitudes gradually decreased over time, becoming
when she was struck by an automobile while attempting unavailable 40 to 60 days after onset (42).
to aid a stranded motorist on a snowy evening. She had a Concerning the sensory NCS, the results are equally
left hemiplegia and right lower limb paralysis, with the varied. Some investigators have found the SNAPs (usu-
latter attributed not only to a femur fracture but also to a ally sural) to be normal (24,27,44,50). Others reported
probable coexisting sciatic nerve lesion. On EDX them as being low in amplitude or unelicitable
examination performed 4.5 weeks after injury, there was (29,31,46,47,51). Even when abnormalities were seen,
evidence of a severe axon loss sciatic neuropathy on the they were noted to be quite variable from patient to patient
right, located at or proximal to the mid-thigh (i.e., and from one side to another in those with paraplegia and
proximal to the motor branches supplying the hamstring quadriplegia (51). They have also been linked to the tone
muscles). However, an inexplicable aspect of the study was of the limbflaccid versus spasticwith the SNAP abnor-
that when muscles in the hemiparetic left lower limb were malities being far more prominent in the former (32).
assessed, solely for comparison purposes, they also showed In regard to the late responses, far less has been pub-
fibrillation potentials. Ultimately, after an extensive EDX lished in the EDX literature about them and there is some-
examination, it was evident that most of the muscles in what more agreement regarding the findings in limbs
both the left upper and lower hemiparetic limbs contained affected with UMN disorders. Concerning F-waves, ini-
fibrillation potentials in the distribution of all segments, tially (within the first 4 weeks of onset), they show
with a distal-proximal gradient. In contrast, no NEE decreased responsiveness: a decrease in the F-wave per-
abnormalities were present in the muscles of the normal sistence, the average F-amplitude ratio, or both. Later,
right upper limb. Moreover, extensive motor and sensory they are more persistent, and more of them are of large
NCS performed on the left lower limb were normal, in amplitude. As a result, their averaged amplitudes are
sharp contrast to those done on the right. increased, as are their durations (52,53). The latencies,
according to Fisher, are slowed whereas, according to
In regard to NCS changes in limbs affected by UMN dis- Liberson, they may or may not be slowed (42,54). Con-
388 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
tiple neoplasms within the intraspinal canal, which and the C8, T1 distributions on the right. The extent of
originated from an ependymoblastoma in the mid- denervation in different intrinsic hand muscles was
thoracic region. variable, as reflected by the median (recording thenar)
An elderly patient rendered quadriplegic by an auto- and ulnar (recording hypothenar and FDI) CMAPs
mobile accident 13 years previously, who had bilaterally. The right median CMAP and the left ulnar
regained enough lower limb strength to ambulate CMAP, recording hypothenar, were both less than one
with a walker, was referred with a 4-year history of mV in amplitude. In contrast, the left median CMAP was
constant burning sensation in the left lower limb; only slightly low (4.8 mV), whereas the remaining ulnar
these symptoms were attributed to an ischemia- motor CMAPs bilaterally (right hypothenar and bilateral
caused nerve problemischemic monomelic neu- FDI) were normal. The patient was being reassessed in
ropathy. This disorder has characteristic EDX find- the EDX laboratory because of the recent onset of new
ings, which were not seen when the EDX weakness in the distribution of his right ulnar nerve. This
examination was performed on his left lower limb was accompanied by loss of light touch and position
(and the right lower limb for control). sense in the right fifth digit. (Pain and temperature
Several months prior to his EDX examination, a mid- sensation were abnormal throughout most of both upper
dle-aged man was scissored between players while limbs because of the cervical syrinx.) On EDX
refereeing a professional football game. Since that examination, in addition to the findings noted on the
time he had experienced paresthesias and sensory loss earlier assessment, the right ulnar SNAP (recording D5)
of the left lower limb and weakness of the right. On was now unelicitable, while that on the left remained
clinical examination, his quadriceps DTRs were brisk normal. The right ulnar motor CMAPs (hypothenar and
bilaterally, right greater than left, while the Achilles FDI) were now considerably lower in amplitude and
DTRs were normal bilaterally. Sensory loss was substantial slowing of the ulnar motor CV at the elbow
noted throughout most of his left lower limb, extend- was noted along the ulnar motor fibers supplying both
ing to the inferior portion of the left abdomen. Prior muscles; NEE now revealed fibrillation potentials and
to his EDX examination, one of his physicians had substantially more MUP loss in the right ulnar nerve-
attributed his hyperreflexia to a remote neck injury, innervated hand muscles. Thus, it was obvious that the
and his sensory complaints to either a mid-lumbar new right upper limb symptoms were caused by a
radiculopathy, or a femoral neuropathy, or possibly superimposed ulnar neuropathy at the elbow, and not
an ilioinguinal nerve entrapment. A CT scan of the due to progression of his cervical syrinx.
lumbosacral spine was normal but did not visualize
above the L3 level. On EDX examination, requested
Coexisting Spinal Cord and
to assess for a left lumbosacral radiculopathy, the
Peripheral Nerve Lesions
only abnormality noted on bilateral lower limb eval-
uation was incomplete MUAP activation in the mus- Plexopathies and mononeuropathies are found with some
cles of the right lower limb; the sural and superficial frequency in patients with SCDs. These may involve nerve
peroneal sensory SNAPs on the left were normal and fibers derived from spinal cord segments situated rostral
equal to those on the right. Based on the rarely made or caudal to the involved segments, or they may originate
EDX diagnosis of suggestive Brown-Sequard syn- from the damaged segments themselves (49,6062).
drome, the patient underwent a myelogram, which In regard to PNS lesions occurring superior to a
revealed a T10 disc herniation with some spinal cord SCD, brachial plexopathies and various mononeu-
compression. ropathies are found in the upper limbs in chronic para-
plegic patients with some frequency. One example of this
At times the EDX examination can definitely prove is crutch palsy: an infraclavicular plexopathy caused by
that new symptoms in a patient with a long-standing SCD excessive pressure placed on terminal nerves (most often
are caused by a superimposed PNS lesion, as the follow- the radial) in the axilla as the result of crutches that are
ing case report illustrates. ill fitted or used improperly. Similarly, ulnar neu-
ropathies in the hand can result from the misuse of
CASE REPORT. (FDI first dorsal interosseous; D crutches and walkers (63). The more common situation,
digit) This middle-aged man had had bilateral upper however, concerns mononeuropathies that develop in
limb motor and sensory changes for several years, chronic wheelchair users. Hand symptoms suggestive of
because of a cervical syrinx. Pain and temperature peripheral nerve compromise are surprisingly common
sensations were decreased from the C2 through C5 levels in patients who are active wheelchair users, ranging from
on the left, and the C3 through T1 levels on the right. A 45 to 74 percent (49,60). Most often, the underlying
prior EDX examination had demonstrated prominent nerve lesion is CTS. These probably are the result of
MUAP loss in the C7 through T1 distributions on the left chronic compression of the median nerve caused by the
392 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
increased carpal canal pressure that occurs during the are the ulnar nerve along the elbow segment in quadri-
extremes of wrist flexion and wrist extension. In one plegia, and the common peroneal nerve at the fibular
series of seventy-seven paraplegic patients, thirty-eight head in both quadriplegia and paraplegia. As noted in
(49 percent) had clinical changes suggestive of CTS (61). an earlier section, one of the alternate theories for the
In two other series, consisting of thirty-one and forty- presence of fibrillation potentials being found in the mus-
seven patients, the presence of CTS was confirmed by cles of hemiplegic, quadriplegic, and paraplegic limbs is
EDX examination in 55 to 64 percent of patients hav- that they are caused by one or more of such focal
ing these symptoms (49,60). The percentages were much mononeuropathies (31).
more diverse, however, in regard to the number of
patients who had bilateral CTS: 13 percent in one series;
43 percent in another. The percentages also varied sig- SOME SPECIFIC SPINAL CORD DISORDERS
nificantly in regard to the incidence of ulnar neuropa-
thy in the same two groups of patients: 19 percent in one, In this section, the EDX findings of three different SCDs
40 percent in another. In Aljure et al.s series of forty- will be discussed.
seven patients, nineteen (40 percent) patients had com-
bined CTS and ulnar neuropathy, and in twelve (25 per-
Anterior Horn Cell Disease
cent) of them both of these PNS lesions were bilateral
(60). Overall, there was very close agreement between This category contains many distinct disorders, only a few
the two series regarding the number of patients with one of which will be reviewed.
or more upper limb mononeuropathies: 66 to 68 percent
(49,60). Among the forty-seven patients studied by
Poliomyelitis
Aljure et al., although only 51 percent had symptoms
or signs suggestive of median or ulnar mononeu- Caused by one of three types of neurotrophic viruses, this
ropathies, 63 percent of them had EDX evidence of disease commonly occurred worldwide, often in epidemic
mononeuropathy (61). Whether the incidence of these form, until the introduction and widespread use of the
upper limb mononeuropathies is related to the duration Salk injectable vaccine between 1953 and 1956. Since that
of the paraplegia is controversial; at least two studies period, very few cases of acute poliomyelitis have
have indicated that it can be (49,60,61). Because these occurred in developed countries. Consequently, most
mononeuropathies can compromise the independent practicing electrodiagnosticians have never assessed
functioning of these patients, whose activities of daily patients with acute poliomyelitis. Instead, they are much
living are so dependent on their upper limbs, their early more likely to have encountered patients with the resid-
detection is of particular importance. uals of remote poliomyelitis, to which their recent onset
Plexopathies and mononeuropathies can involve of symptoms may or may not be related.
nerve fibers that derive from the level of the SCD. These The EDX findings in patients with acute
have two separate causes: poliomyelitis consist of the following:
They can develop at the same time as the focal SCD The SNAPs are invariably normal (unless some
does, and for the same reason thus brachial plexus superimposed mononeuropathy is present).
fibers can be injured simultaneously with the cervi- The CMAPs are normal, low in amplitude, or unelic-
cal spinal cord by trauma. Similarly, ischemia caused itable, depending on the specific spinal cord seg-
by compromise of the abdominal aorta can simul- ment(s) involved and the particular muscles used for
taneously damage both the caudal spinal cord as recording. Typically, when the cord segments sup-
well as the roots and plexuses derived from it. plying the recorded muscle are affected, the CMAP
They can develop at various times after the SCD is amplitudes are decreased in proportion to the degree
established and for various reasons. Often the EDX of axon loss. The motor CVs, in contrast, usually
examination can demonstrate or confirm that sepa- are within the normal range.
rate CNS and PNS lesions coexist. On NEE, fibrillation potentials and reduced MUAP
recruitment are noted in muscles innervated by the
Peripheral nerves that originate from spinal cord involved segments. The severity of changes can vary
levels caudal to a focal SCD can manifest focal abnor- substantially from one muscle to another and uni-
malities. Thus, patients with SCDs involving the cervi- lateral as opposed to bilateral changes at a given
cal or thoracic segments can develop focal mononeu- spinal cord level are typical. Fasciculation potentials
ropathies inferior to the level of the lesion, in the paretic and cramp potentials may or may not be seen. An
and paralyzed limbs, caused by both traction and com- important point is that any MUAPs present are nor-
pression. Probably the nerves most commonly involved mal in configuration, because the duration of the
THE ELECTRODIAGNOSTIC EXAMINATION IN SPINAL CORD DISORDERS 393
lesion is such that collateral sprouting has not had As noted earlier, the most common reason for
time to occur (6,8). patients with poliomyelitis to be referred to the EDX lab-
oratory is because they had an acute bout of the disease
With remote poliomyelitis (disorders of at least 12 decades earlier and now are developing new neuromus-
months duration and typically of many years), the EDX cular symptoms. These new symptoms are attributed to
findings are substantially different from those described their remote episode of poliomyelitis, a condition called
above, primarily because of the motor unit remodeling that post-polio syndrome. Although poliomyelitis produces
has occurred as the result of collateral sprouting. Typically, impressive EDX findings, both acutely and as permanent
the EDX examination reveals that the SNAPs are normal, residuals, post-polio syndrome per se causes no distinc-
unless a mononeuropathy (characteristically CTS) is super- tive EDX changes: the EDX findings in patients with
imposed, or unless the patient is more than 60 years of age, remote poliomyelitis who deny new symptoms are essen-
in which case the lower extremity SNAPs may be unelic- tially indistinguishable from those who are experiencing
itable. Concerning the motor NCS, in some instances the them. Thus, the NEE changes are, for practical purposes,
axon loss initially was so severe that the recorded muscle identical between the two groups. Because of this, the
was totally or near totally denervated. Unelicitable or very main reason EDX examinations are performed on
low amplitude CMAPs result when attempts are made to patients with remote poliomyelitis is not to diagnose post-
record from them. In many cases, however, the CMAPs polio syndrome but, rather, to confirm that the patient
are within the normal range in amplitude, even when the actually had poliomyelitis, or to detect some other disor-
recorded muscles, based on subsequent NEE, had under- der that may be responsible for their symptoms. In regard
gone substantial denervation at the time of the acute attack. to the former reason, many middle-aged to elderly
The CMAP amplitudes are misleading in this regard, patients who, for most of their lives, have considered
because they are indicative of the total number of muscle themselves victims of poliomyelitis as infants or young
fibers in the recorded muscle responding to the stimulus, children, have completely normal EDX examinations.
rather than the total number of nerve fibers supplying the Such findings are totally incompatible with their ever hav-
recorded muscles. Thus, they are reflecting the substantial ing had a bout of acute paralytic poliomyelitis, because
amount of collateral sprouting that has occurred. On NEE, poliomyelitis invariably leaves lifelong, often severe, EDX
fibrillation potentials, and occasionally fasciculation poten- residuals, such as very prominent chronic neurogenic
tials, may be seen in minimal to modest numbers in scat- MUAP changes on NEE, even when the clinical abnor-
tered muscles. Frequently the fibrillation potentials are very malities are minimal. Also, as noted earlier, because
low in amplitude, because they are being generated by very severe, widespread, permanent MUAP changes are char-
atrophic muscle fibers. Typically, reduced MUAP recruit- acteristic of remote poliomyelitis, it can be exceedingly
ment is seen in various amounts in different muscles, and difficult, if not impossible, to identify any superimposed
the MUAPs show very prominent chronic neurogenic neuromuscular disorder, whenever the diagnosis of the
changes. MUAPs of three or more times normal duration latter depends principally on detecting MUAP changes on
and amplitude are commonly observed. Such so-called NEE. In the presence of remote poliomyelitis, the MUAPs
giant MUAPs (a term formerly in common use but now very often are so distorted that any additional alterations
discouraged) are seen with such regularity in remote of them cannot be discerned. Among the disorders in the
poliomyelitis and so infrequently in all other disorders, that category are ALS, radiculopathies, and myopathiesthe
any time they are encountered during the NEE on a patient, very ones most often included in the differential diagno-
immediate inquiry should be made regarding whether that sis of patients who have a history of remote poliomyelitis
patient ever had poliomyelitis. (This important fact very and who are now developing progressive weakness (6).
often is not mentioned by referring physicians, either
because they themselves are not aware of it, or they do not
Amyotrophic Lateral Sclerosis
consider it pertinent for the electrodiagnostician to know.)
These grossly abnormal MUAPs frequently are found not ALS is the most common type of AHC disease assessed in
only in clinically normal as well as abnormal muscles in the most EDX laboratories. It differs from all other AHC dis-
symptomatic limbs, but also in muscles of the reputedly orders in that it characteristically manifests UMN, as well
unaffected limb(s). Both patients and their physicians tend as LMN, changes. The EDX findings in ALS vary consid-
to seriously underestimate the extent of spinal cord involve- erably among patients, depending on the distribution, dura-
ment with poliomyelitis, often strikingly so. Occasionally, tion, and progression of the disease process. In a few
NEE of a muscle reveals only a marked mechanical resis- patients, the disease appears to be almost generalized from
tance on needle insertion: no electrical activity at all seen onset. Frequently, in these patients, the process is rapidly
in it. These findings indicate that the muscle was totally progressive. Far more often, ALS begins in one or two spinal
denervated at the time of the acute attack and was never cord segments and then gradually spreads to involve others.
reinnervated (6,9). Often it begins in the AHCs in the C8 and T1 segments,
394 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
thereby presenting as unilateral or bilateral painless hand because of the duration of the lesion; most patients with
wasting. The L5 spinal cord segment also is commonly ini- ALS are studied within a year or so of onset, long before
tially involved. Clinically, these produce a painless foot drop. they can develop, via extensive collateral sprouting, the
On EDX examination, the following typically are unusually large MUAPs seen so often with remote
seen with ALS: poliomyelitis. Reduced MUAP recruitment typically is
prominent also, at least in some muscles. However, as
The SNAPs are normal for agealthough there are noted, ALS has a UMN component as well as a LMN one.
a few reports describing sensory NCS abnormali- When the UMN influence is great, reduced MUAP recruit-
ties with ALS (1416). ment may not be seen because of incomplete MUAP acti-
The CMAPs are normal or abnormal (i.e., low in vation; that is, the UMN lesion controls the MUAP firing
amplitude or unelicitable), depending on whether pattern (9). In this regard, it is interesting to note that the
the appropriate spinal cord segments are involved fibrillation potentials seen in muscles of limbs that are
and, if they are, the severity of axon loss. Because obviously spastic (have hyperactive reflexes and positive
the standard upper limb motor NCS (i.e., median, Babinski sign) in ALS patients invariably are considered
ulnar) are recorded from hand muscles innervated evidence of LMN damage and not UMN involvement.
by the C8 and T1 spinal cord segments, one or more Thus, where ALS is concerned, the concept that fibrilla-
low-amplitude CMAPs are found with some fre- tion potentials can be seen with UMN disorders, because
quency. If ulnar motor (recording FDI) NCS are of transsynaptic degeneration, is ignored.
added, in a significant number of patients, a split The EDX examination can be very helpful for dis-
hand is seen: the ulnar motor CMAP (FDI) is low tinguishing ALS from certain other generalized disorders
in amplitude or unelicitable, whereas the ulnar with which it can be confused clinically, such as
motor CMAP (hypothenar) is normal, or only polymyositis or myasthenia gravis with prominent bulbar
slightly low in amplitude. Often, in these circum- symptomatology. Unfortunately, however, it cannot dis-
stances, the median motor CMAP (recording thenar) tinguish a radiculopathy from early ALS, when the latter
also is affected. Why the muscles on the lateral initially presents in a monosegmental distribution. Simi-
aspect of the hand are frequently much more larly, ALS restricted to the L5 and S1 spinal cord segments
severely denervated than those on the medial aspect bilaterally cannot be distinguished from a cauda equina
of a hand is unclear, but this finding has diagnostic lesion by EDX examination. Thus, as noted previously,
significance. In our experience, with proximal the EDX presentation of ALS is somewhat nonspecific, in
lesions, it is seen only with AHC disease (6,9). that it is simply that of an intraspinal canal lesion. The
diagnosis rests principally on the diffuse nature of the
The diagnosis of ALS in the EDX laboratory, simi- active and chronic denervation with ALS, and the pres-
lar to that of all other AHC disorders, rests principally on ence of fasciculation potentials in at least some muscles.
the NEE findings. Typically, an extensive NEE is required, A pertinent note is that ALS cannot be diagnosed by EDX
consisting of sampling at least one muscle innervated by examination in a patient with remote poliomyelitis
every segment in the upper and lower limbs, as well as the because the findings, for the most part, are identical.
mid to lower thoracic paraspinal muscles, and, if neces- Although fibrillation potentials may be abundant with
sary, one or two muscles innervated by the cranial nerves. ALS, this feature is not diagnostic in itself.
The goal is to find widespread fibrillation potentials and
suggestive chronic neurogenic MUAP changes with at
Kennedys Disease
least some fasciculation potentials. The presence and den-
sity, if they are present, of fibrillation potentials in any This sex-linked recessive AHC disorder involves the sen-
given muscle tends to vary directly with the degree of clin- sory and motor neurons in both the bulbar and spinal
ical weakness it manifests. In contrast, fasciculation cord segments. On EDX examination, a very unusual
potentials, which often are even more widespread than combination of changes typically is found. This consists
the fibrillation potentials, tend to be more abundant in of NCS abnormalities suggestive of a pure sensory
less affected muscles. (This is to be expected, because fas- polyneuropathy (i.e., low amplitude or unelicitable
ciculation potentials are generated by intact motor units, SNAPs), along with NEE abnormalities indicative of a
whereas fibrillation potentials are generated by muscle very chronic AHC disease (i.e., prominent chronic MUAP
fibers of degenerated motor units.) changes, with sparse numbers of fibrillation potentials),
The chronic neurogenic MUAP changes typically but often with abundant fasciculation potentials, espe-
seen with ALS usually are far less impressive than are those cially in the face. In contrast to the sensory NCS and NEE,
frequently observed with remote poliomyelitis. Thus, the motor NCS most often are normal, although they may
although MUAPs of increased duration are common, those be of low amplitude if the recorded muscles are substan-
of increased amplitude as well are relatively rare. This is tially denervated (6).
THE ELECTRODIAGNOSTIC EXAMINATION IN SPINAL CORD DISORDERS 395
Spinal Cord Injuries voluntary MUAPs. On the left, the infraspinatus, deltoid,
biceps, and brachioradialis appeared normal; fibrillation
Severe focal trauma to the spinal cord is ... one of the potentials were present in the triceps, pronator teres, and
most catastrophic injuriessocially, economically, and flexor carpi radialis. In the triceps, no voluntary MUAPs
physicallythat can occur... (64). These lesions affect could be activated, while in the remaining two muscles,
very predominantly males (more than 82 percent in one markedly reduced MUAP recruitment was noted; in the
series) and those in younger age groups: 80 percent of flexor pollicis longus, extensor indicis proprius, abductor
patients are less than 45 years old. The median age of pollicis brevis, and first dorsal interosseous, no voluntary
occurrence is 25 years, the most common age is 19 years. MUAPs could be activated, but no fibrillation potentials
Fracture-dislocation of the spine, most often caused by were seen. Thus, the EDX examination confirmed the
sudden, violent flexion, is responsible for the majority of asymmetry of the cervical SCI; the lesion appeared to
acute spinal cord injuries (SCI). The most common sites be at the level of the C6 cord segment on the right and
for these are the C5C6, C6C7, and the T12L1 junc- the C7 segment on the left.
tions. Whenever substantial spinal cord damage occurs
without fracture-dislocation, usually it is the result of a
Intraspinal Canal Neoplasms
hyperextension injury to the cervical spine in a patient
with spondylosis. The most common causes for acute SCI Neoplasms that involve the spinal cord portion of the
are vehicular accidents, falls, gunshot and stab wounds, CNS are considerably less frequent than those that
and diving accidents (64). involve the brain. In one series, the latter were almost
The EDX examination has a limited role in the ini- seven times more common (2). Three types of intraspinal
tial assessment of patients with these injuriesfar less of canal neoplasms are recognized:
a role, for example, than that of neuroimaging studies.
Nonetheless, at times EDX examinations can be quite intramedullary, which arise within the substance of
helpful, especially by demonstrating the presence or the spinal cord, e.g., astrocytomas, ependymomas,
absence of coexisting plexopathies. Moreover, later in the and hemangioblastomas;
course, it can detect the presence of superimposed plex- extramedullary-intradural, those situated outside
opathies and mononeuropathies, a task for which neu- the spinal cord, in the meninges or roots, e.g., menin-
roimaging studies are of no benefit. giomas, schwannomas, and filum terminale ependy-
momas; and
CASE REPORT. (D digit; LAC lateral ante- extramedullary-extradural, those in the epidural tis-
brachial cutaneous nerve) A 20-year-old man sustained sues or the vertebral bodies. The latter include
a cervical cord injury in a diving accident, which epidural tumors that are primary (multiple
rendered him quadriplegic. The clinical findings were myeloma, osteogenic sarcoma, and chordomas), and
slightly asymmetrical from side to side, with those on the those that are secondary (metastatic neoplasms).
right being at a slightly higher cervical cord level. This Prostate and breast cancer frequently metastasize
asymmetry was confirmed on EDX examination. On to the vertebral column and epidural space.
NCS, all SNAPs were normal and equal bilaterally,
including those that assess the C6 DRG (median, The relative frequency of intraspinal canal neo-
recording D1, LAC), the C7 DRG (median D2, median plasms varies from one series to another but epidural
D3), both C6 and C7 DRG (radial, recording thumb tumors, overall, are the most frequent (2,3).
base), and the C8 DRG (ulnar, recording D5). On motor Pain is probably the most common symptom with
NCS, the routine median (recording thenar) and ulnar intraspinal canal neoplasms. It may be present for months
(recording hypothenar) were normal bilaterally, as were before other symptoms or segmental signs appear. At some
axillary motor NCS (recording deltoid) bilaterally. The point in their course, these patients frequently have symp-
musculocutaneous (recording biceps) and radial motor toms and signs in a radicular or multiradicular distri-
(recording brachioradialis) CMAPs were asymmetrical, bution and, for this reason, many are referred to the EDX
with those on the right being low in amplitude (4 mV vs. laboratory for assessment. Although the EDX findings typ-
12 mV, musculocutaneous; 5 mV vs 13 mV, radial). On ically are nonspecific in regard to etiology, their presence
NEE on the right, the infraspinatus and deltoid appeared frequently initiates the appropriate neuroimaging studies
normal. Fibrillation potentials and severely reduced (if they were not already planned). This is illustrated by
MUAP recruitment were present in the biceps and the following case history; in this instance, the symptoma-
brachioradialis with lesser but similar changes in the tology was somewhat unusual in that pain was not present.
pronator teres; the flexor pollicis longus, extensor indicis
proprius, first dorsal interosseous, and abductor pollicis CASE REPORT. (EDB extensor digitorum brevis;
brevis contain neither fibrillation potentials nor TA tibialis anterior; AH abductor hallucis) A 32-year-
396 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
old man developed spontaneous weakness of plantar 2. Adams RD, Victor M, Ropper AA. Principles of Neurology, 6th
ed. New York: McGraw-Hill, 1997.
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Arch Phys Med Rehabil 1969; 50:271273.
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diagnosis of multiple right lumbosacral radiculopathies. poliomyelitis. Phys Med Rehabil Clinics N Amer 1994; 5:559569.
An EDX examination revealed the following. The sensory 9. Wilbourn AJ. Electrodiagnostic evaluation of the patient with pos-
sible ALS. In: Belch JM, Schiffman P (eds.), Amyotrophic Lateral
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CMAPs (recording EDB) were normal on the left, but very 10. Cole JL. Central nervous system physiology. In: DeLisa JA, Gans
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peroneal motor CMAPs (recording TA) were normal on 11. Grundy BL, Friedman W. Electrophysiological evaluation of the
the left and low in amplitude on the right (7 mV vs. 2.4 patient with acute SCI. Critical Care Clinics 1983; 3:519548.
12. Little JW, Stiens SA. Electrodiagnosis in SCI. Phys Med Rehabil
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and the M-components of the H-wave test bilaterally, 13. Dumitru D. Electrodiagnostic Medicine. Philadelphia: Hanley Bel-
were normal and equal in amplitude from side to side. The fus Inc., 1995.
14. Shefner JM, Tyler HR, Krarup C. Abnormalities in the sensory
H-reflex was unelicitable on the right, while low in action potential in patients with amyotrophic lateral sclerosis.
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potentials were present in moderate numbers in L5 and 15. Mondelli M, Rossi A, Passero S, Guazzi GC. Involvement of
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17. Pullman SR, Rubin M. Large amplitude sensory nerve action
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the L5 and S1-innervated muscles bilaterally were chronic 18. Goldkamp O. Electromyography and nerve conduction studies
in 116 patients with hemiplegia. Arch Phys Med Rehabil 1967;
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gluteus medius. The EDX findings were interpreted as 19. Aisen ML, Brown W, Rubin M. Electrophysiologic changes in
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42:623626.
involving the L5 and S1 segments/roots bilaterally, more 20. Brown WF, Snow R. Denervation in hemiplegic muscles. Stroke
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Spontaneous electromyographic potentials in chronic spinal cord
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23. Liberson WT, Yhu HL. Proximal-distal gradient in the involve-
CONCLUSION ment of the peripheral neurons in the upper extremities of hemi-
plegics. Electromyogr Clin Neurophysiol 1977; 17:281286.
24. Spaans F, Wilts G. Denervation due to lesions of the
The EDX examination can be helpful in the assessment central nervous system. J Neurology Sciences 1982; 57:291305.
of suspected SCDs and of the complications arising from 25. Spielholz NI, Sell GH, Goodgold J, Rusk HA, Greens SK. Elec-
trophysiological studies in patients with spinal cord lesions. Arch
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nationNCS, NEE, and special studiescan be benefi- involvement in hemiplegia. Arch Phys Med Rehabil 1969;
50:632637.
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assessment of spinal cord lesions. Arch Phys Med Rehabil 1976;
57:7074.
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29
Electrophysiologic
Evaluation of the
Spinal Tracts
n various disorders of the spinal cord the posterior tibial nerve at the ankle, but any other acces-
When stimulating a nerve in the arm, it is mostly tiple sclerosis, stroke, and in the critical care unit, SEPs
possible to consistently recognize an Erbs potential (N13/ can be applied clinically.
P1314), reflecting the voltage difference recorded In SCI, as well as in stroke patients, SEPs can have
between the cervical spine and the midfrontal scalp (Fz), prognostic value in the acute and subacute stages
and an N20 in recordings made between the contralateral (1,3,4,7,8). Although less specific, SEPs seem to be rather
hand area of the scalp (C3'on the left scalp/C4' on the sensitive in predicting outcome in the acute phase, mean-
right scalp) and Fz. The cervical N13 probably reflects ing that when SEPs are absent, prognosis seems to be
postsynaptic activity in the spinal cord, whereas P14 poor. When they are present, certainly if latency times are
reflects activity in the medial lemniscus. The N20 is prob- within normal limits, a better outcome can be expected.
ably generated in the primary somatosensory cortex. The specificity might increase when combining SEPs with
Similarly, P37 and N45 components are found in MEPs.
recordings over the vertex of the scalp (Cz) with respect In cervical spondylytic myelopathy, SEPs can be
to a cephalic reference following stimulation of the pos- helpful in the evaluation of the severity and level of the
terior tibial nerve in the lower extremities. The N20 and lesion, certainly if combined with other electrodiagnos-
N45 components are followed by a number of different tic techniques, as well as in the follow-up of patients after
peaks, according to the site of recording over the scalp surgery or other applied treatment or rehabilitation pro-
and probably reflecting distinct cortical generators (1). cedures (9,10).
Comparable to the cervical N13, a negative potential can In the intensive care unit, patients in coma are
be detected over the cauda equina and over the thora- unlikely to recover from their condition when the corti-
columbar spine, related predominantly to postsynaptic cal responses of the SEPs are bilaterally absent. This is
activity in the lumbar spinal cord (6). especially true for atraumatic (e.g., anoxic) coma in adult
Finally, the central somatosensory conduction time patients (5).
(CSCT) can be determined as being the time interval In radiculopathy, the clinical application of SEPs is
between the major negative peak identified in the spine limited, certainly when SEPs are elicited by multiseg-
(e.g., N13 for the cervical spine) and the initial major neg- mental nerve trunk stimulation. Therefore, dermatomal
ative peak of the cortical response (e.g., N20). It can be stimulation might have a higher diagnostic yield. But,
helpful in detecting slowed transmission through the cen- even then, scalp-recorded dermatomal SEPs have shown
tral neuraxis, for example between the cervicomedullary to be abnormal in only 20 to 25 percent of patients with
junction and the cortex. lumbosacral compressive root lesions (11,12) and in a
In addition to absolute SEP component and inter- similar or even lower number of cases with cervical
component latencies, interside latency differences are radiculopathy (13).
taken into account. Responses are considered to be abnor-
mal if these exceed the mean value for control subjects
by more than 2.5 or 3 standard deviations. Less strin- MOTOR EVOKED POTENTIALS
gent criteria may lead to high false-positive results. The
presence or loss of specific components is also important Fritsch and Hitzig (14), in 1870, and Ferrier (15), in 1873,
in determining SEP abnormality. SEP amplitude and mor- already showed that the exposed motor cortex could be
phology changes, on the other hand, are less reliable activated by electrical stimulation, and that this excita-
because of the wide variability in normal subjects. Inter- tion was propagated along the descending motor tracts.
side amplitude differences of more than 50 percent, how- This method offered an interesting alternative to studies
ever, may be of some significance. of spontaneous and induced brain lesions to reconstruct
the functional topography of the motor cortex (16).
In 1954, Gualtierotti and Paterson tried to stimulate
Clinical Applications
the unexposed cerebral cortex, which at that time was
Altered SEPs are not specific for the age or the nature of found to be very painful (17). It was only in 1980 that
the underlying pathology and provide limited informa- Merton and Morton developed a technique for percuta-
tion on the exact location of the lesions proximal to the neous delivery of individual electrical stimuli over the
dorsal root ganglion. Although SEP abnormalities are eti- motor cortex of awake humans to elicit contralateral limb
ologically nonspecific, they can be very helpful in several movements (18). This was probably the beginning of a
clinical conditions, in the diagnosis of numerous spinal new era in neurophysiologic research and clinical neuro-
disorders, in determining prognosis, in evaluating treat- physiology.
ment, and in following up patients, for example, during In 1965, Bickford and Fremming developed a stim-
their recovery or rehabilitation. ulator capable of producing powerful magnetic pulses
Especially in spinal cord injury (SCI), spondylytic and demonstrated noninvasive peripheral nerve stimula-
myelopathy, spinal cord compression, radiculopathy, mul- tion by pulsed magnetic fields (19). However, for techni-
ELECTROPHYSIOLOGIC EVALUATION OF THE SPINAL TRACTS 401
cal reasons it remained impossible until 1982 to record insulated copper coils together with other electronic cir-
the obtained muscle action potentials (20). The method cuitry, such as temperature sensors and safety switches.
was further refined, and in 1985, Barker et al. introduced The main enclosure and the stimulating coil are inter-
a new type of magnetic cortical stimulator based on the connected by means of a flexible high-power cable.
principle of electromagnetic induction (21). Magnetic stimulators work by charging one or more
Electromagnetic induction, producing a current in energy storage capacitors and then rapidly transferring
a conductive object by using a moving or time-varying this stored energy from the capacitor(s) to the stimulat-
magnetic field, was first described by Michael Faraday ing coil when the stimulus is required. The difficulty in
in 1831 at the Royal Institute of Great Britain, and was producing magnetic nerve stimulators is related to the
the most relevant experimental observation for magnetic high discharge currents, voltages, and power levels
stimulation. Faraday wound two coils on an iron ring and involved in producing a brief magnetic pulse. Typically,
found that, whenever the coil on one side was connected 500 J of energy has to be transferred from the energy stor-
with or disconnected from a battery, an electric current age capacitor into the stimulating coil in about 100 s.
passed through the coil on the other side. The iron ring Power, measured in watts, is equivalent to joules per sec-
acted as a channel, linking the magnetic field from the ond. Consequently, the power output of a typical mag-
first coil to the second. A change in the magnetic field, netic stimulator during the discharge phase is 5,000,000
related to the changing current in the first coil, induced a watts (5 MW) sufficient to provide the electricity neces-
current in the second coil. In fact, the iron ring only sary for 1,000 homes for 1/1000th of a second. During
improved the coupling efficiency between the two coils the discharge, energy, initially stored in the capacitor in
and made the experiment more practical to perform, but the form of electrostatic charge, is converted into mag-
the ring could be dispensed with if sufficient primary cur- netic energy in the stimulating coil in approximately 100
rent was delivered. This is the case in noninvasive mag- ms. This fast rate of energy transfer produces a time-vary-
netic stimulation, where the stimulating coil acts as one ing magnetic field build-up, which induces tissue currents
coil, space as the medium for the flow of the magnetic in the vicinity of the coil in the order 1 to 20 mA/cm2.
field, and the conductive living body as the second coil. Since the magnetic field strength falls off as the dis-
Mainly because transcranial magnetic stimulation tance from the stimulating coil increases, the stimulus
(TMS) is less invasive and painless, the number of clin- strength is maximal close to the coil surface. The stimu-
ical studies has increased over the last decade and many lation characteristics of the magnetic pulse, such as depth
clinical applications have become available (2225). The of penetration, strength, and accuracy, depend on the rise
technique provides reliable information about the func- time, peak magnetic energy transferred to the coil, and
tional integrity and conduction properties of the corti- the spatial distribution of the field (21). The rise time and
cospinal tracts and motor control in the diagnostic and peak coil energy are governed by the electrical charac-
prognostic assessment of various neurologic disorders. teristics of the magnetic stimulator and stimulating coil,
It allows physicians to follow the evolution of motor whereas the spatial distribution of the induced electric
control and to evaluate the effect of different therapeu- field depends on the coil geometry and the anatomy of the
tic procedures. region of induced current flow.
In 1985, the magnetic stimulators originally pro-
duced at Sheffield, UK were equipped with circular coils
Principles of Magnetic Stimulation
of 90 mm mean diameter. These proved to be most effec-
A magnetic field is generated by passing an electric cur- tive for the stimulation of the human motor cortex con-
rent through a coil of wire. A magnetic pulse produced trolling the upper limbs with a large cortical representa-
from an electric current pulse will induce a current in an tion, and also for stimulation of spinal nerve roots. To
electrically conductive region, such as the human body. date, circular coils with a mean diameter of 80 to 100 mm
If the induced current is of sufficient amplitude and dura- are the most widely used in magnetic stimulation.
tion, it will stimulate neural tissue in its vicinity in exactly Presently, two type of magnetic stimulators are avail-
the same way as conventional electrical stimulation. able: monophasic (such as the Magstim 200 stimula-
Magnetic nerve stimulators typically consist of two tor) and polyphasic (such as the Cadwell stimulator),
distinct parts: a high-current pulse generator producing which modifies the way in which cerebral structures are
discharge currents of 5,000 A or more; and a stimulat- excited.
ing coil producing magnetic field strengths of 1 Tesla or Electrical and magnetic transcranial magnetic stimu-
more, with a pulse duration of about 1 ms. The genera- lation activate the brain at different sites (26). Whereas the
tor consists of a capacitor charge/discharge system electrical stimulus excites the corticospinal neurons directly,
together with the associated control and safety electron- the magnetic stimulus excites these neurons transsynapti-
ics. The stimulating coil, normally housed in molded plas- cally, thus explaining the extra delay of a few milliseconds
tic covers, consists of one or more tightly wound and well in magnetic stimulation as compared to electrical.
402 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
The physical characteristics of the frequently used Response latency shortening during voluntary con-
90 mm-diameter monophasic coil (Magstim Company, traction is likely to reflect the application of the size prin-
Whitland, UK), are an inside diameter of 66 mm, an out- ciple of Henneman: the first corticomotoneuron cells to
side diameter of 123 mm, 14 turns, a peak magnetic field fire during a voluntary contraction are those that conduct
strength of 2.5 Tesla, and a peak electric field strength of most slowly; with increasing contraction larger, faster
510 V/m. conducting neurons are recruited (30). Moreover, single
motor unit studies in humans have demonstrated that the
first motor units to be stimulated magnetically are the first
Physiologic Mechanisms
to fire under voluntary control, and are of relatively long
As previously mentioned, transcranial stimulation pro- latency (29). Later, larger units with faster conducting
vides the first objective laboratory measurement of cor- axons have shorter latencies.
ticospinal tract function in humans without surgical expo- With electrical stimulation however, an additional
sure. The motor evoked potential procedure consists of mechanism for the latency decrease is possible. To under-
transcranial stimulation followed by measurement of the stand this one must know that a series of positive waves
compound muscle action potential (CMAP) from differ- is recordable from the contralateral corticospinal tract
ent limb and trunk muscles. when an electrical stimulus is delivered to the exposed
Magnetic stimulation allows the physician to safely, motor cortex of animals such as cat, baboon, and mon-
easily, and effectively stimulate most neural structures, key (31,32). The first of these waves is termed the direct
unimpeded by fat and bone, and without discomfort to or D wave; later descending volleys are indirect or I
the patient. Until now, the technique has been mainly waves. The D wave is so called because its latency is too
applied for stimulation of the peripheral and central short to allow for an interposed synapse, and it is there-
motor pathways. Responses following magnetic stimu- fore conducted directly in fast pyramidal tract axons. The
lation can be recorded in a standard fashion from either D wave is most probably produced by the stimulation of
the nerve or the muscle. Signal averaging is usually not proximal nodes of axons of larger corticospinal tract neu-
necessary. Compound motor evoked potentials from var- rons, whereas I waves appear at higher intensities of stim-
ious muscles can be obtained in response to magnetic ulation and are probably caused by synaptic activation of
transcranial motor cortex stimulation, and nerve root, the same corticospinal neurons, but through intracortical
plexus, and peripheral nerve stimulation. neural elements. There is only minimal temporal disper-
When the CNS is stimulated, the stimulation thresh- sion between the D and I waves, consistent with both
old can be reduced by approximately 30 percent, the being conducted in the fast corticospinal tract axons. The
response amplitude can be increased and the response excitation of the alpha motor neuron cells of the spinal
latency reduced by some 1 to 6 ms (usually about 12 ms) cord requires temporal and spatial summation of stim-
through preactivation of the target muscle. This tech- uli. A stimulus to the cortex will produce a volley of I
nique, referred to as facilitation, has been described in waves, possibly preceded by a D wave, according to the
considerable detail (2628). The phenomenon of facili- stimulus characteristics; anterior horn cell firing and thus
tation was noted in an early stage of the original trans- EMG activity will result. When comparing muscle
cutaneous electrical stimulation studies of Merton and responses to magnetic stimulation with those electrically
Morton, and is equally prominent with magnetic brain induced, magnetic responses are of longer onset latency
stimulation (29). by around 2 ms (in hand muscles), and are of simpler
The relationship between background force and waveform, shorter duration, and larger amplitude. These
CMAP amplitude is approximately linear with electrical differences suggest that electrical and magnetic stimula-
stimulation, whereas a small background contraction of tion activate the motor pathways at different sites. It is
the order of 5 percent maximum has a striking facilitat- probable that electrical stimulation excites corticospinal
ing effect with magnetic stimulation. neurons directly, causing an initial D wave and subse-
There are several different processes of facilitation. quent I wave volleys, and that magnetic stimulation acts
It is probable that facilitation occurs both at spinal and transsynaptically, the better synchronized and longer
cortical levels. When attention is focused on accurate latency response being caused by a more homogeneous
force production in a particular muscle, then facilitation site of excitation results in I waves, but no D wave,
occurs at small forces, which is likely to involve cortical impinging on the spinal motor neuron. However, later
mechanisms. Presumably, during spinal facilitation, more studies have shown that it is also possible to produce a
spinal motor neurons are recruited by an unchanged D wave in the pyramidal tract with magnetic stimulation
descending volley because their excitability is raised by when changing the current direction in certain (monopha-
the descending voluntary input, whereas the cortical facil- sic) coils.
itation depends on an actual increase in the descending Keeping these multiple descending volleys in mind,
volley caused by the magnetic stimulus. and coming back to the phenomenon of facilitation, an
ELECTROPHYSIOLOGIC EVALUATION OF THE SPINAL TRACTS 403
additional mechanism for the latency decrease is possible certain regions of the motor cortex, and that one has to
with electrical stimulation: when the muscle is relaxed, the take into account that the size, latency, and duration of
initial D wave may be insufficient to discharge the motor the MEPs are critically dependent on the type, intensity,
neuron; thus summation with the following I wave may be and localization of the stimulus as well as on the excitabil-
required to fire the anterior horn cell, with a resultant ity of the cortical and spinal motoneurons.
longer latency response. During voluntary activity, there Depending on the purpose of the test, there are many
are likely to be motor neurons near enough to threshold important parameters that can be measured in cortical
for activation to occur and the D wave causes a shorter stimulation such as stimulation threshold, MEP latency,
latency with voluntary contraction. Whether summation MEP amplitude, response morphology, central motor
of I waves contributes in the same fashion as in magnetic conduction time, silent period duration, fatigue, intra-
stimulation is uncertain, but it is probable that the varia- cortical inhibitory and excitatory pathways, and others.
tion in MEP onset latency observable when a series of The central motor conduction time (CMCT) can be
brain stimuli is delivered, whether magnetic or electrical, calculated by subtracting the latency in response to spinal
is related at least in part to this phenomenon. root stimulation from the latency in response to cortical
The synaptic delay for discharge by the motoneuron stimulation (37,38). But it may be also calculated by using
has two components: the excitatory postsynaptic poten- the F wave latency (39):
tial (EPSP) delay ( 0.3 ms) and the delay for the EPSP
to reach firing level. With strong stimulation of the motor CMCT Latency from cortex to muscle
cortex, the number of corticospinal neurons discharging, F latency (motor terminal latency 1)
and thus the amount of spatial summation of EPSP, is 2
increased, thereby shortening the delay for motoneurons
to attain firing level. When the muscle is relaxed, the CMCTs calculated using F waves usually exceed
motoneuron requires increased excitation to reach firing those based on root stimulation, which stimulates the
level, such as could be provided by temporal summation peripheral motor axons distal to the ventral roots.
of motoneuron EPSPs elicited by direct and indirect cor- CMCT can be affected by a number of factors at sev-
ticospinal discharge. The need for indirect corticospinal eral levels, including the activation time of the pyrami-
discharge would impose an additional delay in the CMAP dal tract neurons, the transmission time between motor
attributable to cortical synaptic delays and thus largely cortex and spinal motor neurons, the activation time of
account for the difference observed ( 2ms) in CMAP motor neurons, the time between motoneuron discharge
latency in contracted and relaxed muscle. and the site of stimulation of the motor root, and prob-
Another phenomenon (inhibitory) is the interruption ably others (40).
of the ongoing voluntary muscle contraction produced by The size of electromyographic responses to cortical
magnetic transcranial stimulation of the motor cortex. This stimulation or MEP amplitude can be affected by the type
phenomenon appears as a period of EMG silence lasting of cortical stimulator (high-voltage electrical or magne-
about 100 to 150 ms, which is defined as the silent period toelectrical) and by the stimulus intensity, as well as the
(3335). This silent period is produced by a mixture of cor- activation of other muscles.
tical and spinal inhibitory effects. Approximately the first CMCT can be increased and MEP size reduced by
50 ms of the silent period are caused by both cortical and several factors such as reduced excitability of the motor
spinal mechanisms. From this point onwards, spinal mech- cortex, slowed conduction between the motor cortex and
anisms are progressively less important and the cortical spinal motor neurons, several factors at the motor neu-
inhibitory mechanisms act on the neural elements of the ron level, reduced conduction velocities in motor axons,
corticomotoneuronal system at motor cortical level: a mag- and the like.
netic stimulus given during the second half of the silent Clinical applications of transcranial stimulation
period does not produce MEP, whereas electrical stimula- include SCI, multiple sclerosis, anterior horn cell disor-
tion evokes almost unchanged muscle responses. In other ders (e.g., amyotrophic lateral sclerosis), spondylotic
words, the unexcitability of the motor cortex after a sec- myelopathy, stroke, radiculopathy, various neuropathies,
ond magnetic stimulus indicates that the motor cortex per epilepsy (41,42), degenerative ataxic disorders such as
se is inhibited, whereas the excitability with electrical stim- cerebellar ataxia and Friedreichs ataxia (4345), cranial
ulation implies that corticospinal axons and spinal nerve disorders (mainly the facial nerve: 46,47), pathol-
motoneurons are not inhibited (33,34,36). ogy of the respiratory muscles, and several others. Tran-
scranial stimulation can also be used in the operating
room during surgery, where monitoring motor conduc-
Clinical Applications
tion is a useful indicator of the integrity of the central
Studies in normal subjects have shown that there is a motor pathways, especially during neurosurgical opera-
somatotopic localization and preferential excitability of tions (48), as well as in the intensive care unit (49). Just
404 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
like SEPs, MEPs can have prognostic value, mainly in SCI In stroke, MEPs not only correlate well with clini-
and stroke. Finally, it can be useful in follow-up of motor cal motor function, but can have prognostic value in the
function during treatment and rehabilitation. (sub)acute stages. In some studies, abnormalities in cen-
In multiple sclerosis (MS), most authors found tral motor conduction were shown to be a better indica-
clearly delayed CMCTs (5052) Mills and Murray, 1985; tor of outcome than SEPs (Macdonell et al., 1989). MEPs
in up to 79 percent of patients with definite MS (53). seem to have a high specificity, but a much lower sensi-
Absent MEPs were mostly seen in those MS patients with tivity in predicting functional outcome. More specifically,
marked clinical disabilities. Increased CMCTs were often when MEPs are present in the acute phase, outcome usu-
accompanied by dispersed and reduced size of MEPs, but ally is favorable, whereas absent MEPs in the acute stages
no correlation was found between CMCT and duration are often not correlated with poor outcome (7476).
of the disease. In MS patients with sexual dysfunction, Absent MEPs seem to be most characteristic of corti-
prolonged CMCTs of the pelvic floor muscles were seen calsubcortical infarction (Macdonell et al., 1989).
(54). A higher rate of MEP abnormalities was found when Finally, MEPs can be useful in the follow-up of
altered MRI signal was localized within the parietal cor- motor function during treatment and rehabilitation,
tex, centrum semiovale, and the internal capsule (55). mainly in SCI and stroke patients, which not only is inter-
In anterior horn cell disorders, more specifically in esting for the therapeutic staff, but also for the patients
patients with amyotrophic lateral sclerosis (ALS), pro- motivation (7677).
longed or absent MEPs were found, either with high-volt-
age electrical stimulation (37,52,56) or with magneto- References
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30 Acute Nontraumatic
Myelopathies
his chapter on acute and chronic non- causes of that syndrome; e) read the more-detailed
horn cell (lower motor neuron) disease also reflects the Pathologic processes involving the dorsal columns
loss of about 50 percent of anterior horn cell function (3). cause gait ataxia, because the brain is deprived of infor-
Anterior horn cell disease may cause severe muscle atro- mation about the position of the feet. If the lesion affects
phy, with the loss of up to 80 percent of muscle volume, the cervical cord, there is also upper extremity ataxia.
whereas paralysis from UMN disease usually causes no Vision is able to compensate for loss of position sense to
more than 20 percent loss of muscle volume. Anterior a great degree and thus minimize sensory ataxia. Dorsal
horn cell dysfunction is frequently accompanied by fas- column disease also causes paresthesias, which are
ciculations in the muscle that it innervates. described as tingling, numbness, crawling, or deadness,
Acute paralysis caused by spinal cord disease is and which are felt mainly in the distal parts of the extemi-
accompanied by muscle hypotonia and the loss of tendon ties. Paresthesias probably result from ectopic discharge
and cutaneous reflexes, caused by the sudden withdrawal in damaged dorsal column axons and may be present
of activity descending to anterior horn cells from the cere- before any abnormalities are detectable on neurologic
bral cortex and the brain stem. Some of these pathways examination (7). Similar sensations can be evoked by elec-
produce inhibition and others, facilitation, of anterior horn trical stimulation of the dorsal columns. A special type
cell motor neurons, but the overall effect is facilitation. This of paresthesia of dorsal column origin is Lehrmittes phe-
lack of facilitation causes the motor neurons to become nomenon, in which the patient experiences an electric
hyperpolarized to a degree such that their preserved con- shock-like sensation originating over the cervical spine
nections with dorsal root afferents from muscle spindles, and extending down the back and sometimes into the
cutaneous receptors, and the like. are unable to cause suf- extremities. Dorsal column function is tested by examin-
ficient membrane depolarization to generate action poten- ing the patients ability to perceive changes in the position
tials in the motor neurons. This syndrome of areflexia and of toes and fingers without visual cues and by the ability
hypotonia is referred to as spinal shock (4). Over several to perceive tuning fork vibration. Position sense is not lost
days to several weeks, muscle tone and reflexes return, until about 75 percent of posterior column axons have
probably because of the activation of silent synapses ceased to function (2).
(synapses on motor neurons connected to dorsal root affer- Dysfunction of the lateral spinothalamic tract causes
ent fibers that have not been active). Also, there is evidence decreased perception of pain and temperature on the con-
that dorsal root afferent fibers sprout to occupy synap- tralateral side of the body, one or two dermatomes below
tic sites vacated by the degenerating descending fibers (5). the level of the lesion. Spinothalamic tract lesions usu-
When this occurs muscles, become hypertonic and tendon ally do not cause paresthesia. Patients become aware of
reflexes become hyperactive (spasticity). their pain and temperature deficit when they experience
In more slowly progressive weakness of spinal ori- painless cuts or burns. The bilateral destruction of the lat-
gin, a gradual replacement of descending facillatory influ- eral spino-thalamic tracts abolish erection, ejaculation,
ences by dorsal root and horn facilitatory influence results and orgasm.
in slowly developing spasticity without an intervening
phase of hypotonia and hyporeflexia.
Pain
Spasms are a phenomenon related to, but not iden-
tical with, spasticity in which activation of small myeli- Pain associated with spinal cord disorders can be classi-
nated and unmyelinated dorsal root afferents excites fied as being of three types (8). Two of these, local pain
motor neurons in several adjacent spinal segment to cause and radicular pain, are varieties of nociceptive pain; that
the contraction of synergistic muscles. The most common is, pain generated from pain receptor and proceeding
types of spasms are flexor, adductor, or extensor spasms from there through spinothalamic pathways. The other
in the legs of patients with paraplegia or quadriplegia (6). type, central neuropathic pain, is rarely seen as a pre-
Spastic paralysis is usually associated with the pres- senting or early manifestation of spinal cord dysfunction.
ence of an extensor plantar response (Babinskis sign). It results from damage to the spinal cord pain transmis-
sion pathways producing what might be called a hallu-
cination of pain.
Sensory Abnormalities
Local pain arises from pain receptors in the para-
All sensory information passing from the periphery to the vertebral muscles or from the bones or ligaments of the
brain, except from the head, passes through either the spine, although it may come from the spinal cord itself,
dorsal columns or the spinothalamic tracts of the spinal probably from pain receptors in the blood vessels of the
cord. The dorsal columns convey sensations of toe and cord. Local pain is most intense centered over its source.
finger position and of touch from the ipsilateral body. The It may extend laterally to some degree. When the cervi-
lateral spinothalamic track mediates temperature and cal vertebrae are involved, it may extend to the shoulders.
pain sensation from the opposite side. An anterior Involvement of lumbar vertebrae can cause pain in the
spinothalamic track also carries touch sensation. hips and buttocks, and pain originating in thoracic ver-
ACUTE NONTRAUMATIC MYELOPATHIES 409
tebrae may spread several inches to either side of the mid- onset of spasticity. Because of a small-capacity hyper-
line. Less than 5 percent of local back or neck pain orig- reflexic bladder, patients experience urinary frequency,
inates from the spinal cord or cauda equina. About 20 urgency of micturition, and urge incontinence. If the
percent is caused by abnormalities of the spine, and the slowly progressive condition affects the conus medullaris
cause of the remaining 75 percent is unknown (9); pre- or cauda equina, the bladder and sphincter muscles slowly
sumably it originates in the paravertebral structures. become atonic and hyporeflexic, producing symptoms of
When local pain is of spinal cord origin, it usually indi- infrequent urination, urinary retention, and overflow
cates the presence of a mass lesion (tumor, abscess, incontinence. Bladder symptoms are usually a late and,
hematoma) or some acute process, such as transverse frequently, an inconspicuous symptom in slowly pro-
myelitis or infarction. gressive spinal cord conditions (12).
Radicular or nerve root pain is caused by the involve-
ment of or traction on dorsal root ganglia. The pain pro-
Altitudinal Neurologic Deficit
duced by a herniated intervertebral disk is the best-known
example of this type of pain. The pain radiates into the A level below which the patient has sensory, motor, or
peripheral distribution of the nerve root involvedcervi- reflex abnormalities and above which sensory, motor, and
cal roots into the arm, thoracic roots around the chest or reflex function is normal is the hallmark of spinal cord
abdomen, and lumbar roots into the legs. disease.
Central pain, sometime called funicular pain, is com-
monly seen in patients with SCI but, except for
intramedullary spinal cord tumors, only rarely in other SPINAL CORD AND
spinal cord disorders. The onset of pain is months or years CAUDA EQUINA SYNDROMES
after injury. It is usually burning or lancinating in qual-
ity and occurs in an area of decreased or absent sensation. All spinal cord disorders, whether acute or chronic, will
It may occur in the perianal area and buttocks or diffusely present with neurologic signs and symptoms indicating
below the level of SCI (10). that the spinal cord is involved primarily dorsally, ven-
Neurogenic intermittent claudication, probably a trally, laterally, centrally, or totally. (An additional syn-
type of peripheral neuropathic pain, is seen in patients drome of pure motor involvement also exists.) The only
with lumbar spinal stenosis and spinal cord vascular other way an intraspinal abnormality can cause neuro-
abnormalities. In these patients, standing or walking logic symptoms is by involvement of the cauda equina.
causes dull back and leg pain, sometime associated with Each of these syndromes may be complete, in which there
numbness and weakness in the legs. In the neurogenic pre- is a total loss of function, or incomplete, in which case
sentation, it is distinguished from peripheral vascular function is impaired but not totally lost. The neurologic
intermittent claudication by the absence of any signs of history and examination provides the data by which these
vascular insufficiency in the legs. Standing, without walk- syndromes can be easily identified, greatly simplifying dif-
ing, causes symptoms in patients with neurogenic clau- ferential diagnosis.
dication caused by spinal stenosis, but does not occur in
claudication caused by peripheral vascular disease (11).
Dorsal Cord Syndrome
Dorsal cord syndrome results from the bilateral involve-
Bladder Symptoms
ment of the dorsal columns, the corticospinal tracts, and
Bladder dysfunction occurs in practically all acute spinal decending central autonomic tracts to bladder control
cord conditions in which the cord is affected bilaterally, centers in the sacral cord. Dorsal column symptoms
with resulting leg weakness or paralysis. The bladder par- include gait ataxia and paresthesias. Corticospinal tract
ticipates in the syndrome of spinal shock. Like the extrem- dysfunction produces weakness which, if acute, is accom-
ity muscles, the paralyzed detrusor and bladder sphinc- panied by muscle flaccidity and hyporeflexia and, if
ter muscles become flaccid and areflexic, which results chronic, by muscle hypertonia and hyperreflexia. Exten-
in an inability of the bladder to store urine; continual sor plantar responses and urinary incontinence may be
dribbling incontinence results. The detrusor and sphinc- present.
ter muscles eventually become hypertonic and hyper- The causes of a dorsal cord syndrome include acute
reflexic and automatic voiding is provoked by bladder myelopathies such as multiple sclerosis, or chronic
distention. If the acute condition involves the conus myelopathies, such as Friedreichs ataxia, subacute com-
medullaris or cauda equina, the state of detrusor and bined degeneration, vascular malformations, primary
spincter muscle flaccidity persists. progressive multiple sclerosis, epidural tumors, intradural
If the spinal cord condition is of slower onset, there extramedullary tumors, vacuolar myelopathy, cervical
is no stage of flaccidity and areflexia, but rather the slow spondylolytic myelopathy, and atlanto-axial subluxation.
410 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
Ventral Cord Syndrome The causes of a central cord syndrome include acute
myelopathies, such as neck hyperextension with cervical
Ventral cord syndrome usually includes tracts in the spondylolysis, or chronic myelopathies, such as
anterior two-thirds of the spinal cord, which include the syringomyelia or intramedullary tumor.
corticospinal tracts, the spinothalamic tracts, and
descending autonomic tracts to the sacral centers for
Total Cord Syndrome
bladder control. Corticospinal tract involvements pro-
duces weakness and reflex changes, as in the dorsal cord Total cord syndrome results from the cessation of func-
syndrome. A spinothalamic tract deficit produces the tion in all ascending and descending spinal cord path-
bilateral loss of pain and temperature sensation. Tactile, ways, which results in the loss of all types of sensation
position, and vibratory sensation are normal. Urinary and loss of movement below the level of the lesion. Loss
incontinence is usually present. of bladder and bowel control also occurs.
The causes of a ventral cord syndrome include acute The causes of a total cord syndrome include acute
myelopathies, such as spinal cord infarction or interver- myelopathies, such as spinal cord vascular malformation,
tebral disk herniation, and chronic myelopathies, such decompression sickness, epidural or intramedullary
as intervertebral disk herniation or radiation myelopathy. abscess, transverse myelitis, spinal cord hemorrhage and
simulated paraplegia.
Lateral Cord Syndrome
Pure Motor Syndrome
Lateral cord syndrome, also known as BrownSequard
syndrome, involves the dorsal column, corticospinal tract, Pure motor syndrome may involve only the upper motor
and spinothalamic tract on only one side of the spinal neuron bilaterally, in which case the patient manifests
cord. Lateral cord syndrome produces paresthesias and incoordination and weakness with hyperreflexia and
weakness on the side of the lesion and loss of pain and extensor plantar responses; or it may involve only the
temperature on the opposite side. The dorsal column is lower motor neuron bilaterally, and be manifested by
sometimes not involved, in which case the partial syn- weakness with muscle atrophy and fasciculations; or it
drome will be modified to consist of weakness and reflex may involve both the upper and lower motor neurons
changes on the side of the lesion and loss of pain and tem- bilaterally and simultaneously.
perature on the opposite side. The unilateral involvement The causes of a pure motor syndrome include
of descending autonomic fibers does not produce bladder chronic myelopathies, such as HTLV-I myelopathy, hered-
symptoms. itary spastic paraplegia, primary lateral sclerosis, cervical
The causes of a lateral cord syndrome include acute spondylolytic myelopathy, amyotrophic lateral sclerosis,
myelopathies, such as spinal cord infarction, or chronic progressive muscular atrophy, post-polio syndrome and
myelopathies, such as intramedullary tumors or intra- electric shock induced myelopathy.
dural extramedullary tumors.
Cauda Equina Syndrome
Central Cord Syndrome
Cauda equina syndrome is caused by the loss of functions
Central cord syndrome is characterized mainly by loss of two or more of the eighteen nerve roots constituting
of pain and temperature sensation in the distribution the cauda equina. Each nerve root has incoming sensory
of one or many adjacent dermatomes at the site of the fibers from one of the lower extremity or perineal der-
spinal cord lesion, caused by the dysruption of cross- matomes and outgoing motor fibers to a lower extrem-
ing spinothalamic fibers. Dermatomes above and below ity myotome. Bilateral involvement of the cauda equina
the level of the lesion have normal pain and tempera- at the L5S1 vertebral level produces low back pain that
ture sensation. Within the involved dermatomes, touch radiates down the posterior aspects of both thighs and
sensation is normal because touch fibers pass into the calves, weakness of plantar flexion of the feet, loss of
posterior columns immediately after entering the spinal ankle jerks, and bladder paralysis. Lesions of progres-
cord. As a central lesion enlarges, it may encroach on sively higher levels, such as L45, L34, L23 or L12,
the medial aspect or the corticospinal tracts or on the add progressively higher-level sensory, motor, and reflex
anterior horn gray matter, either of which will produce loss to the syndrome.
weakness in the analgesic areas. Fibers mediating the The causes of an acute cauda equina syndrome
deep tendon reflexes are interrupted as they pass from include intervertebral disk herniation. Chronic cauda
the dorsal to the ventral horn, thus causing tendon equina syndrome may be caused by intervertebral disk
reflex loss in the analgesic areas. There are usually no herniation, lumbar spondylolysis, arachnoiditis, epidural
bladder symptoms. tumor or intradural extramedullary tumor.
ACUTE NONTRAUMATIC MYELOPATHIES 411
DIAGNOSTIC PROCEDURES TO EVALUATE diagnosis of ALS. Although many other spinal cord con-
SPINAL CORD DISEASE ditions are associated with muscle atrophy caused by den-
ervation, the demonstration of EMG abnormalities does
Magnetic resonance imaging (MRI) has revolutionized the not ordinarily add information that might alter the diag-
imaging of spinal cord diseases, and improvements on the nosis or management.
classic spin-echo method of imaging provide additional Lumbar puncture is currently much less frequently
insights into the nature of spinal disease. MRI is the pro- done than even a decade ago. Although many spinal cord
cedure of choice for the evaluation of spinal cord diseases. conditions are associated with abnormalities of the cere-
It can, without moving the patient, provide images in mul- brospinal fluid (CSF), these findings, such as an increase
tiple planes and directly display images of the spinal col- in protein content, are completely nonspecific. Lumbar
umn, vertebral bone marrow, vessels, exiting nerve roots, puncture should not be done until an MRI has been per-
cerebrospinal fluid, and the spinal cord. If motion can be formed to exclude a mass lesion involving the spinal cord,
controlled, it is possible to distinguish cord gray and white because as many as 10 percent of patients with mass
matter. However, current MRI is very motion sensitive and lesions show neurologic deterioration following the pro-
thus requires extensive patient cooperation and patience. cedure. This is thought to be caused by the impaction of
Newer sequences, such as fluid attenuated inversion recov- the mass against the spinal cord, provoked by the creation
ery (FLAIR), short tau inversion recovery (STIR), and spec- of a CSF pressure differential above and below the lesion.
troscopy hold out promise to help diagnose acute cord In myelopathies thought to be caused by multiple sclero-
infarct, distinguish spinal cord compression from metasta- sis, CSF demonstrating excessive gamma globulin and
tic versus osteoporotic spine fractures, identify the cell type oligoclonal bands strongly supports the diagnosis (16).
of tumors, and distinguish radiation myelitis from tumor The findings of a CSF pleocytosis and the identification
and infection. With advances in technology, spinal mag- of a specific infectious agent by polymerase chain reac-
netic resonance angiography implies that a noninvasive tion is most helpful in patients with the syndrome of acute
screen for vascular malformation may be forthcoming. For transverse myelitis (17). Very high CSF protein levels
the evaluation of cortical bone and calcified structures, as ( 100 mg percent) are rarely seen in patients with spinal
well as in the MR-incompatible patient, computed tomog- cord disease, except in association with neoplasms.
raphy (CT), without or with intravenous or intrathecal
contrast, can often substitute.
Electrophysiologic testing is useful in some spinal RELAPSING-REMITTING
cord abnormalities. Somatosensory evoked potential will SPINAL MULTIPLE SCLEROSIS
be abnormal in all conditions involving the dorsal
columns (13). In cases that show abnormalities of posi- Relapsing-remitting spinal multiple sclerosis (16,18,19)
tion and vibratory sensation, the procedure is mostly is the most common cause of an acute spontaneous spinal
redundant unless both spinal cord disease and polyneu- cord syndrome in patients 15 to 50 years old. Multiple
ropathy are simultaneously present. In some conditions, sclerosis (MS) plaques most commonly occur in the dor-
the somatosensory evoked potentials become abnormal sal half of the spinal cord and involve the cervical and
before clinical symptoms appear, which may afford the upper thoracic cord more commonly than the lower tho-
opportunity to treat the condition earlier. One situation racic or lumbar areas. There is a tendency for plaques to
in which somatosensory evoked potentials are particu- occur bilaterally and symmetrically.
larly valuable is in the evaluation of simulated (hysteri- Bilateral plaques in the thoracic dorsal columns pro-
cal) paraplegia, in which they can be demonstrated to duce paresthesias in the legs and trunk and gait ataxia. If
be normal in a patient claiming to have no sensory per- one thoracic dorsal quadrant of the cord is involved, uni-
ception. Central motor conduction studies can be used lateral lower extremity paresthesias and weakness result.
in the same manner in patients who think themselves par- If this lesion is bilateral, the patient is paraparetic, with loss
alyzed. Nerve conduction studies will be abnormal in sev- of position and vibratory sense in the lower extremities and
eral spinal cord conditions having an associated periph- disturbed bladder function. If these lesions involve the cer-
eral neuropathy such as subacute combined degeneration vical cord, the upper extremities are similarly involved.
(14) and Friedreichs ataxia (15), although how this infor- An attack of MS typically evolves from first symp-
mation is clinically useful is not apparent. Nerve con- tom to maximal deficit over the course of 1 day to 1 week,
duction studies in patients thought to have ALS have although onset may be faster or slower. Remission from
rarely shown multifocal motor conduction block, thus maximal deficit to maximal improvement usually occurs
indicating a motor peripheral neuropathy that is poten- over 1 to 3 months, but may be slower or faster. Most
tially treatable. Needle electromyography is useful in con- commonly, patients have an attack about every 2 years.
firming muscle denervation which should be demon- Although almost all MS symptoms improve, some
strated in at least three limbs to support a clinical are more likely to do so than others. Sensory symptoms
412 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
remit completely in over three-fourths of cases. Motor of the cord bilaterally over one or several spinal seg-
symptoms are less favorable. About half of patients with mentsproduce a similar pattern of neurologic deficit
monoparesis or hemiparesis remit completely, but fewer called the anterior spinal artery syndrome, which consists
than 20 percent of patients with quadriparesis or para- of paralysis and loss of pain sensation below the level of
paresis recover to complete normality. Only 15 percent of the lesion, with sparing of position and vibratory sensa-
patients with bladder symptoms regain complete control. tion. Loss of bladder control usually occurs. Onset is sud-
When spinal cord symptoms occur in a patient den and is frequently associated with back pain over the
known to have MS, there is no diagnostic problem. How- area of infarction. Occlusion of the anterior spinal artery
ever, in 30 to 60 percent of cases, spinal cord symptoms is seldom the cause of this syndrome.
are the first manifestation of the disease (20,21). In such MRI scanning using spin-echo technique is usually
cases, it may be possible to establish the existence of other normal for the first 24 hours following an acute spinal
silent lesions in the brain stem or optic nerves using cord infarct. Magnetic resonance diffusion-weighted
brain stem auditory or visual evoked potentials. At least imaging is more sensitive to early ischemia than spin-echo
half of patients in whom spinal cord symptoms are the imaging and so may provide earlier evidence of acute neu-
first manifestation of MS show brain MRI findings typ- ronal damage similar to the abnormalities of early cere-
ical of the disease. Also, about half of such patients show bral infarcts. Spectroscopy of the spinal cord may demon-
oligoclonal banding and selective gamma globulin eleva- strate acute changes in phosphorous metabolites such as
tion on spinal fluid examination. ATP (phosphorous spectroscopy) and the accumulation
If an attack of MS is disabling, it might be useful to of lactate from anaerobic glycolysis (using hydrogen spec-
treat the patient with high-dose intravenous or oral troscopy). However, diffusion imaging and spectroscopy
steroids, which have been shown to shorten the time to are not yet in clinical use.
maximal improvement. Steroids do not, however, increase When spin-echo images become abnormal, the first
the quality of recovery or affect the frequency or inten- signal change that becomes obvious is prolongation of T2
sity of future attacks. relaxation time. The infarct becomes brighter than spinal
Subcutaneous interferon-beta-1b (Betaseron), cord on T2 weighted sequences. On strongly T2 weighted
intramuscular (Avonex) or subcutaneous (Rebif) inter- sequences, the bright CSF signal may hinder the detection
feron-beta-1a, and subcutaneous glatiramer acetate of the infarct. Often, therefore, intermediate weighted
copolymer 1 (Copaxone) have all been shown to spin-echo images (also known as proton density images)
decrease the frequency of MS attacks. These treatments or FLAIR images are preferred. On T1 weighted spin-
may also delay the onset of disability caused by MS, echo images, the infarct has a prolonged T1 relation time,
although this is not certain at this time. and it becomes darker than the spinal cord. During the
course of an acute infarct, swelling of the spinal cord can
occur. This should not be confused with the enlargement
SPINAL CORD INFARCTION of the cord caused by a tumor. As the infarct becomes sub-
acute, the signal tends to return toward normal and
Spinal cord infarction (22) may be caused by insufficient swelling subsides. Often, however, there is some remain-
blood flow in the aorta or segmental arteries or the arter- ing T2 prolongation. In addition, during this stage, con-
ies of the spinal cord itself, such as the anterior or poste- trast can cause intramedullary enhancement, which
rior spinal arteries. The causes of such flow insufficiency appears bright on T1 weighted images. Thus, enhance-
might be a dissecting aortic aneurysm, surgical procedures ment may occur in infarcts as well as spinal cord tumors
on the aorta, embolic or arthrosclerotic obstruction, or and infection. Because the signal pattern of T1 and T2
stenosis of a segmental or spinal cord artery. Infarction is prolongation can occur in many spinal cord diseases, it
frequently precipitated by severe systemic hypotension or is nonspecific and must be correlated with the history,
cardiac arrest. Most infarcts involve most of the gray mat- physical findings, and laboratory values.
ter below the affected spinal segment, or both gray and About half of patients show substantial motor
white matter of the anterior two-thirds of cord in one or recovery following spinal cord infarction. Generally, these
several segments bilaterally. Less common patterns of are younger patients who never have total paralysis dur-
infarction include both gray and white matter of the entire ing the acute phase of infarction.
cord over one or several spinal segments, or gray and white
matter of the anterior two-thirds of the cord unilaterally,
or the white matter of one posterior column. Most infarcts INTRASPINAL HEMORRHAGE
occur in the thoracic spinal cord.
The two most common patterns of infarctionall Intraspinal hemorrhage (23) may occur into the epidural
of the gray matter below the affected spinal segment, or space, the subdural space, the subarachnoid space, or into
of the grey and white matter of the anterior two-thirds the spinal cord itself (hematomyelia). The usual causes are
ACUTE NONTRAUMATIC MYELOPATHIES 413
The rapid change from a higher to a lower pressure tinence follow. These symptoms may amount to total or
environment causes inert dissolved gases in blood and tis- partial sensory and motor loss.
sue to form bubbles, which may be intra- or extravascu- Spinal fluid examination may be normal, but fre-
lar. Within the central nervous system, extravascular bub- quently shows a lymphocytic pleocytosis and increased
bles may compress or tear axons, whereas intravascular spinal fluid protein. Gamma globulin may be selectively
bubbles may obstruct arteries or veins and cause infarc- elevated.
tion. When the nervous system is affected, the spinal cord Magnetic resonance scans in patients with transverse
is involved in 75 percent of cases. myelitis and with acute disseminated encephalomyelitis
Symptoms in the form of paresthesias, weakness, show similar changes. The scans are either normal or
and incontinence occur almost immediately after surfac- show nonspecific cord swelling and edema. Thus, there is
ing from a dive. If the brain is involved, there may be con- commonly an intramedullary fusiform cord enlargement
fusion, coma, seizures, vertigo, or amurosis. (swelling) with subtle to obvious T1 prolongation and
The treatment of decompression sickness is recom- generally recognizable T2 prolongation (edema) with a
pression, as soon as possible. This requires the use of a bright segment or segments on T2 weighted imaging.
specialized chamber, which is usually available in areas Contrast enhancement is not a prominent feature. Spec-
where recreational diving is common. Ninety percent of troscopy and diffusion imaging are experimental. In cases
cases have a favorable outcome if treatment is begun of ADEM, or the first attack of MS, cranial MRI is use-
within a half-hour of surfacing, whereas only 50 percent ful to demonstrate the more characteristic distribution
avoid sequelae if treatment is delayed more than 6 hours. of lesions. Acute MS often enhances. Thus, if some lesions
Decompression sickness has a significant mortality rate can be shown to enhance and others remain enhancement
(5 to 10 percent). free, the imaging equivalent of the clinical criterion of dis-
semination in time is present. In addition, if lesions can
be shown in disparate tracts, the analogy to dissemina-
ACUTE TRANSVERSE MYELITIS tion in space is created. One particular form of myelitis
is from the idiopathic entity sarcoid. In the right clinical
Acute transverse myelitis is a clinical syndrome mani- scenario, this disease should be suspected. MRI with con-
fested by weakness and sensory loss, generally in a sym- trast shows a frequently unique pattern, with an intra-
metrical pattern, below the affected spinal segment (25). medullary lesion combined with leptomeningeal enhance-
The term transverse myelitis implies that the whole cross ment. Myelitis secondary to radiation is suspected when
sectional area of the spinal cord is involved (26). Symp- this clinical scenario is present. In addition to the signal
toms evolve over several hours to several weeks. changes of myelitis, there is often a change in the bone
Pathologically, several or many spinal cord segments marrow from red to fatty marrow (signal hyperintensity
are involved. In some cases, the process is primarily one of in the spinal marrow on T1 spin echo images) that exactly
demyelination; in others, necrosis involves all cord elements. corresponds to the radiation port.
The disorder is thought to be immunologically medi- About one-third of patients recover completely,
ated, in most cases, because of its propensity to be pre- another third do not improve at all, and one-third
ceded by some type of vaccination (rabies, smallpox, improve but have residual neurologic deficit.
tetanus, influenza, polio) or by a specific viral disease In about 5 percent of cases, the syndrome of acute
(measles, mumps, chicken pox). In other cases, the transverse myelitis represents the first attack of MS. Iden-
antecedent is unknown. The immunologic hypothesis is tification of these cases may be difficult (28). Clinically,
further supported by the ability to produce a clinically the neurologic deficit in transverse myelitis is almost
and pathologically similar disorder in experimental ani- always symmetrical on the two body sides, whereas that
mals (experimental allergic encephalomyelitis) by the in MS is seldom so. The spinal fluid findings can be the
injection of central nervous system tissue. same. MRI scanning of the head may be helpful if the typ-
Rarely, transverse myelitis may be caused by spinal ical areas of periventricular demyelination characteristic
cord infection caused by herpes simplex virus, types 1 or of MS are found, although this occurs in only about half
2, varicella-zoster virus, cytomegalovirus, or EpsteinBarr of patients ultimately shown to have MS. Visual and (or)
virus. Immunocompromised patients are usually involved. auditory evoked potentials may be abnormal, thus mak-
The diagnosis is made by spinal fluid analysis for antivi- ing the diagnosis of MS likely.
ral antibody and by polymerase chain reaction. Therapy
with acyclovir is sometimes helpful (27).
The initial symptoms are usually paresthesias involv- INTERVERTEBRAL DISK HERNIATION
ing the lower extremities. About one-third of patients
have pain over the involved segments of the spinal cord, Intervertebral disk herniation usually occurs in a dorso-
most commonly, the upper thoracic. Weakness and incon- lateral direction into the nerve root canal, because the
ACUTE NONTRAUMATIC MYELOPATHIES 415
FIGURE 30.2
scious purposes. Electrophysiologic testing will demon- 16. Paty DW, Ebers GC. Multiple Sclerosis. Philadelphia: FA Davis,
1998; 66.
strate normal function in the sensory and motor tracts 17. Fishman RA. Lumbar puncture and cerebropinal fluid examina-
of the spinal cord in patients with simulated paraplegia. tion. In: Rowland LP (ed.), Merritts Textbook of Neurology,10th
Note the pattern of sensory loss in Figure 30.4. The ed. Philadelphia: Lippincott Williams & Wilkins, 2000.
18. Noseworthy JH, Lucchinetti C, Rodriguez M, et al. Multiple scle-
upper border of the anesthetic area outlines the iliac crest rosis. N Eng J Med 2000; 343:938952.
and the inguinal ligament, rather than a dermatome. 19. Compston A, Ebers G, Lassmann H, et al. McAlpines Multiple
There is also sensory sparing of the penis and scrotum. Sclerosis. 3rd ed. London: Churchill Livingstone, 1998.
20. Shibasaki H, McDonald WI, Kuroiwa Y. Racial modifications of
clinical picture of multiple sclerosis: Comparison between British
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31 Chronic Nontraumatic
Myelopathies
AMYOTROPHIC LATERAL SCLEROSIS ness and atrophy of intrinsic hand muscles. Fasciculations
are noted in the forearms, arms, shoulders, and lower-
myotrophic lateral sclerosis (ALS) extremity muscles. Tendon reflexes are hyperactive, and
combination of loss of disc height, annular bulging, and SUBACUTE COMBINED DEGENERATION
facet hypertrophy conspire to narrow the central canal,
the lateral recesses, the neural foramina, or all of the Subacute combined degeneration (SCD) (14) is a
above. If the patient also has congenitally short pedicles, myelopathy caused by vitamin B12 (cobalamin) defi-
sagittal-oriented facet joints, or both, the situation is exac- ciency. Although young individuals are occasionally
erbated. If there is motion at the facet joints, synovial involved, almost all patients are older than 40 years and
cysts may develop that also encroach on the spinal canal most are older than 60 years.
from the posterior lateral direction. If the narrowing Pathologically, cobalamin deficiency causes degen-
occurs in an area of the spine where the cord may be com- erative changes in both central and peripheral nervous
promised, the thecal sac will be narrowed (particularly system myelin. In the spinal cord, this degeneration
visible on long TR axial views) and the cord will appear appears to begin in the dorsal columns and then spreads
to take up a larger percentage of the spinal canal (and may anteriorly and laterally to involve the dorsal half of the
superficially appear to be enlarged). Later in the course lateral columns (Figure 31.2). The upper thoracic spinal
of the disease, as the spinal cord atrophies, the thecal sac cord is most severely affected. In severe cases, axons are
assumes a more normal size. The spinal cord signal will involved as well.
not necessarily change during the course of the disease. The initial symptoms are usually sensory, with pares-
It is difficult to predict the natural history of patients thesias in the hands and feet. Gait ataxia is the next most
having symptomatic cervical or lumbar spondylosis. common symptom. Only a few patients complain of weak-
Some patients remain stable or improve without any ness. In patients with a very recent onset of symptoms, the
treatment, whereas others progressively deteriorate. In neurologic examination may be normal. However, most
patients with mild symptoms, a cervical collar may be all patients show impaired vibratory or position sense in the
that is needed. Patients with more severe symptoms and lower extremities. Most patients have an ataxic gait. Ten-
(or) neurologic deficit are frequently treated with surgi- don jerks may be increased or decreased (because of periph-
cal decompression using an anterior or posterior eral nerve involvement). Weakness may or not be present
approach. About two-thirds of patients improve after and is usually mild. Plantar responses are extensor in some
surgery. The remainder are unchanged or worse. patients. Bowel or bladder symptoms are uncommon.
The cause of cobalamin deficiency in almost all
patients is lack of the gastric intrinsic factor with which
RHEUMATOID ARTHRITIS dietary vitamin B12 must be bound to be absorbed by the
OF THE CERVICAL SPINE mucosa of the small intestine. Cobalamin is also neces-
sary for red cell production. However, one-quarter of Pathologically, demyelination of the dorsal columns and
patients with cobalamin deficiency do not have anemia. the dorsal half of the lateral columns occurs, with promi-
The diagnosis of spinal cord disorder (SCD) is con- nent vacuoles within the demyelinated tracts. These vac-
firmed by demonstrating a low serum vitamin B12 level uoles are within the myelin sheath itself. The pathologic
( 170 pg/ml by the radioassay method). To show that appearance is strikingly similar to the changes seen in the
the low level of cobalamin is having metabolic conse- subacute combined degeneration of the cord caused by
quences, high levels of homocysteine and methylmalonic Vitamin B 12 deficiency. These changes are seen in almost
acid, both of which require cobalamin for their metabo- half of all AIDS patients coming to autopsy. The mecha-
lism, should be present. The presence of serum intrinsic nism by which AIDS causes these spinal cord findings is
factor antibody confirms the diagnosis of pernicious ane- unknown.
mia. If antibody is absent and levels of homocysteine and Only about a fourth of patients demonstrating vac-
methylmalonic acid levels are increased, a Shilling test is uolar myelopathy at autopsy have symptoms during life.
done, in which the patient is given radioactive cobalamin The most common clinical presentation is a rapidly pro-
by mouth and urinary cobalamin is measured. If this is gressive spastic ataxia. Upper-extremity function is usu-
low, the patient is given a second dose of radioactive ally normal. On neurologic examination, patients show
cobalamin with intrinsic factor which, if the patient has an ataxic gait, lower-extremity weakness and hyper-
pernicious anemia, will result in an increased amount of reflexia, extensor plantar responses, and loss of position
radioactive cobalamin in the urine. and vibratory sensation. Many have urinary incontinence.
If cobalamin deficiency is diagnosed, patients are Most patients die within 6 months of developing symp-
given 500 to 1,000 micrograms of vitamin B12 daily by toms of myelopathy. AIDS-related dementia is present in
injection for 5 days and then maintained on the same dose more than half of patients. There is no effective treatment
given once each month. If the patient wishes to avoid injec- for vacuolar myelopathy.
tions, 1,000 micrograms daily by mouth can be given (15). MRI of the spine is normal. Spinal fluid examina-
Half of all treated patients with CSD recover com- tion may show nonspecific abnormalities, such as protein
pletely when treated. Patients with severe neurologic deficit elevation. SEPs are abnormal, which adds no information
prior to treatment and patients who have been symptomatic in a patient who has position and vibratory sense abnor-
for more than 6 months before treatment also improve when malities on neurologic examination. However, SEPs may
treated but are unlikely to recover completely. be abnormal in AIDS patients without clinical symptoms
of myelopathy.
FRIEDREICHS ATAXIA
VASCULAR MALFORMATIONS
Friedreichs ataxia (16) is an autosomal recessive myelopa- OF THE SPINAL CORD
thy. Most patients become symptomatic between ages 2
and 16 years and all by age 20. All patients have progres- The nomenclature of these vascular malformations has
sive gait ataxia, dysarthria, absent tendon reflexes in the lacked consensus, resulting in many different names for
legs, extensor plantar responses, and decreased position the same abnormality (18). An effort is currently under-
and (or) vibratory sense in the legs. Most also have scol- way to standardize terminology.
iosis and cardiomyopathy. Sensory nerve conduction stud- For ease of understanding, spinal cord vascular
ies and SEPs are usually abnormal. Ten to 20 percent of abnormalities are here classified as extramedullary mal-
patients develop diabetes. Patients become unable to walk formations, which are on the surface of the spinal cord
within 10 to 20 years after onset and rarely survive beyond and intramedullary malformations, which are within the
early middle age. Pathologically, there is degeneration of substance of the spinal cord itself.
dorsal root ganglion cells, the dorsal columns, the lateral Extramedullary malformations result from the
corticospinal tract, and the dorsal spinocerebellar tract. establishment of a fistula between an artery supplying and
The diagnosis is usually confirmed by demonstrating the a vein draining the cord. The direct shunting of arterial
characteristic chromosomal defect, which is especially use- blood under high pressure into veins causes them to dilate
ful in form fruste cases. There is no effective treatment and elongate. This shunting usually takes place in the
for the neurologic abnormalities. nerve root canal or involves the anterior spinal artery on
the ventral surface of the cord. These dilated serpentine
tangles of vessels lie, most commonly, on the dorsal sur-
VACUOLAR MYELOPATHY face of the lower thoracic or lumbar cord. Less often,
when the anterior spinal artery is involved, they are on
Vacuolar myelopathy (17) is a recently described (1985) the ventral or lateral surface. For the most part, they are
spinal cord disorder occurring in the later stages of AIDS. considered to be acquired lesions, because they are not
424 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
SYRINGOMYELIA
SPINAL EPIDURAL TUMORS symptoms may not appear until months or even years
later. Eventually, patients notice weakness and sensory
Spinal epidural tumors (27) are almost all malignant; symptoms below the level of the tumor. Bladder symp-
about 20,000 cases occur per year in the United States. toms occur late in the course of the disease. Despite their
Most of these represent metastatic tumors to the spine; location lateral to the spinal cord, a lateral cord
these metastases extend into the epidural space and com- (BrownSequard) syndrome only occurs in about 5 per-
press the spinal cord or cauda equina. Only about 5 per- cent of cases, although motor weakness is usually first
cent are tumors that have metastasized to the epidural noted on the side of the tumor. The treatment of meningo-
space itself. The lung and breast are the site of the pri- mas and neurofibromas is surgical removal, which is usu-
mary lesion in about 50 percent of cases. A variety of ally curative.
other carcinomas, myelomas, and lymphomas account for
the other 50 percent. Some cases occur in patients known
to have cancer but, not infrequently, the spinal metasta- INTRAMEDULLARY TUMORS
sis is the first manifestation of the malignancy.
Rarely, the spinal cord itself is the site of a metasta- Intramedullary tumors (30), arising within the spinal cord
tic tumor, usually from the lung. The clinical features are itself, are most commonly ependymomas or astrocy-
indistinguishable from a malignant epidural tumor (28). tomas, with ependymomas being about twice as common
Local pain followed by radicular pain or simulta- as astrocytomas. Ependymomas tend to occur in the
neous local pain and radicular pain is usually the first lower half of the spinal cord (and cauda equina), whereas
symptom of an epidural tumor. After an interval, (e.g., 4 astrocytomas are mostly in the upper half of the spinal
months for lung cancer), signs of myelopathy in the form cord. Ependymomas are usually well demarcated and his-
of weakness and sensory loss below the level of the lesion tologically benign. Astrocytomas, conversely, are more
appear. Bladder symptoms occur in over half of cases. infiltrative, and about 25 percent have the histologic char-
Untreated, these symptoms progress to an anesthetic acteristics of malignancy.
paraplegia or quadriplegia with total loss of bowel and Ependymomas and benign astrocytomas are slowly
bladder function. growing lesions; patients are frequently symptomatic
Metastatic epidural and intramedullary tumors are months or years before the tumor is demonstrated and
treated with high-dose corticosteroid administration and treated. Local neck or back pain is usually the first symp-
radiation therapy, which should begin as soon as the diag- tom, followed by weakness and sensory symptoms below
nosis is made. Patients seldom improve, but neurologic the level of the lesion. A lateral or central cord syndrome
deterioration is halted or slowed. Most patients die within is present in some patients. Because about half of all
6 months after the diagnosis is made. ependymomas arise from the filum terminale, a cauda
equina syndrome frequently occurs. Bladder symptoms
are a late occurrence.
INTRADURAL EXTRAMEDULLARY TUMORS The optimal treatment of intramedullary spinal cord
gliomas is curative surgical removal. This is frequently
Intradural extramedullary tumors (29), or tumors in the possible with ependymomas and benign astrocytomas.
subarachnoid space, unlike epidural tumors, are histo- The 10-year postoperative survival with these tumors is
logically benign, very slow growing, and usually com- about 80 percent. In malignant astrocytomas, however,
pletely curable by surgical removal. Meningiomas, which postoperative 10-year survival is only about 15 percent.
arise from arachnoidal cells, and neurofibromas (Schwan- MRI of spinal tumors includes standard spin-echo
nomas), which arise from Schwann cells of the spinal images, often supplemented by extra sequences such as
nerve roots, are about equally common, each constitut- STIR or diffusion weighted techniques, to evaluate the
ing about 25 percent of primary spinal tumors. Both of spinal bone marrow, or T1 weighted, contrast-enhanced
these tumor types arise lateral to the spinal cord: neu- runs to evaluate intraspinal disease. As a general concept,
rofibromas from the dorsal nerve root and meningomas all tumors are initially imaged in at least two planes to
from arachnoidal cells near the dentate ligament. Both evaluate location and extent. Scans performed for follow-
tumor types may involve any portion of the spinal cord up or to assess spinal block may be single-plane views to
although meningomas most commonly occur in the cer- answer the simple or emergent question.
vical and thoracic areas. Both tumors usually become Using standard MRI, tumors can be divided using
symptomatic between 30 and 60 years of age. Meningo- the same classic divisions of myelography, extradural,
mas are more common in women. intraduralextramedullary, and intramedullary. Evalua-
Both meningiomas and neurofibromas present with tion using spectroscopy is still experimental.
local neck or back pain and frequently with radicular pain In general, tumors will have T1 and T2 prolonga-
as well. Because the tumors are slow growing, spinal cord tion and will usually enhance. Tumors originating in the
CHRONIC NON TRAUMATIC MYELOPATHIES 427
16. Harding AE. The Hereditary Ataxias and Related Disorders. Edin- GW (eds.), Handbook of Clinical Neurology, Vol 32. Amsterdam:
burgh: Churchill Livingstone, 1984; 57103. North Holland Publishing, 1978; 255327.
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Churchill Livingstone, 1995; 8084. ditions. In: Joynt RJ, Griggs RC (eds.), Clinical Neurology, revised
18. Detwiler PW, Porter RW, Spetzler RF. Spinal arteriovenous mal- edition, Vol 3, Chapter 45. Philadelphia: Lippincott Williams &
formations. Neurosurg Clin N Am 1999; 10:89100. Wilkins, 1998; 23.
19. Watson JC, Oldfield EH. The surgical management of spinal dural 27. Byrne TN, Benzel EC, Waxman SG. Diseases of the Spine and
vascular malformations. Neurosurg Clin N Am 1999; 10:7387. Spinal Cord. Oxford: Oxford University Press, 2000; 166205.
20. Andersson PB, Waubant E, Gee L, et al. Multiple sclerosis that is 28. Schiff D, ONeill BP. Intramedullary spinal cord metastases: Clini-
progressive time of onset. Arch Neurol 1999; 56:11381142. cal features and treatment outcome. Neurology 1996; 47:906912.
21. Paty DW, Ebers GC. Multiple Sclerosis. Philadelphia: F.A. Davis 29. Schutta HS. Spinal tumors. In: Joynt RJ, Griggs RC (eds.), Clini-
Co., 1998; 66. cal Neurology, revised edition, Vol 3, Chapter 44. Philadelphia:
22. Nijeholt GJL, Barkhof F, Scheltens P, et al. MR of the spinal cord Lippincott Williams and Wilkins, 1998; 2636.
in multiple sclerosis: Relation to clinical subtype and disability. Am 30. Byrne TN, Benzel EC, Waxman SG. Diseases of the Spine and
J Neuroradiol 1997; 18:10411048. Spinal Cord. Oxford: Oxford University Press, 2000; 227232.
23. Shaw MDM, Russell JA, Grossart KW. The changing pattern of 31. Goldwein JW. Radiation myelopathy: A review. Med Ped Oncol
spinal arachnoiditis. J Neurol Neurosurg Psychiatry 1978; 1987; 15:8995.
41:97107. 32. Macbeth FR. Radiation myelopathy: Estimates of risk in 1,048
24. Dolan RA. Spinal adhesive arachnoiditis. Surg Neurol 1993; patients in three randomized trials of palliative radiotherapy for
39:479484. non-small cell lung cancer. The Medical Research Council Lung
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32 Multiple Sclerosis
429
430 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
nition for stress. For example, the loss of employment nancy is contemplated. Most MS specialists discontinue
may be very stressful to one person but a relief to some- these treatments if a patient becomes pregnant. However,
one else. The perception of stress is likely to be more some pregnant MS patients on interferon beta or glatiramer
important than the event itself. Chronic, daily, long-term acetate have carried their babies to term without teratogenic
stress may be more important than intermittent acute effects. However, interferon beta is an abortifacient. The
stressful events. This issue requires more research. chemotherapeutic agents used to treat MSincluding
mitoxantrone, methotrexate, azathioprine, and cyclophos-
phamideare teratogenic and reduce fertility.
Womens Issues
Women have a higher risk for MS (16) as well as other
immune-mediated diseases, such as Hashimotos thy- DIAGNOSIS OF MULTIPLE SCLEROSIS
roiditis, myasthenia gravis, rheumatoid arthritis, Sjgrens
syndrome, systemic sclerosis, and systemic lupus erythe- Whereas the diagnosis of MS is usually easily determined,
matosus (17). Sex hormones can have a profound effect missed diagnoses are still common in patients presenting
on the immune system. Sex hormone receptors are found with unusual symptoms. With the new drugs to treat MS,
on lymphocytes and macrophages. Some sex hormones these misdiagnoses have potentially damaging conse-
downregulate and others upregulate the immune system. quences. Before the FDA approved Betaseron in 1993,
In an early study (1969) on rodents, birth control pills there was no drug approved by the FDA for the long-term
available at the time were shown to have a protective treatment of MS. Therefore, neurologists would some-
effect on experimental allergic encephalomyelitis (EAE) times withhold the diagnosis of MS from the patient to
(18), an animal model of MS. Recently, estriol, a natu- reduce anxiety. The unusual but potential risk of suicide
rally occurring estrogen, has shown some beneficial and insurance issues also prompted some physicians to
affects in MS in a small pilot study. withhold the diagnosis of MS. As a hedge, many neurol-
The effect of pregnancy on lessening MS exacerba- ogists used the term demyelinating disease.
tions has been discussed. In one study, the risk for an MS is a very complex and heterogeneous disease. No
attack in the last trimester of pregnancy was reduced by two patients are alike. Because 85 percent of MS starts
70 percent. However, there was a 70-percent increase in as a relapsing remitting course, acquiring a history of
exacerbation rates during the first 3 months postpartum relapses and remissions is very important. An MS attack
(12). The postpartum risk may be decreased with current is defined as a new neurologic symptom, or the recrude-
immunomodulating therapy, but no studies have been scence of an old symptom, that lasts more than 24 hours
published. and is spaced 1 month apart.
Breast-feeding does not appear to increase the risk MS patients have multiple attacks of demyelination,
of postpartum exacerbations (19). The disease does not in different parts of the nervous system, occurring over
seem to have an effect on reproductive functions. One time. A progressive neurologic disease may indicate the
study (Reference) demonstrated that there was no differ- diagnosis of MS, especially in an older age group.
ence in long-term disability among women who had no The neurologist makes the diagnosis of MS by per-
children, one child, or two or more children (19). forming a neurologic history and physical examination
Some studies have indicated that symptoms of MS followed by an MRI. Sometimes cerebral spinal fluid
and MRI activity may be related to hormonal effects dur- (CSF) and evoked potential tests can be very helpful.
ing the menstrual period (20,21). However, most neurologists who find the typical history,
Although MS may not affect reproductive functions, the typical examination, and the typical MRI for MS do
it may have a significant effect on sexual function. Reduced not proceed to other diagnostic tests.
lubrication, vaginal numbness, decreased orgasms, and Although MRI has revolutionized our ability to
genital pain associated with or without intercourse can be diagnose MS early in its course, it is not the perfect diag-
seen in women having MS. Many of these symptoms can nostic answer. Sometimes, nonspecific white matter
be treated if specific inquiries are forthcoming between the lesions or white matter lesions with an onset over the age
physician and the patient. Lubricants, vibrators, and anti- of 40 years are interpreted as compatible with MS.
spasticity agents have all been shown to help specific symp- Therefore, these patients may be treated inappropriately.
toms. Sildenafil citrate (Viagra) has been shown to pro- However, the most prominent issue is failing to diagnose
foundly improve male sexual function, but has not been early MS, when it can be more easily treated. The MRI
adequately studied in females. test includes T2 and T1 with and without gadolinium
Oral contraceptives and hormone replacement ther- images. The FLAIR MRI increases the sensitivity for MS
apy are not contraindicated in MS. The FDA approved lesions. Gadolinium enhancement images with the MRI
immunomodulating therapiesinterferon beta, glatiramer indicate acute activity of the disease, whereas T2 images
acetate, and mitoxantroneare not recommended if preg- reflect more the total burden of the disease in the brain.
432 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
erative component of the disease. PPMS and PRMS are understood. It has a powerful anti-inflammatory effect.
both mainly degenerative forms of the disease. This dis- It antagonizes many of the negative effects of interferon
tinction may become important, because the current gamma (IFN), which upregulates the immune system.
immunomodulating therapies work on inflammation and It reduces bloodbrain barrier permeability and thereby
possibly not on the degenerative component. These treat- inhibits the activated T-cells from getting into the brain.
ments may have less usefulness in late SPMS and PPMS. In a European, randomized, placebo-controlled,
A new consideration is the recently named clinically double-blinded study, Interferon 1-b has also been
isolated syndrome (CIS). This is the initial, clinical neu- shown to decrease the progression of SPMS as well as
rologic event where the risks of relapse and a diagnosis reduce relapses and MRI lesions (25). It has been
of RRMS are high if the MRI shows active disease at the approved for treatment of SPMS in Europe and Canada.
time of the CIS. Interferon 1-a (Avonex and Rebif) A North American trial of interferon 1-b in SPMS
has been effective in patients with CIS by delaying the showed the same positive effects on exacerbation rates
next attack and, therefore, the diagnosis of MS (23). and MRIs but did not show an effect on disability (26).
Its FDA application for use in SPMS is pending.
The side effects of Interferon 1-b vary among
IMMUNE MODULATION THERAPY between patients. A flu-like syndrome, especially early
in the course of treatment, is the most common side effect
Before the release of Betaseron in 1993, there was no (24). This can be minimized by initiating therapy with
widely accepted or FDA approved treatment to alter the dose escalation and the liberal use of ibuprofen or aceta-
long-term disease course. Corticosteroids, especially minophen before, during, and after the injections. Injec-
intravenously at very high doses (1,000 mg per day) for tion site redness is another side effect of the every-other-
3 to 10 days, were utilized for patients with moderate to day subcutaneous injection. Several strategies are used
severe exacerbations of MS. No evidence showed that this to minimize this side effect, including allowing the mate-
treatment altered the long-term prognosis of the patient. rial to sit at room temperature for 20 minutes, using top-
Chemotherapeutic agents, such as cyclophosphomide ical corticosteroids on the site of injection, rubbing an
(Cytoxan) and azothiaprine (Imuran) were used with ice cube on the site of injection, massaging the injection
mixed results and potential toxicities (see Table 32.4). site, and using an autoinjector. The autoinjector is avail-
able for all subcutaneous forms of treatment. This elim-
inates the need for a patient-initiated needle stick for
Interferon 1-b (Betaseron)
patients with an aversion to sticking themselves. It also
Betaseron was shown to reduce the exacerbation rate by increases the target areas for self-injection to include the
one-third and reduce MRI lesions by 80 percent or more buttocks, which is a hard-to-reach location for needle
(24). In fact, the burden of disease on T2 weighted MRIs injection. Necrotic skin lesions occur in about 4 percent
actually improved in the 2-year Betaseron trial. The dis- of patients on interferon 1-b. These lesions may warrant
ability data showed a trend toward effectiveness of the discontinuation of the therapy.
Betaseron, but this was not statistically significant. Neutralizing antibody is seen in all three interferon
Interferon 1-bs mechanism of action is not clearly beta products. Their significance is debated, because most
TABLE 32.4
Immunomodulation Therapies
Interferon -1b (Betaseron) 28 MIU/week Subcutaneous, every RRMS (World) and SPMS
other day (Europe and Canada)
Interferon -1a (Avonex) 6 MIU/week Intramuscular, once Relapsing Form (World)
a week
Interferon -1a (Rebif) 36 MIU or Subcutaneous, three RRMS (Europe and Canada)
18 MIU/week times a week
Glatiramer acetate (Copaxone) 20 mg/day Subcutaneous, daily RRMS (World)
Mitoxantrone (Novantrone) 12mg/m2 Intravenous, every Progressive MS
three months for 23 years
434 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
of antibody-positive patients turn antibody negative from a few seconds to 30 minutes (29). The cause of this
within a few months. Neutralizing antibody is more likely systemic reaction is unknown. It occurred in 15 percent
seen in lower doses of interferon 1-b (Betaseron) and of patients in the pivotal trial of glatiramer acetate. Glati-
interferon 1-a (Rebif) than in patients on higher doses. ramer acetate is associated with antibody production in all
More research must be conducted to clarify this confus- patients. However, this is not a neutralizing antibody and
ing situation. is not thought to affect efficacy in MS.
Is there evidence of effectiveness beyond the 2 years
of the pivotal trials? Longer-term observations have been
Interferon 1-a (Avonex and Rebif)
fraught with problems, because patients are no longer
Interferon 1-a is available in two preparations, but these blinded, may switch medications, and many are lost to
are essentially the same drug. Avonex, given intramus- follow-up. However, data collected after the 2-year piv-
cularly once a week at 30 ugm, is approved in the United otal trials have shown effectiveness for interferon 1-a
States for relapsing forms of MS. Their pivotal trial (Rebif), interferon 1-b (Betaseron), and glatiramer
showed an effect on exacerbation rate, MRI, and dis- acetate (Copaxone). Some interferon 1-btreated
ability (27). Rebif is effective in treating RRMS and has patients and placebo-treated patients were followed for 5
been approved for use in Canada and Europe. The United years. A statistical difference in the burden of disease on
States Orphan Drug Act prohibits it from being released MRI and a greater than 30 percent reduction of exacer-
until 2003. It is given subcutaneously 3 times a week in bations remained (30). The exacerbation rate reduction
either 18 million international units per week or 36 mil- did not reach statistical significance because of the lim-
lion international units per week (28). ited number of patients. An interferon 1-a (Rebif) trial
The side effects of Avonex and Rebif are mainly has presented 4-year data that shows the sustained effi-
a flu-like syndrome, which is usually transient (27,28). cacy of Rebif. Long-term studies following the pivotal
All of the interferon beta studies have shown that some trial of Avonex were not attempted. Glatiramer acetate
patients acquire neutralizing antibody. Avonex has the patients in the pivotal trial have continued to be followed
lowest incidence of neutralizing antibody. Neutralizing for 7 years (31). Those who stayed on the medication
antibody between the interferons does cross-react have had essentially no increase in disability, and their
between interferon 1-b and interferon 1-a, at least to exacerbation rate remains lower than predicted from nat-
some extent. Again, the significance of neutralizing anti- ural history studies of MS.
body is debated. The clinical rule of thumb is to switch a
patient from the interferon betas or increase the dose of
Mitoxantrone (Novantrone)
interferon betas if the patient is worsening clinically, with
more than expected exacerbations or progression. MRI Mitoxantrone is a chemotherapeutic agent used to treat
lesion progression studies can also be helpful. neoplastic disease. Clinical trials of mitoxantrone have
been relatively small, but have shown a significant bene-
fit, thus it was approved by the FDA for the treatment of
Glatiramer Acetate (Copaxone)
progressing MS. The effectiveness was shown on reduc-
Glatiramer acetate is a random selection of four amino tion of relapse rate, disability, and MRI lesions (32,33).
acids that make up peptides. Its first efficacy was shown Because of potential cardiac toxicity, it is only recom-
in experimental allergic encephalomyelitis (EAE) in mended for use for 2 to 3 years before it must be discon-
rodents. Early trials showed promising results in relaps- tinued. It is given intravenously every 3 months at a dose
ing remitting MS but not in chronic progressive MS of 12 mg/m2. Regular cardiac evaluation is recommended.
(SPMS and PPMS were not separated in this trial). The Another potential issue with any chemotherapy, includ-
mechanism of action of glatiramer acetate is not fully ing mitoxantrone, is the incidence of secondary neoplasias
understood. It likely decreases the activation of T-lym- later in life. Mitoxantrone-treated cancer patients do have
phocytes peripherally, before they cross the bloodbrain an increased risk of leukemia later in life. The actual risk
barrier. Although it does not have the powerful anti- of secondary leukemia for mitoxantrone in MS patients
inflammatory effectiveness of the interferon betas, it has is unknown. Some MS studies have shown no occurrence
shown efficacy in RRMS in reducing exacerbations and of secondary leukemia, whereas some of the cancer
MRI lesions (29). It is given subcutaneously every day at patients had as great as a 2.9 percent occurrence of sec-
a dose of 20 mg. ondary leukemia (32).
The major side effect of Copaxone is transient injec- Mitoxantrones place in the treatment of MS has not
tion site pain and minimal skin redness. Most patients tol- been firmly established. Its usefulness is limited to 2 to 3
erate these side effects well. A more frightening, but harm- years because of cardiotoxicity. Therefore, some neurol-
less, side effect is a systemic reaction of chest tightness or ogists would not use this drug unless the patient had
pain, flushing, anxiety, and shortness of breath lasting failed interferon 1-b, interferon 1-a, and glatiramer
MULTIPLE SCLEROSIS 435
acetatein effect, as a stopgap measure until some- different, although that difference has been narrowing in
thing better comes along. Some neurologists use mitox- the last several years.
antrone in progressive forms of MS without trying inter- Most physicians treating MS patients are troubled
feron 1-b, interferon 1-a, or glatiramer acetate. Studies by a fragmented healthcare system for people with
are being conducted to evaluate the usefulness of mitox- chronic disabilities. This fragmentation, miscommunica-
antrone as an induction agent in combination with the tion, and lack of communication is problematic for the
other medications. The theory is that mitoxantrone may MS patients care. The ideal long-term management of
effectively shut down the disease early in the MS course. MS patients resides in the rehabilitation team approach
Then, other immune modulating drugs could provide to patients. The management of MS patients not only
protection in the long term. No data is available to sup- involves diagnosis and medical management, it involves
port this theory at present. preventive maintenance and addresses family issues,
employment issues, and vocational issues. Education and
care coordination are key to successful long-term disease
EXPERIMENTAL THERAPY management. Patient self-responsibility and empower-
ment are new concepts for many neurologists treating MS
Several clinical trials are in progress on a number of other patients. The systematic use of patient advocacy groups
treatments. None of these has been proven effective. Exam- in the community, such as the National Multiple Sclero-
ples of these experimental treatments include stem cell sis Society (NMSS) and the Multiple Sclerosis Association
transplantation or bone marrow transplantation, intra- of America (MSAA), can make a huge difference in the
venous immunoglobulin (IVIG), T-cell vaccination, mon- quality of life of the MS patient.
oclonal antibodies, interferon alpha, cytokine regulation, The specific components of disease management
metalloproteinases, altered peptide ligands, insulin-like include prevention, diagnosis, acute medical treatment,
growth factor-1, and nerve growth factor. Recently, long-term medical treatment, rehabilitation, psychoso-
chlamydia and human herpes virus VI have become sus- cial adaptation, community integration, and end-of-life
pect agents for the cause of MS. Treatment trials for both issues. The best practice approach to the MS patient
of these agents are in progress. A potentially promising starts with a better doctorpatient communication sys-
area of investigation for future treatments includes neu- tem and the integration of healthcare providers and ser-
roprotective agents for the degenerative component of MS. vices. As team care concepts become part of the neurol-
ogists management of MS patients, the patients quality
of life will be benefited greatly.
REHABILITATION AND SYMPTOM Now that research results have provided positive
MANAGEMENT outcomes, many barriers to MS rehabilitation to mini-
mize the functional loss will be available. The concept of
Twenty-five years ago, rehabilitation of MS was thought maximum rehabilitation potential is nonoperative in
to be a waste of resources because MS was a progressive MS care, because the issues for the MS patient continu-
disease, and patients will get worse anyway. In the mid ally change and are life long.
to late 1970s, multiple sclerosis comprehensive centers Some clinicians believe that rehabilitation should
were developed and rehabilitation was integrated into the start only when the patient has significant disability. In
treatment. However, there was a paucity of publications fact, rehabilitation cannot start too early after the diag-
measuring the outcomes of rehabilitation in MS. In recent nosis. The issues of adaptation, wellness, exercise, nutri-
years, numerous studies have shown the effectiveness of tion, energy conservation, psychosocial equilibrium,
MS rehabilitation in the inpatient and the outpatient set- stress management, and education are part of the post-
ting (3449). Comprehensive short-term and long-term diagnosis treatment plan. Family involvement cannot be
therapy has significant benefit in increasing function, overemphasized: for example, divorce rates decrease in
despite progressive disease. comprehensive care centers.
Most MS symptoms are treated with rehabilitation Some doctors believe that MS patients with severe
techniques as well as medication (5052). Much of the disabilities cannot benefit from rehabilitation. However,
disability in MS is related to spinal cord involvement; in these patients rehabilitation plays a role to maximize
therefore, the rehabilitation issues in MS are similar to function and to create safe environments, as well as to
those in SCI. However, there are many key differences in prevent secondary disabilities and medical complications.
the treatment of the symptoms of MS versus SCI. The maintenance rehabilitation concept for MS
Rehabilitation medicine physicians are often the patients was formalized by Dr. Randall Schapiro in St.
principal care providers for SCI, whereas neurologists are Paul, Minnesota. His active ongoing rehabilitation inter-
more often the principal care providers for MS. Their ventions successfully increase function and decrease com-
backgrounds and orientation to disease can be markedly plications over a long period of time.
436 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
chotherapy, dealing with adaptation and coping, early in catheterization is the best treatment for patients who have
the disease is important. Family counseling is also an inte- urinary retention. A dyssynergic bladder, where both the
gral part of better understanding the disease. When bladder wall and the urinary sphincter contract at the
depression becomes manifest, psychotherapy and med- same time to cause urinary retention and reflux of urine
ications are used concurrently. Tricyclic antidepressants toward the kidneys, can be treated with intermittent
(TCAs) were the mainstay of MS depression treatment for catheterization and alpha-blockers such as terazosin
many years. However, their anticholenergic side effects (Hytrin) or phenoxybenzamine (Dibenzyline). Baclofen
made many MS symptoms worse. Blurred vision, dry (Lioresal) may also help the dyssynergic bladder.
mouth, constipation, and urinary retention are some of
the problems with tricyclic antidepressants. Therefore, the
Bowel Dysfunction
SSRIs or trazodone, nefazodone (Serzone), and bupro-
pion (Wellbutrin) are becoming the mainstays in the Bowel dysfunction usually means constipation, but diar-
medical management of depression. Other newer antide- rhea and incontinence also occur. Medications for other
pressants will also likely be helpful in MS. The drug treat- MS symptoms and dehydration can play a significant role
ment of depression need not be lifelong, especially if there in aggravating constipation. The first step in treating
is adequate psychotherapy. bowel dysfunction is a bowel-training program. Often,
patients are trained to establish a regular pattern of bowel
elimination. The gastrocolic reflex, especially after break-
Cognitive Dysfunction
fast, can be utilized if the patient prepares for a bowel
Cognition dysfunction was not thought to be a major part movement at that time. A warm beverage may also help
of MS symptomatology in the past. Now we realize that stimulate a bowel movement. A high-fiber diet and hydra-
cognitive dysfunction may occur early and may be unrec- tion are the next steps to treatment of most bowel dys-
ognized for a long period of time. Therefore, cognitive test- function. Stool softeners may also be helpful. Oral stim-
ing might be helpful early in the disease course. Fatigue ulants (laxatives) for bowel movements can lose their
and medications for other MS symptoms may contribute effectiveness over time because of habituation. A glycerin
to cognitive dysfunction. The medical treatment for cog- suppository, which contains no medication, is preferred
nitive dysfunction has not been extensive. The results of initially to the stimulant suppositories such as Ducolax.
small studies have been mixed. However, cognitive retrain- Enemas are effective, but should be saved for fail-
ing helps patients adapt to the disabilities and increases the ures with the preceding treatments. Digital stimulation
quality-of-life issues in patients with cognitive problems. can also be effective if other treatments fail.
Immunomodulating therapy has also been shown to help
cognitive problems in some clinical trials.
Sexual Dysfunction
Physicians and patients seem to be joined in a conspir-
Bladder Dysfunction
acy not to discuss or treat most sexual dysfunction. The
Bladder problems are among the most common MS military model of dont ask, dont tell seems to prevail
symptoms, causing social isolation and a reduced qual- as part of this conspiracy. However, sexual dysfunction
ity of life. The evaluation of bladder dysfunction starts is common in both men and women. Sexual dysfunction
with a detailed history and post-void residuals measure- in women may be more prevalent than in men. However,
ment or bladder ultrasound. More formal bladder dys- erectile dysfunction seems to be the center of attention
function testing using urodynamics is performed by a of most neurologists. Both men and women may have
urologist. The treatment for bladder dysfunction starts altered libidos, altered genital sensation, decreased fre-
with education and counseling. Fluid intake is extremely quency and intensity of orgasms, or pain during sexual
important, although drinking more fluid may lead to leak- intercourse. Vaginal dryness is a common complaint in
ing or frank incontinence. However, fluid deprivation can women. Sometimes, sexual intercourse may stimulate
lead to dehydration, increased retention, bladder infec- massive muscle spasms or pain.
tion, stone formation, constipation, and other side effects The treatment of sexual dysfunction may be as sim-
such as dry mouth, dizziness, and nausea. Medication for ple as vaginal lubrication or vibrators. However, the man-
a small spastic bladder usually begins with tolterodine tar- agement of sexual dysfunction begins with education and
trate (Detrol), because it has the least anticholinergic side counseling about the specific pathophysiology of sexual
effects. Oxybutynin (Ditropan XL) can be given once a dysfunction. Sexual activities can occur in many forms
day with fewer anticholinergic side effects than regular that do not require sexual intercourse. Intimacy is a spe-
oxybutynin. No medication is very effective for the large, cial connection that is not necessarily tied to intercourse.
hypotonic bladder. Bethanechol (Urecholine) usually The medical treatments for fatigue, depression, blad-
fails to significantly benefit the patient. Intermittent der dysfunction, bowel dysfunction, and muscle spasms
438 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
are important issues in the treatment of sexual dysfunc- SCI patients. MS patients tend to come from higher
tion. Pharmacologically, sildenafil citrate (Viagra) has socioeconomic backgrounds and are more likely to be col-
been very effective for penile erection problems. One lege educated. Childbearing issues and genetics are more
problem associated with Viagra is that patients may important in MS. A major goal in MS is to find treatments
focus on the act of sexual intercourse as the ultimate in to prevent damage over time, whereas SCI prevention
sexuality. This is unlikely to be the case: communication management is confined to the immediate post-injury
and intimacy likely play a greater role in quality of life phase. Because of the location of many MS lesions above
in the long run. the spinal cord level, more problems with cognition,
vision and eye movement, speech and swallowing distur-
bances, paroxysmal symptoms, cerebellar tremors and
Dysphagia
incoordination are likely.
Dysphagia occurs in 20 to 25 percent of MS patients. Reimbursement for rehabilitation in SCI is easier to
Patients may have a delay in swallowing, where food achieve than long-term rehabilitation in MS. Although it
enters the pharynx before the pharyngeal motor response is obvious that SCI patients need rehabilitation, the ben-
has been initiated. This leaves the airway open, which efit of rehabilitation in MS is less accepted, in spite of
leads to aspiration and possibly pneumonia. The motil- numerous recent articles demonstrating efficacy.
ity of food through the pharynx may also lead to ineffi-
cient swallowing and aspiration. Reduced tongue move-
ment, incoordination, and poor lip closure or facial tone CONCLUSION
may also decrease swallowing. Esophageal peristalsis dys-
function is theoretically possible in MS, although very few This chapter previews some of the complexities in the
studies have been completed. diagnosis, disease course, and treatment of MS symp-
The treatment of dysphagia is difficult, but a speech toms. The integrated, comprehensive treatment approach
and language pathologist can provide treatments to among rehabilitation therapists, the physicians, and other
improve dysphagia. Surgical procedures to prevent aspi- members of the healthcare team is the key to the suc-
ration have not been very successful in MS patients. Dys- cessful long-term management of MS patients (5557).
phagia is also related to fatigue: for example, some MS The disease management approach in MS provides
patients take a long time to finish a meal. Chewing education, self-management, and empowerment oppor-
becomes difficult and, by the end of the meal, fatigue may tunities for the patient. This not only helps the patient but
produce coughing, regurgitation of food, and aspiration. also helps the caregiver and healthcare professionals
Therefore, it is recommended that patients having prob- involved in treatment. A well-designed system of care
lems with dysphagia have more frequent but smaller accomplishes the ultimate goal of a higher quality of life
meals to prevent fatigue. for MS patients.
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440 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
ain in persons with spinal cord traumatic SCI: 76 percent had pain on admission to a
in areas innervated by segments three levels or more sified as SCI complex regional pain syndrome (CRPS)
above the neurological level of injury, and which are not pain: (1) it is not limited to the territory of a single
specifically known to be more common after SCI. peripheral nerve or root; (2) it is disproportionate in
Type 6. SCI at level mechanical/musculoskeletal pain is intensity to what is expected; and (3) it is associated
nociceptive pain related to bony, ligamentous, or mus- with edema, skin blood flow abnormality, or abnormal
cular injury, and which is localized to areas within two sudomotor activity.
segments either above or below the neurological level of Type 12. SCI below level mechanical/musculoskeletal
injury. pain occurs caudal to the two dermatomal levels below
Type 7. SCI at level nociceptive visceral pain occurs the neurological level only in persons with a neurologi-
primarily in those with a neurological level of injury cally incomplete spinal cord injury or a complete injury
below T7. It originates from damage, irritation, or disten- with a zone of partial preservation (ZPP) extending to
tion of internal organs or their supporting ligamentous the level of the pain. Depending on the degree of
structures. retained sensation, the presentation of this nociceptive
pain may vary. Severe spasticity often presents as SCI
Type 8. SCI at level central pain is neuropathic pain
below level muscular pain.
which occurs within two dermatomal levels either above
or below the neurological level and is not related to Type 13. SCI below level nociceptive visceral pain origi-
nerve root damage but to actual segmental spinal cord nates from damage, irritation, or distention of internal
damage. The character is typically one of tightness, organs or their supporting ligamentous structures in
pressure, or burning in thoracic level injuries and of those with a neurological level above T7. In persons
numbness, tingling, heat, or cold in cervical level with a complete SCI, this pain is characteristically vague
injuries. The distribution is typically bilateral, involving and poorly localized. It may be associated with symp-
single or multiple adjacent dermatomes over the shoul- toms of autonomic dysreflexia, anorexia, altered bowel
ders, arms, or hands for cervical injuries; single or multi- patterns, nausea, or vomiting, any of which may be
ple dermatomal bands about the chest for thoracic more prominent than the pain itself.
injuries; and single or multiple adjacent dermatomes to Type 14. SCI below level central pain is neuropathic
the groin or legs for lumbar injuries. Furthermore, rather pain which occurs caudal to the two dermatomal levels
than being paroxysmal, SCI at level central pain is typi- below the neurological level. Its distribution is generally
cally continuous in its presence. not dermatomal but regional, enveloping large areas
Type 9. SCI at level radicular pain is neuropathic pain such as the anal region, the bladder, the genitals, the
arising from nerve root damage occurring within two legs, or commonly the entire body below the neurologic
dermatomal levels either above or below the neurologi- level. The character is often described as burning or
cal level of injury. The character of the pain is often aching, although other descriptors have included pres-
described as stabbing, shooting, or electric shock sure, heaviness, cold, numbness, and pins and nee-
like, although it has also been described as burning or dles. It is usually continuous in presence, although the
aching. It is generally unilateral and paroxysmal in its intensity of the pain can fluctuate in response to a num-
presence, radiating in a dermatomal pattern. The pres- ber of factors including psychological stress, anxiety,
ence of allodynia or hyperesthesia is common, although fatigue, smoking, noxious stimuli below the level of
hypoalgesia or analgesia can occur. When radicular injury, and weather changes.
pain is associated with spinal instability, it may be exac- Type 15. SCI below level neuropathicother pain
erbated by spinal movement. occurs only in persons with a neurologically incomplete
Type 10. SCI at level compressive neuropathy pain SCI or with a zone of partial preservation (ZPP) extend-
occurs in a specific peripheral nerve distribution distal ing to the level of the pain. It includes all the neuro-
to the root level in areas innervated within two der- pathic types of pain which are not specifically known to
matomal levels either above or below the neurological be more common after SCI, and which occur in areas
level of injury and is attributed to compression of a spe- innervated by segments more than two levels below the
cific peripheral nerve. Symptoms most often include neurological level of injury.
either spontaneous or evoked numbness or tingling in a
specific peripheral nerve distribution. The highest neurologic level should be used if the neu-
Type 11. Spontaneous or evoked pain, allodynia, or rologic level is not the same on the right and left sides of
hyperalgesia, that is localized within two dermatomal the body.
levels either above or below the neurological level of
injury and fulfills the following three criteria can be clas-
444 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
sons with a neurologicly incomplete SCI or with a zone plegia, both with and without a rotator cuff impingement
of partial preservation (ZPP) extending to the level of the syndrome, were compared with regard to shoulder
painand visceral); and one SCI below-level neuropathic strength, the athletes with rotator cuff impingement exhib-
subtype, (i.e., central). In addition, two other above-level ited decreased shoulder adduction and external and inter-
subtypes of pain exist: one nociceptive, the other neuro- nal rotation strength and increased abduction to adduc-
pathic; and one below-level neuropathic subtypein per- tion and abduction to internal rotation strength ratios (27).
sons with a neurologicly incomplete spinal cord injury or These findings led the authors to conclude that shoulder
with a zone of partial preservation (ZPP) extending to the muscle imbalance, with relative weakness of the adductors
level of the painwhich are intended to encompass all and external and internal rotators, may be a factor in the
other types of pain that are not specifically known to be development and perpetuation of rotator cuff impinge-
more common after SCI. These three latter orphan sub- ment in wheelchair athletes with paraplegia.
types have been given the generic name, other, to be Specific treatment (and prevention) strategies for
combined with the regional localization, (i.e., either shoulder pain originating from the rotatator cuff in persons
above-level, at-level, or below-level and the division into with SCI should include strengthening, stretching, opti-
either nociceptive or neuropathic pain types). mizing posture, and the avoidance of activities that pro-
mote impingement (28). Strengthening of the dynamic
shoulder stabilizers should occur in a balanced fashion. A
SCI ABOVE-LEVEL NOCICEPTIVE PAIN program should emphasize strengthening the posterior
shoulder musculature, including the external rotators, the
Mechanical or Musculoskeletal Pain posterior scapular muscles (rhomboids and trapezius), and
the adductors, because wheelchair use promotes the
SCI above-level mechanical or musculoskeletal pain is strengthening of the antagonists to these muscles (i.e., the
nociceptive pain that occurs in areas innervated by seg- anterior shoulder musculature) (27,28). Stretching of the
ments two levels or more above the neurologic level of dynamic shoulder stabilizers, especially the anterior shoul-
injury. The etiology of this pain may range from the var- der musculature, which often becomes hypertrophied and
ious soft tissue or bony injuries sustained during a trau- contracted through constant use during wheelchair propul-
matic event, such as a motor vehicle accident, to muscle sion and transfer activities, is also necessary in achieving a
strain related to muscles being used after a period of dis- balanced shoulder. In one controlled study of wheelchair
use, such as occurs after a lengthy acute hospitalization users, an intervention consisting of a 6-month exercise pro-
and during initial rehabilitation with transfer training and tocol that included two exercises for stretching the anterior
muscle strengthening exercises (19). One specific com- shoulder musculature and three exercises for strengthening
mon subtype of above-level mechanical or muscu- the posterior shoulder musculature, was found to be effec-
loskeletal pain is shoulder pain. tive in decreasing the shoulder pain that interfered with
functional activities (29). Optimizing the sitting posture,
with a goal of achieving the normal alignment of shoulder,
Mechanical or Musculoskeletal Shoulder Pain
head, and spine, both through posture exercises and
The prevalence of shoulder pain in persons with chronic through the provision of proper wheelchair seating systems,
paraplegia (data taken from questionnaires sent to com- (the latter often designed to allow a greater positive angle
munity dwellers) ranges from 40 to 50 percent (2123). of the seat plane with respect to the floor), can also help
Bayley, who reported a prevalence of shoulder pain of 30 decrease impingement during everyday activities (28).
percent in hospitalized veterans with paraplegia indepen- Lastly, activities that promote impingement, that is, activ-
dent in transferring, noted that 75 percent of these had ities in which the arm is abducted or flexed more than 90
chronic impingement syndrome and subacromial bursitis degrees, especially when combined with internal rotation,
and 16 percent had aseptic necrosis of the humeral head, should be identified by a thorough functional evaluation.
(four out of five of this latter group used alcohol at least Alternatives must be sought and implemented (28).
moderately) (24). Sie, who reported a prevalence of shoul- Adjuncts to the active treatment strategies for shoul-
der pain of 36 percent in outpatients with paraplegia fol- der pain originating from the rotatator cuff should also
lowed in a SCI clinic, noted that of the fifteen persons include, as necessary, the application of ice; relative rest;
agreeing to work-up, two had pain referred from the cer- anti-inflammatory medications; injections of steroid into
vical spine; eleven had pain that was orthopedically appropriate bursae or joints; and direct or indirect supras-
related, including tendinitis, bursitis, capsulitis, and capular nerve blocks. Most of these have been shown to
osteoarthritis; and two remained with the cause undeter- be effective in decreasing the pain originating from the
mined (25). An earlier study of a similar group revealed rotatator cuff in persons without SCI (3032).
bicipital tendonitis as the most common cause of shoul- Caution is warranted in the undertaking of surgical
der pain (26). When twenty wheelchair athletes with para- treatment of rotator cuff tendonopathies in persons with
PAIN MANAGEMENT IN PERSONS WITH SPINAL CORD DISORDERS 445
SCI, because the outcomes literature is sparse and not par- Unlike in persons without SCI who have CTS, rest-
ticularly favorable. Five persons with paraplegiaa total ing pressures in the carpal tunnel of persons with para-
of six shoulders with rotator cuff tearsunderwent sur- plegia and CTS are normal, and the presenting complaints
gical repair at one institution, but only one repair of a are usually of hyperesthesias of the thumb and index fin-
small tear limited to the supraspinatus muscle was ger and not of nighttime pain and wrist and hand pain
deemed successful, as evidenced by decreased pain and (40). Sie has suggested that the syndrome could be more
increased strength and range of motion (ROM) postop- accurately labeled as repetitive contact neuropathy (25).
eratively. The other repairs of large tears did not result The pathophysiology of this syndrome is thought to result
in an improvement in shoulder function or ROM (28). from a combination of repetitive trauma, as occurs in the
Another study reported three persons with paraplegia and propulsion of manual wheelchairs, and ischemia from
one with tetraplegiaa total of six shoulders with rota- repetitive marked increases in carpal canal pressures, as
tor cuff tearswho underwent repair with anterior occurs with push-up ischial pressure reliefs (persons with
acromioplasty and, when indicated, repair of the paraplegia often elevate their buttocks away from their
supraspinatus tendon. All were noted, at least initially, seating surface by using their arms with elbows and wrists
to have decreased pain and a functional capacity that locked in extension for 15 seconds every 15 minutes) or
approached or equaled preoperative levels (33). transfers from one seating surface to another (38).
The ulnar nerve may be involved in a manner simi-
lar to the median nerve; Davidoff found in a convenience
Autonomic Dysreflexia Headache Pain sample of thirty-one persons with paraplegia that three
A severe headache, usually described as pounding, had electrodiagnostic evidence of an ulnar mononeu-
associated with an elevated blood pressure, and often, ropathy at the wrist (39).
diaphoresis, piloerection, cutaneous vasodilation above The prevention and treatment of repetitive contact
the level of injury, bradycardia or tachycardia, nasal neuropathies at the wrist should begin with the cushion-
stuffiness, conjunctival congestion, and mydiriasis in a ing of the volar aspect of the wrist by use of padded gloves
person with SCI and a neurologic level most commonly and the avoidance of weight bearing on an extended wrist
above T6 indicates the presence of autonomic dysreflexia by substituting a neutral wrist position whenever possi-
(AD) (3436). The syndrome occurs in response to nox- ble. A side-to-side ischial pressure relief or forward lean
ious stimulation below the neurologic level and usually ischial pressure relief can often be substituted for the
responds promptly to the removal of the offending stim- push-up ischial pressure relief as well.
ulus. The most common cause is a distended bladder, but Surgical carpal tunnel release should only be per-
other causes may include bowel impaction, tight cloth- formed for pain that doesnt respond to conservative
ing, a localized abscess, a broken bone, or a urinary sys- measures or when progressive neurologic deterioriation
tem calculus. is observed, because a carpal tunnel release may actu-
ally leave a nerve more exposed to the direct trauma
that is thought to be a major contributory factor to this
SCI ABOVE-LEVEL NEUROPATHIC PAIN condition (41).
bilizing spinal orthosis. Persistent complaints require a over the rhomboids, levator scapulae, and supraspinatus
thorough evaluation, which may include obtaining the and infraspinatus muscles. A postulated mechanism for
appropriate spinal imaging to look for major possible scapular pain is based on the presence of a relative imbal-
causes of the pain. Depending on the specific cause, inter- ance in residual innervation to muscles of the shoulder
ventions may include surgery. Minor causes include mus- girdle caused by a mid-cervical spinal cord lesion,
cle strain after exercise, or soft tissue and incisional pain whereby increased scapular retractor muscle strength,
after surgery. The minor causes usually respond to non- compared to the scapular protractor muscle strength, may
surgical measures within days or weeks. One specific lead to abnormal scapulothoracic motion and secondary
common subtype, mirroring that of above-level mechan- inflammation and contracture of the muscles and joint
ical or musculoskeletal pain, is shoulder pain. capsule.
Although the treatment of musculoskeletal shoulder
pain in persons with tetraplegia depends on the exact eti-
Mechanical or Musculoskeletal Shoulder Pain
ology, a few principles should be emphasized. If a per-
The prevalence of shoulder pain in persons with chronic son who has tetraplegia has weak shoulder stabilizers and
tetraplegia is similar to that of persons with paraplegia, shoulder pain that is lessened when the humeral head is
ranging from 40 to 60 percent (22,23). Sie, reporting an manually reduced by an examiner into the glenoid fossa,
overall prevalence of 46 percent in outpatients with emphasis should be placed on proper positioning in the
tetraplegia, noted that twenty-seven of sixty-two indi- wheelchair with adequate arm support, whether through
viduals had shoulder pain that was orthopedically related, the use of an arm trough or a lap tray, so that the humerus
twenty had pain referred from the cervical spine, and nine remains within the glenoid fossa. When range-of-motion
other cases had pain from a variety of other known and (ROM) exercises are performed, the shoulder should not
unknown causes, including spasticity, heterotopic ossifi- be flexed or abducted beyond 90 degrees unless upward
cation, and syringomyelia (25). In a convenience sample rotation of the scapula and external rotation of the
of eleven veterans with tetraplegia, with a total of fifteen humerus also occurs; this will both minimize stress on the
shoulders painful for less than 6 months, who underwent glenoid capsule and minimize impingement of the rota-
work-up for their shoulder pain using a protocol designed tor cuff. If transfers, positioning, and bed mobility are
to determine the etiology of musculoskeletal shoulder performed with the assistance of another person, the
pain using physical exam, plain radiography, and arthrog- assistant must be trained to assist without putting undue
raphy, six shoulders were determined to have capsular stress on the arms of the assisted. Excess strain on a shoul-
contracture or capsulitis; four to have rotator cuff tears; der with already weak dynamic shoulder stabilizers will
two to have anterior instability; and one shoulder each to lead to strain on the static shoulder stabilizers, the liga-
have rotator cuff impingement, osteoarthritis, and ments of the capsule, and eventually to single or multidi-
osteonecrosis (42). Using this same protocol in thirteen rectional instability. If there is some active motor strength
veterans, with a total of fifteen shoulders that had been in the dynamic shoulder stabilizers, a balanced strength-
painful for more than 6 months, five shoulders were ening program can theoretically help reduce the instabil-
determined to have anterior instability; three shoulders to ity. Finally, performance of a daily stretching program,
have multidirectional instability; three shoulders to have especially if there is spasticity or a significant muscle
capsular contracture or capsulitis; and one shoulder each imbalance, with the goal of obtaining balanced shoulder
to have Charcot arthropathy, rotator cuff tear, rotator muscle strength with full ROM in all directions, is impor-
cuff impingement, and scapular pain (42). tant for the prevention of capsular contracture and cap-
In 1991, Silfverskiold observed that although 78 sulitis.
percent of persons with tetraplegia noted shoulder pain
within the first 6 months of injury; this decreased to 33
Visceral Pain
percent, 6 to 18 months later; of the persons whose pain
persisted, all exhibited spasticity (43). The nociceptive pain sensation that originates from dam-
Another type of pain, occasionally seen in persons age, irritation, or distention of internal organs or their
with mid-cervical levels of tetraplegia, is a musculoskele- supporting ligamentous structures is often altered by SCI.
tal pain originating in the muscles supporting the shoul- Reported causes of visceral pain in persons with SCI
der girdle, labeled scapular pain by Silfverskiold. In a con- include fecal impaction, bowel obstruction, bowel infarc-
venience sample of forty persons with tetraplegia for less tion, bowel perforation, cholecystitis, choledocholithia-
than six months who were evaluated for shoulder pain, sis, pancreatitis, appendicitis, splenic rupture, urinary
six were diagnosed with scapular pain. Six to 18 months bladder perforation, pyelonephritis, and the superior
later, only three remained with persistent pain (43). mesenteric artery (SMA) syndrome (4450).
Scapular pain is centered along the medial-dorsal border The sensory innervation to the parietal peritoneum
of the scapula, with associated tenderness to palpation is segmental and correlates roughly with the truncal der-
PAIN MANAGEMENT IN PERSONS WITH SPINAL CORD DISORDERS 447
matomes. Therefore, when the parietal peritoneum is irri- to localize abdominal pain to the right lower quadrant
tated at a level with at least some retained sensation, pre- of the abdomen early in the disease course. In the same
cise localization is usually possible. In contrast, sensa- study, three persons with paraplegiaone with a T11
tion from the internal organs is not segmental. Thus, neurologic level, another with a T12 level, and a third
when the visceral peritoneum or an internal organ is irri- with a L4 levelwere operated on for perforated
tated, localization is usually not precise in those without bowel. Only one developed increased abdominal
SCI, and even less so in those with SCI. spasm, but all three were able to localize abdominal
The anatomic pathways for visceral sensation are pain well. In ten persons with paraplegia at neurologic
less understood than the anatomic pathways for somatic levels between T6 and L1 who were operated on for
sensation. One putatitive anatomic pathway for the con- acute bowel obstruction, all but one, a person with a
duction of visceral sensation is that visceral afferents fol- T12L1 level, experienced crampy abdominal pain,
low sympathetic pathways. Evidence of this was demon- similar to that described in persons without SCI who
strated by Ray in 1947, in persons without SCI, who had have experienced this condition (47).
undergone sympathectomies for hypertension and under-
went gut distention and temperature stimulation via
catheter and direct gut stimulation during laparatomy SCI AT-LEVEL NEUROPATHIC PAIN
under local anesthesia (51). The visceral afferents are
thought to follow postganglionic sympathetic fibers from Radicular Pain
internal organs in the abdomen and pelvis to the par-
avertebral sympathetic chains (44,48,52). These visceral SCI at-level radicular pain is neuropathic pain arising
afferents have wide and overlapping receptive fields from from nerve root damage occurring within two der-
which they originate. They may pass directly into the matomal levels either above or below the neurologic level
spinal cord at the level where they reach the paravetebral of injury. The causes can include both the initial primary
chain, or they may travel either down or up the chain injury, even if there is no evidence of continued impinge-
before entering the spinal cord along pathways parallel- ment or irritation of the nerve roots by bone fragments,
ing the exiting preganglionic sympathetic fibers from the disk material, or scar. The character of the pain is often
spinal cord, (i.e., at the levels anywhere between T1 described as stabbing, shooting, or electric shock-like,
through L3, the only levels from which preganglionic although it has also been described as burning or aching
sympathetic fibers exit the spinal cord) (48). Because vis- (1,16,53). It is generally unilateral and paroxysmal in its
ceral afferents may enter the spinal cord at a level more presence, radiating in a dermatomal pattern. Hyperes-
rostral than that at which they joined the paravertebral thesia or allodynia, (i.e., pain evoked by non-noxious
chains, it is thought that a person with an upper thoracic stimuli), is common in the dermatomes innervated by the
(or lower level) lesion may have at least some preserved damaged nerve roots, although hypoalgesia or analgesia
visceral sensation through this pathway (48). can be seen, especially if the dorsal root ganglions are
Because SCI at-level visceral pain primarily occurs affected.
in persons with a neurologic level below the mid-thoracic
cord, nociceptive stimuli from the internal abdominal and
Central Pain
pelvic organs apparently travel rostrally along visceral
afferents, following the sympathetic pathways. If these SCI at-level central pain is neuropathic pain that occurs
sympathetic pathways are intact and the visceral afferents within two dermatomal levels either above or below the
from a specific inflamed organ join the spinal cord at a neurologic level and is not related to nerve root damage
level rostral to the neurologic level, then the specific vis- but to actual segmental spinal cord damage. The charac-
ceral sensation from this inflamed organ will presumably ter is typically of tightness, pressure, or burning in tho-
be unaltered by SCI. Moreover, depending on the neuro- racic level injuries and of numbness, tingling, heat, or cold
logic level below the mid-thoracic, the parietal peri- in cervical injuries (18). The distribution is typically bilat-
toneum has at least some retained sensation. Therefore, eral, involving single or multiple adjacent dermatomes
although at-level visceral pain may be quite altered, it is over the shoulders, arms, or hands for cervical injuries;
of a less-vague character and more often accurately local- single or multiple dermatomal bands about the chest for
ized than the visceral pain that occurs in individuals with thoracic injuries; and single or multiple adjacent der-
a higher neurologic level (44). matomes to the groin or legs for lumbar injuries. Fur-
In 1985, Juler reported a case series of various thermore, rather than being paroxysmal, SCI at-level cen-
abdominal emergencies in persons with SCI (47). The tral pain is typically continuous in its presence.
study included two persons with paraplegiaone with Nevertheless, it is often difficult to distinguish SCI at-level
a T11 neurologic level, and one with a T12 levelwho central pain from SCI at-level radicular pain, because
were operated on for acute appendicitis. Each was able both may present in the same manner. One specific com-
448 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
mon subtype of at-level central pain is the central pain level type: repetitive trauma combined with ischemia from
of syringomyelia. repetitive marked increases in carpal tunnel canal pres-
sures (38). Whereas above-level compressive neuropathy
pain is primarily a condition of those with paraplegia,
Central Pain of Syringomyelia
at-level compressive neuropathy pain is primarily a con-
A delayed onset of pain after SCI, especially beginning dition of those with low-level tetraplegia. However,
after 1 year, should raise the suspicion of syringomyelia because of the impaired sensation intrinsic to tetraplegia
as the cause of pain (5458). Nashold found a prevalence in the dermatomes overlying the peripheral nerves usu-
for syringomyelia of 65 percent in persons who had late ally affected by mononeuropathies at the wrist or elbow,
onset of pain after SCI (59). The associated presence of the prevalence of symptomatic at-level compressive neu-
bulbar signs and symptoms, especially facial pain, with ropathies is presumably low, occurring only in those per-
at-level pain of late onset is rare, but virtually diagnostic sons with relatively preserved sensation of the lower cer-
for syrinx (60). vical dermatomes. As a result, there have been no studies
Historically, syringomyelia of clinical significance has that have critically evaluated this entity.
been found in approximately 2 to 5 percent of persons
after SCI (5658,61,62). The most common initial symp-
Complex Regional Pain Syndrome Pain
tom of syringomyelia is pain, either unilateral or bilateral
(56,57,63). In one early series of seventeen persons with Spontaneous or evoked pain, allodynia, or hyperalgesia
SCI and syringomyelia, seven presented with pain on that fulfills the following three criteria can be classified as
coughing, seven presented with spontaneous pain, one complex regional pain syndrome (CRPS) pain: a) it is not
each presented with temperature sensation loss and exces- limited to the territory of a single peripheral nerve or root;
sive sweating above the injury, and one was asymptomatic b) it is disproportionate in intensity to what is expected;
(54). The pain of posttraumatic syringomyelia often begins and c) it is associated with edema, skin blood flow abnor-
at the segment marking the neurologic level of injury or mality, or abnormal sudomotor activity (66). In conform-
in a segment just proximal to this; its presence is often ini- ing to the International Association for the Study of Pain
tially intermittent (60). As the condition progresses, often (IASP) classification of chronic pain syndromes, CRPS type
the heralding segment of pain will develop anesthesia to I has replaced the previously favored term, reflex sympa-
pin and temperature sensation and transform into a tem- thetic dystrophy, while CRPS type II has replaced the pre-
porally continuous and characteristically burning type of viously favored term, causalgia (66). CRPS type I differs
pain (56,60). Burning pain or a dull aching pain have been from CRPS type II in that in the latter, an identifiable root
the most commonly reported characters of pain in several or peripheral nerve injury, is present.
large series, although sharp pain, electrical pain, and stab- A number of small retrospective case reports and
bing pain have also been reported as well (5660,64). prospective reviews have noted an association between
Other common presentations, which occur less frequently cervical SCI and CRPS (6773). The diagnostic criteria in
than pain, include worsening spasticity and motor strength these reports vary. One prospective study of sixty persons
(57,63). consecutively admitted to a SCI unit revealed six who
Magnetic resonance imaging (MRI) is the diagnostic complained of diffuse hand pain, swelling, and stiffness
study of choice in the evaluation of syringomyelia, although and had a pattern of diffuse uptake in the third phase of
a computed tomography (CT) myelogram with up to 24- a triple phase bone scana prevalence of 10 percent (70).
hour delayed imaging often shows contrast dye within a No controlled studies of treatment of CRPS after
syrinx cavity in those for whom an MRI is unobtainable. SCI have been performed. Of five persons reported by
The treatment of posttraumatic syringomyelia can Cremer as having CRPS after SCI, three improved after
include a) untethering if there is an associated tethering a short course of oral corticosteroids combined with or
of the spinal cord with the syrinx; b) untethering and place- without desensitization and range-of-motion (ROM)
ment of a shunt if untethering alone is unsuccessful; or c) exercises (71). Of the six persons reported by Gellman,
shunting if there is no associated tethering (63,65). Surgery, three underwent stellate ganglion blocks, with resulting
in addition to theoretically preventing further neurologic relief of symptoms (70). Finally, Levy reported a case of
deterioration, often alleviates pain: 56 percent having had a person with a C6 tetraplegia and an 8-year history of
substantial improvement in neurogenic pain after deteth- CRPS of the right arm that was unresponsive to range-of-
ering, according to one recent study (65). motion (ROM) exercises, contrast baths, transcutaneous
electrical nerve stimulation (TENS), tricyclic antidepres-
sants, and a dorsal rhizotomy. Ultimately, the condition
Compressive Neuropathy Pain
responded to a series of eight stellate ganglion blocks (74).
The presumed pathophysiology of SCI at-level compres- Finally, it is generally thought that an intensive hand
sive neuropathy pain is the same as for the SCI above- therapy program should underlie the treatment of CRPS,
PAIN MANAGEMENT IN PERSONS WITH SPINAL CORD DISORDERS 449
with pharmacologic approaches only added as adjuncts, complete and incomplete SCI, (levels ranging from C4T6)
although the evidence garnered through controlled stud- who were reported to have perforated appendicitis, seven
ies to support the use of physical modalities is limited, were able to localize pain to the right lower quadrant
even for persons without SCI and CRPS (75). (RLQ); the other, who had a C5 complete injury, noted
only shoulder pain (44,47). Seven of the eight persons, not
including a person with a C4 incomplete SCI, exhibited an
SCI BELOW-LEVEL NOCICEPTIVE PAIN elevated pulse rate, an elevated blood pressure, increased
abdominal spasticity, and sweating above the level of the
Mechanical or Musculoskeletal Pain lesion. The majority also exhibited early anorexia and an
elevated temperature (44,47). Of eight persons with both
SCI below-level mechanical or musculoskeletal pain complete and incomplete SCI, (levels ranging from
occurs only in persons with a neurologica;ly incomplete C3T4), who were reported to have other areas of perfo-
SCI or a complete injury with a zone of partial preserva- rated bowel, all had generalized abdominal and shoulder
tion (ZPP) extending to the level of the pain. Depending pain, but only one could localize his pain to the upper
on the degree of retained sensation, the presentation of abdomen (44,47). In addition, all persons with perforated
this nociceptive pain may vary. Severe spasticity often pre- bowel exhibited an elevated pulse rate, blood pressure, and
sents as SCI below-level muscular pain. Painful muscle temperature, as well as sweating above the level of the
spasms respond best to stretching and positioning, local lesion. Increased abdominal spasm was also present in
nerve or muscle blocks, or antispasticity medications. most as well (44,47).
Common antispasticity medications that have been These cases illustrate several points that should be
shown to be effective in treating spasticity after SCI remembered in the evaluation of abdominal pain in per-
include baclofen (Lioresal), a -aminobutyric acid sons with SCI: a) localization and intensity of nociceptive
(GABA) B receptor agonist; tizanidine (Zanaflex), an 2 visceral abdominal pain depends on the level and com-
receptor agonist; and diazepam (Valium), a benzodi- pleteness of neurologic injury; b) referred pain to other
azepine that interacts with the GABA A receptor (7679). sensate areas may overshadow the classical patterns of
Another medication, gabapentin (Neurontin), which is pain typically produced by specific intraabdominal
commonly used in the treatment of neuropathic pain after pathologies, if those classical patterns of pain are located
SCI, has also been shown to be effective in treating spas- in insensate areas; c) for persons with SCI who have a
ticity after SCI, and may be a good choice when treating neurologic level above the mid-thoracic level, the signs
both neuropathic pain and below-level muscular pain sec- and symptoms of autonomic dysreflexia (AD) may pro-
ondary to spasticity (80,81). vide more of a clue that there is intraabdominal pathol-
ogy than the pain itself; and d) other associated symptoms
may include anorexia, altered bowel patterns, nausea
Visceral Pain
and/or vomiting, and a change in the intensity or pattern
SCI below-level visceral pain primarily occurs in persons of abdominal spasticity.
with a neurologic level above the mid-thoracic region.
Depending on the degree of retained sensation below
level, this below-level visceral pain is characteristically SCI BELOW-LEVEL NEUROPATHIC PAIN
vague and poorly localized.
As mentioned in the SCI at-level visceral pain sec-
Central Pain
tion, visceral afferents are thought to follow postgan-
glionic sympathetic fibers from the abdominal and pelvic SCI below-level central pain is neuropathic pain that
organs to the paravertebral sympathetic chains, and from occurs caudal to the two dermatomal levels below the
there to the spinal cord at segmental levels between T1 neurologic level. Its distribution is generally not der-
and L3. This is thought to be the main pathway by which matomal but regional, enveloping large areas such as the
the brain receives nociceptive input from the viscera. A anal region, the bladder, the genitals, the legs, or com-
cervical SCI can disrupt this main pathway, because there monly, the entire body below the neurologic level. The
is no sympathetic outflow rostral to the T1 level. Never- character is often described as burning or aching,
theless, because persons with anatomically complete cord although other descriptors have included pressure, heav-
transections above this level are often still able to describe iness, cold, numbness, and pins and needles
visceral pain, other neural pathways, not yet defined in (1,2,6,7,9,53). It is usually continuous in presence,
humans, are assumed to exist (50,82). One such long- although the intensity of the pain can fluctuate in
purported pathway is the vagus nerve (44,8283). response to a number of factors including psychologic
In one case series, initially reported by Charney and stress, anxiety, fatigue, smoking, noxious stimuli below
later by Juler, of eight persons with both neurologically the level of injury, and weather changes (1,2,4,5,8,84).
450 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
type of injury (110,111). Electrophysiologically, this may ministration of an 2 agonist (122). This suggests that
result in spontaneous or abnormally high-frequency direct NMDA antagonism is a mechanism, at least in part,
action potential generation, which may be blocked by by which TCAs cause analgesia.
local anesthetics and anticonvulsants without blocking Therapeutic TCA analgesic dosage levels may be lower
normal nerve conduction (109111). than therapeutic antidepressant levels. Analgesia is usually
Allodynia is defined behaviorally as pain evoked by achieved more quickly than the antidepressive action. Serum
non-noxious stimuli. Sprouting is one putative long-term drug levels can be helpful in indicating a maximal dose in
adaptive mechanism occurring in SCI at-level radicular nonresponders who have experienced no side effects because
pain. Sprouting helps to explain the allodynia that is com- of inter-individual variability in the response to TCAs.
mon in the dermatomes innervated by the damaged roots. Dose-related anticholinergic side effects, including
Normally, primary afferent A (non-pain) fibers termi- dry mouth, blurred vision, orthostatic hypotension, uri-
nate in Rexeds lamina III and above and not in Rexeds nary retention, constipation, and sedation, can be mini-
laminas I and II of the dorsal horn, where primary affer- mized by a slow dose escalation starting from a low start-
ent C fibers terminate. However, after peripheral nerve ing dose of 10 mg or 25 mg before sleep. Choosing a
injury, primary afferent A (non-pain) fibers may sprout secondary amine compound (e.g., desipramine [Nor-
to terminate in laminas I and II (112,113). A second long- pramin] and nortriptyline [Pamelor, Aventyl]) in lieu
term adaptive mechanism that may be important in SCI of a more anticholinergic tertiary amine compound [e.g.,
at-level radicular pain that occurs after prolonged nox- amitriptyline (Elavil, imipramine [Tofranil], and dox-
ious stimulation is phenotypic switching with regards to epine [Sinequan]) may also improve tolerability.
the synthesis of modulating neuropeptides by primary Although uncommon, TCAs can provoke cardiac dys-
afferents. Primary afferent A (non-pain) fibers have been rhythmias. For this reason, TCAs are containdicated in
shown to begin synthesizing substance P after receiving persons who have a significant conduction abnormalities.
noxious stimulation for longer than 48 hours, thus func- Screening electrocardiograms should be obtained in per-
tionally resembling primary afferent C fibers (114). sons at risk for heart disease.
AnticonvulsantsFirst Generation
TREATMENT
Carbamazepine (Tegretol) is the prototypical drug of the
Oral Pharmacologic Interventions first generation anticonvulsant class that historically has
been used for the treatment of lancinating or shooting
neuropathic central or radicular pain, based on its effec-
Tricyclic Antidepressants
tiveness in the treatment of lancinating or shooting neu-
Amitriptyline is the prototypical drug of the tricyclic anti- ropathic pain of a different etiology, (e.g., trigeminal
depressant (TCA) class, and it has been used for treatment neuralgia), in randomized placebo-controlled trials
of continuous neuropathic central pain based on its effec- (123128). Paroxysmal lancinating or shooting pain is
tiveness in the treatment of continuous neuropathic pain thought to be initiated by abnormal ectopic discharges
of other etiologies in randomized placebo-controlled tri- from injured neurons (129). Carbamazepine inhibits this
als (115117). It must be noted that there is no evidence abnormal ectopic activity (131) at drug levels far below
that TCAs are more effective for one type of neuropathic the threshold needed to block the propagation of action
pain, (e.g., continuous and burning), than another, (e.g., potentials in noninjured nerves (109). Abnormal ectopic
paroxysmal and sharp). discharges have been recorded both in the peripheral and
TCAs are thought to release norepinephrine (NE) central nervous system, from demyelinated peripheral
onto 2 receptors in the dorsal horn (118), an action that afferent axons, neuromas, dorsal root ganglia, the dor-
has long been thought to be the primary mechanism pro- sal horn, and the thalamus (85,86,108,109,130,131). A
ducing analgesia. Both afferent noxious stimulation and major contributing mechanism to the development of
microstimulation of NE-containing brainstem nuclei in ectopic hyperactivity appears to be a change in the pat-
animals have been shown to elicit an increase in spinal tern of expressed neuronal sodium channels, with upreg-
levels of NE (119,120). The microstimulation of NE-con- ulation of some specific sodium channels and downreg-
taining brainstem nuclei in animals caused inhibition of ulation of others, after neuronal injury (110,111).
escape reflexes as well (120). Carbamazepine acts by blocking sodium channels.
TCAs have also been found to bind the NMDA- The usual therapeutic plasma concentration,
receptor complex (121). Intrathecal injections of tricyclics extrapolated from treatment of trigeminal neuralgia,
in animal studies have been shown to decrease NMDA- ranges from 5 to 10 g/ml, but dose-limiting central ner-
induced pain behavior in a dose-dependent manner. This vous system (CNS) side effectsincluding sedation,
decrease in pain behavior was not affected by the coad- ataxia, vertigo, blurred vision, nausea, and vomiting
452 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
often appear above 8 g/ml (132). These CNS side One putative mechanism for its purported action
effects, which resolve with a lower dose, can be avoided relating to the abolition of SCI at-level central pain, SCI
by a slow dose escalation from a low starting dose of 100 at-level radicular pain, and SCI below-level central pain
mg twice a day. The renal, hepatic, and hematologic sys- concerns the ability of gabapentin at therapeutically rel-
tems may also be affected. Monitor these systems by evant concentrations to limit high-frequency repetitive fir-
reviewing a complete blood count (CBC) and serum ing of sodium-dependent action potentials through an
chemistry tests at the beginning of treatment, after 2 indirect, uncharacterized manner (145,146). A high-affin-
weeks, and thereafter at 6 week intervals. Aplastic ane- ity binding site for gabapentin, unrelated to GABA, has
mia is a rare but irreversible hematologic side effect. been identified in the brain and dorsal horn of the spinal
Thirteen fatal cases of aplastic anemia, thought to be a cord as the 2 auxiliary protein subunit of voltage-
result of carbamazepine treatment, were reported sensitive calcium channels (147,148). However, the rela-
between 1964 and 1982 (133). tionship between the gabapentin binding site and the
Other first-generation anticonvusants that are function of calcium channels remains unclear at this time.
thought to have a similar mechanism of action include Gabapentin may interfere directly in the sensitization cas-
phenytoin (Dilantin) and valproate (Depakene). They cade through action at this receptor. Another mechanism
have been less well studied in randomized controlled stud- through which gabapentin may have an effect on noci-
ies for the treatment of pain, although phenytoin has been ceptive processing, especially decreasing allodynia, con-
shown to be superior to placebo in the treatment of pain cerns the indirect ability of gabapentin (not through
associated with diabetic polyneuropathy (128,134,135). GABA receptors and GABA uptake carriers [149] to
increase brain GABA levels, presumably by stimulating
the GABA synthetic enzyme glutamic acid decarboxylase
AnticonvulsantsSecond Generation
(GAD) (150). Gabapentin is actively transported by the
Gabapentin (Neurontin), which has the structure of the L-system amino acid transporter across the wall of the
inhibitory amino acid, -aminobutyric acid (GABA) with small intestine and the bloodbrain barrier (151). Other
an attached cyclohexane ring, is often used in the treatment central actions of unknown significance caused by the
of neuropathic pain because of its good safety profile and administration of gabapentin include a decrease in brain
anecdotal efficacy. It is the prototypical second-generation glutamate levels by the stimulation of glutamic acid dehy-
anticonvulsant used in the treatment of neuropathic pain. drogenase (a glutamate-catabolizing enzyme) and the
Gabapentin has been shown in randomized, double- inhibition of the glutamate synthetic enzyme, branched-
blind, placebo-controlled trials to be effective in treating chain amino acid aminotransferase (150); an increase in
pain associated with diabetic neuropathy, the pain of free plasma serotonin (152); and the decreased release of
which is typically continuous, and pain associated with serotonin, dopamine, and norepinephrine (145).
postherpetic neuralgia, the pain of which may be either Side effects, which may include dizziness and seda-
continuous or paroxysmal (136,137). Moreover, in these tion, may occasionally be seen at a low starting dose (e.g.,
studies, gabapentin treatment was not shown to be sig- 100 mg), although in general the side effects are dose-
nificantly more effective in treating one type of pain, (e.g., dependent and can be minimized by a slow upward dose
burning pain), than another, (e.g., shooting pain), as iden- titration. Doses of up to 3,600 mg per day are commonly
tified on the sensory pain items of the McGill Pain Ques- prescribed.
tionnaire (138140). In an open-label study by Attal, Other second-generation anticonvulsants include
gabapentin was effective in reducing both continuous and lamotrigine (Lamictal) and topiramate (Topamax).
paroxysmal pain, as well as dynamic mechanical and cold They have been less well studied in randomized controlled
allodynia, in patients (including some with SCI) with both studies for the treatment of pain, although lamotrigine
central and peripheral nervous system lesions (141). has been shown to have an analgesic effect in the treat-
In spinally injured rats, the repeated administration ment of refractory trigeminal neuralgia (153).
of systemic gabapentin has been shown to alleviate allody-
nia-like behaviors (142). Gabapentin has also been shown
Nonsteroidal anti-inflammatory drugs
to suppress ectoptic nerve discharge and reverse allodynia-
like behavior in rats with presumed radicular type pain Nonsteroidal anti-inflammatory drugs (NSAIDs) inhibit
caused by partial ligation of the sciatic nerve (143,144). the enzyme cyclo-oxygenase (COX) (154). Although the
Gabapentin seems to be safe. As of 1999, almost 3 major peripheral mechanism of COX is the facilitation of
million people had been treated with gabapentin in var- the transformation of arachidonic acid into the stable
ious studies and open use, without a causal relationship prostaglandins that are important for sensitizing noci-
to a specific, life-threatening organ toxicity (145). Delib- ceptors, COX is an integral enzyme involved with central
erate overdoses associated with suicide attempts have nociceptive processing as well. During central sensitiza-
been reported without neurologic sequelae (145). tion, calcium levels in dorsal horn neurons increase by a
PAIN MANAGEMENT IN PERSONS WITH SPINAL CORD DISORDERS 453
number of different mechanisms and stimulate the metab- mary afferent neurotransmitter release by suppression of
olism of arachadonic acid by phospholipase A2. The voltage-sensitive calcium currents, and direct inhibition
arachadonic acid is then metabolized by COX, as in the of dorsal horn neurons by inhibition of adenyl cyclase and
periphery, into stable prostaglandins. the activation of outward potassium currents (163).
In animals, intrathecal NSAIDs have been shown to Controversy exists regarding the effectiveness of opi-
block pain-related behaviors induced by NMDA or sub- oids in relieving neuropathic pain because of multiple defi-
stance P (155). Conversely, intrathecal arachadonic acid nitions of neuropathic pain, methodologic shortcomings in
has been shown to potentiate pain-related behaviors available controlled clinical trials, different methods of pain
induced by an inflammatory pain model (156). In assessment, and inter-individual differences in response to
humans, NSAIDs have been shown to have central anal- opioid doses (164). Nevertheless, intrathecal morphine has
gesic effects as well (157). been shown to have antinociceptive properties in an ani-
NSAIDs are effective analgesics for most types of mal model, wherein a photochemically induced spinal cord
nociceptive pain and, although they have not been eval- lesion leads to mechanical allodynia in rats that is analogous
uated with randomized controlled trials for neuropathic to SCI at-level and below-level central pain (165). This pho-
pain after SCI, anecdotally they seem to benefit many per- tochemically induced nervous system damage has been
sons with this type of pain as well. applied to a peripheral nerve in nonspinallyinjured animals
Side effects involving the gastrointestinal system as well, simulating SCI at-level radicular pain. Intrathecal
relate to the inhibition of the COX-1 isoform. The COX- morphine administration relieved the resultant abnormal
2 isoforms are found in the nervous system and female pain-related behaviors here as well (166).
reproductive system. Most NSAIDs inhibit COX-1 and Methadone (Dolophine), in addition to being a
COX-2 isoforms equally but COX-2 selective isoforms receptor agonist, is an N-methyl-D-aspartate (NMDA)
have become available in the United States and include receptor channel blocker (167). This additional mecha-
celocoxib (Celebrex) and rofecoxib (Vioxx). nism of antinociception, (i.e., NMDA receptor blockade),
combined with the high potency of methadone when
given in repeated doses, may make it a good choice for
Capsaicin
treating intractable neuropathic pain after SCI. Other
Capsaicin cream is a neuroblocking agent often used top- commonly used opioids used for moderate to severe pain
ically to treat cutaneous dysesthesias. It acts by deplet- include morphine (MS Contin); fentanyl, which is avail-
ing modulating neuropeptides, including substance P, able as a transdermal patch; hydromorphone (Dilaudid);
somatostatin, galanin, and vasoactive intestinal polypep- and levorphanol (Levo-Dromoran).
tide, from primary afferent C fibers, both centrally and If used for intractable pain in persons with SCI, opi-
peripherally (158,159). Skin treated with capsaicin retains oids should be used in accordance with guidelines that
normal sensation, although many persons note an initial have been developed for their use in persons with non-
burning sensation after application, which decreases malignant pain (see Table 33.2) (168,169). The risk of
markedly after repeated administrations. The mechanism addiction, defined as the loss of control over drug use or
of this transient burning pain is presumably the depletion continued use despite harm, is rare in persons with pain,
of substance P from the primary afferent C fibers (158). but without a history of substance abuse, and without a
Capsaicin is most effective when applied multiple times psychiatric history (164,168,170). It is important to note
per day to painful areas for at least a few weeks that because of a high inter-individual variability in the
(158,159). It has been shown to be effective in treating response to opioids, opioid dosing should be titrated until
various types of neuropathic pain, although not specifi- satisfactory pain relief is obtained or undesirable opioid-
cally pain after SCI (158161). Anecdotally, it is of ben- induced side effects occur. The latter assures that the drug
efit to many persons with pain after SCI, especially when is reaching its target receptors (164).
applied to areas that feel cold. The side effects of opioids that seem to be most dif-
ficult to manage are constipation and sedation. With
regard to constipation, the addition of stimulant laxa-
Opioids
tives, bulking agents, or stool softeners often is necessary.
-endorphin, leuenkephalin, met-enkephalin, and dynor- Persons with SCI may lack, depending on the complete-
phin are natural opioids which act at , , and G-pro- ness and the neurologic level of injury, the warning sen-
tein complex opioid receptors (162). Morphine, the pro- sation of discomfort that accompanies constipation, nor-
totypical opioid extracted from opium, is a receptor mal bowel transit times, and voluntary bowel control.
agonist. In the dorsal horn, opioid receptors are found Therefore, close monitoring for ileus must be maintained.
both presynaptically on C fiber primary afferents and post- With regards to sedation, the addition of a stimulant med-
synaptically on intrinsic neurons (163). The mechanisms ication, such as methylphenidate (Ritalin), as an adjunct
of action of opioid agonists include the inhibition of pri- is often helpful to maintain alertness.
454 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
1. Long-term opioid therapy should be considered cause. In rare cases, this may involve referral for
only after reasonable analgesic modalities have formal treatment of an addiction disorder; more
failed. often, therapy must be adjusted and strict controls
2. Opioids should be viewed as a complementary ther- reestablished. Repeated episodes of aberrant drug-
apy that might be combined with other analgesic related behavior necessitate tapering and discontin-
and rehabilitative approaches. uation of treatment.
3. A single practitioner should take responsibility for
the administration and monitoring of therapy.
4. A prior history of substance abuse, severe character
pathology, and a chaotic social situation should be Finally, if systemically administered opioids are not
viewed as relative contraindications to therapy. tolerated because of side effects, intrathecally administered
5. Informed consent should be documented in the opioids should be considered. The long-term intrathecal
medical record. The consent discussion should
infusion of morphine administered by implanted drug
include information about side effects (including the
risk of additive side effects from other centrally act-
pumps has been found to be safe. Histologic changes about
ing drugs and the need to avoid driving and other the spinal cord, noted in both animal studies and human
potentially dangerous activities if cogntive impair- autopsy studies, have only been related to the implanted
ment should occur), the small risk of addiction, and infusion catheter and not to a chemical effect of the infused
the need for clearly defined parameters for dosing. drug (171). Although controlled studies of the treatment
6. Around the clock dosing with long acting medica- of pain after SCI with intrathecal morphine are lacking,
tion is preferred for the management of chronic intrathecal morphine has been shown, alone or in combi-
pain, but as needed dosing may be considered in nation with other medications, to provide relief for many
some patients with widely fluctuating pain. persons with chronic intractable pain, including persons
7. Opioid dosing should be titrated until either satis- with pain after SCI (172174).
factory pain relief or undesirable opioid-induced
side-effects occur. Side effects should be managed
and a trial with alternative opioids should be con- N-methyl-D-aspartate Antagonists
sidered if dose-limiting side effects are a major
problem in establishing a favorable treatment. If a Intrathecal administration of N-methyl-D-aspartate
dose cannot be stabilized at a level associated with (NMDA) receptor antagonists have little effect on the
benefits that clearly exceed disadvantages, therapy responses to acute nociceptive stimulation in normal ani-
should be discontinued. mals, but markedly decrease touch and heat hyperalge-
8. Following dose titration, the dose should be stabilized sia after nerve injury (175177). Ketamine is a noncom-
and not changed by the patient without prior consent
petitive NMDA receptor antagonist that acts by blocking
of the physician. A monthly quantity of drug should
be established. Some patients should be offered a
open the NMDA channel by an allosteric mechanism
defined smaller quantity to be used for transient (178). When administered systemically, it has been shown
exacerbation of the pain (so-called rescue doses). to have analgesic effects in persons with SCI below-level
9. Initially, patients should be evaluated at least central pain in one controlled cross-over study of nine
monthly. Once dosing is stable, visits may be less patients (179). Nevertheless, the systemic use of ketamine
frequent. has been limited by side effects including hallucinations,
10. At each visit, the physician should assess the dizziness, and drowsiness (180). The use of ketamine
patient for (1) the degree of analgesia; (2) the occur- intrathecally has been limited by neurotoxicity; lympho-
rence of side effects; (3) functional status (physical cytic vasculitis of the spinal cord and subpial vacuolar
and psychosocial); and (4) any evidence of aberrant myelopathy have both been reported (181,182).
drug-related behavior. This assessment should be
clearly documented in the medical record.
11. Evidence of aberrant drug-related behavior, such as
drug hoarding or uncontrolled dose escalation, NONPHARMACOLOGIC AND
should be carefully evaluated. The clinical response NONPSYCHOLOGIC INTERVENTIONS
developed from the assessment should stop the
behavior and appropriately manage the underlying Spinal stabilization with instrumentation and fusion is
indicated for an unstable spine that causes SCI at-level
PAIN MANAGEMENT IN PERSONS WITH SPINAL CORD DISORDERS 455
mechanical pain. Surgical decompression and stabiliza- electrical recordings of both spontaneous and C-fiber
tion is also indicated to relieve compression on nerve evoked signals had 100 percent relief at 3 months to 5
roots that cause SCI at-level radicular pain. Pain origi- years (194). These results contrast starkly with what had
nating from a syrinx often disappears with drainage of been previously reported by other investigators using the
the syrinx, and SCI at-level nociceptive pain caused by conventional approach.
spinal instrumentation is often relieved by the removal The DREZ procedure may have limited use in the
of the offending instrumentation. There are few other treatment of intractable SCI at-level radicular or central
instances where nonpharmacologic and nonpsychologic pain as it has been conventionally performed and in the
interventions are of proven benefit for chronic pain after treatment of SCI below-level central pain if guided by
SCI, except possibly the dorsal root entry zone (DREZ) intramedullary recordings, but it is not recommended cur-
procedure, which is reviewed below. Some common con- rently, as it has been conventionally performed, for the
troversial invasive and noninvasive techniques and pro- treatment SCI below-level central pain.
cedures are also discussed below.
Motor Cortex and Deep Brain Stimulation
Spinal Cord Stimulation
Motor cortex stimulation involves the placement and
Spinal cord stimulation involves the percutaneous implan- electrical activation of an implanted subdural electrode
tation and electrical activation of single- or multiple-chan- grid over the motor cortex. It has been described only
nel electrodes within the epidural space over the dorsum recently for the treatment of various types of neuropathic
of the spinal cord. It has not been found to be effective in pain, in a limited number of uncontrolled studies
treating either radicular or central pain after SCI (183188). (195197). Only a few persons with pain after SCI have
reportedly been treated with this technique, and the
results have been mixed (195).
DREZ Ablation Procedures
Deep brain stimulation involves the placement and
The dorsal root entry zone (DREZ) has been defined as electrical activation of implantable electrodes within the
the proximal portion of a dorsal nerve root and its adja- somatosensory thalamus, which includes the ventropos-
cent superficial layers of dorsal horn. Destruction of this terior lateral (VPL) and venteroposterior medial (VPM)
area, using either radiofrequency or laser coagulation, as nuclei; the caudal medial thalamic areas around the third
first described by Nashold, has been shown to be some- ventricle, which includes the periventricular grey matter
what effective in reducing SCI at-level radicular and (PVG), and adjacent nuclei, centralis medialis (CM), and
central pain, but not SCI below-level central pain parafascicularis (Pf); and near the junction of the third
(85,189193). As conventionally performed, the coagu- ventricle and the sylvian aqueduct, the rostral ventral peri-
lation is performed two levels above and one or two lev- aqueductal gray matter (PAG) and caudal ventral PVG.
els below the level of injury, thus causing an ascent of the This intervention has not been found to be effective for
sensory neurologic level by two segments. Two large stud- pain after SCI (198,199).
ies of persons with SCI at-level radicular or SCI at-level
central pain treated with conventional DREZ lesioning
Acupuncture
have been performed. In the first study, Friedman
reported that twenty-three of thirty-one persons under- Acupuncture is a neurostimulatory technique in which
going the procedure had good relief (defined as lack of small solid needles are inserted into the skin at varying
pain or pain that did not require analgesics or interfere depths, typically penetrating the underlying musculature
with daily activities). In the second, Rath reported that (200). Meta-analyses evaluating its efficacy in multiple
twelve of seventeen had fair or good relief (191,193). types of chronic pain have been inconclusive (201204).
In 1993, Edgar reported a method of recording
abnormal signals in the DREZ before selectively lesion-
Transcutaneous Electrical Stimulation (TENS)
ing the areas with abnormal signals. He noted that forty-
two of forty-six persons with spinal cord pain in his series, In 1975, Davis reported the results of the use of transcu-
(predominantly of an SCI below-level central type pain), taneous electrical stimulation (TENS) on thirty-one per-
when treated with selective lesioning, had at least 50 per- sons with various types of pain after SCI, including radic-
cent relief (85). In 1999, Falci reported that nine of eleven ular, central, and pain at the site of injury. Effectiveness,
persons with SCI below-level central pain treated with defined as a reduction in pain medication or continued
DREZ guided only by spontaneous intramedullary elec- use of the device, was noted only in those who had a com-
trical recordings had at least 50 percent relief, whereas ponent of the pain at the site of injury and not in those
twenty-one of twenty-five persons with SCI below-level with radicular or central pain (205). In 1978, Hachen
central pain treated with DREZ guided by intramedullary reported the use of TENS in thirty-nine persons with a
456 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
similar array of pain types, finding at 3 months slight or was of minor consideration, pain displacement from one
moderate pain relief for most of the persons with pain at area of the body to another less threatening area, replace-
the site of vertebral trauma, almost complete relief for ment of painful sensations with more tolerable sensations,
one-half of the persons who had radicular pain, and no or a direct or indirect suggestion that the pain will no
effect for most of the persons with central pain (206). longer be experienced (213). The postsuggestion phase
Thus, TENS may be a useful adjunct for persons with SCI involves continuation of the new behavior following ter-
at-level mechanical or musculoskeletal pain or radicular mination of hypnosis (207). Strong evidence suggests that
pain, if the more-direct approaches to treating these types hypnosis is effective in cancer pain and some evidence
of pain have been unsuccessful. shows that it is effective for other chronic pain types as
well (207,213,214).
PSYCHOLOGIC INTERVENTIONS
CONCLUSION
The goal of relaxation, hypnotic, and cognitive-behav-
ioral techniques with regard to treating chronic pain after Pain in persons with SCI should not continue to be a com-
SCI is to teach and subsequently to enable the patient to mon and significant problem. Certain issues, if addressed
perform the techniques himself, manage his pain over the in a timely manner, may lessen the impact of this sec-
long term, and minimize the impact of the pain on his life. ondary condition after SCI. First, clinicians must be aware
These techniques are not brief interventions to abolish of the problem and be knowledgeable about current state-
pain. of-the-art treatments. Second, a standard classification
Cognitive-behavioral therapy (CBT) aims to alter based on mechanism must be adopted to facilitate com-
negative thoughts, attitudes, and emotions through edu- munication between clinicians and researchers. Third,
cation, skills acquisition, cognitive and behavioral basic research must focus on the basic neurobiology of
rehearsals, and generalization (207). For chronic pain of pain after SCI. Fourth, open-label studies, followed by
multiple etiologies, not necessarily related to SCI, CBT randomized controlled studies, must be performed to
has been shown in a recent meta-analysis to be effective evaluate both the pharmacologic and the nonpharmaco-
in changing pain experience, mood/affect, cognitive cop- logic interventions that have been identified through basic
ing and appraisal (reduction of negative coping and research as promising potential relief from pain. And
increase in positive coping), pain behavior and activity fifth, psychologic and alternative medical interventions
level, and social role function when compared to wait- must be assessed in a controlled manner.
ing list control conditions (208). Other meta-analyses that
evaluated CBT for chronic back pain also showed effec-
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fibers. Group Ia (primary sensory) afferents convey both and lead to increased alpha motor response to normal Ia
phasic and tonic stretch information. Group II fibers input (1).
innervate static nuclear bag and nuclear chain fibers and Reciprocal Ia inhibition decreases the chance for the
convey information on the tonic/static change in muscle cocontraction of antagonistic and agonistic muscles dur-
length. Gamma motor efferent fibers innervate the intra- ing the stretch reflex or during voluntary movement. Nor-
fusal contractile elements that stiffen the region of the mally, Ia inhibitory interneurons are controlled by descend-
nuclear bag fibers; these gamma fibers maintain spindle ing excitatory pathways. Evidence exists for decreased
sensitivity during skeletal muscle contraction. excitability of Ia inhibitory neurons after rostral lesions
of the CNS. This dysfunction could lead to an increased
cocontraction and weakness of voluntary movement (30).
The Golgi Tendon Organ
Nonreciprocal Ib inhibition has been found to be decreased
The Golgi tendon organ is sensitive to muscle tension and or even replaced by facilitation in stroke and SCI patients
is innervated by myelinated Ib sensory afferents. The with spastic paresis and spastic dystonia, but not in sub-
Golgi tendon organ is highly sensitive to the muscle ten- jects without spastic dystonia (31).
sion created by active muscle contraction, but it has a high Patients with spastic paresis from SCI show
threshold to detecting passive stretch. Stimulation of Ib increased, rather than decreased, levels of recurrent Ren-
afferents leads to an inhibition of the homonymous motor shaw inhibition. Renshaw cells are inhibitory neurons
neuron and its synergists and to excitation of its antago- that are stimulated by colateral axons from alpha motor
nistic motor neurons. The reaction has been called the neurons. When an alpha motor neuron fires, it stimulates
inverse myotactic reflex because its actions oppose those a Renshaw cell that, in turn, inhibits the initiating motor
of the stretch (myotactic) reflex. It is also called Ib non- neuron and its synergists. The Renshaw cell also inhibits
reciprocal inhibition. It should be noted that this reflex the la inhibitory interneuron associated with the initiat-
is stimulated by muscle tension, whereas the stretch reflex ing motor neuron. Because the Renshaw cell inhibits Ia
is stimulated by a change in muscle length. Thus, the inhibitory interneurons as well as agonist alpha motor
Golgi tendon organ has been hypothesized to function neurons, increased Renshaw cell activity may contribute
as part of a muscle tension feedback system (29). to spasticity by decreasing reciprocal Ia inhibition (32).
Hyperexcitability of alpha motor neurons may contribute
to spasticity. Examples of those primary changes in mem-
Hyperreflexia
brane properties that would be expected to produce
The stretch reflex may be viewed as a feedback system increased alpha motor neuron discharge include a reduc-
with muscle length as the regulated variable. The gain, tion in area of dendritic membranes, deafferentation den-
or inputoutput relationship, of the stretch reflex to a dritic hyperexcitability, and an increase in the number of
given change in muscle length is kept low by descending excitatory synaptic inputs by sprouting (1).
influences when the individual is at rest, and the gain is
enhanced when physical demand for performance is
Polysynaptic Connections
needed. Hyperreflexia is an example of the abnormal seg-
mental reflex regulation associated with an upper motor If one considers all segmental connections, most reflex
lesion. Theoretically, hyperreflexia could result from pathways are polysynaptic. For example, Ia reciprocal
decreased inhibitory mechanisms, hyperexcitability of inhibition depends on interposed interneurons connect-
alpha motor neurons, peripheral nerve sprouting, and ing sensory afferents and antagonistic motor neurons to
increased gamma fiber activity. opposing muscle groups. In addition to input from the
Long-term reductions in inhibition may contribute to periphery, interneurons receive excitatory and inhibitory
hyperreflexia. Examples of inhibition types are: recurrent signals from descending pathways. The modulation of
Renshaw inhibition, reciprocal Ia inhibition, presynaptic excitatory and inhibitory input to interneurons allows
inhibition, nonreciprocal Ib inhibition, and inhibition from supraspinal centers to control joint stiffness. The interneu-
group II afferents. Various lines of research have supported rons that mediate Ib nonreciprocal inhibition connect
464 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
inhibitory agonist and excitatory antagonistic motor neu- cuits responsible for ipsilateral flexion and crossed limb
rons. At rest, Ib nonreciprocal inhibition opposes the extension also receive descending inputs and coordinate
actions of the stretch reflex. Ib interneurons receive con- voluntary limb movements.
vergent input from Ia spindle afferents, low-threshold
cutaneous afferents, and joint afferents, as well as exci-
Exteroceptive versus Proprioceptive Responses
tatory and inhibitory input from descending pathways.
The afferents for Golgi tendon organs make polysynap- A cutaneous stimulus may modulate the activity of par-
tic connections, via interneurons, to homonymous motor ticular motor neurons. Touching an area of skin may
neurons and synergist and antagonist motor neurons. cause a reflex contraction of specific muscles, usually
Because the Golgi tendon organ is exquisitely sensitive those beneath the area of stimulation. This is called a local
to active muscle tension, and because Ib interneurons sign and is an exteroceptive response. Cutaneous stimuli
receive short-latency convergent input from Ia spindle may not always produce observable contractions. They
afferents, cutaneous afferents, joint afferents, and may have subthreshold or facilitative effects. Proprio-
descending pathways, those interneurons receiving Ib ceptive information is transmitted from muscle spindles
afferent innervation are likely to play an important role and Golgi tendon organs via group Ia, II, and Ib afferents.
in exploratory movements of the limbs. For example, the
interneuron receiving Ib afferent information would
mediate the inhibition of agonist muscle contraction if a GOALS OF TREATMENT
limb encountered an unexpected obstacle, thus reducing
force against the impediment. Also, Ib inhibition could Because spasticity results from neurologic dysfunction
function to decrease muscle contraction at the extreme within a variety of regions in the CNS, the associated loss
range of joint motion. The net effect of Ib inhibition dur- of voluntary motor function can be highly variable
ing volitional activity depends on inputs from multiple among patients with symptomatic spasticity. Further-
sources. Recurrent Renshaw inhibition takes place via more, there is great variability among individuals with
polysynaptic connections to alpha motor neurons via spasticity. For example, the skill level and functional
Renshaw cells and Ia inhibitory interneurons. expectation of children with spastic CP differs greatly
Although few monosynaptic connections have been from adults with MS, SCI, or cerebrovascular disease.
observed for group II afferents, the majority of connec- Even among patients with a similar diagnosis, the per-
tions are polysynaptic and involve several classes of formance of specific physical functions depends mainly
interneurons. Most group II afferents from muscle origi- on the ability to use controllable muscle strength and the
nate from muscle spindles, but some originate as free motivation to perform the task regardless of the effort
nerve endings or in other types of receptors. Experimen- required. It is often difficult to predict the contribution
tally, group II fibers activate flexor synergistic muscles and of spasticity to interference with otherwise usable vol-
inhibit physiologic extensors. Unopposed, group II-medi- untary muscle control to do a specific task. For exam-
ated activity produces tonic activation of physiologic limb ple, a C4 quadriplegic can use a mouth stick or suck-and-
flexors. Group III and IV afferents originate from deep puff actuator to operate a computer, telephone, and
muscle and cutaneous receptors. Group IV fibers are numerous adapted electronic devices and a head con-
small diameter afferents, originate as free nerve endings, troller to operate an electric wheelchair, but the presence
and serve nociceptive and thermoregulatory functions. of spasticity can alter the sitting position so that control
Both types of fibers convey impulses generated by extreme over the adaptive devices is lost.
pressure, heat, and cold. The reflex responses to these Several other factors may contribute to functional
stimuli are predominantly bilateral flexion. The afferent limitations. Spasticity can be caused or exacerbated by
fibers that produce generalized reflexive flexor move- pain. Generally, the presence of pain is a significant nega-
ments have become known collectively as flexor reflex tive contributor to the quality of life. The time and energy
afferents (FRA). The size of reflex response depends on required to complete a task may change more with spas-
the intensity of the stimulus. However, the response to ticity treatment than the actual ability or inability to com-
cutaneous stimuli is not always one of generalized flex- plete the task. For example, severely spastic lower extrem-
ion as, for example, in the pattern of ipsilataeral flexion ities may make dressing slow and difficult; whereas,
accompanied by contralateral extension with activation antispasticity treament may reduce the time required for
of group II and III fibers. Different modalities of stimuli the task. Increased age, degenerative joint disease, muscle
can have different effects. For example, after certain neu- and skin atrophy, and chronic anemia contribute to a gen-
rologic lesions, pressure applied to the plantar surface of eralized decline in health and reduced function, which can
the foot produces a marked extension of the leg, known diminish the ability to cope with spasticity.
as extensor thrust. In contrast, a pinprick in the same area It is important to assess the relative contributions
leads to flexion withdrawal of the limb. The spinal cir- of muscle hypertonicity and paresis to the patients
MANAGEMENT OF SPASTICITY 465
deficits, because treatment should be directed toward that relationships of central antispasticity drug actions to their
component of the upper motor neuron syndrome that is putative receptor substrates.
most disabling. Although spastic patients are reported to
have disturbances of gait speed, timing, kinematics, and
Gamma-Aminobutyric Acid (GABA)
EMG patterns, the relative impact of spasticity on gait
remains speculative (3336). For example, knee exten- The main inhibitory neurotransmitters in the CNS are
sor muscle torque correlates with the speed of comfort- gamma-aminobutyric acid (GABA) and glycine. In the
able walking, but accounts for only 30 percent of the 1940s, Sir John Eccles characterized the physiologic
variance in gait speed in spastic stroke patients (37). action of GABA on the Ia mediated spinal reflex by presy-
Young concluded that not all abnormalities underlying naptic inhibition. GABA-containing cells are typically
spastic gait are caused by spasticity and, thus, are not small interneurons. A common experimental paradigm
affected by antispasticity drug treatment (38). for eliminating these small interneuron GABA cells, while
leaving long tracts intact, is to apply a localized ischemia.
Conversely, lesions of long tracts, such as a spinal tran-
PHYSICAL MODALITIES section, will not diminish the number of GABA interneu-
rons below the level of transection. Once GABA is
Although reviewed elsewhere in this text, the use of phys- released by GABAergic interneurons, free GABA is
ical modalities in the management of spasticity is an released to bind to receptors on the postsynaptic mem-
important element. Spasticity can be reduced for variable brane. The classical GABA receptor has been charac-
durations by passive range-of-motion exercise (3941); terized as having a number of cell membrane protein sub-
tendon pressure (42); application of cold, warmth, or unitsalpha, beta, and gamma. Once the receptor is
vibration; splinting; bandaging; massage; low-power activated by GABA binding, the chloride ionophore chan-
laser; and acupuncture (43). Electrical stimulation of the nel is stimulated, resulting in membrane hyperpolariza-
spinal cord has been reported to result in a reduction of tion. In the situation where an axo-axonal connection
spasticity (44,45), although the measurement of spastic- exists between a GABAergic interneuron and the termi-
ity in these studies has been questioned (46). Several inves- nal of an Ia afferent, then hyperpolarization of that mem-
tigators have shown that electrical stimulation of the legs, brane will result in a decrease in excitability, decreased
hands, or perineal nerves may decrease spasticity in excitatory transmitter release, and subsequently, reduced
patients with SCI, stroke, or traumatic brain injury motor neuron firing. Thus, presynaptic inhibition of the
(4754). Other physical modalities that have been afferent neuronal terminal reduces motor neuron output
reported to ameliorate spasticity include magnetic stim- without direct inhibition of motor neuron excitability.
ulation over the thoracic spinal cord (55) and topical Because GABA does not cross the bloodbrain barrier, it
application of 20 percent benzocaine (56). is useful as an oral antispasticity agent.
Pharmacologic treatment is often required in the man- BACLOFEN (LIORESAL). Baclofen [4-amino-3 (4-
agement of spasticity. The specific pharmacologic effects chlorophenyl) butanoic acid] is a structurally similar to
may be directed toward an alteration of transmitters or GABA. Baclofen binds to and activates bicuculline-
neuromodulators by the suppression of excitation (glu- insensitive GABA receptors, termed GABA B receptors.
tamate); enhancement of inhibition (GABA, glycine); a GABA B receptors occur both pre- and postsynaptically.
combination of both (noradrenaline, serotonin, adeno- Upon binding with the presynaptic terminal, the influx
sine, and various neuropeptides); or action on periph- of calcium is restricted, membrane hyperpolarization
eral neuromuscular sites. Numerous substances, sum- occurs and endogenous transmitter release is reduced
marized in Table 34.2, have antispasticity effects. (57,58). Postsynaptic binding, on the Ia sensory afferent
However, only three prescription pharmaceuticals have terminals, increases potassium conductance and causes
been approved by the U.S. Food and Drug Administra- membrane hyperpolarization. With reduced excitability of
tion for the treatment of spasticity related to CNS disor- the primary afferent terminal, presynaptic inhibition is
der. These are baclofen, diazepam, and dantrolene considered to be enhanced, thus resulting in a decrease in
sodium. Tizanidine is an alpha-2 selective noradrenergic excitatory neurotransmitter release (see Figure 34.1).
agonist, new in the U.S., for the treatment of spasticity Baclofen activation of GABA B receptors causes inhibition
related to SCI or MS. Figure 34.1 illustrates the presy- of gamma motor neuron activity, reduced drive to
naptic inhibition of excitatory transmitter release from intrafusal muscle fibers, and reduced muscle spindle
a hypothetical sensory afferent nerve terminal and the sensitivity (59). The net effect of baclofen is to produce an
466 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
TABLE 34.2
Drugs Reported to have Antispasticity Properties
EFFECTS ON GABA
Dantrolene Dantrium Hydantoin derivative, inhibits calcium release into the sodium sarcoplasmic
reticulum.
Lamotrigine Lamictal Phenyltriazine, blocks voltage-sensitive sodium channels, preventing EAA
release.
Riluzole Rilutec Blocks voltage-sensitive sodium channels, preventing EAA release
4-aminopyridine Neurolan Blocks postspike potassium reuptake
EFFECTS ON MONOAMINES
FIGURE 34.1
The putative sites of actions for various antispasticity medications, are illustrated for a hypothetical cell with numerous recep-
tors (classical GABA-chloride ionophore shown with subunits, high- and low-affinity benzodiazepine receptors shown as type
I and II, respectively), a GABAergic interneuron, and an effector cell with receptors for excitatory transmitters. Excitatory trans-
mitter function is reduced by a) presynaptic inhibition of release from sensory nerve terminals via GABAergic (type A and B),
noradrenergic (alpha-2), and voltage sensitive ion (sodium) channels; and b) excitatory transmitter blockade (illustrated within
the effector, postsynaptic cell). The GABA A agonists listed are not discussed in the text because of significant toxicity, lack of
availability or inadvisability.
inhibition of monosynaptic spinal reflexes and, to a lesser is wide intersubject variation in baclofen absorption and
extent, polysynaptic spinal reflexes. Numerous reports elimination. Baclofen dosing may be initiated with 5 mg
corroborate the clinical efficacy of oral baclofen in the three times per day and increased gradually to a thera-
treatment of spasticity related to MS or SCI (6067). peutic level. The recommended maximum dosage is 80
Baclofen is rapidly and extensively absorbed after mg per day in four divided doses; however, there are
oral administration. It is mainly excreted by the kidney in reports of improved therapeutic effects with higher
unchanged form; however, 15 percent is metabolized in dosages (68) and an indication that higher dose pre-
the liver. Therefore, liver function parameters should be scription is not uncommon (69). Because baclofen treat-
monitored periodically during baclofen treatment and the ment can produce sedation, patients should be cautioned
dosage should be reduced in patients with impaired renal regarding the operation of automobiles or other danger-
function. The therapeutic half-life ranges from 2 to 6 ous machinery and activities made hazardous by
hours, with a mean of approximately 3.5 hours, but there decreased alertness. Also, because baclofen is excreted by
468 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
the kidneys, patients with renal impairment may require The signs of diazepam intoxication are somnolence
a low dose. The effects of chronic baclofen treatment dur- progressing to coma. Although benzodiazepines are gen-
ing human pregnancy are largely unknown. In some erally regarded to have a wide margin of safety, the rate
patients, seizure control has been lost during treatment of fatal benzodiazepine poisoning in Britain over a decade
with baclofen and abrupt discontinuation of baclofen can was found to be 5.9 per million prescriptions (77). Near-
produce seizures, confusion, hallucinations, and rebound term infants born with benzodiazepine intoxication are
muscle spasticity with fever. at particular risk. A 22-year-old, whose first baby was at
Oral baclofen is a widely prescribed pharmaceutical 36 weeks of gestation, consumed 250 to 300 mg of
in North America, with few reports of major toxicity. How- diazepam. She was somnolent but responsive. The fetal
ever, massive overdose with oral baclofen has been heart rate showed decreased variability and absence of
reported, including a case report of a 57-year-old woman accelerations. The benzodiazepine antagonist, flumaze-
who ingested 2 grams of baclofen, which caused coma and nil, 0.3 mg was given to the mother intravenously. Within
hypoventilation. She was given naloxone, 50 percent dex- 5 minutes, behavioral arousal in the mother and
trose, and activated charcoal. Initially the blood pressure improved fetal heart rate variability were observed (78).
was low, then systolic hypertension was noted, followed 16 The symptoms of withdrawal from high-dose
hours later by bradycardia and hypotension. Pupils were diazepam ( 40 mg per day) are anxiety and agitation;
small and unresponsive and tendon reflexes were absent. restlessness; irritability; tremor; muscle fasciculation and
Plasma baclofen concentrations over time showed first- twitching; nausea; hypersensitivity to touch, taste, smell,
order elimination kinetics and a half-life of 8 hours (70). light, and sound; insomnia, and nightmares; seizures;
hyperpyrexia; psychosis; and possibly death. The inten-
sity of the symptoms, and hence the risk of death, is
Benzodiazepines
related to the prewithdrawal dose. Symptoms of with-
In general, the pharmacologic and antispasticity effects of drawal from low-dose benzodiazepine (< 40 mg per day)
benzodiazepines are mediated by a functionally coupled are more likely if the patient has taken the drug consis-
benzodiazepine-GABA receptor chloride ionophore com- tently for more than 8 months. Onset of symptoms occur
plex (7173). High-affinity and low affinity receptors exist, 1 to 2 days after a short-acting benzodiazepine is stopped
as well as long-acting and short-acting benzodiazepines. or 2 to 4 days for a long-acting benzodiazepine. Even
The relative length of action is related to the duration of when the benzodiazepines are withdrawn slowly over 4
activity and rate of metabolism of the parent compound to 6 weeks, withdrawal symptoms may persist for 6
as well as the production and elimination of pharmaco- months (79).
logically active metabolites. Diazepam, chlordiazepoxide,
and clonazepam are considered to be long-acting benzo- CLONAZEPAM (KLONOPIN, RIVOTRIL).
diazepines, whereas oxazepam, alprazolam, and Clonazepam is indicated for the suppression of
lorazepam are considered to be short-acting, without sig- myoclonic, akinetic, or petit mal seizure activity, alone
nificant production of active metabolites. Generally, ben- or as an adjunct. It is well absorbed after an oral dose,
zodiazepines cross the placental barrier and are secreted with maximum blood concentrations occurring in 1 to
into breast milk. The benzodiazepines are metabolized 2 hours. It is 98 percent bioavailible, with 1 percent
extensively, mostly by the microsomal enzymes of the liver. excreted in the urine; 86 percent is bound in plasma.
Clonazepam is a cytochrome P3A substrate. It has a 7-
DIAZEPAM (VALIUM). Diazepam is a benzodia- amino metabolite and a half-life of 18 to 28 hours.
zepine that suppresses behavioral arousal, agitation, or Clonazepam is used in spasticity mainly to suppress
anxiety. It reduces polysynaptic reflexes and has muscle spasms at night that disturb sleep. It is most commonly
relaxant, sedation, and antispasticity effects (7476). prescribed as 0.5 to 1 mg at night. For patients who find
Commonly, diazepam therapy is initiated with a bedtime morning sedation excessive, the tablet can be broken in
dose of 5 mg, increased to 10 mg as needed. Diazepam half and 0.25 mg taken at night.
is well absorbed after an oral dose. Peak blood level
occurs in 1 hour. It is metabolized to the active CLOBAZAM (FRISIUM). Clobazam is a 1,5-
compound N-desmethyldiazepam (nordazepam), then benzodiazepine with anticonvulsant properties that has
oxazepam. The half-life of diazepam (including active not been reported as a treatment for spasticity. It is likely
metabolites) is 20 to 80 hours, and it is 98 to 99 percent that the differences observed between clobazam and the
protein bound. The significance of the protein binding 1,4-benzodiazepines is the variation of agonist action at
is that in patients with low serum albumin, such as is the high-affinity benzodiazepine receptor. It is well
often the case in SCI and stroke patients, the incidence absorbed after an oral dose and reaches peak plasma
of undesirable sedation is increased. Daytime therapy is concentration in 1 to 4 hours. Clobazam is extensively
initiated with 2 mg and increased as needed. metabolized to a number of active metabolites, N-
MANAGEMENT OF SPASTICITY 469
desmethylclobazam being the most important. The half- pharmacologic effect is to reduce calcium flux across the
life of clobazam is 1030 hours and 3646 hours for N- sacroplasmic reticulum of skeletal muscle. This action
desmethylclobazam; both increase with the age of the uncouples motor nerve excitation and skeletal muscle
patient. Clobazam is available as a scored 10-mg tablet. contraction (80,81). The oral formulation is prepared as
The recommended daily anticonvulsant dose for adults is a hydrated sodium salt to enhance absorption
5 to 15 mg per day that can be given in divided doses or (approximately 70 percent), which occurs primarily in the
as a single nighttime dose. The recommended maximum small intestine. After a dose of 100 mg, the peak blood
dose is 80 mg, and it is recommended to periodically concentration of the free acid, dantrolene, occurs in 3 to
monitor liver function tests, as will all benzodiazepines. 6 hours and the active metabolite, 5-hydroxydantrolene,
occurs in 4 to 8 hours. Dantrolene sodium has been shown
MIDAZOLAM (VERSED). Typically used as an to produce a dose-dependent decrease in the stretch reflex
adjunct for anesthesia, midazolam is not currently used as (82) and percentage decrease of grip strength (83).
a treatment for spasticity because of its intravenous Dantrolene is lipophilic and crosses cell membranes well,
formulation and the profound state of amnesia produced achieving wide distribution and significant placental
by this benzodiazepine. Recently, however, it has been concentration in the pregnant patient. Liver metabolism
reported to be useful as a pretreatment for children by mixed function oxidase and cytochrome P450
receiving botulinum toxin injection. The midazolam produces a 5-hydroxylation of the hydantoin ring and
solution is mixed with a sweet tasting fluid and given orally. reduction of the nitro group to an amine, which is then
Because midazolam can cause significant impairment of acetylated. After oral administration, urinary elimination
oxygenation, oxygen saturation and vital signs should be of 15 to 25 percent of the unmetabolized drug is followed
constantly monitored. The available concentrations are 1 by urinary excretion of the metabolites. The median
and 5 mg/ml. Published pharmacokinetic data relates to elimination half-life is 15.5 hours after an oral dose, and
intramuscular or intravenous administration, which show 12.1 hours after an intravenous dose.
that midazolam and its active metabolite, 1-hydroxymethyl The majority of placebo-controlled clinical trials of
midazolam, have a short half-life of 1 to 2.8 hours each. dantrolene have shown a reduction of muscle tone, ten-
don reflexes, and clonus, and increased passive motion
CLORAZEPATE DIPOTASSIUM (TRANXENE). (84). Mixed conclusions have been drawn regarding the
Clorazepate is a pro-drug for desmethyldiazepam and is effects of dantrolene sodium on gross motor performance
rapidly decarboxylated to it in gastric juice. Like and strength. In comparative trials with spasticity of dif-
clobazam, it has not been reported to be a treatment for ferent etiologies, some have suggested that the best
spasticity. The half-life of clorazepate is 1 to 3 hours, but responders to dantrolene sodium are stroke patients,
the half-life for desmethyldiazepam is as long as 106 whereas others suggest that SCI patients improve most.
hours. It is available in 3.75 mg or 7.5 mg capsules. However, most investigators agree that patients with MS
do not generally benefit with dantrolene treatment (84).
VIGABATRIN (SABRIL). Vigabatrin, gamma-vinyl In four trials of children with CP, dantrolene sodium was
GABA (4-amino-hex-5-enoic acid), is a GABA analog that found to be superior to placebo. The degree of improve-
irreversibly inhibits the activity of GABA transaminase. ment appeared greater in children than adults. One study
GABA transaminase exists in neurons and glia to regulate found dantrolene to be superior to baclofen; another sug-
the intracellular balance of GABA, glutamate, and gested equal efficacy to diazepams. In addition to its anti-
intermediate substances in energy metabolism, such as spasticity effects, dantrolene has been used in the treat-
alpha-ketoglutarate. The inhibition of GABA transaminase ment of malignant hyperthermia and the neuroleptic
results in an elevation of GABA levels in the brain. malignant syndrome (81). In addition, dantrolene has
Vigabatrin is approved in Canada for the treatment of been reported to be useful in the treatment of hyperther-
drug-resistant seizures. Vigabatrin is well absorbed after mia following abrupt baclofen withdrawal (85,86).
an oral dose and the peak plasma concentrations occur At least thirteen clinical reports of overt hepatotox-
within 2 hours. It is widely distributed in the body and icity appear in the literature, five of which report hepa-
eliminated mainly by the kidneys. The plasma half-life is tonecrosis. The overall incidence of hepatotoxicity in a
5 to 8 hours in young adults and 12 to 13 hours in the large group of patients receiving dantrolene sodium for
elderly. The initial starting dose of vigabatrin is 500 mg more than 2 months is reported to be 1.8 percent. Symp-
twice per day and is often quite sedating. tomatic hepatitis occurred in 0.6 percent and fatal hepati-
tis in 0.3 percent, with the greatest risk in females, older
than 30 years of age, taking more than 300 mg/day for
Drugs Affecting Ion Flux
more than 60 days.
DANTROLENE SODIUM (DANTRIUM). Dantrolene The initiation of antispasticity treatment with
sodium is a hydantoin derivative whose primary dantrolene sodium should begin with 25 mg once daily,
470 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
increasing every 4 to 7 days, by 25 mg increments, to 100 ficial in treating spasticity of various etiologies (100).
mg four times per day. The dosage at which the antici- Tizanidine is an alpha-2 agonist like clonidine, but does
pated therapeutic response occurs with least side effects not induce a consistent change in blood pressure or pulse
should be the maintenance dose. as clonidine does (101). However, symptomatic hypoten-
sion has been reported when tizanidine is taken with an
LAMOTRIGINE (LAMICTAL). Lamotrigine is a antihypertensive drug; therefore, the concomitant admin-
phenyltriazine with anticonvulsant properties. It is istration of tizanidine and antihypertensive drugs should
thought to act on voltage-sensitive sodium channels to be avoided.
stabilize neuronal membranes and inhibit the release of Tizanidine is well absorbed after an oral dose, with
excitatory amino acid transmitters. Lamotrigine has extensive first-pass hepatic metabolism to inactive com-
shown analgesic action in animal models of acute and pounds that are subsequently eliminated in the urine.
chronic pain (87). Recently, clinical trials with Therefore, tizanidine should be used with caution in
lamotrigine have shown promise in the treatment of patients with known liver abnormality. Because the most
chronic pain states as well as in the treatment of spasticity common side effects reported during the clinical trials
and concomitant chronic central pain (88). Skin-related with tizanidine include dizziness and drowsiness, it is rec-
adverse events have been reported during lamotrigine ommended to begin tizanidine therapy with a single dose
treatment, particularly in patients taking valproic acid. of 2 to 4 mg at bed time. The titration of tizanidine should
be tailored to the patient, and the maintenance dosage is
RILUZOLE (RILUTEC). Riluzole is an approved the one at which the therapeutic goals have been met with
putative treatment for amyotrophic lateral sclerosis the least side effects. The scored tizanidine tablets contain
(ALS). It reportedly blocks the action of voltage-sensitive 4 mg. Dosage increases of 2 to 4 mg every two to four
sodium channels, thereby preventing the release of days are recommended; however, most clinicians experi-
excitatory aminoacids. It is associated with a reduction enced with tizanidine recommend a slow and gradual
in stiffness associated with ALS (89). upward titration, particularly for MS patients. The max-
imum recommended dosage is 36 mg per day.
Effects on Monoamines
CLONIDINE (CATAPRES, DIXIRIT). The mech-
anism by which clonidine produces any of its many
TIZANIDINE (ZANAFLEX). Tizanidine is an pharmacologic effects remains speculative; however, the
imidazoline derivative and, like the alpha-2 adrenergic most commonly described action by which clonidine
agonist clonidine, binds to both alpha-2-adrenergic and lowers blood pressure and heart rate appears to be an
imidazoline receptor sites both spinally and supraspinally alpha-2 mediated inhibition of locus coeruleus neurons,
(90,91). In the spinal transected cat, tizanidine has been thereby reducing the discharge rate of these neurons and
shown to decrease reflex activity, especially polysynaptic decreasing tonic facilitation on sympathetic preganglionic
reflex activity (92). This finding has been corroborated fibers; this causes the blood pressure to decrease (102,103).
by the observation that tizanidine has an antinociceptive Clonidine also has a spinal site of action that is alpha-2
effect in animal models (9395). Tizanidine and selective. Autoradiographic binding of H3-clonidine in the
clonidine are imidazoles with antispasticity properties dorsal horns of the spinal cord can be blocked by
that affect alpha-2 noradrenaline receptors (96,97) and yohimbine (104). Clonidine has been shown to produce
may restore or enhance noradrenergic presynaptic marked inhibition of the short-latency alpha-motoneuron
inhibition in spastic patients. Several European and response to group II muscle afferent stimulation in the
American studies have shown that tizanidine is safe and spinalized cat, perhaps by augmenting presynaptic
equal in effectiveness to baclofen and diazepam, but with inhibition (105). The antispasticity effect of clonidine
a favorable tolerability profile. One trial, in which treatment in SCI humans has been consistent with the
muscle strength was carefully tested in a variety of enhancement of alpha-2 mediated presynaptic inhibition
spastic patients, showed improvement during tizanidine of sensory afferents (97,106,107). Clonidine is 95 percent
treatment (98). bioavailible after an oral dose; 62 percent is excreted in the
Studies using tizanidine have shown the facilitation urine and the half-life is 5 to 19 hours. Because some
of vibratory inhibition of the H-reflex, associated with patients may be very sensitive to the effects of clonidine,
clinically useful antispasticity effects in patients (99) the initial dosage should be low. The 25-microgram
observations similar to those seen with clonidine (97). In formulation, available in Canada (Dixirit), can be taken
addition, the antispasticity effects of tizanidine are man- orally twice per day to begin treatment. The dose can be
ifest without inducing muscle weakness (98). Tizanidine increased every three days by one tablet per day, as a three-
has been tested in a number of clinical trials in Europe or four-times-per-day dosage regimen. Alternatively,
and has been found to be safe, well tolerated, and bene- clonidine is available in the U.S. as a transdermal patch
MANAGEMENT OF SPASTICITY 471
with two dosage formulations, 0.1 mg or 0.2 mg per day; acting muscle relaxant, whose antispasticity effects were
these have been reported to be a useful treatment for tested by the then newly described Ashworth scale in
spasticity (108,109). The patch is designed to deliver the 1964 (14). In that trial of twenty-four MS patients, one
indicated amount of clonidine daily for 7 days. Blood patient was much improved, sixteen improved, two
pressure and pulse rate should be monitored periodically slightly improved, and five showed no change. With an
during treatment, because bradycardia and hypotension average dose of 350 mg, four times per day, drowsiness
are common side effects of clonidine. Additional side was the main side effect.
effects are dry mouth, ankle edema, and depression.
Cannabinoid
CYPROHEPTADINE (PERIACTIN). Cyproheptadine
has been used safely for more than 20 years in the
treatment of itching associated with hives, vascular CANNABIS (CESAMET, MARINOL). Over 4,000
headache, and anorexia. It is a histamine and a serotonin years ago, the ancient Chinese used the leaves of the
antagonist. It has also been used in the treatment of Cannabis sativa plant for medicinal purposes. The main
repetitive spontaneous abortion. Cyproheptadine has active alkaloid is delta-9-tetrahydrocannabinol (THC),
been reported to decrease clonus in people with spasticity available as a prescription pharmaceutical, dronabinol.
caused by SCI or MS (110). In patients whose gait is Nabilone is the synthetic cannabinoid, marketed as
limited by clonus, cyproheptadine normalizes muscle Marinol or Cesamet. The clinical indications for
firing patterns and increases walking speed (111). In a dronabinol and nabilone are nausea related to
comparative clinical trial, cyproheptadine had anti- chemotherapy treatment. In 1842, OShaughenessy
spasticity efficacy similar to clonidine and baclofen in SCI reported the use of hemp extract to treat muscle spasms
patients (112). associated with tetanus (116). In 1890, Reynolds
Cyproheptadine is available in 4-mg tablets. Treat- described the toxic effects and therapeutic use of cannabis
ment should be initiated as 4 mg at bed time, increasing in the treatment of epilepsy, chorea, and nocturnal spasms
by a 4-mg dose every three to four days. The most com- (117). Anecdotal reports by patients with spasticity caused
monly effective and tolerable dose is 16 mg in divided by MS or SCI suggest that smoking marijuana has a
doses, such as 4 mg four times per day. The maximum muscle relaxing effect (118,119). In a trial of thirteen
recommended dose is 36 mg per day. patients with MS who failed to have adequate spasticity
control using baclofen, diazepam, or dantrolene sodium,
CHLORPROMAZINE (THORAZINE, LARGACTIL). five completed the trial. Dronabinol, after an oral dose is
Chlorpromazine, a well-known sedative/antipsychotic, 4- to 12-percent bioavailable, less than 1 percent is
depresses the monosynaptic reflex to a greater extent excreted via the urine and 95 percent of the drug is bound
than polysynaptic reflexes (113,114). In an open clinical to plasma proteins. The half-life is 20 to 44 hours. It is
trial, resting muscle tone decreased with chlorpromazine formulated as 2.5-mg, 5-mg, or 10-mg capsules. Nabilone
treatment. Adverse effects include sedation and the is formulated as a pulvule containing 1 mg, with a
potential risk of tardive dyskinesia. recommended dose of 1 to 2 mg twice per day.
tural abnormalities and reduce the need for splinting and dose of BTXA (Botox) for individual muscles, based on
bracing. Reduction of thigh adductor tone may improve practitioner experience.
perineal hygiene and ease of nursing care (120). Of course, In a double-blinded, placebo-controlled studies of
local therapies may be used in conjunction with other forms botulinum toxin in spasticity in a cohort of MS patients,
of antispasticity therapy, such as antispastic drugs given ten nonambulatory patients with spastic contraction of
orally or intrathecally. the thigh adductor muscles that interfered with sitting,
positioning, cleaning, or urethral catheterization were
PHENOL/ALCOHOL. Until recent years, phenol has evaluated (120). The results indicated a significant bene-
traditionally been the most widely used injectable agent fit of botulinum toxin versus placebo at 6 weeks in reduc-
in the treatment of spasticity (121). Phenol acts by tion of the spasticity score (mean, baseline to 6 weeks;
protein denaturation of nerve fibers, and in low botulinum toxin: 7.9 to 4.7; placebo 6.8 to 7.8), and
concentrations, has the properties of a reversible local hygiene score. No adverse effects attributable to botu-
anesthetic. Alcohol may also be injected locally, with its linum toxin therapy were noted. Dykstra et al. reported
mechanism of action thought to be a dehydration effect a reduction of detrusor-sphincter dyssynergia in seven out
on nerve tissue, resulting in sclerosis of nerve fibers and of eleven SCI men using EMG-guided botulinum toxin
myelin sheath destruction. These agents may be injected (20 to 80 units) injections through the perineum into the
at motor points, identified by electrical stimulation, or
directly into nerve. The complications of phenol or
alcohol injection include pain, dysesthesias that may last TABLE 34.3
from weeks to months, arrhythmias, variable duration of Suggested Botox Doses for
effect, and incomplete irreversibility. Subarachnoid block Spastic Limb Muscles in Adults
has been performed with intrathecal phenol or alcohol,
although this therapy may result in severe weakness and DOSE # OF
sphincter dysfunction (122). MUSCLE UNITS/VISIT SITES
rhabdosphincter (124). The only adverse effects noted lowing successive days until a therapeutic response is
were transient hematuria following injection, which observed. The initial 24-hour dose can be estimated by
resolved within 24 hours. A large multicentered study of two methods. To estimate the 24-hour dose one can either
botulinum toxin in CP recruited children from 2 to 8 simply double the threshold dose or consider the duration
years of age with spasticity limiting ankle dorsiflexion of the antispasticity effect. For example, if the antispas-
(125). Botulinum toxin at a total dose of 4 units /kg was ticity effect of the test injection persisted for 8 hours, that
injected into one or both gastrocnemius muscles. The is 1/3 of a 24-hour period. The estimated 24-hour dose
results indicated significant improvement of physician rat- in this example would be 3 times the threshold dose or,
ing scale scores in the BTXA-treated group, with partic- in general, the 24-hour dose is the product of the thresh-
ular benefits in ankle position and gait. old dose and the inverse of the therapeutic response dura-
Several open-label studies reported benefits of bot- tion proportion of 24 hours.
ulinum toxin injections in the management of spasticity
in stroke patients (126129). Simpson et al. reported a INTRATHECAL MORPHINE (INFUMORPH). The
multicentered randomized, double-blind, placebo-con- direct infusion of morphine into the epidural or intrathecal
trolled study of botulinum toxin in post-stroke upper space has been reported to be a highly effective
extremity spasticity (130). The results indicated that the antispasticity as well as analgesia treatment (135). Also,
group receiving high dose BTXA (300 units total) expe- intrathecal midazolam (Versed) has been reported to be
rienced a significant reduction in the elbow and wrist effective but limited by sedation (136). In a series of four
flexor tone, as well as an improvement in physician and patients with the combination of severe pain and
patient global assessment scales. However, the functional spasticity, both conditions were controlled for up to 8
measures (Fugl-Meyer, Rand 36-Item Health Survey 1.0, months with daily dosages of 2 to 4 mg. However,
Functional Independence Measure, Function Status tolerance, pruritis, nausea, hypotension, urinary retention,
Index, pain assessment, and caregiver dependency scales) and respiratory depression can occur with an intrathecal
did not improve. Further studies of BTXA in the treat- morphine bolus dose of a little as 0.4 mg (137,138).
ment of spasticity are in progress.
Surgical Interventions
Intrathecal Infusion
Generally, the two surgical subspecialties involved in the
treatment of spasticity are orthopedic and neurosurgery.
INTRATHECAL BACLOFEN (LIORESAL). The The goals of orthopedic surgery are to increase mobility,
intrathecal route of baclofen administration results in a decrease the use of external aids, correct or prevent defor-
marked increase in potency compared to the oral mity, and ultimately maximize function (139). The tech-
treatment. The intrathecal dose is approximately 1/100th niques used by the orthopedic surgeons include tendon
of the oral dose (131). The development of reliable lengthening, muscle/tendon transfer (i.e., the split ante-
implantable drug delivery systems has permitted baclofen rior tibial transfer, SPLATT); contracture release, capsu-
to be administered by chronic slow infusion into the lotomy, osteotomy, resection arthroplasty, arthrodesis,
intrathecal space. Several studies have demonstrated the epiphyseodesis, ankle fusion, and spine fusion (139,140).
effectiveness of intrathecal baclofen for the treatment of The techniques used by the neurosurgeons involve the
spasticity (7,8,131134). The utilization of this highly interruption of the spinal reflex arc by neurectomy, neu-
effective antispasticity treatment is limited by the invasive rotomy, rhizotomy, selective rhizotomy, dorsal root entry
nature of the treatment and the cost of the implantable zone lesion, and myelotomy (Bischoff or T-shaped). Addi-
devices. However, for patients in whom severe spasticity tional techniques include cordotomy, cordectomy, and the
is the cause for hospitalization, intrathecal baclofen can implantation of stimulators or drug infusion devices. The
be cost effective by reducing the need for inpatient ideal neurosurgical procedure would be low in cost,
hospitalization or 24-hour nursing care (7,8). extremely safe, require a small operation with complete
The pre-implantation evaluation method involves relief of spasticity, yet produce total preservation of vol-
the determination of whether intrathecal baclofen will untary movement, sensation, sphincter, and sexual func-
be effective and, if so, at what dose. A test intrathecal tion. Unfortunately, a surgical therapy that could achieve
injection by lumbar puncture can be done and a starting this optimal outcome does not exist currently (141).
dose of 25 g given by slow injection. The patients mus-
cle tone should be evaluated hourly for 4 to 6 hours. A
therapeutic effect is deemed to have occurred when the CONCLUSIONS
Ashworth score of a selected muscle group decreases by
one grade. If a therapeutic response is not obtained, then The most often asked question by patients who receive
trials with 50, 75, or 100 g could be conducted on fol- medical treatment for spasticity is, Once I start taking pills,
474 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
will I have to take pills for the rest of my life? Spasticity, 13. Cole B, Finch E, Gowland C, Mayo N (eds.), Physical Rehabili-
tation Outcome Measures. Baltimore: Williams & Wilkins, 1995.
like the underlying neuropathology it represents, is in a con- 14. Ashworth B. Preliminary trial of carisoprodol in multiple scle-
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be thoroughly assessed, the goal of treatment should be 15. Lee KC, Carson L, Kinnin E, Patterson V. The Ashworth scale:
A reliable and reproducible method of measuring spasticity. J
determined by agreement between the practitioner and Neuro Rehab 1989; 3:205209.
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should include the avoidance of spasticity-aggravating fac- ment. Physiotherapy 1976; 62:358361.
17. Snow BJ, Tsui JKC, Bhart MH, Varelas M, Hashimoto SA, Calne
tors and frequent range-of-motion exercise (142). Patients DB. Treatment of spasticity with botulinum toxin: A double blind
receiving antispasticity medication should be reviewed peri- study. Ann Neurol 1990; 28:512515.
odically to assess response to treatment, side effects and 18. Penn RD, Savoy SM, Corcos D, Latash M, Gotlieb G, et al.
Intrathecal baclofen for severe spinal spasticity. N Engl J Med
adverse effects, to monitor the optimal use of mobility aids, 1989; 320:15171554.
wheelchair seating (if applicable), and to monitor the 19. Halstead LS, Seager SWJ, Houston JM, Whitesell K, Dennis M,
accompanying neurologic impairment. Patients who are Nance PW. Relief of spasticity in SCI men and women using rec-
tal probe electrostimulation. Paraplegia 1993; 31:715721.
surgical candidates require a thorough initial investigation 20. Nance PW, Bugaresti J, Shellenberger K, Sheremata W, Martinez-
in which it is established that skeletal growth and postop- Arizala A, et al. Efficacy and safety of tizanidine in the treatment
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44(Suppl 9):S4452.
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Acknowledgments Foundation Activities, Inc. 1993.
25. Environmental Status Scale, Question 4, Minimal Record of Dis-
The author acknowledges, gratefully, the assistance of the fol- ability for Multiple Sclerosis. National Multiple Sclerosis Society,
lowing individuals: Dr. David Simpson, Dr. Kimberly Safman, 1985; 44.
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476 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
utonomic dysfunction is a universal ANS also involve: a) supraspinal controlling and inte-
FIGURE 35.1
Autonomic nervous system of the head and neck. EWN nucleus EdingerWestphal; SSN superior salivatory nucleus; ISN
inferior salivatory nucleus and the dorsal vagal complex of the medulla; DMN X dorsal motor nucleus of the vagus nerve;
CT chorda tympani; III oculomotor nerve; VII facial nerve; IX glossopharyngeal nerve; X vagus nerve; SCN short
ciliary nerve; GSPN greater superficial petrosal nerve; GN glossopharyngeal nerve; LSPN lesser superficial petrosal nerve;
NA nucleus ambiguus; G ganglion; T thoracic.
from the pterygopalatine ganglion join the maxillary parasympathetic efferent axons in the vagus nerve
nerve and innervate the lacrimal glands, blood vessels of synapse with postganglionic parasympathetic neurons in
the palate, nasopharynx, and sinuses. Other preganglionic ganglia on or near the organs they innervate (heart,
axons in the facial nerve travel in the chorda tympani and bronchi, esophagus, stomach, pancreas, kidney, and intes-
join the lingual nerve to synapse in the submandibular tine to the splenic flexure of the colon).
ganglion and innervate submandibular and sublingual Parasympathetic preganglionic neurons are also
glands. found in the intermediolateral (IML) cell column of the
Preganglionic axons in the glossopharyngeal nerve sacral spinal cord in segments S2S4 and exit the central
branch at the level of the jugular foramen and course to nervous system (CNS) via the sacral ventral roots and the
the lesser superficial petrosal nerve, which exits the fora- spinal nerves and then continue to the pelvic viscera as
men ovale with the third division of the trigeminal nerve the pelvic nerve. The sacral preganglionic parasympa-
to synapse on postganglionic neurons in the otic ganglia. thetic efferent axons of the pelvic nerve synapse with post-
Postganglionic axons from the otic ganglia form the ganglionic parasympathetic neurons in the ganglia of the
auriculotemporal nerve and innervate the parotid gland. pelvic plexus. Postganglionic axons innervate the
The dorsal vagal complex consists of the nucleus descending colon, rectum, urinary bladder and sexual
ambiguus and the dorsal motor nucleus. Preganglionic organs (1).
AUTONOMIC DYSFUNCTION IN SPINAL CORD DISEASE 479
The sympathetic preganglionic component of the the pons and medulla to interneurons in the spinal cord
ANS is purely spinal. Sympathetic preganglionic neurons influence the IML cells (1). The NTS receives afferents
(SPNs) are found in the IML cell column of the thoracic from the viscera and functions as an integrating center for
and lumbar spinal cord in segments T1L2 (1) and exit reflex activity as well as a relay station to the hypothala-
the CNS via the thoracolumbar ventral roots. The sym- mus and limbic systems.
pathetic segmental outflow can vary if the brachial plexus
is prefixed or postfixed so that the outflow can start as
high as C8 or as low as T2 and end at L1 or L3 (1). The SPINAL CORD REFLEX
thinly myelinated preganglionic fibers exit via the ven- ORGANIZATION OF THE ANS
tral roots as the white rami communicantes. Many sym-
pathetic preganglionic fibers synapse in the paravertebral The SNS and the parasympathetic nervous system (PNS)
ganglia, which are paired and lie next to the spine from regulate visceral function largely through autonomic
the cervical to the sacral segments. There are three cervi- reflexes (1). Experimentally, a variety of distinct auto-
cal paravertebral ganglia: the superior cervical ganglion, nomic reflex pathways can be elicited by electrical stim-
the middle cervical ganglion, and the stellate ganglion. ulation of afferent nerves. These reflexes are character-
There are usually eleven thoracic ganglia, four lumbar ized by their afferent spinal input and the autonomic
ganglia, and four or five sacral ganglia (1). At the level efferent pathway function (e.g., smooth muscle contrac-
of the coccyx, the two sympathetic ganglia chains join in tion). Some of these reflexes are relayed at the level of
the single ganglion impar (1). Sympathetic preganglionic the spinal cord, whereas others require a relay in
axons can synapse in paravertebral ganglia at the segment supraspinal structures. The relay in the CNS can be mod-
of their exit or can pass up or down several segments of ulated by a wide range of afferent inputs as well as by
the sympathetic chain before synapsing. One sympathetic descending inputs, such as those from the hypothalamus.
preganglionic axon will synapse with several postgan- The details of the integration of autonomic spinal reflex
glionic neurons. Postganglionic axons are unmyelinated pathways with descending supraspinal systems are largely
and leave the paravertebral ganglia via the gray rami com- unknown (3).
municantes and exit via the segmental spinal nerves. The control circuits in the spinal cord consist of pre-
Some sympathetic preganglionic axons pass through ganglionic neurons; spinal interneurons, both segmental
the paravertebral ganglia without synapsing and consti- and propriospinal (i.e., projecting over many spinal seg-
tute the splanchnic nerves that innervate three preverte- ments); and their synaptic connections (3). The various
bral ganglia: a) celiac ganglion, b) superior mesenteric autonomic control circuits in the spinal cord interact with
ganglion and c) inferior mesenteric ganglion (IMG), as afferent spinal input from the periphery and descending
well as the adrenal medulla. Sympathetic nervous system spinal pathways. Although the synaptic connections of
(SNS) stimulation of the adrenal medulla results in the autonomic interneurons are largely unknown, it is likely
release of norepinephrine (NE) and epinephrine (E). NE that circuits similar to those identified in the somatomotor
excites alpha-receptors, with only slight excitation to system (Renshaw interneurons, 1a interneurons and 1b
beta-receptors. Epinephrine excites both alpha- and beta- interneurons) also exist in the spinal autonomic systems (3).
receptors (1). Postsynaptic axons from the prevertebral Central regulation of autonomic function occurs
ganglia course to the abdominal and pelvic viscera as the chiefly in the hypothalamus, brainstem, and spinal cord.
hypogastric, splanchnic, and mesenteric plexuses. Unlike the relay of the central message to the target organ
Sweat glands, piloerector muscles, and most small in the somatic motor system, the autonomic target can be
blood vessels receive only sympathetic innervation. Dif- innervated by more than one final common autonomic
fuse sympathetic nervous system discharge results in pathway. In addition, the central autonomic message may
pupillary dilatation, increased heart rate and contractil- be changed quantitatively in the autonomic ganglia or at
ity, bronchodilation, vasoconstriction of the mesenteric the neuroeffector junction by neural, hormonal, or other
circulation, and vasodilation of skeletal muscle arterioles. processes (3). Following interruption of the spinal cord,
This is the fight or flight defense reaction. there is residual autonomic function of the isolated spinal
Supraspinal neurons involved in the control systems cord associated with thermoregulation, evacuation of the
of the ANS are located in the nucleus of the tractus soli- urinary bladder and colon, regulation of sexual function,
tarius (NTS), nucleus ambiguus, dorsal motor nucleus of regulation of blood pressure, and other reflexes.
vagus, dorsal raphe nucleus, medullary reticular forma-
tion nuclei, locus ceruleus, hypothalamus, limbic system,
and the primary sensory and motor cortex (1). The hypo- AUTONOMIC PHARMACOLOGY
thalamus has uncrossed sympathetic descending path-
ways to the midbrain, lateral pons, and medullary retic- Acetylcholine (Ach) is the neurotransmitter for all pre-
ular formation. Descending reticulospinal pathways from ganglionic axons, both parasympathetic and sympathetic.
480 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
Ach is also the neurotransmitter for all postganglionic systems (7). Supraspinal vasomotor centers generate the
parasympathetic axons. NE is the neurotransmitter resting tone of sympathetic nerve discharges. Reflex activ-
released by most sympathetic postganglionic axons, ity, central respiratory modulation, and brainstem and
except those that innervate sweat glands that release Ach. spinal neural circuits all may generate the periodic dis-
Acetylcholine receptors are of two types: muscarinic charges of sympathetic nerves (7). The descending input to
and nicotinic. Preganglionic axons synapse on nicotinic sympathetic preganglionic neurons involved in regulating
receptors and postganglionic axons synapse on mus- resistance vessels is predominantly sympathoexcitatory,
carinic receptors. There are five identified subtypes of from the glutamatergic neurons of the rostroventrolateral
muscarinic receptors, M1 through M5. Muscarinic recep- reticular nucleus (RVL). There are also sympathoinhibitory
tors are involved in peripheral and central cholinergic descending inputs from GABAergic, glycinergic, and
responses. Information on the subtype selectivity of mus- adrenergic neurons onto SPNs, although it is thought that
carinic agonists and antagonists, as well the pattern of inhibiting excitatory inputs, such as those from the RVL,
expression of muscarinic subtypes in the many tissues of largely produces SPN inhibition (7).
the body, is incomplete (4). Preganglionic sympathetic neuron projection to the
NE receptors are divided into alpha () and beta () heart are located in spinal cord segments T1 to T4,
adrenoceptors (AR). Although there are many exceptions, whereas the preganglionic neurons for the peripheral vas-
-AR are excitatory and -AR are inhibitory. -AR are culature are found mainly in spinal cord segments T1 to
divided into 1-AR and 2-AR, based on the relative L2 (8). A small proportion of the neurons in the IML col-
potency of certain alpha agonists and antagonists (5). 1- umn are interneurons. The cholinergic SPNs innervate the
AR are further divided into 1A-, 1B-, and 1D-AR. 2-AR adrenal medulla as well as noradrenergic neurons in sym-
are further divided into 2A/D-, 2B-, and 2C-AR. 1-AR pathetic ganglia, which in turn innervate blood vessels and
are coupled to a G protein to stimulate phospholipase C, the heart. The heart is also innervated by three branches
which promotes the hydrolysis of phosphatidylinositol bis- of the vagus nerve (superior cervical cardiac, inferior cer-
phosphate, thus producing inositol trisphosphate and dia- vical cardiac, thoracic cardiac). Preganglionic efferent
cyldlycerol, which are second messengers that mediate parasympathetic axons in these branches form synapses
intracellular Ca2+ release and smooth muscle contraction with neurons in the ganglia of the cardiac plexus.
(5). 1-AR are mostly postsynaptic and excitatory, and 2- The adrenoceptors that are innervated by the SNS
AR are usually presynaptic and inhibitory. Presynaptic a2- and contribute to basal vascular tone are 1-AR in arter-
AR inhibit the release of NE from sympathetic axons and ies, 1- and 1-AR in the heart, and 2- and 2-AR in
result in sympathetic inhibition. veins (5). 1-ARs are the most important in the mainte-
-AR are divided into 1-, 2 -, and 3-AR. All - nance of vascular tone by the SNS. Although the 1-AR
AR subtypes signal by coupling to a stimulatory G pro- subtype mediating vasoconstriction in humans is unclear,
tein, thus leading to activation of adenylyl cyclase and the in rats 1A-ARs are prominent and 1B-ARs and 1D-ARs
accumulation of the second messenger, cAMP (5). 2-ARs are involved in the responses to exogenous agonists (5).
that do not respond to NE can be considered as hormone Afferent fibers from the heart project via either the
receptors for circulating E from the adrenal medulla and sympathetic cardiac nerves or the vagus nerve (9).
are not functionally innervated by sympathetic nerves (5). Parasympathetic afferent fibers from sensory receptors in
Peptides coexist in individual neurons, along with the heart are conveyed via the cardiac branches of the
the classical Ach and NE transmitters. Dozens of neu- parasympathetic vagus nerve, have cell bodies in the jugu-
ropeptides have been identified, and one of their functions lar and nodose ganglia, and project centrally to the NTS.
is to augment the action of the classical transmitter. There is also animal evidence that a portion of vagal affer-
ent neurons in the nodose ganglion project to the upper
cervical spinal cord via a supraspinal pathway not involv-
CARDIOVASCULAR DYSFUNCTION IN SCI ing dorsal root ganglia (10). The sympathetic afferents
course in the inferior cervical and thoracic sympathetic
In high SCI, the SNS is decentralized, and the isolated cardiac nerves, then via white rami communicantes to cell
spinal cord is no longer under control from supraspinal bodies in the dorsal root ganglia (T1 to T5). The tradi-
structures. The cardiovascular results of SNS decentral- tional view is that afferent sympathetic cardiac fibers
ization range from the potentially life-threatening auto- mediate cardiac nociception, and afferent vagal cardiac
nomic dysreflexia, reflex bradycardia, and cardiac arrest fibers mediate cardiovascular reflexes (9). However, vagal
to a low resting blood pressure, orthostatic hypotension, afferent fibers may have some role in the mediation of car-
limited cardiovascular responses to exercise, and changes diac nociception, such as pain referred to the head and
in the skin microcirculation(6). neck (perhaps via afferent projections to the cervical
Arterial pressure is regulated by a rapid neuronal spinal cord). Afferent sympathetic fibers may also trans-
mechanism as well as slower hormonal and renal control mit some cardiovascular reflexes.
AUTONOMIC DYSFUNCTION IN SPINAL CORD DISEASE 481
The arterial baroreflex is the most important mech- interesting to speculate that the increased blood pressure
anism for the CNS regulation of arterial pressure (7). The response to hypercapnia in quadriplegic subjects may
arterial or high-threshold baroreceptors are located in the have resulted from AVP release.
carotid sinus and walls of the aortic arch. The cardiac or
low-threshold baroreceptors are located in the atria and
ventricles. A decrease in arterial blood pressure inhibits HYPOTENSION
firing and a rise in blood pressure increases firing in affer-
ent arterial baroreceptor fibers that project through the After a high spinal cord transection, systemic arterial pres-
vagus and glossopharyngeal nerves, with cell bodies in the sure drops (18), owing to a drop in cardiac output and
petrosal and nodose ganglia, to terminate in the NTS of in total peripheral resistance. Resistances of the muscle
the medulla (7). Baroreceptor-mediated sympathoinhibi- and visceral vascular beds are decreased equally (19). Ani-
tion is predominantly a brainstem reflex mediated via mal studies have found a reduction in sympathetic nerve
ventral medullary neurons (11). Evidence also exists that activity following acute SCI, with the exception of the
baroreceptor inhibition of sympathetic activity can be mesenteric and splenic nerves. However, the firing pattern
exerted at the spinal cord level, suggesting that some of sympathetic nerves following acute SCI in animals
fibers conveying baroceptor afferent information course changed from a rhythmic synchronized discharge to a less
directly to the spinal cord (12). synchronized one (23,26,27). It has been postulated that
Peripheral chemoreceptors that sense a fall in oxy- the less-synchronized firing pattern of sympathetic nerve
gen or a rise in carbon dioxide are located in the carotid activity that remains following SCI in experimental ani-
bodies and the aorta and have afferent fibers in the glos- mals may not support vascular tone adequately, regard-
sopharyngeal and vagus nerves. The brain also has less of the magnitude of SPN discharge (23,24,26,27).
chemoreceptors that sense carbon dioxide. Peripheral With no connection between the medullary barore-
chemoafferents terminate in the NTS and relay sympa- ceptor systems and the spinal cord, sympathetic activity
thoexcitatory responses via the RVL medulla (RVLM) to cannot be increased to compensate for postural changes.
spinal vasomotor neurons. In spinal animals, the In humans, orthostatic hypotension occurs after acute SCI
chemoreflex may also stimulate the release of arginie (28), but after a time the ability to maintain blood pres-
vasopressin (AVP) (also known as antidiuretic hormone sure in a sitting position improves (29). Improvement may
(13). Excitatory amino acid receptors in the rat RVLV be caused by the development of spinal reflex control of
appear to be involved in mediating the sympathoexcita- blood pressure or by long-term regulation by renal fluid
tory pressor response to carotid body chemoreceptor control (6). Alternatively, Bravo and coworkers suggest
stimulation. In addition, carotid body chemoreceptor that the decrement in arterial pressure and heart rate imme-
stimulation in rats results in a pressor response associated diately after SCI may result from acetylcholine release from
with an increase in AVP release; this enhancement of AVP parasympathetic fibers and the cholinergic stimulation of
release is also mediated by excitatory amino acid inputs nitric oxide (NO) release from endothelium (30).
in the RVL medulla (13). In contrast to intact subjects, quadriplegic subjects
In response to systemic hypoxia, perfusion of vital have a lower resting concentration of catecholamines
organs must be maintained. Hypoxia results in the acti- (3134) and no significant increase in either NE or E when
vation of peripheral chemoreceptors, and hypercapnia quadriplegic subjects change from a lying to a sitting posi-
results principally in the activation of central chemore- tion (31). This appears to be caused by a reduction in NE
ceptors (14). Chemoreceptor reflexes result in vasocon- release and not by a change in NE clearance (35). Resting
striction, principally in skeletal muscle vascular beds (15) skin blood flow is greater in quadriplegic than in control
via activation of the SNS. Research on the cardiovascu- subjects (31,36). This is in keeping with observations of
lar responses to hypercapnia in quadriplegic subjects is diminished resting vasoconstrictor tone and much lower
inconclusive. In one study of mechanically ventilated plasma NE levels. Submaximal and maximal exercise
quadriplegic subjects, no change in heart rate was results in increases in plasma NE in controls and paraplegic
detected in response to elevation of carbon dioxide (16). subjects, but not in quadriplegic subjects (32).
In another study, six quadriplegic subjects with complete Animal studies have been unable to attribute the
lesions above the sympathetic outflow responded to normalization of arterial pressure following spinal cord
breathing carbon dioxide by increased blood pressure, transection to either increased vascular sensitivity to a
both in the horizontal and the tilted positions, which sug- constantly low level of sympathetic activity or a steadily
gests either a peripheral or spinal cord effect of hyper- increasing level of sympathetic nerve discharge (37). The
capnia (17). Because chemoreceptor stimulation in spinal enhanced pressor response to NE in humans with cervi-
animals has been shown to produce a pressor response cal SCI reported by Mathias and colleagues (38) may not
that seems to be mediated via excitatory amino acids in have been caused by denervation sensitivity, as suggested,
the RVL medulla and increases in AVP release (13), it is but may have resulted from the absence of baroreceptor-
482 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
mediated sympathoinhibition (37). However, Arnold and renin release by the juxtaglomerular cells in response to
coworkers found a left shift of the doseresponse curve the decreased renal perfusion that accompanies low arte-
for local infusions of NE to reduce dorsal foot vein diam- rial pressures (44). Renin acts on angiotensinogen in the
eter, suggesting that -AR responsiveness is increased in plasma, forming angiotensin, which is converted to
quadriplegic persons (39). In animals, cooling enhances angiotensin II, a major vasoconstricting hormone.
the NE-induced contraction of saphenous vein smooth Increased release of aldosterone is probably a direct effect
muscle mediated via 2-AR, but does not enhance those of the increased serum renin level (29).
contractions mediated via 1-AR (5). Temperature dif- The secretion of AVP by the posterior pituitary gland
ferences between control and quadriplegic subjects may is predominantly controlled by changes in plasma osmo-
account for the different responsiveness to infused NE lality, which are sensed by osmoreceptors in the hypo-
found in cutaneous veins. NE activates postjunctional 1- thalamus; and changes in blood volume and blood pres-
AR to contract the smooth muscle of major arteries; how- sure relayed from cardiovascular receptors in the carotid
ever 2-AR contributes to the vasoconstriction caused by sinus and thorax via the glossopharyngeal and vagal cra-
NE, particularly in cutaneous arteries and veins (5). nial nerves to the NTS, and thence to the paraventricu-
Hyperresponsiveness of 2-AR in cutaneous veins to lar and supraoptic nuclei of the hypothalamus (45). In
infused NE may not necessarily indicate that 1-AR in one study by Kilinc and coworkers, control subjects with-
arteries are similarly hyperresponsive. Because -AR in out SCI had a nocturnal increase in AVP level, whereas
arteries contributes most to systemic vascular tone, quadriplegic or paraplegic individuals did not (46). Quad-
hyperresponsiveness of 2-AR alone may not contribute riplegic or paraplegic individuals had an increased urine
to arterial pressure normalization following SCI. volume at night relative to controls. Kilinc hypothesized
Normalization of blood pressure following SCI may that daytime pooling of blood in paralyzed legs, with an
occur because autoregulatory controllers of blood flow associated reduction of central venous pressure, was fol-
completely dominate within 24 hours following spinal lowed by a redistribution of volume during recumbency
cord transection, with the result that any remaining sym- that resulted in increased blood pressure, which prevented
pathetic vasoconstrictor activity is without effect. nocturnal increases in AVP and thus nocturnal polyuria.
Hypotension following spinal cord transection may there- It has been suggested that SCI patients with severe noc-
fore be principally the result of a decrease in sympathetic turnal polyuria should have AVP levels checked and, if
activity to vascular beds with relatively weak autoregu- necessary, be treated with desmopressin acetate (DDVAP)
latory properties, such as skin and skeletal muscle (37). at bedtime (47).
The development of spasticity may contribute to the The infusion of hypertonic saline cause plasma AVP
recovery of arterial pressure owing to the increased cen- to rise in both control and quadriplegic subjects; however,
tral venous volume that results from enhanced venous at any given level of plasma osmolality, plasma AVP
return and to physical compression on the arterial side tended to be higher in the quadriplegic subjects than in
of the skeletal muscle beds, which thus increases vascu- the control subjects (43). Unlike control subjects, quad-
lar resistance (37). riplegic subjects demonstrated an increase in mean arte-
rial pressure (MAP) without an increase in heart rate as
a result of hypertonic saline infusion. In quadriplegic sub-
RENIN-ANGIOTENSIN SYSTEM AND AVP jects, water loading resulted in a normal suppression of
urine osmolality but subnormal free water clearance dur-
Animal studies have indicated that after lesions of RVL ing maximal water diuresis, despite appropriately sup-
medulla, arterial pressure is maintained by the renin- pressed levels of plasma AVP (43). Plasma AVP increased
angiotensin system and by AVP secretion (40). Studies in following head-up tilt in both control and quadriplegic
animals have also shown that arterial pressure following subjects, but the increase was significantly greater in the
spinal cord transection is related to the level of salt and quadriplegic group (45). These studies indicate that quad-
water intake (41) and in part dependent on angiotensin II riplegic persons have appropriate cardiovascular and
vasoconstrictor activity (37). Persons with chronic quad- osmotic control of AVP secretion, but increased sensitiv-
riplegia exhibit decreased urinary sodium excretion and ity to the pressor effect of AVP. The increase in postural
expansion of the extracellular fluid compartment (42). release of AVP may be responsible for the oliguria seen
The vasoconstrictor activity of the renin-angiotensin in persons with SCI after prolonged sitting (45).
system and AVP, as well as efferent renal sympathetic The infusion of AVP did not change MAP or heart
nerve activity (ERSNA), all may enhance renal retention rate in control subjects, but did result in a marked rise in
of sodium and water, which influences arterial pressure MAP and bradycardia in quadriplegic subjects. The
in the long term. Research findings show that quadri- bradycardic effect of AVP in quadriplegic subjects was
plegic persons have increased plasma renin levels and high probably the result of baroreflex activation of the intact
normal aldosterone levels (28,43), probably because of vagal efferents, secondary to the rise in MAP. The rea-
AUTONOMIC DYSFUNCTION IN SPINAL CORD DISEASE 483
son for the pressor response is not clear; it may have been or disinhibition of sympathetic systems. In intact animals,
caused by increased sensitivity to peripheral- or spinal- noxious cutaneous mechanical stimulation elicited
mediated pressor vascular effects of AVP or because increases in heart rate, blood pressure, ERSNA, and car-
baroreflex-mediated inhibition of sympathetic tone was diac sympathetic efferent nerve activity, while the same
interrupted by the spinal cord lesion (45,48) stimulus in acutely spinalized animals elicited even larger
In control subjects and in persons with SCI below increases in cardiovascular responses (60). This pro-
the L1 level, blood pressure rises with sitting and is lower priospinal somatocardiovascular excitatory reflex had a
when they are recumbent. In quadriplegic subjects, blood strong segmental localization and was under the influence
pressure is lower when sitting than when lying down (43). of tonic descending supraspinal inhibition.
This rise in blood pressure during recumbency has been In animals, spinal cord transection was shown to
attributed to fluid shifts into the central compartment and convert a previously sympathoinhibitory response to a
subsequent increased venous return and stroke volume. nonnoxious stimulus into a sympathoexcitatory one.
This expansion of central blood volume after recumbency, Chemo- and mechanoreceptors in the small intestine,
and the accompanying elevation in blood pressure inhibit- peripheral vasculature, or urinary bladder of spinal cord
ing the release of AVP, may explain the diuresis associated intact rats, activated by capsaicin, result in depression
with recumbency in quadriplegic persons (49). of cardiovascular SPNs (61). However, bladder disten-
sion or intravesical capsaicin [which activates afferent C
fibers (62)] in spinal cord-transected rats activated a
AUTONOMIC HYPERREFLEXIA reflex excitatory response conveyed by pelvic nerve affer-
ents that probably involved activation of SPNs via pro-
Autonomic hyperreflexia (AH) (also known as autonomic priospinal pathways (61). Altered reflex responses in
dysreflexia) is manifested by hypertension, sweating, SPNs may account for the massive autonomic hyper-
headache, and bradycardia (5053) and is most often reflexia displayed by quadriplegic persons, particularly
associated with SCI at or above the T6 level. This disor- responses to stimuli arising from the rectum and urinary
der is reported to affect 30 to 90 percent of quadriplegic bladder.
and high paraplegic persons (54). In individuals with SCI, In humans, the afferent pathway that mediates the
common sources of afferent stimulation that result in AH autonomic hyperreflexia in response to bladder disten-
include bladder distension, pressure sores, childbirth, and sion is not clear. Complete sacral dorsal rhizotomy
rectal distension (55). reduced, but did not eliminate, AH in response to
Somatic and visceral afferent input enters the spinal intradural sacral ventral root stimulation for bladder
cord below the level of the spinal cord lesion and projects emptying (63). However, dorsal rhizotomy alone may not
to the IML cell column via propriospinal pathways. Spinal eliminate all bladder afferents, because the existence of
sympathetic pathways linking the supraspinal cardiovas- ventral root afferents has been well established (64,65).
cular centers with the peripheral sympathetic outflow are It is also possible that the afferent pathway was via tho-
interrupted at the level of the injury, but the parasympa- racic and lumbar dorsal roots, and then via afferent fibers
thetic efferent pathways through the vagus nerve, as well in the hypogastric nerve.
as the afferent arc of the baroreceptor reflex through the Krassioukov and Weaver identified morphological
glossopharyngeal and vagus nerves, are intact after SCI. changes in SPNs following spinal cord transection in rats
Bradycardia results from activation of efferents in the (66). SPNs caudal to the spinal transection demonstrated
vagus nerve coursing to the sinoatrial node. Descending dendritic degeneration and a decrease in soma size within
sympathoinhibitory projections through the spinal cord, 1 week after transection, which was reversed by 1 month
which would normally result in vasodilatation, are dis- post transection. Krenz and Weaver found that the time
rupted by SCI, and the bradycardia alone is not adequate frame of degeneration and recovery of the dendritic arbor
to reduce blood pressure. Treatment requires that the elic- of SPNs correlated to the time frame for first reduced and
iting cause be eliminated or that pharmacologic treatment then enhanced vasomotor reflexes in the rat (67). The
be instituted. For reviews see Lee et al. (56); Naftchi et al. SPNs caudal to the SCI appeared to be innervated by
(57); Comarr and Eltorai (58); and Amzallag, (59). spinal interneurons after losing descending synptic input.
Several pathophysiologic mechanisms responsible In a mouse model of SCI, Jacob and coworkers described
for AH have been postulated and include: a) disinhibition the development of autonomic hyperreflexia in associa-
of sensory pathways; b) disinhibition of the sympathetic tion with the sprouting of calcitonin gene-related pep-
systems; c) altered reflex responses in SPNs; d) re-inner- tide fibers below the level of injury (68). Klimaschewski
vation of SPNs by spinal interneurons; and e) denervation found an increase in the innervation of SPNs by substance
hypersensitivity. Pcontaining nerve fibers in rats following SCI (69).
Sympathetic hyperactivity after spinal cord transec- Krentz and coworkers found that blocking nerve growth
tion might result from disinhibition of sensory pathways factor (NGF) prevented primary afferent sprouting in
484 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
spinal cord transected rats and decreased the hyperten- hyperreflexia (defined as involuntary bladder contrac-
sion induced by colon stimulation (70). tions with increased detrusor pressure of at least 60 cm
H2O) or detrusor-external urethral sphincter dyssynergia
(DESD) (75). DESD has been defined as the presence of
TREATMENT OF AUTONOMIC involuntary contractions of the external urethral sphinc-
HYPERREFLEXIA ter during involuntary detrusor contractions. DESD has
been reported to occur during bladder contractions that
Pharmacologic treatment of AH is aimed primarily at pro- are evoked either during urodynamic studies or by supra-
ducing direct vasodilatation (calcium-channel blockers, pubic tapping in as many as 86 percent of persons with
nitrates, hydralazine, diazoxide), central (spinal) 2- SCI (77). For a review of the urodynamics of SCI see
adrenoceptor agonist activity (clonidine) (71), or gan- Watanabe (78).
glionic blockade (mecamylamine). Nifedipine (a short-act- Bladder mechanoreceptors are activated by bladder
ing dihydropyridine calcium channel blocker) has been distension, mucosal deformation, and a shift in bladder
reported to increase the incidence of acute coronary events, position (79). Fluid-induced bladder distension results in
and its use in routine treatment of hypertension is not rec- the activation of mechanoreceptors that provide the sen-
ommended (72). Because it is unknown whether the treat- sory input needed to facilitate the activation of both
ment of AH with nifedipine in patients with SCI is also supraspinal- and spinal-mediated micturition reflexes (80).
associated with similar hazards, caution is advised in the Sensory inputs from the bladder to the human lum-
use of nifedipine in elderly patients, particularly those with bar spinal cord arise from T11 to L1, and perhaps as far
primary hypertension or cardiovascular disease. proximal as T9; sensory inputs from the bladder to the
Some 1-adrenoceptor blocking agents have been human sacral spinal cord arise from S2 to S4 (81). Affer-
used in the prophylactic management of autonomic dys- ent fibers in the pelvic nerve projecting to the sacral spinal
reflexia. Prazosin, 3 mg bid, was effective in reducing the cord are responsible for the initiation of micturition. In
number of sever episodes of AH and in reducing the aver- cats, A-fiber bladder afferents respond to bladder dis-
age rise in systolic and diastolic blood pressure during tension whereas C-fiber bladder afferents respond pri-
an episode (73). Recurrent symptoms of AH, such as marily to noxious stimulation (82). A-fiber bladder
headache, sweating, and flushing of the face, together afferents initiate the supraspinal micturition reflex
with an increase in blood pressure, have been successfully whereas C-fiber bladder afferents are thought to con-
treated with the once-a-day selective 1-adrenergic block- tribute to bladder hyperactivity following SCI in humans.
ing drug, terazosin (1 to 10 mg in adults and 1 to 2 mg Preganglionic parasympathetic neurons in the IML
in pediatric patients) (74). Phenoxybenzamine is a long- of the sacral cord segments S2 to S4 project preganglionic
acting -adrenergic blocker commonly used in a dose of fibers into the pelvic nerve to the pelvic plexus (79). In the
20 to 40 mg/day to prevent AH (52). There are conflict- human bladder, some pelvic plexus neurons are in the
ing reports as to the effectiveness of phenoxybenzamine bladder wall (79). Parasympathetic postganglionic nerves
in preventing autonomic hyperreflexia (52). excite detrusor smooth muscle via release of Ach acting
on muscarinic receptors in the bladder muscle or by the
release of adenosine triphosphate acting on purinergic
DISORDERS OF MICTURITION IN SCI receptors in the bladder muscle (82). The M3 muscarinic
receptor subtype mediates bladder contractions (82). The
The lower urinary tract functions are to store and peri- parasympathetic input to the urethral smooth muscle is
odically release urine. These functions are performed by inhibitory and mediated by nitric oxide (NO) (83).
the smooth muscle of the urinary bladder (detrusor) and Activation of prejunctional 5-HT4 receptors facili-
urethra and the striated muscle of the external urethra tates cholinergic transmission in detrusor smooth mus-
sphincter. Micturition requires the coordinated activation cle and activation of postjunctional 5-HT2 receptors in
of these muscles, which is controlled by supraspinal struc- detrusor smooth muscle results in a bladder contraction.
tures controlling autonomic and somatic nerves. Cisapride, a 5-HT4 agonist at doses of 10 mg q. i. d.(four
Complete SCI proximal to the sacral spinal cord times a week) for three weeks, has been used to improve
results in an upper motor neuron lesion characterized by bladder emptying in micturition disorders associated with
a hyperreflexic detrusor, whereas injuries involving the detrusor hypocontractility (84).
sacral cord or cauda equina result in a lower motor neu- SPNs that project to the lower urinary tract are
ron lesion characterized by an areflexic detrusor (75). In found in spinal cord segments T10 to L2 in humans (79).
a study of 489 persons with spinal cord lesions from a Most preganglionic sympathetic fibers project through
variety of causes, all those who had suprasacral spinal the lumbar splanchnic nerves to the inferior mesenteric
cord lesions without evidence of additional sacral spinal ganglion (IMG), and a smaller number of preganglionic
cord or cauda equina involvement had either detrusor sympathetic fibers project via the sacral paravertebral
AUTONOMIC DYSFUNCTION IN SPINAL CORD DISEASE 485
chain ganglia into the pelvic nerve to the pelvic plexus. The activation of the parasympathetic pathways to
The hypogastric nerve (also known as the presacral nerve) the detrusor muscle and the inhibition of somatic input
is composed of both preganglionic and postganglionic to the intrinsic EUS are the essential neuronal events that
sympathetic fibers that pass from the IMG to the pelvic initiate release of urine. Reflex contractions of the urinary
plexus (86). Most preganglionic sympathetic fibers inner- bladder and the release of urine that occurs in response
vating the pelvic viscera form synapses in the IMG or the to bladder distension are mediated via a parasympathetic
pelvic plexus (79,86). reflex pathway consisting of an A-fiber afferent limb and
The sympathetic postganglionic nerves release NE, a preganglionic parasympathetic efferent limb in the
which inhibits detrusor smooth muscle via 2- or 3-AR, pelvic nerve (94,95). Spinal afferent pathways ascend in
contracts the smooth muscle of the bladder trigon and the lateral funiculus or the dorsal funiculus of the spinal
urethra via 1-AR, and inhibits (via 2-AR) or facilitates cord and terminate in the nucleus gracilis and periaque-
(via 1-AR) parasympathetic ganglionic transmission ductal grey (PAG). It is thought that neurons in the PAG
(87). The 1A-adrenoceptor is the major subtype in the relay information to the pontine micturition center (PMC)
prostate and urethra (82). The 1-AR blockers, doxazosin to initiate micturition (87). Neurons from the nucleus gra-
and terazosin, used in the treatment of bladder outlet cilis seem to carry nociceptive information that is relayed
obstruction are not selective enough on the urethra to to the thalamus and cortex (87). The descending pathway
eliminate cardiovascular side effects. The selective 1A- of the micturition reflex is also in the dorsolateral funicu-
adrenoceptor antagonist tamsulosin has fewer cardio- lus (96). The spinobulbospinal micturition reflex can be
vascular side effects (5). Bennett and coworkers have sug- modulated at the spinal level by a variety of afferent
gested using terazosin as a first-line treatment of vesical inputs from the colon, vagina, penis, or perineum at var-
sphincter dyssynergia (VSD) prior to contemplating ious sites, including primary afferent terminals, interneu-
surgery (88). rons, or bladder preganglionic neurons. (253) Glycine, an
The urethral sphincter mechanism has two parts: the inhibitory amino acid, contributes to the inhibition of
internal and external urethral sphincter (EUS) (89). The EUS motor neurons during micturition (97).
internal sphincter is the smooth muscle of the urethra that The major excitatory neurotransmitter in the CNS
extends from the bladder outlet through the pelvic floor control of micturition is glutamate (82). Both NMDA and
(89). The striated muscle of the EUS also has two com- AMPA receptors are involved. In the rat, glutamate recep-
ponents: the intrinsic EUS, which lies completely within tors at the level of the lumbosacral spinal cord are
the urethral wall; and the extrinsic EUS, which is formed involved in processing the afferent input from the urinary
by the skeletal muscle fibers of the pelvic floor and uro- bladder and mediate segmental inteneuronal excitation
genital diaphragm (90). The extrinsic EUS is innervated of parasympathetic preganglionic neurons (PGNs) (82).
by the somatic pudendal nerve. However, the extrinsic In the cat, the PMC elicits voiding in response to gluta-
EUS may have autonomic innervation as well (89,91). mate injection (98). The PMC is under tonic GABAner-
The EUS is the most important active mechanism for gic inhibition (98). At the sacral spinal cord level, GABA
maintenance of urinary continence (89). agonists inhibit sacral parasympathetic PGNs (82).
The human striated urethral sphincter (removed Baclofen, a GABA B agonist, decreases bladder hyperac-
from patients who had died of diseases not related to the tivity in patients with spinal cord pathology (99).
urinary tract) was found to have a dense innervation by During continence, the spinal vesicosympathetic
cholinergic nerves and a sparse innervation by adrenergic reflex pathways allow the urinary bladder to accommo-
nerves and by nerves reactive for neuropeptide Y (NPY) date larger volumes by increasing the tone of the bladder
(92). The human striated urethral sphincter derived from neck, by depressing impulse transmission from the sacral
patients with lower motor spinal cord lesions was found spinal cord in pelvic vesical ganglia, and by direct inhi-
to have abundant innervation by adrenergic nerves (91). bition of the detrusor muscle (100). An intersegmental
NPY-reactive nerves have been found in most intrinsic spinal pathway elicits vesicosympathetic reflexes with
EUS fibers in patients with areflexic bladders caused by afferents in the pelvic nerve and efferents in the hypogas-
lower motor neuron SCI (93). NPY is known to be colo- tric nerve (87). The physiologic significance of the sym-
calized in noradrenalin-containing neurons and to inhibit pathetic innervation of the urinary bladder is unclear.
NE release (92). In addition the spinobulbospinal pathway, which is
The human smooth urethral sphincter (removed thought to mediate normal micturition, a spinal micturi-
from patients who had died of diseases not related to the tion reflex pathway has been identified (101). Research
urinary tract) was found to be innervated by cholinergic by de Groat and Ryall found that the spinal micturition
and adrenergic nerves as well as nerves reactive for NPY reflex was present in some intact cats and in all cats with
and galanin (92). VIP-reactive nerves have been found in chronic spinal cord transection (95). It is thought that this
striated and smooth urethral sphincter muscle derived spinal pathway, which has a C-fiber afferent limb and
from patients with bladder cancer or SCI (92). parasympathetic postganglionic efferent limb in the pelvic
486 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
nerve, mediates automatic micturition following chronic tactile stimulation of the penis (107). In patients with a
spinal cord transection (94). C-fiber afferents, which usu- LMN lesion, erections occur less often (26 percent) and
ally do not respond to bladder distention, may become are usually psychogenic in origin, obtained by visual or
mechanosensitive after SCI and may elicit the spinal mic- imaginative stimuli (107). Erection is primarily a spinal
turition pathway as well as bladder hyperreflexia fol- reflex modulated by supraspinal influences and results
lowing SCI. from the relaxation of smooth muscle in the penis (108).
Most bladder C-fibers are capsaicin sensitive. Cap-
saicin is a neurotoxin that activates vanilloid receptors on
Anatomy of Male Sexual Function
sensory neurons (82). Intravesical capsaicin or resinifer-
atoxin (a potent analog of capsaicin) can reduce bladder The penis receives innervation from both somatic
hyperactivity and the autonomic hyperreflexia induced (motor/sensory)and autonomic (sympathetic/parasympa-
by bladder distention (102). thetic) pathways. Somatic afferent innervation to penile
Tachykinins may play a role in detrusor hyper- skin is carried in a terminal branch of the pudendal nerve
reflexia after SCI (103). Various afferent neurotransmit- called the dorsal nerve of the penis (DNP), which projects
ter neuropeptides, including substance P, neurokinin A, to the sacral spinal cord via the S2S4 dorsal root gan-
calcitoningene-related peptide, vasoactive intestinal glia (109). Physiologic activation of afferent pathways in
polypeptide, pituitary adenylate cyclase-activating pep- the DNP elicits multiple sexual responses, including penile
tide, and enkephalins are contained in urinary bladder erection, seminal emission, and ejaculation (110,111).
afferent neurons (82). Many of these neuropeptide neu- Somatic efferent innervation to the ischiocavernosis and
rotransmitters are contained in C-fiber bladder afferents bulbospongiosus striated muscles arise in Onufs nucleus
and are released from the nerve endings into the bladder in sacral segments S2S4 and are conveyed in the puden-
by noxious stimulation. Afferent neuropeptides may also dal nerve. The contraction of the perineal striated mus-
be released at the afferent terminals in the spinal cord. cles enhances an erection that is already present (112).
Neurokinin1 antagonists increase the micturition thresh- Sympathetic preganglionic fibers from T11 to L2 and
old in normal bladders. In rats, both neurokinin1 and neu- parasympathetic preganglionic fibers from S2 to S4 are
rokinin2 antagonists inhibit detrusor hyperactivity involved in erection and ejaculation (113). Parasympathetic
induced by either local application of capsaicin (82) or by preganglionic fibers from the sacral spinal cord project in
SCI (103). This suggests a role for neurokinin1 and neu- the pelvic nerve to the pelvic plexus (109). One pathway
rokinin2 receptors in C-fiber mediated spinal bladder for sympathetic preganglionic fibers to the penis is from the
reflexes and neurokinin1 receptors in the A-fiber thoracolumbar spinal cord through the paravertebral chain
supraspinal micturition reflex. ganglia to project via either the pelvic nerve and plexus into
Cutaneovesical reflexes have also been described. In the penile nerve (also known as the cavernous nerve), or
rats, cutaneous stimulation resulted in a reflex bladder the pudendal nerve to the penis. The other pathway for
contraction both before and after spinal cord transection sympathetic preganglionic fibers is through the lumbar
(104). Neonatal cats exhibit a perineal-bladder reflex splanchnic nerves to the IMG. Sympathetic postganglionic
mediated in the spinal cord that disappears in adult life, fibers from neurons in the IMG as well as preganglionic
and this reflex reappears after spinal cord transection in fibers project through the hypogastric nerves to the pelvic
adult cats (105). Activation of spinal cutaneous soma- plexus. Sympathetic and parasympathetic postganglionic
tovesical reflexes may be responsible for voiding elicited efferent fibers project from the pelvic plexus to the penis
by suprapubic tapping or the pulling of pubic hair in per- via the penile nerves (114).
sons with SCI above the sacral outflow.
Erectile Dysfunction in SCI
MALE SEXUAL DYSFUNCTION IN SCI Penile flaccidity is maintained by sympathetic efferents
and -AR (227). Both 1- and 2-AR have been found
Most cases (82 percent) of SCI occur in males during their in human corpus cavernosum (115). The predominant
reproductive years (mean age, 26 years) (106). Male sex- subtype is the 1-adrenoceptor, although stimulation of
ual dysfunction following SCI can be classified as upper prejunctional 2-AR may inhibit the release of the nona-
motor neuron (UMP) lesion or lower motor neuron drenergic noncholinergic (NANC) mediator of penile
(LMN) lesion. UMN lesion occurs when the SCI is above erection. This is thought to be nitric oxide (NO) (116).
the sacral (S2S4) level, so that sacral reflex arcs are Reflex erections are mediated via the sacral
intact. LMN lesions occur when there is direct injury to parasympathetics, through afferent input from the puden-
the conus medullaris or cauda equina. dal nerve and efferent output via the pelvic, then penile
Erections occur in most patients with UMN lesions (cavernous), nerves. Reflex erections are organized in the
(94 percent) and are usually reflex erections obtained by sacral spinal cord (113). The phenomenon of psychogenic
AUTONOMIC DYSFUNCTION IN SPINAL CORD DISEASE 487
erections in paraplegic men with complete sacral lower Many agonists, including 1-AR agonists, Ach,
motor neuron lesions and abolished reflexogenic erections angiotensin, and AVP, bind to receptors that are coupled
indicates that a pathway for erection from the sympa- to the phosphatidylinositol cascade, which releases inos-
thetic outflow exists (113). The sympathetic proerectile itol 1,4,5-triphosphate (IP3). IP3 opens calcium channels
outflow may be via the hypogastric to the penile (cav- on the sarcoplasmic reticulum without depending on
ernous) nerves (112). changes in the smooth muscle membrane potential. This
Erection involves parasympathetic cholinergic and results in an increase of sarcoplasmic calcium, which trig-
NANC mechanisms (112,117). NO is the NANC neu- gers smooth muscle contraction (115).
rotransmitter primarily thought to mediate erection. It is Intracavernosal injection of vasoactive substances
a smooth muscle relaxant and vasodilator (106). It is such as papaverine, phentolamine, and prostaglandin E1
unlikely that Ach causes erection by a direct action on (PGE1) are useful for erectile dysfunction after SCI. The
smooth muscle fiber postjunctional muscarinic receptors main action of papaverine is nonselective PDE inhibition.
because isolated human corpus cavernosum smooth mus- Phentolamine is a competitive antagonist of 1- and 2-
cle cells contract in response to cholinergic stimulation AR. Intracorporal injection of phentolamine in combi-
(106). Ach may contribute to erection by inhibiting the nation with VIP is a safe and effective means of treating
release of NE via stimulation of muscarinic receptors on nonpsychogenic erectile dysfunction (125). The main
adrenergic nerve terminals (118) or by modulating NO action of PGE1 is probably to increase the intracellular
release by endothelial cells and/or nerves in the penis concentration of cAMP in corpus cavernosum smooth
(115). muscle cells.
NO is essential for the erectile process (115). NO In a study published in 1996, Watanabe found that
relaxation of vascular smooth muscle is mediated through the vacuum erection device (VED) and pharmacologic
activation of guanylate cyclase to produce cyclic guano- penile injection were the most popular treatments for
sine monophosphate (cGMP) (106). Activation of a erectile dysfunction in SCI, and papaverine was the most
cGMP-dependent protein kinase (cGK I) leads to penile common drug used (126). The vacuum erection device
erection (115). Neurons and endothelial cells in the cor- has been reported to be effective in up to 93 percent of
pus cavernosum synthesize and release NO. Phosphodi- SCI patients (126,127). The penile prosthesis has been
esterases (PDEs) catalyze the hydrolysis of cAMP and associated with high rates of mechanical failure (43 per-
cGMP (115). Sildenafil (Viagra) increases cGMP by inhi- cent) and low rates of improved sexual function (41 per-
bition of cGMP-specific phosphodiesterase V that results cent) (106). Biering-Sorensen and Sonksen recommend
in penile erection (106). Sildenafil has been found safe and the use of a penile venous constrictor band for men with
effective for erectile dysfunction caused by SCI (119121). SCI and satisfactory erections of insufficient duration
Nitroglycerin is thought to cause smooth muscle (128). Otherwise, these authors recommend sildenafil as
relaxation via enzymatic liberation of NO (115). Trans- the first line of treatment and intracavernous injection of
dermal nitroglycerin causes erections following SCI PGE1 as the second line of treatment for men with SCI
(122,123). Although transdermal nitroglycerin was not and erectile dysfunction.
as effective as intracorporal PGE1 in causing erections,
the only complication was mild headache (122,123).
Ejaculatory Dysfunction in SCI
NO and vasoactive intestinal peptide (VIP) are colo-
calized in cholinergic nerves in the human penis 115). VIP Emission of semen and seminal fluid is primarily under
has also been implicated as a mediator of the noncholin- sympathetic control. Emission is followed by closure of
ergic nonadrenergic mechanism of erection (116,117) and the bladder neck and contraction of the bulbourethral
is known to relax smooth muscle from the human penis striated muscles, the latter being mediated by pudendal
(116,124). VIP stimulates VIP receptors in the penis; this somatic efferents (113). About 5 to 10 percent of men
leads to an increase in cAMP, which in turn activates a with complete SCI experience ejaculation or seminal emis-
cAMP-dependent protein kinase (115). sion (113,129,130).
Continuous transmembrane calcium influx through For purposes of obtaining semen for artificial
calcium channels is needed to sustain the contraction of insemination, an ejaculation reflex can be obtained by
human corporal smooth muscle and maintain penile flac- vibratory stimulation of the frenulum and lower surface
cidity (115). The second messengers, cAMP and cGMP, of the glans penis (131). The afferent pathway of this ejac-
increase the activity of a calcium-sensitive K+ channel in ulation reflex likely involves the pudendal nerves (DNP)
human corporal smooth muscle. This results in hyper- and ascending tracts from the sacral spinal cord to the
polarization, which is coupled to a decrease in trans- thoracolumbar T12 to L1 preganglionic sympathetic
membrane calcium flux through calcium channels, thus nerves (129,132). Penile vibratory stimulation (PVS) to
resulting in smooth muscle relaxation and penile tumes- the glans penis triggers an ejaculatory reflex . PVS is more
cence (115). successful in lesions above T10 (81 percent) than T10 or
488 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
below (12 percent), and is more successful if hip flexion were able to achieve orgasm; time to orgasm was increased
and bulbocavernosus reflexes are present (77 percent) in women with SCI compared to non-SCI controls (138).
rather than absent (14 percent) (133). Furthermore, the quality of orgasm appeared to be simi-
Electroejaculation is the electrical stimulation of lar for SCI and non-SCI women (138).
efferent sympathetic nerves via a rectal probe. Biering- Three pathways are thought to convey sensory infor-
Sorensen and Sonksen recommend PVS as the first treat- mation from the uterus, cervix, and vagina to the CNS.
ment choice for management of ejaculatory dysfunction These pathways are via the hypogastric nerves to the tho-
in men with SCI, followed by electroejaculation if PVS racolumbar spinal cord (T10L1), via the pelvic nerve to
fails (128). the sacral spinal cord (S2S4), and via the vagal nerve to
the nodose ganglia and NTS (140). Whipple and Komis-
aruk hypothesize that the orgasms obtained in women
FEMALE SEXUAL DYSFUNCTION with complete SCI in response to genital stimulation are
mediated by sensory fibers in the vagus nerve (141).
Sexual dysfunction is common in women with SCI. How- Sildenafil may partially reverse the sexual dysfunc-
ever much less is known about sexual function in females tion commonly associated with SCI in women (142).
with SCI than in males, and sexuality in spinal cord injured Sildenafil has been shown to result in significant increases
women has often been neglected (134,135). A womans in subjective arousal in women with SCI, with maximal
libido and reproductive capability remain intact following responses occurring when sildenafil was combined with
SCI (136). There is transient anovulation in about 50 per- visual and manual sexual stimulation. Sildenafil was
cent of women with SCI, but the preinjury menstrual pat- found to be well tolerated with no evidence of significant
tern is reestablished in 3 to 6 months (134,136,137). adverse events (142).
Anovulation is thought to be a result of the stress of the
trauma and is not related to the level of injury or its degree
Childbirth
of completeness. Mean menstrual cycle length and the
duration of menses have been shown to fall within the nor- Pain in the first stage of labor is caused by uterine con-
mal range for fertile, able-bodied women, regardless of traction and cervical dilatation (136). Because the affer-
level or completeness of injury (137). Menarche is not ent innervation of the uterus arises from T10 to LI, labor
delayed if the SCI is preadolescent. The fertility rate and is painless for women with an SCI above T10. In the sec-
miscarriage rate are the same for women with SCI as for ond stage, labor pain is from the perineum, innervated by
the general population of sexually active women (136). the pudendal nerve and spinal cord segments S2 to S4.
The four components of the sexual response cycle About 25 percent of pregnant spinal cord-injured women
excitement, plateau, orgasm, and resolutionare all pre- are unable to detect the onset of labor (136).
sent, but may vary in degree in women with SCI Efferent uterine innervation arises from T10 to T12
(134,135). The normal female sexual excitation phase (134). In women with SCI above the T10 level, uterine
consists of vaginal lubrication, swelling of the clitoral contractions are effective and labor progresses normally
gland, and congestion of the labia (134). With complete (134). The intensity of uterine contractions is not reduced,
SCI there is absence of lubrication (reflex or psychogenic) and labor is of short duration, often with spasm of the
when the injury is situated between T10 and T12, thus abdominal muscles (143). Most women with an SCI level
indicating that preganglionic neurons at this level of the above T6 develop autonomic hyperreflexia concomitant
spinal cord constitute the final efferent pathway for both uterine contractions (75,144). A significant increase in the
reflex and centrally mediated lubrication (134). Reflex incidence of premature delivery has not been documented
lubrication does occur with lesions above T9 and psy- (136). Women with SCI may be expected to have a rea-
chogenic lubrication is present with injuries below T12 sonably normal pregnancy outcome, provided that spe-
(134). Sipski and coworkers found that preservation of cific complications, particularly autonomic hyperreflexia,
T11L2 sensory function was associated with psy- are anticipated and managed properly (143,145).
chogenic genital vasocongestion (138).
Thirty-six percent of eleven women with complete
SCI, 67 percent of six women with incomplete SCI and pre- GASTROINTESTINAL DYSFUNCTION IN SCI
served light touch T11L2, and 63 percent of eight women
with incomplete SCI and preserved pinprick T11L2 were The function of the gastrointestinal (GI) tract is to retain
able to achieve orgasm in a laboratory-based assessment nutrients and eliminate waste. The motility, secretion, and
(139). Sipski found that 17 percent of women with com- absorption processes of the GI tract are regulated via
plete lower motor neuron dysfunction involving S2S5 exocrine, endocrine, and neural mechanism (146).
spinal segments were unable to achieve orgasm, whereas Paracrine regulation occurs when a sensing cell releases
59 percent of women with other levels and degrees of SCI a mediating chemical, which influences the function of
AUTONOMIC DYSFUNCTION IN SPINAL CORD DISEASE 489
nearby cells. Endocrine regulation occurs when a sens- mary afferent stimulation (150). Stretching of the intes-
ing cell releases a hormone into the circulation to effect tine also stimulates intrinsic primary afferents. The
the function of a distant target cell. Neural mechanisms smooth muscle of the intestine contracts when stretched,
involve sensory receptors that reflexively alter the secre- and this muscle contraction distorts the intrinsic affer-
tory or motor activity of the gut. GI reflex pathways may ent neuron cell body thereby activating a mechanosensi-
be located entirely within the gut or involve the brain tive ion channel to generate an action potential (150). The
and/or spinal cord. SCI results in abnormalities of the intrinsic primary afferents synapse with each other as well
motor function of the gut rather than in absorptive or as with interneurons and motor neurons of the myenteric
secretory dysfunction. plexus (150). Enteric interneurons are involved in motor
reflexes. These interneurons project axons orally or
Enteric Nervous System anally, and so are called ascending or descending; they
form multisynaptic pathways over the length of the gut.
The stomach and intestinal wall contain intrinsic neurons Extrinsic primary afferent neurons have cell bodies
that are part of the enteric nervous system, a system that in the nodose ganglia, with axons in the vagus nerve, or
has been referred to as the third division of the autonomic cell bodies in the dorsal root ganglia, with axons in the
nervous system. It is noteworthy that there are as many pelvic nerve and sympathetic nerves. They convey sensa-
neurons in the enteric nervous system (108) as there are tions of satiety, nausea, and pain to the CNS (147). Extrin-
in the spinal cord (146). Neurons of the enteric nervous sic primary afferent neurons are described as mucosal
system are not postganglionic parasympathetic neurons, receptors, muscle receptors, and serosa receptors.
and the majority of enteric neurons do not have direct Mucosal receptors respond to mechanical stimuli,
contact with parasympathetic preganglionic axons. The such as gentle stroking of the epithelium, lumenal chem-
enteric nervous system has two divisions that are con- icals, cold, or lumenal osmolarity (151). Three types of
nected by nerve processes: the submucosal plexus (Meiss- chemoreceptors found in the small intestine are likely
ners plexus), which innervates the mucosa and regulates responsible for feedback control of gastric emptying by
secretion; and the myenteric plexus (Auerbachs plexus), nutrients: osmoreceptors, lipid receptors, and receptors
which innervates the circular and longitudinal smooth for amino acids (151). Glucoreceptors and pH receptors
muscle layers and regulates motility. are also present in the small intestine. How stimulation
The enteric nervous system has three types of nerve of intestinal receptors mediates changes in gastric emp-
cells: motor neurons that innervate smooth muscle cells; tying has not been entirely resolved.
interneurons that connect different neurons; and intrin- Muscle receptors in the gastric, duodenal, and jeju-
sic primary afferent neurons. Although over twenty sub- nal smooth muscle respond to distension and contraction
stances have been identified as putative neurotransmitters of the viscus and have afferent fibers in the vagus nerve
in the enteric nervous system, the main neurotransmitters (152). Serosal receptors are activated by mechanical stim-
of excitatory motor neurons of the enteric nervous sys- uli. The splanchnic nerves, which convey afferents from
tem are acetylcholine and substance P, whereas the main the gut to the spinal cord, are thought to mediate painful
neurotransmitters of inhibitory motor neurons are VIP sensations, although vagus nerve afferents may also be
and NO (147,148). involved in gut nociception (153). The sensation of
Enteric motor neurons are categorized as muscle hunger is reduced in vagotomized patients (154).
motor neurons, secretomotor neurons, enteric vasodila- Extrinsic efferent innervation of the GI tract occurs
tor neurons, and motor neurons to enteric endocrine cells. through the parasympathetic and sympathetic divisions
These can be excitatory or inhibitory. Secretomotor neu- of the ANS. The GI tract from the esophagus to the dis-
rons result in water and electrolyte secretion in the small tal colon is innervated by vagal nerve efferents with pre-
and large intestine, bicarbonate secretion in the duode- ganglionic neurons in the dorsal motor complex of the
num, and gastric acid secretion in the stomach (147). vagus nerves. There is more proximal and lesser distal
In the guinea pig, intrinsic primary afferent neurons vagal innervation of the GI tract.
have been identified with cell bodies in the wall of the
small intestine (149,150). Three classes of intrinsic pri-
mary afferent neurons have been identified: tension-sen- UPPER GASTROINTESTINAL DYSFUNCTION
sitive neurons, mucosal mechanosensitive neurons, and
chemosensitive neurons (147). The intrinsic primary Upper GI tract dysfunction following SCI includes impair-
afferents are thought to be indirectly excited, perhaps by ment of gastric motility, gastric emptying, and intestinal
an intermediate substance. Lumenal chemicals or motility. One-third of persons with complete quadriple-
mechanical stimulation of the mucosa result in the release gia for more than 1 year have been found to have mild
of 5-HT from enteroendocrine cells of the mucosa. 5- GI symptoms such as nausea, diarrhea, constipation, and
HT could be an intermediate substance for intrinsic pri- fecal incontinence (155).
490 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
FIGURE 35.2
Spinal afferent and efferent pathways to the intestine. Although spinal afferents and sympathetic efferents travel via both the
superior mesenteric ganglion (SMG) and the inferior mesenteriac ganglion (IMG), the afferent pathway is depicted traveling via
the SMG, whereas the efferent pathway is depicted traveling via the IMG. Sympathetic preganglionic neurons to the colon and
pelvic viscera project to the SMG and the IMG via the splanchnic nerves. Other sympathetic preganglionic axons enter par-
avertebral chain ganglia and form synapses with postganglionic neurons there. The major role of the sympathetic nervous sys-
tem is to regulate gastrointestinal blood flow via excitatory input onto vasoconstrictor neurons (VC), secretion via excitatory
input onto secretomotor inhibiting neurons (SMI), and intestinal motility via excitatory input onto motor inhibiting neurons (MI).
The MI and SMI neurons mediate their effects by inhibiting enteric motoneurons (EM) in Meissners plexus (MP) between the
longitudinal muscle (LM) and circular muscle (CM), or in the submucosal ganglia (SM) between the CM and mucosa (Muc).
Vagal input (i.e., preganglionic) neurons form synapses on EM in the intrinsic ganglia of the gastrointestinal tract (not shown).
Intrinsic afferent neurons have cell bodies in the MP, whereas extrinsic afferent neurons have cell bodies in the dorsal root gan-
glia (DRG) and vagal ganglia (not shown). Intestinofugal (IF) fibers constitute the afferent arc of enteroenteric reflex pathways.
Sympathetic ganglia receive synaptic input from preganglionic neurons with cell bodies in the spinal cord, primary sensory
neurons with cell bodies in the DRG, and IF neurons arising in visceral intramural ganglia. [Modified from Furness et al. (147).]
In the abdomen, the parasympathetic preganglionic the sympathetic trunk. Postganglionic sympathetic effer-
efferent fibers to the stomach are conveyed in the vagus ent nerves emerge from the celiac and superior mesenteric
nerve, which in turn gives rise to gastric branches that prevertebral ganglia to run along mesenteric blood ves-
form synapses with neurons in the myenteric and the sub- sels and innervate the small intestine (see Figure 35.2).
mucosal plexuses of the stomach. The actions of the stomach in response to a meal are
Preganglionic sympathetic efferent fibers originate storage, secretion, mixing, and emptying. The fundus of the
in the mediolateral gray matter of the spinal cord and stomach acts as a reservoir to accommodate the meal, while
course through the splanchnic nerves to the celiac and the antrum is both a pump and a grinder. The vagus nerve
superior mesenteric prevertebral ganglia (plexuses). The is important for the motor activity of the stomach. Vagal
stomach receives sympathetic postganglionic fibers prin- stimulation induces relaxation of the fundus and contrac-
cipally from the celiac plexus but also from the left tion of the antrum (151). Mesenteric sympathetic nerve
phrenic plexus, bilateral gastric, and hepatic plexuses, and stimulation inhibits contraction in the small intestine (151).
AUTONOMIC DYSFUNCTION IN SPINAL CORD DISEASE 491
In the normal interdigestive state of the gastroin- via indirect pathways that modulate the release of polypep-
testinal tract of humans, a cyclic wave begins in the stom- tide gastrointestinal hormones (160).
ach and duodenum and migrates to the terminal ileum. Gastric emptying has been reported as delayed in
This activity, characterized by recurring periods of chronic quadriplegic subjects when data on gastric emp-
intense, regular motor activity, is known as phase III of tying is studied for at least 2 hours (160,161). Segal and
the interdigestive motor complex (IDMC) and is also coworkers identified a biphasic pattern of gastric empty-
referred to as the migrating myoelectric complex (MMC) ing where the initial (2030 minutes) gastric emptying
(156). The IDMC usually begins in the antrum and (which resembled the patterns seen in control and para-
migrates to the proximal duodenum in a coordinated plegic subjects) was followed by a delayed second phase
manner that is interrupted by feeding. Phase III activity (161). Lu and coworkers demonstrated a normal cuta-
is followed a period of less-intense activity (phase IV), neous electrogastrogram (EGG) in twelve patients with
then by quiescence (phase I), then by irregular contrac- complete cervical SCI (155). Abnormalities in the EGG
tions (phase II) (157). The phases of the interdigestive have been associated with gastric motor dysfunction, but
state disappear after a meal and are replaced by the ongo- if there is antral-duodenal motor incoordination after
ing phasic contractile activity of the fed pattern (157). The SCI, as described by Fealey (160) and coworkers, abnor-
phases of the IDMC and the fed pattern of gastrointesti- mal gastric myoelectrical activity may correlate poorly
nal contractile activity occur independent of nerves with gastric emptying (155). Paraplegic persons have been
extrinsic to the enteric nervous system (157). It is thought reported to have delayed gastric emptying, but this
that in man, the hormone motilin acts on enteric nervous appears to be a nonspecific finding related to prolonged
system neurons in the gastric antrum to control gastric immobilization (162).
emptying and trigger the MMC (158). Erythromycin has
motilin agonist properties, and its administration can
Lower Gastrointestinal Dysfunction
induce a MMC (158). One case report describes the suc-
cessful resolution of refractory SCI-induced gastropare- The most obvious lower gastrointestinal dysfunction
sis with eythromycin lactobionate (159). associated with SCI is the loss of voluntary control of the
In humans, gastric distension and ileus occur imme- initiation of defecation. The most common problems
diately after traumatic spinal cord transection, suggesting complicating the neurogenic bowel are poorly localized
abnormal gastrointestinal motility. In long-term quadri- abdominal pain (in 14 percent of cases studies by Stones
plegic persons, an intact supraspinal sympathetic pathway group); difficulty with bowel evacuation (20 percent);
is not an absolute requirement for initiation and propa- hemorrhoids (74 percent); abdominal distension (43 per-
gation of antral phase III motor activity. No significant dif- cent); and autonomic hyperreflexia arising from the gas-
ferences appear in the duration of phases of the IDMC, trointestinal tract (43 percent) (163). As many as 27 per-
cycle length of the duodenal IDMC, or the propagation cent of persons with SCI have significant chronic
velocity of phase III of the IDMC from the duodenum to gastrointestinal problems, and those with more complete
the jejunum among subjects with quadriplegia (neurologic injuries are more likely to have symptoms (33 percent)
level above T1); low paraplegia (neurologic level below than those with incomplete injuries (6 percent) (163). In
T10); or an intact spinal cord (160). In normal subjects, one survey of patients with SCI, 30 percent regarded
90 percent of phase IIIs originated in the antrum and symptoms colorectal dysfunction as worse than the symp-
migrated to the duodenum and jejunum, whereas in sub- toms of bladder and sexual dysfunction (164).
jects with high SCI, fewer than 40 percent of phase IIIs The extrinsic innervation of the lower GI tract is
originated in the antrum. About 80 percent of quadriplegic conveyed by parasympathetic, sympathetic, and somatic
subjects had dissociation between antral and duodenal nerves. Afferent fibers in the vagus nerves supply the small
phase III motility, manifested primarily as a pattern of per- intestine and the proximal two-thirds of the colon, coin-
sistent antral activity. In one subject with prominent recur- ciding with the vagal efferent innervation. Vagal afferents
rent autonomic hyperreflexia, there was marked antral respond to distension and contraction of the colon. Other
hypomotility. Antral quiescence was associated with the afferent fibers from the colon travel with both sympa-
degree of reflex vascular hypertension that resulted from thetic and parasympathetic axons and have cell bodies
spontaneous and suprapubic pressure-induced autonomic in the lumbar and sacral dorsal root ganglia.
hyperreflexia, whereas duodenal motility was unaffected The parasympathetic outflow to the colon and
(160). This suggests that motility in the antrum is modi- anorectum originates from brainstem nuclei and the
fied by central sympathetic input and that excessive sacral spinal cord. The ascending and transverse colon
splanchnic sympathetic outflow may delay gastric empty- receives parasympathetic efferent fibers from the poste-
ing by inhibiting antral motility (160). Fealey and cowork- rior vagus nerve, and the left half of the colon and rectum
ers suggested that sympathetic activity may influence gas- receives them from the pelvic nerve. Preganglionic
tric motility via a direct neural pathway to the gut wall or parasympathetic neurons project from the sacral seg-
492 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
ments S2 to S5 through the pelvic nerves and pass to the a tonic inhibitory influence with a spinal organization,
left colon and anorectum via the pelvic plexus (86). and the proximal colon appears to be influenced by nei-
The sympathetic supply to the right colon arises ther spinal nor supraspinal tonic inhibition.
from T6 to T12 and that to the left colon and upper rec- When food is ingested into the stomach, there is an
tum arises from L1 to L3 (165). Sympathetic pregan- increase in colonic motor activity that is called the gas-
glionic neurons to the colon and pelvic viscera project to trocolic reflex (169). The afferent limb of this reflex can
the superior and IMG via the lumbar splanchnic nerves be blocked by intragastic lidocaine, and the increase in
(86). Postganglionic fibers from the superior mesenteric distal colonic spike activity can be blocked by anti-
ganglion innervate the colon from the cecum to the dis- cholinergics or naloxone, indicating the participation of
tal transverse colon. Postganglionic fibers from the IMG cholinergic and opiate receptors. Colonic cyclical orga-
project via lumbar colonic nerves, which run along the nization in the rat, including enhancement of distal
inferior mesenteric artery to innervate the left side of the colonic cyclical activity as a secondary response to feed-
colon. Postganglionic fibers from the IMG also project ing (gastrocolic reflex), persists after ablation or section
via the hypogastric nerves and join the pelvic plexus (also of the spinal cord (168). This demonstrates thatat least
known as the inferior hypogastric plexus) to innervate the for the distal coloncolonic cyclic organization is not ini-
distal colon (86). Other sympathetic preganglionic axons tiated by lumbar spinal or supraspinal influences. The
enter paravertebral chain ganglia and form synapses with local enteric nervous system, the prevertebral ganglionic
postganglionic neurons there (85). system, or both, are probably responsible for the cyclic
Some sympathetic postganglionic neurons are vis- organization of distal colonic motility in rats. However,
ceral vasoconstrictor neurons and innervate vascular the gastrocolic reflex in the transverse colon persists in
smooth muscle, while others are motility-regulating (MR) rats after spinal cord transection but not after spinal cord
neurons, which innervate visceral smooth muscle. ablation, thus suggesting that it was organized in the
Whereas some sympathetic postganglionic neurons con- spinal cord (168).
trol the effector organs directly, other sympathetic post- Analysis of colonic myoelectric spike activity of the
ganglionic neurons control the effector organs indirectly rectal mucosa 6 to 15 cm from the anus revealed that, in
by influencing other peripheral neurons, such as those in the fasting state, persons with SCI above T10 who were
the submucosal ganglia (Meissners plexus) or myenteric also more than 3 months post injury, had significantly
ganglia (Auerbachs plexus) of the enteric nervous system greater basal colonic myoelectric spike activity than con-
or in the prevertebral ganglia, via pre- or postsynaptic trol subjects (170). Meal stimulation increased basal spike
mechanisms (143). activity (gastrocolic reflex) in the control group compared
The majority of lumbar sympathetic postganglionic to the fasting state. In the SCI group there was no signif-
neurons are noradrenergic, but many contain a peptide icant increase in myoelectric spike activity after the meal,
as well (166). Sympathetic ganglia receive synaptic input compared to the fasting state. Because feeding did not sig-
from preganglionic neurons with cell bodies in the spinal nificantly increase the already high basal spike activity
cord, primary sensory neurons with cell bodies in the dor- of persons with SCI, the gastrocolic reflex could not be
sal root ganglia, and neurons arising in visceral intra- demonstrated. The loss of a tonic inhibitory supraspinal
mural ganglia (enteric nervous system). The peripheral influence may cause the increase in basal colonic spike
ANS may mediate the complex reflex functions of the gas- activity following SCI (170). The absence of a gastrocolic
trointestinal system without the involvement of either reflex in this study is consistent with studies by Glick and
supraspinal or spinal cord influences. colleagues (171), who recorded at 12 to 18 cm from the
Electrical stimulation of the sympathetic supply to anus; their findings differ from those of the study of Con-
the colon results in contraction of the internal anal sphinc- nell and associates (172), who recorded intact gastrocolic
ter and relaxation of the colon and rectum (86). Studies reflexes at 15, 20, and 25 cm from the anus (143).
in the rat have shown that the sympathetic inhibition is Fecal material entering the rectum results in relax-
mediated by postjunctional -AR on colon smooth mus- ation of the internal anal sphincter (IAS) (rectoanal
cle, whereas sympathetic-induced contractions, which are inhibitory reflex), but contraction of the external anal
most prominent in the distal colon (although present in sphincter (EAS) to prevent incontinence (holding reflex)
the proximal colon), are mediated by postjunctional (173). Internal anal sphincter relaxation occurs after rec-
smooth muscle -AR (167). tal distension in individuals with SCI above T12 (174).
The lumbar sympathetic outflow exerts a tonic Complete sacral posterior rhizotomy did not eliminate the
inhibitory influence on the motility of the colon. Studies IAS relaxation induced by rectal distention. However,
in rats show that there is differential sympathetic inhibi- reflex contraction of the EAS in response to either rectal
tion of the proximal, transverse, and distal colon (168). distention or increased intraabdominal pressure was elim-
The distal colon receives a tonic inhibitory influence with inated by sacral posterior rhizotomy, thus indicating that
a supraspinal organization, the transverse colon receives these contractions are mediated by a spinal reflex (174).
AUTONOMIC DYSFUNCTION IN SPINAL CORD DISEASE 493
Voluntary relaxation of the EAS and the puborectalis (2-adrenoceptors are activated by circulating NE or E
muscle results in stool elimination (174). released from the adrenal medulla (174).
In persons with complete SCI above T12, transit of The bronchial circulation is controlled by sympa-
contents is slowed throughout the large bowel, regardless thetic vasoconstrictor and parasympathetic vasodilator
of the level of the spinal cord lesion (175). Studies have nerves (185). It is thought that the bronchial vasculature
identified a) prolonged rectosigmoid transit times (175); is under tonic sympathetic influence and results in vaso-
b) transit delays more marked in the descending colon, constriction mediated by -adrenoceptors (185). Pul-
sigmoid, and rectum than in the cecum, ascending colon, monary vascular tone is also regulated by nonadrener-
and transverse colon (176,177); or c) transit delays gic, noncholinergic neural mechanisms and humoral
involving the entire colon (178). Krough and coworkers mechanisms.
found that persons with supraconal SCI had generalized Pulmonary receptors convey afferent information
colonc dysfunction, whereas persons with conal or cauda via the vagus nerve (186). Slowly adapting receptors
equina lesions had severe rectosigmoid dysfunction (179). (SAR) and rapidly adapting receptors (RAR) detect lung
The obvious clinical implication of prolonged tran- volume and changes in lung volume (187). Afferent fibers
sit time throughout the left and right colon is that treat- from these receptors terminate in the nucleus tractus soli-
ment of colorectal dysfunction in patients with SCI should tarius (NTS) in the medulla and can reflexively inhibit
include not only rectal agents but also prokinetic agents or excite inspiration (187). The RAR in the large bronchi
to reduce transit time in the entire colon (178). Cisapride and carina appear to be the most important receptors for
has been shown to improve chronic constipation and the initiation of cough (188). There is also sensory C-fiber
colonic transit times in persons with quadriplegia or para- nociceptor innervation of the airways. C-fiber axon stim-
plegia (180,181). After reports of serious cardiac arryth- ulation causes bronchial vasodilatation via a peripheral
mias, Cisapride was withdrawn from the United States vagal axon-reflex mechanism that depends on the release
market, however, it continues to be available under a lim- of vasodilator neuropeptides from axon terminals (189).
ited-access program for patients for whom other drug An axon-reflex occurs when the impulse propagated cen-
treatments fail (182). Nizatidine, an H2-receptor trally by the stimulation of one branch of the peripheral
inhibitor, appears to exert a prokinetic action in the end of an afferent axon invades a second peripheral
human colon, but studies in patients with SCI are lack- branch of the same axon and results in the release of neu-
ing (183). ropeptides from the peripheral end of the second affer-
ent axon branch. Activation of C-fiber afferents in the
lungs and airways also causes reflex brochial vasodilation
PULMONARY DYSFUNCTION through afferent and parasympathetic efferent pathways
in the vagus nerve (189). Respiration is also controlled by
Although pulmonary problems are common following chemosensors that detect hypoxia and hypercapnia.
SCI, they are usually secondary to impairments in inspi- Peripheral chemoreceptors are located in the carotid and
ratory and expiratory function, with associated abnor- aortic bodies and also project to the NTS (187).
malities in gas exchange that are largely the result of Medullary neurons are also chemosensitive to CO2 (190).
ineffective cough mechanisms and not autonomic dys- Loveridge and coworkers identified impaired ven-
function. However, changes in breathing patterns, airway tilatory lung function in sitting following complete
hyperresponsiveness, ventilatory drive, and control of C5C8 SCI at 3 months post injury, with improvement
ventilatory responses have been identified. between 3 and 6 months. They also found that the sigh
The parasympathetic nervous system, via the vagus reflex is retained in quadriplegic subjects, albeit they take
nerve, is the dominant pathway in the control of more big breaths in the supine rather than in the sitting
bronchial smooth muscle tone (184). Stimulation of position (191).
cholinergic nerves results in bronchoconstriction, mucus Otherwise-healthy subjects with quadriplegia have
secretion, and bronchial vasodilation (184). An inhibitory been shown to have airway hyperresponsiveness to
nonadrenergic noncholinergic (i-NANC) pathway also aerosolized methacholine, histamine, and ultrasonically
mediates bronchodilitation (184). The neurotransmitters nebulized distilled water (192195). Aerosolized iprat-
of the i-NANC pathway are colocalized with ACH in the ropium bromide blocked the airway hyperreactivity asso-
parasympathetic nerves. The main neurotransmitter of ciated with aerosolized methacholine and ultrasonically
the i-NANC system in human airways appears to be NO nebulized distilled water, but did not inhibit airway hyper-
which may be coreleased with ACH and VIP (184). Both reactivity associated with histamine inhalation (193).
NO and VIP have potent smooth muscle relaxing prop- Nebulized -agonist metaproterenol sulfate blocked both
erties. Although (2-adrenergic receptors are expressed on histamine- and methacholine-associated airway hyperre-
human bronchial smooth muscle, no innervation from the activity (193). In one report, subjects with high paraple-
sympathetic nervous system has been shown (184). These gia (T1T6) who demonstrated airway hyperresponsive-
494 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
ness to methacholine had significantly lower FEV1 than at the same level of end-tidal PCO2, independent of
subjects who did not demonstrate hyperreactivity (195). whether end-tidal PCO2 was increased by increasing
Singas and coworkers hypothesized that loss of the abil- FICO2 or by decreasing tidal volume or frequency. Simon
ity to stretch airways by deep breathing may cause air- (203) and coworkers, concluded that afferent feedback
way hyperresponsiveness in quadriplegic subjects. from some part of the chest wall was needed to produce
There are conflicting reports on the response of a volume- and frequency-dependent inhibition of inspi-
quadriplegics to hypercapnia. Normally, ventilatory drive ratory muscle activity during mechanical ventilation.
is increased by hypercapnia (primarily via central The sensation of the need to cough and of conges-
chemoreceptor stimulation) and hypoxia (primarily via tion has been reported to remain after high cervical spinal
peripheral chemoreceptor stimulation) (14). Several stud- cord lesions (202). It has also been shown that ventilated
ies have found a blunted ventilatory drive in response to quadriplegic subjects can reliably perceive an increase in
hypercapnia (196198) while another reports normal PCO2 in the physiologic range as an uncomfortable sense
ventilatory chemosensory responses to hypercapnia and of air hunger (16). Ventilated quadriplegic subjects
hypoxia (199). The latter results may have been caused maintained at a constant but elevated level of end-tidal
by the inclusion of quadriplegic subjects with incomplete PCO2 experienced a similar sensation of air hunger
spinal lesions. Manning (197) and coworkers could not when tidal volume was decreased (197). The sensation
attribute the blunted respiratory drive found in quadri- of dyspnea in response to increases in end-tidal PCO2 is
plegic subjects to expiratory muscle weakness or altered preserved in spinal cord-intact humans, paralyzed with
chest wall mechanics. They hypothesized that the blunted total neuromuscular blockade, thus suggesting that
ventilatory drive in response to hypercapnia may be chemoreceptor activity can lead to discomfort in the
caused by the loss of supraspinal control of the sympa- absence of any respiratory muscle contraction (204).
thetic nervous system, because similar blunted responses
have been reported previously in individuals with auto-
nomic dysfunction. Unlike Manning and colleagues, Lin THERMOREGULATORY DYSFUNCTION
(198) and coworkers found that the reduction of minute
ventilation (VE) per increase in PCO2 was normalized by The impairment of temperature regulation is a recognized
maximal voluntary ventilation (MVV) (i.e., VE / hazard for persons with SC1 (205). Body temperature con-
PCO2/ MVV), thus suggesting that muscle weakness trol in homeotherms is regulated by behavioral and auto-
was the primary factor contributing to the diminished nomic mechanisms. Behavioral mechanisms include
ventilatory response. changing the thermal environment, changing posture, and
The control of a range of ventilatory responses changing the amount of insulating clothing. Physiologic
tidal volume, respiratory frequency, inspiratory and expi- mechanisms include vasomotor adjustment, sweating,
ratory durations, and mean inspiratory airflowdoes not shivering and nonshivering thermogenesis, piloerection,
appear to require afferent pathways from the rib cage and and panting, of which only the first three are of importance
intercostal muscles (200,201). A group of ventilated in humans. Thermoregulatory dysfunction is most severe
quadriplegic subjects detected changes in tidal volume of for persons with a complete cervical SCI, because shiver-
as little as 100 ml just as well as did a group of ventilated ing can only occur above the level of the injury and hypo-
control subjects (202). The sensory information that thalamic control of sympathetically mediated vasomotor
allowed the quadriplegic group to detect changes in lung control and sweating is lost below the level of the lesion.
volume could arise from visceral lung afferents that pro- Some degree of thermoregulation via activation of local or
ject to the CNS in the vagus nerve. Mechanical ventila- spinal vasomotor and sudomotor reflexes has been iden-
tion is known to inhibit inspiratory muscle activity in tified in SCI patients. Nonshivering thermogenesis, or
humans (203). In control and C2 quadriplegic subjects chemical thermogenesis secondary to increases in cellular
with intact sternocleidomastoid (SCM) efferents and metabolism, although important for some animals, has not
afferents, inspiratory effort, as evidenced by electromyo- been clearly demonstrated in humans. Behavioral modifi-
graphic (EMG) activity in the SCM muscle, was elicited cation is an important mechanism for thermoregulation in
by increasing end-tidal PCO2. In both these groups, the persons with complete high-level SCI (206).
onset of inspiratory muscle activity occurred at a higher The classic cold and warm sensors are located in the
end-tidal PCO2 when inspiratory activity was elicited by hypothalamus and the skin; however, there is evidence
increasing the inspired fraction of CO2 (FICO2), than of mesencephalic, medullary, spinal, and intraabdominal
when it was elicited by decreasing the tidal volume or fre- temperature sensors (207). Afferent fibers from periph-
quency during mechanical ventilation. In C1 quadriplegic eral warm and cold receptors, with cell bodies in the dor-
subjects with efferent innervation of the SCM muscle via sal root ganglia, enter the spinal cord and ascend con-
the accessory nerve, but lacking afferent innervation of tralaterally to the medial lemniscus and thalamus and
the SCM, the onset of inspiratory muscle activity occurred have further projections to the hypothalamus (208). The
AUTONOMIC DYSFUNCTION IN SPINAL CORD DISEASE 495
preoptic area of the hypothalamus is implicated as the subjects was less than that reported for hyperthermic
generator of the thermal set point and central integrator spinal cord-intact men. Freund and coworkers (217) sug-
of thermoregulatory responses (208). Efferents from the gested that one reason for the attenuated response of FBF
hypothalamus control thermoregulatory vasomotor and to whole-body heating was diminished thermoregulatory
sudomotor tone as well as nonshivering and shivering effector outflow that resulted from the diminished affer-
thermogenesis via descending noradrenergic and cholin- ent input which, after SCI, could originate only from above
ergic fibers that exit the spinal cord below C7 (208). Vagal the level of the lesion. Tam and colleagues (218) reached
afferents, conveying metabolic information from the a similar conclusion. They reported that a person with T6
periphery to the brain, are important for thermoregula- paraplegia had to achieve a higher core temperature
tion during fever and may also play a role in body tem- threshold (37.2 to 37.9C) to generate sweating and
perature regulation (209). related vasodilatation responses, compared to the core
Cooling the skinregardless of whether central temperature threshold (36.2 to 37.1C) of a normal con-
body temperature changescauses increased heat pro- trol subject. It can be concluded that paraplegic persons
duction via shivering (210). In paraplegic subjects, deep appear to exhibit markedly attenuated skin blood flow
or central temperature receptors sensitive to cold are also inresponse to hyperthermia and thus are limited in their
able to initiate shivering above the level of the SCI, and ability to dissipate excess heat (217).
these receptors can act independently of the temperature Conscious control of behavior depends on sensory
of the skin above the SCI (210). Researchers have postu- appreciation of temperature. In one study, paraplegic sub-
lated that in normal humans, these central cold receptors jects did feel hot as their oral temperature was raised 1
may be backup mechanism that comes into play if there to 1.5C by heating insensate skin (217). However, in the
is a loss or diminution of skin cold receptor reflex activ- case of one T8 paraplegic individual, researchers reported
ity (210). In humans, shivering does not occur below the that a fall in central temperature to 36.2C was not asso-
level of a complete SCI, and therefore shivering is thought ciated with a conscious appreciation of cold (219). The
not to be a spinal reflex (210). perception of warm and cold after SCI appears to depend
Cooling of peripheral or central areas results in a on the temperature of the sentient skin.
reduction of skin blood flow and a simultaneous increase The primary and principal stimulus that elicits the
in flow to central vascular regions (208). In control sub- thermoregulatory sweating response is a change in core
jects, cooling or warming of one hand elicits a cutaneous temperature (220). Sweat glands receive dual innervation
vasomotor response of the contralateral hand and both by both cholinergic and adrenergic fibers and are stimu-
legs (211). Several investigations (211213) have failed to lated by cholinergic, -adrenergic, and -adrenergic ago-
observe a similar cutaneous vasomotor response below the nists; however, cholinergic stimulation provokes the
level of SCI; others have observed vasomotor responses largest response (221). Spinal segments T2 to T4 supply
below the level of SCI in both primates and humans sweat glands on the head and neck, T2 to T8 to glands
(214216). Tsai and coworkers (214) reported that all of the upper limbs, T-6 to T-10 to the trunk, and T11 to
vasomotor responses to cooling or warming in the para- L2 to the lower extremities (221). Normell (222) provides
plegic lower extremities of men were absent following an outline of the segmental arrangement of the ther-
acute SCI (T5 to T11), but by 4 months after injury the moregulatory vasomotor innervation of the skin of the
ipsilateral local vasomotor responses to warming and cool- trunk and lower extremities. Autonomic dermatomes
ing in the paraplegic lower extremities returned to normal, overlap several segments above and below the somatic
and by 18 months after injury, the crossed vasomotor level. In several reports, spinal lesions are associated with
reflex to cooling and warming recovered to normal. anhidrosis below the level of the lesion (221,222). Nor-
Paraplegic men have been shown to have significant mal evaporative cooling is maintained in persons with
differences in skin blood flow response to hyperthermia spinal injury by increased compensatory sweating from
when compared to intact control subjects (217). When sentient skin (220,221).
only insensate skin was heated (to 40C) in paraplegic sub- Reflex sweating occurs below the level of lesion in
jects, few or no increases in forearm blood flow (FBF) SCI during autonomic hyperreflexia. There is also evi-
occurred, even with an elevation of oral temperature of dence for thermal reflex sweating below the level of SCI
1 to 1.5C over 59 to 71 minutes, although the subjects in humans. A 1961 study by Seckendorf and Randall
exhibited mild sweating on the upper body. In contrast, a demonstrated low-intensity sweating in five patients with
normal subject exhibited vigorous vaso- and sudomotor anatomically complete lesions of the spinal cord (T3T8)
responses to the same pattern of heating that was given (223). In 1991, Silver, Randall, and Guttmann recorded
to the paraplegic subject. With whole-body heating of sweat responses of nine patients with physiologically
paraplegic subjects, all but one exhibited sweating above complete lesions of the spinal cord, including six with cer-
the level of the spinal cord lesion, and FBF increased in all vical lesions (224). Although sweating (up to 30 percent
subjects. However, the increase in FBF in the paraplegic of what would be expected in controls) occurred on the
496 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
entire cutaneous surface below the level of SCI in every the rostral ventrolateral medulla mediate hypertension induced
by carotid body chemoreceptor stimulation. Naunyn Schmiede-
patient in response to environmental heating, the sweat- bergs Arch Pharmacol 1994; 349:549554.
ing was not sufficient to prevent an elevation in body tem- 14. Somers VK, Mark AL, Zavala DC, Abboud FM. Contrasting
perature in patients with cervical SCI. effects of hypoxia and hypercapnia on ventilation and sympa-
thetic activity in humans. J Appl Physiol 1989;67:2101-6.
In quadriplegic subjects, sympathetic skin nerve 15. Somers VK, Mark AL, Zavala DC, Abboud FM. Influence of ven-
recordings of vasoconstrictor and sudomotor impulses, tilation and hypocapnia on sympathetic nerve responses to
from below the level of SCI made while ambient temper- hypoxia in normal humans. J Appl Physiol 1989; 67:20952100.
16. Banzett RB, Lansing RW, Reid MB, Adams L, Brown R. Air
ature was varied, demonstrated no changes in sympa- hunger arising from increased PCO2 in mechanically ventilated
thetic outflow despite cooling that reduced tympanic quadriplegics. Respir Physiol 1989; 76:5367.
temperature 2C (225). During this study, abdominal 17. Downey JA, Chiodi HP, Miller JM, III. The effect of inhalation
of 5 percent carbon dioxide in air on postural hypotension in
pressure over the bladder, as well as mechanical and elec- quadriplegia. Arch Phys Med Rehabil 1966; 47:422426.
trical skin stimulation applied distal to the level of SCI, 18. Hilton S. The central nervous contribution to vasomotor tone.
induced bursts of neural impulses recorded in the sym- In: Magro A, North Atlantic Treaty Organization, Scientific
Affairs Division, (eds.), Central and Peripheral Mechanisms of
pathetic skin nerve fascicles also below the level of SCI, Cardiovascular Regulation. New York: Plenum Press, 1986;
indicating the presence of spinal vesicosympathetic and 465486.
somatosympathetic reflexes. It has long been noted that 19. Yardley CP, Fitzsimons CL, Weaver LC. Cardiac and peripheral
vascular contributions to hypotension in spinal cats. Am J Phys-
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21. Mannard A, Polosa C. Analysis of background firing of single
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36 Syringomyelia
degrees residual kyphosis and more than 25 percent canal teroften unilaterally (27). The longitudinal fluid dis-
stenosis are twice as likely to develop a posttraumatic section may result from pulsatile increases in subarach-
syrinx (11). Cord tension may pull open cystic cavities noid fluid pressure caused by cough, sneeze, straining or
and traction cord vessels causing gray matter ischemia; Valsalva, Cred pressure over abdomen, weight lifting,
recurrent gray matter infarction may coalesce into a forward-lean pressure release, and quad coughing
syrinx (10,11,21). Not only SCI, but also other spinal (19,2932). These intraabdominal or intrathoracic pres-
cord pathology (e.g., spinal cord tumors, spinal stenosis, sure surges are transmitted via the valveless inferior vena
infectious meningitis, disc protrusion, spinal cord arteri- cava and azygous veins to the epidural venous plexus;
ovenous malformation) can predispose to syringomyelia, subarachnoid pressure either surges pump fluid into the
perhaps also by tethering the spinal cord, causing spinal syrinx cavity via perivascular spaces (VirchowRobin
cord ischemia, and impairing CSF flow in the subarach- spaces; 26,28,33) or causes fluid in the syrinx to slosh
noid space. Uncommon traumatic cyst-to-subarachnoid rostrally (24,34). In an ovoid syrinx, pressure is greatest
fistulas may allow the more direct transmission of sub- where the curvature is greatestthus, syrinxes extend at
arachnoid pressure surges to the cyst and lead to more the poles and become long tubular structures (35). The
rapid syrinx development (2,5,23). perivascular spaces of VirchowRobin may act as one-
A variety of factors may contribute to syrinx way valves, allowing fluid from subarachnoid space into
enlargement. Posttraumatic syrinxes, as a type of non- the syrinx but limiting egress; this may explain the high
communicating syrinx, do not communicate with the intrasyrinx pressures and protein concentrations in some
fourth ventricle or with the central canal (2426); rather, syrinxes (4,36). The dilated spinal cord may also be com-
they likely arise from posttraumatic cysts at the zone of pressed against spondolytic spurs, thus leading to addi-
cord injury that take on fluid from the subarachnoid tional neurologic decline (37).
space, increase in pressure, and extend rostrally and/or The symptoms and signs of early PTS are often non-
caudally by dissecting through intermediate gray mat- specific; even some patients with markedly elongated
A B C D
FIGURE 36.1
MRI of posttraumatic syrinx. Serial magnetic resonance images are shown of a posttraumatic syrinx in a 57-year-old male with
T3 traumatic paraplegia, who developed L hand weakness and pain at 8 months post-SCI; examination revealed loss of tendon
reflexes and loss of pinprick sensation in the L upper limb. The initial MRI (A) showed a large dilating syrinx ascending rostrally
from the thoracic level of SCI through the cervical spinal cord to the medulla. He underwent arachnoid dissection/duraplasty at
12 months post-SCI with gradual resolution of his L hand weakness and partial resolution of pain. Despite sustained recovery of
L hand strength and improvement in pain symptoms, follow-up MRIs at 21 months (B,C) and 30 months (D) post-SCI showed
a persisting, dilating syrinx extending rostrally to the medulla. MRI images A, B, and D are T2-weighted; image C is T1-weighted.
SYRINGOMYELIA 503
syrinxes that dilate the spinal cord may have minimal (7,11,42), CSF flow from cardiac-gated images (43),
symptoms (Figure 36.1). The symptoms and signs are poorly demarcated T2-weighted signal hyperintensity at
often unilateral early in PTS (4,7,38), consistent with a the rostral extent (44), flow void sign on T2-weighted
unilateral syrinx on MRI; they may become bilateral later. images suggesting high pressure (22), and association
Pain is a common presenting symptom and is seen in 36 with spinal stenosis (11). MRI limitations are that metal
to 80 percent of PTS patients (46,38). The pain is often may distort cord images (e.g., rods, wires, plates, screws,
localized to the site of the original injury or may radiate bullet fragments, etc.) and, rarely, false-positive and false-
to the neck or upper limbs. The pain is often aching or negative MRIs for syrinxes may occur. False-positive MRI
burning and aggravated by coughing, sneezing, straining, diagnosis may be caused by myelomalacia (i.e., ischemic
postural change, or body vibration. Ascending loss of cord damage/gliosis and microcystic degeneration), cord
deep tendon reflexes and ascending loss of pain and tem- hemorrhage, and tumor (29,45,46). False-negative MRIs
perature sensation are common early signs (46,39). The for PTS can occur when the signal density of the pro-
ascending reflex and sensory loss is often unilateral, con- teinaceous syrinx fluid is similar to neural tissue (46).
sistent with a unilateral syrinx on MRI. Loss of pain and Gadolinium, as an intravenous contrast agent, can
temperature sensation with preserved touch, position, and improve the distinction between syrinxes and intra- or
vibration sense is referred to as dissociated sensory loss extramedullary tumors, arteriovenous malformation, and
(46,39); it is caused by a disruption of crossing multiple sclerosis (MS) plaques. CSF velocity imaging,
spinothalamic fibers by the syrinx but sparing of poste- using images gated to the cardiac cycle, can aid in distin-
rior column fibers. Worsening weakness is also common guishing syrinxes from cystic myelomalacia and arach-
in PTS (46,39,40) but typically develops after ascend- noid cysts.
ing sensory loss (7,18). Muscle fasciculations and atro- Other imaging studies may be useful for evaluating
phy are noted later. PTS and for distinguishing it from other progressive
Less-common findings are increased or decreased myelopathies. Conventional spine X-rays with flexion
spasms, hyperhidrosis, loss of reflex bladder emptying, and extension views may help diagnose spinal instabil-
worsening orthostatic hypotension, scoliosis, central ity, disc space infection, and spinal stenosis. Metrizamide
and/or obstructive sleep apnea, new Horners syndrome, myelogram with immediate and delayed CT scans can aid
reduced respiratory drive, impaired vagal cardiovascular diagnosis of PTS, arachnoiditis, disc herniation, etc. (47).
reflexes, and sudden death (4,41). With high cervical and Electrodiagnostic findings in posttraumatic
brainstem involvement by the syrinx, diaphragm paraly- syringomyelia include features of mixed upper and lower
sis, sensory loss in the trigeminal nerve distribution, dys- motoneuron involvement (4,48,49). Early findings are: a)
phagia, dysphonia, and nystagmus may be seen. Loss of prolonged segmental conduction on F-wave studies, and
protective pain and temperature sensation can lead to b) prolonged descending motor conduction on motor
neurogenic arthropathy, pressure sores, and burns. evoked potential (MEP) testing. Later electrodiagnostic
Syringomyelia is variable in its presentation and in findings include a) reduced compound muscle action
its natural history. Long, dilating, rapid-onset syrinxes are potential (CMAP) amplitude; b) enlarged motor-unit
most symptomatic. These manifest as weakness and loss action potential (MUAP) amplitude, consistent with
of function, but some long and dilated syrinxes manifest motoneuron loss and compensatory motor axon sprout-
minimal symptoms. Syrinx size on MRI does not corre- ing by spared motoneurons; and c) reduced maximal fir-
late with symptoms or degree of disability. Short dilating ing rate of motor units during maximal voluntary effort,
syrinxes may manifest as weakness and loss of function, consistent with reduced upper motoneuron input to
particularly in lower limbs of those with incomplete SCI. motoneurons. Sensory nerve action potentials (SNAPs)
Short or long, dilating or nondilating syrinxes can cause are preserved, except when there is associated peripheral
neuropathic pain. Regarding natural history, syrinxes can nerve entrapment, which is common. Muscle membrane
spontaneously resolve, worsen then plateau, worsen inter- instability is a late finding, likely because lower motoneu-
mittently, or worsen continuously. Syrinxes with an early ron loss is gradual and there is ongoing compensatory
onset post-SCI often worsen most rapidly. sprouting to minimize persisting denervation in a muscle.
Magnetic resonance imaging (MRI) is usually defin- Neurologic monitoring guides treatment. Large
itive for diagnosing posttraumatic syringomyelia. An syrinxes on MRI may be minimally symptomatic. Some
intramedullary cyst with well-defined margins, which is argue to surgically decompress the syrinx only when the
eccentric, extends beyond the limits of the original cord patient becomes symptomatic. Others warn that early
injury, does not communicate with the central canal or treatment is needed or neuronal loss may advance silently
the fourth ventricle, and is isodense with CSF (i.e., low- and become irreversible. If nonoperative treatment is cho-
density T1-weighted image, high-density T2-weighted sen, then close neurologic monitoring is needed to assure
image) is characteristic. Syrinx MRI features that are asso- that the patient is neurologically stable. The clinical neu-
ciated with neurologic decline include long and wide rologic examination may not be sufficiently sensitive to
504 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
detect neurologic decline before irreversible damage has delayed (5,29,55). Surgical options include a) syringos-
occurred. Neurologic monitoring can include pinprick tomy to drain the syrinx (56); b) shunting (syringo-
self-examination by the patient, serial quantitative subarachnoid, syringo-pleural, or syringo-peritoneal;
strength testing, serial electrodiagnostic studies (F-waves, 29,5560); c) dissection of arachnoidal scar and
MEPs, EMG); and serial MRI. Neurologic monitoring is duraplasty to reconstruct the subarachnoid space
also needed postoperatively to assure persisting benefit (6163); and d) cordectomy (33,36,55).
from the syrinx surgical decompression. Nonoperative treatment is favored by some, if there
Posttraumatic syringomyelia can lead to a neuro- is no apparent or minimal neurologic decline (6,7,51,52),
logic decline of sufficient degree to cause functional loss. although in most studies, follow-up is for less than 10
In severe cases, a fully independent person with paraple- years. Rossier and colleagues (4) stated ...a patient, if fol-
gia may decline to tetraplegia with total dependence for lowed long enough, will eventually show some signs of
transfers and self-care (3,50). Another common scenario progression. Syrinx patients who are managed conserv-
for functional loss in posttraumatic syringomyelia is the atively must be monitored closely; if they show significant
incomplete tetraplegic patient; such persons regain walk- decline, then prompt surgical treatment may be needed.
ing and bowel/bladder control during initial rehabilita- Surgery yields improved strength and improved pain
tion and then lose walking and bowel/bladder control control in some but not all, whereas sensation does not
years later because of a posttraumatic syrinx. In addition usually recover. Reduced syrinx size on postoperative MRI
to disability from either ascending cord involvement or predicts a good surgical result (65), but complete syrinx
long-tract cord involvement in those with incomplete SCI, resolution is not essential for a good clinical outcome. With
other factors can contribute to functional loss in PTS, either shunting or arachnoid dissection/duraplasty, there
including severe neuropathic pain, extensor spasms and is recurrence of neurologic decline by 5 years in as many
neuropathic joints. Noncystic myelopathy, such as myelo- as 50 percent of patients (55,63). In some, the failure is
malacia and cord tethering, may also cause pain, sensory caused by a recurrence of the syrinx; shunts often occlude
loss, and weakness, but the degree to which they cause because of a collapse of the syrinx cavity around the
functional decline is less clearly described. catheter tip with subsequent glial ingrowth into the shunt
Conservative treatments have been advocated for openings (62). Shunts or duraplasty may fail even though
posttraumatic syringomyelia (6,7,51,52); however, they are the syrinx remains decompressed on MRI; cord tethering
not well studied. Conservative treatment is often clinical by the shunt or recurrent arachnoidal scarring may con-
observation without any specific intervention, but may also tribute. Walking SCI patients (i.e., ASIA D or E) are at
include activity precautions, altering spinal fluid dynamics risk for neurologic decline after shunting (6466); perhaps
by reducing CSF production and by lowering venous pres- the shunt tube compromises descending motor axons adja-
sures, percutaneous drainage of the syrinx, assuring no sec- cent to the syrinx. Ambulatory SCI patients may benefit
ondary causes of myelopathy, and providing ongoing reha- most from conservative treatment or arachnoid dissection
bilitation. Activity precautions include avoid maneuvers or duraplasty. Complete SCI (ASIA A) persons may bene-
that transmit venous pressures to the subarachnoid space: fit from cordectomy, which yields long-term benefit in
high-force exercise, Valsalva and Cred, quad coughing most (33,55).
with direct compression over the inferior vena cava, and
forward-lean pressure releases. Altering spinal fluid dynam-
ics may include agents to reduce CSF production, head-of- CHIARI MALFORMATION CAUSING
bed elevation to 20 degrees at night to lessen rostral slosh SYRINGOMYELIA
of fluid in the syrinx, agents to promote venous dilation
and lower venous pressures, and agents to lessen daytime Syrinxes that are associated with congenital foramen mag-
fluid accumulation caused by orthostatic hypotension. num encroachment (e.g., Chiari I or Chiari II malforma-
Other treatable causes of myelopathy should be addressed, tion) may result from either central canal dilation (i.e.,
including B12 deficiency and spinal stenosis. Percutaneous communicating syringomyelia or hydromyelia) or from an
tap of the syrinx under CT guidance can provide benefit eccentric syrinx cavity in the gray matter of the cord (non-
(53), but often fluid reaccumulates rapidly (54). Perhaps a communicating syringomyelia) (1,6670). Because the two
combination of conservative measures can prevent neuro- types of syrinxes are often indistinguishable by imaging
logic decline without surgery, but this has not been estab- studies, particularly later in the disease, they may be
lished in clinical studies. referred to as syringohydromyelia or hydrosyringomyelia.
Surgical treatment is usually indicated if there is Chiari I malformation is a congenital downward dis-
ongoing or rapid neurologic decline. Surgery should not placement of the cerebellar tonsils through the foramen
be delayed until there is overt weakness; if weakness magnum, into the cervical subarachnoid space. Chiari II
develops, then prompt surgery should be considered, malformation is downward displacement of the cerebel-
because deficits can become irreversible if surgery is lar vermis, pons, and medulla into the foramen magnum
SYRINGOMYELIA 505
and elongation of the fourth ventricle. Chiari I malforma- retract upward with age, MRI interpretation depends on
tion typically presents clinically in young adults, whereas age-appropriate controls. Tonsilar herniations greater
Chiari II malformation presents in infants and is usually than 6 mm are significant up to age 10, greater than 5 mm
associated with myelomeningocoele and hydrocephalus. for ages 10 to 30, and greater than 4 mm for ages over
Chiari I malformation is the most common cause of 30. However, 30 percent of persons with cerebellar her-
syringomyelia. Although it usually presents clinically in niations of 5 to 10 mm are asymptomatic.
young adult years, it may manifest first in an infant or Differential diagnosis for syringomyelia of Chiari I
older adult (1,71,72). The duration from onset of symp- malformation includes multiple sclerosis (MS); spinal
toms to diagnosis is typically 3 to 7 years (1). The earliest muscular atrophy, amyotrophic lateral sclerosis (ALS);
symptom is often headache or neck or arm pain aggravated spinocerebellar ataxias, cervical disc and posttraumatic
by straining or cough; neck pain may be accompanied by syringomyelia from trauma, arteriovenous malformation,
torticollis. Neurologic findings depend on the structures arachnoiditis or meningitis, neurofibromatosis, and from
involved. It is sometimes difficult to distinguish direct fora- spinal cord or brainstem tumors (e.g., ependymoma,
men magnum compression from cervical syrinx compro- astrocytoma, hemangioblastoma).
mise. The syrinx is often noted at the C46 bony levels but Treatment of Chiari I malformation involves poste-
may extend rostrally or caudally the full length of the cord. rior fossa decompression with a suboccipital craniectomy,
Dissociated sensory loss in a capelike distribution over the with or without dural patch grafting, and cervical
neck and arms is characteristic (i.e., loss of pain and tem- laminectomy with fenestration or shunting of the syrinx
perature sense with sparing of touch, vibration, and posi- cavity. In patients with mild symptoms, 70 to 80 percent
tion sense); dissociated sensory loss results because cross- report improvement (7476). Some suggest that direct
ing spinothalamic tract fibers carrying pain and surgery of the syrinx should be undertaken only after
temperature sensation are most affected, whereas poste- craniocervical decompression has failed (1,74,75). A
rior column sensory fibers are spared. Hand and arm top-down approach has been suggested for surgical
weakness develop as lower motoneurons of the cervical treatment of Chiari II malformation: shunt for hydro-
cord are affected. Long-tract myelopathic symptoms cephalus, then posterior fossa decompression for Chiari
develop in the lower limbs with further expansion of the malformation, then syringo-pleural shunt for
cervical syrinx. Worsening scoliosis is common, particu- syringomyelia if needed. Surgical decompression of the
larly in childhood-onset syringomyelia of Chiari I malfor- posterior fossa typically involves occipital craniectomy,
mation. Other associated craniocervical abnormalities of laminectomies of C1 and C2, lysis of arachnoidal adhe-
Chiari I malformation may include basilar impression, sions, and generous fascia lata dural grafting to attempt
KlippelFeil anomaly (congenital fusion of cervical verte- to restore CSF flow across the foramen magnum (1).
brae); and atlanto-occipital assimilation. Syrinx extension Surgery for syringohydromyelia is as described for post-
into the brainstem can lead to sleep apnea, eye movement traumatic syrinxes: myelotomy, shunting, or dural graft.
abnormalities (e.g., nystagmus, oscillopsia); dysphagia, After posterior decompression, with or without syrinx
aspiration, vertigo, truncal ataxia, and poor balance. shunting, 50 percent or more patients improve; in those
Chiari II malformation often presents in infancy as with syrinxes, about one-third improve, one-third stabi-
stridor, weak cry, nystagmus, and apnea or in childhood lize, and one-third deteriorate further after surgery (1).
as gait abnormality, spasms, worsening incoordination, Postoperative neurologic decline can result from recur-
and nystagmus. Later, it may lead to loss of head con- rent syringomyelia, occipital C1C2 instability,
trol, arm weakness, spasms, and tetraparesis. pseudomeningocoele, tethering of the spinal cord, menin-
Syringohydromyelia may arise from fluctuations in gitis, and extradural abscess (1,7476).
subarachnoid space pressure, which create hydrostatic Chiari II malformation that becomes symptomatic
forces that drive CSF into the spinal cord (73). The causes in infancy is the leading cause of death in children with
of these alterations in CSF pressure include bony nar- myelomeningocele (1). Respiratory compromise and
rowing of the foramen magnum, as in achondroplasia or recurrent aspiration are particularly worrisome. Less-
basilar invagination, Chiari I and II malformations, severe symptoms may stabilize by 1 year of age. Older
intramedullary and extramedullary tumors, and sub- children with Chiari II malformation do better with cer-
arachnoid scarring secondary to trauma, hemorrhage, or vical spinal cord decompression; improvement is reported
infection. In patients with Chiari II malformations, hydro- in 60 to 80 percent of patients.
cephalus may contribute to syrinx formation. Rostral or
caudal extension of the syrinx may result from rapid
changes in intraspinal pressure, such as those caused by CONCLUSIONS
coughing, straining, or sneezing.
MRI confirms the diagnosis, but asymptomatic ton- Syringomyelia, whether caused by a congenital Chiari
silar herniation is common. Because cerebellar tonsils malformation or a spinal cord injury, can be severely dis-
506 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
abling, even fatal; yet, some patients are stable for 16. Silberstein M, Hennessy O. Cystic cord lesions and neurological
deterioration in spinal cord injury. Paraplegia 1992; 30:661668.
decades. Syrinxes should be diagnosed early by identify- 17. Bursell J, Little JW, Stiens SA. Electrodiagnosis in spinal cord
ing the characteristic dissociated sensory loss and tendon injured patients with new weakness or sensory loss. Arch Phys
reflex loss and by obtaining an MRI. If little or no ongo- Med Rehabil 1999; 80:904909.
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37
Dual Diagnosis:
Spinal Cord Injury
and Brain Injury
ehabilitation after spinal cord injury of the post-injury course of recovery is important because
limited pre-injury education, along with motivational deceleration trauma, history of loss of consciousness,
problems, severe depression, pain, fatigue, and preoccu- presence of neurologic indicators, and the need for ven-
pation with adjustment, may impair neuropsychologic tilatory support. Sixty-four percent of these high-risk
test performance (47). The results of neurologic evalu- SCI subjects were rated as mildly impaired or worse. The
ation for TBI may be normal in SCI patients, however, patients who had pre SCI learning disabilities (one-third
neuropsychologic examination may reveal cognitive of the sample) had scores reflecting the greatest impair-
deficits. These tests are sensitive to problems of attention, ment. The group with cognitive deficits but no preinjury
concentration, memory, and judgment in these patients. learning disabilities was found to be the most likely to
The reported prevalence figures for cognitive deficits have sustained TBI at the time of SCI. Alker (26) found
in patients with SCI vary, depending on with definition of a 16 percent incidence (10/61) of concurrent cerebral and
cognitive deficits is used and on the specific procedures cervical injury in a 1975 review of 146 patients who died
that are used for assessment and measurement in a par- at the scene or in the emergency room shortly after arrival.
ticular study. According to a number of studies (216), Roth (2) and associates administered a comprehensive
40 to 60 percent of acute SCI patients demonstrate cog- motor-free neuropsychologic test battery to eighty-one
nitive dysfunction. These cognitive deficits result from acute SCI patients and sixty-one controls matched on age,
various forms of cerebral damage, including concurrent gender, level of education, and geographic location.
or premorbid traumatic brain injury (TBI), chronic alco- Patients were tested a mean of 72 days post-injury. The
hol or substance abuse, premorbid learning disability, the results of this study suggested that patients had general-
use of psychoactive medication or motivational or other ized cognitive deficits. Although attention, concentration,
problems. Other studies have demonstrated similar find- and initial learning were often impaired, long-term mem-
ings that associate the rate of TBI in SCI populations, ory for well-established verbal material was relatively pre-
when SCI is the primary injury (1725). served. Abilities to acquire new knowledge, adapt to new
When TBI is the primary diagnosis, the incidence of situations, and shift to new mental sets also were rela-
SCI is much lower than the incidence of TBI when the pri- tively impaired, as was word retrieval and syntax use.
mary diagnosis is SCI. Data from the Spinal Cord Model Shrago (19) reviewed the records of fifty survivors
Systems report that 28.2 percent of SCI patients have at of highway accidents with injury to the cervical spine and
least a minor brain injury with loss of consciousness, reported a 53 percent incidence of concurrent head trauma
whereas 11.5 percent have a TBI severe enough to demon- when the upper cervical spine was injured and a 9 percent
strate cognitive or behavioral changes (24). rate when the damage occurred at lower levels. Head
Several investigators have reported the magnitude trauma was not well defined in this paper and included
and nature of neuropsychologic test abnormalities among contusions, abrasions, lacerations, or skull fractures.
SCI patients using a broad range of assessments of cogni- Bivins (27) reported a series of seventeen victims of
tive function. Unfortunately, applicability of the findings cardiorespiratory arrest seen in the emergency department
from many of these studies has been limited. The distinc- following blunt trauma and found a 24 percent incidence
tion between the diagnosis of a concomitant TBI, which of injury to the cervical spine. It is possible that the more
is a specific diagnosis, and cognitive deficits, which is a serious head injuries do not survive the dangerous aggra-
more general problem with any potential contributing fac- vation of depressed respiration following the head injury
tors is often ignored. It is important to separate these two by decreased ventilation resulting from the paralysis of
issues, because they have different definitions, frequencies, respiratory muscles by the spinal injury.
and most important, implications (21). Bucholz (28) reported a postmortem series of one
Davidoff and associates (6), in one of the first stud- hundred consecutive patients dying in motor vehicle acci-
ies of the prevalence of cognitive deficits in SCI patients, dents. He found twenty-four cervical injuries (24 percent),
found that, based on the clinical histories of impaired con- he found that twenty were located between the occiput
sciousness and posttraumatic amnesia at the time of and the axis. There was no statistically significant differ-
injury, twelve (40 percent) of the thirty patients had sus- ence in associated injuries, including cerebral damage,
tained mild TBI concurrently with SCI. Six of the remain- between patients with cervical injury and those without
ing eighteen, or 20 percent of the entire sample, had a pre- such injury. Porter (29) found that in sixty-two (29 per-
morbid history of TBI. Only twelve patients (40 percent) cent) of 216 nonacute SCI mortalities there was evidence
had no history of TBI at any time, as defined by loss of of significant associated injury to the brain or skull on
consciousness, posttraumatic amnesia, or both, before pathologic examination. Davis (30) and associates
or at the time of SCI. Wilmot and associates (7) studied reported that 46 percent of autopsied spinal cord injuries
sixty-seven SCI patients who were considered at high risk had demonstrated pathology of the brain. Most cases
for what they called occult head trauma. High risk were upper as compared to lower cervical lesions. Young
patients were defined as those who met one or more of (31) recorded major head injuries coincident with para-
the four selection criteria, which were quadriplegia from plegia at 16 percent and with quadriplegia, 7 percent.
DUAL DIAGNOSIS: SPINAL CORD INJURY AND BRAIN INJURY 511
Wagner and associates (32) found that of those with head ria groups used in the studies reviewed above. The
injuries, complete tetraplegic individuals had severe head absence of preinjury measures of cognitive performance
injuries, as clinically assessed at 24 hours, whereas com- in all of these studies is the reason for the limited applic-
plete paraplegic individuals had mild injuries. Patients ability of these studies. In addition, there are indications
with incomplete lesions also sustained head injuries but that the SCI population as a whole tends to function intel-
these more often were mild in both paraplegic and lectually in the low average range, even when there is no
tetraplegic individuals. Numerous authors have stressed evidence of cognitive impairment (3,38).
that the cervical spine injuries seen in association with Occult head injury is often not diagnosed until the
head injury primarily involve the atlanto-occipital junc- patient is in the rehabilitation setting. The head injury may
tion, C1, or C2. Damage to the cervical spine cord at this first be apparent when the patient demonstrates an inabil-
level is generally fatal and may account for the greater ity to learn care because of cognitive deficits such as
rate of combined injury seen in autopsy reviews. impaired new learning, impaired short term memory, and
It has been asserted that mood can affect attention, the inability to organize information. The presence of a
concentration, memory, and other cognitive functions. head injury dramatically complicates the patients rehabil-
This is a particularly relevant factor in SCI patients, who itation. Cognitive deficits will limit or complicate the
have a depression rate estimated about one-third (3335). patients ability to adapt to physical limitations, learn com-
Richards and associates (36), in their study of thirty-one pensatory skills, and achieve a higher level of independence,
SCI inpatients, noted that deficits in arousal and perfor- as compared to those with SCI only. Behavioral dysregu-
mance did not show up strongly in an unnatural and lation is a sequela of head injury. The resultant emotional
deprived environment. However, they believed that depres- liability, poor impulse control, noncompliance, and
sion or anxiety might be major contributing factors in per- decreased insight will impair the patients ability to fully
formance and arousal deficits in recently injured patients. appreciate the situation and proceed through the stages of
Davidoff and associates (37) found a minimal relationship adjustment to disability. The patient with a dual diagnosis
between neuropsychologic performance and depression. of head injury and SCI usually has a longer length of stay.
Their findings suggest that the magnitude and nature of This is partially caused by the impact of cognitive deficits
cognitive deficits is unrelated to the presence or severity on the ability to learn care. The impact of cognitive deficits
of depression in SCI patients, and that these two psycho- and behavioral dysregulation in preventing these individ-
logic issues should be treated as separate problems. uals from independently managing their own care continue
To identify the incidence of cognitive deficits in SCI after discharge (even if the brain injury has been identified).
patients with no obvious TBI, Kreutzer and associates (10) Once discharged from the rehabilitation setting these indi-
conducted neuropsychologic testing of sixty-two consec- viduals are at greater risk for hospital readmission, UTIs,
utive SCI patients. Their testing revealed a variety of bowel impaction, skin breakdown, substance abuse, non-
deficits, when compared with the normal ranges reported compliance with care, depression, and delayed adjustment
in the neuropsychologic testing literature. Impairments to injury (3942). For the patient with an undiagnosed head
were found to be most prevalent in visual learning, verbal injury, the incidence of these complications is not only
learning, visual organization, and attention. greater, but also guaranteed. A comprehensive, multidisci-
To determine the relative contribution that con- plinary approach is crucial to form a successful long-term
comitant TBI and alcohol or substance abuse may have plan of care which addresses this patients complex needs.
on cognitive performance, Davidoff and associates (9) To avoid complications, treatment guidelines must be cus-
administered a comprehensive motor-free neuropsycho- tomized, with great attention paid to the patients specific
logic test battery to 116 patients with acute SCI and 69 cognitive limitations.
control subjects. The SCI patients were divided into three Several considerations are necessary to perform a
groups: SCI only, SCI-TBI, and SCI-abuse (alcohol or proper and thorough assessment of neuropsychologic
drug abuse history with no concomitant TBI). Statistically functioning in SCI patients. Factors such as reduced or
significant differences were found between all patient and absent upper-extremity function, poor endurance, and
control mean scores for all but three of the eighteen tests limited sitting tolerance require that appropriate modifi-
used. Impairments were present in the SCI-abuse group, cations be made in the design and administration of a
suggesting chronic deficits in this group of patients. comprehensive test battery.
In summary, the coincidence of SCI and cognitive
impairment has been demonstrated to vary depending on
which definition of cognitive impairment is used. Various RISK FACTORS FOR CONCOMITANT SPINAL
authors have used the presence or absence of posttrau- CORD INJURY AND BRAIN INJURY
matic amnesia, loss of consciousness, neuropsychologic
test scores, clinical ratings, and etiology of injury, either As in SCI, in TBI, males between the ages 15 and 35 years
alone or in varying combinations, to establish the crite- were primarily affected. Alcohol and/or drug use com-
512 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
bined with impulsive risk-taking behaviors are some of DEGREES OF BRAIN INJURY
the personality traits found to be involved in the major-
ity of these injuries. Also alcohol intoxication is a fre- Mild head injury is the most common type of brain injury.
quent factor at the onset of injury (39). The mechanism The Glasgow Coma Scale (GCS) includes score of 1315
of injury varies and is regional. In urban areas, motor at lowest point after resuscitation, with no loss of con-
vehicle accidents (MVAs), acts of violence, and falls pre- scious or brief loss of conscious that lasts less than 20
dominate, whereas farming injuries and falls are seen in minutes, and a normal CT scan or MRI of the brain.
rural areas. Beach communities and summer months have Moderate head injury, which corresponds to a lower GCS
a large number of surfing, skiing, and recreation-related score of 9 to 12, denotes that the patients lowest level of
injuries. Ethnic breakdown varies and is regional consciousness could be described as combative or lethar-
(3941). gic. The patient may or may not have followed com-
mands, but did not respond appropriately to questions.
Severe head injury corresponds to a lowest GCS score of
BIOMECHANICS OF DUAL INJURY 8 or lower (3 is the minimum).
Hypoxia, which results from ischemia, disrupts aer- tive of the Brain Trauma Foundation, the American Asso-
obic oxidative respiration. The following mechanisms have ciation of Neurological Surgeons, and the Joint Section
been proposed as disruptive to cell membranes: progressive on Neurotrauma and Critical Care. These guidelines will
loss of phospholipids, cytoskeletal abnormalities, reactive be summarized (54).
oxygen species, lipid breakdown products, and loss of intra-
cellular amino acids. Prolonged ischemia contributes to per-
Resuscitation of Blood Pressure and
manent damage of the cell membrane, irreversible injury,
Oxygenation
and cell death. Key events in any ischemic lesions include
energy depletion and activation of anaerobic glucolysis. Post injury hypotension or hypoxia increases morbidity
Cytoskeletal integrity and homeostatic mechanisms are dis- and mortality. The guidelines define hypotension as sys-
rupted and contribute to the cellular death because of the tolic blood pressure (SBP) 90 mmHg, and hypoxia as
presence of excess calcium, sodium, and chloride (5355). apnea, cyanosis, or PaO2 60 mmHg. Treatment rec-
Hypoxia and perfusion status play a significant role in the ommendations are to maintain mean arterial pressure
secondary injury process in TBI and SCI. After an ischemic 90 mmHg to maintain cerebral perfusion 70 mm Hg.
insult, the complex buildup of metabolic products, such
as free radicals, dysregulation of calcium, and excitatory
Intracranial Pressure Monitoring
neurotransmitters, contributes to the disruption of neuronal
cellular integrity. The death of cells is the final and ultimate The aggressive treatment of elevated ICP has become crit-
result of cellular injury and occurs by two distinct processes: ical to acute brain injury management. Normal ICP is 0
necrosis and apoptosis. In necrosis there is a rapid decline to 10 mmHg. Most use 20 to 25 mmHg as the arbitrary
in the ability of the cell to maintain homeostasis, and it upper limit, but it may vary among medical centers. Effec-
quickly becomes swollen and disintegrates, releasing its tive management of elevated ICP (defined as 2025
content. Apoptosis occurs as a normal process during torr) has been shown to have significant impact on out-
which abnormal and defective cells are removed. Apopto- come (5558). ICP monitoring is not without risks and
sis occurs in more orderly fashion because it is a gene-reg- is generally not recommended in mild or moderate head
ulated mechanism. Adequate perfusion to the penumbra injuries, although it may be used in a conscious patient
region can potentially preserve neuronal cell function after with traumatic mass lesion. Intracranial pressure (ICP)
a CNS insult. The penumbra contains cells that are at risk monitoring is recommended for patients with severe head
unless reperfusion is reestablished within a certain window. injury (Glasgow Coma Scale of 3 to 8 after cardio-pul-
Treatment programs based on the maintenance of monary resuscitation) and abnormal CT (hematoma, con-
cerebral perfusion and the control of intracranial pressure tusion, or compressed basal cistern). ICP monitoring is
have proven to be of critical importance to the functional recommended in those with normal CT if two or more
outcome of TBI survivor. The correction of systemic para- of the following are present: age 40 years, unilateral
meters such as hypoxia, hypotension, and hyperglycemia or bilateral motor posturing, and systolic BP 90 mmHg.
is critical to the current concept of limiting secondary The goal of ICP monitoring is to maintain adequate
injury. Novel pharmacologic therapies are currently being cerebral perfusion and oxygenation. Continuous moni-
developed, all geared at inhibiting and limiting the extent toring of ICP and BP is the only reliable measurement of
of secondary injury. cerebral perfusion pressure (CPP). The CPP is the mean
arterial blood pressure minus the ICP. Low CPP can be
deleterious to vulnerable or ischemic parts of the brain.
ACUTE INTERVENTIONS Conversely, increasing CPP can increase cerebral perfu-
sion. The recommendation is to maintain a CPP at min-
The combination of a head injury with SCI poses special imum of 70 mmHg. A hypotensive patient can sustain
problems of management. A severe head injury may ini- harmful sequelae from only marginal elevations of the
tially mask the diagnosis of a spinal injury, because the ICP. In contrast, an elevated BP could be protective in a
inability to move the limbs may be attributed to the head patient with high ICP. In person with brain injury, the
injury, and the lack of cooperation may make it difficult MonroKellie doctrine establishes that within the con-
to assess sensory appreciation. The main objectives in the fines of the cranium, ICP is governed by three factors:
management of the severely head-injured patient are to brain parenchymal volume, volume of CSF, and cerebral
treat the complications of TBI (e.g., increased intracranial blood volume (CBV). Cerebral parencymal volume (CPV)
pressure) and to limit secondary brain damage. Ischemia increases after head injury because of the cerebral edema,
is probably the greatest factor contributing to secondary which results in increased ICP. To compensate, CSF is ini-
damage in TBI. tially displaced from the cranium and, subsequently,
The Guidelines for the Management of Severe changes in CBV occur. The arterial system, with one-third
Head Injury was developed in 1995 by the joint initia- of the total CBV, is responsible for autoregulation.
514 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
Autoregulation allows the blood vessels to change in Therefore, mannitol is advised as a small-volume resusci-
diameter in response to metabolic effect. Because autoreg- tation fluid for the acute phase. Limited data suggest that
ulation is compromised in moderate and severe ischemia, intermittent bolus therapy may be more effective than con-
CBF varies passively with perfusion pressure. Ventricu- tinuous infusion. The effective dose range is 0.25g to
lar drainage is an important management technique for 1gm/kg-body weight. Finally, euvoluemia is recommended
the treatment of elevated ICP. The ventricular catheter by adequate fluid replacement. A Foley catheter should
allows the physician not only to monitor ICP but to treat be placed. Most acute SCI (still in ICU) will have a Foley
it effectively by draining CSF either continuously or inter- catheter to drain the neurogenic bladder.
mittently.
Barbiturates
Hyperventilation
Barbiturates are not recommended for prophylactic use.
Hyperventilation remains a highly effective, acute treat- The reported effect is through alterations in vascular tone,
ment for increased ICP via changes in CBV (59). How- decreased metabolism (with subsequent decreased blood
ever, studies have demonstrated that prophylactic treat- flow needs), and the inhibition of free radical mediated
ment with hyperventilation is not beneficial. In some lipid peroxidation. High-dose barbiturate may be rec-
cases, the vasoconstriction obtained with hyperventila- ommended in uncontrollable high ICP that is refractory
tion causes the CBF to drop below ischemic levels. Thus, to all other management. The most commonly used bar-
hyperventilation should not be used for an extended biturate in clinical situations is pentobarbital.
period of time and pCO2 should not be reduced below 28
mmHg. In the absence of increased ICP after head injury,
Glucocorticoids
it is recommended to avoid chronic, prolonged hyper-
ventilation therapy. Brief periods of hyperventilation may Glucocorticoids are not recommended for improving out-
be warranted with acute neurologic deterioration. Longer comes or reducing ICP in patients with severe head injury.
periods of hyperventilation may be necessary if intracra- The majority of studies indicate that glucocorticoids do
nial hypertension was unresponsive to sedation, paraly- not lower ICP or improve outcomes in severe head
sis, CSF drainage, and osmotic diuretics. injuries. In one study, 161 patients were randomized to
This guideline recommends avoiding prophylactic placebo or high-dose dexamethasone (100 mg/ day), fol-
hyperventilation (PaCO2 greater than or equal to 35 lowed by a tapering dose. There was no difference in 1-
mmHg) during the first 24 hours after severe head injury. month survival or 6-month outcome. This information
It can decrease cerebral perfusion (by causing cerebral is in contrast to the proven benefits of the methylpred-
vasoconstriction) during the time that cerebral blood flow nisolone protocols used in acute SCI management.
is reduced. Therefore there is an increased risk of cerebral
ischemia with aggressive hyperventilation.
MEDICAL COMPLICATIONS
Mannitol
Medical issues arise in both brain injury and SCI,
Mannitol is accepted as effective for the control of raised although the etiologies of these issues are often different.
ICP after severe brain injury. It has an immediate plasma An overview of medical complication related to SCI is
expanding effect. Mannitol reduces the relative concen- provided in another chapter.
tration of hematocrit, thereby decreasing blood viscosity,
and increases both cerebral blood flow and cerebral oxy-
Neurologic Complications
gen delivery (6061). The osmotic effect of mannitol is
delayed for 15 to 30 minutes until gradients are estab-
Posttraumatic Epilepsy (PTE)
lished. Because mannitol is excreted entirely in the urine,
a risk of renal failure exists when serum osmolarity exceed It is estimated that approximately 5 percent of all persons
320 mOsm. This effect can last 1.5 hours to 6 or more with TBI who are hospitalized will develop posttraumatic
hours. Indications for mannitol prior to ICP monitoring epilepsy. Patients are at increased risk of developing PTE
are signs of transtentorial herniation or progressive neu- after certain injuries, acute intracranial hemorrhage
rologic decline. Mannitol may be given as repeated or in requiring surgical evacuation within 2 weeks of injury,
intermittent boluses. Avoiding continuous infusion early epilepsy, and depressed skull fracture. Other factors,
decreases the risk of keeping the bloodbrain barrier such as dural tearing, focal signs, posttraumatic amnesia
open too long. This opening for prolonged periods >24 hours, and the presence of foreign bodies are also
can cause a reverse osmotic gradient, with a subsequent considered as increased risks. Seventy five to 80 percent
increase in ICP, by increasing brain cellular swelling. of TBI patients develop PTE within 2 years; the rate is
DUAL DIAGNOSIS: SPINAL CORD INJURY AND BRAIN INJURY 515
no different from general population after 5 years (62). geal and tracheal disorders are not uncommon in this
Most PTE, 50 to 80 percent, is thought to be of the par- patient population.
tial variety, either simple partial or complex partial; 20 to
50 percent of PTE is of generalized type. Recent studies
Deep Venous Thrombosis (DVT)
have shown that using phenytoin (Dilantin) beyond the
first week following a brain injury does not prevent the Incidence of DVT in SCI is very high in the first 12 weeks,
development of late PTE. with the first 2 weeks having the highest rate following
acute injury. The venous stasis that results from muscle
paralysis, and hypercoagulability as a result of the stim-
Posttraumatic Hydrocephalus
ulation of thrombogenic factors following injury are the
True posttraumatic hydrocephalus (PTH) is relatively two major factors contributing to the development of
uncommon. One study estimated the incidence to be in DVT in the SCI population. Damage to the vessel wall
the range of 1 to 8 percent (63). The Model System can occur in SCI patients as a direct result of trauma from
National Database showed that PTH, defined as an the original injury or indirectly from external pressure on
enlargement of the ventricles that requires the placement the paralyzed leg. The incidence of DVT in the TBI pop-
of a shunt or drain, was detected in only 5 percent of ulation is lower than in SCI population. In some
patients (64). PTH should be suspect when patient is not instances, prophylaxis and treatment of DVT in SCI
progressing as expected or deteriorating. The symptoms patients with concomitant TBI requires the use of
of PTH may range from coma to arrest of progress in mechanical devices for prophylaxis and inferior vena cava
rehabilitation. An atypical presentation, such as seizures (IVC) filter placement when anticoagulation is con-
and emotional problems, is possible. Generally, shunting traindicated.
is successful if the pressure is elevated 180 mm H2O
or if the ventricles progressively increase in size.
Gastrointestinal Complications
Gastric emptying and gastric acid secretion may be
Respiratory Complications
altered in SCI patients. The SCI patient is at high risk to
Early in SCI respiratory complications are the primary develop gastric atony and ileus. As with most polytrauma
cause of death. In fact, respiratory system complications, patients, the patient with SCI and TBI has an increased
most commonly pneumonia, are the most common cause risk of stress ulceration during the acute phase. The risk
of mortality in SCI patients at any time. Pulmonary com- is greater in patients with complete injuries above the T5
plications are common in higher-level injuries especially level; bleeding is unusual in patients with lesions below
at the C1 to C4 levels. Complications include acute res- the T5 level.
piratory distress syndrome (ARDS); aspiration, atelecta- A large number of patients have impaired liver func-
sis, bronchitis, bronchospasm, lung abscess, pleural effu- tion, which in most cases is drug induced, mostly from
sion, pneumonia, pneumo/hemothorax, pulmonary phenytoin and phenobarbital; the remainder of cases are
edema, pulmonary thromboembolism, tracheitis, upper caused by acute viral hepatitis. Diarrhea in patients with
respiratory infection, and ventilatory failure. SCI and TBI may be caused by osmotic overload from
Persons with injury below T12 essentially have no tube feeding or due to Clostridium difficile colitis.
impairment of respiratory function. Injuries between T1
and T12 result in some impairment of the abdominal
Swallowing Disorders
muscles, which reduces forceful expiration and cough.
With higher thoracic level injuries there is also impair- Dysphagia is a common problem in both SCI and brain
ment of intercostal muscles, which reduce inspiratory and injury. The SCI patient with a concomitant head injury
expiratory function. With injuries above C8, loss of all is at a greater risk for a swallowing disorder. Dysphagia
abdominal and intercostal muscles occurs with further can be difficult to detect in the head-injured patient.
impairment of inspiration and expiration. C4 is generally Often, these patients are unable to communicate effec-
the highest level of injury at which spontaneous ventila- tively, have difficulty swallowing or can be silent aspira-
tion can be sustained. Injury above this level generally tors. Dysphagia as a result of a head injury may be attrib-
requires mechanical ventilation. uted to a variety of causes. TBI patients usually have one
Respiratory failure is one of the most common com- or more of the following findings on a videofluoroscopy.
plications seen in severely brain-injured patients, neces-
sitating the use of artificial respiration. A recent multi- a delay or absence of swallowing responses,
center study revealed that 39 percent of all severe TBI reduced tongue control,
patients are affected. Patients with combined SCI and TBI reduced pharyngeal transit, and/or
are at very high risk for respiratory complications. Laryn- reduced laryngeal closure, elevation, or spasms.
516 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
Heterotopic Ossification (HO) Class II mild to moderate motor and mild cognitive
dysfunction;
Heterotopic ossification (HO) is the formation of new
Class III, significant motor impairment with mini-
bone in periarticular soft tissues. The methods of diag-
mal cognitive dysfunction;
nosis and treatment for HO are the same for brain-injured
Class IV, moderate cognitive impairment with min-
patients as they are for SCI patients. As HO develops in
imal motor involvement; and
joints, it manifests itself as a loss of range of motion,
Class V, severe cognitive and physical impairment.
edema, and heat. It is a major complication common to
both brain injury and SCI. The incidence of HO in SCI
Class I patients have zero or minimal recurrence and
alone is 16 to 53 percent. In SCI, HO usually does not
best functional improvement. Class V patients have min-
occur above the level of the injury. There is an increased
imal or no gain in motion, and HO recurs in each
incidence of HO in SCI individuals with complete lesions.
instance. Good outcome and low recurrence are antici-
In the head-injured population, HO occurs at a rate of 11 pated in Class I or II patients regardless of location or site
to 75 percent, depending on the center and method of of HO.
study (66). The incidence of HO in TBI patients is most
likely 10 to 20 percent. A clinically significant HO is asso-
ciated with pain or decreased range of motion. HO usu- Autonomic Dysregulation
ally presents 1 to 3 months after injury. It can mimic Autonomic dysregulation may be found in patients with
thrombophlebitis. Shoulder HO consistently forms infer- dual SCI and TBI, which includes hypertension, ortho-
omedially to the joint. HO at the elbow originates ante- static hypotension, and temperature instability (central
rior to the elbow if flexor spasticity is present and poste- fever, hypothermia). Hypertension must be controlled to
rior to the elbow if extensor tone is initially present. avoid added neurologic insult. Patients may have
Although posterior HO forms in small amounts, the diaphoresis, which requires close monitoring for possi-
elbow is the most likely joint to ankylose. The hip has ble dehydration. It is important to monitor for skin break-
three major sites of involvement. HO inferomedial to the down, because the patients skin will be intermittently
hip joint is associated with adductor spasticity and is the soaked. Autonomic dysregulation can take two forms in
most frequent. The anterior HO forms between the the SCI patient. The first form is orthostatic hypotension,
anterosuperior iliac spine of the pelvis and the proximo- which is commonly seen in the early phases of recovery.
lateral femur. The final site of hip HO is situated poste- The second form is autonomic dysreflexia (67). Both sit-
rior, 70 percent of which is seen in the affected part of uations require prompt medical intervention. Autonomic
the body. HO is seen most commonly with brain injured dysreflexia is always considered a medical emergency and
individuals who have experienced prolonged comatose should be treated as such. Both these conditions can
and vegetative states and hypoxic episodes. Spasticity, sometimes be prevented when the patient is well educated
venous stasis, and immobility are risk factors for HO that and vigilant in his or her care. Learning the difference
is common to both brain injured and SCI individuals. between the two is often difficult for the cognitively
Therefore the person with the dual diagnosis of SCI and impaired patient, so family members and caregivers must
brain injury is probably at greater risk to develop HO. also be educated. The patient should always carry a med-
Treatment options for HO are both pharmacologic ical alert card that can instruct anyone in the steps nec-
and nonpharmacologic. They usually consist of aggres- essary to stop dysreflexia. The SCI patient who has a con-
sive range of motion, IV and oral dosing of didronel, use comitant head injury is more likely to experience
of anti-inflammatory drugs (Indocin), and in some cases, orthostatic hypotension and dysreflexia. Because of cog-
radical surgical resection. Patients receiving treatment nitive deficits and behavior issues, these patients often fail
require frequent thorough physician monitoring and fol- to take the actions necessary to prevent these conditions.
low-up. The successful treatment of HO is dependent on These patients often require more family, caretaker, and
compliance with medication and therapeutic regimens. physician involvement to ensure that their day-to-day care
Cognitively impaired individuals are limited in their abil- is of a standard that will prevent these situations.
ity to adhere to this type of regimen. In some case, surgi- Hyperthermic states often result from a defect in the
cal resection is indicated. Because most neurologic recov- central thermoregulatory system (68). It is not uncommon
ery occurs by 112 years, resection should be considered at to see a rapid rise in temperature. Left untreated, a hyper-
this time. The classification of patients according to neu- thermic state will result in further brain damage. Assess-
rologic status was proposed by Garland (66) as the best ing and diagnosing the situation correctly is crucial in
predictor for functional improvement and rate of HO developing the correct treatment. A diagnostic work-up
recurrence after resection: must take place to rule out the possibility of drug fever,
DVT, pulmonary embolism, infection, HO, and others.
Class I, near-normal neurologic recovery; After all these and other causes have been ruled out, then
518 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
the physician can consider the possibility that a central nique. Patients may forget key steps that are critical to
fever is present. If hyperthermia is a regularly occurring complete evacuation in their bowel program. The end
event for the patient, he will then need frequent moni- result is often bowel impaction or chronic UTI. The treat-
toring of his temperature. A cooling blanket that employs ing team involved in the care of patient with dual SCI and
a rectal temperature probe is helpful. Other useful inter- TBI must be very proactive, aware of the exact nature of
ventions include cool showers, fans, and not overdress- the patients cognitive deficits, and develop a plan that
ing the patient. Autonomic dysfunction may resolve over includes compensatory strategies. Extra time is always nec-
time. However, if a patient is discharged from the hospi- essary to provide the extra reinforcement of the teaching.
tal still exhibiting symptoms, patient, family, and care- Often, in the course of teaching, patients become over-
giver education regarding the monitoring and manage- whelmed by the volume of information being presented
ment of this situation is critical in avoiding harmful to them. They then confuse facts later on and make errors
sequelae. when carrying out their programs. Printed information
(memory books) is often necessary to clarify critical aspects
of care. Memory devices and family involvement is key in
Skin Breakdown
preventing complications and setting the patient up for
Skin breakdown is a multifactoral complication of both success. The presence of SCI in the TBI patient further
brain injury and SCI. There are similarities and differ- complicates the bladder function. Urodynamic studies are
ences regarding the risk factors of skin breakdown for usually helpful to accurately determine the bladder dys-
both of these populations. Therefore, when these risk fac- function if the patient is cooperative. Bowel incontinence
tors are combined, as seen in someone with the dual diag- and constipation may be found in patients with SCI and
nosis of brain injury and SCI, it should be understood that TBI. If the level of injury is C5 or higher, or the head injury
the risk for skin breakdown is very great. Appropriate is severe, a caretaker must become responsible for the
interventions in the areas of behavioral management, bowel and bladder program. If the patient is cognitively
treatment of depression, and identification of cognitive capable, he should be well versed in directing another in
deficits is beneficial in the prevention of skin breakdown. his care. A daily or every other day Dulcolax suppository
The SCI individual is at risk for skin breakdown primar- followed by digital stimulation is a good start. Bowel care
ily because of issues of immobility, pressure relief, should be done at the same time of day and in an upright
impaired sensation, and spasticity. Secondary issues tend sitting position. The dietitian can review the current diet
to be those of a psychologic nature, such as noncompli- of enteral feeding to ensure that the patient is receiving a
ance, depression, anger, and adjustment to disability, balanced high-fiber diet. If constipation or impaction
denial, and loss. Other causes of skin breakdown are indi- becomes a chronic problem, consider prescribing laxatives
rect, such as substance abuse, smoking, and impaired cog- or stimulants on a daily basis.
nition. Immobility combined with a decreased level of
consciousness places the individual at particular risk for
Miscellaneous Disorders
skin breakdown. The involvement of nursing, enteros-
tomal therapy, and dietary professionals in the planning Regulatory disturbances including fatigue, changes in
and implementation of aggressive turn schedules, frequent sleep patterns, dizziness, and headaches are not uncom-
skin checks, and adequate nutrition are crucial interven- mon problems in TBI patients.
tions in prevention of breakdown. Cognitive deficits often Perceptual deficits such as possible changes in hear-
limit the patients ability to adhere to a turning schedule, ing, vision, taste, smell, and touch are found in some
perform adequate pressure releases or perform dressing patients.
changes. In these situations, frequent reinforcement, use
of memory devices and family training and education are
important factors for home care. COGNITIVE SYMPTOMS AND BEHAVIORAL
PROBLEMS OF BRAIN INJURY AND
INTERVENTIONS
Bowel and Bladder Management
Managing the bowel and bladder for the SCI patient who An individual who sustains a head injury is left with cog-
also has a significant head injury can be difficult because nitive impairments, behavioral problems, emotional dis-
of both physical and cognitive impairment. In addition, turbances, and personality changes which he cannot com-
some patients may have behavioral problems as a result pletely control. Cognitive deficits include shortened
of TBI. Decreased new learning and impaired executive attention span, memory problems, impaired judgment,
functioning results in an inability to make practical, sim- safety awareness, and problem solving; and slowed pro-
ple adjustments to the program. Impaired memory results cessing. Cognitive impairments can sabotage or compli-
in missed catheterizations or improper bowel care tech- cate a patients rehabilitation by negatively affecting his
DUAL DIAGNOSIS: SPINAL CORD INJURY AND BRAIN INJURY 519
ability to learn his care. A patient with any degree of head instructions, follow a recipe, and perform most functional
injury may exhibit emotional liability, impaired impulse tasks. They cannot do two things at once. Do not expect
control, and a complete host of other behavioral impair- them to be able to watch the kids and make dinner at the
ments. Behavioral impairments and acting out can leave same time and not get frustrated or make a mistake.
him socially isolated, without any support network. A Behavioral problems are common following head
detailed assessment of the individual by the neuropsy- injury. Impulsivity may be caused by poor insight and
chologist is the key to the identification of cognitive judgment when a patient has impaired self-regulation of
impairments. The neuropsychologist performs a detailed desires. He decides on an action and does not think it
assessment, which identifies cognitive impairments and through first or consider the consequences of the action.
target behaviors. From this follows the development of a Personal safety can often be compromised.
comprehensive plan of care, which includes a behavioral A patient will perform unsafe transfers and then fall.
management plan. Cognitive impairments and behavioral He may overeat, drink or smoke. He may be careless with
issues manifest themselves differently from person to per- power tools or driving. He demonstrates apathy, poor ini-
son and must be handled on an individual basis. Evalua- tiation, poor motivation, and the inability to follow
tions, plans, and goals must be established to address through. The patient may desire to take action, but can-
whatever impairments are found, especially those which not motivate himself to initiate the action. He appears
have the greatest impact on functioning. A patient with to be lazy and uninvolved with his care. Bowel and
impaired attention and concentration and decreased abil- bladder routines, pressure releases, and medications will
ity to screen out irrelevant information is easily distracted be missed. He demonstrates emotional instability, lower
and finds it difficult to follow long discussions. He usu- frustration tolerance, resulting in overstimulation char-
ally does better in settings that are structured and short acterized by agitation, angry outbursts, crying, and labil-
in duration. This patient learns new information more ity. This patient appears to be easily irritated by minor
effectively in an environment that is distraction free (no things. He may want to quit a task after one try. He has
TV, radio). Information can be given in written form for great difficulty in tolerating (normal) daily stressors with-
purposes of reinforcement. out breaking down.
Some patients may have an impaired ability to read Because of shortened temper and poor frustration tol-
and write. Packaging directions on medical equipment, erance, issues of safe and effective parenting may arise.
prescription information, and medical information may Attention should be paid to his environment to keep it
get left at the wayside if it cannot be read and understood. structured, simple, and free of elements that trigger agita-
Patient teaching will have to be done by explanation and tion. Periods of activity must be balanced with periods of
demonstration. Having the patient provide a return expla- rest. Disinhibition may be demonstrated by inappropri-
nation or demonstration is one way of assessing what he ate comments or sexual behaviors. Behavioral manage-
has learned. ment plans that target specific behaviors are successful in
Impaired new learning and decreased short-term extinguishing these annoying events. Anxiety requires psy-
memory is another problem. The individual cannot learn chologic intervention. Medication management is required
catheterization programs or other aspects of care as when it is acute enough to prevent a patient from partici-
quickly as others. In this situation written materials (when pating in therapy or learning self-care. A patient may have
appropriate), repetition, and family and caregiver train- extreme fears of falling that prevent him from fully par-
ing are necessary. ticipating in gait training, transfers, and fall recovery. Non-
Other cognitive consequences of TBI include a compliance, denial, and refusal to participate may be
decreased ability to solve problems, organize information, demonstrated by a patient listing numerous reasons why
and correctly sequence complex actions. Patients cannot he is not going to therapy. In these situations a behavioral
problem solve their way through small or big problems contract with mutually agreed on goals, rewards, and con-
that arise in their daily activities; for example what to do sequences is valuable in increasing compliance.
if they drop a catheter on the floor. They may fail to antic- Extinguishing problem behaviors successfully
ipate when medications and supplies run out and plan requires a customized behavioral management plan. The
ahead to reorder. Managing their money and keeping an neuropsychologist is responsible for performing a detailed
accurate checkbook may be impossible without help. Bills assessment to identify specific target behaviors and design-
may not get paid in a timely manner because they can- ing the behavioral plan. The team is responsible for car-
not prioritize which should be paid first. Patients will have rying it out. A successful behavior management plan
difficulty following a recipe or planning or making a din- includes structure, consistency, repetition, and practicality.
ner start to finish. Structure the plan to be well constructed and sound; oth-
Patients with decreased processing skills and an erwise it will fail. The brain-injured patient cannot deal
inability to divide attention have difficulty understanding with generalized, unstructured environments. Lack of
and integrating information. It takes them longer to read structure often results in overstimulation, acting out, and
520 NEUROLOGIC ASPECTS OF SPINAL CORD CARE
a sense of failure. Consistency requires that the patient arousal therapy, multisensory therapy, and coma
adhere to the same tasks with the same people at the same stimulation. This sensory stimulation provides an orga-
time. Unpredictability leads to confusion. Repetition is the nized presentation of direct stimulation in multiple
primary means by which learning can be accomplished. modalities with the hope of advancing patients from an
Practicality involves choosing a target behavior that, unconscious state. Common treatment strategies include
when eliminated, results in increased functioning in the visual, auditory, olfactory, cutaneous, kinesthetic, and
home, community, or self-care. Goals must be directed oral stimulation. Pharmacologic treatment is an inter-
at increasing basic functioning and addressing long-term vention often used to directly increase arousal and facil-
goals. Specific consequences must be established for tar- itate recovery of consciousness of these patients. Med-
geted behaviors. A patient with brain injury cannot gen- ications include stimulants (e.g., methylphenidate,
eralize information; he needs to focus on specific goals dextroamphetamine), and antiparkinsonian medications
or consequences, meaningful rewards. The goals that are (bromocriptine and amantadine).
selected must be viewed as valuable and desirable to the The unconscious patient should undergo range-of-
patient and family. motion (ROM) exercise in order to prevent contractures
and other joint abnormality. The patient should be posi-
tioned to prevent pressure ulcers, edema, and contrac-
REHABILITATION OF THE PATIENT tures. Intervention for spasticity, nutrition, and bowel or
WITH DUAL TBI AND SCI bladder incontinence should be provided.
PHARMACOLOGIC CONSIDERATION
TABLE 37.2
Agents that Influence Neurotransmitters
Multiple and varied medications are used acutely and
long term in the trauma patient. In the SCI patient with
NEUROCHEMICAL AFFECT
mild to moderate brain injury, the physician may choose
medications that, while appropriate to spinal cord med- Cholinergic Memory, concentration,
icine, may be undesirable to use in patients with a brain attention
injury. General concepts include: Dopaminergic Movement, arousal
Noradrenergic Arousal, sleep/wake,
avoid sedating medications, memory, learning
normalize sleep/wake patterns,
Serotonergic Sleep, mood, arousal
maximize medical condition, and
treat adjustment issues, mood, and anxiety.
TABLE 37.3
Some of the Medications used as Neurostimulants
Methylphenidate (Ritalin) Increases dopamine and Increases BP, agitation, insomnia, headache,
norepinephrine activity dysphoria
Pemoline (Cylert) Similar to Ritalin Reported fewer sympathomimetic side effects
Amantadine (Symmetrel) Presynaptic and postsynaptic Lowered seizure threshold, arrhythmias,
dopamine receptor increases BP, headache, nausea
Bromocriptine (Parlodel) Dopamine agonist Same as above
L-Dopa + Carbidopa (Sinemet)
Modafinal (Provigil) Exact mechanism unknown Headache, nausea, nervousness, insomnia
Naltrexone (Revia) Opiod antagonist Nausea
coefficients for the DRS, although the GCS and DRS con- twelve items to FIM to improve sensitivity in assessing the
sist largely of items that tend to lose their predictive value brain injured (78).
after the acute period (75). The physician must remember to factor the brain
The Rancho Los Amigos Scale (Table 37.4) was injury component into the outcome of the dual diagno-
designed to measure recovery; it is widely used to describe sis patient. In general, a better outcome is more chal-
neurobehavioral function. The scale ranges from 1 (no lenging for patient and treating team in the dual-diagno-
response) to 8 (purposeful and appropriate). This scale sis. Recovery in the TBI patient is most rapid after the
can be used to guide the rehabilitation team in a treat- injury and slows down with the passage of time. Many
ment plan (76). people with severe head injuries end up with almost no
The Functional Independence Measure (FIM) is noticeable problems, but others require constant care for
widely used in the general rehabilitation population. The the rest of their lives. In general terms, the longer a per-
assessment incorporates eighteen items including bowel, son remains in coma, the less likely he or she will recover
bladder, self-care, and mobility. The Functional Assess- completely. The full extent of a brain injury may be
ment Measure (FAM) with the FIM (FIM-FAM) adds unknown for months or even years. A significant rela-
TABLE 37.4
Rancho Los Amigos Scale (77)
tionship exists between the impairments in memory, con- 14. Dubo H, Delaney G. 101 spinal cord injuries due to motor vehi-
cle accidents. Proceedings of the Tenth Annual Meeting of the
centration, and learning skills demonstrated during ini- American Spinal Injury Association, March 1984; 3538.
tial inpatient rehabilitation, and the rate of rehospital- 15. Davidoff G, Morris J, Roth E, Bleiberg J. Closed head injury in
ization for SCI patients. The prevention of medical spinal cord injured patients: Retrospective study of loss of con-
sciousness and post-traumatic amnesia. Arch Phys Med Rehabil
morbidity and enhancement of function are two of the 1985; 66:4143.
major goals of SCI rehabilitation. Awareness of the poten- 16. Davidoff G, Thomas P, Johnson M, and et al. Closed head injury
tially adverse effects of cognitive deficits on hospitaliza- in acute traumatic spinal cord injury: Incidence and risk factors.
Arch Phys Med Rehabil 1988; 69:869872.
tion, medical care use, and functional abilities may help 17. Meinecke FW. Frequency and distribution of associated injuries
to reduce their impact on the post SCI course. in traumatic paraplegia and tetraplegia. Paraplegia 1968;
5:196211.
18. Harris P. Associated injuries in traumatic paraplegia and tetraple-
gia. Paraplegia 1968; 5:215220.
CONCLUSION 19. Shrago GG. Cervical spine injuries: Association with head trauma
a review of 50 patients. Am J Roent 1973; July:670673.
When a SCI patient sustains a concomitant head injury, his 20. Steudel WI, Rosenthal D, Lorenz R, Merdes W. Prognosis and
treatment of cervical spine injuries with associated head trauma.
care becomes complex and challenging. Brain injury results Acta Neurochir Suppl 1988; 43:8590.
in a variety of cognitive impairments. Some of these 21. Davidoff G, Thomas P, Berent S, Dijkers M, Dolijanac R. Closed
impairments, such as decreased insight, decreased short- head injury in acute spinal cord injury: Incidence and risk factors.
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learning can profoundly affect the successful rehabilitation spinal injury. J Neurotrauma 1989; 6:177189.
of these individuals. Patients suffering concomitant CHI 23. Saboe LA, Reid DC, Davis LA, Warren SA, Grace MG. Spine
trauma and associated injuries. J Trauma 1991; 31:4348.
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the patients specific cognitive limitations and needs. ciation of head trauma with cervical spine injury, spinal cord injury,
or both. J Trauma 1999; 46:450452.
26. Alker GJ, Oh YS, Leslie EV, et al. Postmortem radiology of head
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VI
MUSCULOSKELETAL CARE
This Page Intentionally Left Blank
38 Musculoskeletal Pain
and Overuse Injuries
ndividuals with spinal cord injury extremity pain to their doctors, and the majority had not
ALTERNATIVE DIAGNOSES
50 and 75 percent (1,2). Because the majority of individ-
uals with SCI rely on their upper extremities for all activ- Is the individuals pain related to a musculoskeletal prob-
ities of daily living (ADLs) and for mobility, this pain can lem? Probably, but not definitely. Anyone who treats or
have a tremendous impact on their quality of life. All the works with individuals with SCI must have an expanded
subjects in one study reported a change in their depen- differential diagnosis. A common diagnosis in individu-
dence on personal care assistants with fluctuations in als with SCI is neuropathic pain. Historical factors that
upper-extremity pain (3). Even when self-care is not can help differentiate neuropathic pain from muscu-
affected, upper-extremity pain can limit an individuals loskeletal pain are:
mobility and endurance (4). In one study, pain was the
only factor found to be correlated with lower quality-of- Type of pain
life scores (5). The largest survey study was completed Neuropathiccan be burning or stabbing and
by Sie et al. (1). This study defined significant pain as pain sometimes aching
requiring analgesic medication, pain associated with two Musculoskeletalusually dull
or more ADLs or pain severe enough to result in cessa- Location of pain
tion of activity. Using this definition, significant pain was Neuropathiccan occur anywhere, often at the
present in 59 percent of individuals with tetraplegia and level of injury or below
41 percent of individuals with paraplegia. Decreased arm Musculoskeletalusually above the level of injury,
and hand function can result in loss of independence, often associated with specific joints or trigger
mobility, and the ability to perform work and leisure points
tasks. Unfortunately, clinicians are not effective at treat- Exacerbating factors
ing these complaints. Subbarao et al. (3) found that 51.5 Neuropathicuncertain; pain can be present con-
percent of 451 individuals with SCI had reported upper- stantly
527
528 MUSCULOSKELETAL CARE
Musculoskeletalusually worse with movement those greater than 20 years post-injury, with tetraplegia.
and activity involving affected joint or region This increase in paraplegic shoulder pain likely represents
repetitive strain injuries from years of transfers and man-
The above list is not complete, and differentiating ual wheelchair use.
neuropathic from musculoskeletal pain is not an easy To get a better idea of the cause of pain, many stud-
task. In addition to the historic factors, musculoskeletal ies have incorporated physical examination and imaging
pain can commonly be reproduced on physical examina- studies. The majority of these studies involve individuals
tion. This is usually not the case for neuropathic pain. with paraplegia. Bayley studied ninety-four veterans with
Syringomyelia is another diagnosis unique to SCI complete paraplegia (10). Each veteran had a physical
(6). In this condition, fluid collects in the spinal cord itself examination focusing on the upper extremity. Thirty-one
and eventually compresses the cord in the rigid spinal patients reported a history of shoulder pain, and twenty-
canal. This condition usually has associated numbness three were found to have signs of impingement on exam-
and pain; weakness can also be a presenting symptom. ination. All twenty-three subjects with pain on examina-
This condition is potentially life threatening and needs tion had X-rays and arthrography that revealed rotator
to be ruled out if there is a high clinical suspicion, or if a cuff tears in 65 percent and aseptic necrosis of the humeral
patient with pain does not respond to treatment as head in 22 percent. Wylie reviewed the medical and sur-
expected. Syrinx frequently requires surgical treatment gical records of fifty-one patients with SCI who were all
and is diagnosed using magnetic resonance imaging more than 20 years post injury (11). He found radi-
(MRI). Many other nonmusculoskeletal conditions, such ographic evidence of shoulder degenerative joint disease in
as radiculopathy, can cause upper extremity pain and 32 percent of these subjects. He reported that patients with
numbness. A broad differential diagnosis is needed when greater activity levels had less evidence of injury.
diagnosing and treating pain in people with SCI. Two studies to date have looked at MRI abnormal-
A potentially common cause of musculoskeletal pain ities in individuals with SCI (12,13). In agreement with
in individuals with SCI is myofascial pain. Although there the study by Bayley, Esobedo et al. found that 57 percent
have been no studies documenting its prevalence in indi- of veterans with paraplegia had rotator cuff tears, with
viduals with SCI, based on the general population and the severity of tears related to age and duration of SCI.
postural issues that accompany SCI, it likely has a high More recent work by Boninger et al. found a relative
prevalence. Appropriately locating the trigger points asso- absence of rotator cuff tears. In the twenty-eight subjects
ciated with radiating pain or numbness can help establish tested (fifty-five shoulders), only a single rotator cuff tear
a diagnosis. In addition, patients should be questioned was seen. The most likely cause for the difference between
related to sleep disturbance, which commonly accompa- the studies is the disparity in populations: the average age
nies myofascial pain. Physical examination showing a of the subjects in Escobedos study was 59 years and the
restricted range of motion and pain at end range may be average number of years post-injury was 26. This is in
indicative of this disorder. Although the underlying marked contrast to the study by Boninger, where the aver-
pathology of myofascial pain may be benign compared to age age was 35 years and years post-injury was 11.5. If
rotator cuff tears or carpal tunnel syndrome, symptoms subjects in the study by Boninger were followed for an
related to this disorder can be quite disabling. additional 15 years, the prevalence of rotator cuff tears
and pain would likely increase.
Other causes for the difference between these stud-
SHOULDER INJURIES ies include different recruitment methods and changes in
wheelchair design over time. In the study by Boninger,
The shoulder is the most common source of muscu- subjects were not recruited from a single clinic but from
loskeletal pain in SCI. This finding is not surprising, as wheelchair vendors and discharge records after acute SCI
the shoulder is built for maximum flexibility and rehabilitation. By recruiting subjects in this manner, indi-
motionnot for weight bearing. The prevalence of pain viduals not seen by a physician were included in the study.
by survey methods varies from 36 to 100 percent (1,79). Individuals who have pain may be more likely to see a
This range in prevalence is caused by many differences physician in regular follow-up. Thus, previous studies
among the studies. Sie interviewed 239 individuals with may have overestimated the prevalence of rotator cuff
SCI (1). He found that in people less than 5 years post- tears. In the past 20 years, the weight and performance
injury, 53 percent of individuals with tetraplegia and 16 of wheelchairs has improved substantially. Most of the
percent of individuals with paraplegia reported shoulder subjects in the study by Boninger used ultralight weight
pain. The difference between the groups is likely caused wheelchairs. As will be discussed later in this chapter, this
by neuropathic pain at the shoulder in tetraplegia. How- could lower the incidence of injury.
ever, by 20 years post-injury, over 70 percent of individ- Another imaging abnormality noted in individuals
uals with paraplegia had pain, a higher percentage than with paralysis is osteolysis of the distal clavicle (see Fig-
MUSCULOSKELETAL PAIN AND OVERUSE INJURIES 529
ELBOW INJURIES
eral nerve damage. No specific study has investigated the als with paraplegia, increased weight was related to
etiology of elbow injuries in individuals with SCI. median nerve slowing and amplitude loss (see Figure
38.2). Controlled for weight, those individuals who hit
the pushrim more frequently and with greater force also
WRIST INJURIES had greater evidence of median nerve damage. This arti-
cle was the first to find a direct link between daily activ-
As at the elbow, wrist pain can be caused by both mus- ities and the incidence of repetitive strain injury in a pop-
culoskeletal and nerve injury. The majority of studies in ulation of individuals with SCI.
this area have focused on carpal tunnel syndrome (CTS).
Sie is one of the few authors who attempted to separate
the symptoms of CTS and other causes of wrist pain. In MUSCULOSKELETAL BACK
Sies article, wrist and hand pain not associated with CTS AND NECK PROBLEMS
was seen 13 and 11 percent of the time, respectively, in
individuals with paraplegia. Sies study found historic or Surprisingly little is written about back pain in individu-
physical examination evidence of CTS in 66 percent of als with SCI. As in much of the literature on pain in SCI,
subjects (1). Gellman, in a study of seventy-seven indi- when a study reports the prevalence of back pain it likely
viduals with T2 or lower paraplegia, found that 49 per- contains some individuals with neuropathic pain. Nep-
cent had signs and symptoms of CTS (24). Both of these muceno performed a mail survey returned by 200 indi-
large clinical series found the incidence of CTS increased viduals with SCI (2). Neck pain was seen in up to 16 per-
with an increased duration of paralysis. cent, depending on the injury level, with individuals with
To add objective criteria to the diagnosis of CTS, a tetraplegic having the greatest incidence. Back or trunk
number of investigators have performed nerve conduc- pain had an incidence as high as 83 percent, with injury
tion studies on this population. Aljure studied forty- at the thoracic level being most associated with this type
seven patients with a SCI below the T2 level and found of pain. The discussion of this article focused on neuro-
electrodiagnostic evidence of CTS in 63 percent and clin- pathic causes for pain, including radiculopathy associated
ical evidence of CTS in 40 percent (25). This study also with the initial injury. It is likely however, that at least part
found an increased prevalence of CTS with a longer of these pain complaints were related to musculoskeletal
duration of paralysis. Tun found that 50 percent of indi- causes. It is clear that more work is needed in the area of
viduals with paraplegia below the T1 level had slowed musculoskeletal back pain in individuals with SCI.
motor conduction of either the ulnar or median nerves Few studies have investigated potential muscu-
at the wrist (26). Forty-four percent of patients with elec- loskeletal etiologies for neck and back pain. It has gen-
trical abnormalities were asymptomatic. Davidoff stud- erally been thought that increased kyphosis and scoliosis
ied thirty-one patients with paraplegia below the T1 level lead to pain, but there has been little research to support
and found electrodiagnostic evidence of CTS in 55 per- this contention. Gertzbein et al. conducted a 2-year
cent and symptoms of numbness or tingling in the hand prospective study of patients with thoracic and lumbar
in 74 percent (27).
From these studies, it is apparent that CTS is a com-
mon problem in individuals with SCI. Most of the stud-
ies found a greater prevalence of abnormalities in nerve
conduction studies than actual clinical symptoms. This
may signify that subclinical nerve damage exists in a num-
ber of these individuals. Whether this subclinical dam-
age goes on to become clinically important has not yet
been determined.
CTS is generally thought to be caused by compres-
sion of the median nerve within the carpal tunnel.
Extremes of wrist flexion and extension have been shown
to greatly increase the pressure within the carpal tunnel,
more so in patients with CTS. Gellman studied patients
with SCI and found that pressures in the carpal tunnel
were higher in wrist flexion in this group than in a group
of controls (28). Recently, Boninger et al. found a direct FIGURE 38.2
relationship between both subject weight and wheelchair
propulsion biomechanics and evidence of damage to the In individuals with paraplegia, increased weight and pusarim
median nerve (29). In this study of thirty-four individu- force was related to median nerve slowing and amplitude loss.
MUSCULOSKELETAL PAIN AND OVERUSE INJURIES 531
spinal fractures. Although this study found a positive rela- as in DiGiovine et al. They suggest that this higher fre-
tionship between kyphosis and pain, the finding was not quency may be a function of the seating position (e.g., a
statistically significant. In a study evaluating mortality, kyphotic posture) inherent to wheelchair propulsion or
morbidity, and psychosocial outcomes of persons more may be related to the amount of active muscle control.
than 20 years post-SCI, Whiteneck et al. reported a 14 These studies found that the vibrations experienced dur-
percent incidence of kyphosis and scoliosis. Boninger et ing usual activities may exceed the standards for risk of
al. compared radiographically determined scoliosis and injury set by the trucking injury.
kyphosis between a control group, a group of individu- To further support this research, DiGiovine et al.
als with tetraplegia less than 2 years post-SCI, and a group assessed perceived comfort while individuals in wheel-
longer than 10 years post-SCI (30). Individuals with chairs traversed obstacles in a variety of chairs (34). This
tetraplegia had significantly higher measures of scoliosis study found that riders were significantly more comfort-
and kyphosis. Interestingly, no difference existed between able in ultralightweight wheelchairs than in lightweight
the two groups with SCI, indicating that the increase chairs. For the purpose of the study, an ultralight chair
spinal curvature occurs early (see Figure 38.3). No rela- was defined as having a high degree of adjustability.
tionship was seen between the degree of scoliosis or Although this article does not provide a direct link
kyphosis and measures of pain. Not surprisingly, this between back pain and type of wheelchair, it does suggest
paper recommended early intervention. that such a link may be present. Further research into this
One group of researchers has investigated vibration and other causes of secondary spine injury and pain is
as a potential etiology for musculoskeletal back pain in needed.
individuals with SCI (3133). For most individuals with
SCI, a majority of their day is spent in a wheelchair. This
research group maintains that during wheelchair propul- TREATMENT AND PREVENTION OF
sion, individuals encounter impact vibrations while tra- MUSCULOSKELETAL INJURIES IN SCI
versing curbs, bumps, and other obstacles, and cyclic
vibrations while traversing uneven or rough terrains. Treating individuals with SCI and musculoskeletal
Studies by this group have found that the spine of an indi- injuries requires insight into the special needs of this pop-
vidual with paralysis reacts differently to vibration than ulation. The single most unique factor is related to their
does an unimpaired spine. A recent study by DiGiovine use of extremities. For the vast majority of individuals
et al. measured vibrations at the wheelchaircushion with SCI, it is nearly impossible to rest an extremity. For
interface while ten unimpaired individuals traversed an an individual with paraplegia, to rest may means going
obstacle course (31). Average resonance frequencies of the from complete independence to complete dependence. In
head in any given direction were reported to be between some cases, it may be necessary to hospitalize a patient
1.5 and 4 Hz, which approaches the fatigue boundaries just to assure adequate rest. For this reason, prevention
of acceptable vibration transmitted to the body in a seated of injuries and early treatment is essential. Specific treat-
position. VanSickle et al., in a study of sixteen manual ment for the injuries discussed are numerous and beyond
wheelchair users (thirteen with SCI), found higher peak the scope of this text. Instead a brief summary is provided,
frequencies while negotiating the same obstacle course and areas where treatment may need to be approached
differently for individuals with SCI are highlighted.
Treatment for most musculoskeletal conditions can
be summarized in the following categories:
Exercise
Modalities
Modification of activity
Bracing
Medications
Injections
Diet
Surgery
Another form of bracing that should be considered for had work-related rotator cuff injuries and returned to the
shoulder and back pain is related to seating. Appropri- same job were at greatly increased risk for reinjury (39).
ate seating can improve posture and, at least theoretically, There is also literature showing that individuals with CTS
reduce complaints related to back, neck, and shoulder related to repetitive tasks may be at greater risk of a worse
pain. Special care should be taken to reduce the natural outcome from release surgery (40,41).
kyphotic posture assumed when sitting in a wheelchair. It may be useful to apply a stepwise approach to the
This can be accomplished through tilt of the seat, avoid- treatment of upper extremity pain in individuals with SCI.
ing sling upholstery, and using specialized cushions and Once pain is appropriately diagnosed, initial treat-
back supports. ment should consist of:
Medications, such as nonsteroidal antiinflammato-
ries (NSAIDs), are a mainstay of treatment to reduce Exercise, modalities, medications, modification of
inflammation. As with all patients, gastrointestinal and activity, patient education, and bracing. Consider
other potential side effects must be explained to the dietary counseling if the patient is overweight.
patient. For pain as a result of fibromyalgia or chronic at this point continued use of a manual wheelchair
pain not associated with inflammation, NSIADs may not is acceptable
have a role. In these cases, it may be worthwhile to con-
sider a medication with a better side effect profile, such if pain persists
as acetaminophen. Although narcotic analgesics have a
potential for addiction, their use should not be ruled out Continue treatment outlined plus, begin exclusive
in individuals with chronic pain. use of a power wheelchair or power-assist wheel-
Injections can be helpful in a number of conditions chair and offer peer counseling,
affecting the upper extremities. Although they can relieve
the symptoms in CTS, symptoms frequently recur (38). if pain persists
The injection of steroids and an anesthetic can also be
effective in lateral epicondylitis. Unfortunately, steroid Surgery as a last resort; power wheelchair use exclu-
injections can weaken ligamentous tissues for prolonged sively after surgery to prevent reinjury.
periods. This is an important consideration when treat-
ing rotator cuff tears. In general, the individual should As in all types of medical care, the patient should
not use the arm in any lifting activity for 3 to 4 days. help to guide treatment. Because changes in wheelchairs
Because rest of the arm is difficult, and the risk of ten- and ADLs affect the patients lifestyle to a great extent,
don rupture is increased in the presence of a tear, this it is important for the clinician to understand the patient
treatment should be used with caution in individuals with from a broad perspective including vocational and avo-
SCI. Injection of a local anesthetic may also play a role cational activities. These types of discussions help the
in the treatment of trigger points. patient understand the treatment approach and assure
Diet, although not traditionally thought of as a greater compliance.
treatment for pain, may be an essential component of a
comprehensive treatment plan that should not be ignored.
Recent work has established a link between weight and PREVENTION
both shoulder injuries and CTS in patients with SCI
(13,29). This link is not surprising, because heavier As with most conditions, prevention is infinitely prefer-
patients have to do more work, and thus place more strain able to treatment. The recipe for prevention is actually the
on joints, during transfers and wheelchair propulsion. At same as the recipe for treatment. Individuals with SCI will
the same time, the ability to perform cardiovascular fit- be well served by an exercise program that maintains joint
ness exercises is diminished. As in the general population, range of motion and prevents muscle imbalance. At the
the mainstay of a weight loss prescription is diet. same time, a focus on ergonomic setup in the home and
Surgery is usually thought of when alternative treat- work environment will reduce the stress on the arm.
ments have not successfully relieved the symptoms. The Appropriate wheelchair setup and good seating and posi-
surgical treatment of CTS and most shoulder injuries tioning should help to prevent both upper-extremity pain
requires absolute rest of the arm for extended periods. and back and neck pain. In addition, clinicians should ask
Once again, to assure proper rest, an individual with SCI patients about pain. A patient may be reluctant to come
may need to be hospitalized during the recovery period to forth with this information, because he is aware of the
assure that rest is achieved without compromising the skin. serious implications arm pain may have on his daily life.
It should also be noted that for rotator cuff injuries there The early treatment of pain could result in fewer symp-
is a greater risk of reinjury when returning to the same toms in the future, and identification of the problem can
activity level. In one study, for example, individuals who serve as a catalyst to healthy lifestyle changes.
534 MUSCULOSKELETAL CARE
References 21. Boninger ML, Cooper RA, Shimada SD, Rudy TE. Shoulder and
elbow motion during two speeds of wheelchair propulsion: A
description using a local coordinate system. Spinal Cord 1998;
1. Sie IH, Waters RL, Adkins RH, Gellman H. Upper extremity pain 36:418426.
in the postrehabilitation spinal cord injured patient. Arch Phys 22. Crane L, Klerk K, Ruhl A, Warner P, Ruhl C, Roach KE. The
Med Rehabil 1992; 73:4448. effect of exercise training on pulmonary function in persons with
2. Nepomuceno C, Fine PR, Richards JS, Gowens H, Stover SL, quadriplegia. Paraplegia 1994; 32:435441.
Rantanuabol, et al. Pain in patients with spinal cord injury. Arch 23. Stefaniwsky L, Bilowit DS, Prasad SS. Reduced motor conduction
Phys Med Rehabil 1979; 60:605609. velocity of the ulnar nerve in spinal cord injured patients. Para-
3. Subbarao JV, Klopfstein J, Turpin R. Prevalence and impact of plegia 1980; 18:2124.
wrist and shoulder pain in patients with spinal cord injury. J Spinal 24. Gellman H, Gelberman RH, Tan AM, Botte MJ. Carpal tunnel
Cord Med 1994; 18:913. syndrome. An evaluation of the provocative diagnostic tests. J
4. Curtis KA, Roach KE, Applegate EB, Amar T, Benbow CS, Bone Joint Surg [Am] 1986; 68:735737.
Genecco TD, et al. Development of the Wheelchair Users Shoul- 25. Aljure J, Eltorai I, Bradley WE, Lin JE, Johnson B. Carpal tunnel
der Pain Index (WUSPI). Paraplegia 1995; 33:290293. syndrome in paraplegic patients. Paraplegia 1985; 23:182186.
5. Lundqvist C, Siosteen A, Blomstrand C, Lind B, Sullivan M. Spinal 26. Tun CG, Upton J. The paraplegic hand: Electrodiagnostic studies
cord injuries. Clinical, functional, and emotional status. Spine and clinical findings. J Hand Surg [Am] 1988; 13:716719.
1991; 16:7883. 27. Davidoff G, Werner R, Waring W. Compressive mononeu-
6. Rossier AB, Foo D, Shillito J, Dyro FM. Posttraumatic cervical ropathies of the upper extremity in chronic paraplegia. Paraple-
syringomyelia. Incidence, clinical presentation, electrophysiolog- gia 1991; 29:1724.
ical studies, syrinx protein and results of conservative and opera- 28. Gellman H, Chandler DR, Petrasek J, Sie I, Adkins R, Waters RL.
tive treatment. Brain 1985; 108:439461. Carpal tunnel syndrome in paraplegic patients. J Bone Joint Surg
7. Nichols PJ, Norman PA, Ennis JR. Wheelchair users shoulder? [Am] 1988; 70:517519.
Shoulder pain in patients with spinal cord lesions. Scand J Reha- 29. Boninger ML, Cooper RA, Baldwin MA, Shimada SD, Koontz
bil Med 1979; 11:2932. AM. Wheelchair pushrim kinetics, weight and median nerve func-
8. Pentland WE, Twomey LT. The weight-bearing upper extremity in tion. Arch Phys Med Rehabil 1999; 80:910915.
women with long term paraplegia. Paraplegia 1991; 29:521530. 30. Boninger ML, Sauer T, Trefler E, Hobson D, Burdett R, Cooper
9. Gellman H, Sie I, Waters RL. Late complications of the weight- RA. Postural changes with aging in tetraplegia. Arch Phys Med
bearing upper extremity in the paraplegic patient. Clin Orthop Rehabil 1998; 79:15771581.
Related Res 1988; 233:132135. 31. DiGiovine CP, Cooper RA, Wolf EJ, Hosfield J, Corfman T. Analy-
10. Bayley JC, Cochran TP, Sledge CB. The weight-bearing shoulder. sis of vibration and comparison of four wheelchair cushions dur-
The impingement syndrome in paraplegics. J Bone Joint Surg [Am] ing manual wheelchair propulsion. Proceedings 23rd Annual
1987; 69:676678. RESNA Conference. Orlando, FL: RESNA Press, 2000; 429431.
11. Wylie EJ, Chakera TM. Degenerative joint abnormalities in 32. VanSickle DP, Cooper RA, Boninger ML, DiGiovine CP. Analy-
patients with paraplegia of duration greater than 20 years. Para- sis of vibrations induced during wheelchair propulsion. J Rehab
plegia 1988; 26:101106. Res Dev 2001; 38(4):409421.
12. Escobedo EM, Hunter JC, Hollister MC, Patten RM, Goldstein 33. Tai C, Liu D, Cooper RA, DiGiovine MM, Boninger ML. Analy-
B. MR imaging of rotator cuff tears in individuals with paraple- sis of vibration during manual wheelchair use. Saudi J Disabil
gia. AJR 1997; 168:919923. Rehabil 1998; 4:186191.
13. Boninger ML, Towers JD, Cooper RA, Dicianno BE, Munin MC. 34. DiGiovine MM, Cooper RA, Boninger ML, Lawrence B, Van-
Shoulder Imaging abnormalities in individuals with paraplegia. J Sickle DP, Rentschler AJ. User assessment of manual wheelchair
Rehabil Res Dev; 2001; 38(4):401408. ride comfort and ergonomics. Arch Phys Med Rehabil 2000;
14. Yu YS, Dardani M, Fischer RA. MR observations of postraumatic 81(4),490494.
osteolysis of the distal clavicle after traumatic separation of the 35. Werner RA, Franzblau A, Albers JW, Armstrong TJ. Median
acromioclavicular joint. J Comput Assist Tomogr 2000; mononeuropathy among active workersare there differences
24:159164. between symptomatic and asymptomatic workers. Am J Ind Med
15. Roach NA, Schweitzer ME. Does osteolysis of the distal clavicle 1998; 33:374378.
occur following spinal cord injury? Skeletal Radiology 1997; 36. Boninger ML, Baldwin MA, Cooper RA, Koontz AM, Chan L.
26:1619. Manual wheelchair pushrim biomechanics and axle position. Arch
16. Scavenius M, Iversen BF. Nontraumatic clavicular osteolysis in Phys Med Rehabil 2000; 81(4):608613.
weight lifters. Am J Sports Med 1992; 20:463467. 37. Cooper RA, Fitzgerald SG, Boninger ML, Prins K, Rentschler
17. Burnham RS, May L, Nelson E, Steadward R, Reid DC. Shoul- AJ, Arva J, OConnor TJ. Evaluation of a pushrim activated
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J Sports Med 1993; 21:238242. 82(1):702708.
18. Koontz AM, Boninger ML, Towers JD, Cooper RA, Baldwin MA. 38. DArcy CA, McGee S. Does this patient have carpal tunnel syn-
Wheelchair propulsion forces and MRI Evidence of Shoulder drome? JAMA 2000; 283:31103117.
Impairment. 23rd Annual Meeting of the American Society of Bio- 39. Gazielly DF, Gleyze P, Montagnon C. Functional and anatomical
mechanics, Oct. 21, 1999; 296297. results after rotator cuff repair. Clin Orthop Related Res 1994;
19. Ferrara MS, Davis RW. Injuries to elite wheelchair athletes. Para- 4353.
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20. Nyland J, Robinson K, Caborn D, Knapp E, Brosky T. Shoulder carpal tunnel syndrome. Hand Clin 1996; 12:731743.
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39 Management of
Long Bone Fractures
he treatment principles for hip and angular deformities and shortening of the lower extremi-
and demand complex medical and nursing care (35). Upper Extremity (UE)
Programs for skin, bowel, and bladder care, and even-
tual rehabilitation are necessary. Therefore, the man- Fifty-three long bone UE fractures were evaluated (5). No
agement of these acute fractures should facilitate nurs- significant difference between nonoperative and opera-
ing and medical care and rehabilitation without tive treatment was detected with respect to time to union,
compounding the patients disability. Fracture treatment rehabilitation, range of motion, or orthopedic complica-
should also produce a high rate of union, maintain tions when standard orthopedic fracture management
length, prevent angular deformities, and maximize func- principles were followed. The incidence of medical com-
tional return (6). plications, such as deep venous thrombosis and decubi-
tus ulcers, occurred more frequently in the nonoperatively
treated group. Early operative treatment is preferred for
General
these fractures, unless closed treatment can be carried out
Because high-energy accidents often cause multiple predictably with a functional orthosis, allowing the
injuries, emergent stabilization of organ systems is vital patient to participate in the rehabilitation process.
prior to treatment of the extremity fractures (1,7). Femur
fractures are the most common long bone injury, followed
Conclusion
by tibia, humerus, radius, and ulna fractures. Recent data
at specific lower-extremity (LE) fracture sites has shown The trend toward ORIF of all long bone fractures in acute
improved outcome with operative stabilization regardless SCI has been evolving over decades. Initially, all fractures
of the neurologic status. were treated nonoperatively. Later, nonoperative treat-
ment was reserved for complete injuries with functionless
extremities. Recent studies have demonstrated that non-
Femur
operative treatment retards healing and leads to short-
Twenty-seven femoral shaft fractures were recently ening and angular deformities. We now feel that most LE
reviewed (6). Fracture outcome was evaluated in the com- fractures should undergo ORIF whether the patient is
plete and incomplete groups as well as operative versus neurologically complete or incomplete. Upper extremity
nonoperative fractures. One-third of the nonoperative fracture care should follow general orthopedic principles,
femurs displayed a tendency toward nonunion. Angular with a tendency toward ORIF.
and shortening deformities were also significantly higher
compared to the operative group. Decubitus ulcers in the
nonoperative group were more severe and required a FRACTURES IN CHRONIC SCI
higher number of surgical procedures than those occur- (PATHOLOGIC FRACTURES)
ring in the operative group, resulting in additional bed-
rest (8,9). Early operative stabilization of femoral shaft Fractures in patients with established SCI are usually
fractures was the preferred method of treatment regard- caused by relatively minor injuries, although major acci-
less of the patients neurologic status. Finally, nonopera- dents may occur (10). Paraplegic patients have a higher
tive fractures demonstrated a decreased, not increased, incidence of fractures than quadriplegic patients (11).
healing potential, which is in contradistinction to the gen- This difference results from the greater degree of function,
eral consensus. mobility, and participation in various physical activities.
The fracture rate is ten times greater in individuals with
complete lesions of the cord compared to those with
Tibia
incomplete lesions (12). The overall incidence of patho-
The outcome of thirty-four tibia fractures in acute SCI logic fractures in chronic SCI patients, treated at SCI cen-
was also the subject of a recent report. Fractures treated ters, has been reported at 1.4 to 6 percent. This is prob-
nonoperatively healed at a prolonged rate, with nearly 50 ably a low estimate, because many patients are treated
percent proceeding to delayed union or nonunion. Open locally and/or as outpatients and do not report to SCI cen-
reduction and internal fixation (ORIF) enhanced the rate ters. Many patients with trivial swelling in painless
and time to union. Except for type III open injuries, frac- extremities may never seek medical attention.
tures treated with early ORIF had the least orthopedic Patients with established SCI have numerous altered
and medical complications. Routine ORIF of tibia frac- physiologic factors that effect the management of long
tures in both incomplete as well as complete SCI patients bone fractures. These patients have a negative nitrogen and
was advocated (4). Incomplete patients routinely became calcium balance, as well as impaired skin sensitivity, which
ambulatory with orthopedic devices. Freedom from results in a propensity to develop decubitus ulcers, along
shortening and angular deformities facilitated this with poor wound and fracture healing. They may have
orthotic care. ongoing, frequent urinary tract infections and bacteremia.
MANAGEMENT OF LONG BONE FRACTURES 537
Superficial and deep wound infections should be antici- cation. Acetabular erosion may result from osteoporotic
pated. Marked lower extremity spasticity may cause sig- bone. Outcomes of cementing of the prosthesis versus
nificant angular and rotary deformities, displacement of bony ingrowth have not been determined.
fracture fragments, as well as penetration of the fracture Intertrochanteric fractures with pin and side plate
fragments through the skin. Significant spasticity may pre- may result in a failure of fixation because of the osteo-
clude the use of nonoperative management for shaft frac- porosis. Nonoperative management is associated with
tures. Casts may cause skin breakdown and splints may varus deformity of the hip, which is often acceptable. The
not provide adequate immobilization. On the other hand, new intramedullary hip fixation devices offer an
osteoporosis may prevent adequate fixation (13). improved fixation technique, which is currently our pre-
Chronic, complete SCI males lose 33 percent of their ferred operative method if surgery is deemed necessary.
bone mineral density (BMD) at the knee within a year
of injury. Complete SCI females lose 40 to 45 percent of
Femoral Shaft
their BMD within a year of their injury. Patients with
known LE fracture have a 50 percent or greater loss of Femoral shaft fractures may be very difficult to manage
BMD at their knee. This 50 percent reduction of BMD nonoperatively, especially in the spastic patient.
at the knee seems to be the fracture threshold for this pop- Nonunions are commonplace in displaced fractures, as
ulation. The SCI female is at fracture risk at the knee soon has been established in the acute SCI population. Casts
after her SCI. These populations should be counseled and splints are frequently inadequate. External fixation,
regarding their osteoporosis and potential for fracture. although effective, is not without its own set of compli-
Unexplained limb swelling, especially knee and ankle cations, such as pin infection fixation failure through
swelling, should cause them to seek medical attention osteoperotic bone and stress fracture through the pin site
(13,14). at a later date. Immobilization by external fixation may
At Rancho Los Amigos National Rehabilitation interfere with patient positioning and predisposes to
Center (RLANRC), a review of all patients with chronic trophic ulcers (16). The treatment of choice for displaced
SCI and a pathologic fracture from 19911995 was femoral fractures or nondisplaced fractures that lose
undertaken. The study included seventy-three patients reduction during nonoperative methods is interlocking,
with ninety-nine fractures. The fracture location varied, intramedullary rodding. Locking is necessary, because the
but the overwhelming anatomic location was in the lower femoral canals are wide and rotational deformities as well
extremity. A breakdown of the lower-extremity fractures as nonunion cannot be prevented with standard nails.
revealed the supracondylar femur, followed closely by the
proximal tibia, had the highest concentrations of injury.
Distal Femur/Proximal Tibia
All of the fractures healed, and based upon this review,
fracture management guidelines in this patient popula- These non- or minimally displaced fractures are the most
tion were developed. commonly encountered fractures in the SCI patient popu-
lation. Well-padded splints or knee immobilizers are the best
methods of treatment (17), providing adequate immobi-
General
lization along with a low incidence of skin breakdown. Fre-
Supracondylar fractures of the femur are the most com- quent inspection of the skin is required. Pillow splints have
monly encountered in the SCI population, followed by been widely utilized in the past, but are bulky and make
proximal tibial and then tibial shaft fractures. The high mobilization difficult. Long leg cylinder casts are occasion-
incidence in these regions is consistent with the loss of ally used when these other nonoperative methods fail.
bone mineral in this region. The cause of the fracture was Surgery for displaced fractures may be necessary (10). The
frequently a fall out of wheelchair on the osteoporotic use of retrograde IM nails through the knee is advantageous.
part of the knee and torsion to the LE.
Tibial Shaft
Hip
Management of tibial shaft fractures can be treated non-
Displaced femoral neck fractures are uncommon, but operatively if the tibia is non- or minimally displaced.
pose a difficult management dilemma. If left untreated, Displaced fractures require interlocking intramedullary
the femur may migrate proximally, unbalancing the pelvis fixation.
and causing pressure sores (15). Displaced femoral neck
fractures result in nonunion even when reduced and
Ankle
pinned, because fixation failure occurs. Prosthetic
replacement may be prudent, although dislocation after Ankle fractures usually exhibit low-energy fracture pat-
surgery may occur, as well as late subluxation and dislo- terns. Such a fracture can be treated with a padded pos-
538 MUSCULOSKELETAL CARE
terior splint or cam walker. Healing may be either bony nails if technically possible. Distal femoral and proximal
or fibrous union without complications. Varusvalgus tibial fractures can be treated with well-padded splints.
angular deformities are unacceptable, because malleolar Most displaced tibial shaft fractures require an inter-
pressure sores may develop. locking intramedullary nail. Distal tibia, ankle, and foot
fractures respond to splinting. Upper-extremity fracture
Foot management should follow general orthopedic principles.
Metatarsal base and toe fractures are usually nondis-
placed and unite without consequence using a soft splint. References
Splinting in the equinous position may lead to a fixed 1. Comarr A, Hutchinson R, Bors E. Extremity fracture of patients
deformity. with spinal cord injuries. Am J Surg 1962; 103:732.
2. McMaster W, Stauffer ES. The management of long bone frac-
ture in the spinal cord injured patient. Clin Orthop 1975; 112:44.
3. Garland D, Reiser T, Singer D. Treatment of femoral shaft frac-
UPPER EXTREMITY tures associated with acute spinal cord injuries. Clin Orthop 1985;
197:191.
4. Garland D, Saucedo T, Reiser T. The management of tibial frac-
Preservation of upper-extremity function is especially tures in acute spinal cord injury patients. Clin Orthop 1986;
important in these patients. The management of patho- 213:237.
logic fractures, which only occur in the aged SCI popu- 5. Garland D, Jones R, Kunkle R. Upper extremity fractures in the
acute spinal cord injury patients. Clinic Orthop 1988; 233:110.
lation and in the occasional quadriparetic patient, should 6. Eichenholtz S. Management of long-bone fractures in paraplegic
follow general orthopedic principles with an emphasis on patients. J Bone Joint Surg 1963; 45A:299.
preserving function. Bone mineral loss is not significant 7. Sobel M, Lyden J. Long bone fracture in a spinal cord injured
patient. J Trauma 1991; 31:1440.
and fixation devices are not prone to failure. 8. Baird R, Kreitenberg A. Treatment of femoral shaft fractures in
the spinal cord injury patient using the Wagner leg lengthening
device. Paraplegia 1984; 22:366.
9. Freehafer A, Hazel C, Becker C. Lower extremity fractures in
CONCLUSION patients with spinal cord injury. Paraplegia 1951; 19:367.
10. Keating JF, Ken M, Delorgy M. Minimal trauma causing fractures
A high index of suspicion of an extremity fracture is nec- in patients with spinal cord injury. Disability and Rehabilitation
essary in the assessment of paralyzed patients who 1992; 14108.
11. Ragnarsson K, Sell GH. Lower extremity fractures after spinal
develop a swollen extremity. A careful history of any cord injury: A retropective study. Arch Phys Med Rehabil 1981;
trauma, however minor, should be carefully sought in 62:418.
patients with SCI, spina bifida, and end-stage multiple 12. Ingram RR, Suman RK, Freeman MA. Lower limb fractures in the
chronic spinal cord injury patient. Paraplegia 1989; 27:133.
sclerosis. Late-onset swelling may be the only sign for 13. Garland D, Stewart C, Adkins R, Hu S, Rosen C, Liotta F, Wein-
diagnosis. Other general signs, such as an increase in spas- stein D. Osteoporosis after spinal cord injury. J Orthop Research
ticity or sweating, should also alert the clinician to the 1992; 10:371.
14. Garland D, Maric Z, Adkins R, Stewart C. Bone mineral density
possibility of an occult fracture. about the knee in spinal cord injured patients with pathological
The management of extremity fractures in patients fractures. Contemp Orthop 1993; 26:375.
with pathologic fractures varies depending on the loca- 15. Garland D. Clinical observations on fractures and heterotopic
ossification in the spinal cord and traumatic brain injured popu-
tion. Hip fractures continue to be a difficult management lation. Clin Orthop 1988; 233:86.
problem because of the poor fixation obtained in the 16. Baird R, Kreitenberg A, Eltorai I. External fixation of femoral
osteoporotic bone using current fixation devices. Some shaft fractures in spinal cord injury patients. Paraplegia
1986;24:183.
intertrochanteric, subtrochanteric, and femoral fractures 17. El Ghatit A, Lamid S, Flathy T. Posterior splint for leg fractures
should be managed with interlocking, intramedullary in spinal cord injured patients. Am J Phys Med 1981; 60:239.
40
Bone Loss and
Osteoporosis Following
Spinal Cord Injury
s with many aspects of spinal cord mechanical loading was initiated in astronauts (hypograv-
tus, they have been replaced as the primary tools for eval- developed by several clinical researchers for the focused
uating skeletal status. assessment of the midshaft and distal femur and proxi-
Immediately after injury, urinary excretion of calcium mal and midshaft tibia, because these represent common
and hydroxyproline increases; these are the first markers fracture sites in persons with SCI. The nature of DXA is
of bone resorption. In general, the proportions of release that it provides site-specific quantification of bone mass
of these metabolites indicate that osseous tissue is resorbed and thus allows accurate quantification of regional skele-
as a whole (i.e., osteopenia) rather than demineralized (i.e., tal response. This is in contrast to systemic-level assess-
osteomalacia) and that while both formation and resorp- ment of bone metabolism from urine and serum markers,
tion rates are increased in acute SCI (i.e., elevated bone which have limited value for the evaluation of regional
turnover), resorption is disproportionately increased, thus responses.
resulting in a net loss of bone tissue. Blood calcium levels Evidence of bone loss is restricted to regions below
are infrequently elevated in adults post-SCI, although the level of lesion, consistent with a causal influence of
hypercalcemia is fairly common in pediatric SCI (1). Table unloading (elimination/reduction of mechanical stimuli).
40.1 summarizes the acute changes in metabolites and hor- Presumably, incompleteness of injury and residual
mones related to bone turnover (27). The majority of mechanical loading may attenuate some of the bone loss
these markers return to normal levels within 1 year post- (911), but there has been relatively little investigation
SCI, indicative of adaptation to a new skeletal steady state. of this effect. It has also been suggested that spasticity may
However, parathyroid hormone (PTH) and vitamin D lev- protect against bone loss (12), but this has not been
els may remain low and calcitonin high even after this (8). demonstrated conclusively and several studies report a
These metabolic responses, both acute and long-term, do negative effect (13,14).
not seem to differ between patients with tetraplegia and
paraplegia.
Acute Pattern
A reduction in bone mass appears to begin immediately,
Regional Specificity
although there are few studies documenting a change in
Currently, the measurement of bone mineral density by BMD in the very early days and weeks following acute
dual energy X-ray absorptiometry (DXA) is the most SCI. Lack of such data is attributable primarily to diffi-
commonly used methodology for the assessment of bone culty with patient measurement in the initial post-injury
mass, usually reported as bone mineral areal density phase (because of other medical or surgical complications
(BMD) in g/cm2. Standard measurement sites include the or contraindications for moving the patient). An addi-
proximal femur, lumbar spine (L1L4), and the total body tional limitation may arise related to the accuracy and
(although the latter is primarily used for body composi- sensitivity of DXA technology; there may be a minimal
tion and research studies rather than for clinical assess- decrement of bone tissue that cannot be measured, and
ment). In addition, regional scanning protocols have been immediate loss may be insufficient to be detected.
TABLE 40.1
Serum and Urinary Markers of Bone Response to Spinal Cord Injury
Urinary calcium Osseous tissue breakdown Elevated excretion, peaks at 810 weeks at 2x
normal
Urinary hydroxyproline Osseous tissue breakdown Elevated excretion, peaks at 3 months, remains
high for as long as 8 months
Serum total alkaline phosphatase Bone formation (nonspecific) Gradual elevation over 59 weeks post-SCI
Serum osteocalcin Bone formation Low initially, increased during 6 months post-SCI
Urinary pyridinoline and Bone resorption Increases to peak at 23 months post-SCI
deoxypyridinoline;
Serum N-telopeptide of
collagen type 1
PTH Bone turnover Initially low, may return to normal after 6 months
25-hydroxy Vitamin D Bone turnover Decreased, despite normal serum calcium
BONE LOSS AND OSTEOPOROSIS FOLLOWING SPINAL CORD INJURY 541
Initially, bone mass is rapidly lost in the lower paralysis in tetraplegia and positively to overloading
extremity at a rate of approximately 1 to 2 percent per (caused by manual wheelchair mobility) in paraplegia
month, with a reduced rate of loss by the end of the first (12,2224).
year at most sites. There appears to be more rapid loss Specific findings vary depending on the patient pop-
in the distal femur and proximal tibia compared with ulation and measurement technology, but the general pat-
the proximal and midshaft femur (13,1517). In one tern remains consistent across all studies. Further, lower-
study that employed peripheral quantitated computed extremity bone loss does not differ between individuals
tomography (pQCT), local bone envelope changes with tetraplegia and paraplegia; this is not surprising,
within the tibia were assessed. During the first 2 years because the alteration in mechanical environment is essen-
post-SCI, cortical, subcortical, and trabecular regions tially the same for both. Table 40.2 summarizes findings
of the tibia lost 33, 44, and 52 percent of bone mass, from a number of studies that have determined regional
respectively (18). responses to chronic SCI throughout the skeleton
Bone mass reduction throughout the lower extrem- (1315,1719,22,2428).
ity appears to continue for the next 1 to 3 years at a It should be noted that all clinical findings regard-
slower rate, but few prospective studies systematically fol- ing long-term bone response are taken from retrospective
low patients for longer than 1 year, so these specific data studies. Thus, the responses noted in these studies are
are lacking. From available reports, the initial period of derived from cross-sectional data and use comparisons
accelerated bone loss seems to resolve between the sec- with peak or age-matched ambulatory reference values to
ond and fourth year post-injury. estimate change. Further, the majority of studies have
Vertebral bone mass is not decreased after SCI and not demonstrated an independent effect of age or dura-
paralysis; instead, increased BMD of 6 to 11 percent has tion of paralysis, although there is one recent report by
been reported (13,19,20). A reasonable explanation for Baumann and colleagues on co-twins (as a surrogate for
the lack of observed bone loss in the vertebrae is that the a prospective study), which indicates a continued bone
trunk remains functionally loaded because of wheelchair decrement with a longer duration of SCI (29). Epidemi-
sitting and mobility regardless of the paralysis of spinal ologic factors, such as selective mortality and nonequiv-
musculature. In addition, a possible explanation for the alent cohorts, could contribute to bias and error in the
observed increase in vertebral bone mass may be that very interpretation of cross-sectional findings as representative
early bone loss occurs acutely because of initial immobi- of long-term changes.
lization or bedrest, but this loss is subsequently reversed
with wheelchair sitting. No systematic studies have
explored these theoretical mechanisms, but the pattern
of normal or increased vertebral bone mass has consis- TABLE 40.2
tently been described in a number of published reports. Regional Bone Response to
In contrast to most published accounts, one study used Chronic Spinal Cord Injury
QCT to evaluate vertebral bone density and identified
significant cancellous bone loss that was not observed DIFFERENCE IN BONE MASS AS A
General versus Individual obtained by QCT and bending stiffness was calculated
Response and Gender Effect from a specialized measurement device (31). Cross-sec-
tional area, maximal moments of inertia, and bending
The response rate (following acute SCI) and magnitude stiffness were reduced in individuals with SCI, compared
of loss (in chronic SCI) can vary tremendously among with ambulatory controls. Although the determination of
individuals, but this is rarely discussed or studied. An indi- geometric properties and calculations of bone strength are
vidual response that varies from the norm or average not yet commonly available measurement technologies,
response may be caused by preinjury bone mass, pre- there has been growing interest throughout the osteo-
injury habitual activity patterns (i.e., regular participa- porosis community regarding how such measurements
tion in sports versus sedentary lifestyle), nutritional sta- might augment the interpretation of bone mass measured
tus both pre- and post-injury, hormonal status, and by DXA.
underlying genetic factors. As yet, there have been no
studies to elucidate the potential role of such influences
Measurement Considerations
on individual bone responses, and no algorithms have
been developed to guide individual patient management. Finally, it is important to be aware of technologic issues
Generalized patterns and average responses are certainly that can influence bone densitometric measurement. Per-
useful for understanding the impact of paralysis on the haps more than any population, individuals with SCI pre-
skeleton, but more focused research remains to be done sent unique challenges to the assessment by DXA. Patient
on the contributions of other factors and systems, in order positioning can be extremely difficult because of joint
to most effectively assess and manage the skeletal health contractures in the hip, knee, and occasionally ankle; and
of individual patients. care must often be taken to avoid extended pressure to
Because the great proportion of persons with SCI are the heel or buttocks, especially in the presence of decu-
male, most of the studies have reported findings only for bitus ulcers. Although egg crate foam or other padding
men or include only a small sample of women. One study can be used to relieve pressure, this can also contribute
focuses on bone response in women with SCI and reports to improper positioningthe body region might be ele-
similar rates and patterns of loss as those observed for vated or rotated away from the standard position. It is
men with SCI for spine, hip, and proximal tibia, although frequently impossible to assess a given region by DXA
a greater increase in spinal bone mass is noted, as well as because of the presence of extensive heterotopic ossifica-
a finding of continued loss of tibial bone with longer tion (HO)whereas less-severe or undiagnosed HO may
duration of injury (30). invalidate any accurate assessment of bone mass when it
occurs in the area of measurement even though it may not
be readily apparent (32). Osteophytes or other calcific
Geometric Changes and
abnormalities in the spine can also falsely elevate bone
Estimates of Bone Strength
mass. Thus, it is essential that DXA assessment be per-
Bone mass, as determined by DXA, is the most commonly formed by an experienced technologist who fully under-
used measurement for the determination of skeletal sta- stands the issues of correct patient positioning and that
tus and prediction of fracture risk. A measurement of DXA findings be interpreted by an experienced clinician
BMD in a specific region is roughly equivalent to the who understands the potential sources of measurement
cross-sectional area of the region scanned and is thus a error, especially those caused by skeletal artifact. It is best
reasonable estimate of bone strength in compression. if both have specific knowledge of these issues as they
However, the strength of bone to resist a bending or tor- relate to patients with SCI.
sional load is dependent on its geometry and distribution
of mineralized tissue. Determination of the geometric
Synopsis
properties of bone has been accomplished by use of QCT
and by calculations derived from densitometric measure- Beginning immediately after SCI, rapid bone loss occurs
ments, as well as by direct, noninvasive mechanical test- in paralyzed, sublesional regions, seemingly caused by the
ing (various prototype devices have been used; none is cessation of habitual loading and in proportion to the
commercially available or widely used at this time). In one magnitude of those loads. The majority of this effect is
study, geometric properties were assessed in the midshaft noted in the lower extremity, with no difference between
femur from cortical measurements derived from DXA paraplegia or tetraplegia. A divergent effect is observed
and concurrent radiography; calculation of cross-sec- in the upper extremity: bone is gained with paraplegia and
tional moments of inertia and polar moments (I and J) lost with tetraplegia. Bone loss is rapid initially and
provided estimates of rapid reduction in bone strength appears to resolve by the fourth year post-SCI. In con-
in parallel with reduction in bone mass (17). In another, trast, no reduction in bone mass is observed in the spine,
direct imaging of tibial cortical bone geometry was presumably because mechanical loading is maintained via
BONE LOSS AND OSTEOPOROSIS FOLLOWING SPINAL CORD INJURY 543
an upright posture; rather, a slight increase is observed values between those who do and do not fracture, there is
in this region. The majority of studies have used DXA evidence of significantly reduced bone mass in persons
technology which, if applied and interpreted appropri- with SCI who have sustained a fracture compared with
ately, provides an accurate quantification of regional bone those who have not (31,34,35). Therefore, there is cer-
mass response, although heterotopic ossification and joint tainly a need for further determination of the factors that
contractures can contribute to measurement and inter- predispose to elevated fracture risk, and guidelines should
pretation error. There may be a differential response in be established regarding the interpretation of regional bone
cancellous versus cortical bone (greater versus less loss, mass as predictive of fracture for individuals with SCI.
respectively), but this remains to be thoroughly investi-
gated by the use of QCT technology. Although there
Etiology of Bone Fractures
appears to be agreement that a new level of bone home-
ostasis is achieved at a reduced skeletal mass, evidence More than 90 percent of long bone fractures after SCI
of an ongoing, long-term response to paralysis is incon- occur in the lower extremity and are distributed approx-
sistent; neither is there clear indication of an independent imately evenly in the femur and tibia, with a concentra-
age effect. Finally, research into the sources of individual tion around the knee. The pattern and morphology of
variation in bone response is needed to best guide indi- these fractures is distinct in several ways from those
vidual patient assessment and management. expected with the osteoporotic syndrome in the general
population, wherein the majority of fractures occur in the
proximal femur and thoracic vertebrae (compression). By
LONG BONE FRACTURESOCCURRENCE contrast, in persons with SCI, the majority are spiral frac-
AND RISK FACTORS tures of the diaphyses and simple, bending fractures of
the distal femur and/or proximal tibia. The latter fre-
Bone Density and Fracture Risk
quently involve displacement of the shaft into the epiph-
Throughout the osteoporosis literature, decreased bone ysis, often after the original fracture event (Kiratli, unpub-
mass is considered indicative of elevated fracture risk, lished data).
although there is significant overlap between those with Because the location and morphology of lower
low bone mass with and without fracture history as well extremity fractures appears to be distinct in individuals
as those with normal bone mass who have experienced with SCI, the underlying causes might also be expected
fracture. Although there have been attempts to establish to differ from those in the general population. That is,
a fracture threshold for the general population, this has whereas supported body weight imposes one of the most
been of limited value because of the complexity of frac- significant risks in the osteoporotic syndrome for spon-
ture etiology. In 1994, a World Health Organization taneous vertebral and hip fractures in nonparalyzed indi-
Study Group designated, for the spine, a cutoff of 1.0 viduals, this factor is essentially removed in persons with
standard deviation (SD) below peak BMD (i.e., bone mass SCI; the majority of long bone fractures in SCI patients
measured by DXA) as indicative of osteopenia and more occur as a result of falls from the wheelchair and during
than 2.5 SD below peak as indicative of osteoporosis (33); transfer activities (3638). Fractures also commonly
this has been adopted by Medicare for reimbursement occur during low-strain activities such as ADLs and
purposes. However, there is no evidence that would sup- range-of-motion exercises but rarely result from trau-
port the application of this designation for clinical diag- matic causes. Finally, a portion of fractures have no
nosis in the SCI population nor has there been sufficient known etiology, which may be due to a lack of awareness
study of the magnitude of bone loss associated with frac- of the causal event rather than indicative of a sponta-
tures in this population to warrant an alternate thresh- neous fracture.
old designationwhich would have to be defined specif-
ically for each skeletal region. Further, a body of research
Rate of Occurrence
indicates that nonskeletal factors contribute significantly
to bone fracture in the general population, which explains Relatively few studies of fracture epidemiology have been
some of the variability in fracture prediction based on undertaken in this population over four decades
bone mass alone. Similar factors (such as susceptibility to (36,3842). Some of these estimates are confounded by
fall, velocity and acceleration of activity, soft tissue the inclusion of fractures that occurred at the time of ini-
padding, contact substrate, etc.) would be expected to tial injury, which makes the results difficult to interpret;
apply for individuals with SCI, although the etiology of furthermore, most of these data have been derived from
fractures may be different. retrospective chart review and thus neglect any fractures
Nonetheless, although normal bone mass values treated outside the hospital under study. Overall popula-
for a person with SCI are substantially lower than ambu- tion prevalence (percent who have ever experienced a
latory reference values, and there is overlap in bone mass fracture divided by population at risk) appears to be as
544 MUSCULOSKELETAL CARE
high as 25 percent (37), with an incidence (percent who guidelines for follow-up of fractures in this population
have experienced a new fracture divided by population and data on long-term outcomes are lacking.
at risk) estimated at 2 percent per year (38). Many indi- Depending on the location and severity of the frac-
viduals experience more than one fracture and some have ture, the person with SCI may experience greatly reduced
as many as five or six, although there does not seem to independence and the need for increased care and assis-
be evidence of refracture risk at the site of a previous frac- tance during fracture recovery. There have been no pub-
ture. Depending on the specific study and population, var- lished reports documenting these impacts, but data col-
ious estimates have been published regarding mean dura- lected at our institution indicate that a significant
tion of SCI at the time of fracture; estimates are generally proportion of patients report the following (Kiratli,
in the range of 11 to 15 years post-SCI. Consistent with unpublished data):
the pattern of bone loss, fracture occurrence appears to
be somewhat lower in the first 5 years after injury, but Hospitalization
there is no evidence of an association with time since Reduced independence, often because elevated or
injury (37,38); that is, the frequency of fractures does not extended leg rest limits wheelchair mobility and
increase with longer duration of SCI, although this restricts self-care activities
deserves additional study. Time lost from work or school because of hospital-
Fractures seem to occur more frequently in persons ization and mobility issues
with paraplegia than tetraplegia, in those with complete Need for additional attendant care and/or medical
rather than incomplete injuries, and in those with flaccid assistance at additional expense
rather than spastic paralysis (36,39,40). Although no
studies explore these observations in any detail, there may
Synopsis
be a component of risk related to activity level and ele-
vated exposure to risk; persons with greater mobility and As in the general population, reduction in bone mass con-
levels of participation may be at higher risk of activities tributes to elevated fracture risk, but the causes and mech-
that might result in fracture. anism of bone failure seem to differ for the SCI population.
The majority of fractures occur in the lower extremity, and
these are concentrated around the knee. However, no con-
Symptoms of Long Bone Fracture
sensus guidelines exist for predicting the fracture risk in this
In contrast to acute fracture in nonparalyzed individu- population from specific bone mass values, and consistent
als, the fracture event may go unnoticed by an individ- estimates of population prevalence and incidence are lack-
ual with SCI because of the absence of sensation. Symp- ing in the literature. Fractures most commonly result from
toms of a fracture may include local swelling and edema, nontraumatic events such as falls from the wheelchair as
elevated temperature, increased spasticity, and autonomic well as during transfers and ADLs. Clinicians should be
dysreflexia; occasionally, there will be an obvious defor- aware of the heightened fracture risk following SCI and
mity in the limb because of a displaced fracture. While be alert for symptoms such as swelling, edema, increased
attempting to perform a transfer or manually moving a spasticity, etc., that might indicate a fracture of which the
limb, the person who has unknowingly sustained a frac- patient is unaware. Generally, successful bony union is
ture may notice that a portion of the limb is displaced or achieved, with malalignment and shortening occurring
unstable. In some instances, the fracture can be heard commonly. Regardless of healing outcome, the impact of
(loud pop) rather than felt. Because fractures often long bone fracture can be substantial, requiring hospital-
result from minimal impact, patients and clinicians should ization and resulting in reduced mobility and independence
be aware of the physical symptoms regardless of whether and the need for increased assistance.
a fall or other traumatic event has taken place. The assess-
ment should include manual examination, and radi-
ographs should be ordered. THERAPEUTIC INTERVENTIONS
especially for pharmacologic therapy, and studies of all Several studies, however, have generated findings
types of interventions are ongoing. suggesting a positive effect on bone mass, although the
results are somewhat inconsistent. Hangartner and co-
workers (18) demonstrated a reduction in tibial bone loss
Weight Bearing
with early FES intervention, whereas Belanger and asso-
Attention has traditionally focused on the reintroduction ciates (50) observed a recovery of bone mass in the dis-
of mechanical loading through supported weight bearing tal femur and proximal tibia, but not the tibial diaph-
or tilt table standing as a means to reduce or reverse the ysis, after 24 weeks of quadripceps FES, at least 1 hour
bone loss caused by paralysis. This approach seems intu- per day, 5 days per week. Finally, Mohr and colleagues
itive, because the primary component of unloading is the (51) observed an increase in tibial bone mass after 12
cessation of upright standing and walking. Unfortunately, months of FES training, but this effect was not sustained
no studies have yet been able to document a significant when the level of training was reduced. In another study,
skeletal effect. Treatment regimens usually use a standing although no significant effect on bone mass was found
frame or leg braces, for 30 to 45 minutes per day, 3 to 5 in a 9-month FES study of nine individuals, a subset of
times per week, for up to 6 months. Presumably, these four who trained at a high workload ( 18 watts) were
amounts of loading are insufficient to replace the magni- found to have increased bone mass in the distal femur
tude, frequency, and duration of mechanical stimuli attrib- after 3 months (52). Among the various studies that have
utable to habitual normal ambulation. Further, standing been performed, training protocols, subject characteris-
protocols usually involve static standing, whereas normal tics, and measurement procedures differ, thus confound-
mechanical stimulation of bone (e.g., walking) occurs ing a comprehensive consolidation of findings.
through repetitive, cyclic loading (bending and unbending). One published report describes a bone fracture
In one study, a protocol using a functional electri- occurring during FES, and the investigators warn that
cal stimulation modality coupled with ambulation underlying bone fragility should be considered before
(Parastep1 Ambulation System) was implemented in an undertaking a training program (53).
attempt to reproduce the combined influences of muscle
activity and axial loading (43). However, no change in
Pharmacologic Therapy
femoral neck bone mass was demonstrated after 12 weeks
of training. Weight bearing and simulated ambulation The third category of intervention has targeted meta-
were accomplished through a graded program but not bolic processes, primarily by use of bisphosphonates to
fully realized until near the completion of the program. inhibit or slow bone resorption. In the past, attempts
Finally, one recent report of an early intervention at treatment with calcitonin, etidronate, clondronate,
program of standing and treadmill walking has yielded and toludronate have been mostly unsuccessful in
evidence suggesting a preventive effect (44). Peripheral reversing loss of bone mass, although there has been evi-
QCT was used to assess cancellous bone density in the dence of a positive effect on bone turnover markers
distal tibia following a 25-week program, and little or (5457). That is, these agents have been shown to
no reduction in bone mass was noted in patients who reduce markers of bone resorption and thus indicate
stood or ambulated, whereas untreated patients lost 8 to that they may have an effect on preventing of bone loss.
10 percent bone mass. However, no treatment effect was However, there has been little convincing evidence of a
noted for the cortical shell. The sample was small and het- protective effect on bone mass (in some cases, bone mass
erogeneous, but this may be the first study to implement was not directly measured).
an intervention trial with direct monitoring of bone mass Recently, intraveneous pamidronate has been shown
response in the acute period following SCI. to reduce bone mass decrement after acute SCI by approx-
imately 50 percent during a 12-month trial (58). Patients
with complete (ASIA A) and incomplete (ASIA D) lesions
Functional Electrical
were studied, and this finding was most apparent in those
Stimulation (FES)
with incomplete SCI who were able to regain ambulatory
Similarly, FES has been used to reintroduce mechanical status. A significant reduction in bone loss was observed
loading through muscular contractions as a preventive with pamidronate treatment in the proximal and distal
measure. However, perhaps for reasons similar to those femur but not in the proximal tibia. This was a nonran-
cited in the previous section (short duration of loading, domized, controlled prospective trial with a relatively
relatively weak contractions, insufficient muscle mass), small sample (twenty-four patients total, treated and non-
there is little conclusive evidence of a protective influence treated). Limitations cited by the authors include the
on bone mass, although there are many reports of posi- inconsistent findings (no effect at the tibia); evidence from
tive effect on muscle mass (4549). Further, most of these the resorption marker, NTX, of insufficient dosage fre-
studies have been performed on postacute patients. quency; and the necessity of intravenous injections.
546 MUSCULOSKELETAL CARE
Nonetheless, this study provides credible support for the proven therapeutic measures. In the past decade,
potential role of this therapeutic agent. increasing numbers of studies have been conducted to
Another recent study provides promising evidence quantify bone loss after SCI and evaluate the effects of
of a positive effect of alendronate (Fosamax) in reduc- various injury-related characteristics (level and com-
ing bone loss after acute SCI (59). After 18 months of pleteness of injury, duration, etc.) Measurement
treatment with alendronate and calcium, significantly less methodologies have included DXA, QCT, and mechan-
bone loss was observed in proximal femur and distal tib- ical testing devices. Skeletal sites have included lumbar
ial BMD, compared with patients receiving only calcium. spine and proximal femur (standard DXA and QCT
There was no evidence of an effect in the tibial diaphysis. sites) as well as regions throughout the femur and tibia,
These preliminary data were presented in a recent con- each especially designed and described in individual
ference abstract (Sept 2001), so it is not possible to crit- clinical centers and labs to assess the regions where frac-
ically evaluate the full study. Nonetheless, these findings tures are most prevalent and, therefore, bone loss is
represent a substantial sample (fifty-one patients) and a expected to be most pronounced.
randomized, controlled study design and thus constitute
encouraging support for a treatment effect.
Generalizable Knowledge
At this time, there are several ongoing clinical trials
to evaluate the preventive or treatment effects of several From this body of clinical research, generalizable knowl-
bisphosphonates in both acute and chronic SCI; the results edge includes the following:
of these studies are anticipated in the next few years.
Bone loss begins early in skeletal regions below the
level of paralysis/paresis
Synopsis
There appears to be greater and more rapid loss of
Proposed countermeasures for osteoporosis include the cancellous than cortical bone, and a pattern of
restoration of mechanical loading through upright weight increased magnitude of loss in more distal skeletal
bearing and assisted ambulation or stimulation of mus- regions
cular activity by FES and inhibition of bone resorption by Residual mechanical loading of the limbs, with
pharmacologic means. In general, attempts to restore incomplete injury and residual or recovered ambu-
mechanical loading have been disappointing, although a lation, may attenuate the pattern and rate of bone
few studies have reported positive effects. Thus, although loss
currently no widely accepted physical regimens to prevent Reduction in bone mass leads to increased fracture
bone loss following SCI exist, there may be hope for a risk, but there is a substantial overlap among those
mechanical countermeasure to be defined and validated who do and do not (or have yet to) sustain a frac-
in the future. Until recently, as with pharmacologic treat- ture, even at very low bone values
ment of osteoporosis in the general population, study A great proportion of lower-extremity fractures
results have been inconclusive to identify an effective ther- results from low or insignificant trauma, including
apeutic agent to prevent bone loss post-SCI. However, falls from wheelchair, during transfer activities, and
recent studies indicate the positive effects of pamidronate during ADLs, rather than from accidents (such as
and alendronate (i.e., reduction in acute bone loss), and MVA) and high impact incidents
other current clinical research may further substantiate Although few studies have demonstrated the effec-
these results. Still, specific therapeutic regimens have not tiveness of any countermeasure, such efforts are
been defined, and the clinical use of bisphosphonates for ongoing; recently, there have been promising
the reversal of bone loss has yet to be recommended for accounts of preventive outcomes from long duration
patients with SCI. Finally, it is expected that the use of FES, early standing and assisted walking, and intra-
physical or pharmacologic countermeasures, if specific venous pamidronate in the acute stage.
regimens can be proved successful, would be most effec-
tive for preventing early bone loss rather than for restora-
Recommendations for Clinical Management
tion of bone mass in long-standing SCI.
The recommendations for clinical management of the
patient with SCI with regard to bone loss and fracture
CONCLUSION prevention should include the following:
Although it has long been known that osteopenia and Individual assessment of bone mineral status at sev-
osteoporosis occur following SCI and lead to increased eral sublesional skeletal sites and prospective follow-
fracture risk, there are no consensus-derived clinical up, particularly between the initial injury and 4 years
guidelines for assessment and management and no post-SCI
BONE LOSS AND OSTEOPOROSIS FOLLOWING SPINAL CORD INJURY 547
Physical rehabilitation to include standing, if possible, 9. Dauty M, Perrouin-Verbe B, Maugats Y, Dubois C, Mathe J.
Supralesional and sublesional bone mineral density in spinal cord-
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Patient, family, and caregiver education regarding the impact of SCI. Topics in Spinal Cord Injury Rehabilitation
2001; 6:4760.
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spine and lower extremities years after spinal cord lesion. Para-
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Topics in Spinal Cord Injury Rehabilitation 2001; 6:3746.
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43. Needham-Shropshire B, Broton J, Klose K, Lebwohl N, Guest R, immobilization hypercalcemia using calcitonin and etidronate.
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Sussi E. Changes of tibia bone properties after spinal cord injury: effect on bone density in patients with acute spinal cord injury.
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electrical stimulation-induced lower extremity cycling on bone spinal cord injury. Arch Phys Med Rehabil 1999; 80:243251.
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1990; 71:207209. abstract]. J Bone Min Res 2001; 16 (Suppl 1):s219.
41
Functional Restoration
of the Upper Extremity
in Tetraplegia
ith continued improvements in the for tetraplegic patients possessing active wrist extension.
FIGURE 41.2
in transfer that it cannot be reliably expected to do use- team members must play a role in the decision-making
ful work after transfer. process and must share in the frustrations as well as the
Moberg (11,12) encouraged us to also consider rewards.
remaining sensory resources as well. If sufficient propri-
oception remains in any part of the hand (typically the
thumb and index fingers), the patient can control his hand PALO ALTO EXPERIENCE
without having to keep it in view. If the hand lacks pro-
prioception, the patient may instead use his eyes for affer- In 1977, a multidisciplinary upper-extremity clinic was
ent control. However, lack of proprioception limited the established as part of the Spinal Cord Injury Center at
patient in performing bimanual activities. Today, we the PAVAHCS. This clinic was staffed by rehabilitation
equate static two-point discrimination of less than 12 to physiatrists, therapists, psychologists, orthotists, and sur-
15 mm as indicating the presence of proprioception. The geons. During the past 23 years, over 400 tetraplegic vet-
classification was extended somewhat to include a deter- eran patients have been examined. Of this group, 145
mination of the presence or absence of active elbow exten- patients elected to undergo some type of functionally ori-
sion. This system was adopted by the International Fed- ented upper-extremity surgery. The primary functional
eration of Hand Surgery Societies and it is used by goals depended on those muscle and sensory resources
essentially all surgeons involved in the care of the upper remaining under voluntary control following neurologic
extremities of these patients. stabilization.
Deltoid-to-Triceps Transfer
Two surgical procedures are advocated for restoring
active elbow extension. In the United States, transfer of
the power of the posterior half of the deltoid to the tri-
ceps tendon, as described by Moberg (12), is preferred.
We prefer this procedure when the posterior deltoid is
strong and the elbow has near-normal passive extension.
The procedure, performed under general anesthesia,
involves detaching insertion of about one-half of the del-
toid (usually the posterior half) from the humerus and
connecting this portion of the muscle via the triceps ten-
don insertion into the olecranon process of the ulna (Fig-
ure 41.8). Several technical modifications have been
described, but the goals are similar. After surgery, the
FIGURE 41.7
SURGICAL RECONSTRUCTION
Elbow Extension
Erik Moberg brought to our attention the importance of
active elbow extension for the SCI patient. The wheel-
chair-bound individual depends on good shoulder and
elbow power and stabilization to push a wheelchair,
transfer from bed to chair, and to perform pressure
releases to prevent pressure sores. For the tetraplegic
patient, lack of a functional elbow extension results in a FIGURE 41.8
much-reduced functional environment The world of the
tetraplegic patient is determined by the range of motion The operative steps for the deltoid-to-triceps transfer are
of his or her upper extremity. Without the ability to illustrated.
FUNCTIONAL RESTORATION OF THE UPPER EXTREMITY IN TETRAPLEGIA 557
B
A
C D
FIGURE 41.10
become a candidate for surgical reconstruction of key sion or very strong wrist extension, because Grade 4 is
pinch. The brachioradialis is truly a useful spare part. such a subjective parameter.
Patients in the O or OCu-2 category are potential
candidates for the creation of a lateral or key pinch, as
Key Pinch Procedure
described by Moberg (6). Conceptually, this is a very sim-
Patients functioning at the O or OCu-2 level can actively ple operative procedure and, importantly, is essentially
extend the wrist against gravity and against some resis- totally reversible should the patient decide he was more
tance. This is a presenting feature for a large number of functional before surgery. This is a consideration for a
our patients. This correlates to the C5C6 functional clas- patient with weak wrist extension who is many years from
sification. They may still have relatively weak wrist exten- his injury. The key pinch procedure may be combined with
FUNCTIONAL RESTORATION OF THE UPPER EXTREMITY IN TETRAPLEGIA 559
A
brachioradialis-to-ECRB transfer if greater wrist exten-
sor power is deemed advantageous. It represents an auto-
matic pinch in that the tendon of the thumb flexor, the
flexor pollicis longus (FPL), is anchored to the palmar sur-
face of the radius under such tension that with wrist exten-
sion the thumb tip is pulled against the side of the index
finger (Figure 41.11). The other fingers are usually left sup-
ple and the patient frequently must learn to roll these dig-
its into some flexion to provide a platform against which
the thumb can act (Figure 41.12). Gravity is needed to flex
The illustration demonstrates the proper position for stable This IC OCu-2 patient has undergone bilateral Moberg key
key pinch between the pulp of the thumb and the radial side pinch procedures as described above, A and B. He achieved
of the index finger. Note the bow-stringing of the tendon of 4 pounds of pinch force. He was able to discard all his
the flexor pollicis longus as forceful pinch is achieved. orthoses.
560 MUSCULOSKELETAL CARE
Active Key Pinch the side of the index finger irrespective of wrist position.
Further wrist extension augments the power of the bra-
After gaining experience with the key pinch procedure
chioradialis transfer (Figure 41.15). Although we have
described by Moberg, we have chosen to modify the key
had less experience with this transfer, other colleagues in
pinch operation for the patient with very strong wrist
the United States have enjoyed good results and very sat-
extension, meaning a strong O or OCu-2 or OCu-3
isfied patients (22). In several instances, we have per-
patient. These patients do not require augmentation of
formed bilateral hand surgery in OCu-2 or OCu-3
wrist extension by, for example, brachioradialis transfer.
patients and have chosen to provide a traditional key
Instead of tenodesing the FPL to the radius as described
pinch via FPL tenodesis on one side and active key pinch
above, in a single-stage procedure the following steps are
via brachioradialis muscle transfer to the flexor pollicis
accomplished (Figure 41.14):
longus tendon on the opposite side. The patients have
enjoyed the different functional attributes of each method
1. The carpometacarpal (CMC) is fused to pre-posi-
and find that certain functions are easier with one extrem-
tion the thumb tip to contact the index finger mid-
ity, whereas others are more efficiently performed with
dle phalanx.
the opposite hand.
2. The extensor pollicis longus (EPL) tendon is
anchored to the extensor retinaculum on the dorsum
of the wrist. Grasp- and Release-Procedures
3. The brachioradialis muscle tendon unit is trans-
For our tetraplegic patients who possess additional
ferred to the tendon of the flexor pollicis longus.
motor resources distal to the elbow, more complicated
reconstructions are possible but not always indicated.
Using this procedure, the patient depends on grav-
ity to flex the wrist and tighten the extensor pollicis
longus tenodesis, thus opening the grip. Then by actively
contracting the brachioradialis, the thumb flexes against A
FIGURE 41.15
FIGURE 41.14
Following surgery, the patient is able to open the grip (A) by
The operative steps to achieve active key pinch are illustrated. allowing gravity to flex the wrist. This causes the thumb to fully
Power for pinch comes from transferring the brachioradialis extend. By contracting the brachioradialis, the patient can
into the tendon of the flexor pollicis longus. achieve strong key pinch, irrespective of wrist position (B).
FUNCTIONAL RESTORATION OF THE UPPER EXTREMITY IN TETRAPLEGIA 561
These patients are, of course, also candidates for either We have seen so few patients in this international
procedure described above if reversibility seems an category that we cannot determine outcome in a mean-
important consideration. In the early years of our expe- ingful manner. Nonetheless, the procedure is predictable.
rience, we frequently offered only key pinch recon-
struction for OCu-4 and even OCu-5 patients. As we
Strong Grasp and Release
have gained experience, so we have gained confidence
in the teams decisions. As we have gained confidence in Patients in the international classification OCu-5 category
being able to achieve a reliable outcome, we have usually have a functioning triceps. They are also func-
extended the riskbenefit equation to include more com- tioning at some reasonable level of efficiency because they
plex procedures. frequently have some residual effective natural flexor ten-
The OCu-4 patient has four strong muscles func- odesis in their paralyzed fingers and thumb. Decisions
tioning distal to the elbow usually the brachioradialis: the regarding the appropriateness of surgery for this category
two radial wrist extensors, the extensor carpi radialis need careful consideration (24,25). However, these
longus and extensor carpi radialis brevis and, frequently, patients have the most to gain from carefully planned and
the pronator teres muscle. In addition, the majority of executed surgery.
OCu-4 have some function (albeit minimal) in the flexor We have performed several variations of the two-
carpi radialis. They can flex the wrist with some force stage grasp-and-release procedure, described for the OCu-
when the forearm is pronated with gravity assisting the 4 group above, except that an additional muscle is avail-
weak flexor carpi radialis. For these patients, we have able for transfer. Typically, we have tried to avoid fusion
devised a two-stage procedure that takes advantage of the of any joints. Extension of the fingers or the release phase
presence of two expendable muscles for transfer, the is obtained either via extensor tenodesis, as described
extensor carpi radialis longus and the brachioradialis. above, or occasionally by active muscle transfer to the
Prerequisites for surgery include near-normal passive combined tendons of the thumb and finger extensors. The
wrist movement and reasonably flexible fingers. flexor phase includes several procedures to maximize the
At the initial procedure, we test the power of the versatility of thumb pinch and provide powerful finger
extensor carpi radialis brevis under local anesthesia to flexion. This includes provision for some ability to abduct
be certain that this muscle is sufficiently strong to extend the thumb away from the palm. The goal of pinch is still
the wrist. If this is the case, we proceed to fuse the car- directed toward more of a lateral, side, or key pinch but
pometacarpal joint of the thumb to preposition the thumb frequently the patient can also pinch closer to the index
ray for pinch and, at the same time, attach the tendons fingertip and can manipulate smaller objects, such as a coin
of the extensor digitorum communis and extensor polli- into a telephone coin receptacle, with some efficiency.
cis longus to the dorsum of the radius. This is referred to These patients have truly gained the most from their
as the release phase. After a period of healing with wrist surgery, particularly in terms of efficiency of movement
flexion, the fingers and thumb extend. This is a very nat- and function. Instead of requiring 1 hour to dress them-
ural and synergistic motion and is easily learned. selves in the morning, they accomplish the same tasks in
Some weeks to months later, the flexor phase is per- 10 to 15 minutes. Few do many more tasks postopera-
formed. Two active muscletendon transfers are per- tively, but all perform these tasks with much greater effi-
formed, including transfer of the extensor carpi radialis ciency and less expenditure of energy (Figure 41.16).
to the combined tendons of the flexor digitorum profun-
dus, so that this muscle smoothly flexes all of the fingers;
OCu-6, OCu-7, and OCu-8 Level Patients
and transfer of the brachioradialis to the tendon to the
flexor pollicis longus. These transfers permit the patient Patients classified at the OCu-6 level possess active digi-
to actively close the fingers around an object and flex the tal extension but lack thumb extension. These patients,
fingers to provide a platform against which the thumb can as is true of the OCu-5 category, already have very func-
actively pinch through the power of the brachioradialis tional hands and surgery should be approached cau-
transfer. The hand and wrist are immobilized for about tiously. Again, however, they can achieve truly spectacu-
4 weeks and then exercised under the supervision of our lar results by all parameters including pinch strengths
therapist. Because they are synergistic transfers, reedu- equal to 5 Kg and grasp strengths between 10 and 15 Kg.
cation is relatively easy and quick. Many months are nec- The results of surgery can be a nearly normal hand except
essary before full strength is achieved. Also, because the that ulnar intrinsic function is not reestablished. They
patient lacks active finger extension, daily digital exten- require only adding an extensor force for the thumb and,
sion exercises are necessary to avoid the development of at the same operation, multiple tendon transfer to achieve
finger flexion contractures. A static night splint that main- balanced thumb pinch and strong finger grasp. Therefore,
tains the proximal interphalangeal joints in near full only one procedure is necessary and their period of depen-
extension should be worn essentially indefinitely. dence is minimal.
562 MUSCULOSKELETAL CARE
Other Presentations
Some injury patterns do not fit easily into the interna-
tional classification. Patients with so-called central cord
B injuries have hands that defy classification. These patients
require prolonged studies and frequent reexamination
before formulating a surgical plan. Temporary nerve
blocks have been particularly helpful in determining the
procedure of choice.
FUNCTIONAL NEUROMUSCULAR
STIMULATION
A B
FIGURE 41.18
The lateral or key pinch grasp pattern is demonstrated. This is useful for acquiring smaller objects. Grasp openA. Grasp closedB.
564 MUSCULOSKELETAL CARE
A B
FIGURE 41.19
The tip-to-tip or opposition pattern is illustrated. This is useful for grasping larger objects. Grasp openA. Grasp closedB.
RESULTS AND CONCLUSIONS these patients according to the proposed major preoper-
ative goals.
Recent reports show encouraging results for tendon trans- The first goal was restoration of elbow extensor sta-
fers in the tetraplegic upper extremity (3034). With con- bilization and active elbow extension. Two surgical pro-
tinued experience, we have found it necessary to modify cedures were employed, with relatively specific indica-
surgical indications and procedures more or less contin- tions for each. Most commonly, transfer of posterior
ually. We recommend videotaping the hand performing deltoid to triceps, as popularized by Moberg was per-
several standard functions, such as opening a package or formed. It was performed exclusively until 1985. Begin-
holding a pen, both pre- and postoperatively. We recom- ning in 1985, for patients presenting with a preoperative
mend formalizing the recordkeeping process and the team elbow contracture greater than 30 degrees, contracture
dynamics and decision-making processes. release was combined with medial routing of biceps to tri-
Because of the significant functional demands placed ceps, as popularized by Zancolli.
by tetraplegic patients on their upper limbs, especially the We examined twenty-one patients who had under-
need to bear weight on their hands, the durability of oper- gone elbow extensor reconstruction more than 10 years
ative procedures performed to enhance upper limb func- prior. In the fifteen patients who had posterior deltoid-to-
tion in tetraplegic patients has been questioned. Percep- triceps transfer, ten had bilateral transfers. All fifteen had
tions about the durability of some of the earlier surgical required a motorized wheelchair as their primary means
recommendations, such as the surgically created, wrist- of movement before surgery. Ten years following surgery,
driven flexor-hinge hand, still influence the referral pat- nine now used a push chair as their standard chair, and
terns to surgeons of modern day physiatrists and spinal four others used a push chair at least some of the time.
cord rehabilitation specialists. To provide some current Three patients had undergone bilateral posterior deltoid-
perspective to this issue, we were able to locate and per- to-triceps transfer; they were able to self-transfer in the
sonally examine forty-five patients who had been oper- early postoperative period. All three continued to be able
ated on at least 10 years prior to evaluation. We analyzed to perform this monumental (for a tetraplegic) task. In the
FUNCTIONAL RESTORATION OF THE UPPER EXTREMITY IN TETRAPLEGIA 565
posterior deltoid-to-triceps group, four patients had had, on average, a more powerful grasp. This may be a
required a preliminary release of elbow flexion contrac- product of preselection, however, because the stronger
ture. One of the four examined 10 years postsurgery had patients were selectively chosen for intrinsic substitution.
developed a recurrence of contracture greater than 30 We conclude from this long-term analysis that care-
degrees. fully chosen upper-limb reconstructive procedures in
In the six patients having biceps-to-triceps transfer properly educated patients are both effective and durable.
(all needing contracture release), two could use a push Systematic postoperative reevaluation of their upper
chair, but not exclusively so. None had developed a recur- limbs should become a standard part of the more typical
rence of elbow contracture. interval examinations of more generally studied systems
The second goal was the restoration of pinch for the such as renal and bladder function, blood pressure, and
weaker patients and, and for the stronger patients, pinch pulmonary status. Aside from their brains, the upper
and grasp, as well as the ability to open the hand. Inter- limbs remain the most important residual resource for
national classification OCu-2 patients typically had a key tetraplegic patients. Frequent reevaluation of their upper
grip fashioned by tenodesis of the flexor policis longus limbs makes ultimate good sense.
to the radius. Seven were evaluated at more than 10 years. As mentioned earlier, few of our patients perform
Five had maintained pinch strength essentially equivalent many new activities. Typically, a good or excellent result
to that demonstrated 6 to 12 months following surgery. means that the patient performs many of the same func-
Overall pinch strength in this group was 25 newtons. tions but with much greater efficiency. The rewards for sur-
One patient lost to follow-up for many years was an geons, rehabilitation medicine specialists, and therapists are
O2 patient. He presented with hands typical of Hansens best expressed by one of our patients who replied to a ques-
disease, with dissolution of thumb phalanges, presumably tion requesting his feelings on his outcome, its not as
the result of forceful thumb use in an insensate and less much as I hoped for, but its much more than I ever had.
than fully self-aware patient. This unique finding does
point to the potential for harm when strong grasp is
restored in the totally insensate limb that is then subjected References
to weight-bearing.
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between their initial surgery and the date of long-term 10. Campbell CC, Koris MJ. Etiologies of shoulder pain in cervical
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42 The Medical Management
of Pressure Ulcers
lthough thought to be completely rial processes involved in pressure ulcer development and
8.9 percent respectively. Whiteneck et al. (4) reported a treatment of pressure ulcers, but an untold amount of
23 percent incidence of pressure ulcers among persons expense is borne by the individual because of loss of
with SCI at the C4 or higher level during the first 5 years income and added family expenses (5,6,328,29). The
following rehabilitation, and an incidence of 30 percent human and societal costs of pressure ulcers are immea-
6 or more years following injury. surable, and costs far greater than monetary are incurred
Pressure ulcers are the most common preventable by the individual, including human suffering, pain, dis-
cause for hospitalization in numerous studies. Davidoff figurement, and body image change (30). The loss of pro-
(5) found 39 percent of people with SCI rehospitalized ductivity, the effect of prolonged bed rest on health and
in the first year after SCI were admitted for pressure ulcer wellness, and the effect of pressure ulcers on family
treatment. Schryvers et al. (6) reported 34 percent of per- dynamics, depression, and community reintegration
sons with SCI require three or more admissions to the although difficult to measureare significant.
hospital for treatment of pressure ulcers, whereas 25 to
85 percent of all persons with SCI develop a pressure ulcer
severe enough to require treatment at some time during PATHOPHYSIOLOGY
their life (718). Fuhrer et al. (19) found that 33 percent
of persons with SCI living in the community had one or A pressure ulcer is an infarctionno different from a
more pressure ulcers of at least stage 1 severity, and 13.6 myocardial or cerebral infarction. As such, it has an
percent had one or more stage 3 or 4 pressure ulcers. ischemic event, an opportunity for reperfusion, and a
Salzberg et al. (15), studying 219 veterans, reported penumbra with an opportunity for complete or partial
that only 19.6 percent had no history of pressure ulcer recovery. This is a localized event; however, the general
occurrence. A later study of 800 veterans identified that health of the individual contributes significantly to the
62.4 percent of the persons experienced pressure ulcers likelihood of developing a pressure ulcer, and the ability
1 to 52 years following SCI (16). The researchers found to heal it. As stated in our operational definition, pressure
that among persons with a history of pressure ulcers, the ulcers are caused by compression of soft tissues between
number of different pressure ulcer occurrences ranged the proverbial rock and a hard place. Compression of
from one to twenty-four, with a median number of three blood vessels feeding the soft tissues decreases or com-
per individual. More than 77 percent of the pressure pletely obstructs blood flow into these tissues, resulting
ulcers reported in the two studies cited above were stage in local ischemia. If the compression is unrelieved, and
3 or worse. the ischemia prolonged beyond the tissues tolerance, this
More than 70 percent of persons with SCI develop becomes tissue necrosis. Like other models of prolonged
multiple pressure ulcers or a recurrence of previous pres- ischemia, there is likely a central area of necrosis and a
sure ulcers (14,18). Schryvers et al. (6) reported that over surrounding area that is ischemic, but not yet necrotic.
one-third of the pressure ulcers occur at the same site. This surrounding penumbra, if reperfused within a criti-
Death caused by the consequences of pressure ulcers has cal time period, will resolve without necrosis. However,
been found to occur in 7 to 8 percent of persons with SCI if reperfusion is incomplete or delayed, the area of necro-
(20). sis will expand and include much of the penumbra. There
Epidemiologic data not only substantiate that pres- is likely a window of opportunity to rescue soft tissues
sure ulcers are a lifelong threat to persons with SCI, but during this period of ischemia. The duration of the win-
studies have provided insight into the costs to both the dow of opportunity will depend on the tissues ability to
individual and healthcare services when pressure ulcers resist ischemia (based on the general health and condition
occur. Pressure ulcers account for annual treatment costs of the tissue) and the severity of tissue injury. Much of our
in the United States exceeding $1.3 billion per year effort to prevent pressure ulcers includes activities to pre-
(16,21,22). It has been estimated that $66 million is spent vent ischemia by minimizing or attenuating the degree of
in annual hospitalizations for the treatment of pressure tissue compression (mattresses, specialty beds, cushions,
ulcers experienced by persons during the acute phase of turning regimens, etc.) or by identifying early signs of
SCI (12). Over 25 percent of the total cost for treating pressure ulcer (skin checks) and attempting to minimize
persons with SCI throughout their life is the result of pres- the degree of tissue necrosis (avoiding sitting or lying on
sure ulcers. Between $20,000 and $70,000 are spent for an area that looks injured or vulnerable). An important
treatment of each pressure ulcer, and expenses continue piece of the puzzle still missing is early detection of
to escalate at a rate of $1,000 to $5,000 per year impending tissue ischemia and necrosis so that interven-
(1,2327). Hospital charges for each pressure ulcer result- tions to maximize reperfusion and limit the degree of
ing from increased lengths of stay (average 11.9 days), necrosis can be initiated before severe damage takes place.
supplies (average $2,230), additional staff utilization To better understand the pathophysiology and heal-
(average $7,900 or 50-percent increase), and surgical pro- ing of pressure ulcers and to assist with their assessment
cedures (average $15,000) contribute to cost estimates for and management, it is helpful to differentiate between
THE MEDICAL MANAGEMENT OF PRESSURE ULCERS 569
external and internal factors that contribute to pressure ulcer formation in persons with SCI by inhibiting the
ulcer development and delayed healing in the SCI popu- delivery of oxygen and nutrients to ischemic areas (35).
lation. Internal factors may be subdivided into three Researchers have noted normal vasomotion and reactive
groupsthose factors that are present as a direct result hyperemia in persons with SCI under resting conditions,
of the SCI; those factors that are more common in peo- but greatly diminished vascular reactivity to external
ple with SCI, but not a universal result of SCI; and those stimuli. Vascular capacity for dilation, abnormal vascu-
factors that are a result of aging or concomitant disease. lar tone, and increased venous pressures have also been
shown to contribute to a significantly decreased blood
content in tissue following reactive hyperemia (34).
External Factors
Pressure Shear
Although many factors contribute to the development of Shear forces also contribute to the development of pres-
a pressure ulcer, pressure is the most important. The age- sure ulcers. Dinsdale et al. (37) demonstrated that the
old adage, where there is no pressure there is no pres- pressure level capable of disrupting blood flow in the skin
sure ulcer, remains true today. Kosiak (31,32), in his clas- is reduced by half in the presence of significant shear
sic animal experiments, found that deep-tissue necrosis forces. Because capillary loops in the skin are oriented ver-
develops during prolonged episodes of low pressure, recur- tically, they are more resistant to direct, vertically oriented
rent pressure without adequate relief, or short episodes pressure. However, if lateral forces (shear) are exerted on
high pressure. He reported a positive relationship between them, they are readily kinked, resulting in dermal
tissue damage and duration of pressure. He also found that ischemia and necrosis. Therefore, any force that exerts
tissue damage could be prevented with frequent episodes both pressure and shear is more likely to result in dermal
of pressure relief, even in the face of high pressures. tissue injury.
Tissues vary in their sensitivity to pressure-induced
ischemia. Muscle is most sensitive because of its high
Positioning
metabolic activity and ease of vessel compression,
whereas skin is most resistive because of its lower meta- The presence of limb contractures, scoliosis with or with-
bolic activity and the orientation of capillary loops in the out pelvic obliquity, or severe spasticity will aggravate
dermis (33). The time between the onset of pressure and positioning problems. These abnormalities in positioning
occurrence of tissue damage varies from person to person in turn contribute to problems of pressure and shear.
but may require as little as 1 to 6 hours of constant min- Abnormal positioning increases pressures over specific
imal pressure. Even when initial signs of pressure damage bony prominences and creates a higher risk for pressure
are recognized early and relief measures initiated, necro- ulcer development because of higher pressures and shear
sis to deep muscle and fat may have already occurred (25). forces. Most guidelines recommend that persons at
This supports the anectodal reports of patients who state bedrest be repositioned every 2 hours, and that persons
that their pressure ulcer presented clinically as a deep in wheelchairs practice pressure relief activities every 20
crater many days following a pressure event, (i.e., missed to 30 minutes. However, studies on healthy individuals
transfer or fall), without any surface indication until the and elderly nursing home residents have demonstrated
wound appeared. The deep tissues become ischemic and that skin temperature, redness, and pressure at the
eventually necrotic, whereas the superficial tissues appear sacrum, ischium, and trochanter significantly increase and
relatively normal until the wound opens. show signs of early tissue destruction after as little as 1
Reactive hyperemia, the normal physiologic to 1.5 hours of a static position. Pressure over bony
response to pressure, signals an increased vascular per- prominences of persons with SCI during wheelchair sit-
fusion to the upper dermis and corrects the local meta- ting has been demonstrated to increase following as lit-
bolic debt accumulated during ischemia. However, pro- tle as 10 to 15 minutes (3840). It remains unclear how
longed pressure may lead to a toxic build-up of long a person may remain in one position without pres-
metabolites in tissue and ultimately an inflammatory sure injury. The answer will likely depend on the tissue
response (3436). Impaired reactive hyperemia, examined tolerance to pressure and will vary from person to per-
by microciruclation studies, has been demonstrated to son. Positioning to prevent and manage pressure ulcers
occur in persons with SCI and is accompanied by an is a critical issue to address for all persons with SCI.
abnormal resting blood flow, referred to as diminished
vasomotion. Because vasomotion is centrally mediated
Excessive Skin Moisture and Maceration
via the nervous system and locally controlled by myogenic
mechanisms, it is postulated that impaired vasomotion Maceration decreases the skins tolerance to mechanical
further aggravates the multifactorial process of pressure stress, making dermal injury more likely to occur during
570 MUSCULOSKELETAL CARE
an episode of shearing and pressure. Urine, feces, and lower degree of hydroxylation of collagen-specific amino
sweat are potential irritants to tissue and serve to break acids in skin biopsies taken from below the level of injury.
down the bodys normal barrier against damage and This may lead to diminished tensile strength of the skin
infection. Conversely, skin permitted to become exces- after SCI. Urinary excretion of collagen metabolites is
sively dry because of dehydration, lack of environmental increased in people with SCI, possibly indicating an
humidity, or extended periods of cold is also susceptible underlying defect of collagen metabolism.
to damage (41).
Muscle Atrophy
Internal Factors Related to SCI
Atrophy of muscle below the level of injury results in a
decreased surface area to distribute pressure during sit-
Paralysis and Sensory Impairment
ting or lying and theoretically provides less padding
Young (42) found that 33 percent of persons with para- of bony prominences. It is unclear if the padding pre-
plegia and 47 percent of persons with quadriplegia devel- sent in neurologically intact individuals is due to muscles
oped pressure ulcers during their initial hospitalization. or adipose tissue. Clearly persons with excess adipose tis-
However, the greatest difference was found in persons sue will develop pressure ulcers, as do people with severe
with complete SCI versus incomplete SCI at all levels of muscle atrophy. Further research in this area is necessary
injury. Persons with complete quadriplegia had an inci- to understand this.
dence of 57 percent, compared to 39 percent for persons
with incomplete quadriplegia. Persons with complete
Altered Peripheral Circulation
paraplegia had an incidence of 41 percent, whereas per-
sons with incomplete paraplegia had only 22 percent inci- Altered circulation is frequently seen after SCI. Decreased
dence. Fuhrer et al. (43) reported that the presence of a venous return because of impaired muscle pump in the
pressure ulcer was more likely in persons with a lower legs and loss of normal vasoconstriction during alter-
ASIA motor score and greater disability as measured by ations of blood pressure and posture are especially com-
the Functional Independence Measure (FIM). mon. Venous and lymphatic congestion may contribute
The loss of protective sensation results in a decreased to pressure ulcers in the pelvic region in the same way that
awareness of tissue ischemia. In the fully sensate person, venous congestion contributes to venous stasis ulcers in
tissue ischemia is painful. In the fully sensate person, early the lower extremities. The alterations in vasoconstriction
detection of an uncomfortable sensation results in a con- may result in transient hypotension, which may be clini-
scious or unconscious shift of the body. However, in a per- cally important if other factors contribute to put a person
son with decreased or absent sensation, the usual signals at risk for pressure ulcer development.
of impending tissue damage are not present and no behav-
ioral changes result. In the case of myocardial infarction,
Common Internal Factors Not a
chest pain leads to an immediate change of behavior (stop
Universal Response to SCI
doing whatever one may be doing and seek immediate
medical assistance). Myocardial ischemia may be reversed
Endocrine Abnormalities
if appropriate action is taken acutely to prevent reper-
cussions. Early symptoms allow early interventions. Conditions such as SCI, trauma, major surgery, liver fail-
Because of the loss of sensory feedback, persons with SCI ure, myocardial infarction, burns, alcoholism, and mal-
are not able to take appropriate action (immediately get nutrition have been found to be associated with the sup-
off the area of ischemia) and ischemia proceeds to infarc- pression of gonadotropin secretion and secondary
tion. Persons with SCI are taught to reposition themselves hypogonadism. A primary testicular disorder causing ele-
frequently during the day, independent of sensation and vated gonadotropin levels may also occur as part of the
to inspect their skin at least twice each day. These activ- cascade of chronic illness and debilitation (46). Wang (47)
ities are much more complicated when the person with reported that 12.7 percent of sixty-three otherwise healthy
SCI has high tetraplegia and is not able to perform inde- men with SCI for an average of 6 years exhibited low
pendent weight shifts or skin inspections. serum testosterone levels. Approximately one-third of the
subjects had low serum thyroxine, thyrotropin, cortisol,
growth hormone, and plasma adrenocorticotropic hor-
Collagen Metabolism
mone accompanying the diminished serum testosterone
Collagen metabolism appears to be altered below the level levels. Testosterone, an essential element for collagen
of injury in SCI. Donovan et al. (44) demonstrated an up integrity and health, influences tissue responsiveness to
to five times slower wound healing below the level of both pressure ulcer formation and healing. Although there
spinal cord injury. Rodriguez (45) also demonstrated a is a dearth of studies testing the relationship between
THE MEDICAL MANAGEMENT OF PRESSURE ULCERS 571
hypogonadism and the formation of pressure ulcers in per- increased body temperature leads to at least a 28-percent
sons with SCI, it is hypothesized that men who are hypo- increase in the levels of metabolites, especially lactate and
gonadal following SCI may be more likely to develop a urea, in the sweat. Mahanty et al. (51) noted a significant
pressure ulcer and to experience delayed wound healing. rise in skin temperature within 2 to 5 minutes about the
Diabetes may impair wound healing or increase the ischium and sacrum of SCI patients sitting in wheelchairs.
risk of pressure ulcer development in persons with SCI. The peak temperatures were proportionally related to the
Wound healing is delayed in the presence of poorly con- magnitude and duration of applied pressures. Vistnes (52)
trolled blood sugars. The use of corticosteroids for other demonstrated that elevated skin temperature is greatest
medical conditions will also delay wound healing. over areas subjected to repeated pressure stress even when
Hypogonadism, hypothyroidism, and other endocrino- pressure relief activities are performed. Finestone et al. (53)
logic disorders should be sought in the case of nonheal- determined that a correlation exists between erythema size
ing pressure ulcers. and skin temperature following prolonged pressure on tis-
sue over bony prominences.
Anemia
Internal Factors Associated with
Persons with pressure ulcers often experience a mild ane-
Concomitant Disease and Aging
mia caused by decreased appetite, loss of serum and elec-
trolytes from the ulcer, secondary infection, and a general
Malnutrition and Vitamin or Mineral Abnormalities
debilitated status (25). Persons with a hemoglobin level
of less that 10 mg/dl have been shown to demonstrate Nutritional problems lead both to increased likelihood
delayed wound healing. Many persons with SCI and pres- of pressure ulcer development and decreased ability of
sure ulcers have evidence of anemia, often with a hema- the body to heal. Serum albumin has been found to cor-
tologic picture of an anemia of chronic disease. Tissue relate positively with pressure ulcer stage and negatively
metabolism may be impaired because of decreased oxy- correlate with pressure ulcer risk. It has also been shown
gen-carrying capacity, thus resulting in poor wound heal- that pressure ulcers improve with nutritional support.
ing. Perkash et al. (48) studied sixty-five males with SCI Because of the long half-life of serum albumin, other tests,
treated at a Veterans Affairs medical center between 6 including pre-albumin and transferrin may be more sen-
months and 32 years following injury and reported that sitive measures of change in nutritional status. With
52.3 percent experienced anemia without clinical and aging, our ability to absorb nutrients, including fat, pro-
hematologic evidence of hemolysis or hemorrhage. More tein, and minerals declines.
than two-thirds of these subjects found to be anemic had Vitamin C is necessary for the hydroxylation of pro-
tetraplegia, and the anemia was mild in all cases. Signif- line to hydroxyproline, which is critical for normal col-
icant fluctuations occurred in the hemoglobin levels from lagen formation. Likewise, zinc is necessary for collagen
day to day and hour to hour for all the persons with SCI synthesis and zinc deficiency leads to poor wound heal-
included in this study. Approximately half of the subjects ing. However, zinc deficiency is rare except in cases of
with anemia demonstrated decreased serum iron and severe malnutrition, and excess zinc interferes with cop-
decreased serum iron binding capacity but no other per absorption and may become toxic.
abnormalities in nutritional status. All subjects studied
who had large deep pressure ulcers were anemic, and 83.3
Smoking and Peripheral Vascular Disease
percent of the persons who were anemic had pressure
ulcers. Burr et al. (49) found an incidence of 49 percent Adequate blood flow is necessary for wound healing. In
of anemia for persons with SCI at least 1 year post-injury. addition to the vasomotor changes that occur after SCI,
Evidence from these studies substantiates that anemia is smoking and other risk factors for peripheral vascular dis-
a common occurrence for many persons with SCI, but the ease increase the risk for pressure ulcer development.
question of whether anemia is a precursor or consequence Peripheral vascular disease decreases arterial blood flow
of pressure ulcer development remains unanswered. to tissues, resulting in a decreased ability to heal. Smok-
ing also decreases arterial oxygen tension and causes an
immediate effect on tissue oxygenation. Therefore, smok-
Increased Tissue Temperature and Fever
ing has both an acute and long-term effect on the heal-
Increased tissue temperature leads to higher metabolic ing of pressure ulcers.
demands in deep tissues. This increases the likelihood that
a relatively brief episode of pressure will lead to deep-
Immune Function
tissue damage. Many patients report that their pressure
ulcer developed during an illness with accompanying fever. The natural and adaptive immune responses have been
Polliack et al. (50) reported that any event accounting for noted to be strikingly altered immediately following SCI.
572 MUSCULOSKELETAL CARE
Multiple communicating pathways exist between the ner- structure of the healed wound. Wound margins contract,
vous, endocrine, and immune systems and are associated granulation tissue fills the defect, and epithelial cells
with blood vessel function and lymphocyte processes. migrate from the wound margins to close the wound. This
Adhesion molecules, necessary for wound healing and phase is anabolic, whereas the inflammatory and matu-
adaptation to inflammation, have been noted to be ration phases are catabolic. The maturation phase occurs
reduced in immobile persons with SCI, but return to nor- from day 21 to 2 years. Collagen fibers reorganize,
mal levels when activity is increased. The intricate rela- remodel, and mature, gaining tensile strength. This
tionship between nutritional status, functional life-style, process continues until the scar has regained 70 to 85 per-
neuromuscular impairment, and immune function is often cent of the tissues original strength (57).
disrupted in persons with SCI and is considered by some Superficial partial thickness ulcers will heal with-
scientists to prompt the development of pressure ulcers and out scarring through regeneration of epithelium. This
affect the rate of healing from pressure ulcers (54). takes only 1 to 2 weeks because the epithelial cells migrate
from dermal appendageshair follicles and sweat glands.
Full-thickness superficial wounds heal by epithelial migra-
Aging
tion from the wound margins. The ulcer size will deter-
Thinning skin having less tolerance to trauma and shear mine the speed of healing. Deep pressure ulcers require
forces, commonly occurs with advancing age unrelated to much longer to heal, depending on the size of the wound.
SCI. Changes in muscular strength and activity level These wounds heal by a combination of wound contrac-
caused by immobilization, advancing age, or chronic ill- tion, granulation, and epithelialization.
ness also put an individual at higher risk for pressure Increased bacterial load is a common complication
ulcers. This may occur in a person with SCI during pro- that causes wounds to stagnate in the healing process. The
longed immobilization for medical complications includ- characteristics of unhealthy granulation tissue include
ing treatment for another pressure ulcer. superficial bridging, friability, and delay of expected heal-
ing rate (58). Bacterial load may lead to local or systemic
infection. Fever, leukocytosis, erythema, edema, indura-
Psychologic Factors
tion, odor, and increased drainage are standard signs of
Numerous psychologic and social factors have been infection. In the discussion of chronic wounds, however,
described in relation to pressure ulcers. Richards et al. occult infection is thought to be the primary culprit when
(55) studied the psychologic and social factors that pre- wounds do not progress. Robson (58) determined that
dispose persons with SCI to pressure ulcers. They found chronic wounds with bacterial loads of more than
that pressure ulcers correlated with advanced age, fewer 100,000 organisms/ml did not heal normally. Although
persons living in the home, and lower verbal intelligence. this is the definition of wound infection, 34 percent of the
They also found that the presence of pressure ulcers cor- stagnant wounds determined to be clinically free of infec-
related with subscores on the Minnesota Multiphasic Per- tion had bacterial counts 100,000 organisms/ml. This
sonality Inventory (MMPI) of low ego strength and self- study also pointed out that beta-hemolytic Streptococ-
esteem, and a high degree of impulsive behavior without cus can impair healing at lower colony counts.
thought of consequences.
Pathology of Nonhealing Wounds
NORMAL WOUND HEALING Pressure ulcers may stall in any of the phases of healing
somehow losing the ability to move from one phase to
This subject is covered in greater detail in Chapter 43. In another. Pressure ulcers may stall in the inflammatory phase
brief, normal wound healing has three phases. The because of delayed or blocked onset of the inflammatory
inflammatory phase, which begins within 4 hours of phase, as is seen with the use of glucocorticoids. Prolonged
injury, usually lasts from 4 to 6 days. Neutrophils arrive or heightened intensity of acute inflammation may also
first to kill bacteria and prevent colonization of the retard wound healing. Ongoing acute inflammation is com-
wound. Macrophages, which arrive later, kill bacteria, monly seen in chronic nonhealing wounds. Histologic find-
clean up the cellular debris, and recruit the fibroblasts ings of ongoing acute inflammation include perivascular
needed for tissue repair by secreting soluble cytokines. infiltrates of neutrophils, degraded extracellular matrix and
The balance between collagen synthesis and collagen abnormal fibroblasts, and a high content of neutrophil-spe-
breakdown is thought to be moderated by activated cific enzymes and proteinases in chronic wound fluid.
macrophages (56). The proliferative phase occurs Pressure ulcers that stall in the proliferative or tis-
between days 2 and 24, when granulation tissue is gen- sue-formation phase may do so because of failure to form
erated. Fibroblasts stimulate the ongoing production of granulation tissue because of the persistence of causative
collagen, which is responsible for the tensile strength and factors (pressure, ischemia, infection), inadequate com-
THE MEDICAL MANAGEMENT OF PRESSURE ULCERS 573
to the person with SCI and all SCI care providers. Reha-
TABLE 42.1
bilitation of the person with SCI should emphasize under-
Guidelines for Assessment and Prevention of
standing the risk factors that contribute to pressure ulcers,
Pressure Ulcers and Complications Following
working with the healthcare team for pressure ulcer man- Development of Pressure Ulcers: Managing
agement, and assuming personal accountability for health Modifiable Risk Factors
maintenance.
Conservative wound care includes a recurring cycle ASSESSMENT PREVENTION STRATEGIES
of prevention, assessment, and treatment with the per-
son as the focal point for all activities. The degree of EXTERNAL
health care intervention required is directly proportion-
ate to the persons ability to independently satisfy the Pressure Pressure relief every 1530 minutes
requirements of prevention, assessment, and treatment. during wheelchair sitting.
The Norton scale and the Braden scale are two of Turning every 2 hours when in bed.
the most common tools clinicians use to analyze a Use wheelchair cushions and mat-
patients risk for pressure ulcer development. The Norton tresses that distribute pressure.
scale has been tested extensively (61). Physical condition, Shear Keep clothing and linen wrinkle free.
Reduce sliding in bed and friction
mental state, activity, mobility, and incontinence are each
during transfers.
assigned a numeric value from 1 to 4. Protect extremities from trauma.
Scores may range from 5 to 20, with lower scores Positioning Correct flexible deformities.
being indicative of higher risk. The onset of risk begins Accommodate inflexible deformities.
with a score of 14, and a person is at high risk with a score Moisture/body Maintain clean dry skin.
of 12 or less. One study reported low reliability among hygiene
registered nurses who used the Norton scale and dis- Medication use Recognize that many medications can
agreement among experts as to its face validity (61). place the person at risk for pressure
The Braden scale, however, has been shown to be ulcers by clouding consciousness.
highly reliable when used by registered nurses (62). This Monitor for sedation or confusion.
scale has been tested in critical, acute, and extended-care BEHAVIORAL
settings with raters who were registered nurses, licensed
vocational nurses, and nurse assistants. It allows the asses- Smoking Smoking results in both short-term
sor to assign a numeric value, to six different indicators and long-term complications for
with potential scores from 4 to 23. Sensory perception, wound healing. Encourage smoking
skin moisture, physical activity, nutritional intake, friction cessation.
and shear, and the ability to change body position are each Alcohol use Alcohol use may interfere with behav-
iors to prevent pressure ulcers. Moni-
given a numeric value which is totaled to indicate a
tor for evidence for alcohol abuse.
patients potential for developing pressure ulcers. A patient
Cognitive Provide supervision for cognitively
with a Braden scale score of 16 or less is considered to be function impaired persons.
at risk. The exception is the geriatric patient who may be
at risk even with a score as high as 18. It is also interest- INTERNAL
ing to note that in SCI, values for sensory perception, activ-
ity, mobility, and friction/shear are all affected. Thus a per- Paralysis and Frequent pressure relief, regular skin
sensory loss inspection.
son with quadriplegia can achieve a maximum possible
Fever Be alert for pressure injury during
score of only 15, putting him at high risk for pressure ulcer febrile illness.
development even when not compromised. Risk assess- Anemia Maintain adequate hematocrit by eat-
ment scales specific to SCI have been proposed (15,16) but ing a well-balanced diet with appropri-
have had limited testing for validity or reliability. ate level of iron supplementation.
Many intrinsic and extrinsic factors can place a per- Malnutrition Maintain healthy body weight and
son at risk for the development of pressure ulcers or nutritional levels by eating a well-
delayed healing from pressure ulcers. Table 42.1 has been balanced diet.
constructed from Pressure Ulcer Prevention and Treat-
ment Following Spinal Cord Injury: A Clinical Practice
Guideline for Health-Care Professionals (63) as a gen- people are the first-line defenders against pressure injury.
eral guideline to be used by all individuals on the health- Education may be augmented with behavioral modifica-
care team, including the person with SCI. tion techniques, although these techniques have not been
Education of the staff, patient, and caregiver(s) is demonstrated to be effective in all situations. The great-
also an important component of prevention since these est effect has been seen in preventing the recurrence of
THE MEDICAL MANAGEMENT OF PRESSURE ULCERS 575
pressure ulcers based on intensive follow-up and moni- lying wound will require increased nursing care to turn
toring (64,65). Prevention of pressure ulcers is a multi- the patient more frequently or utilize a specialty bed to
disciplinary concern requiring full participation by the minimize pressure during bedrest.
person with SCI.
Assessing Wound Size
Principle #2: Correct the Underlying Factors
Wound size is used to monitor a wounds progress toward
Once an ulcer develops, a complete assessment should closure and provides information to determine the appro-
be done to determine the cause of the pressure ulcer, to priate wound care product to use. Attention must be paid
document the severity of the wound, and to determine to define how the measurements were taken. Even among
an appropriate management strategy. The purpose of skilled clinicians, objective measurement is difficult and
ongoing wound assessment is to document healing (or may be unreliable. Numerous techniques have been devel-
failure to heal) and to modify the management plan oped to objectively determine wound size. Three-dimen-
accordingly. By consistently using the same techniques sional linear measurement is the most frequently used
to monitor wound presentation and healing, the clinician technique among wound care professionals. The length,
will be able to identify the causative factors of the ulcer width, and depth of the cavity are measured and stated
and direct conservative healing. By using objective data in centimeters. A long-handled cotton-tipped applicator
points to support progress within a treatment plan, the and a disposable ruler are standard tools for measure-
clinician is able to provide justifiable, expeditious care ment. Linear measurements have been thought to be unre-
to patients with even the most complex wounds. Frequent liable because of a lack of standardization for which
assessment of the pressure ulcer location, size, staging, dimensions constitute the length or width. In addition,
wound bed, exudate, wound margins, and the periulcer there is error of up to 0.5 cm with each measurement
skin should be performed and recorded. Because vari- using this system. It is good practice for the whole team
ability exists between the assessment skills and to measure the dimensions using the same technique. One
approaches for each practitioner, it is advisable to stan- recommendation is to define length as the wound mea-
dardize the format for daily recording of pressure ulcer surement along the bodys head to toe, or longitudinal,
characteristics and assign the task of assessment to a lim- axis. Width is the distance from epithelial edges across the
ited number of observers. hip to hip, or transverse, axis. Depth is measured from
the wound base to the surface of the skin at the deepest
point, measuring perpendicular to the skin surface.
Assessing Wound Location
Undermining and tracking in the wound bed are also
The location of an ulcer provides clues to the cause of the measured and recorded. Undermining is defined as an
wound and facilitates communication with other clini- extension of the wound under the surface of the skin that
cians. The location of a wound is classically described is parallel to the surface of the skin. This would be indica-
by the underlying bony prominence, e.g., ischium, tive of a combination shear and pressure injury. Track-
sacrum, etc. Pressure ulcers involving the occiput, scapu- ing or tunneling is defined as a tract heading away from
lae, lateral trochanter, fibular head, lateral malleli, and the wound base in any direction. These variables are most
heels are typically developed while lying down either effectively documented as components on a clock face
supine or, in the case of the trochanter, in a lateral decu- (e.g., 2 cm tract at 12 oclock, undermining from 25
bitus position. Conversely, pressure ulcers over the oclock position). This provides consistency and allows
ischium, coccyx, and posterior trochanter are developed for reasonably reliable location of the area by other clin-
while sitting. Pressure ulcers in persons with SCI are icians. The PUSH tool developed by the NPUAP is a
reported to occur 16 to 19 percent of the time in the wound assessment tool designed to standardize wound
sacral area, 31 to 43 percent of the time about the ischial measurements that incorporates these recommendations.
tuberosities, 16 to 26 percent of the time about the Wound tracings are an easily obtained measurement
trochanters, and 10 to 12 percent in the lower extremi- that are both inexpensive and produce minimal discom-
ties (6,27). A person with scoliosis and a pelvic obliq- fort for the patient. Tracings are not accurate in the pres-
uity is likely to develop a pressure ulcer over the ischium ence of significant wound depth. Tracings also require
and posterior trochanter, whereas a person with tight both consistent patient positioning and agreement by clin-
hamstrings and a posterior pelvic tilt will develop a pres- icians as to what is the exact edge of the wound. Some
sure ulcer over the coccyx. Knowing the relationship clinicians trace the extent of undermining on the surface
between location and etiology is critical to healing a cur- of the skin and trace or measure the entire wound area,
rent ulcer and preventing a new pressure injury. Modifi- not just the wound opening. Improvements in tracing size
cations of posture and seating systems are frequently nec- are a good indicator of wound contraction, but not vol-
essary in the presence of a sitting wound. However, a ume changes.
576 MUSCULOSKELETAL CARE
Wound photography is a commonly used method to used to define which tissue layers are affected by pressure
document wound healing. With the advent of the digital injury. However, pressure ulcers do not typically have a
camera and increased use of the Internet, wound pho- clear delineation of tissue planes, as may be found in sur-
tography is being used more widely. Telemedicine, using gical wounds or lacerations. Therefore, it is difficult to
static digital images (store-and-forward technology) or define the actual tissue planes affected. Second, staging
real-time video, has been used to monitor pressure ulcers implies that a wound progresses as it develops or wors-
in the home setting. Digital images may be entered in the ensstarting as a shallow wound and progressing to a
computer and retrieved by an off-site assessor for patients deep wound. However, in pressure injury, tissue necrosis
in remote locations. Photo images may be displayed in the is worse in the deep tissues. Deep pressure ulcers start
patients medical record to document progress toward with pressure necrosis in the deep muscle and fat and
healing. The old adage a picture is worth a thousand work their way out to the surface. Shallow ulcers, which
words has been used with efficiency to defend both care- are more likely to be caused by shear forces, begin in the
givers and institutions in this increasingly litigious culture dermis and only progress to deeper wounds if there is a
of healthcare. However, photos are two-dimensional significant component of pressure.
images that do not accurately capture wound depth, However, if there is enough pressure to disrupt
undermining, tracking, odor, or exudate. Color may also blood flow and result in ischemia of the relatively avas-
be distorted because of lighting. Photo images may, how- cular subcutaneous tissue, the deeper soft tissues (such
ever, provide powerful support to linear measurement. as muscle), which have higher metabolic requirements,
would very likely also become involved. Therefore the
wound would not worsen by progressing from a Stage 2
Assessing Wound Stage
to a Stage 3.
Staging is often considered the cornerstone of wound Rather it would progress directly to a Stage 4. Like-
assessment. When staging pressure ulcers, two key con- wise, nonblanching redness with induration (i.e., a Stage
cepts must be recognized. First, a wound cannot be accu- I pressure ulcer), indicates the presence of significant
rately staged if there is necrotic tissue in the base. Sec- deep-tissue ischemia. The body is responding to this
ondly, there is no reverse staging. Wounds do not heal by injury with edema and vasodilatation. A Stage I ulcer is
restoring normal tissue planes, but by scar formation. actually the heralding lesion for a deep pressure ulcer.
Therefore, for example, once a Stage 4, always a Stage 4 This is early evidence of deep ischemic injury and there
until it is completely healed. Table 42.2 provides a widely is a high risk of ulceration, even if all pressure is removed.
used, standard staging classification system based on the Third, studies have shown very low interrater reliability
depth of tissue involvement. Staging has a number of real between practitioners using staging systems, especially
and conceptual limitations that interfere with its useful- when a difference exists in the level of educational expo-
ness in managing pressure ulcers in SCI. First, staging is sure to the principles of wound management (6668).
The ideal classification system for pressure ulcers
should have its roots in pressure ulcer pathophysiology
especially the concept of pressure- and/or shear-induced
TABLE 42.2 ischemia. It should also provide assistance in making clin-
Pressure Ulcer Staging System ical management decisions. A revision of the classifica-
tion system based on the depth of the wound, and its heal-
Stage 1 An area of nonblanchable erythema involv- ing mode, rather than the presumed tissue planes involved
ing intact skin. This is the heralding lesion is presented in Table 42.3. Pressure ulcers under this
of pressure injury. revised system would be classified as either superficial or
Stage 2 Partial-thickness skin loss involving the deep. Superficial pressure ulcers may be further subdi-
epidermis and may travel up to, but not vided into partial-thickness (indicating the presence of
through the dermis. Often presents as an intact dermal appendages in the base) and full-thickness
abrasion or blister.
categories. Superficial partial-thickness wounds are equiv-
Stage 3 Full-thickness skin loss that involves subcu- alent to Stage 2 pressure ulcers in the previous staging sys-
taneous tissue and travels up to, but not
tem. Superficial full-thickness wounds wound are equiv-
through the fascial layers. Often a deep,
round opening with or without undermining.
alent to superficial Stage 3 pressure ulcers. Deep pressure
ulcers are equivalent to deep Stage 3 and Stage 4 ulcers.
Stage 4 Full-thickness tissue injury involving the
loss of muscle, connective tissue, joint cap-
Stage 1 pressure ulcers from the previous staging system
sule, and even bone. Presents as a deep would be included with deep pressure ulcers because they
opening with support structures either pal- herald the onset of deep-tissue ischemia. This classifica-
pable or visible in the base. tion system will also provide direction for clinical man-
agement decisions.
THE MEDICAL MANAGEMENT OF PRESSURE ULCERS 577
the second objective in its six-point directive. The tioning source of the pressure ulcer. Observing him sitting
selection and management of support surfaces is critical in the wheelchair will reveal positioning problems includ-
for tissue load distribution in the person with SCI. ing scoliosis, pelvic obliquity, or sacral sitting. It will also
Interface pressure evaluation can be helpful in iden- identify problems of inadequate back support, incorrect
tifying contributing positional and equipment factors. A footplate height, or other evidence of poor fitting or
sensor pad is placed between subject and support surface adjustment of the chair. Looking at the wheelchair cush-
to determine sitting pressures and the distribution of pres- ion itself is also essential. Some key questions to ask
sure on the sitting surface. The goal of interface pressure include the following. If it is an air cushion, is it over- or
assessment is to identify areas of high pressure and pres- under-inflated? If a gel cushion, is there evidence of too
sure distribution. This method adds important informa- little gel or is the cushion placed incorrectly in the chair
tion for tissue pressure management. However, it should so that the patient is not sitting on the gel pads? If the
not be the only method used to evaluate a cushion or seat- cushion is made of foam, how old is it?
ing system. Hands-on and hands-under evaluation of
the individuals seating surface is essential. Interface pres-
Modifiable Factors
sure measurement only measures the interaction occur-
ring at the interface of the support surface and the sub-
ject, not pressure or its effects on deeper tissues. These NUTRITION. A careful evaluation of the patients
pressures do not tell us how much pressure is too much nutritional state is essential. Pressure ulcers compete with
in the deep tissues of persons with SCI. the body for energy stores, amino acids, and micronutrients,
The term tissue interface pressure is often used usually doubling the bodys demand for the specific
interchangeably with capillary closing pressure. These nutritional intake of protein, calories, vitamin C, and zinc.
concepts, however, are not interchangeable. Capillary Most research has focused on the enhanced intake of
closing pressure is stated to be 12 to 32 millimeters of protein and calories as adjunctive to pressure ulcer healing.
mercury (mmHg) and is a measurement of the pressure It has been shown that persons with low albumin levels
needed to collapse capillaries at the skins surface. Early (3.5 gm/dl) have between a 2.8 and 8.6 greater likelihood
experiments by Landis (70) demonstrated arteriolar clos- of developing and recovering more slowly from pressure
ing pressure to be 32 millimeters of mercury (mmHg). ulcers than persons with normal serum albumin (71,72).
This number has come to be accepted as the theoretic crit- Persons with chronic pressure ulcers often show signs
ical pressure for tissue ischemia. Landis also reported a of malnutrition, and require aggressive nutritional
continuum of pressure across the microvascular bed, management. Dietary supplementation with an average of
dropping to 20 mmHg in capillary loops and 12 mmHg 2 grams of protein per kilogram of body weight per day
in venules. These pressures were determined in the finger- and between 3000 and 3500 calories per day are generally
nail bed of healthy subjects at the level of the heart. We required for proper wound healing (73). Table 42.4
do not know what the true closing pressures are deep in illustrates the nutritional requirements for SCI patients who
soft tissues where pressure ulcers occur. Therefore, the have a pressure ulcer.
goal of interface pressure evaluation and seating system Periodic chemistry studies including serum albumin,
adjustments is to create even pressure distribution across prealbumin, and transferrin, and 24-hour urine analysis
the seating surface, and not rely on a predetermined tar- for nitrogen balance are recommended in the manage-
get pressure. It is also well documented that the pressure- ment of nutrition for persons with pressure ulcers. Eval-
sensing devices themselves may affect the readings they uation of the hemoglobin, hematocrit, and lymphocyte
yield. Thus, using tissue interface pressures alone to deter- count also been found to provide strong indicators for
mine the efficacy of products is inadvisable. nutritional status and should be utilized for persons with
Often the patient knows or has an idea of what pressure ulcers.
caused the pressure ulcer. Many attribute it to a missed Trauma, infection, and chronic illness initiate a
transfer, a slip in the bathtub, a cushion failure, or a fall. physiologic stress response by the body that increases the
Sometimes the attributed cause is correct, other times energy demands and results in a catabolic state of protein
patients attribute the cause to an unlikely source, and breakdown and hypermetabolism. Circulating hormones,
other times they do not know. If the patient believes he prompted by the stress response, result in an increase in
knows how it happened, one should look carefully at that cortisol and catecholamines, but a decrease in the ana-
event, even if is seems trivial. Review of the method and bolic steroids, growth hormone, and testosterone.
frequency of weight shifts and care of the wheelchair Although catabolism can rapidly appear within weeks fol-
cushion may also be revealing. lowing injury, restoration of a steady anabolic state may
Evaluating the wheelchair and cushion of the per- take months of medical management. Many patients with
son with a sitting pressure ulcer is essential to under- pressure ulcers are in a severe catabolic state, which must
standing the cause of the ulcer and correcting the posi- be taken into account when planning treatment. The cor-
THE MEDICAL MANAGEMENT OF PRESSURE ULCERS 579
TABLE 42.5
Approaches for Debridement of Pressure Ulcers
and granulation tissue. Occasionally it is necessary to Thin film dressings are frequently used for dry eschar
postpone healing to control infection. The use of silver because they are not absorptive and the wound bed can
sulfadiazine cream has been show to control bacterial be easily visualized. Hydrocolloid dressings are frequently
load better than povidone iodine without causing dam- used if the wound has some drainage. This product is
age to fibroblasts (76). effective because it maintains a fluid environment while
Hydrotherapy is usually considered a component of absorbing excess exudate. In both cases, dressings should
mechanical debridement. The gentle movement of the be changed every 1 to 3 days and the wounds must be
water over the necrotic tissue can help to remove it with carefully evaluated for infection. Autolytic debridement
less pain or discomfort (58). In a controlled trial of is advantageous to the patient because it causes very lit-
twenty-three people with Stage 3 or 4 pressure ulcers, tle discomfort, is highly selective, and can be easily dele-
Burke et al. found that daily whirlpool significantly gated to the patient or caregiver. The disadvantages are
enhanced wound healing (77). Pulsatile lavage has been that this technique is very slow, an odor is often produced
used as an effective bedside alternative to whirlpool ther- with the liquefaction of the necrotic tissue, and the patient
apy. This powered irrigation device directs irrigation fluid or caregivers must be vigilant to identify infection.
selectively and can be used to cleanse heavily draining or Autolytic debridement is contraindicated when there is
necrotic ulcers. The disadvantage of hydrotherapy is that evidence of active infection in the wound.
it is costly and requires significant time on the part of a
specially trained person to coordinate the setup, use, and
Principle #4: Moist Wound Healing
maintenance of the equipment.
Once the pressure ulcer is clean, the clinician may pro-
Enzymatic ceed with moist wound healing. The choice for the type
of dressing will depend on ones knowledge of the prop-
Chemical agents, also known as enzymatic debridement erties of the wound care products and a good wound
agents, are used to breakdown necrotic tissue. This assessment. The depth of the wound and the amount of
method is slower than sharp or mechanical debridement, exudate will frequently determine the type and frequency
but is selective. Enzymatic agents are most effective in of dressing change. The ultimate goal is to keep the
wounds with a relatively small burden of slough or wound environment moist to allow contraction, granu-
necrotic tissue. They are virtually ineffective against hard, lation, and epithelialization with the least amount of
dry eschar. However, if the eschar is cross-hatched with dressing and personnel time. Table 42.6 summarizes com-
a large-bore needle or blade prior to their application, monly used dressings for wounds, based on depth and
enzymatic agents can be used with success. Enzymatic amount of exudate.
debridement should be avoided in large wounds because Many products have been created around these
it is cost prohibitive. basic principles. A list of the most common types in their
generic form may be found in Table 42.7.
Autolytic
Autolytic debridement involves the use of occlusive dress-
ADJUNCTIVE TREATMENT FOR
ings over a wound to allow the bodys own enzymes to
PRESSURE ULCERS
break down necrotic tissue. The viable tissue is separated
from the nonviable by the bodys own enzymes and white
Electrical Stimulation
blood cells and the liquefied necrotic tissue is cleaned
away with each dressing change. Selecting the best type In one study of seventeen persons with SCI, electrical
of occlusive dressing depends on the wound assessment. stimulation (ES) using high-voltage pulsed current
THE MEDICAL MANAGEMENT OF PRESSURE ULCERS 581
TABLE 42.6
Approaches for Moist Wound Healing Based on Depth and Degree of Exudate
MODERATE/
MINIMAL DRAINING HEAVY DRAINING MINIMAL DRAINING MODERATE/HEAVY DRAINING
TABLE 42.7
Wound Care Products
WOUND CARE
PRODUCTS PROPERTIES USES
Gauze Woven fiber which absorbs excess exudate, Used with saline as wet-to-moist dressing for
Regular maintain moist wound environment, fills large ulcers with heavy drainage that requires
tracts and undermining. changing three times a day.
Sodium Impregnated Absorbs excess exudate, maintains moist Can be applied in ulcers with tracts and
wound environment, fills sinus tracts undermining
and undermining. Requires more nursing time due to three times
Draws moisture into the wound bed by a day dressing change
osmotic action. Used for moderate- to heavy-draining ulcers
Finer weave of gauze minimizes sticking of that can be changed one or twice a day.
new tissue upon removal. Ribbon type dressing which can be applied in
More absorptive capacity than regular gauze. tracts or undermining.
Less frequency of dressing change Can be used in clean and infected ulcers.
(cost effective).
Contraindications: very dry or profusely
draining ulcers.
Transparent Membrane No absorptive capacity. For superficial clean ulcers with no drainage.
Semipermeable polyurethane membrane Used to protect healed injuries which are
that allows gas exchange but not liquids. superficial and not draining.
Prevents bacterial entry due to pore size. Abrasions/skin tear.
Can visualize ulcers.
Promotes epithelial migration.
Protects skin from shearing and friction
(secondary skin).
Contraindications: torn skin, friable skin;
exudating wounds.
Hydrocolloid An occlusive dressing; does not allow For shallow, superficial ulcers.
oxygen exchange. Facilitates autolytic debridement of eschars.
Protects, absorbs excess drainage.
Forms gel with wound exudate and create
a moist environment.
Can be changed minimum every 23 days.
Has the tendency to peel off epidermis if
changed frequently, so may consider
changing to another type of dressing
with more absorptive capacity.
continued on next page
582 MUSCULOSKELETAL CARE
TABLE 42.7
Wound Care Products (continued)
WOUND CARE
PRODUCTS PROPERTIES USES
(HVPC) was shown to increase the rate of healing of cific pressure ulcers studied eighty persons with pressure
pressure ulcers compared to placebo at 5, 15, and 20 sores divided between three different ES waveforms and
days (78). Stage 2 wounds healed completely in both the a control group (80). One hundred fifty four wounds
ES and placebo groups. All Stage 3 and 4 ulcers (n=6) met all the selection criteria and were evaluated for
in the ES group showed consistent reduction in size effect of waveform on healing. Electrically balanced
compared to baseline, whereas only three of seven Stage asymmetric biphasic waveforms showed the highest rate
3 and 4 ulcers in the placebo group showed consistent of complete healing during the study. The mechanism of
reduction in size. In another study of 150 persons with enhanced wound healing using ES is not fully under-
SCI and pressure ulcers, low-frequency pulsed currents stood. Baker et al. (80) states that the most likely expla-
(LFPC) (i.e., alternating current) were compared to nation is neural changes caused by the ES, which
direct current and control groups (79). Researchers increases blood flow to the tissue.
found that healing is faster when pressure ulcers are Although electrical stimulation has been shown to
treated with ES compared to controls. Their data also be effective in increasing the healing rate of chronic pres-
suggested that LFPC was superior to direct current in sure ulcers, ES has not had wide usage throughout the
affecting healing rates. They found that pressure ulcers United States. The reasons for this are many; however,
treated with LFPC healed twice as fast as those treated this form of treatment should not be overlooked for per-
with standard care (control). A third survey of SCI-spe- sons with SCI and difficult-to-heal pressure ulcers.
THE MEDICAL MANAGEMENT OF PRESSURE ULCERS 583
TABLE 42.8
Thermal and Nonthermal Effects of Therapeutic Ultrasound
THERMAL NONTHERMAL
in Table 42.8. Although there are no studies of the bene- extensive degloving injury of the torso was managed in
fits of ultrasound in pressure ulcers in SCI, the theoreti- his hospital room with conscious sedation for all of his
cal benefits suggest that it may in time prove to be an dressing changes and was able to control his pain with a
effective adjunctive treatment for pressure ulcers in SCI. self-administered medication pump when the dressing
was in place. Prior to the use of the VAC in this patient,
he had to be placed under anesthesia for each dressing
Vacuum-Assisted Closure (VAC)
change (99).
VAC has become widely used in the last 10 years to con- VAC therapy is often used as an adjunctive therapy
servatively manage recalcitrant wounds. This product for conventional surgical intervention. This product has
involves the use of a foam sponge that is placed into the been used to prepare a wound for placement of a split-
wound, connected to a drainage tube, and attached to a thickness skin graft, decrease the size of a defect that will
pump that pulls negative pressure on the wound bed. The undergo traditional myocutaneous flap surgery or
foam may be open or closed cell and is selected based on enhance flap viability by evacuating excess edema after
the type of wound and its characteristics. Tubing is flex- surgery. Flap surgery in SCI patients has a variable suc-
ible but cannot be collapsed. The pump is capable of sev- cess rate. Recurrence has been tied strongly to substance
eral settings and can be run continuously or intermittently abuse and was found to be as high as 79 percent in peo-
based on the characteristics of the wound. Initially a bed- ple who sustained a traumatic cord injury (100). Further
side device that limited patient mobility, it has been devel- information needs to be collected to directly tie the use
oped to operate on a battery pack if the full-size unit is of VAC therapy to the positive long-term success rate of
necessary. For wounds with less drainage, the mini-VAC flap surgeries.
is now being used and is so small that it fits handily in a However, disadvantages to the use of the VAC exist.
small carrying case the size of a normal fanny pack. Sub- Rarely, wounds were found that did not react favorably
atmospheric pressures are achieved by the application of when treated with the VAC. In one study (96), wounds
a thin film dressing once the sponge has been carefully cut that did not respond were chronic in nature and often had
and trimmed to fill the wound bed and any tunneling or underlying pathology, most commonly osteomyelitis. The
undermining. Sealing this device to provide an occlusive VAC cannot be used in wounds with necrotic tissue or
dressing is critical to the function of the machine. cancer in the margins. For obvious reasons, the VAC is
Continuous negative pressure is used initially to also contraindicated in wounds that involve fistulae and
remove any excess edema in the periwound skin and has should be used with caution in abdominal wounds that
been shown to decrease bacterial load in contaminated require grafts to close the abdominal wall. Cautious use
wounds (96). The removal of excess fluid in the wound is also recommended in anticoagulated patients and
bed promotes moist wound healing and evacuates the wounds with initially difficult hemostasis or vessel grafts.
chronic wound fluid that is thought to retard granulation Because it decreases the frequency of dressing
tissue formation. Negative pressure further assists the changes and limits a persons exposure to these painful
body by encouraging angiogenesis. These new blood ves- procedures, its initial cost should not be daunting.
sels will carry oxygen and nutrients to the wound bed Although the need for trained clinical staff to place and
(97). monitor it may limit its placement as a first-line option,
In one of the original studies on porcine subjects, the VAC must be considered if a wound fails to respond
small full-thickness wounds were found to granulate 62 to traditional conservative management.
percent faster than the wet-to-dry control group (98).
Later studies have confirmed the positive effects on many
types of wounds in settings ranging from the home to A BRIEF PRIMER ON SUPPORT SURFACES
acute care. The VAC may be used on a wide variety of
wounds including Stage 3 and 4 pressure ulcers, surgical Passive protection in the form of support surfaces and
dehiscence, traumatic wounds such as avulsion, ampu- intensive nursing care is essential early after acute SCI. As
tations, or degloving injury, flaps and grafts, vascular the person is medically stabilized and begins rehabilita-
ulcers, and diabetic ulcers. tion, he will be required to take a more-active role in pres-
There are several advantages of the VAC in relation sure ulcer prevention. Passive protection through the
to patient tolerance and nursing time. The frequency of appropriate prescription of wheelchair cushions and bed
the dressing change is most often three times each week, overlays is needed throughout a persons life with SCI.
thereby decreasing nursing time. Although nursing duties Advances in technology have produced a variety of sta-
are increased on VAC change days, nursing activity drops tic and dynamic support surfaces available for this patient
overall when compared to wet-to-dry dressings changed population.
three times each day. Patients have less pain during their Lying support surfaces are usually grouped more
dressing changes. In one case study, a person with an specifically into overlays, mattress replacements, and full-
THE MEDICAL MANAGEMENT OF PRESSURE ULCERS 585
framed products. Overlays are products that lie on top pressures during inflation of the cylinders, but that is fol-
of the standard hospital mattress. They are used in addi- lowed by a period of very low to no pressure when the
tion to the standard mattress and bed frame. Mattress cylinders are deflated. Different overlays are run on differ-
overlays can be grouped specifically into several addi- ent time schedules for inflation and deflation and each
tional categories that include foam, air, gel, and water. product has different inflation pressures. Alternating air
Mattress replacements are placed on the hospital bed overlays are designed to prevent constant, unrelieved pres-
frame and are used instead of the standard hospital mat- sure against the skin. There is some suggestion that these
tress. Full-framed products are also called specialty beds. products also enhance blood flow and decrease the collec-
Specialty beds replace the whole hospital bed unit, includ- tion of metabolites in dependent areas by promoting flow
ing the mattress and its frame. in the low-pressure venous and lymphatic systems (102).
Although air overlays are an excellent link in the
chain of pressure ulcer prevention and treatment, they
Mattress Overlays
require more vigilance on the part of the clinician or care-
Foam is widely used for inexpensive, low-maintenance giver than foam. Whether static or dynamic, air overlays
pressure reduction. To choose the most effective foam for are susceptible to two enemies: bottoming out and shear.
pressure reduction, the clinician must understand the con- A product is said to bottom out if the clinician places a
cepts of contours, base height, density, and indentation hand between the standard mattress and the overlay and
load deflection. Contours are simply the description of is able to feel the patients bony prominence with upward
the surface of the foam pad. Examples of this are egg motion of the fingertips. An inch of air between the patient
crate, slashed, and smooth. Currently there is no data to and the clinicians hand is normally expected. When a
suggest that any type of contour is superior. product bottoms out, the air is distributed toward the ends
The height of the foam from its base to where the con- of the overlay, and the persons body is not supported.
volutions begin is referred to as base height. Foam over- Shear injury occurs when the head of the bed is ele-
lays with a base height of 2 inches did not provide signifi- vated and the torso slides toward the foot of the bed. This
cant pressure reduction when compared to a standard force is transmitted to the deeper portion of the superfi-
hospital mattress unless the patient was restricted to prone cial fascia, which is relatively mobile. The movement of
or supine position (101). Significant pressure reduction was the fascia puts the blood vessels in this area under stretch
seen, however, in convoluted foam with a 4-inch base under and possibly even occlusion, because they are essentially
both the scapula and sacrococcygeal area although mean anchored in place. This force produces extensive tissue
readings under the trochanter were not reduced (75). The damage and eventual tissue dissection along the fascial
advantages of foam are that it is easy to use, has no main- plane; this is demonstrated as undermining (103). The
tenance, is generally billed as a one-time fee for the patient, head of the bed should optimally be kept below 30
is lightweight, comes in many sizes, and is not affected by degrees to decrease the likelihood of shear injury in immo-
punctures. The disadvantages are that a plastic sheet must bile persons, such as those with acute SCI. Air overlays
be used to protect the foam from incontinent episodes, are contraindicated in people over 250 pounds, those with
which may increase heat and moisture; washing decreases unstable spines, or those who require traction.
the flame-retardant coating; and foam has a limited life. Gel support surfaces provide antishear surfaces. Gel
Air overlays may be divided into either air filled or overlays are generally made up of gel flotation pads com-
low air loss. Air-filled overlays are composed of inter- posed of silicone or Silastic (a silicone elastomer). This
connected cells that are inflated with a pump to a pres- fluid emulsion tends to allow excellent immersion of the
sure determined by the manufacturer in conjunction with patient, produces pressure reduction, and comes in many
the size of the patient they must support. Barring any styles. Advantages include a lower risk of bottoming out,
punctures, after inflation the air pressure may be main- minimal maintenance, ease of cleaning and, in the over-
tained easily by the patient or caregiver. The air may move lay form, inexpensive daily rental cost. Gel products are,
passively from one chamber to another or within the same however, very heavy and have no mechanism to control
chamber to accommodate changes in patient position. moisture.
Low-air-loss overlays are the second type of static air Water beds or water-filled overlays are known to be
product and are composed of air-filled cells whose pres- useful in the prevention of pressure ulcers and have lower
sure is controlled by a continuous flow of air produced tissue interface pressures than standard hospital mat-
by an electric pump. These products provide slight air tresses (104,105). These products disperse the pressure of
movement against the skin. the body over the entire support surface and are often
Alternating air overlays consist of a number of air- used with great success in the home. Both overlays and
filled cells or cylinders that are inflated and deflated at reg- beds have dangers in the acute or long-term care setting.
ular intervals by a compact pump attached to the bed. Tis- The maintenance of water temperature, weight of the
sue interface pressures are higher than capillary closing filled product, leak prevention, and exact fill levels all
586 MUSCULOSKELETAL CARE
make water-filled products a challenge for caregivers. In too unstable to be turned. The bed consists of a hospital
one study comparing multiple products, one canine test bed frame that holds air-filled pillows. These pillows may
subject actually drowned while sedated when he bit a hole be interconnected or be powered by the same pump but
in the cover of his water bed (106). have separate air-inflow tubes. This is superior to an air
overlay because these products offer a depth of immer-
sion that overlays cannot and there is no chance for the
Mattress Replacements
patient to bottom out if the product is working properly.
Mattress replacements are used in place of standard hos- For this additional therapy, there is additional cost.
pital mattresses. These products are thought to reduce tis-
sue interface pressures when compared to standard hos-
Wheelchair Cushions
pital mattresses. Replacements vary in design. Products
may be all foam separated into various components, a Static cushions may be foam, gel filled, air filled, or made
combination of gel and foam, or of foam and air. A firm, of other synthetic materials that reduce interface pressures
high-density foam periphery with segments of varying and redistribute body weight. Air-filled cushions were
density at the head, seat, and foot is a common design. precursors to the dynamic cushion in providing support
that is effective in preventing bottoming out under con-
ditions of uneven loading as occurs in areas supporting
Full-framed (Specialty) Beds
bony anatomy. Their effectiveness in pressure ulcer pre-
Full-framed products or specialty beds are the high end vention has been shown to depend on their internal infla-
of the specialty-surface spectrum. All require some form tion pressure. Thus, pressure-mapping systems designed
of electric pump and are used as treatment products. The to quantify interface pressures in terms of the ratio of sur-
three basic groups are high air loss, low air loss, and face pressure against inflation pressure have been suc-
kinetic therapy. Whereas kinetic therapy beds were orig- cessfully used to customize air-filled seating surfaces and
inally created as a skin treatment product,they are now predict their benefit in preventing pressure ulcers. In a
used in the acute care setting for the treatment of pul- study by Krouskop et. al, (101), three types of air-filled
monary conditions and spinal fractures. High and low air- cushions were evaluated using pressure mapping to test
loss beds have been designed to provide pressure relief for differences in performance. Results from their study indi-
patients who may not be turned or who have Stage 3 or cated that overinflated air-filled cushions are more effec-
4 pressure ulcers on more than one turning surface. tive, although the interface pressure is greater than with
underinflating. An interface pressure of 35 mmHg was
determine effective for preventing pressure ulcers, but the
High Air-loss Beds
impact of variations in interface pressure was also greatly
High air-loss beds are also called air-fluidized beds. This affected by the shape and pliability of the material used
refers to the use of a high air flow through the silicon to construct the cushions.
beads, which makes the beads collectively perform like The relationship between body weight, build, and
a liquid. This product, which was originally designed to degree of interface pressure during wheelchair use has
treat people with burns, is composed of a metal frame also been identified as critical in the evaluation of wheel-
that looks like a bathtub and silicon beads contained in chair cushions. It has been demonstrated that the SCI
a loose-fitting sheet. When the electric pump is turned off, population is composed of many more thin persons than
the bed rapidly becomes firm enough to easily reposition the normal population. Pressure distribution on a seat-
the patient or even perform effective cardiopulmonary ing surface differs by body build; thus, thin individuals,
resuscitation. When the pump is engaged, the patients with more prominent bony tuberosities, experience
lower body is immersed in the bed, giving the illusion of greater interface pressures than heavier individuals over
floating because of the movement of the beads under the ischial processes and trochanters but less over remaining
sheet. Alkalinity (pH 10), warm temperature (30 to surfaces. Weight and length of lower extremities can also
32C), and entrapment of microorganisms within the affect total or average interface pressures for subjects
beads all contribute to the reported bactericidal effect of using wheelchair cushions (108).
this bed (107). The air-fluidized bed is most commonly Kernozek and Lewin (109) conducted a study to
used during the weeks following myocutaneous flap compare interface pressures while SCI subjects were sta-
surgery or in patients with multiple severe pressure ulcers. tically seated on a Jay cushion versus interface pressures
on the same cushion during locomotion of the wheelchair.
The objective of the study was to determine if peak pres-
Low Air-loss Beds
sure and pressuretime intervals were sufficiently
Low air-loss beds are used to treat patients who have pres- dynamic during a subjects usual routine of daily activi-
sure ulcers on more than one turning surface or who are ties. Peak pressures varied 40 percent from the peak to
THE MEDICAL MANAGEMENT OF PRESSURE ULCERS 587
the minimum throughout the time of wheelchair loco- to early healing of pressure ulcers, will lend great success
motion, and remained 35 to 42 percent greater than peak to clinicians to promote beneficial outcomes during each
pressures during static seating. Because pressuretime persons time of healing.
intervals were found to be comparable between the two
seating conditions, the cumulative effect of the peak pres-
sures were also expressed as equitable for the two condi- References
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43 The Surgical Management
of Pressure Ulcers
And a certain poor man named Lazarus was cov- waiting to be conquered by some modern scientific break-
ered with sores even the dogs were coming and lick- through. Until a half-century ago, few surgeons were
ing his sores. Luke 16:20-21. involved in pressure ulcer treatment, because these
wounds were considered indicators of a terminal illness.
he care of the patient afflicted with
T
Even today, the general perception is that they occur in
spinal cord dysfunction, whether the infirm and aged or in people bedridden with incur-
caused by trauma or disease, is mul- able diseaseshence the persistence of the term bed
tifaceted and requires an interdisci- sores. As a result, the care of these wounds in the past
plinary team approach. The surgeon is a necessary mem- was often neglected and attention was diverted to the
ber of the team and must be knowledgeable about the other seemingly more pressing medical problems that are
broad spectrum of spinal cord medicine. Although pri- common in these patients.
marily concerned with the surgical management of the pres- For the individual afflicted with a debilitating illness,
sure ulcer, the reconstructive surgeon must be familiar with the infirmities of the aging process, or the sequelae of neu-
the frequent concomitant lifetime medical and socioeco- rologic dysfunction from any cause, the specter of failed
nomic problems encountered in this special patient popu- skin integrity is forever present. Although pain may not
lation. In the same manner, other members of this team be a factor in many of these wounds, foul odor, the rit-
should be familiar with the special surgical aspects of the ual of numerous dressing changes, and the need for fre-
patients care. With this in view, the focus of this chapter is quent medical attention at a clinic or office or even hos-
not so much on the technological aspects of flap surgery pitalization can be a heavy burden for those afflicted with
but rather on the basic concepts of the role of surgery in the these wounds.
repair of pressure ulcers and the contributions of the sur- For the patient undergoing a rehabilitation program,
geon as a team member in the surgical management of these the development of a pressure ulcer and subsequent oblig-
wounds in this challenging group of patients. atory bed rest can severely curtail and prolong the reha-
bilitation process. Prolonged bed rest in the newly injured
THE PRESSURE ULCER DILEMMA patient contributes to the atrophy of muscle and bone
(1,2), urinary tract infections and stone formation (3),
In general, pressure ulcers and the treatment thereof is not venous thrombosis, and mental depression as these
thought of as one of the exciting frontiers of medicine patients struggle to cope with their devastating injury.
591
592 MUSCULOSKELETAL CARE
Not only are these wounds disruptive of an indi- surgical tome written in the late 1930s, granted only one
viduals daily activities, their treatment is costly, having page to discuss the surgical treatment of these wounds (8).
an estimated range of between $15,000 to $40,000 to The reason for the surgeons neglect of pressure
treat a single pressure ulcer (4). The total national cost ulcers prior to World War II is that patients with bed
of the medical treatment of these wounds exceeds $1,335 sores had a short life expectancy. Any significant trauma
billion a year (5). Based on the premise that an ounce to the spinal cord was considered a mortal injury, and the
of prevention is worth a pound of cure, approximately development of bed sores was an ominous sign. Of the
$500 million is spent each year on the seemingly unlim- American combat troops in World War I who sustained
ited number of preventative pressure-relief products that injuries to the central nervous system, only 20 percent sur-
are available on the market. An estimate of the cost of the vived long enough to be brought home. Of these sur-
pound of cure when prevention fails is in the range of vivors, half were dead within a year, usually from infec-
an additional $200 million just for drug therapy alone (4). tion and sepsis originating in the genitourinary tract or in
Hirshberg et al. reported that the cost of hospitalization, their numerous necrotic pressure ulcers (9).
durable medical equipment, home nursing care, physician Because this mortality rate was considered the nor-
management, and transportation supports a $20 billion- mal outcome of a central nervous system injury from time
a-year industry (6). immemorial, there was little interest in the untreatable
In addition, there are numerous nonmedical fac- ulcer that was considered an integral part of the neuro-
tors, both social and economic, that have compounded logical syndrome (10). It was taught that any surgical
these obvious medical treatment costs at a significantly approach was doomed to failure because of infection and
higher annual multibillion dollar amount. It is clear that the pathophysiology of SCI. In 1940, Donald Monro, the
the theoretically preventable pressure ulcer is a major father of modern SCI treatment in the United States,
player in the competition for societys available health- taught that even the simple lancing of an abscessed wound
care resources and that prevention must be a major goal was contraindicated in SCI patients. Such abscesses
in the care of the at-risk patient population. should be treated by needle aspiration to avoid spread-
ing the infection by cutting tissue and causing fatal blood
poisoning (11).
FACTORS IN COST ESCALATION
months, with a range between 1 and 72 months. Over- and that initiates an incredibly complex wound healing
all, twenty of the twenty-two paraplegic patients (91 per- process. This wound healing process is a dynamic inter-
cent) had developed an ulcer at either the surgical site or active process involving hematogenous and tissue cells
at a new site within an average time of 19.2 months. In along with humoral components of the activated clotting
comparison to these patients, there were no recurrences and complement pathways. The inflammatory process
following surgical closure in four of the nonparaplegic releases numerous cytokines and other growth factors,
patients, either at the surgical site or at a remote site. The eventually leading to the production of extracellular
remaining nonparalyzed patients did not undergo surgery matrix components, proteases, and various cytokine
because of their unstable medical or geriatric condition. growth factors and inhibitors that interact to result in a
Experiences such as those of the Evans group and healed wound. Wound-healing research into this process
others have reinforced the opinion of some physicians has led to the development of recombinant exogenous
that, because of the failure of operative intervention to growth factors that have been applied to wounds in order
cure the disease, conservative (nonsurgical) methods of to promote wound healing (17,18). A recent study sug-
wound care are the treatments of choice for pressure gests that the preoperative application of these recombi-
ulcers in SCI patients. It is granted that not all patients nant growth factors may contribute to the successful sur-
should be considered candidates for the surgical closure gical closure of chronic pressure ulcers (19).
of their wounds. The elderly, the infirm, or those suffer- This classic wound-healing process has three over-
ing from terminal disease should be humanely cared for lapping phases, distinguished by the prevailing dominant
by nonsurgical means in a hospice or nursing home set- biologic process present during a particular sequential
ting. However, with the appreciation of potential com- time frame. They are the inflammation phase, the tissue-
plications such as sepsis, osteomyelitis, or even malignant formation phase, and the tissue-remodeling phase.
transformation (14,15) in the chronic pressure ulcer, as Numerous reviews are available detailing this incredible
well as the cost of care and the effects on the quality of and fascinating process (2022) for those interested; these
life associated with prolonged bed rest, conservative will not be discussed here.
wound care in the rehabilitative patient is not the answer The different gradations of wounds depend on the
either. The pre-WW II philosophy of institutional care for extent of the injury. A wound may be relatively trivial,
these patients is not appropriate today. such as a contusion or an abrasion. Here, the wound heal-
The problem remains that when pressure ulcers in ing inflammatory cascade normally leads to the restora-
the SCI patient are successfully healed by either the con- tion of tissue integrity within 1 or 2 weeks by the regen-
servative secondary intention route or by invasive eration of cloned epithelial and/or endothelial cells, so
surgery, there is a significant recurrence rate of from 5 to that the healed wound is fully restored to its functional
91 percent (16). The culprit is not the ulcer and its par- and aesthetic preinjury state. The remodeling phase is
ticular mode of treatment per se but the underlying patho- minimal to nonexistent, because there is usually no scar
logic condition combined with psychosocial issues that tissue formation.
perpetuate this disease or syndrome. In a laceration, this same wound healing process of
It is obvious that neither surgery nor nonsurgical inflammation and cellular proliferation leads to the
treatment of pressure ulcers in SCI patients is curative. restoration of tissue integrity in a week or two (healing
The selection of the surgical procedure or the level of the by primary intention). In a cutaneous laceration extend-
surgical skill employed has not been the issue in recur- ing completely through the dermis, there is a combination
rence in most studies (12,16). It is the ounce of preven- of the processes of epithelial regeneration and scar tissue
tion and not the pound of cure that must be addressed formation that results in the formation of a new type of
to improve the quality of life in these individuals. The edu- tissue called scar-epitheliuman inferior although satis-
cation of the SCI patient and caregiver, appropriate reha- factory substitute for the destroyed dermis that, unlike
bilitation, social and economic issues, and psychologic epithelium, does not regenerate. The final remodeling or
factors such as depression and patient compliance are all maturation phase of this scar tissue may take 6 months
elements in the pressure ulcer equationcomponents as or longer.
equally important as the SCI pathologic syndrome itself. In a wound with loss of tissue, as in an ulcer, the
A lifelong holistic treatment plan must be pursued. healing process is more challenged than in the primary
intention healing of a laceration. With time and a pro-
longed inflammatory process the tissue deficit, if limited,
THE PRESSURE ULCER AS A WOUND eventually heals by a) contraction of scar tissue and b)
epithelialization. This process is known as healing by sec-
A wound is an injury that disrupts the integrity of tissue ondary intention. Although restoring the integrity of the
and normally results in an acute inflammatory response protective mechanical barrier of skin, such healed wounds
(cause and effect) that is intended to limit further injury are devoid of skins normal physiologic functions. Scar
594 MUSCULOSKELETAL CARE
epithelium lacks nerve endings, pilosebaceous and sweat burns as a measure of wound depth. In the case of the
glands, and hair follicles, and is relatively ischemic com- pressure ulcer, where the vertical dimension of depth is
pared to the adjacent normal skin. the major prognosticator of tissue repair, the term Stage
The seriousness of the injury and the ability of the is used and is analogous to the old burn wound termi-
wound to heal itself normally correlate with the degree of nology of Degree. As in burns, this clinical staging of pres-
tissue injured. With significant tissue destruction, the sure ulcers is useful in assessing the extent of the injury
capability of the organism to heal and restore tissue con- and is used in the development of a surgical or a nonsur-
tinuity in a reasonable time frame is severely strained, if gical treatment plan (see Table 43.1). The present con-
not arrested (wound-healing exhaustion). Wounds that sensus grading classification is derived from the original
fail to heal become chronic and debilitating to the patient numeric system proposed by Shae in 1975 (28).
because of the prolonged and enhanced inflammatory In the Stage 1 injury, similar to a first-degree burn
process. The systemic catabolic effects of the extended such as sunburn, the erythema of the invoked localized
period of inflammation may be seen in the malnutrition inflammatory response is the harbinger that tissue injury
and anemia that one frequently sees in patients with has occurred (Figure 43.1A). The observable cutaneous
chronic pressure sores. vasodilatation is in response to the release of chemotac-
It is now recognized that there is more to the devel- tic cytokines and vasoactive mediators by the injured or
opment of wound chronicity than the failure of a wound activated inflammatory cells. Because there is no tissue
to heal within a certain time frame. Studies of the mole- loss other than superficial desquamation, this inflamma-
cular environment of acute and chronic wounds indicate tory phase resolves without calling forth the classic fibro-
a difference in the microenvironment of acute and chronic plasia of the proliferative phase of wound repair. Its per-
wounds. Tarnuzzer and Schultz demonstrated that fluid sistence distinguishes it from the short-lived reactive
specimens collected from chronic wounds were not mito- hyperemia of reperfusion that follows a transitory dis-
genic and blocked the DNA synthesis normally stimulated ruption of capillary flow that may be occasioned by a
by acute wound fluid (23). brief period of external pressure. A Stage 1 pressure injury
It has been noted by others that fluid from chronic must be heeded as a warning sign that death of tissue is
wounds inhibits the proliferation of keratinocytes, fibro- about to take place. Treatment must be instituted in the
blasts, and vascular endothelial cells, elements necessary form of immediate and complete pressure relief until the
for the healing of wounds (24,25). Bucalo et al. also affected tissue recovers.
observed that cell adhesion was inhibited by chronic The presence of a blister, an abrasion, or the bluish
wound fluid (26). It would appear that the reduced level discoloration of a contusion portends a more significant
of growth factors in chronic wounds, along with the pres- injury than in a Stage 1 lesions. This Stage 2 injury (Fig-
ence of cytokine inhibitors and proteases normally
involved in the remodeling phase of wound healing, inhibit
the reparative processes initiated in an acute wound.
TABLE 43.1
Stages of Severity of Pressure Ulcers
CLASSIFICATION OF PRESSURE
SORE SEVERITY Stage 1 Nonblanchable erythema of intact skin.
Stage 2 Partial-thickness skin loss with preservation
A system of classifying the degree of severity of the wound of deep dermis and adnexal skin glands.
is helpful in planning the type of treatment needed to heal May involve entire dermis but not subcuta-
these wounds. In the case of an acute thermal injury, the neous fat (abrasion, blister, contusion, shal-
tissue destruction is usually relatively superficial but can low ulcers).
be serious when it involves a large portion of the body Stage 3 Full-thickness skin loss, with damaged or
surface area, thus evoking not just a localized inflamma- destroyed subcutaneous fat down to deep
tory response, but a severe systemic inflammatory reac- fascia. Tendency to undermining and sinus
tion (27). Such an enhanced systemic response is injuri- formation.
ous to the entire organism and can be brought under Stage 4 Full-thickness loss to muscle, bone, joint,
control only by closing the wound with skin grafts. and the supporting structures (ligaments,
Like the major and minor burn wounds, the seri- tendons, joint capsule). Undermining,
bursa, and sinus tracks not uncommon.
ousness of a pressure ulcer is determined by the extent of
the tissue destruction and the magnitude of the inflam-
Adapted from: Treatment of Pressure Ulcers. In Clinical Prac-
matory response. In thermal injury, the terms partial- tice Guidelines, Number 15. Rockville Md.: U.S. Department
thickness burn and full-thickness burn have replaced Health and Human Services, 1994.
the old terminology of first-, second-, and third-degree
THE SURGICAL MANAGEMENT OF PRESSURE ULCERS 595
FIGURE 43.1B
FIGURE 43.1C
Stage 2 pressure ulcer is a wound with a partial loss of skin
that may only involve the epidermis (abrasion, blister) but can Stage 3 pressure ulcer with full-thickness loss of skin. The
include a limited loss of the dermis preserving the deep der- subcutaneous fat may suffer a partial or full-thickness loss
mal elements without the involvement of subcutaneous fat. extending to the deep fascia overlying an intact muscle layer.
596 MUSCULOSKELETAL CARE
Stage 3 pressure ulcer with lateral destruction of the relatively A presumed Stage 3 ulcer with undermining or sinus forma-
ischemic subcutaneous fat with undermining of the more vas- tion may actually be a Stage 4 A ulcer because of unsuspected
cularized skin. involvement of muscle tissue remote from the cutaneous
wound.
shallow, Stage 3 ulcers may heal in a reasonable time tion. A more-extensive Stage 4 pressure ulcer may extend
frame by secondary intention through the process of con- to bone and joint, thus resulting in periostiitis,
traction and epithelialization. osteomyelitis, or even pyoarthritis. This type of wound
Like dermis, muscle tissue is more vascular than sub- involving bone or joint is classified as a Stage 4B ulcer
cutaneous fat and more resistant to pressure-induced by Eltorai (29) because of the more serious nature of the
ischemia. However, if the injury is severe enough, it too injury and its more complex treatment. Such an ulcer is
will necrose and result in a Stage 4 wound (Figures 43.1E obviously a more serious wound and not normally
and 43.1F). Undermining and sinus tract formation is amenable to treatment without surgical intervention (Fig-
common and difficult to heal without surgical interven- ure 43.1G).
Stage 4 A pressure ulcer extending into or completely Stage 4 B ulcer with involvement of muscle with extension
through the underlying muscle layer. A reactive periostitis through the joint capsule into the joint space. In some sys-
may be present but cortical bone is not eroded in a Stage 4 tems of pressue ulcer classification, involvement of muscle
A ulcer. and joint is considered a single Stage 4 wound.
THE SURGICAL MANAGEMENT OF PRESSURE ULCERS 597
COMPLICATIONS OF PRESSURE and is in fact contraindicated, because the end result will
SORE NEGLECT be the loss of control of the bacterial invaders and the pro-
liferation of resistant organisms.
The early and late complications of pressure ulcers must An acute infectious process, accompanied by chills,
be addressed. The goal of all treatment (surgical and non- fever, and an elevated white blood cell count indicates the
surgical) is healing of the wound, and this requires the bacteremia of systemic sepsis. Empirical systemic intra-
prevention of complications that disrupt the normal venous antibiotic therapy based on clinical judgment must
wound healing process and that can lead to a chronic non- be initiated and adjusted as soon as sensitivity studies are
healing ulcer. The prevention of complications in the available. In addition to antibiotics, debridement of the
acutely injured tissue begins with the recognition of the devitalized tissue and drainage of all abscess pockets must
injury and the removal of the causative agent; treatment be carried out. Appropriate studies should be undertaken
begins with pressure relief. Without pressure relief and if osteomyelitis is suspected.
the control of infection, a superficial Stage 1 or 2 ulcer
will progress into the deeper tissues and become a more
Sepsis and Septic Shock
serious Stage 3 or Stage 4 ulcer.
In the acute pressure sore setting, the bacterial invasion
(infection) of body tissues will initiate a local inflamma-
Bedrest
tory response that seeks to eradicate the undesired
Pressure relief in the wheelchair ambulant patient who invaders. It is frequently accompanied by fever and ele-
develops a pressure ulcer usually entails bedrest that has vation of the white blood cell count. If uncontrolled, the
its own subset of complications. Attempting to avoid fur- local infection leads to further tissue destruction, con-
ther tissue damage and seeking to heal the ulcer requires verting a Stage 2 ulcer to a Stage 3 or 4 ulcer. Should bac-
the removal of the main causative agent of the wound teremia develop, there is the potential for generalized sep-
unremitting pressure. The immobility and reduced phys- sis having a marked systemic inflammatory response that
ical activity accompanying enforced bedrest, however, may adversely produce dysfunction in one or more organ
increase the loss of bone and muscle mass (1,2) and systems. Furthermore, this ensuing systemic inflamma-
encourage the development of joint contractures, par- tory response syndrome (SIRS) can precipitate a poten-
ticularly in spastic SCI patients. Loss of upper body tially lethal septic shock phase, with subsequent multi-
strength in paraplegics confined to bed will require sub- organ failure (27,30).
sequent physical therapy focused on the recovery of mus-
cle strength before they can safely do transfers and oper-
Periostitis/Osteomyelitis
ate their manual wheelchairs. Bedrest also places the
patient at risk for developing additional pressure ulcers. The Stage 4 ulcer may involve bone by direct extension
Consequently, avoidance of this cascade of complica- of the infected pressure ulcer and, if inadequately treated,
tions by healing the wound and the returning the patient will doom any wound healing treatment plan. The inflam-
to the ambulant state should be accomplished as early as matory involvement of the periosteum (periostitis) is fre-
feasible. quently involved in Stage 4 ulcers that extend to bone sur-
faces without involving the bone itself and should not be
confused with osteomyelitis. The clinician must know if
Infection
there is indeed a focus of osteomyelitis underlying the sur-
The invasion of bacteria following the loss of the integrity face periostitis, because one reason for an early recurrence
of the protective skin barrier results in a local invasive following apparently successful pressure sore surgery is
wound infection that is nourished by the dead and dying the presence of an unrecognized nidus of chronic bone
tissue of the pressure ulcer. The establishment of this local infection. In such a situation, the chronic infection may
infection is a major contributor to the tissue destruction manifest itself by the development of a draining sinus tract
that ensues. It is generally accepted that infection is pre- or an abscess rather than a typical pressure ulcer.
sent when a quantitative assay of the cultured organism(s) Preoperative diagnostic efforts involving nuclear
exceeds 105 organisms per gram of tissue. A bacterial bur- scanning combined with routine radiographic studies and
den that is less than that figure is not considered an infec- swab cultures are frequently resorted to in an attempt to
tion, but the wound is said to be colonized. In the afebrile rule out the presence of infected bone. Such studies not
patient with a normal white blood cell count and a only can be costly but may also be misleading. A preop-
chronic or healing subacute ulcer, wound cultures will erative swab culture of the wound with suspected
reveal a variety of organisms, but only as colonizers, and osteomyelitis has poor correlation with the organisms
infection is usually not present. The use of systemic or actually infecting the bone and should not be relied on for
topical antibiotics is not warranted in colonized ulcers the selection of antibiotic therapy (31).
598 MUSCULOSKELETAL CARE
Standard X-ray studies are the most cost-effective is indeed bone involvement. Acute and chronic inflam-
imaging study, but may give a false-negative or false-pos- matory cell infiltration, marrow fibrosis, and microab-
itive results in 50 percent of cases (32). The inflammation scesses are the microscopic criteria used to confirm the
associated with an adjacent pressure ulcer, pressure- diagnosis of osteomyelitis. If there is no inflammation, the
related bone changes, severe osteoporosis, hypertrophic bone changes are most likely related to pressure effects.
bone formation, osteosclerosis, synovitis, and degenera- Thus, bone cultures must be correlated with the bone his-
tive joint disease (DJD), as well as previous surgeries with tology, because contamination from the colonized soft tis-
partial ostectomies, may confuse the interpretation of sue wound can occur. In addition, correlation of the
these studies (33). Although more expensive, the CT scan histopathology and cultures of the surgically resected
can be helpful in demonstrating fluid collections, bony osseous specimen should be made in planning postoper-
erosion, and joint involvement. ative antibiotic therapy.
Radionuclide bone scanning (see Chapter 3) with Adequate ablative surgery of Stage 4 ulcers entails
technetium methylene-99m diphosphate (99mTcMDP) the resection of the bone forming the base of the ulcer.
can be helpful when using a multiphase flow study pro- Postoperative specimens of resected bone should be sent
tocol but only has a moderate degree of sensitivity and for gram staining and culture and sensitivity studies
specificity. Even so, radionuclide imaging studies are often before placing the surgical specimen in formalin or other
reported as compatible with but not diagnostic of fixatives. In addition, a biopsy specimen from bone
osteomyelitis; clinical correlation advised, and false-pos- remaining in the wound bed following a partial ostectomy
itive and -negative results do occur. A bone scan should should be submitted for these same studies, because the
be correlated with other laboratory studies and a careful results will aid in the decision of whether to embark on
clinical evaluation of the patient. a 6-week postoperative course of IV antibiotics. Often the
The best radionuclide results require the concomi- disease is cured by the partial ostectomy and such a
tant comparison of the Tc99m bone scan with a gallium67 prolonged and expensive treatment is not warranted even
radionuclide scan. Of the five patterns of activity associ- if the resected bone has changes compatible with
ated with a combined gallium67 and technetium99m study, osteomyelitis. Again, the definitive diagnosis depends on
when the gallium67 scan shows a larger area of relatively the results of the culture and the histologic examination
more intense uptake than the technetium99m scan, the of the presumed involved bone.
probability of osteomyelitis approaches 100 percent (34).
The definitive diagnostic test remains the bone
Spasticity and Contractures
biopsy, and all other studies must be gauged against it.
Lewis et al. compared plain X-rays, Tc99m bone scans, CT Increased spasticity may be considered a complication of
scans, white blood cell counts (WBC), erythrocyte sedi- unhealed pressure ulcers, because its presence can inhibit
mentation rates (ESR), and preoperative needle biopsies or disrupt the healing process whether by surgical or non-
done in the operating room prior to debridement (singu- surgical means. The open wound may be subliminally
larly or in various combinations) with the definitively perceived as a noxious stimulus and trigger spasms that
diagnostic postoperative resected bone specimen. His can be considered as aggravated withdrawal reflexes. A
group concluded that the combination of an ESR greater new-onset infected pressure ulcer or abscess qualifies as
than 120, a WBC greater than 15,000, and a plain pelvic a noxious stimulus, and increased spasticity may be the
X-ray was as useful as the more costly Tc99m scan alone. first clue that an infection is present. Conversely, one of
This combination of studies gave a sensitivity of 73 per- the adverse effects of spasticity is the causation of pres-
cent and a specificity of 96 percent. Their conclusion was sure ulcers secondary to the shear-generated erosions of
that radionuclide scanning was not necessary (35). skin associated with repeated episodic and convulsive
The preoperative diagnosis often is simply made the muscle spasms. It therefore goes without saying that con-
old fashioned way by exploring the base of the ulcer with trol of spasticity is essential in the prevention and treat-
a gloved finger and palpating the exposed and eroded ment of pressure ulcers. A special section on the medical
bone or bony spicules that confirm bone destruction. management of spasticity, a major consequence of SCI is
Even probing a sinus tract with a cotton-tipped applica- included in this textbook (see Chapter 34).
tor can transmit to the clinician the sensation of rough- In the patient with SCI, contractures are a compli-
ened and eroded bone. cation linked with spasticity and aggravated muscle
If a course of preoperative antibiotics is planned for imbalance and are associated with pressure ulcers.
presumed osteomyelitis, laboratory confirmation of the Untreated spasticity leads to a myriad other SCI com-
offending organisms and their antibiotic sensitivities is plications, such as spontaneous fractures of long bones,
essential. A pre-op punch bone biopsy is the diagnostic dislocations, pain, joint ankylosis, and deformities that
gold standard. The bone biopsy should include the his- prevent proper seating in a wheelchair. Spasticity and
tologic examination of the specimen to verify that there associated contractures, being causative agents in pres-
THE SURGICAL MANAGEMENT OF PRESSURE ULCERS 599
Mechanical debridement using wet-to-dry saline capillaries are sealed (tissue welding) by the thermal energy
dressing, when properly performed, is an effective debrid- of the monochromatic light, absorption of debris and bac-
ing technique. However, the dressings should not be teria does not readily occur. This markedly reduces the
allowed to become too dry if the goal is healing by sec- postoperative chills and fever of the bacteremia that often
ondary intention. Wound desiccation can be a problem, accompany sharp debridement. In addition, swab cultures
particularly for patients in an air-fluidized bed. As they following laser debridement are uniformly sterile and the
dry, in theory, saline dressings tend to become hypertonic closure of debrided wounds often can be accomplished at
and absorb fluid from the wound to maintain a physio- the time of debridement (4246).
logic balance, thus helping to reduce wound edema. An additional pearl concerning wound debridement:
Wound debris tends to adhere to the gauze and frequent the dry, hard eschar, without evidence of putrefaction or
changing of the dressing mechanically removes the surrounding cutaneous inflammation, that is frequently
necrotic material. Whirlpool hydrotherapy is relatively found over the posterior calcaneous usually does not
ineffective compared to diligently performed wet-to-dry require debridement and is better left intact. Contraction
saline dressings. and epithelialization under the periphery of the eschar
When confronted with a granulating wound that is will take place similar to the mummification and autoam-
obviously not surgically clean, as manifested by a per- putation that is observed in the dry gangrenous toe. How-
sistent purulent drainage, the use of a broad spectrum ever, should evidence of necrosis under the eschar be
topical antibiotic such as silver sulfadiazine cream 1 per- noted, as indicated by softening of the eschar and/or the
cent might be considered. The use of topical antiseptic presence of perieschar inflammation or frank pus, the
solutions such as providone-iodine, hydrogen peroxide, eschar should be removed because incipient or frank
or Dakins solution and other chemicals utilized to clean infection is present (47).
up the wound are to be used with caution, because they
are toxic to keratinocytes and fibroblasts in vitro (40,41).
Clinically, their use for a brief period prior to surgical INDICATIONS FOR SURGICAL
closure appears to have no observable adverse effect on INTERVENTION
subsequent wound healing. Nevertheless, cytotoxic anti-
septic agents or the prolonged use of topical silver sulfa- Not all pressure ulcers require surgical closure, particu-
diazine are not recommended in a wound healing by sec- larly if the wound in question is expected to heal in a few
ondary intention. Topical agents must never be weeks or months by an alternative noninvasive method.
substituted for good, diligent wound care. Neither should a patient at the end of life be subjected
Sharp debridement at the bedside involves resection to a major reconstructive procedure. In general, how-
of the diseased tissue down to viable tissue, but has the ever, Stage 3 and 4 ulcers do not meet the promise of
disadvantage of inducing bleeding and being less selective expedient healing and are candidates for a surgical pro-
than enzymatic debridement and maggot therapy. In the cedure, particularly where bone or joint is involved (see
toxic and septic patient, surgical debridement is best Table 43.2).
accomplished in the operating room accompanied by the Ulcers with sinus tracts usually fail to heal by conser-
administration of IV antibiotics. vative means and require some surgical intervention, if only
The surgeon has a variety of tools available for the
debridement of abscessed necrotic tissue. The scalpel and
scissors have given way to other safer modalities that
reduce the likelihood of bacteremia and dissemination of TABLE 43.2
the infection as blood and lymphatic vessels are disrupted. Indications for Surgical Interventions
Electrocautery, normally an operating room procedure,
is less sanguineous and reduces the dissemination of bac- Necrotic/infected tissue requiring surgical debridement
teria, but also can injure viable tissues and causes coag- Stage 3 and 4 ulcers with or without bone/joint involve-
ulation necrosis and tissue desiccation. ment
The use of the CO2 laser, although slower than other Nonhealing sinus or fistulous tracts
surgical extirpative methods, is more selective in limiting Significant undermining of adjacent tissue
tissue damage and is usually bloodless. The monochro- Chronic, nonhealing ulcers
matic, collimated (laminar) light energy is absorbed by Chronic ulcers with scarred, fibrotic bed and/or
water and is transformed into thermal energy, boiling the keratinized peripheral rim
cellular water and rapidly vaporizing the purulent debris. Heterotopic (ectopic) bone formation
Surface tissue injury is limited, because the penetration of Wound complications not responding to local wound
the destructive beam is confined to only a few hundredths care
of a millimeter (0.23 mm at 40 watts of power). Because
602 MUSCULOSKELETAL CARE
to unroof the chronic tract. Sinus tracts leading to bone Vital to the program is a multidisciplinary team of oper-
should suggest the presence of osteomyelitis, and penetra- ating room personnel, staff nurses, dieticians, the primary
tion through a joint capsule is indicative of pyarthrosis. care physician, physiatrists and various therapists, infec-
With joint involvement, the infectious process usually tious disease specialists, medical social workers, case
erodes the joint cartilage and bone, and debridement of the managers, mental health personnel, and chaplains, among
infected tissue followed by wound closure is indicated. The others. The definitive goal of pressure ulcer wound care,
sinogram is a useful tool in determining the course of a sinus like all rehabilitation endeavors, is to return the patient
tract particularly where joint involvement is suspected. to a safe community environment after attaining the max-
Surgery is also indicated where there is significant imum restoration of function and the prevention of ulcer
undermining in the subcutaneous tissue plane (Figures recurrence. This is best accomplished by closing the
43.1D and 43.1F). Healing of these wounds by secondary wound with the minimal amount of functional disabil-
intention is significantly delayed or even arrested, thus ity and including the entire treatment team in the post-
leading to a chronic nonhealing wound. Any ulcer that operative recovery process.
becomes chronic and no longer responds to nonsurgical To accomplish appropriate wound closure by sur-
measures should be considered a candidate for surgical gical means, there are certain historically proven basic
closure. Stage 3 or 4 chronic ulcers are often accompa- principles and rules of surgery that every well-trained sur-
nied at the skin level by a hard encircling ischemic fibrous geon must know and abide by to achieve these goals while
ring (annulus fibrosis) that prevents the process of wound avoiding complications and disastrous outcomes (see
contraction. This situation is often seen when there is a Table 43.3). As an obvious example, purulent abscesses
significant ulcer crater. Because epithelial migration should be drained.
requires a skin-level granulating surface, any significant The removal (debridement) of all devitalized or
depth to a wound will inhibit epithelial migration. Epithe- necrotic tissue is a basic concept in the surgical treatment
lium does not like to grow downhill thus resulting in of wounds. Exposed tendons, with loss of their nourish-
the accumulation of the new cells at the lip of the ulcer ing tendon sheath, must be included in the debridement
which, with time, become hyperkeritinized. both proximally and distally under the adjacent intact soft
In a chronic wound with arrested healing, the deeper tissue because these denuded tendons will not survive.
tissues may be covered by a dull layer of pale granula- Effective wound debridement is essential in controlling
tion tissue, which indicates the presence of significant infection prior to any wound closure. During the subse-
chronic fibrous scar tissue having diminished circulation quent surgical closure of pressure ulcers, the excisional
and reduced resiliency. Hypertrophic granulation tissue debridement of the entire colonized granulating pyogenic
(proud flesh) may also be seen in chronic wounds and membrane lining the ulcer and any involved bone is
sometimes is accompanied by polypoid masses of granu- required. Adjacent ischemic scar tissue secondary to a pre-
lation tissue. Such tissue lacks fibrotic scar cells and is viously healed ulcer should also be excised, leaving only
spongy to the touch. Granulation tissue of this type is healthy well-vascularized tissue.
not conducive to epithelial migration and should be Other principles include the elimination of any
removed by the application of silver nitrate or scraping dead space in the surgically closed wound, ensuring
the wound bed with a tongue blade. wound hemostasis and control of hemorrhage, postop-
Pressure ulcers complicated by the presence of erative wound drainage by a closed wound evacuation
ectopic bone are a special problem and are discussed more system to avoid potential collections of blood or serum
fully in Chapter 44. When associated with underlying het- exuding from the raw wound surface, and the restoration
erotopic ossification (HO), surgical repair may fail unless
the problem of this soft-tissue bone is addressed also. The
pressure effects over the unyielding bone mass can also
be a causative factor in the initial development of the ulcer TABLE 43.3
and should be addressed like any osseous pressure point Basic Principles of Wound Surgery
to diminish the probability of recurrence. In addition,
osteomyelitis involving this extraskeletal bone, secondary Adequate wound Preservation of blood
to direct involvement by the ulcer, may be present, as sug- debridement supply
gested by a chronic sinus tract. Obliteration of dead space Control of infection
Accurate hemostasis Postoperative wound
drainage
BASIC SURGICAL PRINCIPLES Restoration of tissue planes Adequate nutrition
Tension-free wound closure Avoidance of secondary
The operative treatment of pressure ulcers in persons with injury
SCI involves not just a surgeon and an agreeable patient.
THE SURGICAL MANAGEMENT OF PRESSURE ULCERS 603
FIGURE 43.5
of trochanteric ulcers with involvement of the hip joint, situations is not always inevitable and preservation of the
osteomyelitis of the proximal femur, ankylosis secondary limb by a Girdlestone arthroplasty (upper femorectomy)
to heterotopic bone formation, and flexion contractures is the preferred treatment.
(6062). As discussed earlier, there are both nonsurgical Garthorne R. Girdlestone originally described his
and surgical alternative procedures other than amputa- operation in 1943 (63,64), having developed the proce-
tion that are available to control the lower extremity con- dure earlier in India for the surgical treatment for tuber-
tractures associated with pressure ulcers. culosis of the hip. The procedure involves resection of the
There are indeed situations where an amputation is infected femoral head and any involved proximal femur
necessary, particularly in an ischemic and gangrenous as well as all involved soft tissue, including the joint cap-
limb or uncontrolled infection in the distal extremity. sule. The acetabulum is thoroughly debrided of involved
Amputation, however, must be weighed against the loss cartilage and synovia with a curette. Repair usually uti-
of support granted by the lower limbs and subsequent lizes a local muscle, such as the vastus lateralis or biceps
body imbalance in the wheelchair-confined patienta femoris, for obliteration of the resulting dead space (Fig-
problem that can lead to recurrent ulcerations of the but- ure 43.7). The transposed muscle can be covered by a
tock and ischial areas. In addition, there are psychologic split-thickness skin graft but usually the wound can be
aspects for the patient who has lost so much and now closed primarily or, where there is a large soft tissue
experiences the addition visible loss of a portion of his deficit, by a more substantial local flap such as the ten-
body. Serious considerations of alternative methods of sor fascia lata flap.
wound closure must be pursued and amputation only In cases where the infection is not under control,
considered as a last resort. reconstruction following femoral head resection is usu-
Concerning Stage 4 ulcers that involve a joint such ally delayed until the wound is clinically free from puru-
as the hip, if uncontrolled, the infection not only destroys lent drainage or other signs of infection. The wound is
the joint capsule, synovial lining, and articular cartilage packed open, with frequent dressing changes as needed,
but also can erode into the adjacent bone and establish until an elective delayed secondary closure of the wound
frank osteomyelitis (Figure 43.6). Ulcers involving the can be safely accomplished. Careful attention to any
interphalangeal joints of the toes are best treated by nutritional deficit should be corrected during this often-
amputation, because there is minimal morbidity. But protracted time of wound preparation.
amputation for involvement of major joints such as the Postoperatively, the involved extremity is immobi-
knee or hip is a significant additional handicap in the lized by splinting it to the opposite limb, utilizing an
patient with SCI. Disarticulation of the extremity in such abduction pillow and a well padded mummy wrap of
FIGURE 43.6
FIGURE 43.7
Patient with a Stage 4 B pressure ulcer involving the hip joint
with destruction of the joint capsule resulting in dislocation Vastus lateralis muscle flap being elevated in preparation for
of the femoral head. An upper femorectomy and repair with rotation into the acetabulum following resection of the upper
a vastus lateralis muscle flap and split-thickness skin graft femur and femoral head (Girdlestone procedure) for a Stage
successfully closed the defect with preservation of the limb. 4 B pressure ulcer involving the hip joint.
606 MUSCULOSKELETAL CARE
resilient material and Kerlex and/or Ace wrap (65). scar tissue and the contaminated (colonized) pyogenic
Some authors have recommended an orthopedic external ulcer bed, along with a tension-free primary closure,
fixator device with pins in the iliac crest and the posterior should be adequate to close these wounds.
femoral shaft (66) but such a device is awkward and a dif- When the above conditions for primary repair are
ficult nursing problem, as well as requiring a continuous absent, then flap reconstruction is required. Although a
proning position on the part of the patient. Quadripleg- split-thickness skin graft is less complicated than a flap
ics are not able to prone because of the interference with in cases with tissue loss, the use of skin grafts is usually
diaphragmatic breathing, and aspiration from eating in not adequate or appropriate in the case of pressure ulcers,
the prone position is a real risk. In an osteopenic patient, particularly over a bony prominence. Skin grafts are eas-
the use of an external fixator has its own problems, ily traumatized over pressure points and their donor sites
because pins may loosen and become infected. add an additional Stage 2 wound that frequently is diffi-
Successful closures of ulcers involving the hip joint cult to heal in SCI patients.
by upper femorectomy have been reported recently in Skin grafts can be used in conjunction with flap
the range of 90 to 100 percent (67,68), and the proce- surgery where there is a distal donor defect, usually in a
dure is considered the definitive treatment of hip non-weightbearing area, following the rotation or
pyarthrosis associated with Stage 4 pressure sores. The advancement of a flap to cover an ulcer site. In such cases,
only other surgical option, as mentioned previously, is we prefer to obtain the graft from an area where the skin
total hip disarticulation. is abundant, such as the anterior abdominal wall. Such
Other more radical approaches to major pressure donor sites are usually small and can be primarily sutured
ulcers of the pelvic region include the hemipelvectomy following excision of the raw dermal bed. This technique
and the hemicorporectomy, both of which are accompa- avoids the wound healing problems of the classic skin
nied by a high morbidity and a dismal survival rate graft donor site (a Stage 2 wound left to heal by secondary
(69,70). Although such procedures have been considered intention) located on the insensate thigh; these sites are
in patients with massive pressure ulcers, such procedures easily traumatized during the required frequent reposi-
are best reserved for the occasional patient with advanced tioning of the paralyzed patient confined to bed.
malignancy that requires palliation. The contemporary utilization of muscle in pressure
ulcer surgery is partially for the bulk it supplies in the nec-
essary obliteration of any dead space following ulcer
WHEN A GAP, SWING A FLAP excision and partially for the theoretical padding over the
underlying bony prominence. This initial muscle bulk,
The development and evolution of soft-tissue flap surgery however, diminishes in time as the muscle atrophies, los-
has been the basic instrument in the surgeons weapons ing of 30 to 50 percent of its mass within a year follow-
system for the closure of chronic pressure ulcers. It is not ing surgery. This loss of bulk is probably related to the
the intent of this treatise to discuss the technical creation direct pressure effects in the sitting patient and the asso-
of the variety of such flaps, because there is an abundant ciated periods of pressure-associated ischemia; muscle tis-
literature available on this subject for interested clinicians sue is less resistant to ischemia than skin.
(71,72). The intent is to familiarize those caring for SCI The main use of the muscle flap, however, is because
patients with the concepts behind the principles of flap of its superior blood supply. There is an abundant axial
reconstruction in the surgery of pressure ulcers. (longitudinal) circulation within muscle bellies. Their
From the beginnings of pressure ulcer surgery in the derivative perforators lead to the overlying subcutaneous
late 1940s and early 1950s, the primary workhorse has fat and skin and are the principle source of blood supply
been advancement or rotational skin and subcutaneous for skin. This axial pattern of blood supply permits the
flaps (73,74). Such transfers of large tissue mass required transfer of larger flaps than could be accomplished with
an intact blood supply, as distinguished from free skin the random skin flaps used by earlier surgeons, where the
grafts. When planning a procedure, a general principle of circulation depended on the internal labyrinth of sub-
reconstructive surgery is to select the least complicated dermal capillaries. The flaps dominant circulation of low-
operation that will accomplish the goal of the intended pressure, subdermal capillaries limits the size of a random
surgery without undo morbidity, because the more com- flap (Figure 43.8) and its length-to-width dimension could
plicated the procedure, the greater the risk of complications. not exceed a 2.5:1 ratio without resorting to a multistaged
Flap surgery is not always mandated. In the case of (delayed) flap. It is now appreciated that the main blood
a small nonhealing Stage 3 ulcer that has failed to heal supply to the skin is derived from the perforator vessels
after a trial of bedrest and appropriate local wound care, arising from the axial circulation of the underlying super-
it would be unwise to mobilize a large adjacent flap when ficial muscles that traverse the subcutaneous fat to ter-
a simple primary closure would do the job. Careful atten- minate in the arborescen circulation of the dermal capil-
tion to hemostasis following complete debridement of laries (Figure 43.8C). An additional type of axial
THE SURGICAL MANAGEMENT OF PRESSURE ULCERS 607
FIGURE 43.8
alyzed patient by the nursing staff can disrupt the sutures blood and serum are not infrequent. Such collections
and lead to wound dehiscence. make excellent culture media for bacteria and contribute
Another source of stress on the healing suture line to the not-uncommon wound infection problem. Any sig-
that may cause wound dehiscence is the problem of nificant hematoma will require a return to the operating
uncontrolled spasticity that may increase following room for evacuation of the clot and resuturing of the
surgery because of the noxious stimuli from the surgical wound. Seromas may persist during the postoperative
site. Every effort should be made to control any spastic- period and require multiple aspirations. If they do not
ity prior to surgery, but when severe spasticity fails to respond to repeated aspirations, it is useful to replace the
respond to treatment, the patient probably should not be aspirated serum with a sclerosing solution such as sodium
considered a candidate for flap surgery. morrhuate or tetracycline (86).
The problem of spasticity in wound healing has its It is said that postoperative complications are best
converse side in patients with flaccid paralysis. Over the treated preoperatively. This maxim is clearly applicable
years, these patients gradually lose their inactive muscu- to SCI patients. Comorbid medical conditions should be
lar tissue, having it replaced by poorly vascularized adi- optimized, not neglecting the problem of malnutrition
pose tissue that is more easily injured by pressure. Con- that is fairly common even in obese patients. Spasticity
sequently, these muscles have a reduced capacity to must be addressed and attention given to clearing the uri-
support any substantial flap that may be needed in the nary tract of stones and bacteria and attaining a surgi-
repair of a pressure ulcer. cally clean granulating wound that is free from puru-
In addition to disuse atrophy of muscle mass and lence and necrotic tissue.
bone (osteopenia), the vascular tree also atrophies below
the level of injury, as blood is shunted to areas where the
metabolic needs are greater. Combined with the general- POSTOPERATIVE CARE
ized atherosclerosis of aging, there is a relative or absolute AND AMBULATION
ischemia of the affected tissues in long-term SCI patients.
Even with the use of a well-vascularized muscle flap in the Following flap surgery, consideration of the duration of
repair of pressure ulcers, flap circulation can be a prob- bed confinement and the need for a special pressure-relief
lem and lead to flap failure and the loss of essential tis- or pressure-reduction bed should be a part of the post-
sue (the part you needed to cover the defect). The prob- operative care plan. In patients unable to assume a prone
lem of smoking is a major contributor to this problem of position, the use of an air-fluidized (A-F) bed for 2 to 4
flap failure. As to this well-documented risk factor, some weeks postoperatively has been found to be cost-effective
surgeons consider smoking an absolute contraindication aid in successful surgical outcomes, particularly in the
to surgical repair. presence of additional pressure ulcers. Patients with sacral
Circulation difficulties in a flap may be caused by flaps are able to tolerate a supine position without com-
either ischemia (inflow failure) or be secondary to venous promising the flap repair. The average pressure over the
congestion (outflow failure) as evident by a dusky cyan- sacrum in an A-F bed is 37.1 mmHg, compared to 52.4
otic appearance and a prolonged capillary refill time of mmHg for a standard hospital bed (87). In fact, the uni-
greater than 2 to 3 seconds. Hyperbaric oxygen treat- formly distributed pressure on the flap in the high-air loss
ments have been useful in the salvage of these vascular- air-fluidized bed seems to promote the adherence of the
compromised flaps (85). flap to the underlying raw wound surface, thus reducing
Because pressure ulcers are colonized under the best the incidence of seroma and bursa formation under the
of circumstances, the relatively hypoxic and edematous healed flap.
tissue struggling to recover from the surgical insult is an As soon as the clinical situation is deemed safe, the
excellent environment for the multiplication of residual patient is transferred to a low-air loss bed and allowed
pathogenic organisms that may lead to a subsequent to be on a prone gurney, if that is tolerated. Should the
wound infection. Most surgeons, therefore, provide intra- patient be a smoker, the use of the prone gurney is delayed
venous prophylactic antibiosis in the early postoperative until it is felt that the wound is healed sufficiently to tol-
period, and it is prudent to extend the perioperative pro- erate an expected return to that habit by the patient.
phylaxis for 72 hours following surgery, rather than the A self-contained closed drainage system, such as the
normal single intraoperative dosing recommended for JacksonPratt device, is a usual accompaniment of pres-
clean surgical cases. Antibiotic treatment can then be sure ulcer flap surgery. It is usually needed for about 10
extended, based on the most recent (collected in the OR) to 14 days and its removal is guided by the reduction of
sensitivity studies, if the clinical condition warrants it. the wound drainage to about 5 to 10 cc per 8-hour shift,
Even with the use of postoperative closed-wound at which time the drainage in a healthy wound is mostly
drainage and the careful attention to the obliteration of serous rather than sanguineous. These devices do obstruct
dead space during the surgical repair, collections of by clot formation so diligence must be maintained.
610 MUSCULOSKELETAL CARE
ing Concept of Systemic Inflammation. In: Fein AM, Abraham 55. Eltorai I, Khonsari F, Montroy RE, Comarr AE. Urinary fistulae
EM, Balk RA, Bernard GR, Bone RC, Dantzker DR, Fink MP after radical ischiectomies in surgery of ischial pressure sores.
(eds.). Sepsis and Multiorgan Failure. Baltimore: Williams & Paraplegia 1985; 23:379385.
Wilkins, 1997: 310. 56. Chang N, Mathes SJ. Comparison of the effects of bacterial inoc-
28. Shae JD. Pressure sores: Classification and management. Clin ulation in musculocutaneous and random-pattern flaps. Plast
Orthop 1975; 112:89100. Reconstr Surg 1982; 70:1.
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30. Wheeler AP and Bernard GR. Current concepts: Treating patients 58. Georgiade N, Pickrell K, Maguire C. Total thigh flaps for exten-
with severe sepsis. N Engl J Med 1999; 340: 207214. sive decubitus ulcers. Plast Reconstr Surg 1956; 17:220.
31. Rousseau P. Pressure ulcers in an aging society. Wounds 1989; 59. Royer J, Pickrell K, Georgiade N, Mladick R, Thorne F. Total thigh
1(2):135141. flaps for extensive decubitus ulcers. Plast and Reconst Surg 1969;
32. Sugarman B, Hawes S, Musher DM, et al. Osteomyelitis beneath 44(2):109118.
pressure sores. Arch Intern Med 1983; 143:683688. 60. Van Enst W. Hip disarticulation in patients with paraplegia and
33. Deloach ED, Check WE, Long R. Diagnosing osteomyelitis under- a survey of the treatment of pressure sores and its complications
lying pressure sores. Contemp Ortho 1993; 27(3):240246. Arch Chir Neerl 1963; 15: 249.
34. Tumeh SS, Aliabadi P, Weissman BN, McNeil BJ. Chronic 61. Cannon B, OLeary JJ, ONeil JW, Steinsieck R. An approach to
osteomyelitis: Bone and gallium scan patterns associated with treatment of pressure sores. Ann Surg 1950; 132:760.
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35. Lewis VL, Bailey MH, Pulawski G, Kind G, Bashioum RW, Hen- femoral resection and vastus lateralis flap in the treatment of het-
drix RW. The diagnosis of osteomyelitis in patients with pressure erotopic ossification in patients with spinal cord injury. Contemp
sores. Plast Reconst Surg 1988; 81(2):229232. Orth1995; 31(6):341347.
36. Eltorai I, Montroy RE. Muscle release in the management of spas- 63. Girdlestone GR. Acute pyogenic arthritis of the hip: An operation
ticity in spinal cord injury. Paraplegia 1990; 28:433440. giving free access and effective drainage. Lancet 1943; 1:419.
37. Marjolin JN. Ulcere. In: Dictionnaire de Medecine. 1820. 64. Girdlestone GR, Somerville EW. Tuberculosis of Bone and Joint.
38. Bell D, Young JW, Banchereau J. Dendritic cells. Adv Immunol Oxford: Oxford University Press, 1952; 101.
1999; 72:255324. 65. Eltorai I. The girdlestone procedure in spinal cord injured patients:
39. Sherman RA, Wyle F, Vulpe M. Maggot therapy for treating pres- A ten year experience. J Amer Paraplegia Soc 1983; 6(4):8586.
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18(2):7174. from sores requiring proximal femoral resection. Ann Plast Surg
40. Johnson AR, White AC, McAnalley B. Comparison of common 1988; 21:246250.
topical agents for wound treatment: Cytotoxicity for human 67. Rubayi S, Pompan D, Garland D. Proximal femoral resection and
fibroblasts in culture. Wounds 1989; 1(3):186192. myocutaneous flap for treatment of pressure ulcers in SCI. Ann
41. Teepe RGC, Koebrugge EL, Lowik CWGM, Petit PLC, Bosboom Plast Surg 1991; 27(2):132138.
RW, Twist IM, Boxma H, Vermeer BJ, Ponec M. Cytotoxic effects 68. Evans GRD, Lewis VL Jr, Manson PN, Loomis M, Vander Kolk
of topical antimicrobial and antiseptic agents on human ker- CA. Hip joint communication with pressure sore: The refractory
atinocytes in vitro. Trauma 1993; 35(1):819. wound and the role of Girdlestone arthroplasty. Plast Reconstr
42. Stellar S, Meijer R, Walia S, Mamoun S. Carbon dioxide laser Surg 1993; 91(2):288294.
debridement of decubitus ulcers: followed by immediate rotation 69. Pearlman NW, McShane RH, Jochimsen PR, Shirazi SS. Hemi-
flap or skin graft closure. Ann Surg 1974; 179:230237. corporectomy for intractable decubitus ulcers. Arch Surg 1976;
43. Eltorai I, Glantz G, Montroy RE. The use of the carbon dioxide 111(10):11391143.
laser beam in the surgery of pressure sores. Internat Surg 1988; 70. Aust J B, Absolon K B. A successful lumbosacral amputation,
73(1):5456. hemicorporectomy. Surgery 1962; 52:756.
44. Montroy RE. Pressure ulcers. In: Achauer BM, Vander Kam VM, 71. Mathes JM, Nahai F. Reconstructive Surgery, Principles, Anatomy,
Berns MW (eds.), Lasers in Plastic Surgery and Dermatology. New and Technique. Edinburgh: Churchill Livingstone,1997.
York: Thieme Medical Publishers, 1992; 164175. 72. McGraw JB, Arnold PG. McCraw and Arnolds Atlas of Muscle
45. Lutchman GD, Staffenberg DA, Herz BL. The carbon dioxide and Musculocutaneous Flaps. Norfolk: Hampton Press Publish-
laser: Decubitus debridement and sterilization. In: Lee BY, Herz ing Company, 1986.
BL, (eds.), Surgical Management of Cutaneous Ulcers and Pres- 73. Conway H, Griffith BH. Plastic surgical closure of decubitus ulcers
sure Sores. New York: Chapman and Hall, 1998; 141154. in patients with paraplegia. Am J Surg 1956; 91:946.
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47. The Treatment of Pressure Ulcers; Clinical Practice Guideline 1981; 67(2):177187.
Number 15. Rockville, Md.: The U.S. Department of Health and 76. Ortichocea M. The musculocutaneous flap method: An immedi-
Human Services, Dec 1994; 49. ate and heroic substitute for the method of delay. Br J Plast Surg
48. Lamon JD, Jr, Alexander E, Jr. Secondary closure of decubitus 1972; 25:106.
ulcers with aid of penicillin. JAMA 1945; 127:396. 77. Nakajima H, Fujino T, Adachi S. A new concept of vascular sup-
49. Davis, JS. The operative treatment of scars following bedsores. ply to the skin and classification of skin flaps according to their
Surgery 1938; 2:1. vascularization. Ann Plast Surg 1986; 16:1.
50. Kostrubala JC, Greeley PW. The problem of decubitus ulcers in 78. Yamamoto Y, Nohira K, Shintomi Y, Igawa H, and Ohura I.
paraplegics. Plast Reconstruct Surg 1947; 2:403412. Reconstruction of recurrent pressure sores using free flaps. J
51. White JC, Hudson HW, Kennard HE. The treatment of bed sores Reconstr Microsurg 1992; 8:433.
by total excision with plastic closure. US Nav Bull 1945; 45:445. 79. Park S, Koh KS. Superior gluteal vessel as recipient for free flap
52. Conway H, Kraissl CJ, Clifford RH, Gelb J, Joseph JM, Leveridge reconstruction of lumbosacral defect. Plast Reconstr Surg 1998;
LL. The plastic surgical closure of decubitus ulcers in patients with 101:1842.
paraplegia. Surg Gynec Obst 1947; 85:321. 80. Hartrampf CR Jr, Scheflan M, Black PW. Breast reconstruction
53. Comarr AE, Bors E. Radical ischialectomy in decubitus ischial with a transverse abdominal island flap. Plast Reconstr Surg 1982;
ulcers complicating paraplegia: Report of 100 cases. Ann West 69(2):216.
Med Surg 1951; 5:210213. 81. Drever JM. The lower abdominal transverse rectus abdominis
54. Comarr AE, Bors E. Perineal urethral diverticulumcomplication myocutaneous flap for breast reconstruction. Ann Plast Surg
of removal of ischium. JAMA 1958; 168: 20002003. 1983; 10(3):179.
612 MUSCULOSKELETAL CARE
82. Mixter RC, Wood WA, Dibbell DG. Retroperitoneal transplan- 85. Nemiroff PM. Enhanced healing of compromised skin flaps and
tation of rectus abdominis myocutaneous flap to the perineum and skin grafts. In: Camporesi EM, Barker AC, (eds.), Hyperbaric
back. Plast Reconstr Surg 1990; 81:437. Oxygen Therapy. Bethesda, MD: Undersea and Hyperbaric Med-
83. Kroll SS, Pollock R, Jessup JM, Ota D. Transpelvic rectus abdo- ical Society 1991; 149157.
minis flap reconstruction of defects following abdomininal-per- 86. Baeke JL. Treating seromas (letter). Plast Reconstr Surg 2000;
ineral resection. Am Surg 1989; 55:632. 106(5):1224.
84. Tobin GR, Day TG. Vaginal and pelvic reconstruction with dis- 87. Sachse RE, Fink SA, Klitzman B. Multimodality evaluation of pres-
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Surg 1988; 81:62.
44 Heterotopic Ossification
xisting since the debut of the human nature, as first described by Guy Patin in 1692 (2). The
matic brain injuries (TBI), where the severity of the injury osteoblasts that are no longer synthesizing collagen and,
(Glasgow Coma Scale) correlates with the extent of the when exposed to parathyroid hormone, secrete enzymes
ossification measured in square centimeters (14). As in that begin to dissolve osteoid as part of the third or
the cases reported by Dejerine and Ceillier, it is most com- remodeling phase. The primary cell involved with bone
monly associated with spasticity and is always below the resorption, however, is the osteoclast, which is rich in the
level of injury. Neurogenic HO seemingly differs from enzyme acid phosphatase and secretes collagenase and
that seen following direct muscle trauma, such as in other enzymes that are involved in the hydrolysis of col-
myositis ossificans and invasive hip/pelvis surgery, in that lagen and demineralization of osteoid. This balanced
the site of the neurotrauma (brain, spinal cord) is anatom- active metabolic process maintains the equilibrium of the
ically remote from the affected site. mature bony skeleton throughout life.
Conversely, there are reports of HO occurring in The mineralization of the osteoid matrix in the sec-
children with cerebral palsy following iliopsoas and other ond phase involves the deposition of crystalline hydro-
muscle release procedures (15,16) and also following rhi- xyapatite [Ca10(PO4)(6(OH)2], which is then followed by
zotomy with femoral osteotomy (17), in which local mus- the last step in osteogenesisthe remodeling phase
cle, tendon, and bone trauma occurs in the treatment of involving the coupled actions of resorptive osteoclasts and
a spastic central nervous system disorder. bone-reforming osteoblasts. This synergistic, ongoing
The cause of ectopic bone formation is unknown. In process is under the control of various hormones such as
cases such as THA and myositis ossificans, it is reason- estrogens, testosterone, parathormone, and calcitonin, as
able to make an association with the direct muscle injury well as Vitamin D metabolites and prostaglandins. In
and the evoked inflammatory response of repair. But the addition, numerous cytokine growth factor superfamilies,
HO following CNS trauma, with no apparent direct rela- such as skeletal growth factor, insulinlike growth factor,
tionship to the locus of HO formation, resurrects the transforming growth factor, tumor necrosis factor,
ideas of Charcot, who in 1879 put forth a theory, in the acidic/basic fibroblast growth factor, and platelet-derived
case of pressure ulcers, of the traumatic release of a neu- growth factor are involved (19,20). The activity of this
rogenic trophic factor (18). A more contemporary cascade of inflammatory factors in bone formation sug-
hypothesis, however, concerns the general inflammatory gests the possible mechanism and the rationale for the use
response that follows noninfectious and infectious acute of anti-inflammatory drugs, such as indomethacin, in the
injury and involves cellular, circulating, and locally active prevention of HO in at-risk patients.
mediators and effectors. This is known as the Systemic The exact cause of the eccentric formation of ossi-
Inflammatory Response Syndrome (SIRS) (19). Such a fication in the soft tissue of trauma (localized or remote)
response can affect the pluripotential mesenchymal stem patients remains unknown but most investigators believe
cells known to be resident in soft tissues and would HO arises as the result of unknown triggers (possible
explain the association of SCI and TBI and HO in the inflammatory cytokines) that induce the differentiation
early weeks following the acute injury. of pluripotential mesenchymal stem cells resident in soft
tissues into osteoprogenitor cell lines that then differen-
tiate into osteoblasts and the other cell lines involved in
THE PATHOGENESIS OF HETEROTOPIC bone formation. Such stem cells, common in the bone
BONE FORMATION marrow, are now known to be present in a wide variety
of tissues such as adipose tissue and muscle (21,22). Bosch
Because HO is normal bone, it undergoes the same three et al. studied different subpopulations of mouse skeletal
main stages of osteogenesis as does skeletal bone: a) The muscle and their response to the induction of alkaline
formation of an extracellular matrix (osteoid); b) the min- phosphatase after exposure to bone morphogenetic pro-
eralization of this newly formed osteoid to form bone; tein-2 (BMP-2). An isolated subpopulation of adult mus-
and c) bone remodeling and maturation. The process cle cells with an adenovirus construct encoding BMP-2
involves osteogenic cells derived from pluripotential mes- was injected into the hind limb of immune-compromised
enchyme cells of the bone marrow stroma: the mice. These cells appeared to actively participate in the
osteoblasts, osteocytes, and osteoclasts. Intervention for formation of ectopic bone. The authors felt that their data
the prevention or treatment of HO addresses these supported their hypothesis that osteoprogenitor cells
sequential stages of new bone formation. reside within skeletal muscle and were induced to differ-
The osteoblasts synthesize and lay down the pre- entiate into osteoprogenitor cell lines (23).
cursors of collagen I that make up 90 to 95 percent of Bone morphogenetic protein (BMP), first described
the extracellular matrix or osteoid tissue. They also pro- by Urist (24), is a bone matrix protein. It has been found
duce other noncollagenous proteins of osteoid tissue, such to have at least fifteen family members (25), most of
as the proteoglycans of the ground substance, and are rich which belong to the transforming growth factor beta
in alkaline phosphatase. The osteocyte is derived from (TGF-) superfamily that plays an essential role in the for-
616 MUSCULOSKELETAL CARE
FIGURE 44.3
injured SCI patient on prophylactic DVT anticoagulation this group with eighty-four patients in a placebo group.
therapy, adjustments in dosing should be considered to There was no difference in the incidence of HO in the two
prevent bleeding secondary to the effect of NSAIDs on groups, but the treated group had considerably less HO
platelets. NSAIDs may also exacerbate stress-induced and less functional disability than the control group.
peptic ulcer disease with associated upper gastrointesti- Treatment, however, was started relatively late in the nat-
nal tract bleeding, which, although rare, may follow neu- ural history of neurogenic HO, ranging from 20 to 121
rotrauma (the so-called Cushing ulcer). days after the injury, and treatment was only for 3 months
There has been extensive experience in the use of the rather than the 6 months now recommended; thus limit-
diphosphonate EHDP in the treatment of HO. EHDP has ing the value of the studys results.
no effect on osteoid development but does prevent the con- As an alternative to the sometime ineffective med-
version of amorphous calcium phosphate compounds into ications, low-dose radiation has been advocated in the
hydroxyapatite crystals, a critical step in the mineralization immediate postoperative period for patients at high risk
of the osteoid matrix (27,40). EHDP has been used since to develop HO and may be combined with an NSAID as
the 1960s, and a number of treatment protocols have been an additional prophylactic measure (44). It is theorized
put forward, but the effectiveness of EHDP has been nei- that the ionizing radiation alters the nuclear deoxyri-
ther proved nor disproved (27,41). Banovac recommends bonucleic acid (DNA) of rapidly dividing cells and blocks
giving EHDP intravenously for three days, administering the differentiation of the soft tissue pluripotential mes-
300 mg over 3 hours for 3 days, followed by an oral dose enchymal cells into the osteoblastic stem cells that give
of 20 mg/kg/day for 6 months (42), the anticipated 6-month rise to the osteoblasts, osteoclasts, and osteocytes needed
period of HO activity seen in the majority of cases. for osteogenesis.
Because EHDP only affects the mineralization of For this reason, postoperative low-dose radiation
osteoid, theoretically it should be continued indefinitely, has been employed in patients deemed to be at high risk
and recurrence of varying amounts of HO have been for developing HO following THA and in patients with
reported following the discontinuance of the drug established HO undergoing surgical intervention. Because
(27,41). Although there have been reports of good results of the theoretic danger of inducing malignant change, the
with minimal recurrence following treatment for a year use of low-dose radiation therapy is reserved for only
or longer, concerns remain for aggravating the sometimes those deemed at high risk for developing recurrence of
severe osteoporosis found in SCI patients, where a loss their HO.
of bone mass of as much as 40 percent is known to occur Protocols differ as to single-dose or fractionated
in the first year following injury. Because of this combined therapy delivered over a course of several days. Advocates
detrimental effect of skeletal osteoporosis and reduced of fractionated protocols feel that 1,000 centigrays (cGy)
osteoid mineralization, a potential complication of pro- delivered at 200 cGy per day over 5 days is an effective
longed use of diphosphonates is a pathological (sponta- and safe inhibitor of bone growth. This dose was believed
neous) fracture of a long bone; this has been observed in to be equally as effective as a 2,000 cGy fractionated pro-
animal experiments (43). This limited window of EHDP tocol (45). Ayers and his group reported on a number of
treatment of 6 months may permit the process of calcifi- studies using both single-dose and fractionated radiation
cation of nascent osteoid matrix to proceed following dis- protocols that supported the effectiveness of low-dose
continuance, because the ossification process was not radiation therapy following THA surgery (46).When
arrested but only delayed. therapy was delayed more than 4 to 5 days, however, suc-
The question of routine medical prophylaxis with cessful outcomes were reduced in both protocols com-
EHDP in newly injured SCI patients is not well defined pared to treatment initiated in the first 24 to 48 hours,
but is probably not indicated because any ectopic ossifi- even with half the radiation used in delayed protocols.
cation activity in most patients usually subsides within 6 The Ayers group reported that single-dose radiation had
months without serious clinical consequences. The main greater tissue effects than fractionated radiation of equal
value is in patients who go on to develop ankylosis or near or greater total cGy, and they concluded that a single
ankylosis; this cohort represent only a limited small per- treatment in the immediate postoperative period was
centage of SCI patients. Those at risk may be difficult to superior to delayed or fractionated therapy (46). A num-
identify in the predominantly youthful newly injured SCI ber of other authors report equal success with single-dose
cohort with few of the known risk factors present in the radiation therapy of between 600 to 700 cGy, given in the
older THA patient (12). The potential for complications immediate postoperative period (47,48). It would seem
in the SCI soon-to-be osteopenic population diminishes that either single or fractionated radiation between 600
the value of general prophylaxis. and 1,000 cGy is effective, but that therapy should be ini-
Stover et al. (41) prophylactically treated eighty-two tiated early in the postoperative recovery phase during the
patients with disodium etidronate (20 mg/kg for 2 weeks early inductive phase of the pluripotential mesenchymal
and 10 mg/kg for an additional 10 weeks) and compared stem cells transformation into osteoprogenitor cell lines.
620 MUSCULOSKELETAL CARE
Kienapfel et al. (49) compared radiation with the to permit closure of the defect. Because recrudescent HO
NSAID indomethacin, using a single dose of 600 cGy is common, limiting the surgical trauma is the most
between the 2nd and the 4th postoperative day in one acceptable approach and postoperative HO prophylaxis
group and in a comparable second group, 50 mgm BID is indicated (40,43,4650).
of indomethacin for 6 weeks, again starting on the first Where there is a need to restore joint mobility in
day following surgery. They found that each method had Brookers Class IV fused joints (27,53), surgery is the only
comparable results in its effectiveness in the prevention option. In the case of a fused hip joint, the goal should
of postoperative HO and both modalities were signifi- be limited to gaining the degree of mobility that is con-
cantly more helpful than a third control group that sistent with sitting in a wheelchair. It should not be the
received no prophylactic postoperative treatment. complete ablation of the pathology, because the surgery
Adjunctive treatment to maintain the mobility of of extensive HO is difficult and there is a high rate of com-
major joints is a basic tenet of rehabilitation medicine. plications. Limiting the procedure to a simple wedge
Maintenance of the mobility of the major joints of immo- resection (osteotomy) of a long bone distal to an anky-
bile patients is essential to avoid contractures and fibrous losed joint, creating a pseudoarthrosis that permits sitting
ankylosis of joints from disuse. This goal is usually in a wheelchair, is a safer plan than total extirpation of
accomplished by passive range-of-motion (ROM) exer- the entire mass of bone. In other cases where HO is asso-
cises by trained therapists. This is particularly important ciated with osteomyelitis and persistent drainage from a
in any bedridden patient and should not be avoided for deep sinus tract, a sinogram is indicated to ascertain
fear of initiating HO, because gentle range of motion does whether the joint space is involved, because a more major
not seem to be a causative factor in the etiology of HO. procedure, such as a Girdlestone resection, may be needed
The majority of SCI patients in a rehabilitation program (Figure 44.6) (53,54).
do not develop clinically significant HO, and when Garland states that resection of HO should not be
nascent HO is in the preclinical phase, the first clue to its undertaken during the 6-month period following onset
possible presence may be the recognition by the thera- (27). Not only may the process of HO formation be self-
pist of diminishing ROM during therapy. limiting and not clinically important but the majority of
Where there is loss of joint mobility following TBI workers in the field feel that surgical trauma should be
with spasticity, forceful joint manipulation under anes- withheld during the inflammatory phase of active ossifi-
thesia every 2 to 3 months as neurologic recovery takes
place is a useful adjunct in the prevention of contractures.
It should be limited to no more than three manipulations
and does not seem to lead to HO formation (27). How-
ever, some workers have avoided such manipulation of
major joints, believing that such activity may increase the
inflammatory response or result in trauma with bleeding
and hematoma formation and contribute to the onset of
HO or adversely affect the volume of ectopic bone being
formed.
The detractors do so on the basis of experiments in
rabbits in the early 1980s in which forceful and traumatic
manipulation of the limbs caused direct trauma to mus-
cle tissue with hematoma formation that precipitated the
development of centers of ossification in the injured mus-
cle tissue (50,51). Such a setting is more in keeping with
myositis ossificans than the HO of neurogenic origin, in
which there is no recognized local injury. In any event,
there is a consensus that forceful exercises should not be
initiated until the inflammatory signs heralding the acute
onset of HO, if present, have subsided.
The surgical treatment of established HO should
have limited objectives. In the majority of patients who FIGURE 44.6
do not have clinically significant HO, surgery is usually A 57-year-old C5 tetraplegic with a chronic draining sinus of
not indicated. Where the need to close a pressure ulcer is the hip associated with massive HO formation of many years
the goal, however, the underlying ectopic bone mass duration. Sinogram showed a deep sinus tract but without
should be removed to the degree that the pressure point extravasation into the anatomical acetabulum from which the
is eliminated and flap tissue can be mobilized sufficiently femoral head has been displaced.
HETEROTOPIC OSSIFICATION 621
cation if exacerbation of the process is to be limited. Mat- formation. HO is usually self-limiting over a period of 6
uration of the process, as evidenced by a return of the SAP months and may require no active intervention. In the rel-
to baseline, the bone scan, decreasing or at baseline, and atively few cases that progress to bony joint ankylosis,
the radiograph, indicating only mature bone are criteria surgery is the sole option available. Because of a high rate
assisting the clinician in surgical decision making. Such of significant complications, however, surgeons are reluc-
a situation should exist in the majority of cases within 6 tant to attempt more than a limited operation, seeking only
months to a year following the onset of HO. In a limited to restore a sufficient degree of joint mobility to allow the
number of patients where there is X-ray evidence of con- patient to gain wheelchair ambulation. Because recurrence
tinued progression beyond 6 months, the presence of sig- is the most common surgical complication, prophylaxis
nificant amounts of HO, persistent elevation of the SAP, with NSAIDs, EHDP, and low-dose radiation either sin-
continued radionuclide uptake, and a poor response to gularly or in combination early in the postoperative period
prophylactic medications, surgery should be delayed for is indicated. It is expected that the present line of research
18 to 24 months (27). Delaying the needed surgery into the molecular and cellular causes of this potentially
beyond that time frame, however, increases the risk of debilitating process will eventually lead to interventions
intraoperative and postoperative long bone fractures that will prevent this unusual complication of SCI.
caused by the presence of markedly osteoporotic bone.
Surgical complications (46,47) include excessive
bleeding, a high rate of wound infection including References
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13. Riedel B. Demonstration line durch ach Hagiges Umhergehen total
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45 Cardiovascular Fitness
after Spinal Cord Injury
any studies published over past essential activities such as locomotion, transfers, and other
623
624 MUSCULOSKELETAL CARE
with tetraplegia, they are only marginally more fit Changes in Peripheral Vascular
(14,16). No evidence suggests that the levels of fitness Structure and Function
for those with SCI will improve without a committed
The volume and velocity of lower-extremity arterial cir-
effort on their part to increase daily physical activity.
culation is significantly diminished after SCI, with a vol-
Cardiovascular disease (17), hyperlipidemia (18),
ume flow of about half to two-thirds that reported in
insulin resistance (19), musculoskeletal decline (5), and
matched subjects without paralysis (30,31). This so-
visceral obesity (20) are complications of aging commonly
called circulatory hypokinesis(32) results from the
reported among those with SCI, and their onset occurs
loss of autonomic control of blood flow as well as
earlier in life than observed in those without disability.
diminished regulation of local blood flow by the vas-
Fortunately, these complications are successfully treated
cular endothelium (30). The lowering of volume and
in persons without disability using exercise training,
velocity contribute to the heightened thrombosis sus-
although the selection of exercise activities for persons
ceptibility commonly reported in those with acute and
with SCI to accomplish the same goals is more limited.
subacute SCI (23,33). A contributing factor to throm-
The consequences of imprudent exercise are also more
bosis disposition appears to be a markedly hypofibri-
serious than those experienced by persons without dis-
nolytic response to venous occlusion of the paralyzed
ability. It is thus important to identify effective and avail-
lower extremities (34,35), a poor response likely attrib-
able exercise activities that reduce the risks of physical
utable to low blood-flow conditions in the paralyzed
dysfunction and cardiovascular diseases sustained by per-
lower extremities or interruption of the adrenergic path-
sons with SCI while not increasing injury risks or has-
ways that regulate fibrinolysis for persons with an intact
tening musculoskeletal deterioration. The following sec-
neuraxis (36,37).
tions will address how individuals with SCI can exercise
under voluntary and electrically stimulated control, and
the common risks of their exercise participation.
EXERCISE RECONDITIONING
OF THE CIRCULATORY SYSTEM
The benefits of arm training are widely reported for (102). Thus, higher intensities, durations, and frequencies
persons with paraplegia, although their performance is lim- of exercise training may be required to invoke significant
ited by the circulatory dysregulation accompanying tho- exercise-related lipid/lipoprotein changes, as is the case for
racic SCI (113117). Despite having relatively normal car- persons without paraplegia, who often fail to alter their
diac output, individuals with injuries below the level of lipid profiles following low-intensity exercise training, but
sympathetic outflow at T6 have significantly lower resting do so at higher intensities (127).
SV and higher resting HR than persons without paraple-
gia (116118). The significant elevation of resting and exer-
cise HR is thought to compensate for a lower cardiac SV ARM RESISTANCE EXERCISE
imposed by pooling of blood in the lower-extremity venous
circuits, diminished venous return and cardiac end-diastolic Whereas strengthening of muscles spared by SCI would
volumes, or frank circulatory insufficiency (116119). appear to benefit those with physical disability, few stud-
Adjustments to the adrenergic systems after SCI may also ies have tested effective exercise training programs to do
regulate the excessive chronotropic response to work, so. The most commonly reported symptom of upper
because higher resting catecholamine levels and exagger- extremity physical dysfunction among persons with SCI
ated catecholamine responses to physical work have been is pain (128,129), and the most common site, the shoul-
reported in paraplegics with mid-thoracic (T5) cord injuries der joint (128,130134). Rotator cuff dysfunction, tears,
(120). These exceed resting and exercise levels of both high- and impingement are common (135,136). Although a sin-
level paraplegics and healthy persons without SCI gle cause for shoulder pain has not been identified, dete-
(120,121). Hypersensitivity of the supralesional spinal cord rioration and injury resulting from insufficient shoulder
is believed to regulate this unusual adrenergic state and strength, range, and muscle endurance are commonly
dynamic, which contrasts the downregulation of adrener- cited (133135,137,138). Pain that accompanies wheel-
gic functions observed in persons with high thoracic and chair locomotion and other wheelchair activities report-
cervical cord lesions (120122). edly interferes with functional activities, including upper-
An exaggerated HR response is experienced during extremity weight bearing for transfers, high-resistance
physical activity by persons with paraplegia (32). This find- muscular activity in extremes of range of motion, wheel-
ing is consistent with reports in which subjects with para- chair propulsion up inclines, and frequent overhead activ-
plegia require higher levels of oxygen consumption to per- ity (128,132134). In eighty-four patients with paraple-
form the same work intensity as subjects without SCI gia at least 1 year post-injury, fifty-seven (67.8 percent)
(103,114,117,120,123), and may represent a limiting fac- complained of pain in one or more areas of their upper
tor in the performance of activities of daily living extremities. Onset was most common during transfer
(117,124,125). Because the sympathetic nervous system activities, and severity increased as time following injury
regulates hemodynamic and metabolic changes accompa- lengthened (128). Given evidence that wheelchair loco-
nying exercise (126), the elevated oxygen consumption and motion is a major source of pain and dysfunction for per-
HR response to work in paraplegics with injuries below sons with SCI, its use as a conditioning mode ought to
T5 may be caused by adrenergic overactivity accompany- be limited to cases in which training specificity and skill
ing their paraplegia (120,121). Despite these hemody- acquisition for an athletic event are sought.
namic and metabolic limitations, significant improvements Unlike studies examining the effects of endurance
in blood lipid profiles and cardiovascular risk accompany exercise on the cardiovascular system, strengthening of the
their exercise training. For example, significant inverse upper extremities in persons with paraplegia has received
relationships have been reported between VO2peak and the limited attention. In a study of Scandinavian men (most of
total cholesterol (TC) to high-density lipoprotein choles- whom had incomplete low thoracic lesions), a weight train-
terol (HDL-C) ratio, serum triglycerides, and the low-den- ing program with special emphasis on the triceps (for elbow
sity lipoprotein cholesterol (LDL-C) to HDL-C ratio in extension during crutch walking) was undertaken for 7
subjects with paraplegia, as well as a direct significant asso- weeks with modest but significant increases in VO2peak
ciation between VO2peak and the HDL-C to apoprotein A- observed following training, accompanied by increased
1 ratio (16). These findings suggest a direct association strength of the triceps brachii (139). Another study (140)
between cardiovascular risk indices and fitness similar to examined the effects of arm ergometry in subjects assigned
that observed for persons without paraplegia. In another to high intensity (70 percent of their VO2peak) or low inten-
study, blood samples were analyzed for TC, TG, HDL-C, sity (40 percent of their VO2peak) for 20 or 40 minutes per
and LDL-C in subjects undergoing wheelchair ergometry session, respectively. Strength gains were limited to subjects
training conducted at 50 to 60 percent and 70 to 80 per- assigned high-intensity training, and occurred only in the
cent of the HR reserve. The high-intensity training group shoulder joint extensor and elbow flexor muscles. Other-
experienced increased HDL-C and decreased TG, LDL- wise, no changes in shoulder joint abduction or adduction
C, and TC to HDL-C ratio, though not their VO2peak strengths were reported, and none of the muscles that move
CARDIOVASCULAR FITNESS AFTER SPINAL CORD INJURY 627
or stabilize the scapulothoracic articulation or chest were encountered will be similar to those experienced by per-
stronger following training. These results suggest that arm sons without paralysis, although complications such as
crank exercise is ineffective as a training mode for upper- general overuse may be exaggerated in persons with SCI.
extremity strengthening because it fails to target the mus- It should further be anticipated that their occurrence will
cles most involved in the performance of ADLs. compromise daily activities to a far greater extent the
The use of resistance exercises to improve fitness has overuse or injury arising in persons without SCI.
been reported by several investigators. Conditioning of Various complications may be experienced by indi-
five paraplegic and five tetraplegic subjects three times viduals exercising under the control of electrical current.
weekly for nine weeks was reported using a hydraulic fit- The foremost of these complications involves episodes of
ness machine. Exercises were limited to two maneuvers: autonomic dysreflexia, which can occur in individuals
chest press/chest row and shoulder press/latissimus pull having injuries at and above the T6 spinal cord level
(141). Significant increases in average VO2 and power (147,148). Recognition of these episodes, withdrawal of
output, as measured by arm ergometry testing, were the offending stimulus, and the possible administration
observed at the conclusion of the study, although no test- of a fast-acting peripheral vasodilator may be critical in
ing was conducted that directly measured strength gain preventing serious medical complications. Prophylaxis
in any muscle groups undergoing training. Another study with a slow calcium channel antagonist or selective adren-
focused training on strengthening of the scapular muscles, ergic antagonist may be needed prior to exercise
although this study focused solely on the scapular retrac- (149151). It is known that wheelchair racers intention-
tor muscles when comparing seated rowing and a stan- ally induce such autonomic dysreflexia during exercise as
dardized scapular retraction exercise, and did so only for an ergogenic aid by restricting urine outflow through a
concentric actions (142). The authors found that higher Foley catheter (152). This is a dangerous practice that
levels of retractor activation were obtained during back- should be discouraged by healthcare professionals.
ward than forward wheelchair locomotion, and suggested Fracture and joint dislocation are risks of exercise in
that rowing was effective for improving scapular retrac- osteopenic bones caused by the asynergistic movement of
tor activity and cardiorespiratory fitness. A recent study limbs against the force imposed by either electrical stim-
observed reduced shoulder pain following a series of ulation or the device used for exercise (153). This explains
shoulder resistance exercises using elastic bands (138). why these activities are contraindicated for individuals
The most effective resistance training program for with severe spasticity, or spastic response to the intro-
persons with paraplegia has been a circuit resistance exer- duction of electrical current (47). Small risks of post-exer-
cise program modeled after successful training exercises cise hypotension (58) are associated with lost vasomotor
used by individuals without physical disability (143,144). responses to orthostatic repositioning (65), although these
Subjects in this study underwent exercise using a series of episodes can abate after upper limb training.
six alternating isoinertial resistance arm exercises on a Precautions to prevent overuse injuries of the arms
multistation gym and high-speed, low-resistance arm and shoulders must be taken for those participating in
ergometry (145). Emphasis was placed on strengthening upper extremity exercise (8,154,155). Because the shoul-
of the upper back and rotator cuff muscles, and stretch- der joints are ill designed to perform locomotor activi-
ing of the normally tightened pectorals. After 12 weeks ties but must do so in individuals unable to walk, injuries
of exercise conducted 3 times weekly, subjects with para- may compromise essential activities of wheelchair propul-
plegia experienced a 30 percent increase of VO2peak and sion (156), weight relief (157), and depression transfers
a 12 to 30 percent increase in isoinertial strength. Gains (156) and profoundly influence normal daily activities in
in fitness and strength exceeded those previously persons with SCI. In addition, individuals with SCI often
reported, following either arm endurance exercise or lack sudomotor responses below their level of injury and
strength training in this subject population. More are thus challenged to maintain thermal stability
recently, this conditioning program has been shown to (158160), although these effects are less pronounced as
improve the lipid profiles of individuals with paraplegia; the level of SCI descends (73,161) and when exercising in
reduce their cardiovascular disease risk as assessed by ele- an environment controlled for temperature and humidity
vation of the HDL-C; and significantly lower LDL-C and (162,163). Thus, attention should be paid to hydration
the TC to HDL-C ratio (146). and, if possible, limiting the duration and intensity of
activities performed in intemperate environments.
UNIQUE EXERCISE
COMPLICATIONS AND CAVEATS CONCLUSIONS
Special consideration is required when designing exercise Many persons with SCI lead sedentary lifestyles imposed
programs for persons with SCI. Some of the exercise risks by choice, limited sparing of the muscles needed to initi-
628 MUSCULOSKELETAL CARE
ate and sustain body movement, or narrow exercise obic power in spinal cord injured men. Med Sci Sports Exerc
1991; 23:409414.
options. These sedentary lifestyles compromise their 17. Bauman WA, Spungen AM, Raza M, Rothstein J, Zhang RL,
health and function as they age with their disability. Zhong YG, Tsuruta M, Shahidi R, Pierson RN Jr, Wang J, et al.
Although exercise injuries need to be anticipated and spe- Coronary artery disease: Metabolic risk factors and latent dis-
ease in individuals with paraplegia. Mt Sinai J Med 1992;
cial caveats observed, many exercise options are available 59:163168.
for persons with SCI that reverse the effects of immobi- 18. Bauman WA, Spungen AM. Disorders of carbohydrate and lipid
lization deconditioning and sedentary living. Evidence metabolism in veterans with paraplegia or quadriplegia: A model
of premature aging. Metabolism 1994; 43:749756.
suggests that desirable multisystem adaptations accom- 19. Duckworth WC, Solomon SS, Jallepalli P, Heckemeyer C, Finnern
pany many of these exercises, which likely enhances the J, Powers A. Glucose intolerance due to insulin resistance in
ability of persons with SCI to lead more healthy, active, patients with spinal cord injuries. Diabetes 1980; 29:906910.
20. Zlotolow SP, Levy E, Bauman WA. The serum lipoprotein profile
satisfying, and productive lives. Greater emphasis on the in veterans with paraplegia: The relationship to nutritional fac-
effects of conditioning exercise across the spectrum of tors and body mass index. J Am Paraplegia Soc 1992; 15:158162.
injuries following complete and incomplete spinal cord 21. McKinley WO, Jackson AB, Cardenas DD, DeVivo MJ. Long-
term medical complications after traumatic spinal cord injury:
paralysis is needed to better examine functional and A regional model systems analysis. Arch Phys Med Rehabil 1999;
health changes resulting from increased physical activity 80:14021410.
and exercise pursuits as persons age with SCI. 22. King ML, Lichtman SW, Pellicone JT, Close RJ, Lisanti P. Exertional
hypotension in spinal cord injury. Chest 1994; 106:11661171.
23. Grossman WE, Braunwald T, Mann LP, McLaurin Green LH.
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10:171176. 162. Ishii K. er al. Effects of upper limb exercise on thermoregulatory
144. Mosher PE, Nash MS, Perry AC, LaPerriere AR, Goldberg RB. responses in patients with spinal cord injury. Appl Human Sci
Aerobic circuit exercise training: Effect on adolescents with well- 1995; 14:149154.
controlled insulin-dependent diabetes mellitus. Arch Phys Med 163. Price MJ, Campbell IG. Thermoregulatory responses of spinal
Rehabil 1998; 79:652657. cord injured and able-bodied athletes to prolonged upper body
145. Jacobs PL, Nash MS, Rusinowski JW. Circuit training provides car- exercise and recovery. Spinal Cord 1999; 37:772779.
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REHABILITATION
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46 Wheelchairs and Seating
heelchairs and their seating sys- orthotics designed to meet the mobility demands of the
635
636 REHABILITATION
removable armrests, a single cross-brace frame, and solid severe orthopedic impairments. If necessary, specialized
tires (11). Swing-away footrests add weight to the wheel- seating can be adapted to most childrens wheelchairs.
chair. However, they make transferring into and out of
the wheelchair easier. Armrests provide some comfort and
Lightweight and Ultralight Wheelchairs
stability to the depot wheelchair user, and they can aid
in keeping clothing off the wheels. Depot chairs typically The rehabilitation wheelchair is designed to be used as a
fold to reduce required storage area, and to fit into the mobility device (1215). The selection and configuration
automobile of the borrower. Solid tires are commonly of a rehabilitation wheelchair involves understanding the
used to reduce maintenance. Solid tires dramatically needs of the intended user. Some rehabilitation wheel-
reduce ride comfort and add weight. (Pneumatic tires are chairs can be assigned to a category by their intended use
recommended for outdoor usage.) There is very little, if or intended user.
anything, that can be adjusted to fit the user on a depot Those who have weakness of the upper and lower
chair. Typically, only the leg-rest length is adjustable. extremities can gain maximal benefit from wheelchair
Depot chairs are available in various seat widths, seat propulsion by combining the use of their arms and legs
depths, and backrest heights. These dimensions must be or by using their legs. The design and selection of a foot-
specified to the manufacturer or distributor. drive wheelchair depends greatly upon how the user can
Not all wheelchairs are propelled by the person sit- take greatest advantage of his/her motor abilities. The
ting in the wheelchair. In many hospitals and long-term strength and coordination of the users legs must be deter-
care facilities wheelchairs are propelled by attendants. mined to decide whether it is best to pull or to push with
The design of these wheelchairs requires special consid- the legs. When pushing, the user moves with her back in
eration. If the wheelchair is to be solely propelled by the direction of motion (16). The design of the chair is
attendants with no assistance from the rider, then there affected by whether the user pushes or pulls. Typically if
may be no need for larger drive wheels. Attendant-pro- the user pulls the chair along with her leg(s), then a wheel-
pelled wheelchair designs must consider the rider and the chair with the footrests removed is effective.
attendant as users. The rider must be transported safely If the person pushes the wheelchair, modifications
and comfortably. The attendant must be able to operate to the standard wheelchair design are required (17). Plac-
and easily maneuver safely and without any physical ing the casters at the back of the seat allows for easier to
strain. The design of the wheelchair must be such that the control of the wheelchair. For wheelchairs propelled
attendant can assist the rider with transfers and maneu- solely with the feet, the wheels at the front edge of the seat
ver around the chair in restricted spaces. The push han- do not swivel. The castors should lead the rear wheels in
dles should be such that the attendant experiences no the direction of travel. This helps to reduce the possibil-
undue stress to the hands, arms, and back. Consideration ity of the user flipping over when hitting an obstacle and
must be given for how the chair will be maneuvered over makes the chair directionally more stable. The wheelchair
obstacles. The primary consideration is that the wheel- should be set up so that the userwheelchair center of
chair has two users: the rider and the attendant. People gravity is well within the footprint of the wheelchair. This
who are elderly most commonly use attendant-propelled requires some consideration when positioning the large
chairs. Sometimes these chairs are called gerry chairs, wheels.
in reference to their geriatric users. This type of attendant- Common wheelchair frames center around tubular
propelled wheelchair is typically designed to minimize the construction (8,13,18,19). Manual wheelchairs are gen-
independent mobility of the rider. The rider is seated in a erally made out of lightweight tubing (e.g., aluminum, air-
large recliner type seat. The soft padding, reclined posi- craft steel). The tubing can either be welded together or
tion, small wheels, and large size make it impossible for bolted together using lugs. There are two basic frame
the rider to move the wheelchair and difficult for most types, folding and rigid; and three common frame styles,
riders to exit the wheelchair. Those are used in long-term the box frame, the cantilever frame, and the T or I frame.
care facilities to exercise control over their clients. There The box frame is so named because of its rectangu-
has been considerable discussion about the appropriate lar shape and the frame tubes that outline the edges of the
use of attendant-propelled chairs that significantly impair frame to form a box (8), which makes it very strong and
the riders mobility. very durable. A cantilever frame is so named because of
Childrens wheelchairs may also be attendant-pro- the relative position of the front and rear wheels. When
pelled. With childrens chairs, the philosophy is that the viewed from the side, the wheels appear to be connected
parents or an assistant can propel the chair until the child by only one tube, with the front wheels attached to a can-
is capable of doing so on her own or until the child is tilever beam fixed at the rear wheels. Both box and can-
ready for a powered wheelchair. Childrens wheelchairs tilever frames require cross bracing (i.e., tubes which con-
may also be either attendant-propelled or rider propelled. nect the two halves) to provide adequate strength and
Childrens chairs are semicustom fit for children without stiffness. The T or I frame uses a minimal number of
WHEELCHAIRS AND SEATING 637
tubes. The T frame uses a single tube, called an axle tube, on the seat upholstery. When the seat is lifted, the cross-
mounted to the rear wheels. Another tube is welded or members move upward, pulling the frame together. The
mounted to the axle tube, which extends forward to the users weight keeps the frame from folding when the
single front caster. The caster of a T frame is mounted at wheelchair frame is extended. Cross member folding
the end of the tube, which extends forward from the axle mechanisms are simple and easy to use. However, the
tube. The I frame has another tube mounted at a right wheelchair may collapse when tilted sideways, and the
angle to the front of the center tube, which holds a caster frame becomes taller when folded. Some chairs incorpo-
at either end. rate snaps or over-center locking mechanisms to reduce
Box frames, cantilever frames, T, and I frames stem the problem of frame folding while on a side slope. Fold-
from slightly different design philosophies. The box frame ing cross-brace type wheelchairs are available from every
provides great strength and rigidity. Thus, the wheels are major manual wheelchair manufacturer. There are sev-
mounted to a fairly rigid framework. If designed and con- eral variations of this basic design.
structed properly the frame only deflects minimally dur- The parallel-brace folding mechanism lets the frame
ing normal loading, and the seat cushion and the wheels fold sideways by having the frame cross member hinge
provide most of the suspension. Many manufacturers do forward. Each cross member is hinged in the center and
not triangulate (have tubes crisscrossing the frame) their at each end. When in the extended position, the center
box frame designs. This allows for some flexibility in the hinge is locked. The user releases the lock and pulls for-
frame. A few manufacturers have taken this concept one ward. The chair folds as the user pulls forward. The cross
step further by adding suspension elements to the frame. members can be locked with a pin or a cam mechanism.
Hinges are placed at the front of the seat and elastic ele- The advantages of this design are that the frame is rigid
ments are placed at the back of the seat. The elastic ele- in the extended position, yet it can be folded with the user
ments act to provide some suspension. The flexible ele- in it to permit the negotiation of some narrow entrances.
ments for the suspension can use either metal springs or However, parallel-brace mechanisms are difficult to oper-
polymer dampeners (elastomers). ate and to maintain.
The cantilever frame is based on a few basic princi- Forward-folding wheelchairs have some nice fea-
ples: a) the frame can act as suspension (i.e., there is some tures. Many ultralight wheelchairs incorporate forward
flexibility purposely built into the frame); b) there are folding backrests. The forward-folding wheelchair
fewer tubes and they are closer to the body, which may extends this concept. The design of a forward-folding
make the chair less conspicuous; and c) there are fewer wheelchair involves hinging the front end of the wheel-
parts and fewer welds which makes the frame easier to chair and the backrest. The backrest folds onto the seat
construct. and the front end folds under the seat. Forward-folding
The T and I frames are designed to make the frame wheelchairs can be made very compact if the rear wheels
as light and small as possible. In some cases, durability is are quick release. However, front-folding wheelchairs
sacrificed to provide a very lightweight and unobtrusive require more operations and latches for folding.
design. The T and I frames are popular among active
wheelchair users who enjoy a wheelchair suitable for both
daily use and sports. All of these basic frame types are THE WHEELCHAIRUSER INTERFACE
very functional and have their merits.
There are three commonly used folding mechanisms The wheelchairuser interface is the most critical design
for wheelchair frames: cross-brace, parallel-brace, and factor, and it is the least understood (20). The rider and
forward-folding. Most common folding wheelchairs use the wheelchair must act as one. The chair must be selected
variations of the cross-brace design. Each of these fold- so that the users potential is maximized; the chair should
ing mechanism designs has its relative advantages and the not limit the user. Experience tells us that there is a great
selection depends on the users preferences. deal of variability among individual preferences. The
Cross-brace folding wheelchairs are available with chair must become an extension of the users body, much
either single or double cross-brace mechanisms (8,19). like an orthosis. This requires careful matching of criti-
Double cross-brace designs add some stiffness to the cal chair dimensions to body dimensions, user ability, and
frame. A cross-brace folding mechanism consists of two intended use.
frame members connected in the middle and attached to To properly specify a wheelchair, it is important to
the bottom of a side frame member on one side of the understand the intentions and abilities of the user
chair and to the seat upholstery above the top side frame (4,8,2123). The best wheelchair will not be successful if
member on the opposite side. The cross members are rejected by the user. Therefore the needs, desires, and abil-
hinged at the bottom and pinned together in the middle. ities of the user must be ascertained and incorporated into
When viewed from the back of the frame, the cross mem- the wheelchair selection (2426). The selection of a wheel-
bers form an X. The chair is folded by pulling upwards chair depends on the intended use of the wheelchair. It is
638 REHABILITATION
no longer acceptable to stock a particular model of wheel- as little as 0 degrees and as much as 20 degrees. Seat depth
chair and to provide it to all new wheelchair users. Man- is determined from the length of the upper legs. Gener-
ufacturers who do not consider the intended uses of the ally no more than a 75-millimeter gap should exist
consumer will soon find themselves waiting for business between the front of the seat and the back of the knees
(27). The activities and the environment of the wheelchair when the person is in the wheelchair. This helps to ensure
and the user must be determined to assist in defining the a broad distribution of the trunk weight over the buttocks
geometry, components, and durability of the wheelchair. and upper legs, without placing undue pressure behind
The abilities of the user must be matched with the the knee. Some gap is required to allow the user some
intended use of the wheelchair (28). For some users, many freedom to adjust position. Seat width is determined from
of the desired tasks may be simply accomplished with the width of the persons hips, the intended use, and
existing technology, others will require custom products, whether the person prefers to use side guards (pieces of
and still others will not be able to achieve their goals with plastic, aluminum, or steel sheet placed between the seat
existing knowledge. The type of chair and how it is set up and rear wheels to prevent clothing from rubbing on the
depends on the interaction between the user and the rear wheels) or not. Generally, the wheelchair should be
intended use (person, place, task). The real abilities of the as narrow as possible. Thus, a chair about 1 inch (2.54
user, as related to the intended uses, must be assessed. cm) wider than the users hips is desirable. Side guards
Financial and physical resources are required to or armrests can help to keep clothing clean, and allow the
select and purchase wheelchairs, so the limits of the avail- user and chair to mesh better. Seat widths for adults typ-
able resources must be weighed against the abilities and ically range from 250 to 500 millimeters.
desires of the user. Resources are always limited, and new Backrest height, one of the most critical dimensions,
technology can be costly. The user should be provided depends on the users disability etiology, intended use, and
with the best possible product for the available resources. wheelchair skills. The comfort, stability and control of
Assistive technology is considered medical equipment and the wheelchair is influenced by backrest height. Gener-
thus caution must be taken to ensure the safety of all ally, the lower the mobility impairment, the lower the
potential users. desired backrest height. Commonly, backrest height is
The assistive technology clinician must remain cur- made to be adjustable so that the user can tune it to her
rent with respect to new product developments. There is liking. A lower backrest height permits greater freedom
no purpose in reinventing the wheel, and often an exist- of motion (e.g., leaning, turning) and is less restrictive
ing product can be adapted to meet the users needs. How- (i.e., does not interfere with arms when pushing), but pro-
ever, caution must be observed when modifying existing vides less support than a high backrest. Backrest heights
technology for applications not intended by the manufac- may vary from 200 to 500 millimeters. The backrest
turer, and a rehabilitation engineer should evaluate possi- should be made of a padded pliable material or, when
ble problems that might result from any modifications. made of rigid material, should not only be padded but
There are several critical seat dimensions to be con- mounted to conform to the users changing body position.
sidered during wheelchair selection: seat height, seat angle, It is sometimes desirable to taper the backrest to provide
seat depth, seat width, back height, and backrest angle (29). for comfortable support of a users well-developed upper
The seat base should be made of some stiff material to pro- torso. The backrest angle is often set so that the backrest
vide a rigid base of support for the cushion. Either a pli- is vertical with respect to a level floor.
able material (i.e., canvas, nylon) stretched tightly across The wheelbase and width affect handling and per-
the side frames or a rigid base (i.e., plastic, aluminum, com- formance. The wheelbase affects caster flutter, rolling
posite) works well. Seat height varies based on desired use. resistance, stability, controllability, and the obstacle per-
In general, in an upright sitting position with the hands rest- formance (17,30,31). With a longer wheelbase, the chair
ing on the top of the wheels, the elbow angle should be becomes more stable, but less controllable. Therefore, a
between 100 and 120 degrees for optimal mobility. long chair may be less apt to tip over, but the user may
Seat heights vary from about 300 to 600 millime- not be able to negotiate common terrain. Typically, active-
ters. The seat height depends on the total body length of use rehabilitation wheelchairs are very controllable and
the user. Users with longer lower leg lengths will require maneuverable and hence less stable. This is because users
higher seat heights to achieve sufficient clearance for the prefer control over the chair to stability. A longer wheel-
footrests. There is some flexibility when selecting seat base makes negotiating obstacles like curbs easier because
height, even for taller individuals, because most active the angle of ascent for the center of gravity is reduced
users prefer some seat angle. By tilting the seat towards (32,33). This is an important consideration. A short
the backrest, the user fits more securely into the chair, and wheelbase is more maneuverable, and can get the user
the chair becomes more responsive to the users body lan- closer to furnishings. However, a short wheelbase may
guage. Seat angle also gives the user some pelvic tilt, move more weight over the front wheels, which may
which provides greater trunk stability. Seat angle can be increase rolling resistance and reduce the speed of onset
WHEELCHAIRS AND SEATING 639
of caster flutter. This could also increase the risk of a for- these demands. Typically a wheelchair will be used all day
ward fall. Obviously, tradeoffs must be made. Typically, every day for all major life activities (i.e., activities of daily
the wheelbase of wheelchairs is between 250 to 500 mil- living, work, school, recreation, sport) over a period of 3
limeters. The width of the footprint of the wheelchair is to 5 years. The wheelchair must be designed to regularly
also an important design variable. withstand obstacles (rocks, curbs, bumps, dips, impacts)
The camber or angle at which the main wheels are encountered during a variety of activities. Making the
mounted to the body, is another variable. Camber has sev- frame too flexible causes it to absorb the energy of the rider
eral advantages: the footprint of the chair is widened, cre- and make it inefficient. Making the chair too stiff may
ating greater side-to-side stability; cambered wheels allow cause it to fracture from shock and reduces ride comfort.
quicker turning, cambered wheels help to protect the
hands by having the bottom of the wheels scuff against
Legrests
edges first, thus preventing them from hitting the area
where the hands are in contact with the pushrims, and the Most wheelchair users require support for their feet and
camber positions the pushrims more ergonomically for lower legs. This support is provided by footrests.
propulsion (it is more natural to push down and out) Footrests may be fixed, folding, or swing-away. The
(34,35). Camber angle can be either fixed or adjustable, footrest must provide sufficient support for the lower legs
depending on the frame design. The width of the chair and feet and hold the feet in the proper position to pre-
depends on the width of the frame and the camber angle. vent foot drop or other deformities. The feet must remain
Frame widths for adults range from 300 to 500 millime- on the footrests at all times during propulsion and there-
ters, and camber angles are usually between 0 and 15 fore some type of cradle is recommended. Some wheel-
degrees, with most between 7 and 12 degrees. Thus chairs (i.e., primarily those with swing-away footrests)
wheelchair widths vary from about 550 to 750 millime- use foot stirrups behind the heels of each foot. However,
ters. Generally, for daily use the chair should be as nar- for other wheelchairs it is best to use a continuous strap
row as possible without substantially diminishing the behind both feet, because the riders feet sometimes come
handling characteristics. The wheels should be offset over stirrups during active use, which is not possible with
enough from the seat to avoid rubbing against the cloth- a strap. The feet should not be permitted to be pinched,
ing or body. Narrow chairs are easier to maneuver in an trapped, scratched by the footrests during normal driving
environment made for walking. Some suspension can be activities or when transferring. The frame should be
gained through the use of wheel camber. By adding cam- selected and configured so that the feet sit firmly on the
ber to the rear wheels, the effective stiffness between the footrests, with shoes on, without lifting the upper legs
rolling surface and frame is reduced. from the seat cushion. Care must be taken that sufficient
ground clearance is maintained. The footrests are com-
monly placed between 25 and 50 millimeters from the
Ride Comfort and Durability
ground. Often, the footrests are the first part of the chair
Ride comfort is primarily a function of the frame, wheel to come in contact with an obstacle (i.e., door, wall,
and component stiffness, frame geometry, seat and cush- another chair); therefore, they must be durable.
ion design, and compliance (24,36). Ride comfort is an Rigid wheelchairs often use simple tubes as footrests
important issue because people must sit in the chair from across the front of the wheelchair. By using a tubular rigid
12 to 18 hours per day every day. Because some chairs are footrest, the wheelchair becomes stiffer and stronger.
used by active users, they may travel nearly ten miles per Rigid footrests are used during sports activities, and work
day over a variety of terrain (e.g., grass, carpet, gravel, well for people who are very active in their wheelchairs.
concrete, asphalt, etc.) The frame design must be durable Forward anti-tip rollers can be mounted to rigid footrests.
but should have sufficient flexibility to withstand drop- This is helpful for playing court sports and to reduce the
ping off curbs (up to 35 centimeters high), and also not risk of some forward tipping accidents. Rigid footrests
to break after several thousand miles of road vibration may be used on folding wheelchairs to prevent them from
(37). Most wheelchair suspension comes from the cush- folding. However, for people who like to use one wheel-
ion and the tires. Typically wheelchairs use pneumatic chair for both daily and recreational use, two sets of leg
tires and foam, gel, or air cushions. The frame geometry rests (e.g., one rigid and one split) may help to accomplish
can be used to reduce the effects of the impulse observed this. Folding wheelchairs often use footrests that fold up
when going off a curb, or to minimize road vibration. and leg rests that swing out of the way to ease in trans-
Durability is also a very important issue in wheelchair fers; most people prefer this type of footrest. Swing-away
design (10,13,18,19,36). Wheelchairs may used by leg rests are not as durable as rigid ones, and in some cases
demanding riders and/or receive little maintenance. The manufacturers design swing-away leg rests that bend on
same wheelchair is often used for work or school and impact to absorb the energy of the impact and possibly
recreation. Therefore the chair must be designed to meet prevent serious injury to the wheelchair rider. Elevating
640 REHABILITATION
leg rests can be used for people who can not maintain a properly, two people can lift the rider and wheelchair by
90-degree knee angle or who need their legs elevated for holding onto the armrests. In some cases, armrests are
venous return. Elevating leg rests make the wheelchair designed to pull out if any upward force is applied to
longer, and this has the effect of making the wheelchair them. They are not intended to be used for lifting.
less maneuverable by increasing the turning radius.
Wheel Locks
Armrests and Clothing Guards
Wheel locks act as parking brakes to lock the wheelchair
Armrests provide a form of support and are convenient during transfers to other seats, and when the rider wishes
handles when the rider leans to one side or the other. Arm- to remain in a particular spot. When locked, they keep the
rests are also helpful when attempting to reach higher wheelchair in place to allow the rider to push things from
places. Some people even use their armrests as tools to the chair, and they provide stability when desired. There
nudge items off high shelves. Armrests are commonly are a variety of wheel locks used to restrain wheelchairs
used to assist with pressure relief by performing a push- when transferring or parked. High lock brakes are most
up. By placing an arm on each armrest and pushing common, and they require the least dexterity to operate.
upward, some wheelchair users are capable of lifting their Extension levers can be added for people with limited
buttocks from the seat. This helps to improve blood flow reach or minimal strength. Some wheel locks are over-cen-
to the lower extremities and reduces the risk of develop- ter and are either engaged or disengaged. Other wheel
ing a pressure ulcer. There are three basic styles of arm- locks allow a selection of the braking force; these are some-
rest: wrap-around, full-length, and desk-length. Wrap- times called sweeper brakes, because the wheelchair can
around armrests mount at the back of the wheelchair, still be pushed in some positions, thus allowing the user
onto the frame below the backrest in most cases. The arm- to push and then sweep a broom to clean a floor. Wheel
rest comes up along the back of the backrest canes and locks are standard equipment on wheelchairs, and they are
wraps around to the front of the wheelchair (which is how simple to mount if the wheelchair does not come equipped
the wrap-around armrest gets its name). The major with locks from the manufacturer.
advantage of this design is that the armrest does not Wheel locks may be push-to-lock or pull-to-lock.
increase the width of the wheelchair, as with other types Most people prefer push-to-lock brakes because wheel
of armrest. Wrap-around armrests are popular among locks are more difficult to engage than to disengage. Rid-
active wheelchair users. The most significant drawback ers often find it easier to push with the palm than pull with
of this design is that the armrest does not serve as a side the fingers. High wheel locks are often mounted to the
guard to keep the riders clothing away from the wheels. upper tube of the wheelchairs side frame. Low wheel locks
Full-length and desk-length armrests are similar in are usually mounted to the lower tube of the wheelchairs
design, the main difference being the length of the arm- side frame. Low wheel locks require more mobility to oper-
rest. Full-length armrests provide support for nearly the ate. They also alleviate the common problem of hitting the
entire lower arm. They are popular on electric powered thumb, which plagues high-wheel lock users. This prob-
wheelchairs because they provide a convenient and func- lem can be addressed for high-wheel lock users by select-
tional location for a joystick or other input device. Full- ing retractable (i.e., scissors or butterfly) wheel locks. This
length armrests provide good support for the arms, but type of wheel lock helps to prevent jamming the thumbs
they make it difficult to get close to some tables and desks; and can accommodate a wide variety of camber angles.
hence the design of desk-length armrests. Both of these The major drawback of retractable wheel locks is that they
types of armrests include clothing guards to protect cloth- are more difficult to use than other types of wheel locks.
ing from the wheels. These types of armrests are mounted The wheel lock must be positioned properly with respect
to the side of the wheelchair and may add as much as 5 to the wheel to operate effectively. If the wheels are repo-
cm (2 inches) to the width of the wheelchair. sitioned, then the wheel locks must be repositioned. Tire
Armrests can be fixed or height adjustable. Height- pressure also affects the grip of the wheel locks.
adjustable armrests may move up and down to accom-
modate the length of the riders trunk and arms. Most
Hybrid Wheelchairs
armrests can be moved to provide clearance for transfer-
ring in and out of the wheelchair, and to allow a person Recently, electric-powered wheelchairs have been devel-
to lean over the sides of the wheelchair. Armrests are oped that are activated by applying a force to the pushrim
either removed or flipped back. Both styles commonly use of the wheel (21). These wheelchairs are called pushrim-
a latch, which is operated by the user. It is important to activated power-assist wheelchairs (PAPAWs). The pushrim
have latches on armrests because armrests form conve- force is regulated by sets of linear compression springs and
nient places for people to hold when attempting to pro- recorded by a simple potentiometer that senses the relative
vide assistance. If the armrests and latches are designed motion between the pushrim and hub. The potentiometer
WHEELCHAIRS AND SEATING 641
signals from both wheels are interfaced to a microcon- Consequently, for individuals with differing levels of
troller. The microcontroller algorithm coordinates control impairment or strength for their right versus left sides, the
of each wheels motor. The software simulates inertia (i.e., power assist can be adjusted accordingly. Choosing power
allows for a coast); compensates for discrepancies between levels also depends on whether the individual user is going
the two wheels (e.g., differences in friction); and provides to be indoor on a relatively smooth surface (e.g., tile, hard-
an automatic braking system activated when applying a wood flooring) or outdoor on more rugged terrain. Push-
reverse torque to the pushrims. The system is supplied with ing a button on the hub of each wheel completes the selec-
power by a single custom-designed nickel-cadmium (NiCd) tion of these levels. Each time the e.motion is turned on,
or nickel-metal hydride (NiMH) battery. The wheels have the power levels default to the previous selected level. In
quick-release axles. The entire unit has a mass of about 10 addition, when going down small slopes or hills, appli-
kg, and a range of about 12 km on a single charge. cation of slight pressure on the push rim engages the
In a study by Cooper et al., the Yamaha Motor Cor- motor controlled brakes and slows the descent of the
porations JWII PAPAW reduced the physiologic demand wheelchair. Each battery is a 24-volt, 1.9 amp, NiCd bat-
of wheelchair propulsion. This is beneficial for most tery and has a range of 4 to 6 miles on one charge (depend-
wheelchair users, particularly the aging or those in a tran- ing on the weight of occupant and the terrain). Each bat-
sitional status between manual and powered chairs. In tery pack weighs about 4.5 pounds and is mounted within
addition, it eases long-distance propulsion by facilitating the wheel hub. Consequently, each drive wheel adds about
less-strenuous maintenance of higher speed (Figure 46.1). 26 pounds to the existing wheelchair. The e.motion, like
ALBER GmbH has developed a pushrim-activated the JWII PAPAW, has the capability to benefit those indi-
power-assist wheelchair called the e.motion, which is cur- viduals who may be hesitant to transition to powered
rently awaiting FDA approval. Like the JWII PAPAW, the mobility and want to maintain physical control over their
e.motions power-assist wheelchair provides the flexibil- mobility needs for as long as possible.
ity of both power and manual wheelchair propulsion
when needed. The hubs of each wheel have gearless
brushless motors independently attached to them. Unlike ELECTRIC POWERED WHEELCHAIRS
the JWII PAPAW, this means that each wheel can be
adjusted independently. There are three levels of power Electric powered wheelchairs were first invented around
assist and variable sensitivity: Level 1 (25 percent power the turn of the century (38). The first U.S. patent describ-
assist), Level 2 (50 percent power assist), Level 3 (100 ing an electric powered wheelchair was approved around
percent power assist). 1940 (39,40). However, the very early designs were
impractical and did not receive significant attention.
There was also little demand for powered wheelchairs,
because powered wheelchairs are most beneficial to peo-
ple with severe disabilities, and the long-term prognosis
for people with severe disabilities was poor at that time.
The medical advances that occurred and were imple-
mented during the Second World War enabled more peo-
ple with severe disabilities to survive. Moreover, the
emphasis changed from acute treatment to long-term
rehabilitation. These changes created a demand for bet-
ter wheelchairs and, more specifically, for powered wheel-
chairs. The first practical electric powered wheelchairs
used starter motors and batteries from automobiles.
These early wheelchairs provided limited mobility for
some people with upper-extremity impairments. Relays
were soon used to provide greater control over electric
powered wheelchairs. Next, the transistor provided much
better control and allowed for the development of spe-
cialized control interfaces for the user. The number of
electric powered wheelchairs expanded during the 1960s
and 1970s. However, powered wheelchairs still suffered
from being bulky, inefficient, and unreliable. The 1980s
FIGURE 46.1 saw the implementation of microprocessors and metal-
oxide-semiconductor-field-effect-transistors (MOSFETs),
A pushrim-activated power-assist wheelchair (PAPAW). both of which helped to improve reliability. The micro-
642 REHABILITATION
processor provides improved control and greater ability Indoor/outdoor powered wheelchairs can be further
to match the characteristics of the chair to the abilities divided into categories by the design of the seating sys-
of the user and the MOSFETs improved efficiency. tem. The simplest form of seating system is a linear seat-
Because of their low-cost, extra MOSFETS have been ing system. A linear seating system refers to a planar seat
used to provide some redundancy. and back with fixed angles and orientations. For people
The 1990s brought about changes in frame design, who have low sitting tolerance or who need to change
as manufacturers began to develop frames designed posture to perform some activities of daily living, a reclin-
specifically for electric powered wheelchairs. The most ing seating system may be beneficial. A reclining seating
significant change was separating the seat from the frame. system allows the angle of the backrest to be changed
This is referred to as a powered wheelchair base design. (e.g., from upright to reclined). For people with very low
With the introduction of this concept, other innovations, sitting tolerance, severe spasticity, or homodynamic prob-
such as rear wheel suspension, were developed. lems, a tilt-in-space seating system may be appropriate.
Tilt-in-space systems allow the backrest, seat, and legrests
to tilt as a unit, without changing their orientation with
Differentiation of Powered Wheelchairs
respect to each other, but as a unit change with respect
Twenty years ago, there were very few differences to the wheelchairs chassis. Changing the users position
between the designs of powered wheelchairsmost were in space has other advantages besides assisting with pos-
simply iterations of manual wheelchair design. As the ture and sitting tolerance. Stand-up wheelchairs assist
powered wheelchair market grew and products devel- users in performing many activities of daily living. Stand-
oped, powered wheelchairs began to evolve. Eventually, up wheelchairs may have some physiologic benefits
consumers, clinicians, engineers, and manufacturers rec- because of the users upright posture, and have been
ognized the need for product differentiation. Moreover, reported to provide psychologic benefits to the user. Some
as more manufacturers of electric powered wheelchairs people cannot use stand-up wheelchairs because they do
emerged to address the specialized needs of the con- not have the range of motion or their bones may be too
sumer, manufacturers wanted to have their products be fragile. However, some of the benefits of a stand-up
distinguishable from those of other manufacturers wheelchair can be obtained by using a variable seat height
(41,42). wheelchair. The most common function of variable seat
Powered wheelchairs can be grouped into several height wheelchairs is to provide seat elevation, although
classes or categories. The most common groupings are some wheelchair models come with both seat elevation
based on the functions provided by the wheelchair and the and lowering. Lowering may extend to a few inches from
intended use. A convenient grouping by intended use is the floor, while rising can be as high as 3 feet (91.44 cm)
primarily indoor, both indoor/outdoor, and active from the floor to the base of the seat.
indoor/outdoor. Indoor wheelchairs have a small footprint
(i.e., area connecting the wheels) that allows them to be
Power Wheelchairs
maneuverable in confined spaces. However, they may not
have the stability or power to negotiate obstacles out- People with severe disabilities might need power wheel-
doors. Indoor/outdoor powered wheelchairs are used by chairs for mobility purposes. Individuals who have a
people who wish to have mobility in the home, school, moderate level of trunk control can use conventional
office, and community, but who stay on finished surfaces power wheelchairs. Conventional power wheelchairs con-
(e.g., sidewalks, driveways, flooring). Both indoor and tain a standard seat system. Alternative seating systems
indoor/outdoor wheelchairs conserve weight by using have been designed to provide customized seating in the
smaller batteries. This often results in reduced range. standard power wheelchair seat. Power wheelchairs are
Some wheelchair users want to drive over unstruc- distinguishable by their integrated frame and seat. Their
tured environments, travel long distances, and move fast. appearance is most similar to that of a conventional
Active indoor/outdoor wheelchairs may be best suited for wheelchair. Power wheelchairs may use a range of drive
these individuals. The active indoor/outdoor-use wheel- wheels from 8 to 20 inches (20.32 cm to 50.8 cm) in
chairs include those with suspension systems and the use diameter. Either belts or gearboxes may drive the drive
of a power base design is increasing. A power base sepa- wheels. All powered mobility systems are generically
rates the seating system from the main chassis of the lumped under the title powered wheelchair.
wheelchair. With power bases, the main chassis consists
of the motors, drive wheels, casters, controllers, batter-
Power Bases
ies, and frame. The seating system (e.g., seat, backrest,
armrests, legrests, footrests) is a separate integrated unit. Power bases are simply powered wheelchairs consisting
Often, seating systems from one manufacturer are used of a power drive system on a mobile platform. Power
on a power base from another manufacturer. bases are developed for those people with minimal trunk
WHEELCHAIRS AND SEATING 643
In-Wheel Motors
Another means of providing powered mobility to a man-
ual wheelchair is to use in-wheel motors. In-wheel motors
are wheel hubs made from a motor. The hub is much
larger than that of a standard manual wheelchair so that
the motor is of sufficient size to provide function power.
In-wheel motors use a battery pack, which attaches to the
frame. The most common means of attaching the battery
is to use a small bag slung under the wheelchair. In-wheel
motors are commonly made with quick-release axles to
simplify transport. Either a joystick or switches can be
used to control the motors (i.e., right and left side) to pro-
vide electric powered mobility for a manual wheelchair.
One must exercise some caution when converting a man-
ual wheelchair to an electric powered wheelchair. Man-
ual wheelchairs are not designed to accommodate the FIGURE 46.2
additional weight, and this may reduce the stability, dura-
bility, and ease of propulsion when in the manual mode. IBOT in balance function.
The speed possible with a powered unit is also likely to
be greater than that possible with a manual chair. More-
over, powered wheelchairs drive much differently than state-of-the-art software and hardware, the IBOT
manual wheelchairs. A skilled manual wheelchair rider responds to changes in terrain. The IBOT is controlled by
may pop the casters over small bumps without noticing. a set of central custom computers that help to maintain
However, when the chair is converted to being electrically stability and provide the user with control. The IBOT
powered, the caster may be damaged in short order. When incorporates a variety of sensors for attitude control,
used properly, with appropriate training and awareness speed control, self-diagnosis, and for changing opera-
of the trade-offs, power conversion units can provide tional functions. The IBOT programming uses a voting
valuable assistance. process with its redundant computers to determine the
actions of the device. The IBOT software also records the
operation of the device and maintains a fault log. A pio-
ROBOTIC WHEELED MOBILITY SYSTEMS neering feature of the IBOT is that the device contains
an internal modem that allows the device to communi-
Computer technology is increasingly used in assistive cate with the manufacturer or a service representative at
devices. This has led to the investigation of remote mon- a distance. This provides the potential to download fault
itoring and programming. The Independence 3000 IBOT logs to determine whether periodic maintenance is nec-
Transporter (IBOT) (Figure 46.2) acts as an electronically essary and possibly to upload software changes.
stabilized mobility device for people with disabilities Currently, people with a functional limitation involv-
(www.indetech.com). The IBOT uses three computers ing ambulation will often use a wheeled mobility device
and a number of sensors and actuators to provide a vari- to achieve their mobility goals. Those with either bilateral
ety of functions. The IBOT is a technologically advanced upper extremity, or unilateral upper and lower extremity
mobility device designed to overcome many of the limi- control and strength may use a manual wheelchair. Peo-
tations of currently available devices. By incorporating ple who lack the ability to propel a manual wheelchair,
644 REHABILITATION
who lack the endurance to propel a manual wheelchair, ing there are always two wheels on a step. Because of the
or have severe upper extremity pain may use a powered cluster design and the specialized controls incorporated
wheelchair or scooter. All current wheeled mobility devices in the IBOT, the device can be balanced above two wheels.
are limited in their functional use. Many environments are Balance function acts to raise the seat height for reaching
not accessible using a wheeled mobility device (e.g., stairs, high objects or for looking a standing person in the eyes.
high curbs, steep ramps, high objects, soft surfaces, uneven It also decreases the turning radius for confined spaces.
terrain). Performance trade-offs are numerous and must Surface restrictions for balance function include wet floors,
be considered for each individual. Independence 3000 ice, and snow. Driving on areas of soap, oil or grease spills
IBOT Transporter, as an electronically stabilizing device, should be avoided.
provides the opportunity to enhance mobility without
some of the traditional trade-offs.
The IBOT is intended for use as an advanced mobil- SELECTION OF ELECTRIC
ity system, which provides the user with five separate POWERED MOBILITY
functions for operation. The functions allow the user the
following capabilities: When selecting the most appropriate wheelchair, it is
important to involve a multidisciplinary team of support
Indoor mobility (e.g., driving on finished surfaces: personnel in the decision making process (4145). This
carpet, tile) assessment team will examine the needs of the wheelchair
Outdoor mobility (e.g., traverse uneven terrain, user, the environments encountered, the activities per-
steep inclines, grass, sand, gravel, curbs) formed in the chair, and the amount of training or instruc-
Stair climbing (e.g., independently, assisted) tion that might be required to use the chair safely and
Elevated height, with a reduced turning radius (e.g., effectively. The decision to accept the chair should ulti-
reaching the top shelf in a cabinet, having an eye- mately be made by the person spending the most time in
level conversation with someone standing, maneu- itthe wheelchair user. The family and caregiver should
vering in a confined space) also provide input, because they will be the next most
Ability to transport the device in some motor vehi- affected by the choice of chair.
cles without the use of a motorized lift. The assessment team usually consists of a variety of
rehabilitation professionals. A physician usually signs the
The IBOT can traverse nonuniform surfaces and prescription for the wheelchair. An occupational or phys-
steep inclines, climb curbs, and negotiate some stairs. The ical therapist conducts the evaluation process. The ther-
mobility system is based on a chair mounted through link- apist also works closely with a rehabilitation engineer or
ages to a wheeled base. The IBOT has four primary a professional certified in rehabilitation technology by
wheels, each controlled through its own electric motor, Rehabilitation Engineering and Assistive Technology
and two caster wheels. The two sets of drive wheels on Society of North America (RESNA) during the assessment
either side of the chair form a cluster. Each cluster may process. Together they have the skills to assess the needs
rotate about its central axis while the wheels may rotate of the user, and will take into account the environment
about their hubs. This provides the IBOT with more the chair will be used in and the activities that will be per-
degrees of freedom than an electric powered wheelchair. formed in the wheelchair.
The IBOT is operated via a hand-controlled joy- Those involved in the wheelchair selection process
stick and a user panel containing several switches. The should learn about the many powered wheelchairs avail-
controls are attached to an armrest. The IBOT can be oper- able on the market. Magazine articles and commercial
ated in five functions: standard function, four-wheel func- database sources such as ABLEDATA are good places to
tion, stair function, balance function, and remote function. begin your research. Wheelchair manufacturers can be
In standard function, the IBOT operates much like an elec- contacted directly or through the Internet for additional
tric powered wheelchair. When driving in four-wheel func- information.
tion, the clusters are allowed to rotate. This provides the The funding agency or third-party payer for the
freedom to negotiate obstacles or uneven terrain. The wheelchair should be contacted early to determine the
IBOT can also be used to negotiate stairs that have at least amount and nature of costs that will be covered. In addi-
a single railing. In stair function, the IBOT responds to tion to the purchase price, the chairs long-range mainte-
the reaction forces generated by the user against the stair nance costs should also be considered. This may be an
railing, or an assistant can initiate stair negotiation by important factor in making the final decision.
using the handles on the back of the device. Cluster rota- Once the model and pricing options have been inves-
tion is used to negotiate stairs. As the clusters rotate, with tigated, the needs of the individual should be assessed by
the wheels fixed at the central axis, a wheel from each clus- examining the environment and regular activities of the
ter transitions from one step to another. During stair climb- wheelchair user. Most peoples activities can be placed
WHEELCHAIRS AND SEATING 645
into one of three categories: daily living, vocational/edu- batteries (4648). The maximum overall weight of the chair
cational, and leisure. Activities of daily living include can be an important consideration if the wheelchair user
those activities done in the home environment, such as does not have access to mass transit, or a van equipped with
transferring in and out of bed, bathing and other toilet a lift or ramp. For all users, regardless of functional ability,
activities, and cooking. Access to a range of heights might skills need to be developed and tuned to enable the safe oper-
be important to reach objects in the home and other envi- ation of a particular wheelchair, with its given performance
ronments. Other daily living activities include running characteristics, in specific environments.
errands, performing chores, shopping for groceries, and With an understanding of the individuals need or
stopping by the post office. Vocational/educational activ- desire to perform different activities, the most important
ities can involve maneuvering within an office, outdoor factor in the wheelchair selection process is the user. An
construction site, agricultural setting, or school environ- inventory of the different kinds of activities important to
ment. Peoples professions are often highly specialized and the user will give an idea of that persons level of inde-
specific requirements may exist for mobility within a lab- pendence or dependence on others, as well as a sense of
oratory, operating room, courtroom, clean room, or their physical abilities. To verify this, some simple tasks
machine shop. Leisure activities, pursued in such places can be performed by the user to determine the lateral and
as community centers, restaurants, movie theaters, and frontal stability of trunk, hand and arm strength, and the
recreational environments, often place the most demands fine motor skills of the fingers.
on the wheelchair. Many powered wheelchair users brace some portion
The wheelchair model chosen should also be com- of their hand against the control box and use their hand
patible with the public and/or private transportation and arm coordination to operate the joystick. Gross arm
options available to the wheelchair user, such as a bus, car, function, in many cases, can be used to operate a joystick.
or van. If the wheelchair user plans to travel by air, only If the user does not have the hand function or coordina-
modular wheelchairs that operate on sealed gel batteries tion to operate a joystick input device, other options are
should be considered; chairs using other power sources are available. Other parts of the body, such as the chin or foot
not permitted on commercial planes. In other countries, can operate a modified joystick, or a sip-and-puff control
access to buses and trains can be an important requirement. can be used.
In all environments, the surface conditions will Be aware, however, that as the physical and func-
impose the greatest restrictions on the type of wheelchair tional abilities of the user decrease, the cognitive abili-
that is most appropriate. The regularity of the surface, ties required to operate an alternative control typically
its firmness, and stability are important in determining increases (49,50). A sense of an individuals cognitive
the tire size and wheel diameter. The performance of the function can be assessed through verbal and visual skills,
wheelchair is often dictated by the need to negotiate the rate and accuracy with which he can perform vari-
grades, as well as height transitions, such as thresholds ous tasks, spatial problem solving abilities, and his under-
and curbs. The clearance widths in the environment will standing of the bodys position in space. Visual skills,
determine the overall dimensions of the wheelchair. The including sight accuracy and depth perception, are also
climate the chair will be operated in, and the need to be important for safe operation of a powered wheelchair.
able to operate in snow, rain, changing humidity and tem- Programmable wheelchair controllers allow reduction of
perature levels, and other weather conditions, are impor- the maximum velocity, acceleration, and deceleration
tant considerations. rates of the wheelchair (5154). To assist persons with
Overall, a variety of performance characteristics of more severe cognitive or visual limitations, technologies
the wheelchair should be considered with regard to the are being developed that enable the wheelchair to follow
environment and activities that the individual is trying walls, navigate through doorways, and stop when other
to perform. The maximum speed of the wheelchair is objects are contacted.
important when using the wheelchair to travel longer dis- It is also important to assess for spasticity in the
tances, such as across campus or across town. The obsta- hands or arms. For persons with spasticity, particularly
cle climbing ability of the chair will help determine the tremor in the hand, modern controllers have filters that
riders access to environments that do not meet ADA can be adjusted to give smooth wheelchair control (55).
Accessibility Guidelines. Static and dynamic stability A magic arm or similar positioning technology will enable
characteristics (the tippiness of the chair) will deter- positioning the joystick in numerous places to optimize
mine how safely the rider will be able to negotiate many the users joystick operating function. Finally, once the
of these nonideal environments. appropriate choice and technologies in powered mobility
The overall dimensions of the chair will determine how have been made, significant time should be spent opti-
well the wheelchair user can maneuver in tight quarters. The mizing the seating set up to maximize the riders function.
range of the wheelchair will indicate how long and far the Before individuals ride away in their new powered
user can go in a given day without having to recharge the mobility, they should go through a wheelchair-training
646 REHABILITATION
program (56). This is particularly important for younger indicate the position of the joystick. This type of joystick
children. Wheelchair users can first practice basic maneu- is called a resistive joystick. As the joystick is moved left,
vers in controlled environments that meet ADA Accessi- the electrical resistance is increased, and as the joystick
bility Guidelines. They should practice negotiating uneven is moved to the right, the electrical resistance is decreased.
surfaces, slope transitions (i.e., level to sloped surfaces) in Another independent change in resistance is used to indi-
both the uphill and downhill directions, and maneuver- cate the position of the joystick fore and aft. A change in
ing through tight environments. Once these skills are mas- inductance can also be used to signal the position of the
tered, they should gradually tackle more challenging envi- joystick. Two independent inductors (i.e., coils) vary with
ronments, such as steep grades and step transitions that the position of the inductive joystick in the same manner
exceed ADA Accessibility Guidelines (57). The rider that the resistance does with resistive type. Digital joy-
should always practice with an appropriate lap belt and sticks based on a new standard called M3S are being
chest support in place, as well as spotters standing by to introduced, especially in the European market. These joy-
assist if needed. The wheelchair user should also be given sticks may offer improved immunity to interference from
an opportunity to experience the limits of the technol- sources such as police radios, cellular phones, ham radios,
ogy he is using (5859). By setting up and practicing in and even the electric motors of the wheelchair itself.
safe environments, users can experience the limits of the
lateral stability, as well as the front and rear stability of
Sip-and-Puff
their chair, so that they have a better understanding of
its performance limits. Those with tetraplegia, who do not have functional use
of their arms or hands, may use sip-and-puff switches. A
sip-and-puff access device consists of a replaceable straw
ACCESS DEVICES located near the mouth. The wheelchair is controlled by
a sequence of pulling and pushing air through the straw
The primary function of an access device is the control with the mouth. These systems can be set up in a variety
of the powered mobility system (6061). Secondary appli- of configurations. Generally, the user will sip a specific
cations are for use with environmental control systems number of times to indicate a direction, and puff to con-
and computer access. By using the same control interface firm the choice and activate the movement of the wheel-
for both the powered wheelchair and the secondary sys- chair. It is common for an auxiliary display to be used
tems, the seating position and control selection can be with sip-and-puff to provide feedback to the user. This
optimized for multiple purposes. A critical consideration display is often an LCD flat panel, which is capable of dis-
when selecting or designing a user interface is that the playing about 20 characters.
ability of the user to accurately control the interface is
heavily dependent upon the stability of the user within
Switches
the wheelchair. Often, custom seating and postural sup-
port systems are required for a user interface to be truly An array of switches can be used for the directional input
effective. The placement of the user interface is also crit- of a wheelchair. The control scheme is similar to that of
ical to its efficacy as a functional access device. a switched joystick, except a combination of switch acti-
vations is rarely used for directional input. Switches are
indicated for individuals who have good control over an
Joysticks
anatomic site not usually used to control a wheelchair. An
A joystick is the most common access device for powered individual with a disability might, for example, have bet-
wheelchair systems. When a powered wheelchair is ter motor control over a foot rather than a hand. An array
ordered and nothing is specified, a joystick is almost of large switches mounted to the footrest could then be
always supplied by the powered wheelchair manufacturer. used as a direction input. Switches are also a primary
Joysticks can either be switched or proportional. Switched means for computer access. Often one switch is used to
joysticks respond to discrete positions of the joystick. Usu- toggle between the functions of computer access and pow-
ally four switches are implemented. There are eight dis- ered wheelchair control. In computer access mode, the
crete states, with four positions being defined by the acti- switches are used with any number of coding schemes.
vation of a single switch and four positions being defined These coding schemes include International Morse
by the activation of two switches together. Proportional Code, automatic scanning, step scanning, inverse scan-
joysticks get their name because the control output sent ning, array scanning, and multilevel scanning. Morse code
to the wheelchair from the joystick is in proportion to how has the advantage of only requiring one switch (although
far the joystick is pushed from the center position. two switches can be used), but generally places the high-
The control output can be one of several electrical est cognitive demands on the user of all of the different
signals. A change in electrical resistance can be used to types of coding schemes. The common feature of the scan-
WHEELCHAIRS AND SEATING 647
ning methods is that the selection choices proceed until (62,63). Body measurements are typically made with the
the user chooses the correct function or types the correct consumer in the seated position. However, some thera-
character. The act of selection can be through a time delay pists prefer to make body measurements on a mat-table.
or the activation of a switch. In automatic scanning, the Both methods work well, provided the therapist is famil-
choices are presented to the user on a computer screen iar with the measurements to be made and has adequate
or LCD panel with a preset time delay between each experience. Body measurements are best made with a steel
choice. When the desired choice is presented, the user or nylon tape measure designed specifically for making
must activate a switch before the next choice is shown. body measurements. Such a tape measure is called an
In step scanning, the user uses the switch to move from anthropometric tape measure, and it incorporates a spring
one choice to the next with each press of the switch. A attached to the end to limit the amount of force applied
delay on any one choice indicates acceptance of that func- to soft tissue (64). This helps reduce the error caused by
tion or character. Inverse scanning is the reverse opera- one person applying a small amount of force and caus-
tion of automatic. The switch is held down until the ing a small distortion of soft tissue, and another person
desired choice is reached, and then released to finalize the applying a larger force and causing a larger distortion of
selection. All three of the scanning methods can be fur- soft tissue, thus resulting in different measurements for
ther modified to use more than one switch. One such the same consumer. Calipers can also be used to get more
modification would be to use one switch to step through accurate breadth and depth measurements. However,
the choices and a second switch to accept the choice. calipers are primarily used by specialized seating clinics
Array scanning uses four switches to guide a cursor and research laboratories.
through an xy grid of choices; a fifth switch with a time Probably the most obvious body measurements are
delay is used for the selection process. The switch arrange- the consumers height and weight. The consumers weight
ment in array scanning is highly applicable for both pow- is critical to obtaining a wheelchair that is suitably strong
ered wheelchair control and computer access, because of to accommodate them. For example, most wheelchairs
the similarity in skills that are necessary to master each task. are tested with 100-kilogram (220 pound) test dummies.
Multilevel scanning is similar to the above methods, The test dummies are made of wood or aluminum and
but after the selection of a choice, a new menu is pre- are stiffer than a real person, but nevertheless the wheel-
sented. The final function or character is entered from this chair needs to be rated for the end users weight. Many
new menu. The number of levels can also be increased. manufacturers claim in their owners and service manu-
With all of the possibilities for computer access and the als that their wheelchairs are rated for 250 pounds. When
ability to use unusual access sites, switches provide for selecting a wheelchair for someone who weighs more than
one of the most versatile powered wheelchair control 220 pounds, it is best to inform the wheelchair manu-
inputs. The disadvantage is the same as for a switched joy- facturer. The wheelchair provider should also verify that
stick: there is no proportional control of the wheelchairs the wheelchair has been tested for a weight greater than
speed with switched control. the actual weight of the client.
The height of the wheelchair user provides infor-
mation about the persons size and can be used to check
Ultrasonic and Infrared
the final wheelchair measurements. For example, the sum
Many of the input devices described in this section can of the sitting height, sitting depth, and lower leg length
be used for more than the control of powered wheel- should be close to the persons supine height (65,66).
chairs. Two common additional uses are environmental Height and weight also provide information about a per-
control and computer access. In some cases, the computer sons stature. This is helpful for people building the wheel-
is mounted to the wheelchair, but often it is at a fixed site. chair, who may never see the client, and for future use
In this case, there must be a method for the linkage of by therapists who can use stature as a quick measure of
the computer to the control device. Older systems, which whether the clients status has changed substantially since
were used primarily for environmental control, used the previous wheelchair was selected.
ultrasonic signals to transmit information. Newer designs The measurements required to select the proper
are based on infrared for both environmental control and wheelchair for an individual are illustrated in Figure 46.3.
computer access. Additional measurements and definitions are used when
specialized seating and postural support systems are
required. The critical body width measurements are
WHEELCHAIR AND SEATING shoulder width, chest width, waist width, and pelvic
MEASUREMENT width. The shoulder width is important when the wheel-
chair user requires shoulder supports or a shoulder har-
For many people, a few simple measurements can be used ness. The chest width is important for determining the
to determine the proper dimensions for a wheelchair width of the backrest and backrest supports. The more
648 REHABILITATION
ing the sagittal plane, the edges from three seat position
planes are used as references for defining an individuals
wheelchair seating. The back plane is a vertical plane that
contacts the posterior-most position of the individuals
back. Typically, the person being measured is placed in
as close to as possible vertical torso posture. When the
individual being measured can assume a normal erect sit-
ting posture, then the back plane will contact the spine
within the thoracic region (67). The seat plane is a hori-
zontal plane that intersects the back plane and contacts
the inferior-most portion of the individuals seated surface
during seated weight bearing. Typically, the point of con-
tact for the seat plane will be the ischial tuberosities. The
FIGURE 46.3
foot plane is also a horizontal plane, which is defined by
the point of contact with the base of the feet. It is impor-
Critical body measurements for wheelchair users. tant to remember that the back plane, seat plane, and foot
plane are imaginary planes used as references. The actual
wheelchair seat may vary substantially from these planes
severely impaired an individual is, the higher the backrest depending on the functional anatomy of the individual.
must be to provide adequate sitting balance. However, if The overall height of the individual in the seated
the backrest is too narrow, it will cause discomfort. If the position is the distance between the foot plane and the top
backrest is too wide it can interfere with the individuals of the persons head. This measurement is important
ability to propel the wheelchair. The backrest width is when considering a persons interaction with his/her envi-
more critical for manual wheelchair users than for power ronment; for example, the seated overall height of an indi-
wheelchair users. The backrest width should be about 2 vidual is going to define whether the roof of a van will
centimeters wider than chest width at the highest point need to be elevated, and by how much. The overall height
on the backrest. also defines how low a person can sit properly in a wheel-
The waist width is important to accommodate arm- chair without having his/her feet contact the ground. The
rests, side guards, and the wheels on some wheelchairs. sitting height is defined as the distance between the seat
Typically, armrests and side guards (i.e., devices used to plane and the top of the head. The sitting height combined
keep clothing from touching the wheels) are at waist width. with the seat height provides information about how high
The distance between the armrests and waist width should a person sits. This helps to define the minimum and max-
be between 5 and 10 centimeters. The dimension becomes imum reach. The sitting depth is the distance between the
more critical for manual wheelchair users. If the armrests back plane and the popliteal fossa (hamstring tendons).
are too far apart, the wheelchair user will have to place the The sitting depth is used to determine the depth of the
arms in forward flexion to reach the pushrims. (This often wheelchair seat. The lower leg length is measured from
described as chicken winging, because the propulsion the popliteal fossa to the foot plane. The lower leg length
stroke resembles a person imitating a chicken.) is used to select and adjust the leg rest length.
The pelvic width is used to determine the seat width Wheelchair footrests come in various sizes, depend-
of the wheelchair. The seat width of the wheelchair should ing on the size of the individuals foot and the flexibility
be as close as possible to the pelvic width for manual of the foot and ankle. The foot length is the maximum
wheelchairs. This allows the wheelchair user to reach the length of the foot with shoe. The overall depth for a seated
pushrims most easily, and places the arm in a position of person is defined as the distance between the tips of the
greater leverage. Active wheelchair users prefer the seat toes to the back plane. This distance is important when
width to be about 1 centimeter greater than the pelvic determining the depth of a tabletop or desktop required
width. However, other wheelchair users prefer greater to accommodate a wheelchair user. The overall depth also
space to accommodate a coat, sweater, or to allow some affects the maneuverability of the wheelchair user. Some
room to adjust seating position. In manual wheelchairs, people require more support than can be provided by a
more than about 3 centimeters difference between seat simple linear seating system. Headrests are often used to
width and pelvic width can have a negative impact on provide support for the head and neck for people having
stroke biomechanics. Power wheelchair seat width mea- higher levels of impairment. The headrest height is related
surements are also important to maintaining the postural to the seated height and acromion height. The acromion
support needed to control the wheelchair. height is measured from each acromion to the seat plane.
Several important seated measurements are made The measurement to each acromion provides information
with respect to the sagittal plane (side view). When view- about postural asymmetry. The distance between the
WHEELCHAIRS AND SEATING 649
occipital protuberance and the back plane is used to spec- or contouring, which requires clinical skills to make foam
ify the range of the headrest fore and aft adjustment work most effectively for pressure relief.
required. Standard polyurethane or urethane foams are made
Experienced clinicians can make the required mea- from plastic, which has been processed to incorporate
surements with the individual either seated or lying on a large numbers of air bubbles. These air bubbles within
mat. Most therapists prefer to make seating measure- the foam cause a cross-link type structure. When foam is
ments with the individual in the seated position because loaded, it behaves like a spring for small forces and dis-
it accounts for the effects of gravity on posture. It is also placements. As greater load and displacement are applied,
important to determine the individuals range of motion the cross-links begin to buckle and the foam begins to
and postural asymmetries. behave nonlinearly. If further load is applied, the cross-
links fully buckle and the foam begins to behave like a
compressible solid. Little is understood about how these
CUSHIONS properties are best manipulated to form a pressure relief
cushion. For this reason people use foams of various den-
Two basic types of seating systems are used with wheel- sities and stiffness. The cross-links of foam change the
chairs: linear systems and contoured systems. Linear seat- forcedisplacement relationship as force is increased. This
ing systems are planar in that the seat and back surfaces nonlinear behavior of foam has not been fully exploited
are flat and only conform to the weight of the user. For in cushion design.
many wheelchair users, a simple linear seating system or The density of foam relates to the amount of air that
a standard contour is very effective (6870). Foam, vis- is trapped in the plastic matrix. Higher-density foams tend
coelastic fluid, dry flotation (air) cushions, and honeycomb to be heavier because they contain more plastic. As den-
are the most common cushion materials (Figure 46.4). sity increases, stiffness also increases. However, stiffness
and density are not the same properties. Stiffness refers
to the slope of the forcedisplacement curve during the
Linear Foam Cushions
linear region (i.e., before the cross-links begin to buckle).
Various densities and types (polyurethane, urethane, T- Foam is also characterized as open cell or closed cell.
foam, Sun-Mate) of foam are commonly used in linear Closed-cell foam is made so that it is less permeable to
seating systems. Foam has been shown to offer the low- fluid.
est maximum pressure over the seating surface when the Foam cushions can be made by contouring a single
appropriate densities and contours are used (7173). piece of foam or by combining foams of different stiff-
However, foam has a tendency to deteriorate at an unde- ness. There are also specialty foams, such as T-foam or
sirable rate (74). A very significant advantage of foam Sun-Mate foam. These specialty foams are made to have
over other materials is that it is inexpensive. The raw gellike properties while maintaining some desirable prop-
material for a foam cushion costs only a few dollars. The erties of foam. T-foam and Sun-Mate foam have negli-
added cost to make a cushion comes from the sculpting gible linear regions. They compress like standard foam,
although more slowly. However, they have memory that
retards them from returning to their resting shape. Hence,
when a person sits on them, they tend to come into equi-
librium with the sitting tissue. The foam then responds
slowly to changes in position. T-foam or Sun-Mate foam
can be used to give some additional stability to standard
foam cushions. Foam is an excellent material for cush-
ions, but it has low durability and loses its pressure-reliev-
ing properties rapidly with use. Most foam cushions need
to be replaced annually.
the seating area. However, the pressure would be dis- accompanied by lower viscosity; lower viscosity provides
tributed indiscriminately. To better control interface pres- greater shock-absorbing properties. Viscoelastic fluid
sure, many air flotation cushions use multiple compart- cushions do not change their properties significantly with
ments, contouring, and baffling. Multiple compartments changes in atmospheric pressure. Users of viscoelastic
allow interface pressure to be regulated from one com- fluid cushions must check with manufacturers about pos-
partment to another. The pressure within a particular cell sible allergic reactions from contact with the viscoelastic
(balloon) will be constant. Shaping the balloon may reg- fluid. Viscoelastic fluid cushions may develop leaks with
ulate the pressure at the skin interface. Balloon shape may time and use.
lower pressure within the tissue by altering the way in
which the body tissue deforms. Some cushions use foam
Honeycomb
in combination with air bladders to control the pressure
within the cushion and, in turn, the interface pressure. Honeycomb materials are made to exploit the linear prop-
The combination of foam and air flotation assumes that erties of some polymers (i.e., types of plastics). When
the compression of the foam within the bladder will reg- these polymers are molded into a honeycomb, they
ulate pressure to direct it away from low-tolerance behave like a collection of springs (similar to the linear
regions and towards regions of higher-pressure tolerance. behavior of foam). Honeycomb cushion materials have
A problem with air cushions is that they may lose an advantage over foam in that the manufacturer can
their air and simultaneously their ability to provide pres- carefully control the properties of the honeycomb mate-
sure relief. Some air cushions require considerable main- rial and the resulting cushion. Honeycomb cushions can
tenance. In some cases, users should carry repair kits with be contoured like foam. However, honeycomb cushions
them when traveling. Air cushions will also change their are typically contoured by the manufacturer to some stan-
properties with changes in altitude. The most common dard shape. Honeycomb cushions can be made to greatly
instance is when flying on an airplane. The air pressure simplify cleaning, which makes them more attractive than
within the cushion can change considerably from take-off foam in institutions that may have multiple people share
to cruising altitude. a single cushion. (Air and gel flotation cushions can also
be made easy to clean.)
A drawback of honeycomb cushions is that they are
Viscoelastic Fluid Cushions
considerably more expensive than foam cushions, and
Viscoelastic fluid cushions use an electrolyte viscoelastic they do not come in a wide variety of stiffness, as foam
fluid in a closed plastic or latex pocket. The viscoelastic does. Honeycomb materials may have a larger linear
fluid conforms to the body and provides a nearly even range than foam. Honeycomb materials may be more
pressure distribution. Viscosity is used to define a fluids durable than foam as well.
resistance to flow. Because viscoelastic fluid behaves like
a fluid, it will work much like air to equalize pressure over
the entire seating surface, because it moves to come to a SEATING BASES
constant pressure within its container. Baffles can be used
to control the flow of the viscoelastic fluid, just as in air Seat bases may either be rigid or a cloth sling (75,76). In
cushions. The pressure distribution can be altered by either case the seat should be stiff and provide a stable
using a stiff base (e.g., plastic or foam) to provide some base for the cushion and user. Sling seats (seat and back-
contouring. The high viscosity of the viscoelastic fluid pre- rests) are constructed of synthetic materials with high ten-
vents it from moving rapidly within its container. This sile strengths. Nylon, cotton canvas, Kevlar, or similar
property allows the viscoelastic fluid cushion to provide materials are used to provide the structural component of
a more stable seating surface than an air cushion. How- the seat or backrest sling. In many instances the structural
ever, the high viscosity of a viscoelastic fluid cushion also material may be enclosed in a vinyl, Naugahyde, or
limits its shock-absorbing properties. other water-resistant case, which acts as the interface to
Viscoelastic fluid cushions often suffer from exces- the user. Vinyl and Naugahyde deform substantially
sive weight for many users, and their properties are sub- under load and are not appropriate as seating structural
ject to changes in temperature. Some types of viscoelas- materials. The exterior shell is often vacuum formed
tic fluids may actually freeze during extremely cold under heat to establish a bond between the shell mater-
weather, and if the cushion becomes cold, the users body ial and the structural material. Seats and backrests may
may need to exert considerable energy to heat the cush- be attached to the wheelchair frame in a variety of ways.
ion. Some cushion manufacturers have altered the weight One of the more common ways is to sew a pocket into
of viscoelastic fluid cushions by making lightweight vis- each lateral edge of the seat base and backrest upholstery.
coelastic fluids. Lightweight viscoelastic fluids may act Then a metal (steel or aluminum) slat is placed into the
more like whipped cream. Light weight is typically upholstery pocket to attach the base and backrest uphol-
WHEELCHAIRS AND SEATING 651
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47 Spinal Orthoses
he use of spinal orthotics has been These devices, along with supplemental stabilization
FIGURE 47.2
FIGURE 47.5
The Halo.
THORACOLUMABAR IMMOBILIZATION
compared to its CASH counterpart. The Jewett is most ulcers. When it is ordered, the nursing staff should be
effective in preventing flexion and extension between T6 instructed by the orthotist in the donning and doffing pro-
and L1. The CASH, clinically viewed as slightly less effec- cedure. The placement should be checked by the nursing
tive than the Jewett, is commonly used for low back pain staff every 4 hours to ensure that migration has not
as well and is tolerated better by most patients compared occurred.
to the Jewett. The TLSO body jacket is available in temporary and
The Taylor TLSO purports to provide anterior flex- off-the-shelf versions as well (Figure 47.9). Although the
ion control of the thoracic and lumbar region without the off-the-shelf versions can be fit quickly and at a reduced
weight, bulk, and donning challenges of the TLSO body cost, the attendant complications from poor fit are
jacket. However, its effectiveness at motion control com- increased significantly compared to the custom molded
pared to the Jewett or TLSO body jacket is controversial versions. In cases of body asymmetries, off-the-shelf body
at best. The TLSO Milwaukee, used historically for sco- jackets are an inappropriate choice and do not accom-
liosis, can in theory be an effective means of thoracic modate to all body shapes. The two basic designs are the
immobilization, but patient acceptance is so dismal that bivalve and the one piece with chest plate. An advantage
it is not a viable option. of the bivalve is that it can be applied while the patient is
The TLSO body jacket shown in Figure 47.8 is a cus- reclined in bed. Typically, the patient rolls over to one side
tom-molded orthosis for maximum balance of comfort and the posterior section of the TLSO is positioned. Then
and efficiency in motion control of the thoracic region. the patient is rolled over to the prone position. At this
Of the noninvasive approaches to controlling thoracic point the anterior section is keyed to the posterior section.
motion, it may well be the most effective. The treatment Velcro straps secure the orthosis firmly in place. Donning
team should keep in mind that limiting thoracic motion the one-piece TLSO body jacket is slightly more awkward
may increase lumbar motion. Indications for the custom- and may generate pain in some patients. The bivalve
molded TLSO body jacket include postsurgical thoracic design is easier to don and may provide increased control.
protection and chronic instability, for which it is an excel- The one-piece TLSO with posterior opening might com-
lent treatment choice. However, the treatment team promise control in the sensitive spinal region. If the open-
should be aware that it is difficult to don and doff and ing is anterior, some efficiency of abdominal containment
because it may migrate on the body, requires frequent may be affected and circumferential compression may be
positional adjustment. Attention to the trim lines is crit- less effective.
ical and requires an experienced orthotist. Generally the The TLSO (wheelchair suspension) is a unique con-
TLSO body jacket is considered somewhat awkward and cept developed at Newington Childrens Hospital, New-
bulky. It will migrate proximally as the bed-bound patient ington, Connecticut (Figure 47.10), which partially sus-
raises and lowers the head of the bed. This may diminish pends the patient and is helpful in the treatment of sacral
comfort levels and increase the risk of orthotic-related
FIGURE 47.9
FIGURE 47.8
The TLSO body jacket is available in temporary and off-the-
The TLSO body jacket. shelf versions.
SPINAL ORTHOSES 661
6. Hartman JT, Palumbo F, Hill BJ. Cineradiography of the braced 9. Brown C, Chow G. Orthoses for spinal trauma and postopera-
normal cervical spine Clinical Orthopeadics and Related Research tive care. Atlas of Orthoses and Assistive Devices, American Acad-
1975; 109:97102. emy of Orthopedic Surgeons 3rd ed. St Louis: Mosby 1997,
7. Lunsford. The effectiveness of four contemporary cervical 251258.
orthoses in restricting cervical motion. Prosthetics Orthotics 1994; 10. Garfin SR. Complications in the use of the Halo fixation device.
9399. J Bone Joint Surg 1986; 68:320325.
8. Lavernia C, Botte M, Garfin SR. Cervical orthoses. Spinal 11. Norton, Brown. The immobilizing efficiency of back braces the
Orthoses for Traumatic and Degenerative Disease, Chapter 30. effect on the posture and motion of the lumbosacral spine. Bone
Philadelphia: W.B. Saunders, 1998, 11971223. Joint Surg 1957; 39-A.1:111139.
48 Upper Limb Orthoses
n tetraplegia, the function of the Often a few of these goals are achieved with one
663
664 REHABILITATION
hand structures (2,9). Dynamic orthoses, such as the of the earliest of these was the flexor-hinge hand splint.
wrist-driven, wristhand orthosis (also known as the Originally designed for the polio patient, this orthosis
wrist-driven, flexor-hinge splint or tenodesis splint) (Fig- transmitted the force generated by active wrist extension
ure 48.1) and the ratchet wristhand orthosis (Figure via a mechanical linkage to paralyzed index and long fin-
48.2), provide prehension to increase independence in gers, enabling finger closure against the thumb (10). The
activities of daily living (ADLs). design of this orthosis evolved into what today is known
as the wrist-driven, wristhand orthosis (WDWHO)for-
merly called flexor-hinge splint or tenodesis splint). This
HISTORIC BACKGROUND orthosis offered prehension capability that had obvious
application for the SCI patient. Individuals with C67
The designs for upper limb orthoses were often originally lesions, with strong wrist extensors and paralyzed finger
developed for patients with conditions other than SCI. One flexors, could utilize the WDWHO to improve function.
The orthosis harnesses wrist extensor power and utilizes
the power of wrist extension to flex the fingers at the
A metacarpophalangeal joints against a stable thumb.
Some patients lacked sufficient wrist extensor
strength to utilize the WDWHO. The development of
external powered designs led to a system that utilized a
CO2-powered artificial muscle to provide proportion-
ally controlled prehension. This system was designed in
1957 at Rancho Los Amigos Hospital in collaboration
with Dr. Joseph McKibben, a physicist whose daughter
contracted polio. Dubbed the McKibben muscle, it fea-
tured a rubber bladder, which was covered with a woven
fabric. This unit was attached to the side of the WHO.
B A
A. Person with C6 tetraplegia using wrist-driven WHO to A. Opening is achieved by activating a button. B. Person with
paint; B. person with C6 tetraplegia using wrist-driven WHO C5 tetraplegia may benefit from the Ratchet WHO for Pre-
to apply makeup. hension.
UPPER LIMB ORTHOSES 665
When pressurized with CO2, the bladder would expand Data on long-term use of upper limb orthoses (13)
against the woven fabric and shorten in length. This in has contributed to the decreased number of prescriptions
turn operated a linkage bar, which propelled the fingers for these items. An additional factor is simply the phi-
into flexion against the stable thumb. A two-way valve, losophy of providing patients with the smallest amount
operated by shoulder shrugging, released the pressure to of equipment possible. With emphasis on patient-centered
allow finger extension (11). care, consumer feedback has become central in shaping
By the mid-1960s, smaller, more-powerful electric trends. Consumers with SCI have been saying all along
motors, brought a shift away from CO2 as a source of that equipment and orthoses make them look more dis-
external power for upper limb orthoses. Electrical exter- abled and often make their life more cumbersome.
nal power was coupled to the WDWHO through the use The efficacy of static splints in tetraplegia has long
of cables and battery-powered motors. Again, there were been questioned and, with a lack of evidence to support
obvious potential benefits to the patient with partial their use, it is hard to justify the ordering of such splints
upper limb paralysis. and orthoses (9,13).
Encouraged by results of the work with the Additionally, cost-containment efforts have resulted
WDWHO, orthotists and biomedical engineers at Ran- in not only decreased LOS, but also a reduction in the
cho Los Amigos Hospital undertook a much more ambi- numbers and types of equipment that patients receive.
tious projecta battery-powered, multidimensional Selection criteria have become more restrictive. Some
upper extremity orthosis that would attempt to duplicate institutions provide equipment only on an outpatient
all major motions of the arm and hand. Designed using basis. Others utilize patient contracts, in which patients
anthropometric measurements, this tongue-switch con- commit to using equipment for specified time periods or
trolled device offered the opportunity for high-level for specific tasks (12). Other cost-cutting measures
tetraplegic patients to achieve greater independence in include the use of prefabricated orthoses on a clinical trial
ADLs. basis. These orthoses cost much less and allow a patient
In practice, however, externally powered systems to demonstrate the potential for the long-term usage of a
typically proved difficult to maintain. Without ready more definitive orthosis.
access to technical support personnel who could repair There is also an ongoing search for new materials
delicate electronic parts, the orthoses fell into disrepair and designs that can reduce weight or result in orthoses
and were discarded. Patient training was, therefore, cru- that are more esthetically pleasing; that is, less stigmatiz-
cial to the successful use of the orthoses. The complexity ing in appearance. This may be accomplished through
of orthotic design required a well-organized training pro- surface coating of metals, such as aluminum anodizing,
gram by occupational therapists. These two factors often or the use of alternative materials, such as plastic or car-
proved a deterrent to continued use by all but the most bon fiber. Designers must always remain aware of the
committed patients. need to maintain an optimum balance between material
Designs reverted to more simple mechanical com- strength and weight. All of this must be accomplished in
ponents, which proved easier to operate and maintain. a cost-effective way.
One design adapted prosthetic harnessing systems to the The increased use of robotics in industry to perform
WDWHO. Upper limb prostheses have long been pow- complex tasks suggests potential applications for the dis-
ered by the use of strapping systems that utilize con- abled population. Experimental work has been done on
tralateral shoulder protraction to operate a cable that robotic arms that can manipulate items such as a com-
opens the terminal device. This principle was applied to puter or feeding utensils. Hillman et al. (14) designed a
the WDWHO with limited success. workstation using a commercially available robot manip-
Current designs continue to utilize simple mechan- ulator controlled by a microcomputer. The arm chosen
ical components, which are more easily maintained. was relatively low in cost and offered a wide range of
motion, plus an open/close gripper.
The module stands on a table and may be controlled
CURRENT TRENDS, PHILOSOPHY AND in real time, under direct control of the user, although this
PRINCIPLES is slower than preprogrammed motions. Control is
accomplished through a scanning menu interface, which
Overall, there has been a significant decrease in the use of is accessed through a two-switch input system manipu-
upper limb orthoses in individuals with SCI since the lated with a mouthstick or hand stick. Initial tasks
1970s (12). This is because of a variety of factors, two included loading taped music into a cassette player, load-
of which have combined for greatest impact: general cost- ing discs into a computer, and manipulating books. Pre-
cutting measures resulting in decreased inpatient length programmed routines were also provided for some tasks.
of stay (LOS), and evidence of lack of long-term orthotic Eight patients were tested, and their reactions were gen-
use in a large percentage of individuals with SCI (13). erally favorable.
666 REHABILITATION
Another device that has shown some benefit for per- are prescribed both unilaterally or bilaterally accord-
sons with C5C7 lesions is the bionic glove (15). Developed ing to the pattern of upper limb weakness. However,
at the University of Alberta, this device utilizes Neuro- there is a great advantage in fitting both arms with
muscular Electric Stimulation (NMES) to stimulate finger mobile arm supports to allow for upper body sym-
flexors, extensors, and thumb flexors to produce functional metry in the wheelchair. With a functional orthosis
prehension through tenodesis action at the wrist. Electrodes such as the WDWHO, it may be tempting to fit some
are imbedded in a glove worn by the patient. Three chan- patients with bilateral orthoses. However, it is
nels of electrical stimulation are used to stimulate the thumb uncommon for patients to achieve a higher level of
and fingers. Control is provided through a wrist position function with two orthoses. Typically, the patient
transducer inside the garment. Wrist motion produces pre- achieves optimal function using one orthosis and
hension through tenodesis action. using the other hand as an assist. If sensation in the
radial fingers or thumb is impaired or absent, as in
the person with C5 tetraplegia, the patient must
EVALUATION AND TREATMENT PLANNING compensate visually, and this makes bilateral use of
the orthoses less effective.
To determine which orthoses would best work for an indi- Patients act as partners in selecting the appropriate
vidual, all data gathered by the rehabilitation team must orthosis by designating which goals and activities
be taken into consideration. Along with physical aspects are meaningful to them. The Canadian Occupa-
such as the neurologic level of the injury, ambulatory sta- tional Therapy Measure (COPM) (17,33) is an
tus, and the presence of pain, psychosocial issues are of excellent tool in determining these goals (18,19).
great importance. Questions relating to the discharge des- The choice of a more permanent orthosis is often
tination, the presence of significant others, and occupa- made after various orthoses are tried and as a con-
tional goals play an important role in choosing the appro- tinuing dialog between the team and the patient
priate orthosis for the individual. about fit and functionality takes place.
In orthosis evaluation and treatment planning, the To help determine initial orthotic selection, two
occupational therapist must consider the following key questions must be answered:
points: What orthoses are needed to prevent further
deformity and complications?
Along with objective physical evaluations, the ther- What orthoses would optimize arm function for
apist must observe the function of the hand. How achieving the persons occupational goals?
does the person pick up objects and manipulate Mounting data on the pattern and time line of recov-
them? Is the tenodesis action functional? Can ten- ery of people with complete and incomplete tetraple-
odesis action be enhanced through splinting? gia helps guide the therapist in choosing the appro-
Answers to questions like these help the therapist priate orthosis before full motor recovery is
and the patient choose the optimal orthosis for the achieved. A patient with complete (ASIA A) tetraple-
ultimate goal of enhancing function. gia who tests with 1/5 radial wrist extensors 1
Some hand tests may prove useful in assessing the month post injury, for example, has a 22 percent of
tetraplegic hand (7). Examples to such tests are the having a 3/5 strength after 1 year (20). This statis-
Jebson Hand Test (16) and the Sullerman Hand tical prediction makes it reasonable for the therapist
Function Test (17). When finger and thumb mus- to concentrate on tenodesis training geared toward
cles are present, pinch and grip strength are also ordering the WDWHO. At first the orthosis may
measured. have an extension assist component. However, as the
Along with routine ASIA (21) sensory tests of pain wrist strengthens over time and endurance improves,
and light touch, proprioception is also checked. In the person will be able to use the orthosis effectively
testing sensory dermatomes of the hand (C6C8), without an extension assist component.
if sensation is present, it is valuable to also check
object identification and/or two-point discrimina-
tion. These tests allow the therapist to predict the ORTHOTIC DESIGN PRINCIPLES BY
need for visual compensation in manipulating FUNCTIONAL LEVEL
objects. If the patient has a deficit in hand tactile sen-
sory input, a small, less-bulky orthosis may be cho- This section reviews the most common orthoses given
sen over a bigger orthosis, to allow the patient to to patients by level of injury. Prior to this discussion it
have an unobstructed view of the hand and object. is important to note the small, but important difference
Choosing orthoses for both upper limbs is common between the ASIA neurologic level and a functional
with static, positioning splints. Mobile arm supports level.
UPPER LIMB ORTHOSES 667
Although not an upper limb orthosis, the mouthstick is Mouthsticks: A. Implement holder (pencil, paintbrush,
described here because it is the only simple, low-tech marker, etc.); B. Pointer (keyboard operations, page turner);
device that is widely available and allows the person with C. Pincer (picking up small, light objects).
668 REHABILITATION
MAS may provide the patient with the ability to drive a therapeutic strengthening of the wrist extensors, while
wheelchair with hand controls versus head operated con- still maintaining proper wristhand position. A wrist-
trols. However, the therapist must make certain that the action, wristhand orthosis (WAWHO) (Figure 48.8),
patient is able to use the MAS on uneven and inclined sur- may be used as a transitional orthosis (6) for the patient
faces, because the MAS is dependent on gravity to keep who is less than 6 months post-injury. The orthosis allows
it stable. free wrist motion and has adjustable stops that limit
Although the MAS may be adjusted for use in a motion to a prescribed range. The stops may be adjusted
semireclined position, an upright position is preferable to accommodate existing and changing strength in wrist
for functional activities. Wheelchair trunk supports are extensors. If extensors are absent, the stops may be
needed to obtain adequate upright position and stability. adjusted to eliminate motion and provide static support
A problem of the standard MAS system is that it sig- in the desired position. As strength increases, increased
nificantly increases the overall width of the wheelchair range of motion may be allowed. Extension assist and
and may cause difficulty going through doorways. A augmentation of weak muscles may be achieved with the
newer design, the linear MAS (Figure 48.7) minimizes this use of elastic bands that cross the wrist.
problem by utilizing a straight rod with no articulations As in individuals with C4 and limited C5 return, the
that is mounted in a housing with linear bearings. This elbow must be maintained in extension to prevent flex-
allows for forward, backward, and horizontal movement ion contractures. To achieve this goal, the patient wears
of the MAS. A recently developed design currently under- an elbow orthosis at night.
going clinical trials, called the Rancho-JAECO MAS, uti- There is no optimal functional orthosis for the per-
lizes carbon composite material and multiple articulations son with C5 tetraplegia. To stabilize the wrist, a hand cock-
to produce an extensive and smooth range of motion, up splint is worn. The universal cuff, mentioned previously,
with decreased width to allow for easier accessibility
through doorways.
is often used for feeding and hygiene. However, its uses are discussed. At the C6 level the patient will have intact
limited. The person must use other adaptive equipment for radial wrist extensors, along with some strength in the
other activities such as writing, grooming, cooking, etc. serratus anterior and clavicular pectoral muscles. Pectoral
The universal cuff is often combined with an orthosis that strength provides the ability to bring the arm across the
supports the wrist in a functional position. chest to midline, thereby allowing activities requiring
bilateral arm use. Intact wrist extensors allow the indi-
vidual to harness the tenodesis action at the wrist to
Ratchet WristHand Orthosis
accomplish dynamic prehension for feeding, hygiene, and
Another, more expensive, custom-made orthosis is the similar activities.
ratchet wristhand orthosis (Figure 48.2a,b). The Ratchet In addition to C6 muscle return, the person with
WHO is a metal orthosis that is controlled externally by functional C7 has return of the triceps and lattisimus
the opposite arm. The ratchet WHO holds the wrist joint dorsi and some return of the extrinsic finger muscles,
in a static position. A thumb post holds the thumb most often return of the extensor group.
abducted and aligned in opposition to the index and mid- The focus in managing the hand of persons with C6
dle fingers to allow prehension. Finger pieces hold the or C7 functional level is optimizing the biomechanic
index and middle fingers in enough flexion to allow pad- dynamics of the wrist and hand to promote effective ten-
to-pad contact with the thumb. A ratchet bar connects odesis grip and pinch (2,8,9,25,26). Because the effec-
the finger pieces to the main body of the orthosis. Clos- tiveness of the tenodesis grip is determined by the pas-
ing is accomplished by motion of the contralateral hand sive properties of the hand (25), great efforts are placed
against the ratchet mechanism to flex the fingers against on preserving and encouraging this mechanism. Various
the thumb. Release is accomplished through a return static and dynamic orthoses are prescribed by different
spring, which is activated by pressing a release button. SCI centers for the purpose of encouraging adaptive
Individual muscle strength and experimentation will shortening of the flexor digitorum profundus, superfi-
determine the easiest way for the patient to manage the cialis, flexor pollicis longus, and protecting the web
operation of the orthosis. This simple mechanical design space (25). Such orthoses wrap the fingers as in a box-
is more easily maintained than more complex electrical ing glove and position the thumb in lateral pinch against
systems. With a properly managed training program, a the index finger. An example of this is the Moberg glove
motivated individual can become proficient in a variety (Figure 48.9),which brings full closure of the fingers. To
of functional activities using the ratchet WHO, includ- encourage the dynamic nature of tenodesis, a simple
ing independent feeding, hygiene, writing, and grooming, splint like the dynamic tenodesis strap (Figure 48.10) is
as well as certain tabletop activities (6). used (7,8). Some of these splints are worn in combina-
In select cases, some individuals with C4 tetraplegia tion with others. The person with functional C6 tetraple-
and C5 return may use the ratchet WHO in combination gia may still need to wear an elbow extension splint at
with the MAS (24). It must be stressed, however, that when night to preserve full extension of this joint. Such
the person requires the use of mobile arm supports with a orthoses become unnecessary for the person with C7
ratchet WHO, his functional capabilities are more limited. tetraplegia. A functional dynamic orthosis, such as the
He requires assistance donning and doffing the orthosis wrist-driven wristhand orthosis, may substitute for lost
and the work area must be set up carefully. Once the opti- pinch and grasp.
mal setup is achieved, however, the individual is able to
perform a variety of tabletop activities.
In general, it has been the authors experience that
therapists and patients prefer the use of orthoses and
adaptive equipment other than the ratchet. This is par-
tially because of its cumbersome operation, appearance,
and excessive weight.
In contrast to the limited functional options of the
person with C5 tetraplegia, individuals with C6C7
injuries have significant muscle return, which allows for
a more favorable orthosisthe wrist-driven, wristhand
orthosis (Figure 48.1A, B), described below.
challenge as more and more individuals with SCI have strate a knowledge of the overall purpose of the ortho-
incomplete injuries and are able to ambulate (24). Patients sis, whether it is positional or functional. This under-
with incomplete injuries pose a special challenge, because standing is important in obtaining cooperation and a will-
proximal muscles of the upper limbs may be weak. The ingness to use the orthosis consistently. This is especially
person may be able to reach a cup, or a toothbrush, but true of the positional orthosis because functional gains
may be unable to deliver it to the mouth because shoul- are not always obvious to the patient. It is important that
der and elbow flexion muscles (C5,6) are weak. In a sit- the patient be knowledgeable about wearing tolerance
ting position, the arm can be supported with a table or and schedule.
chair-mounted mobile arm support. However, this is not Whereas most static orthoses require less training than
so when the person is standing or walking. Although dynamic/functional ones, such education should not be
waist-mounted mobile arm supports are sometimes used, overlooked. Family members and attendants must learn to
they are heavy and cumbersome. Some other simple solu- apply the orthosis correctly and demonstrate the ability to
tions have been documented, such as the dynamic triceps- adequately care for the orthosis prior to taking it home.
driven orthosis (DTDO) (33) invented at the Mayo Clinic. Functional orthoses, most typically the ratchet and
This orthosis assists a person in reaching the mouth and WDWHO, require more lengthy and complex training.
face by extending the contralateral arm. Through the use The patient may be introduced to the orthosis by watch-
of a simple dynamic orthosis, a person can accomplish ing a video or observing another patient using the device
elbow flexion and reach her face. It is important to note, successfully. After demonstrating basic understanding of
however, that unlike upper limb support systems mounted the operation of the orthosis, training with activities begins.
to a wheelchair, no optimal solutions exist yet for the With both the ratchet WHO and the WDWHO, the
ambulating patient with weak upper limbs. patient may begin by practicing opening and closing of
the fingers. The patient becomes familiar with the com-
ponents of the orthosis, particularly the actuating lever at
ORTHOTIC FITTING the wrist with the WDWHO, and the bar of the ratchet
WHO. Both the bar and the actuating lever control the
Along with good orthotic design, proper fit is essential. size of the opening and the resultant prehension. Experi-
Upon delivery, the orthosis must be carefully evaluated to ential learning is by far the best, in teaching the patient
ensure proper fit and function. A fit that is less than opti- to handle different size objects and shapes. The fine-tun-
mal will compromise function. Close communication ing of different settings is accomplished through further
between the therapist, orthotist, and patient is essential. experimentation.
Other team members are encouraged to provide feedback With the presence of any hand sensory impairment,
for best fit and use. The nurse, for example, must learn patients must learn to compensate for sensory impairment
to assist the patient in putting on a night static orthosis with the aid of greater visual attentiveness. Additionally,
and monitor it for proper fit during the evening and night they must learn the importance of skin inspection in
shifts. The patient must be encouraged to effectively direct avoiding excessive pressure from either the orthosis itself
care by communicating clearly with staff and significant or from the forceful prehension achieved.
others about the orthosis. The patient practices grasping, placing, and releas-
Initially, the orthosis is placed on the hand for 30 ing objects. It is best to start with soft, medium-sized
minutes, after which, it is removed and any red areas are objects. Training involves activities at various heights that
noted and evaluated again 1 hour later. If the redness has require pronation and supination. Board games such as
not disappeared, adjustments may be necessary. The Connect Four are excellent for practicing grasp and
patient must be instructed in the importance of skin release. Patients are given choices that are meaningful and
inspection to prevent excessive pressure and skin break- relevant to them.
down. Wearing tolerance is gradually increased until opti- Once basic manipulation is mastered, functional
mal tolerance is achieved. training may follow. Patients will often have specific tasks
they wish to accomplish. For example, patients are typi-
cally interested in relearning self-care activities, such as
PATIENT EDUCATION AND TRAINING feeding and brushing their teeth. The patient can be
expected to become independent in these tasks using the
Key to the success of any orthotic management is the wrist-driven, wristhand orthosis. The patient using the
patients participation and understanding of the program. ratchet WHO may continue to need assistance in setting
The orthosis must complement the goals and life context up for the activity.
of the individual (6,27). Typical activities training with the WDWHO
A systematic approach to orthosis training aids in include feeding, hygiene and grooming; parts of dress-
the successful use of the device. The patient must demon- ing; self-catheterization; home and community skills, such
UPPER LIMB ORTHOSES 673
as computer and desktop-related activities; cooking, man- guidelines are based on general knowledge and trends and
aging money; and manipulating knobs, switches and keys. largely lack evidence to support the use of one orthosis
The opportunity for new patients to interact with over another. This is especially true in the area of posi-
experienced and successful users of similar devices is help- tioning for preventing deformity. To support and improve
ful, and this may occur even before patients receive their the care of individuals with SCI, more studies are needed,
own orthoses. Group activities such as games with particularly those that demonstrate the outcomes of the
patients using similar orthoses, are great opportunities for use of various splints and orthoses.
humorous social interactions and at the same time, for
engagement in repetitive use of the functional orthosis. References
To achieve optimal independence the patient must
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26. Curtin M. An analysis of tetraplegia hand grips. Brit J Occup Ther Ratchet wristhand orthosisA variation of the wrist
1999; 62:444450.
27. Clarke D. Wrist driven, wristhand orthosis use post-discharge by
driven wrist hand orthosis design that allows the
individuals with tetraplegia. Unpublished, 2001. individual with C5 tetraplegia some prehension
28. Shepherd C, Ruzika S. Tenodesis brace use by persons with spinal function.
cord injuries. Am J Occup Ther 1991; 45:8283.
29. Engel W, Kmiotek M, Hohf J, French J, Barnerias M, Siebens A.
Resting wrist-hand splintA static positioning splint
A functional splint for grasp driven by wrist extension. Arch Phys that keeps the wrist and hand in a functional posi-
Med Rehabil 1967; 48:4352. tion. The wrist is at approximately 2030 of
30. Knox C, Engel W, Siebens A. Results of a survey on the use of a
wrist-driven splint for prehension. Am J Occup Ther 1971;
extension, the fingers are in slight flexion and the
15(2):109111. thumb is in abduction.
31. Wise M, Wharton G, Robinson T. Long term use of functional Short opponensA static positioning orthosis that
hand orthoses by quadriplegics [abstract]. Proceedings of the
Twelfth Annual Meeting of the American Spinal Injury Associa-
supports the thumb and fingers in a functional
tion. San Francisco: 1986; 111113. position.
32. Formal C, Smith J. Upper extremity function in spinal cord injury. Tenodesis actionThe normal anatomical action
In: Ditunno J (ed.), Topics in Spinal Cord Injury Rehabilitation
Aspen Publishers 1996; 1(4):114.
whereby, as the wrist is extended, the fingers flex
33. Lawlor B, Stolp-Smith K. A new dynamic triceps-driven orthosis and naturally fall against the thumb in a position of
(DTDO): Achieving elbow flexion in patients with C5 deficits. lateral pinch.
Arch Phys Med Rehabil 1998; 79:13091311.
Tenodesis splintSame as wristdriven wristhand
orthosis.
Universal cuffA simply designed cuff that fastens to
GLOSSARY the individuals hand. A pocket in the cuff allows
attachment of various implements to accomplish
Flexor-hinge hand splintSame as wristdriven wrist feeding, hygiene, etc.
hand orthosis. Wristdriven, wristhand orthosisA dynamic ortho-
Long opponens splint (Also called wristhand orthosis, sis that provides a transfer of power from the wrist
WHO)A static positioning orthosis that supports extensors to the fingers for the purpose of
the wrist and allows the fingers and thumb to rest prehension.
in a functional position.
49 Lower Limb Orthoses
and Rehabilitation
Ann Yamane
rthoses used in the management of skeletal malalignment include the acquisition of patho-
PATIENT EVALUATION
Range of Motion
If range-of-motion limitations are treated, potential align-
ment problems can be prevented (3). For example, a plan-
tarflexion contracture associated with internal rotation
FIGURE 49.2
pensations the individual will use in his pathologic gait peroneals are not innervated, the subtalar joint will be
pattern. In addressing orthotic intervention we must maintained in inversion. In addition, if the strength of the
look at the interaction between the ankle, knee, hip, gastrocnemius and soleus is poor (2/5), the foot and ankle
pelvis, and trunk during both stance and swing phase. are positioned in dorsiflexion and inversion. Although the
For example, an AFO may be designed to substitute for individual has adequate clearance of the foot during swing
inadequate dorsiflexion strength of the tibialis anterior phase, the unopposed dorsiflexion and inversion must be
and toe extensors by controlling plantarflexion range of addressed or a pathomechanical deformity will develop.
motion during swing phase, with a plantarflexion stop
place in dorsiflexion. One question that arises is: What
Sensory Testing
impact will this have at loading response? The normal
eccentric contraction of the ankle dorsiflexors that Joint proprioception of the ankle and knee is vital for the
allows controlled plantarflexion is also impaired. Plac- successful control of the knee. If an individual does not
ing the ankle in dorsiflexion within an AFO eliminates have position sense awareness to place the knee in rela-
this controlled plantarflexion and creates a knee flex- tionship to his ankle, hip, and trunk, he will have diffi-
ion moment during loading response. This situation culty finding stability in a well-aligned position. As a com-
raises our concerns regarding the persons muscle pensation to gain knee stability during stance phase an
strength and control at the knee and hip. Good (4/5) individual may use uncontrolled knee extension to their
quadriceps strength (Table 49.1) is adequate to control end range of motion to prevent knee flexion.
a knee flexion moment. Fair (3/5) quadriceps strength Sensory testing for light touch and pain will assist
has the potential to control a knee flexion moment, but in determining the type of orthotic design considerations
also depends on other factors such as size of the person appropriate in distributing the desired forces. It will also
and the presence of joint proprioception. furnish us with information regarding the type of patient
When assessing a person with an incomplete SCI, the education necessary to prevent any skin breakdown. A
manual muscle testing (MMT) evaluation is especially person without protective sensation will need education
important in determining joint instability in the sagittal on skin observation for redness caused by excessive pres-
and coronal planes and predicting any potential patho- sure over any bony prominence.
mechanical deformities. Our MMT examination looks at
the strength at each joint and assesses the agonist and
Static Alignment Evaluation
antagonist strength for symmetry. For instance, in a case
where the tibialis anterior strength is good (4/5) and the A static alignment evaluation without an orthosis gives
us information with respect to the natural skeletal align-
ment of the individual. This knowledge assists in devel-
oping our hypothesis regarding the impact muscle weak-
TABLE 49.1 ness or spasticity has on joint range of motion, alignment,
Manual Muscle Testing (4) and gait pattern. For example, in observing the static
alignment of an individual with a plantarflexion con-
QUALITATIVE NUMERICAL MUSCLE TESTING tracture caused by spasticity of the gastrocnemius and
GRADE GRADE CRITERIA
soleus, we may see a number of compensations. In addi-
tion to talocrural plantarflexion we may see distal sub-
Normal 5 Completes full ROM against
gravity, maximum
talar eversion, midtarsal pronation, and forefoot abduc-
resistance tion. Proximally, we may observe knee hyperextension,
Good 4 Completes full ROM against internal rotation of the tibia and femur, retraction of the
gravity, strong resistance pelvis, and an anterior tilt of the pelvis.
Fair plus 3+ Completes full ROM against
gravity, mild resistance
Fair 3 Completes full ROM against
Gait Evaluation
gravity If possible, a gait evaluation without any orthotic inter-
Poor 2 Completes full ROM, vention is advantageous to assess the persons alignment
gravity minimized and pathologic gait pattern. Frequently the patient does
Poor minus 2 Completes partial ROM,
not have the necessary stability, and this is not possible.
gravity minimized
Trace 1 Contraction palpated/
In this situation, we correlate our manual muscle testing
Detect visually and range-of-motion results with our static alignment
Zero 0 No muscle contraction evaluation to develop an orthotic recommendation. The
palpated use of temporary evaluative orthoses is another option
available for assessment.
678 REHABILITATION
We must remember that all phases of gait are inter- trolling two adjacent skeletal segments (Figure 49.3). The
woven and that, whereas one orthotic design may be ben- corrective force is located on the convex side of the curve
eficial during one phase of gait, it may prove to be inad- at the joint addressed (Figure 49.3b). Two counteractive
equate or even detrimental during a subsequent phase of forces are positioned on the opposite side above (Figure
gait. Our task is to determine the biomechanical needs 49.3c) and below (Figure 49.3a) the corrective force.
during both swing and stance phases of gait and develop Increasing the distance of the counteractive forces from
an orthotic design that addresses the most important the corrective force increases the lever arms and therefore
issues without creating any additional problems. the effectiveness. Based on the principle Pressure = Total
force/Area of force application, the objective is to dis-
tribute the forces over a larger area to decrease the resul-
GOALS FOR AFO INTERVENTION tant pressures (5). A well-fitting total contact orthosis
that avoids bony prominences and utilizes an appropri-
The most obvious use for an AFO is the control of the ate and effective three-point force system assists in achiev-
ankle joint in the sagittal plane. The AFO can sustain ing this objective.
clearance of the foot during swing phase if there is inad- To provide mediolateral stability at the subtalar
equate strength of the ankle dorsiflexors, including the joint and control excessive subtalar eversion, the three-
tibialis anterior, extensor hallicus longus, and extensor point force system (Figure 49.4) has the corrective force
digitorum longus. The AFO can also simulate push-off
during stance phase, therefore substituting for weak
ankle plantarflexors, which consist of the gastrocnemius
and soleus. The less obvious goals of an AFO include
controlling the position of the ankle in the sagittal plane
to control mild knee hyperextension and knee flexion
instability caused by the weakness of the quadriceps.
Coronal plane stability of the subtalar joint can be
achieved with a well-designed plastic AFO as well as
supination and pronation of the forefoot. Transverse plane
control must also be considered when designing an orthotic
system. With proper stabilization of the subtalar joint,
transverse plane control of the forefoot is obtainable, as
demonstrated by forefoot abduction and adduction. Inter-
nal rotation of the femur and tibia is the more difficult com-
ponent of transverse rotation to control. However, through
proper material selection and appropriate biomechanical
design, we can provide various levels of control. The mate-
rial properties of the polypropylene used in AFOs, along
with any asymmetry of the anterior trimlines of the half-
cylinder shape, may create torsion in the orthosis. Methods
of decreasing transverse rotation in the orthosis include
reinforcements, the use of thicker plastic, and the incorpo-
ration of conventional stirrups and ankle joints.
Through optimal skeletal alignment of the person,
along with appropriate biomechanical controls in our AFO
design, we hope to create a stable base of support to allow
safe and efficient ambulation and prevent the development
of pathomechanical deformities. We must first discuss the
biomechanical controls used in an AFO to understand how
we can achieve our goals for orthotic intervention.
The controls incorporated in orthotic systems are based Three-point force system for correction of genu valgum. B
on three-point force systems that affect alignment by con- corrective force; A,C counteractive forces.
LOWER LIMB ORTHOSES AND REHABILITATION 679
applied proximal to the medial malleolus (Figure 49.4b) caneus (Figure 49.5a) and the medial proximal tibial flare
and at the sustentaculum tali (Figure 49.4c). Pressure (Figure 49.5d).
cannot be applied directly to the bony medial malleo-
lus, therefore the corrective force must be applied over
Plantarflexion Stop
two adjacent areas. The sustentaculum tali (ST) is
located on the calcaneus and, if stabilized correctly by a A plantarflexion stop or posterior stop in an AFO (Figure
ST modification or pad, provides a horizontal ledge to 49.6) is designed to substitute for inadequate strength of
support the talus (6). The two counteractive forces at the the ankle dorsiflexors, including the tibialis anterior, exten-
distal lateral calcaneus (Figure 49.4a) and proximal lat- sor hallicus longus, and the extensor digitorum longus,
eral calf (Figure 49.4d) are above and below the joint during swing phase of gait. This stop is effective by limit-
and as far away from the joint as possible to produce ing the plantarflexion range of motion of the talocrural
longer lever arms. joint. The three-point force system has the corrective force
Subtalar inversion (Figure 49.5) from an unopposed at the shoe instep or ankle strap and two counteractive
tibialis anterior is controlled by the corrective force placed forces: one at the plantar surface at the ball of the foot
proximal to the lateral malleolus (Figure 49.5c) and over and the second on the posterior calf region. An important
the cuboid (Figure 49.5b) if possible. Again, we are concept when evaluating the ankle position is the tibial
unable to apply a direct force over the lateral malleolus angle to the floor (Figure 49.7). We define the tibial angle
and must place the corrective forces adjacent. The two to the floor by bisecting the distal one-third of the tibia in
counteractive forces are located at the distal medial cal- the sagittal plane and measuring this angle in relationship
Three-point force system for correction of subtalar eversion. Three-point force system for correction of subtalar inversion.
Corrective force (b) medial malleolus and (c) sustentacu- Corrective force (c) proximal lateral malleolus and (b)
lum tali. Counteractive forces (a) distal lateral calcaneus cuboid. Counteractive forces (a) distal medial calcaneus
and (d) proximal lateral calf. and (d) medial tibial flare.
680 REHABILITATION
FIGURE 49.7
FIGURE 49.6
The tibial angle to the floor is determined by bisecting the dis-
Articulated plastic AFO with a plantarflexion or posterior stop. tal one-third of the tibia in relationship to the floor while wear-
ing a shoe.
FIGURE 49.8
Conventional metal ankle foot orthosis. Conventional double adjustable ankle joint.
LOWER LIMB ORTHOSES AND REHABILITATION 683
main function of this AFO design is to limit plantarflex- ated. The tibial angle to the floor is critical in determin-
ion range of motion during swing phase when an indi- ing knee stability. Stability is achieved with the ankle at
vidual has weakness of the ankle dorsiflexors. Unfortu- 90 degrees to the floor or slightly posteriorly tilted. The
nately, the inherent flexibility of this design will not length of the footplate may be extended to the end of the
control excessive subtalar eversion, midtarsal pronation, toes to increase the knee extension moment arm. The
and forefoot abduction. The placement of the mechani- ground reaction AFO design is indicated with quadriceps
cal axis of the AFO posterior to the anatomic axis leads strength of fair minus (3/5) (8).
to migration of the orthosis during plantarflexion and Articulated plastic AFO designs (Figure 49.6) are
dorsiflexion range of motion and the potential for skin widely used because they easily provide a system with a
irritation or ulceration on the leg. sagittal plane plantarflexion stop and control in the coro-
The ground reaction AFO (Figure 49.14) is a mis- nal and transverse planes caused by their total contact
nomer, because all AFOs utilize ground reaction forces. design. Clinically, we have found the incorporation of an
This nomenclature has been used historically to describe ankle joint decreases the amount of transverse and coro-
the plastic AFO composed of a solid ankle design and a nal plane control because of the flexibility added to the
pretibial shell. Ground reaction forces induce a knee system by the joint and the interruption in total contact.
extension moment from midstance through terminal Common ankle joints include the Tamarack joint (Fig-
stance by a dorsiflexion stop or anterior stop that limits ure 49.15a), Gaffney joint (Figure 49.15b), and the Okla-
the dorsiflexion range of motion. As the center of mass of homa joint (Figure 49.15c). These are articulations only
the individual is moving forward and tibial advancement and must be used in conjunction with a plantarflexion
is limited by the AFO, a knee extension moment is cre-
Custom fabricated posterior leaf spring ankle foot orthosis. Ground reaction or floor reaction ankle foot orthosis.
684 REHABILITATION
FIGURE 49.15
Hybrid ankle foot orthosis. Knee joints. a. Bail lock, b. Drop lock/Ring lock, c. Offset.
Hybrid KAFOs
Hybrid KAFOs (Figure 49.2) are composed of either a
plastic or laminated thigh and calf section. They incor-
porate a conventional stirrup and ankle joint system that
allows the freedom of maximum sagittal plane adjust-
ment of the ankle position. The total-contact qualities of
the design provide an equivalent degree of control in the
sagittal and coronal planes as in the plastic KAFO
designs. The laminated systems have improved transverse
plane control because of their increased rigidity. The dis-
advantages of this design include increased fabrication
time and expertise, the inability to alter the fit following
volume changes, and increased cost.
RECIPROCATING
GAIT ORTHOSES
Plantarflexion Weakness
The clinical signs of plantarflexion weakness observed
during terminal stance include: a) excessive knee flexion;
b) late heel rise; c) short step length on the contralateral
side. Orthotic intervention provides a simulated push-off
that is accomplished by utilizing a dorsiflexion or ante-
rior stop to limit the amount of dorsiflexion during mid-
stance to terminal stance, thus creating the forefoot
rocker described by Perry (11).
FIGURE 49.20
CLINICAL SITUATIONS
Dorsiflexion Weakness
The clinical observation of dorsiflexion weakness reveals
inadequate clearance of the foot during the swing phase
of gait. Compensations observed during swing-phase gait
evaluation include increased hip and knee flexion. With
the strength of the ankle dorsiflexors less than grade fair
(3/5), a heel strike will most likely be absent. Grade fair
(3/5) muscle strength of the ankle dorsiflexors may pro-
vide adequate clearance of the foot, but a foot slap will
be observed at loading response. The lack of adequate
inversion strength will decrease coronal plane subtalar
joint stability. Our goals for orthotic intervention include:
a) adequate clearance of the foot during swing phase; b)
controlled knee flexion during loading response; c) bal-
anced alignment with respect to inversion/eversion of the
calcaneus or subtalar joint at loading response (10). FIGURE 49.21
Orthotic design options to address dorsiflexion
weakness include: a) a dorsiflexion assist AFO (Figure Dorsiflexion assist plastic ankle foot orthosis.
688 REHABILITATION
itation team that includes the patient, physician, physical 5. Fess EE, Philips CA. Hand Splinting: Principles and methods, 2nd
ed. St. Louis: C.V. Mosby, 1987; 126.
and occupational therapists, and the orthotist. A thorough 6. Carlsen MJ, Berglund G. An effective orthotic design for con-
evaluation is crucial to the success and acceptance of the trolling the unstable subtalar joint. Orthotics and Prosthetics
orthoses, along with an accurate understanding of the bio- 1979; 33(1):3141.
7. Perry J. Gait Analysis: Normal and pathological function, 1st ed.
mechanical needs in relationship to the functional goals Thorofare, NJ: SLACK Inc., 1992; 6063.
of the individual. Patient education in the short- and long- 8. Yang GW, Chu DS. Floor reaction orthosis: clinical experience.
term goals of orthotic intervention is an important factor Orthotics and Prosthetics 1986; 40(1):3337.
9. Perry J, Hislop HJ (ed.), Principles of lower-extremity bracing,
in preventing pathomechanical deformities caused by an New York: APTA, 1967; 3647.
inadequate or inappropriate orthotic treatment plan. 10. Perry J. Gait Analysis: Normal and pathological function, 1st ed.
Thorofare, NJ: SLACK Inc., 1992; 303304.
References 11. Perry J. Gait Analysis: Normal and pathological function, 1st ed.
Thorofare, NJ: SLACK Inc., 1992; 3036.
12. Perry J. Gait Analysis: Normal and pathological function, 1st ed.
1. Fish DJ, Nielsen JP. Clinical assessment of human gait. JPO 1993; Thorofare, NJ: SLACK Inc., 1992; 272275.
5(2):2736. 13. Lehmann JF, deLateur BJ, Price R. Ankle-foot orthoses for pare-
2. Shurr DG, Cook TM. Prosthetics and orthotics, 1st ed. East Nor- sis and paralysis. Phys Med and Rehab Clin of NA 1992;
walk, CN: Appleton & Lange, 1990; 1415. 3(1):139159.
3. Perry J. Gait Analysis: Normal and pathological function, 1st ed. 14. Nixon V. Spinal Cord Injury: A guide to functional outcomes in
Thorofare, NJ: SLACK Inc., 1992; 192195. physical management, 1st ed., Rockville, MD: Aspen Publishers,
4. Hislop HJ, Montgomery J. Muscle testing, techniques of manual 1985.
examination, 6th ed. Philadelphia: WB Saunders, 1995; 26.
This Page Intentionally Left Blank
50 Activities of Daily Living
injured individual and the medical team; ideally this PERSONAL INDEPENDENCE POTENTIAL
involves matching the values and goals that the person
has with the rehabilitation program proposed by the rest The potential for independence in people with complete
of the medical team. Most injured people are motivated spinal cord injury (SCI) has been well described in other
to regain control over various aspects of ADLs, such as chapters. Self-care is further delineated in this chapter
self-feeding and bladder and bowel management, as psy- based on complete injury or particular muscle strength.
chologic adjustment to the physical condition stabilizes. The following examples of personal independence fol-
Although a comprehensive rehabilitation program of lowing rehabilitation training are ideal for people with
independent living may not be accomplished within the incomplete injury.
trend of decreasing length of hospital stay, many self-care Central cord syndrome is a special type of incom-
issues can continue to be resolved through a continuum plete SCI that impairs upper extremity (UE) function
of care and the introduction of problem-solving tech- more than lower extremity (LE) function. This tends to
niques that can be further generalized after discharge. create more deficits with ADL skills rather than mobil-
Self-care, as defined in this chapter, falls within the ity. Bowel and bladder function is usually spared, but toi-
realm of human occupational performance and includes leting can be difficult without UE function. Approxi-
personal care habits such as grooming, dressing, and mately 93 percent of people with this type of injury are
bathing. This term will be used interchangeably with bladder continent at the time of discharge from acute
ADLs. The term of instrumental activities of daily living inpatient rehabilitation (1). Bath mitts, bidet toilets, and
(IADL) will, for the purposes of this chapter, extend to adaptive clothing with finger loops are examples of assis-
learning (access to information through desktop activi- tive devices used to promote more independent toileting
ties, personal interaction); play (age-appropriate leisure skills. Of patients discharged from acute rehabilitation
pursuits); domestic activities (household maintenance, 56.2 percent will be independent or require minimal
shopping); and interpersonal activities (roles within the supervision with ADLs or mobility (1).
691
692 REHABILITATION
A person with a C1C4 central cord injury may be Visual feedback is needed to safely perform ADLs, espe-
ambulatory and able to do ADLs but may require respi- cially in homemaking, where mishandling hot items can
ratory assistance with a ventilator. Unfortunately, if the cause burns. For those who have a very rare (tumor,
anterior horn cells of the diaphragm are involved, this cre- surgery, gunshot wound) thoracic BrownSequard syn-
ates a peripheral nerve injury, which typically disallows drome, they will likely be independent in ADLs without
phrenic nerve pacing. The equipment issues may include adaptive devices.
a rigid cervical orthosis with tracheotomy cut-out and a Anterior cord syndrome causes an incomplete injury
manual wheelchair with ventilator tray. Communication that affects the anterior cord and preserves the posterior
may be accomplished through use of a Passey-Muir valve columns. Variable loss of motor function and pinprick
or sign language/writing. sensation occurs, but light touch and proprioception are
A person with central cord injury at C5 loses shoul- spared. This injury gives the person the advantage of hav-
der and elbow flexion and supination. This compromises ing protective sensation to avoid injury and pressure
overhead activities such as upper body dressing or sores, but neuropathic pain may limit ADLs. Unless this
bathing. Swaying the trunk for UE momentum may person has significant motor sparing that can be used
accommodate overhead activities. A balanced forearm functionally, presentation in ADLs will be similar to com-
orthosis (BFO) could be indicated, but the person is usu- plete injury at the same neurologic level. Strong motiva-
ally ambulatory and not interested in utilizing a wheel- tion, pain control, and spasticity management, if present,
chair. For desktop activities and computer use, a mobile can allow weak muscles to be utilized along with adap-
arm support (MAS) can be utilized. A simple orthosis can tive devices and orthoses to improve function. On litera-
consist of a figure-8 shoulder harness connected to fore- ture review, anterior cord syndrome is the least studied
arm cuffs by flexible rubber tubing. The clamps on the for functional outcome and the above recommendations
cuffs allow for variable arm positioning for ambulatory are based on the authors experience.
ADLs (2). Alternatively, the dynamic triceps-driven ortho-
sis provides elbow flexion by using contralateral elbow
extension to move a cable threaded across the shoulders PERSONAL INDEPENDENCE
and wrist cuffs bilaterally (3). BASED ON OTHER FACTORS
Someone with an isolated C6 central cord injury can
compensate by splinting the wrist in a functional position. Many other factors influence ADL independence as it is
The person with an isolated C7 central cord injury pre- performed out in the community. Medical, psychologic,
sents with weakened triceps strength, which impairs and societal issues all affect a persons ability to utilize
weight bearing through the UE for moving from sit to their reserve neurologic function in daily living tasks.
stand or walking with crutches. This person fortunately Medical issues include cardiopulmonary reserve
will still have enough volitional extension of the elbow to (endurance), spasticity, pain, range of motion (contrac-
eliminate gravity for the majority of daily living tasks. tures/heterotopic ossification), spinal stability, cognitive
Built-up utensils and natural tenodesis or a U-cuff may be status, age, and body type. Psychologic adjustment to dis-
utilized with a C8T1 injury with decreased hand func- ability includes issues such as motivation, family support,
tion. People with central cord injuries at or below T2 can substance abuse, and lifestyle.
become independent in ADLs, parallel to people with Society plays a role in the achievement of indepen-
complete SCI, except that ADL goals are oriented to dence through financial support of needed adaptive
standing or walking positions instead of sitting. devices and equipment, including environment control
BrownSequard syndrome produces relatively units (ECUs) and orthoses, as well as home modifications
greater ipsilateral proprioceptive and motor loss and con- and caregiving. Finally, lifestyle choices and preferences,
tralateral loss of sensation to pinprick and temperature. such as school, work, or other leisure activities, may cause
Usually, bowel and bladder function is spared and the per- the injured person to budget time and energy spent on
son is continent and independent with toileting manage- performing certain aspects of self care in pursuit of more
ment. If the person requires a bowel program with digi- meaningful life activities.
tal stimulation or suppository insertion, a mirror may be
needed. With extensive rehabilitation, this person may
Cardiopulmonary Reserve
become independent in ADLs, similar to someone with
hemiplegia, by using one-handed techniques with adap- With pacing and energy conservation, most daily living
tive devices. The lower the cervical lesion, the more tasks can be performed despite limited cardiopulmonary
rapidily the ADL training can occur, with decreasing reserve. There is a need, however, to consider electric
needs for adaptive devices to address UE paralysis. The mobility to conserve energy for transfers and daily living
rehabilitation of this person will be focused on compen- tasks. Sliding board transfers may also be recommended
sating for impaired sensation on the nonparalyzed UE. over scapular depression-style hopover transfers to avoid
ACTIVITIES OF DAILY LIVING 693
increased UE isometric use if the patient has significant The development of chronic pain in the individual
cardiac disease. with SCI has a significant association with reduced qual-
ity of life and increased dependence in ADLs. Studies
have shown that SCI-related pain increases over time.
Spasticity
Chronic pain, as seen in SCI, is associated with increased
Spasticity can either assist in or impair ADLs. An exam- levels of depression, anxiety, fatigue, and other psy-
ple of how spasticity is used in ADLs, in a person with chosocial problems (4).
C6C7 tetraplegia, is the use of spastic finger flexors for
holding objects, especially if one hand has finger use and
Range of Motion
the other arm offers a stabilization assist. Functional trans-
fers can be facilitated by extensor spasticity of trunk, hip, Impaired joint range of motion can be dynamic, because
and knee. A disadvantage of spasticity is the decreased flex- of spasticity and tone, or fixed, because of soft tissue
ibility and interference in the performance of a functional shortening across a joint, heterotopic ossification, or
activity. A high degree of truncal extensor spasticity may arthrodesis. Some contractures are encouraged to facili-
cause safety issues or more dependence for transfers. tate ADL status. Back extension contractures may assist
Bumps in the road during wheelchair propulsion may trig- with sitting balance. The person with C6 tetraplegia can
ger truncal extensor spasms. This can interfere with safety use finger flexor contractures to increase grasp force by
and optimal sitting posture for driving (especially for some- wrist extension (known as the tenodesis effect).
one with a high cervical injury). People with excessive Generally, however, most contractures limit func-
adductor spasticity of the hip will have difficulty doing tional activities and may interfere with future tendon
bowel and bladder care or perianal bathing. This, in turn, transfers in the upper extremity. A person with C5
can also increase the difficulty of caregiving. tetraplegia will have a decreased ability to use gravity to
extend the elbow and volitional flexion in the presence of
an elbow flexion contracture. In addition, this person can-
Pain
not lock the elbow in extension to do a push-up style pres-
In acute or chronic SCI, pain often limits the ability to sure relief. If someone with C6 tetraplegia has wrist flex-
perform ADLs. Pain can be perceived because of muscu- ion contractures, they prevent the use of the tenodesis
loskeletal injury or be neuropathic because of the SCI or effect for releasing an object from the wrist extension
peripheral nerve or root injury. An appropriate and timely grasp position. LE extension contractures limit the flexi-
pain management program is crucial to allow continued bility needed to do LE dressing, bathing, and some func-
ADL performance, to avoid learned dependence on care- tional transfers.
givers, and to avoid the development of a chronic pain
syndrome. People with acute traumatic SCI often have
Spinal Stability
comorbid diagnoses such as long bone fracture, verte-
bral fracture, herniated discs, and soft tissue injury, which Immediately after traumatic SCI, there are strict bedrest
are all painful if the SCI spares sensation to the same der- and log roll precautions until the spine is stabilized
matome. In complete injuries, with the comorbid mus- through either surgery or appropriate bracing. Prior to
culoskeletal injury below the level of injury, spasticity and stabilization, ADL training is severely limited. The per-
neuropathic pain can be aggravated. This, in turn, can son is usually dependent on nursing care for all self-care
negatively affect independence with ADLs. except possibly self-feeding and grooming after set-up.
With chronic SCI, overuse syndromes, fractures, and Stabilization causes decreased flexibility at the levels that
arthritis in the UE have a direct impact on the person who were either fixed internally through instrumentation or
was previously independent in ADLs. Using correct tech- fusion or externally through bracing.
nique with ADL and mobility tasks can often prevent Cervical multilevel fusion in the person with
overuse syndromes. Acute or overuse injuries necessitate tetraplegia decreases neck flexibility for independent
prompt treatment, including rest; this increases the need mouthstick activities. Additionally, cervical fusion may
for care. In the interim of resting, temporary equipment necessitate the use of extra mirrors in a vehicle for visual
(i.e., adaptive devices, sliding board, electric wheelchair, safety during driving. A rigid cervical orthosis is advan-
mechanical lift, etc.) and caregiver assistance are sug- tageous in supporting the neck in the C1C3 tetraplegia
gested. Painful sensation serves as a reminder for the per- or neurological weakness of cervical muscles in the vari-
son avoid risk factors. Examples of this include per- ous spinal cord diseases. Unfortunately, this orthotic
forming ADLs using ergonomically correct techniques, decreases downward vision, causes difficulties in self feed-
attending to factors such as a heat source during kitchen ing, and limits the ability to use a mouthstick.
activities, or excessive pressure over the ischial tuberos- Multilevel fusion of the lumbar spine and/or multi-
ity while absorbed in a tabletop activity. level internal instrumentation limits flexion of the lum-
694 REHABILITATION
bar spine for LE dressing. Dressing aids and LE flexibil- der management often allows attendance at school with-
ity can compensate for this. The temporary advantage of out an attendant. Yet another device, the mobile arm sup-
a thoracic lumbar sacral orthosis (TLSO) is truncal sta- port (MAS), may have various disadvantages, such as a
bility for functional transfers and sitting for tabletop lack of appeal because of cosmesis, extensive set-up, and
activities. The disadvantages of a TLSO are decreased the level of difficulty for operation.
flexibility for LE dressing and decreased vision of peri- Aging with SCI can be associated with increased dif-
urethral area for self-catheterization in the female. If the ficulty for performing bathing, dressing, and transfers onto
spinal brace is not worn all the time, the person and/or adaptive bathroom equipment. The individuals in one
caregiver must learn how to don and doff this apparel. study reported fatigue, pain, and weakness as the most
common contributing factors to a decline in function (5).
This is probably related to the development of overuse syn-
Cognitive Status
dromes, arthritis, and limited reserve capacity.
To learn compensatory techniques and the use of adap- Increasing age, however, without associated medical
tive devices to maximize independence in ADLs, the comorbidity does not directly affect the ability to do
individual with SCI needs to consistently follow direc- ADLs. New-onset geriatric SCI patients tend to discharge
tions, demonstrate retention of information, and have from acute rehabilitation at lower Functional Indepen-
the ability to problem solve. SCI caused by motor vehi- dence Measure (FIM) scores (6). This may be caused by
cle accidents has a correlative of traumatic brain injury, the length of time required to achieve similar FIM scores
which may affect cognitive skills. Multiple sclerosis can for elders with SCI when compared to their younger coun-
often cause spinal cord disease along with cognitive terparts. In addition, there is a higher incidence of dis-
impairment. charging elders with SCI to nursing facilities in compar-
SCI patients who have significant behavioral prob- ison to young to middle-aged adults with SCI. This could
lems caused by head injury may have difficulty retaining be related to poor social support and/or medical comor-
a consistent caregiver. In addition, they may be at risk bidities. The older person with SCI is likely to have fam-
for further injury or complications because of decreased ily members who are also aging and possibly facing their
safety awareness. People with a dual diagnosis of SCI and own medical and functional issues that preclude the abil-
traumatic brain injury (TBI) may require 24-hour super- ity to provide care (5).
vision, if not physical assistance, to maintain a safe home When the team knows that the patient will be dis-
environment and avoid the medical complications of SCI. charged to a nursing facility, there may be a bias toward
less training in ADLs. There may be a belief that the nurs-
ing facility staff will simply dress and bathe the patient
Age
and not allow sufficient time for the patient to do the
Training children in ADLs is essentially similar to train- activity independently. Independence in self-feeding, how-
ing adults with the same degree of neurologic impairment. ever, is highly important in a nursing facility for increas-
The differences include recognizing those stages of child ing quality of life and decreasing caregiver burden. As the
development that correlate with the introduction of cer- general populations mean age increases, the mean age
tain ADLs. Habilitation is the initial learning of activities. of the SCI population also increases (7,8).
Rehabilitation must be based on a developmental frame-
work that matches the childs current level. The child may
Body Status
need to return to an occupational therapist frequently to
train on new tasks and/or to reassess equipment based on Above-average weight makes functional transfers more
changing body status. difficult and increases assistance needs for caregivers (9).
Therapeutic play activities are incorporated into A protuberant abdomen can decrease vision of the ure-
ADL training to enhance the childs interest. The U-cuff thra. In some cases, excessive girth can contribute to the
can be used as soon as the child begins hand to mouth difficulty of applying and sustaining a condom catheter
activities. For children aged 1 to 3 years, the U-cuff in males because of penile retraction. This often translates
requires custom fitting. Children will tend to use it functionally into a need for an extra transfer back to bed
because it is cosmetically acceptable, easy to don/doff, as an intermittent catheterization program (ICP) may not
and has numerous applications. There is less acceptance be able to be performed while up in the wheelchair (9).
of the short opponens splint because repositioning the Tissue redundancy also interferes with the ability of
thumb with the chin is preferred over wearing the splint. females to easily visualize the urethra for ICP. In addition,
The wrist-driven flexor-hinge (WDFH) orthosis can be a protuberant abdomen obstructs the trunk from ade-
used after age 3 and is custom made for children. It is cos- quately flexing forward while on the bed or wheelchair
metically unappealing but is often the only way the child for independent management of the lower body. Short
can self-catheterize his bladder. Independence with blad- arm length (combined with a long trunk) decreases the
ACTIVITIES OF DAILY LIVING 695
ability to do self-digital stimulation for a bowel routine, and a unilateral FES system, it was shown in a single sub-
self-bladder catheterization in females, and decreases abil- ject study that a person with complete C5 tetraplegia
ity to do functional depression transfers. improved from a baseline of moderate assist to set-up for
Excessive underweight causes bony prominences to self-feeding. In addition, he improved from dependent
protrude excessively, and may interfere with safely per- to minimal assistance in grooming and UE dressing (15).
forming ADLs on surfaces with inadequate padding. Peo- Lack of sensation and proprioception makes the use of
ple with underweight status typically will not have nutri- postoperative occupational therapy extremely important
tional reserves from which to heal pressure ulcers in a for training in using the new grasp capacity. Current stud-
timely manner. ies are trying sensory feedback to close the loop and hope-
fully improve the functional use of the grasp in ADL
tasks. Additional discussion on FES of UE can be found
Surgical Interventions for ADLs
in Chapters 41 and 54.
The surgical treatment of spasticity or contractures can
lead to increased flexibility. This may complement the
performance of self-dressing, bathing, and stability of sit- CHALLENGES
ting balance. UE tendon transplants can improve UE func-
tion for self feeding and grooming (10,11). Various tech- The individual with acute SCI faces many challenges with
niques have been developed to restore active elbow the resumption of self-care tasks. The value of indepen-
extension, wrist extension, finger flexion to enhance dence in mainstream society, superimposed on ever-dwin-
grasp, grasp release, and lateral thumb pinch. Restoring dling community resources, has increased the financial
active elbow extension enhances the ability to perform burden that people with SCI face to resume community
self-care activities that require overhead reaching and participation. An inability to perform self-care activities
allows more complete forward reaching to retrieve is considered a burden of care by the medical commu-
objects. In addition, it lends stability to the elbow, which nity. Upon discharge from a hospital setting, either fam-
can enhance the performance of functional transfers. ily members, other caregivers, or both share the burden
Active wrist extension enhances the use of a tenodesis of care. Medical insurance programs have required reli-
pinch between the thumb and index fingers to pick up able data on which to determine benefits, including cov-
objects. Restoring grasp, grasp release, and lateral thumb erage of durable medical equipment, treatment, and care-
pinch assists grasp for eating, grooming, and dressing. giving assistance (16). The use of an ADL index to
Neural prosthesis implantation became available in measure and define disability has become prevalent as a
the 1990s. This device provides lateral and palmer grasp means to predict a number of issues (17).
for persons with C5 and C6 motor level SCI. The neuro-
prosthesis has both internal and external components, and
Measures of Functional Independence
early studies have indicated that there is a high satisfaction
level with the use of this technology (12). Another surgi- The most widely used ADL scale today in the U.S. is the
cal advance has been made in the use of myoelectric func- FIM, which evaluates nine areas of self-care (including
tional electrical stimulation (FES) in the UE to increase transfers) and has demonstrated a high degree of inter-
ADL function in people with tetraplegia by creating an rater reliability (18). There are eighteen motor and cog-
artificial grasp. Postoperatively, training is done to assist nitive items in total that are measured and compared from
relearning grasp and release techniques for items such as admission to discharge. These components of the FIM
eating utensils, cups, pens, and the telephone. More com- indicate disability caused by physical impairment versus
plex tasks, such as pouring, washing, brushing teeth, and cognitive or communication deficits. It has been used as
handling computer discs have also been demonstrated a predictor of the hours of care-giving assistance that will
(13). The preferred targeted population is the person with be needed (19). In terms of self-care, the FIM specifically
complete C6 tetraplegia, but someone affected with cen- reports the ability to self-feed, groom, bathe, dress the
tral cord syndrome, with an upper motor neuron injury upper and lower body, toilet, and transfer to the toilet and
to C8T1, could be considered. If the anterior horn cell, tub or shower. These measurements do not examine self-
root, or peripheral nerve is involved, FES is not recom- care skills that are typically a part of the rehabilitation
mended. To utilize grip, the hand must be able to be placed program for the individual with higher-level cervical
in space with elbow flexion volitional control and elbow injuries (C1-C5 motor complete), including the use of a
gravitational extension when sitting upright. computer, an ECU, etc. When a sample of occupational
In the person with complete C5 tetraplegia, assis- therapists treating patients at regional SCI centers
tance can be provided with a MAS or the new multijoint responded to a questionnaire, they noted the FIM as prob-
FES system to ensure elbow stability when grasping and lematic in detecting progress for patients with high cer-
releasing (14). Following a bilateral UE tendon transplant vical SCI or disease (20). A trend also exists in the U.S.
696 REHABILITATION
towards using the Minimum Data Set (MDS), a system As the person with SCI is being assessed for the
that has incorporated some aspects of the FIM. application of technology, care must be taken to recom-
The Quadriplegic Index of Function (QIF) is mend devices that will be useful and acceptable to the
another functional measurement. Although the QIF in its final user. A significant number of devices prescribed to
entirety is more sensitive to progress with different activ- patients with tetraplegia during rehabilitation are dis-
ities, it is more time-consuming to administer than the carded by the second year post-injury (26). This may be
FIM. The QIF has been noted to be redundant; there has indicative of continued strengthening or innovation that
been some research regarding reducing the number of occurs in the community. It is important to properly eval-
items to increase the potential for practical clinical uses uate the need for expensive technology to ensure that
(21). Whereas the QIF excludes application to the patient there is a long-term matching of technology and need.
with paraplegia, the Spinal Cord Independence Measure Needs also can change based on a number of factors, and
(SCIM) was designed for all patients with spinal cord the technology must be flexible enough to adapt to these
lesions. It also has the characteristic of being more sen- changes. One study indicates that, although quality of life
sitive than the FIM and has a high degree of interrater improved for persons with SCI using AT devices, the user
reliability (22). environment, adequate training for use of the device, and
There is much activity to develop standards around the match between product and user greatly influence the
the disability experience based on the medical model. ability to perform functional tasks (27). The development
There is also, however, much debate about treating dis- of the AT field is newer, in relation to education and
ability as a more complex syndrome, rather than render- health fields, under the umbrella of rehabilitation; it is in
ing it as a discrete, comparable experience (23). The work the process of developing methods to measure outcomes
in progress of the International Classification of Func- so that the system is driven towards higher efficiency (28).
tioning and Disability identifies that one can be both
healthy and disabled (24). Each individual, no matter
Architectural Barriers and Universal Design
what type or how complete or incomplete his injury is,
will present his own unique sequelae, such as the age Accessibility can make a tremendous difference in the per-
process, culture, role in the family and community, or formance of everyday living activities. Evaluating archi-
medical complications. These factors affect the ability or tectural barriers within the community has evolved into
the desire to perform certain aspects of self-care and par- a practice of universal design (e.g., design features that
ticipate in the community. are inherently accessible to persons with any type of dis-
ability, as well as suiting needs for all people). An exam-
ple of this is modifying curb cuts with a variable texture
Technology
to signal a blind user of a traffic boundary (29). Designs
Many examples of assistive technology (AT) are utilized that benefit wheelchair users also benefit the nondisabled
by the individual with SCI. These can range from some- user. Curb cuts, for example, are used for bicycles and
thing as simple as elastic shoelaces to assist with donning strollers as well as wheelchairs. In the past, the concept
shoes or as complex as a radio-wave, voice-activated ECU of adaptable design features was often used, which
to operate the telephone, computer, or other devices that entailed adapting the design as needed for the individual
plug into an AC outlet. Increasing examples of medical user. These kinds of features often involve a higher cost
technology involve the implantation of devices within the than universal design (30).
body to impact self care (neuroprosthesis for grasp, blad- Starting within the hospital environment, it is cru-
der stimulator, or baclofen pump). cial to arrange the room so that there is reasonable access
The technology available can be low in cost or to the bathroom, bed, call system, communication sys-
extremely expensive, based on how wide a market it is used tems (such as a telephone or computer), and leisure inter-
for. As society changes, the market for AT changes, and ests (stereo system, TV, etc.). It is not always possible to
manufacturers continue to seek to improve products for simulate the home environment during hospitalization;
wider application (25). For example, the universality of a however, efficient access to adaptive equipment (such as
nonskid surface is much less expensive as a rug-holding ICP supplies for night use) helps the person with SCI with
product than a similar product that is sold through med- future problem solving on organizing and arranging nec-
ical catalogs. Occasionally, a member of the rehabilitation essary items within the home environment.
team can improve on a commercially available product Essential access to residential settings often entails
through a minor adaptation. Of course, this type of adap- modifications to the entry, bathroom, kitchen, and bed-
tation often may nullify the warranty on the product. Least room. Entry access often necessitates the construction of
desirable is to have a custom-fabricated device, which a ramp. Ramp access should include nonslip material that
increases the cost of the product and adds complexity to stands up to any number of inclement conditions. The
issues like servicing and repairs and liability considerations. grade should be 8 percent or less; include level platforms
ACTIVITIES OF DAILY LIVING 697
for rest, if it is over 30 feet in length or at the location of adaptations added on to increase access, if access changes
a 180-degree turn; and have two handrails along the length were found to be an undue hardship for the owner.
of the ramp. In snowy or rainy climates, consideration
must be taken for maintaining of the ramp surface; possi-
Self Advocacy and Directing Care
bly adding an overhead canopy will ease this requirement
as well as protect the ramp user from the elements. The theory behind the United States independent living
Door and doorway configuration is variable for movement is that persons with disabilities prefer to view
exterior and interior entries. At the entry, there needs to themselves as consumers, not patients, capable of inde-
be adequate space next to the door to allow the wheel- pendence with supports. The disability rights movement
chair user to be out of the way when opening the door. has propelled thinking toward the redefinition of dis-
Typically, a platform or porch that is 5 5 feet is ade- ability away from earlier concepts of the medical model,
quate, depending on the size of the mobility equipment which focuses on limitations of physical functioning.
being used. The management of an additional screen door Instead, self-advocacy means that disability rights activists
poses an additional challenge. Inside the home, interior can apply pressure on society to provide people with dis-
doors are typically narrower, especially the bathroom abilities with what they need to live more independently
door. Making a right-angle turn from a hallway into a (31). In one study of women with SCI, one of the new
door often requires a turning radius that calls for a wider occupational roles that all took on was that of self-advo-
doorway. This can sometimes be resolved by changing the cate, especially in the areas of architectural and attitudi-
swing of the door, using special hinges that align the door nal barriers (32).
flush to the frame, or removing the door and using a cur- Another new role that people with SCI often find
tain cover for privacy. themselves in is that of employer. Hiring attendant assis-
Frequently, bathroom fixtures need to be reconfig- tance can follow a variety of methods; for example,
ured to allow the person with SCI access into the room. through an agency or advertising in the paper. Factors to
For the individual with outstanding safety and medical consider when self-hiring are whether or not the person
issues, having to perform multiple transfers for bathroom with SCI wants to track such details as withholding
functioning is not recommended; this may precipitate the social security and income taxes during each pay period,
need for a roll-instyle shower. Ideally, there should be and confronting the employee about dissatisfaction with
enough space in the bathroom to store any associated job performance. The components of hiring an attendant
adaptive equipment. Ceiling tracks can be installed to involve checking references, setting up a schedule of
offer another option for dependent-style transfers with assistance, opportunities to train on skills and evaluate
less bulky equipment. performance, and evaluating the compatibility of the
Kitchen access ideally allows the user to circulate relationship.
evenly between the triangulation of stovetop, sink, and Often, having an attendant trained in the hospital
refrigerator. Most kitchen counter heights are prohibitive setting on those tasks with which the person with acute
to use from a wheelchair; setting up a 30- to 32-inch height SCI will need assistance is a good way for the person to
workspace is crucial to preserve the body mechanics and initially assess attendant competency and compatibility.
energy of the kitchen user. Kitchen appliances continue to Learning to direct caregivers appropriately is a skill of
be generally designed with better ergonomics for all users. self-advocacy that can begin in the hospital setting. The
Of course, older-model appliances can be adapted to fit the medical team may be able to provide constructive feed-
need of the user (e.g., refrigerators can have a loop added back about how to organize and frame requests for assis-
to the handle to accommodate decreased arm and hand tance as well as visually and descriptively orienting the
strength). Those refrigerator/freezer models that are most patient to tasks where visual components are normally
accessible are the side by side configuration, or one that obstructed (e.g., wheelchair repair, bowel care, etc.) Hav-
has the freezer unit on the bottom. ing a back-up plan for attendant care is also important,
Within the community setting, accessibility can mean and often means hiring more than one person (33).
many things. The most comprehensive civil rights legisla-
tion to pass in recent years is the Americans with Disabil-
Environmental Control Unit
ities Act (ADA). It covers five basic areas, including access
to employment, state and local government, public accom- ECUs can provide a number of functions to the person
modation, telecommunications, and transportation. The with SCI, including accessing the telephone and electron-
ADA has promoted accessibility in the new construction ically operated appliances, such as a computer, bed, fan,
of public spaces and with major remodeling. Federal build- or stereo; operating lights or thermostat,or opening a door.
ings and services were previously mandated to be accessi- The most-favored functions reported by users were to
ble in the 1970s. Many inaccessible buildings were built access the telephone and control the television. Although
prior to these legislative requirements and may not have studies have shown that use of an ECU decreased the need
698 REHABILITATION
for direct attendant care time, these devices are frequently having a book holder helps to stabilize the reading mate-
not obtained for injured persons after discharge. Practi- rials while turning pages. It may also provide comfort at
tioners qualified to recommend ECUs for use in the home the cervical neck for prolonged reading. The size, qual-
often cite a lack of funding as prohibitive (34). ity, and weight of the book or magazine pages will require
ECUs can be quite simple in function, depending on adjustments to the skill of turning pages.
the need of the individual. Similar to computers, the range Writing typically requires the use of the shoulder,
of inputs to operate the device can involve voice recog- elbow, wrist, and hand in individuals with normal
nition; and infrared, pneumatic, or pressure switches, typ- strength. To compensate for the lack of muscular coor-
ically with dual action. It is often necessary to set up the dination, the range of adaptive devices includes use of a
ECU for multiple position use (e.g., being able to access mouthstick, splints, use of a variety of UE orthotics, or
electronic items while in bed or from the wheelchair). built-up surfaces on the writing utensil, depending on the
Additionally, it is important to prevent a complex set-up type of weakness. The writing implement easiest to use
that necessitates constant intervention: a headset tethered tends to be a felt tip or antigravity pen.
to a computer workstation for sip/puff switch use of a Technology for telephone use continues to evolve, and
telephone may interfere with convenient wheelchair tilt- many individuals prefer to carry a cell phone while out in
backs for pressure releases. the community. Within the home setting, depending on the
physical capabilities of the person and the technical capa-
bilities of the phone, various configurations exist. Push-but-
Computer Access
ton phones are the easiest to dial, and many phones have
Computer access promotes contact with a wider array of the capability of programming frequently used phone num-
individuals and activities and continues to be an increas- bers into memory, thereby reducing the number of buttons
ing trend in todays society. More long distance online pushed. Speaker phones are frequently used, however, this
educational courses and degrees are available over the limits the privacy of the conversation. Another method is
Internet (35). This helps to decrease the mobility require- to utilize a gooseneck set-up that positions the receiver at
ments that the person with SCI faces in educational pur- head level, along with a toggle switch to turn the phone
suits. Electronic discussion groups help to connect indi- on and off. Many phones are now cordless and allow the
viduals with similar interests. In addition, the educational receiver to be carried around the home while maintaining
component of using a computer within the acute reha- good reception. These phones can also be interfaced with
bilitation setting enhances patient learning and self-advo- a headset and an adaptive method of accessing the
cacy. Another big use of the Internet is e-mail. And, the answer/hang up function on the receiver. Additionally, there
ability to use a computer can greatly enhance the poten- are adaptive products, such as a sip/puffactivated phone
tial for employment outcomes. and computerized phone functions that the individual with
In evaluating which alternative computer access high cervical level SCI may use.
should be selected, it is important to match the technol-
ogy to the persons endurance. The reliability that the
technology provides for various computer function (key-
BASIC SELF-CARE
board entry versus mouse function, file management, etc.)
must also be determined. Finally, the feasibility of use in
Self-feeding
educational or vocational applications should be calcu-
lated (36). There are many alternative input methods for Impairments to arm and hand function impact self-feed-
a computer, including but not limited to key latches, voice ing. This can be overcome to varying degrees through
recognition, word prediction, Morse code, using minia- adaptive equipment and technique. In the acute SCI stage,
ture or alternative keyboards with typing sticks, and using people generally have precautions and conditions to work
a keyboard or mouse emulator on screen (35). aroundcertain movements, orthotic devices to protect
spinal integrity, and initial weakness. Ultimately, using
adapative equipment, the individual with limited arm
Other Desktop Activities
placement and tenodesis hand function tends to be inde-
Writing, reading, and telephone use are skills that can pendent for the majority of self-feeding tasks.
apply to leisure and vocational activities. For those indi- Someone with a high-level injury can be independent
viduals without hand or wrist function, the use of a in drinking fluids using a secured drink container and a
WDFH or ratchet splint is one solution for holding a typ- straw at a stationary location; this requires head and neck
ing stick or writing utensil. Turning pages in a book can movement to access. The mount for a drinking container
be accomplished through wetting the ulnar portion of the on a power wheelchair must accommodate the various
hand and pulling the page across, using a typing stick or angles the chair can be positioned in, to prevent spills and
mouthstick, or using an electronic page turner. Often, possible skin damage from hot liquids. Additionally, the
ACTIVITIES OF DAILY LIVING 699
dimensions of a power wheelchair must be taken into con- Turning on a faucet requires that a person have ade-
sideration when attaching a beverage mount, to ensure quate reach to the handle. Handle-free electronic faucets
that the container can be easily removed for refilling and work with a motion sensor at the faucet head. Individu-
cleaning, and to allow the user appropriate access to their als lacking trunk control may need to use compensatory
environment. Many manual wheelchair users also opt for arm movements to flex their trunks more forward to
a beverage mount on the wheelchair. The placement of access the stream of water or the bowl of a sink.
the device should optimize the convenience of retrieving Many of same principles from self-feeding apply to
and replacing the drink container, minimize spillage, and dental hygiene, such as handling a cup for rinsing and
not interface with wheelchair operation. spitting. To compensate for a lack of grasp, it is possible
Beverage use can be independent for someone with to brush the teeth by maneuvering the toothbrush by
adequate shoulder and elbow strength to allow hand-to- rotating the arm. Changing from wrist extension to wrist
mouth pattern movements from a table surface. The flexion while the toothbrush is held in place with the
method by which a beverage can be picked up or set down mouth or through use of an orthotic device, or building
can be adapted, as well as the cup or glass itself. Tenodesis up the handle, increases ease of manipulation. Many peo-
is used to pick up a glass. The amount of fluid or the ple prefer to use an electric toothbrush for greater ease
weight of the drink container can make a difference in the and thoroughness. Managing toothpaste can be done in
ability to hold the drink in a stable manner. Generally, the a number of ways, the easiest being suctioning it right into
use of a straw is helpful to compensate for the lack of neck the mouth or positioning the toothbrush underneath the
movement or trunk stability as the container is approxi- dispenser and using leverage. Flossing can be accom-
mated to the mouth. Some people use a ratchet splint or plished with a commercially produced dental floss holder;
WDFH splint to pick up a beverage. these holders can be adapted to accommodate those indi-
The difficulty of consuming other food items varies viduals who lack finger movement.
because of food texture or consistency. Often, the person For individuals without finger function, facial shav-
with acute SCI will begin training with finger food items or ing can be accomplished most easily through use of an
scooping food with a spoon. Because of weakness, training electric razor. Generally, the on/off button is high-pro-
on self-feeding is often assisted through the use of a MAS file or pressure sensitive, so that it can be manipulated
or overhead sling. Positioning a utensil in the hand can be with the end range of thumb extension. The handle and
accomplished through use of an orthotic, a palmar cuff, or design of the razor is often easy to use with tenodesis,
even weaving the utensil through the fingers, provided there but these can also be adapted with an additional han-
is adequate joint tightness. Additionally, utensils can be elon- dle. Persons using safety razors can use adaptive handles
gated, angled, mounted on a swivel, or have the handle built to allow the blade to be rotated, or they may handle a
up to adapt for a lack of wrist or hand movements. razor using a splint. Safety-razor use necessitates access-
One example of an adaptive method is to mount a ing the dispensers for shaving cream, which can be
fork with the tines angled downward to gain better lever- adapted if the user cannot leverage pressure over the dis-
age for spearing a food item. Cutting food and opening pensing mechanism.
packages tends to involve the highest level of strength or Shaving the armpits or legs is a bigger challenge,
control, but a number of devices are available to open involving adequate trunk stability to lean forward to
soda cans or bottles, and adaptive knives for cutting up access and/or pick up and position legs or to compensate
food are also available. for loss of balance when lifting up one arm. Shaving the
legs may be performed on a bed or wheelchair, depend-
ing on the persons balance skills. For tasks such as don-
Hygiene and Grooming
ning contact lenses, there are often commercially avail-
It is helpful for the person using a wheelchair to have access able methods to reduce the steps to completing the
to a sink that can be approached frontally. Adequate sink activity (e.g., contact solutions that both cleanse and wet
countertop space also provides room to easily access items, the contact). Setting up a variety of positioning and han-
rather than having items tucked into drawers. Sinks with dling adaptations for tasks such as cosmetic applications
a single-lever faucet provide enhanced access to the person may also be necessary.
with a weak grasp. As an alternative, a wash basin on top
of an adjustable-height table can be used, if sink access is
Bowel and Bladder Management
prohibitive. Because of bathroom construction, many
bathrooms are inaccessible to a wheelchair. For individu- The medical team collaborates with the person with SCI
als with limited walking or standing skills, these same prin- on how best to manage the bowel and bladder systems.
ciples of sink access apply; in fact, the countertop may need Various adaptive equipment and methods can be used to
to be raised to help support the individual to maintain the promote independence. In terms of bladder management,
most stable upright position. the individual who uses an indwelling or condom catheter
700 REHABILITATION
with an accompanying drainage bag has a number of mits access to a rolling shower chair to conserve energy
options. The drainage bag can be placed at belly level, knee for other tasks (this equipment decreases the need for
level, or most commonly, below the knee. The level at multiple transfers and therefore, the time for completing
which the drainage bag is placed is based on arm strength, a routine that involves both toileting and bathing).
trunk stability, and possibly the ability to lift the leg to Bathing can be safely accommodated from a seated posi-
access the bag close to a toilet or floor drain. Another tion. The person who utilizes a shower/tub combination
option is the electric leg bag emptier, which allows switch must have adequate strength and balance to manage mov-
access operation (pneumatic versus pressure switch) for a ing the legs over the tub wall during a transfer to a tub
person with a higher-level cervical injury. bench. Independence with full-body bathing requires at
Lower body clothing can often be adapted to allow least the use of adaptive equipment, tenodesis hand func-
easy access, depending on the method being used. For tion, unassisted arm placement, limited trunk stability,
those individuals utilizing an ICP, clothing management and the ability to transfer on and off bath seating. Typi-
skills are crucial, as are unassisted arm placement, ten- cal equipment can include a long-handled sponge or scrub
odesis hand function, limited trunk stability, and the abil- brush, a hand-held shower nozzle (with an adapted han-
ity to utilize adaptive equipment. Following initial dle if necessary), a pump-style or adaptive soap dispenser,
injuries, training for ICP might involve the use of prism and a bath bench or shower commode chair with a pos-
glasses for the individual with cervical bracing to allow sible chest belt adaptation.
visualization of his anatomy.
The body mass distribution of many females, with
Dressing
wider and heavier hips, creates a heavy workload for
pulling pants down and back up over hips to perform ICP. To independently dress the upper body, the person must
A preferred method might entail access through the crotch have at least limited to normal arm placement, tenodesis
of the pants with Velcro or snap closures or the use of hand function, and limited trunk stability using adapta-
a skirt. For all people using ICP, the portability and stor- tions such as loops, Velcro closures, and a buttoner or
age of catheterizing supplies also must be planned for. zipper pull device. Thumb loops can be applied to
Standard catheterizing supplies can include tubes of lubri- brassiere closures or abdominal binders. The various
cant, cleaning supplies (often baby wipes are sufficient), orthoses for cervical-level injuries (specially the halo
possibly leg loops to spread legs, a standard or adapted brace) sometimes prohibit pulling an upper body garment
urinal, catheters, possibly catheter extensions and a ten- over the head. In this case, adaptations can be made to
odesis orthosis, adapted caps for lubricant, and adapted accommodate this lack of neck flexion by enlarging the
scissors if there is a lack of hand function. Additional collar. Being able to adjust the garment behind the back
equipment for women includes adaptive or standard mir- is also necessary. This may require developing a technique
rors and possibly a labia spreader. A surface area on for leaning forward enough in the wheelchair to allow the
which to set these supplies is also needed. back of a garment to be positioned.
In terms of bowel management, the goals are to cre- Dressing the lower body tends to be performed in
ate a routine time for regular bowel evacuation and to bed, to allow the person a multitude of positions from
avoid incontinence. Often this routine is combined with which to leverage clothing onto the lower body. For
bathing, for energy conservation and thorough clean-up instance, the individual wearing a TLSO, with normal
afterward. Skills needed for independence include (at a arm strength, can utilize loops to position the legs for cap-
minimum) unassisted hand placement, partial trunk sta- ping a pant leg over a foot while the back is supported
bility, and tenodesis hand function. Being able to sit (through use of an electric bed, wall, or headboard). Once
upright for the bowel routine often lends quicker results the body jacket has been discontinued, this same indi-
and easier clean-up. Transfers to padded toilet seats are vidual can progress to other methods of dressing minus
more difficult than bed-to-wheelchair transfers, given that adaptive equipment (e.g., from a side-lying position in a
there is less surface area for hand placement and the dan- regular bed, or even sitting in the wheelchair [the latter
ger of added friction to exposed skin over a vinyl sur- requiring significant arm strength]). Having skills to roll
face. For those individuals with limited to no hand func- side to side in bed is important for advancing the garment
tion, equipment such as a suppository inserter and digital over the hips when the individual is dressing in bed.
stimulator may be necessary. The person without hand function benefits from use
of adaptive closures or fastener devices. Often, it is pos-
sible to pull pants on by trapping material between the
Bathing
palmer surfaces of both hands, or using wrist extension
The facilities available for bathing have a significant inside the waist band. Devices used for lower body dress-
impact on the independence with which bathing can be ing include reachers, dressing sticks, and leg loops. Adap-
accomplished. Some people opt for a bathroom that per- tations such as thumb loops can be added to the waist
ACTIVITIES OF DAILY LIVING 701
and a lightweight vacuum. Canister vacuums or mop which uses a specialized wheelchair. Bicycles can be
buckets mounted on a wheeled platform help to conserve obtained with reciprocal or symmetrical hand controls.
energy. To keep floors clean, wheelchair users may want For persons with paraplegia, more physically ori-
to use a covered entryway with outdoor carpeting, which ented sports are available. Activities such as wheelchair
cuts back on the need to frequently maintain a floor racing, all-terrain exploration (dune buggies, motorcy-
(37,38). cles), basketball, tennis, skiing, golf, and aviation are
available through the use of device adaptations or a spe-
cialized sport wheelchair. Care must be taken with the
Leisure Activities/Play
seating component of specialized sports equipment to
Play is a meaningful activity for children that allows them ensure that skin integrity is not compromised.
to explore the environment and relationships between Several studies have demonstrated that athletes with
themselves or others. Use of AT can be one way that a SCI had higher self-esteem and happier mood affect than
child with SCI can participate in play with peers (39). their nonathletic disabled peers. A group of persons with
Leisure activities can consist of participation in activ- SCI described kayaking as a method to shift the focus
ities by oneself or with others. Research indicates a link away from the injury and to add meaning to their daily
between social participation and life satisfaction. One routine (41).
study about quality of time use for persons with SCI
demonstrated that there was higher unemployment with
Community-Living Skills
the consequential increase of free time (40). It is important
for members of the medical team to assess what sorts of Being able to resume attending educational, civic, or reli-
leisure activities have been meaningful to the SCI patient gious activities, participating in recreational or vocational
in the past. During the rehabilitation phase, the person pursuits, managing money and resuming the role of con-
with SCI may be able to resume familiar leisure pursuits sumer are all part of community-living skills. Accessing
as well as add new activities to his repertoire. A sense of transportation gives the person a wider array of choices
self can be developed by having a choice over the kinds of and interests. Many communities now have public trans-
activities that one can select from; having choices is more portation with design features that include a wheelchair
inherent in leisure activities than in other ADLs. lift. This is a great bonus for both manual and power
For the person with high-level tetraplegia and ade- wheelchair users. The public ground transportation user
quate head and neck control, various tabletop activities, needs to be familiar with negotiating whatever mobility
including using a mouthstick in painting, playing cards, device is being used on a wheelchair lift and how it
board games, word games, or computer activities (such accesses the passenger space. It is also crucial for the pas-
as composing music, discussion groups, graphic design, sengers safety that he is able to direct assistance on how
etc.) are available. The use of a mouthstick can begin once to stabilize the device securely within the vehicle. Con-
the external brace (cervical orthosis) is removed. Mouth- temporary urban design features more pedestrian-friendly
stick composition involves fabricating or obtaining a environments that entail closer access to most desired des-
proper fit of orthotic within the oral cavity to decrease tinations. This may preclude the use of public trans-
the possibility of tooth or temporomandibular jaw dys- portation for power wheelchair users (the typical mileage
function from occurring. These orthotics are often cus- from a fully charged wheelchair battery may be 10 miles
tom fitted by a dentist. The terminus appliances should or less). More complex transportation such as flying can
be designed to allow change of use between appliances mean transporting other adaptive equipment besides the
(e.g., from use of a pen to paint brush or typing stick). wheelchair on the flight. Most airlines insist that the trav-
The appliances are typically stored at a docking station. eler with a disability must travel with a personal care
The ability to adjust the length of the mouthstick is also attendant if there are care needs anticipated on the trip
important in how activities can be accomplished. (transfers to a seat, bathroom needs, etc.) Being able to
Devices that attach to a wheelchair or other stable drive an adapted vehicle is another option.
surface can enable the person with lower cervical level Public accommodation within the community con-
SCI to engage in photography, hunting, fishing, and tool- tinues to demonstrate increased accessibility features. Per-
ing leather or wood. Switches can be adapted on a video sons with SCI can prepare for reintegration into the com-
game to allow a bigger switch target than is generally munity by contacting with the site prior to visiting it in
commercially available. Adaptations can be made to pool person. In this way, they can learn about the physical
sticks and table tennis or tennis racquet handles to allow characteristics or types of services offered that may help
playing games that require mobility skills. Water activi- accommodate them.
ties, such as swimming, sailing, canoeing, or kayaking can One environment that individuals with SCI will con-
be accomplished with set-up and/or adaptations to the tinue to frequent is healthcare clinics. In one study, per-
equipment. One game that is highly active is quad rugby, sons with SCI found the accessibility of healthcare clin-
ACTIVITIES OF DAILY LIVING 703
ics did not meet their needs, despite the indications by Young ones flourish under the attention of caring,
clinicians that the facility was accessible (42). Other envi- mature adults. Many individuals with SCI do not resume
ronments that persons with SCI might frequent include paid employment, and this choice may assist the family
grocery stores, which increasingly include timed electric unit in terms of one adult with more time available for pay-
eye door openers, which demonstrates the increased inci- ing attention to their child. Parents with disabilities face
dence of a universal design found in the community. enormous challenges, however, both financially and time-
There are a whole host of skills that are required wise. If the disabled parent develops medical complica-
for independence in the community, such as money han- tions, the available level of attention for childcare may be
dling, the ability to transport and access items from a compromised. It is important that parents solicit as much
wheelchair, problem solving how to deal with barriers, assistance as possible from other adults, especially in the
etc. Being able to transport needed items in a safe, acces- early years of childhood. It was noted in a study of moth-
sible place on a wheelchair is key for catheter supplies, ers with disabilities that the activities of physical play and
money, adaptive equipment, utensil holders, writing disciplining sometimes needs to be assigned to a person
equipment, etc. Within self-serve retail areas, such as a with normal strength and mobility (38,43).
grocery store, many items are out of reach. Being able to
carry a reacher is one solution to this difficulty (37,38).
Individuals with Internet access may prefer to use many CONCLUSION
of the online shopping services that also feature delivery
to the home setting for convenience. As technologic advances continue to develop, more peo-
ple with SCI have the potential to be independent with
ADLs. Additionally, lifetime costs through the use of some
Parenting
AT devices are likely to be reduced. On the other hand,
Special issues must be addressed during the parenting lengths of stay for acute rehabilitation have tended to
phase of infancy, including, but not limited to, posi- decrease, which may negatively affect the ability to live
tioning the infant for feeding, bathing, dressing, chang- independently. The need for documentation to demon-
ing a diaper, sleeping, traveling in a vehicle, and carry- strate the success and efficacy of various rehabilitative
ing the infant from place to place within the household interventions has continued to grow, to increase efficien-
or the community. Infants especially enjoy close prox- cies of funding by third-party payers.
imity to their parents, and it is important to preserve a Many societal factors of recent years have led
wheelchair users body mechanics through keeping the towards increased self-advocacy for independence. Peo-
work of parenting easily accessible. There are a number ple with SCI are a central part of the rehabilitation team.
of commercially available products that can be adapted They are often able to resume much of their self care as
to more easily accommodate a parent using a wheelchair. well as direct how they need assistance. In this era of con-
Often, an infant carrier can be securely affixed to a sumer education, they are often integral for advocating
wheelchair for transport around the household. Such extending their insurance benefits. This can help create
factors as how to easily disassemble this type of set up an opportunity for the person with SCI to work with a
must be taken into account because of the quickly chang- professional on increasing abilities to perform ADLs,
ing needs of an infant throughout the day. For the par- thereby affording more of an opportunity to fully partic-
ent with walking ability, carrying an infant may change ipate in the community.
the gravitational center of balance enough so that it
makes sense to temporarily use a wheelchair. Using a
wheelchair to transport an infant and the accompanying
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51
Recreation and Leisure
Skills for People with
Spinal Cord Disorders
Jo Lemons, T.R.
Nancy C. Cutter, M.D., P.T.
What you do during your working hours determines percent of respondents do not engage in organized leisure
what you havewhat you do during your leisure activities outside the home (8).
hours determines who you are. George Eastman The potential benefits of regular exercise and activ-
ity for persons with SCI include reduced mortality for
ecreation and leisure are some of the coronary heart disease, reduced rate of hypertension and
patient rehabilitation setting. The RT incorporates and dealing with discrimination, problem-solving skills
may offer community reintegration therapy, adapted unique to being disabled, time and energy management,
recreation, leisure counseling, wheelchair sports, aquatic coping skills, and adjustment issues. Frequently, patients
therapy, leisure skill building and leisure education for with SCI may not have had a healthy recreation and
each individual within the program. The full leisure leisure lifestyle before the injury. Sometimes, they were
assessment completed by the RT may include strength engaged in a high-risk behavior at the time of the injury.
and mobility, as in other therapists assessments, but also It is important that they receive education about alco-
includes recreation interests, attitude toward recreation hol, drug, and tobacco use and learn about alternative
and leisure, social and family support, and any previous means of socializing and relaxing. It is also important
leisure barriers before the injury. (See Leisure Assessment that they learn how to be self-advocates in a therapeu-
Form.) tic and successful way.
Adapted Sports and Fitness graphic location and often requires little adaptive equip-
ment? Art can also provide a needed creative outlet, social
The leading cause of death in persons with SCI is car-
interaction, personal enjoyment, and possible employ-
diovascular disease, sometimes at a premature age.
ment. Artists with disabilities not only reap these bene-
Because lack of exercise is a major risk factor for cardio-
fits, but also help to present disability in a context for
vascular disease, fitness assessment and monitoring and
the wider world.
encouragement of activity and fitness are important for
Famous artists with disabilities have had great
persons with SCI (13). Lack of physical activity has been
impact on the art world. Three visual artists with SCI rep-
found to contribute to secondary complications such as
resent very different types of art and views of disability.
urinary tract infections, pressure ulcers, respiratory infec-
Frida Kahlos very personal self-portraits reflect not only
tions, and spasticity (1417).
her view of disability, but that of the medical establish-
For those with SCI to participate in regular physical
ment. One of her most famous works, The Broken Col-
activity, the barriers to fitness activities must be addressed.
umn shows her wearing a metal brace, her body open to
As with the able-bodied population, barriers may include
reveal a broken column in place of her pins.
lack of time, energy, and motivation or lack of convenient
Chuck Close is an American photorealist who spe-
or affordable facilities. However, in the disabled popula-
cialized in close-up portraits. Since becoming paralyzed
tion, barriers may also include handicap parking issues,
in mid-career, he has changed his technique, but his paint-
wheelchair inaccessibility, and restroom and locker room
ings continue to be more about faces than bodies. Ricardo
inaccessibility. Lack of information about accessible fitness
Pealver had always raised social issues through his art.
programs and facilities, and lack of knowledge by the pro-
After he became paralyzed, paintings like Seated Man
gram staff about the specific needs of individuals with dis-
Dancing seem to reflect more personal issues surround-
abilities have been listed in the top ten issues of people with
ing disability, although still in a social context.
a disability in one study (18).
Art can be an expression of personality, a reflection
The recognition of the importance of physical fitness
of social issues or just an enjoyable pastime. The possi-
in SCI dates at least to 1948, when Dr. Ludwig Guttmann
bilities are endless. In the visual arts, classes in painting,
of the Stoke-Mandeville Spinal Injury Center introduced
pottery, sculpture, jewelry, and more are available
the first organized wheelchair sports program. The Stoke-
through local city art centers. These centers are typically
Mandeville Games included twenty-six participants in a
city run, and also through community colleges and local
few events. In 1952, a team from the Netherlands was
musical stores. Actors with disabilities can receive train-
invited and the concept grew. In 1984, an exhibition
ing through local acting schools, art centers, and colleges.
wheelchair track event was held at the Olympics in Los
People interested in literary arts can take classes at local
Angeles. By 1988, the Paralympic games coordinated
colleges or even online. There are integrated dance
with the Olympic games. Today, wheelchair athletes have
troupes throughout the U.S., which use dancers with and
gained more public respect and interest (19).
without disabilities, along with specific wheelchair dance
Despite ones skill level, ability, or disability, there
companies. Information on many of these companies and
is a sport or recreational activity for everyone. Many
opportunities can be found through the National Arts and
modified examples are described below. However, recre-
Disability Center.
ation is only limited by ones own ingenuity. New sports
One need not be an artist to enjoy the arts. For an
and recreational interests for people with disabilities are
audience member or arts patron, most arts venues are
being explored all the time.
wheelchair accessible. Wheelchair seating is being
improved, as new facilities integrate this seating through-
Indoor Recreation out the facility, and ramps are no longer designed for
back door entrances. Many museums and theaters are
Painting/Arts/Crafts also financially accessible, with free days and low-cost
tickets.
Some of the most gifted artists have had a physical dis- Many organizations provide resources and support
ability. Adaptive equipment varies from traditional tools for artists with disabilities. Four of special interest and
to using ones own wheelchair as the artists brush, such contact information are noted:
as at the program at Metro Health in Cleveland. Rancho
Los Amigos produces calendars and cards displaying the 1. The National Arts and Disabilities Center (NADC)
work of former patients. The Veterans Administration is the national information dissemination, techni-
and other organizations provide annual displays and cal assistance, and referral center specializing in the
works of art. field of arts and disability. The NADC is dedicated
What other form of recreation is accessible to all to promoting the full inclusion of children and
people with SCI, regardless of age, sex, ability, or geo- adults with disabilities into the visual-, performing,
708 REHABILITATION
media, and literary-arts communities. Its resource the citizens, businesses, and organizations of the City of
directories, annotated bibliographies, related links, Phoenix to experience and understand other cultures
and conferences serve to advance artists with dis- through the development of long-term, international part-
abilities and accessibility to the arts. nerships. It is a not-for-profit organization that is offi-
cially authorized by the City of Phoenix, and has as its
National Arts and Disability Center core mission the promotion of international relationships.
Tarjan Center for Development Disabilities The mission is accomplished through a variety of pro-
300 UCLA Medical Plaza Suite #3310 grams, including student, educational, cultural, and eco-
Los Angeles, CA 90095-6967 nomic development exchanges that form bridges of inter-
Phone (310) 794-1141 national understanding. The Disabilities Committee is
Internet: http://nadc.ucla.edu able to study, and ultimately propose, how PSCC can fully
incorporate persons with disabilities into all programs
2. The World-Wired-Wheels Art Gallery is an online and activities, as well as create forums for learning and
gallery and support system for artists using wheel- awareness with, and for, the eight sister cities: Calgary,
chairs, a Paralinks service for disabled artists. Canada; Chengdu, China; Ennis, Ireland; Grenoble,
Internet: http://www.paralinks.net/wiredwheelsart. France; Hermosillo, Sonora, Mexico; Himeji, Japan;
html Prague, Czech Republic; Taipei, Taiwan.
The exchange of information about accessibility, atti-
3. The Association of Mouth and Foot Painting Artists tudes towards disability, attitudes towards recreation, and
(AMFPA) is an international association that helps troubleshooting barriers with cooperative partnering, on
artists to distribute their artwork. The AMFPA rep- a global level, has been found to be very successful.
resents 500 mouth- and foot-painting artists in 60
countries around the world.
Writing/Poetry
AMFPA (Association of Mouth and Foot Painting With computer assistive technology, the writer is only lim-
Artists) ited by his own imagination.
9 Inverness Place,
London, W2 3JG
Movies/Theater
Phone 0171 229 4491
Internet: http://www.amfpa.com Most modern theaters have wheelchair-accessible seating
in accordance with the Americans with Disabilities Act of
4. VSA Arts is a national organization dedicated to 1990. These are excellent activities shared by friends and
promoting the creative power in people with dis- family.
abilities. They offer a number of programs, includ-
ing an on-line gallery for artists with disabilities.
Television
1300 Connecticut Ave NW Television is the most common recreational interest of
Washington, D.C. 20036 persons with SCI. It is recommended that other activities
Phone 1 800-933-8721 involving more active participation be encouraged.
Internet: http://www.vsarts.org
Outdoor Recreation
Reading
Aquatic Therapy Program
Reading is enjoyed by all ages and materials can be
obtained through programs like Talking Books, available Therapeutic aquatic exercise is a goal-directed exercise that
at most libraries. Adapted book holders are available, and takes place in a warm pool. Therapeutic pool temperatures
some may have the option of turning pages for the reader are between 90 and 94 degrees F. This temperature closely
if desired. resembles skin and body temperature. The warm water
provides relaxation, support, resistance, increased circu-
lation, and decreased pain and pressure (hydrostatic pres-
International Involvement
sure) to all parts of the body. The pool is a wonderful way
The Phoenix Sister Cities Commission (PSCC) is one of to increase strength and flexibility, and to develop a great
only a few Sister Cities that expands its focus to the dis- exercise program. Aquatic therapy is provided by RT,
ability issues at hand. The Commission is dedicated to physical therapists (PT), and occupational therapists (OT)
creating exceptional people-to-people opportunities for with specific training in the specialization of aquatic ther-
RECREATION AND LEISURE SKILLS FOR PEOPLE WITH SPINAL CORD DISORDERS 709
Hunting
Individual state law regulates hunting with either a bow
or a gun. Many states allow individuals with disabilities
to hunt from vehicles and/or may issue free licenses. Infor-
mation on specific state regulations may be obtained from
each states wildlife management service.
Rock Climbing
Training for rock climbing typically starts by working in
an indoor gym, supervised by trainers. As the participant
progresses with her level of comfort and knowledge of
equipment and functional status, she may continue this
adventurous activity in the outdoors.
FIGURE 51.2
Sky Diving Basketball is one of the earliest adapted sports. It does not
require much additional equipment. Able-bodied athletes can
Sky diving is usually completed as a tandem jump with a compete if they are brave and have a wheelchair. Picture cour-
professional skydiver; generally weight limits for this tesy of Sports-n-Spokes.
activity are imposed.
Water Sports
Adapted watercraft have allowed many people with
tetradriplegia, paraplegia, and other disabilities the
opportunity to drive their own watercraft on the water,
at greater speeds. This modification is available through
Innovative Mobility provided for the Barrow Neurolog-
ical Institutes Day on the Lake event, held every June,
at Bartlett Lake Marina in Carefree, Arizona.
Power Soccer
This active game is played by power wheelchair users
using an 18-inch physio-ball. Four players make up a
team. The ball is played on a regulation basketball court
and the ball must be pushed over the line of play to score.
A Plexiglas guard is adapted to fit over the wheelchair
users feet and is used to control the ball. More details
may be obtained from the Sharp Rehabilitation Center.
FIGURE 51.3
Quad Rugby
Downhill skiing requires specialty equipment and training.
This is a competitive sport, reserved for only the strong The athlete uses two outriggers instead of poles. Most ski
of heart. The game was given its name because of the areas have modified skiing training centers. Picture courtesy
spirit of its intensity. It is played by persons with quadri- of Sports-n-Spokes.
plegia, on a basketball court, in four-member teams. A
volleyball is used to carry across the goal line. A sepa-
rate classification is used to rate the players disability on Snow Skiing
a scale of 0.5 to 3.5. A team is limited to a total of 8
points. The game is regulated by the U.S. Quad Rugby This sport can be for recreation or competition. Sports
Association. include downhill racing, slalom, and giant slalom (Fig-
ure 51.3). Modifications are completed with the sit-ski,
mono-ski, bi-ski, and outriggers. More information can
Road Racing
be obtained from the Disabled Sports, USA (DSUSA), and
This sport dates its heritage to the 1975 Boston the United States Ski Association.
Marathon, in which the first wheelchair athlete partici-
pated. This sport can be paralleled with other road races.
Softball
The wheelchair athlete competes in a division separate
from nondisabled runners. The Wheelchair Athletes of This game is played with the 16-inch, slow-pitch softball
the U.S. oversees wheelchair racing. rules of the Amateur Softball Association. It is regulated
by the National Wheelchair Softball Association. There
is a national tournament held each September.
Trap and Skeet Shooting
In these sports, the disabled athlete can compete along-
Table Tennis
side the nondisabled athlete, using the same rules. Trap-
shoot rules can be obtained from the Amateur Trap- Athletes with impaired hand function may strap the
shooting Association and skeet shooting rules may be paddle to their hand as a modification for this game. It
obtained from the National Skeet Shooting Association. can be played as a single or double event. It is regulated
by the American Wheelchair Table Tennis Association,
and its rules are consistent with the U.S. Table Tennis
Sled Hockey
Association.
This competitive game began more than 30 years ago in
Norway. Able-bodied athletes may play as well as dis-
Tennis
abled athletes. The game is regulated by the American
Sled Hockey Association. It is played at a variety of lev- This is another sport in which the able-bodied and the dis-
els, including at the Paralympics. abled athlete may compete side by side. The U.S. Tennis
712 REHABILITATION
Weightlifting
Rowing
This sport can be enjoyed recreationally or competitively.
Able-bodied and disabled athletes can compete. Mini- Rowing requires only minimal adaptations such as
mal modifications are needed for the weight-training devices for the upper limbs. Able-bodied and disabled ath-
equipment. letes may compete. Information may be obtained from
the City of Tempe, Arizona; City of San Diego Recreation
Departments; or from the Philadelphia Rowing Program
Water Sports for the Disabled.
Canoeing/Kayaking
Sailing
This sport can be enjoyed by both able-bodied and dis-
abled athletes. Typically, people with a high level of injury Sailing is an exciting way to enjoy the water and relax. It
do better in larger, two-person kayaks. The Disabled Pad- can be recreational as well as competitive. Modifications
dlers Committee of the American Canoe Association can allow disabled sailors easier entrance and exit from sail-
be contacted for ideas and suggestions for adaptations boats. Several organizations are involved in disabled sail-
(Figure 51.4). ing. They include the National Ocean Access Project
(NOAP) and the U.S. Yacht Racing Union (USYRU).
Scuba Diving
This is another recreation enjoyed by both the able-bod-
ied and disabled enthusiast. The Handicapped Scuba
Association trains people with disabilities to scuba dive.
Water-Skiing
Imagine rushing 50 or 60 miles per hour on the water in
the hot sun. A modified tobogganlike ski with a seat (sim-
ilar to the sit-ski in downhill snow skiing) is used with
outriggers to pull an athlete behind a high-speed boat.
More information may be obtained from the City of San
Diego Recreation Department; or from the Recreation
Department at Saint Joseph Hospital, Phoenix, Arizona.
Classification
The National Wheelchair Athletic Association (NWAA)
has developed classification systems for wheelchair
sports. There are two basic types of classification systems.
The first type is based on the neurologic examination. The
second type is based on the functional level. Both require
a trained classifier to complete. The neurologic assess-
ment is used for most recreational and community events.
The functional level assessment is a more time-consum-
FIGURE 51.4
ing and elaborate system and is used at the Paralympic
Canoeing/kyaking requires minimal adaptation. Picture cour- level. Classification schemes are controversial but neces-
tesy of Barrow Neurological Institute Rehabilitation Program, sary, to allow fair competition between persons with sim-
St. Joseph Hospital, Phoenix, Arizona, Day at the Lake ilar disabilities. An example of the classification scheme
event. based on neurologic examination follows:
RECREATION AND LEISURE SKILLS FOR PEOPLE WITH SPINAL CORD DISORDERS 713
Class IAAll participants having cervical lesions, Classification must be based on careful neurologic
with complete or incomplete tetraplegia, who have examination, using an accurate muscle-testing procedure.
involvement of the upper extremities, weakness of The complete or incomplete nature of the spinal cord
the trunk and lower extremities, that interferes sig- lesion will influence motor performance and classifica-
nificantly with trunk balance and the ability to walk. tion. Preservation of sensation provides an advantage in
Class IBAll participants having cervical lesions, competition. Functional assessment of the competitor in
with complete or incomplete tetraplegia, who have his wheelchair or observation of the competitor during
involvement of upper extremities , but less than IA, an athletic event may be helpful, and at times essential, to
with preservation of normal or good triceps (4 or 5 make accurate judgment. This assessment is not part of
on testing scale), and with generalized weakness of the above-mentioned classification. The athletes full
the trunk and lower extremities that interferes sig- effort and cooperation is necessary. If the athlete does not
nificantly with trunk balance and the ability to walk. cooperate or attempts to exaggerate the nature of his dis-
Class ICAll participants having cervical lesions, ability, the classifier must place the competitor in a more
with complete or incomplete tetraplegia, who have difficult class in the interest of fairness to other competi-
involvement of upper extremities, but less than IB, tors. The classifier may request that a competitor be re-
with preservation of normal or good triceps (4 or 5 examined the following year. This may be helpful when
on testing scale), and normal or good finger flexion neurologic change is probable, when athlete cooperation
and extension (grasp and release), but without is questionable, or when there are unexplained inconsis-
intrinsic hand function, and with a generalized tencies in the examination. Full notes should be made to
weakness of the trunk and lower extremities that alert future classifiers to the issues in question. It is rec-
interferes significantly with trunk balance and the ognized that this system of classification is not perfect. It
ability to walk. cannot accommodate all types of disability. Flexibility
Class IIParticipants having complete or incom- should be exercised in difficult cases, recognizing that the
plete paraplegia below T1, down to and including purpose of the classification is to allow fair competition
T5, or comparable disability with total abdominal among athletes with a similar degree of disability.
paralysis or poor abdominal muscle strength (02
on testing scale) and no useful trunk sitting balance.
Class IIIParticipants having complete or incom- CONCLUSION
plete paraplegia or comparable disability below T5,
down to and including T10, with upper abdominal A good quality of life is the state of complete physical,
and spinal extensor musculature sufficient to pro- mental, and social well-being and is not merely the
vide some element of trunk sitting balance, but not absence of disease (20). It is entirely possible that
normal. increased involvement in recreational activities may
Class IVParticipants having complete or incom- improve the social, emotional, physical, and spiritual
plete paraplegia or comparable disability below T5, well-being of persons with SCI, thus resulting in an over-
down to and including T10, with upper abdominal all higher quality of life. In fact, the literature supports
and spinal extensor musculature sufficient to pro- that access to social and recreational opportunities is an
vide some element of trunk sitting balance, but not important indicator of quality of life (21).
normal. It is imperative that someone who has sustained a
Class VParticipants having complete or incom- recent injury be introduced during the inpatient rehabil-
plete paraplegia or comparable disability below itation stay to active mentors with SCI. He must also be
T10, down to and including L2, without quadriceps introduced to community resources and adapted to recre-
or very weak quadriceps, and with a value up to and ation and wheelchair sports opportunities offered in their
including 2 on the testing scale, and gluteal paraly- community. This can best be achieved through a thera-
sis. peutic recreation program designed for persons with SCI.
Control and Prevention, National Center for Chronic Disease Pre- 11. US Department of Health and Human Services. Healthy People
vention and Health Promotion; 1996. 2010 (conference edition in two volumes). Washington, D.C.: Jan-
4. Johnson KA, Klaas SJ. Recreation involvement and play in pedi- uary 2000.
atric spinal cord Injury. Top Spinal Cord Inj Rehabil 2000; 12. Guttmann L. Textbook of Sport for the Disabled. Aylesbury, Eng-
6(supp):105109. land: HM&M; 1976.
5. Anson C, Shepherd C. A survey of spinal cord patients: Medical 13. Arch Phys Med Rehabil 2000; Vol 81, 394.
and social characteristics. In: Trends: Research News from Shep- 14. Noreaul, Shephard R. Spinal cord injury, exercise and quality of
herd Spinal Center, Atlanta, Ga.: Shepherd Center 1990. life. Sports Med 1995; 20:226250.
6. Levi R, Hulting C, Seiger A. The Stockholm spinal cord injury 15. Curtis KA, Hall KM, McClanahan S, Dillion D, Brown DF.
study: 4. Psychosocial and financial issues of the Swedish annual Health, vocational and functional status in spinal cord injured ath-
level-of-living survery in SCI subjects and controls. Paraplegia letes and nonathlete. Arch Phys Med Rehabil 1986; 67:862865.
1996; 34:152157. 16. Fentem PH. Exercise in prevention of disease. Br Med Bull 1992;
7. Coyle CP, Kinney WB. Leisure 1990; 24(4):6467. 48:630650.
8. Harrison C, Kuric J. Community reintegration of spinal cord 17. Stotts KM. Health maintenance: Paraplegic athletes and non-ath-
injured persons: Problems and perceptions. Abstracts Digest letes. Arch Phys Med Rehabil 1986; 67:109114.
Annual Science Meeting American Spinal Cord Injury Association 18. Nary DE, Froehlich K, White G. Accessibility of fitness facilities
1987; 13:263267. for persons with physical disabilities using wheelchairs. Top Spinal
9. Pate RR, Pratt M, Blair SN, et al. Physical activity and public Cord Inj Rehabil 2000; 6(1):8798.
health: A recommendation from the Centers for Disease Control 19. Williams T, England M. Sociology, Physical Activity and Spinal
and Prevention and the American College of Sports Medicine. Cord Rehabilitation. Top Spinal Cord Inj Rehab 1997; 3(1):5666.
JAMA 1995; 273(5):402407. 20. World Health Organization, 1985.
10. Ross CE, Hayes D. Exercise and psychological well-being in the 21. Sewell L, Berry ET. Quality of life: Promoting sports and leisure
community. Am J Epidemiol 1988; 127:762771. involvement after spinal cord injury.
52 Vocational Rehabilitation
pinal cord injury (SCI) presents sig- people with high-level tetraplegia 14 to 24 years post
time when they would ordinarily graduate based on com- THE VOCATIONAL REHABILITATION
pletion of academic credits. PROCESS
IDEA requires that Individualized Transition Plans
be developed for each student on an IEP by the time that Many people with SCI have a history of employment and
student reaches 16 years of age. The Transition Plan iden- may even have a job to which they may return. As noted
tifies transition goals, the services necessary to achieve earlier, others will have had a marginal history of employ-
those goals, and who will provide those services. As part ment, have worked in physical labor jobs and have few
of a transition plan, a student might participate in vari- transferable skills, or have no work history at all. In either
ous kinds of career exploration, work tryouts, or perhaps case, the process of vocational rehabilitation begins on
vocational training. Transition goals might include post- the first day of admission to the inpatient rehabilitation
secondary education (e.g., community or technical col- setting, when the expectation is set that most people with
lege, university), employment, or community living. It is SCI can work at meaningful jobs with acceptable pay, and
the intent of IDEA that the transition process include a that the skills the newly injured person acquires in the
collaboration between schools and other key agencies early stages of rehabilitation are the building blocks for
including Vocational Rehabilitation. employment and independence later on. At the Univer-
Physiatrists can play a key role as advocates in the sity of Washington Medical Center and Harborview
transition process by ensuring that the functional limita- Medical Center, a staff of professional rehabilitation
tions of their patients with SCI are addressedbut per- counselors provides vocational rehabilitation and other
haps more important, that their functional strengths are counseling services to inpatients and outpatients. Newly
addressed. Physiatrists will have important information injured SCI patients are seen on admission and through-
about the impact of new assistive technology to enhance out their inpatient stay. As a member of the interdisci-
performance, seating and position modifications for plinary team, the rehabilitation counselor assists in the
employment, management of bowel and bladder issues, identification of educational and career interests, assess-
etc. Transition is a difficult time as students anticipate ment of academic and vocational potential, review of
moving into the world of work, employment, higher edu- existing skills, and counseling around issues related to
cation, and community with far fewer supports in place adjustment, perceived competence, and the like. On dis-
and a fragmented service delivery system. For those stu- charge, the counselor continues following the patient,
dents who have concurrent brain injuries or other cogni- often in collaboration with a state or community agency,
tive or emotional issues, transition can be even more dif- to support efforts to enter and maintain employment.
ficult. Families who do not feel they have received Whenever the process begins, it includes the fol-
appropriate services and have not found the school dis- lowing steps:
trict to be responsive may file complaints through the U.S.
Department of Education Office of Civil Rights, which
Referral
has offices in each state.
The physiatrist is often in an ideal position to identify a
person with SCI who can profit from vocational rehabil-
THE PROFESSION OF itation services. Not only should individuals who are not
REHABILITATION COUNSELING working be considered for referral, but so should indi-
viduals who are working, if there is an indication that
Rehabilitation counselors provide vocational rehabilita- they are dissatisfied with their employment. The voca-
tion and other counseling services to people with disabil- tional rehabilitation process should be driven by the per-
ities and provide consultation to families, employers, and son with SCI and supported by the rehabilitation coun-
healthcare providers (1). Professional Rehabilitation coun- selor and others.
selors have masters degrees in rehabilitation counseling
from graduate programs in rehabilitation counseling
Assessment
accredited by the Council on Rehabilitation Education or
closely related degrees. They have training in applied psy- The assessment begins with a review of records and an inter-
chology; individual, group, and family counseling; med- view with the client, significant others, family members, and
ical, psychosocial, and vocational issues in disability; indi- other key informants. The assessment process includes a
vidual and career assessment; career development; labor review of functional limitations and strengths and an inves-
market and employment issues; job placement; case man- tigation of current and potential barriers to employment.
agement; and service delivery systems. They complete A careful review of educational and a vocational history will
internships and acquire the Certified Rehabilitation Coun- yield a list of transferable skills as well as an employment
selor credential (16). In many states, they will sit for state history. An appraisal of the career interests may be useful
exams to become Licensed Professional Counselors. either through the counseling process or with career assess-
718 REHABILITATION
ment instruments such as the Strong Interest Inventory. Gen- bilitation, job stations or paid employment may serve this
erally, people are more satisfied in employment that is con- purpose. Job stations may be created by collaborating
sistent with their career interests, which may include, for with employers to have the client try out elements of a
example, preferences for working with data versus people, job for a period of time. For example, a 25-year-old with
or in activities requiring creative problem solving versus rou- paraplegia had worked as a welder prior to injury and
tine activities. Similarly, it may be useful to understand an was interested in returning to that line of work. The reha-
individuals temperament or personality style. This can be bilitation counselor set up a job station in the hospital
evaluated by reviewing history, interviews with the client metal shop where the client worked 3 hours a day, 5 days
and other key informants, observation, and/or psychologi- a week, for 3 weeks at metal fabrication and welding. The
cal assessments such as the Jackson Personality Inventory. shop supervisor worked with the client and the rehabil-
If potential rehabilitation goals include a return to itation counselor to evaluate the clients ability to per-
school or pursuit of occupations that require certain lev- form various tasks, and to develop potential accommo-
els of educational attainment, then it is necessary to eval- dations that would allow the client to work more
uate achievement by reviewing educational records, inter- independently. At the conclusion of the job station, the
view, and perhaps through achievement testing using client had increased his endurance for work, his confi-
instruments such as the Wide Range Achievement Test. dence, and had an employment reference from the shop
When inadequate evidence is available to predict the supervisor as he began his search for paid employment.
clients potential to benefit from education or to acquire Federal law allows 160 hours of unpaid employment as
or perform certain kinds of occupational skills, assess- part of the assessment process. Some clients, particularly
ment of aptitude may be appropriate. Generalized apti- those who have had concurrent brain injuries, will
tude for higher education (e.g., community college) may require on-site supervision and training from a job coach
be estimated from standard intelligence tests, or many during their job station. Under these circumstances, the
community college systems offer assessments. General job coach can collect data about the rate of acquisition
occupational aptitudes may be estimated through tests of skill, responsiveness to supervisory feedback, and
such as the General Aptitude Test Battery, and tests are other variables.
available to assess specific aptitudes as well.
When possible, the best assessment involves a review
of actual performance, interview, and real-world tryouts. Labor Market Analysis
The validity of paper and pencil tests is limited. For exam-
As part of the assessment process, the outlook for
ple, only 9 percent of the variance in freshman college
employment in various occupations is evaluated. The
grades can be accounted for by their scores on the
U.S. Department of Labor and state Departments of
Scholastic Aptitude Test (17). Nevertheless, paper and
Employment Security maintain statistics on trends in
pencil assessment instruments may be useful as part of the
occupations, including the number of people currently
career exploration process.
employed part time and full time in each occupational
Another approach to vocational evaluation involves
group, and the percent growth or decline predicted dur-
the use of work samples (18). Work samples are either
ing the next 5 years. Clients can identify the number
organized along various tasks common to many jobs or
of jobs currently available in an occupational group
tasks thought to represent a specific job. For example, one
within their immediate geographical area. They can
might have activities that sample the individuals ability
also find out the wages, tasks, educational preparation,
to manipulate small objects or demonstrate eyehand
and other characteristics of occupations. This infor-
coordination, as in the Valpar system. Commercially avail-
mation is readily available on the associated govern-
able vocational evaluation systems using work samples
mental Web sites.
often include norms that purport to allow the evaluator
to compare the performance of the person being evaluated
with a standard necessary for successful occupational per-
Counseling
formance. These predictions do not hold up well; however,
the vocational evaluation systems may provide a useful Many clients with SCI will find counseling useful to help
way for people with disabilities to try out a variety of occu- them re-evaluate their expectations, enhance their per-
pational activities and for the evaluator to collect obser- sonal adjustment, and understand and react to the per-
vational data about the way the client engages tasks. ceptions and biases of others around them (including fam-
ily members, potential employers, and coworkers). As
part of the counseling process, the clients may take stock
Situational Assessment
of the resources they bring to bear on the issues, the bar-
Situational assessment is the generic term for using real- riers they confront, and compensatory strategies to which
life situations to assess performance. In vocational reha- they have access or can learn.
VOCATIONAL REHABILITATION 719
Selecting a Goal capable, but that they were unable to imagine how he
could accomplish the essential functions of the job they
Through the counseling process, the client evaluates the had open because of his limited dexterity and inability
assessment data and develops a terminal goal and inter- to manipulate print materials. With the intervention of a
mediate steps. For example, a 19-year-old with tetraple- rehabilitation counselor and an assistive technology spe-
gia might choose working as an attorney as her long-term cialist, he was able to secure employment and has been
goal. To achieve this goal, she might develop intermedi- working successfully since.
ate steps which include: a) increasing sitting tolerance;
b) applying for readmission to an undergraduate pro-
gram; c) finding accessible housing; d) finding and train- Supported Employment
ing a personal care attendant; e) identifying transporta- Some people with SCI will require ongoing support in the
tion options; f) obtaining and learning to use adaptive workplace to sustain employment. For some, such as peo-
equipment including voice recognition software; g) begin- ple with high-level tetraplegia, that support may involve
ning classes; h) choosing a college major; i) reappraising assistance in positioning in relation to a task, manipulat-
her long-term goalinterviewing lawyers, law school fac- ing materials, inserting compact disks into computers, etc.
ulty, conducting research on work in the legal profession; They may also require personal care assistance in the
j) taking the Law School Admissions Test; k) researching workplace. Others, particularly those who have significant
various law schools; l) applying to law schools; m) apply- concurrent brain injuries, may require ongoing support
ing for scholarship and financial aid; n) accepting admis- in the form of job coaching. This support may be provided
sion; o) and finishing law school and finding employment. either by a coworker or by a professional job coach.
Having tetraplegia has reduced this students flexibility in
planning. She must complete the same steps she would
have prior to her injury, but will require additional plan- Job Placement
ning to achieve success. When the rehabilitation counselor is assisting with the job
placement process, she begins by searching for jobs that
will meet elements of the clients goal. When potential
Educational or Vocational Reentry
jobs are identified, the rehabilitation counselor conducts
Once the goal setting and planning is completed, the next a job analysis to determine the actual kinds of work done
step in the vocational rehabilitation process is educational in that particular job; the cognitive, social, and physical
reentry, as in the example above, or vocational reentry. In demands of the job; and potential accommodations.
vocational reentry, the client may be able to secure a job Then, in collaboration with the client and the employer,
independently, but often may require assistance in job the goodness of fit of the job with the client is evaluated:
development and placement from the rehabilitation coun-
selor. Although discrimination against qualified individ- Is this job a good match for what the client is seek-
uals with disabilities is illegal, discrimination at the point ing in terms of interest, skills, and distance from
of hiring is difficult to prove and may not be intentional home, social environment, pay, and benefits?
on the part of the employer. Employers may not have Can the client do the essential functions of the job
experience interacting with people with significant dis- with or without accommodation?
abilities and may not be able to imagine their working Given the size of the employer and the employers
productively. Advocacy and assistance in obtaining resources, are the accommodations reasonable?
employment is often an appropriate role for the rehabil- If the client cannot perform the essential functions
itation counselor. of the job, is the employer willing to modify the job
Take, for example, a 34-year-old man with C56 or create a job the client could do by merging com-
tetraplegia. After discharge from inpatient treatment and ponents of other jobs?
6 months of outpatient rehabilitation, he began college, Could the client do the job with additional accom-
taking only one course the first quarter; in 6 years, he modations not paid for by the employer, such as the
graduated with a BA in business with a specialization in support of a job coach?
accounting. One year after graduation, he passed his Cer-
tified Public Accountant exam. Over the next 3 years, he
Job Accommodation
applied for nearly 700 jobs, had numerous interviews,
and worked in volunteer positions, with no offers of Dowler et al. reviewed the requests for information on
employment. His faculty advisor at the college reported accommodation made by 1,000 people with SCI who
that he was an excellent student and, when prospective called the national Job Accommodation Network (19).
employers were contacted, they reported that he had They reported that callers with tetraplegia most fre-
interviewed competently, impressed them as bright and quently reported difficulties with gross and fine motor
720 REHABILITATION
skills such as keyboarding, manipulating small parts, benefits. For people with SCI who are employed, the
holding pens, and sorting. Those with paraplegia reported employer may pay for some services to meet the obliga-
difficulty with driving, heavy lifting, carrying objects, tion to provide reasonable accommodations. Finally,
reaching work areas, etc. As noted earlier, job accom- many legal settlements rising from traumatic SCI include
modations can include modification in work schedules, funding for vocational rehabilitation. In addition to these
modification of tasks, and modification of the environ- individual options, there are several major social service
ment to make it more accessible (e.g., installing electric systems that provide vocational rehabilitation.
door openers or a ramp). Some accommodations are as
simple as removing a desk drawer or raising the height
Federal and State Vocational
of a desk with four wood blocks to permit wheelchair
Rehabilitation Programs
access. Other accommodations are high tech and involve
designing computer interface software and hardware, or The Rehabilitation Act funds a vocational rehabilitation
writing scripts to allow a user to control multiple appli- program in each state with four federal dollars for each
cations using voice recognition. Still other accommoda- dollar appropriated by the state. Some Native American
tions are practical, such as providing a private changing groups have also established federally funded vocational
area and storage for a change of clothing for a male rehabilitation programs. State vocational rehabilitation
employee with tetraplegia who uses a condom catheter in programs are designed to help people with disabilities pre-
case of bladder accident when the condom becomes dis- pare for, obtain, and retain employment. The goal is not
connected during transfers. In many cases, the need for simply to obtain employment, but to assist the person
additional accommodations will become apparent as the with a disability to maximize his or her employment
employee begins settling into the job. potential. The state vocational rehabilitation agencies
Although accommodations for people with SCI may work in collaboration with program participants,
often seem self-evident, this is not the case for individu- employers, and the community. To be eligible for services,
als with concurrent brain injuries. For these individuals, an applicant must have a significant disability that limits
special attention must be paid to designing accommoda- the ability to work. Services include:
tions that serve as compensatory tools for lost cognitive
function. These may include prosthetic memory devices, Medical EvaluationDetermination of a persons
but also may include changes in supervision to include strengths and vocational limitations through
explicit and concrete instruction to the employee, using expert medical, psychiatric, social, and psychologic
both verbal and written instructions. evaluation.
Vocational AssessmentIdentification of a persons
interests, readiness for employment, work skills, and
Long-Term Employment Issues
job opportunities in the community.
Several authors have noted recently that the more years Counseling and GuidanceRehabilitation coun-
of employment an individual with SCI has following selor and participant establish an ongoing relation-
injury, the more functional limitations he or she experi- ship in which they explore evaluation results and
ences (5). This represents the accelerated morbidity asso- labor market opportunities and develop a realistic
ciated with aging with SCI. It is critical that vocational plan for employment.
rehabilitation not be considered complete once a job has RestorationIncrease work potential and ability to
been obtained (10). Proactive, strategic planning with retain a job through use of medical and assistive
periodic intervention will reduce the occurrence of addi- technologies.
tional disability and employment problems in the future. Job PreparationBuild work skills through services
such as volunteer experience, on-the-job training,
vocational and technical education, and higher
VOCATIONAL REHABILITATION SYSTEMS education.
Support ServicesSupport the participant through
In the past, rehabilitation counseling services at some services such as transportation assistance; the pur-
inpatient and outpatient rehabilitation programs were chase of tools, equipment, books, or work clothing;
provided as part of the program fee. Increasingly, hospi- job coaching; and independent-living support.
tal-based rehabilitation counselors and those in the pri- Job PlacementDevelop work opportunities and
vate sector provide fee-for-service rehabilitation counsel- obtain and maintain a job suited to the participants
ing. Some services can be paid for by health insurance interests and capabilities.
policies, and others are paid directly by the recipient. It Follow-upFollow the progress of the participant
is not unusual for personal injury policies and long-term for at least 90 days to ensure that employment is
disability policies to provide for vocational rehabilitation satisfactory.
VOCATIONAL REHABILITATION 721
Post-employmentProvide short-term services prior to return to work, and they will be considered
from time to time necessary to help a participant sus- totally disabled for at least a year or two. Other people
tain employment. with SCI will never return to work. These include people
who cannot resolve health insurance issues, or who live
in rural areas without access to transportation or employ-
State Workers Compensation Programs
ment, or older workers who had relied on their ability to
Each state has a workers compensation program for perform physical labor and who do not have sufficient
workers who sustain work-related injuries. In many transferable skills to work in another field. Under these
states, the program is regulated by a state agency but circumstances, it is the responsibility of the rehabilitation
implemented by commercial insurance carriers that cover counselor, the physiatrist, and other rehabilitation pro-
the employer. Larger employers may be self-insured. In fessionals to advocate for disability benefits and assist in
some states, all phases of the process are conducted by the the application process. Some individuals will have long-
state agency or its contractors. Workers compensation term disability insurance policies, for which the premi-
programs in most states include a vocational rehabilita- ums have been deducted from their pay, but most will not
tion component. In workers compensation rehabilitation, and will rely on various state and federal programs.
the goal is to return the injured worker to his or her pre-
vious job, or a similar job. If neither of these is possible,
Social Security Administration
then depending on the state, the goal may be to return the
injured worker to any suitable employment. Covered ser- The Social Security Administration (SSA) pays disability
vices are similar to those described for the federal state benefits under two programs: Social Security Disability
system, but typically services are only provided with Insurance (SSDI) and Supplementary Security Income
respect to the work-related injury. Workers compensa- (SSI). The medical requirements for disability payments
tion vocational rehabilitation programs typically have are the same under both programs, and a persons dis-
two phases. The first is characterized by the continuing ability is determined by the same process. However, eli-
recovery and rehabilitation of the injured worker. The sec- gibility for SSDI benefits is based on history of employ-
ond phase begins when the injured worker reaches max- ment covered by SSA, whereas eligibility for SSI disability
imum medical improvement. Wage replacement is typ- payments is determined by financial need. SSDI is a fed-
ically paid during both phases. When the injured worker eral long-term disability insurance program funded by
cannot return to previous employment or a similar job, payroll taxes, whereas SSI is a federal welfare program
and does not have the transferable skills necessary to per- for, among others, people with disabilities who are unable
form other suitable work, retraining can be authorized. to work and are poor.
If a worker is determined not to have the potential to The SSA considers a person disabled if he or she is
resume employment, he or she is typically awarded a set- unable to perform any kind of work at a level of sub-
tlement or pension. stantial and gainful activity, and the disability is
expected to last at least a year or to result in death. Appli-
cants should contact a Social Security office as soon as
Veterans Administration
they become disabled, because there is a waiting period
Individuals with SCI who are eligible for services through before some types of disability benefits begin. This wait-
the Veterans Administration (VA) have access to voca- ing period starts with the first full month after the onset
tional rehabilitation services at many comprehensive VA of the disability, as determined by SSA. Filing for disability
system rehabilitation centers and through other commu- benefits can be done by phone, by mail, or in person at
nity access points. VA services are similar to those pro- any office. Spouses and children who are financially
vided through the federal state system. dependent on the disabled worker may also qualify for
disability benefits under certain conditions.
Newly injured individuals with SCI should consider
Other Vocational Rehabilitation Systems
applying for SSA benefits immediately if it seems likely
Various other systems with similar features exist for work- that they will not return to work for at least a year. Once
ers who sustain disabling work-related injuries in federal eligibility is established, depending on the program, recip-
government, off-shore, maritime, and railroad settings. ients are immediately eligible for state Medicaid benefits
or, after a waiting period, federal Medicare benefits.
Under the Ticket to Work legislation of 2000, many recip-
KEY DISABILITY BENEFIT SYSTEMS ients of SSA benefits will be able to continue their
Medicare coverage after they return to work. SSA also
For some people with SCI, an extended period of educa- has several work incentive programs in which a person
tion, retraining, and/or rehabilitation will be necessary with a disability who must purchase services or goods to
722 REHABILITATION
work can deduct those expenses from his or her income. 6. Krause J. Employment after spinal cord injury: Transition and
life adjustment. Rehabil Coun Bul 1996; 28(2):244255.
If, as a result, his or her income after deductions is below 7. Krause J, Anson C. Adjustment after spinal cord injury: Rela-
the threshold, he or she can continue to receive SSA ben- tionship to participation in employment or educational activities.
efits including Medicare. This can be a powerful tool for Rehab Coun Bul 1997; 40:202214.
8. Britell K, Hammond M.Spinal cord injury. In: Hays R, Kraft G,
someone with a high-level SCI who has a strong desire Stolov W, (eds.), Chronic Disease and Disability. New York:
to work but requires personal care assistance on the job, Demos, 1994.
must make lease payments on a van, and needs other ser- 9. Strubreither W, Hackbusch B, Hermann-Gruber M, Stahr G, Jonas
H. Neuropsychological aspects of the rehabilitation of patients
vices to work. with paralysis from a spinal injury who also have a brain injury.
Spinal Cord 1997; 35:487492.
10. Roessler R. Job retention services for employees with spinal cord
injuries: A critical need in vocational rehabilitation. J Applied
CONCLUSION Rehabil Couns 2001; 32(1):39.
11. Rehabilitation Act of 1973, 87 Stat.355, 29 U.S.C. 701 et seq.
Many people with SCI would like to work and can work 12. Rehabilitation Act Amendments of 1974, 89 Stat.2.
13. Pennell F, Johnson J. Legal and civil right aspects of vocational
with appropriate vocational rehabilitation services, but rehabilitation. In: Johnson K, Haselkorn J, (ed.), Vocational Reha-
they face numerous barriers to achieving that goal. They bilitation: Physical Medicine and Rehabilitation Clinics of North
achieve many benefits from employment including eco- America. Philadelphia: Richard Saunders; 1997.
14. Americans with Disabilities Act of 1990, 42 U.S.C. 12101 et seq.
nomic self-sufficiency, health care benefits, and improved 15. Individuals with Disabilities Education Act of 1990, 20 U.S.C.
quality of life. People with disabilities believe that access 1400 et seq.
to employment is a basic civil right and federal laws have 16. National Council on Rehabilitation Education. 2001.
www.nchrtm.okstate.edu/ncre
been enacted to support that belief. Physiatrists and other 17. Anastasi A, Urbina S. Psychological Assessment. 7th ed. Upper
rehabilitation professionals can play a key role in advo- Saddle River, NJ: Prentice Hall; 1997.
cating for full participation in employment for their 18. Brown C, McDaniel KR, Couch R. Vocational Evaluation Systems
and Software. Menomonie, WI: Stout Vocational Rehabilitation
patients by understanding the benefits of employment and Institute, University of Wisconsin-Stout; 1994.
the potential for their patients to participate in employ- 19. Dowler lD, Batiste L, Whidden E. Accommodating workers with
ment, and by taking concrete steps to document both spinal cord injury. J Voc Rehabil 1998; 10:115122.
functional limitations and strengths, identify compen-
satory strategies, including assistive technology and Bibliography
accommodations, and make appropriate referrals to
Johnson K L, Haselkorn J (eds.), Vocational Rehabilitation: Physical
vocational rehabilitation professionals. Medicine and Rehabilitation Clinics of North America. Philadel-
phia: Saunders, 1997.
Parker R, Szymanski EM. Rehabilitation Counseling: Basics and
References Beyond. Austin: Pro-ed, 1998.
723
724 REHABILITATION
encountered in normal urban and rural driving. Driving from falling onto the driver during rapid vehicle maneu-
is not just the ability to operate the controls of a vehicle vers. Some persons with tetraplegia are able to transfer
to move from one location to another at low speed with into a car, but are not able to load the wheelchair. If they
little or no traffic. have a folding wheelchair, they can use a car top loader
(3). After transferring into the vehicle, the consumer
attaches a device to the wheelchair that lifts, folds the
ADAPTIVE EQUIPMENT wheelchair, and stows it in a box, on top of the car.
Devices are also available to load scooters into the trunks
The equipment needed may range from $700 for a basic of cars. If the individual cannot transfer into a car or has
hand control in a car for a consumer with paraplegia, to a wheelchair (such as a powered wheelchair) that cannot
$70,000 for a joystick-type control system in a van for a be accommodated in the above manner, a van is usually
consumer with high-level tetraplegia. The cost difference required.
lies in the type and amount of equipment needed to sub- Consumers who cannot use a car generally choose
stitute for the loss of function. between mini-vans and full-sized vans. Mini-vans are
More-complex driving systems are normally needed smaller, more economical to operate, and fit into home
by people with less function, but can be driven by people garages. Ground clearance can be a problem, because van
with more function. Even the most sophisticated driving floors are typically lowered 10 inches to accommodate a
systems can be driven by able-bodied individuals, as long driver in a wheelchair. Mini-van steering also requires
as they receive training in their use. Less-complex systems more strength than in full-sized vans and there is less
are often installed in parallel to the Original Equipment room to adjust hand controls to take advantage of lever-
Manufacturer (OEM) systems, and a driver can use either age. Full-sized vans have more room for the storage of
system. More-advanced systems replace the OEM sys- vocational or personal items, are based on a truck chas-
tems; some utilize a switch to change from one system to sis for durability, and support a greater variety of modi-
another, and others require the able-bodied driver to use fications.
the adaptive system. Adaptive equipment should be Mini-vans typically require a combination of a low-
matched with the level of individual function to provide ered floor and a rear suspension that lowers the vehicle
sufficient assistance for safe control of the vehicle while for loading and raises it for driving, allowing a wheelchair
reducing cost and the potential for inadvertent over-con- user to enter and exit the vehicle using a ramp. The ramp
trol. There is more than a semantic difference between the either folds out from inside the vehicle or slides from
equipment people need and the equipment people use. under the floor. Persons with tetraplegia using manual
Whereas underequipped consumers are likely to be unsafe wheelchairs may have difficulty negotiating a ramp. Mini-
drivers, overequipped consumers may also be unsafe dri- van access modifications typically cost between $12,000
vers and will have paid far too much for their modifica- and $15,000.
tions. Four basic categories of modifications enable peo- Full-sized vans require a wheelchair lift to raise the
ple with spinal injuries (or other disabilities) to drive: consumer and wheelchair from ground level to van floor
modifications to provide access to the vehicle and the dri- level. If only additional head room is needed, the roof and
ving station, to control the speed and direction of the vehi- doorway can be raised. If improved visibility out of the
cle, to stabilize the driver and protect him or her in the windows or additional head room is needed, the floor is
event of an accident, and to control everything else, lowered. Whereas most mini-vans modified for wheel-
including shifting, horn, and lights (often called secondary chair users have side entry (especially for wheelchair dri-
controls). vers), full-sized vans may have the entry on the side or
rear. Side entry allows more functional seating, especially
in the rear of the vehicle, for passengers. Rear entry typ-
Vehicle Access
ically allows greater freedom in parking lots, because the
Persons with paraplegia are typically able to transfer into wheelchair driver need not rely on neighboring cars leav-
a car independently and to load their wheelchairs into the ing enough room for re-entry into the van. Most vans
car (2,3). Folding wheelchairs can be put into the foot with side lifts (and mini-vans with ramps) require almost
space between the back and front seats. Rigid wheel- 8 feet next to the van for the consumer to enter or exit.
chairs, after removal of the wheels, can be loaded into the A van lift commonly referred to as a rotary lift reduces
back seat or the passenger seat. Some persons with para- the space needed next to the van to 36 inches. Both mini-
plegia transfer in on the passenger side, slide over to the vans and full-sized vans typically use powered doors for
drivers side and pull the wheelchair into the passenger independent access for consumers in wheelchairs.
seat. The increased use of consoles between front seats Although most consumers depend on interior and exte-
has made this option less feasible. If stored on the pas- rior switches for the doors and lifting device, consumers
senger seat, the wheelchair should be secured to keep it who cannot operate the switches can purchase radio con-
DRIVING WITH A SPINAL CORD DISORDER 725
trols. Access modifications to full-sized vans vary con- trol is simply a device that allows the operation of the
siderably, because there are many types available. Costs gas and brake pedals with the hands. Some of the first
range from $6,000 to $12,000. hand controls were made for the Model A Fords (2,7).
Great care must be exercised by the consumer in the In 1967, 35 to 40 percent of approximately 6,000 mem-
selection of the vehicle, not just for function but for avail- bers of the Paralyzed Veterans of America were
ability of modifications (4). Only certain specific mini- tetraplegic. Of these, about 20 members had devised
vans are modified by large companies, called second stage means of independent highway travel (8). This was
manufacturers. Consumers must always check on the fea- about the time hand controls and wheelchair lifts became
sibility of the installation of the modifications needed commercially available.
before purchasing a vehicle. Many consumers have been The most common hand control is called the push-
very frustrated to find that they purchased a vehicle that right angle-pull hand control, seen in Figure 53.1. With
could not be modified to meet their needs. this control, which is mounted under the steering column,
Once inside the van, the consumer may transfer out the consumer pushes forward on a handle to operate the
of the wheelchair into the drivers seat using a device called brakes. For gas, the handle is pulled down towards the
a transfer seat. A wheelchair seat is normally about 6 leg (at a right angle to the direction of the application of
inches higher than a typical van seat. The transfer seat uses the brake). This type of hand control sells for $700 to
a van seat mounted on a special seat base that moves back $1,000, installed. Other varieties of the standard hand
several inches, raises about 6 inches and, if necessary, control include a twist grip (as on a motorcycle) for the
swivels, to facilitate the transfer (2). Many consumers who accelerator or push-pull (push forward for brake, pull
cannot transfer into a car with a standard seat can use a back for gas). There are several varieties of hand controls
transfer seat in a van, because it reduces the horizontal and for special purposes (9).
vertical distance required during the transfer. Many people with tetraplegia find it difficult to push
If the consumer cannot use a transfer seat, he or she down on or to twist a handle for the accelerator. Floor-
must drive from the wheelchair. There are wheelchair mounted push-pull mechanical hand controls help alle-
securement devices designed (and crash tested) specifically viate this problem. This push-pull hand control has a ter-
to hold a wheelchair in the drivers position (5). An indi- minal device (a term used in the industry to refer to the
vidual who drives from a wheelchair and wants to pur- part used by the consumer to interface with the control
chase a new wheelchair will need to determine if a secure- system) mounted on a long rod attached to the floor. The
ment device is available for that chair model. The consumer pushes forward for brake and pulls back for
selection of an appropriate wheelchair is one area in gas. The forces are in the 8- to 12-pound range, but the
which physicians and other rehabilitation staff can make motions are in a direction more favorable to the func-
significant contributions to the success of the consumer tional abilities of a person with tetraplegia. These con-
in the vehicle purchase (3,4). Because the wheelchair sits trols often use a device called a tri-pin for the terminal
higher than a van seat, the vehicle floor will usually need device. The tri-pin has three vertical posts. The rear two
to be lowered to allow proper visibility out of the wind- posts fit on either side of the wrist (in the neutral posi-
shield and better access to the driving controls. A lower tion), and the forward post goes across the palm of the
wheelchair seat height may lead to fewer structural mod-
ifications in a full-size van but may leave a shorter con-
sumer sitting too low in a mini-van. Floor lowering gives
rise to many technical issues, such as positioning of the
fuel tank and structural integrity of the vehicle (6).
In general, exiting the vehicle is the reverse of enter-
ing, with one significant exception. Regardless of how the
vehicle is entered, consumers should never exit vehicles
by backing onto the lift or the ramp. They should always
face out from the vehicle. Consumers have been injured,
even killed, because they backed out over the edge of the
van doorway and fallen, after the lift had been lowered
to the ground.
hand and into the thumb web space. Tri-pins are fre- injured consumers use hand controls, which occupy one
quently used for drivers without a functional grip. Floor hand, only one hand remains for steering. Most hand con-
mounted push-pull hand controls cost from $1,500 to trol users need a terminal device attached to the steering
$2,000. wheel, such as a knob, a cuff, or a tri-pin, which can spin
If the forces needed to operate a mechanical hand freely and maintains constant contact with the wheel dur-
control exceed a consumers functional abilities, the con- ing rapid movements such as a swerve in an evasive
sumer may use a powered hand control. Figure 53.2 maneuver. Some consumers prefer to palm the wheel,
shows a powered control (with the terminal device left but this can result in a loss of control when it is most
off, for better visibility of the control). Powered hand con- needed. If the consumer has difficulty turning the steer-
trols use power from the vehicle (vacuum, hydraulic, elec- ing wheel, the OEM power steering system can be mod-
tric, etc.) to translate the drivers input, on the terminal ified for low-effort steering (reducing the force by half) or
device, into forces from an actuator, which then pushes zero-effort steering (reducing the force to only about a
on the gas and brake pedals. The forces needed to oper- tenth of that originally needed). Mini-vans are about 50
ate this control are measured in ounces (usually under 1 percent harder to steer than full-sized vans and the effort
pound), with a total travel length from full gas to full needed to steer them cannot be reduced as much as on a
brake of approximately 4 inches. A powered hand con- full-sized van. Reducing the effort costs from $1,500 to
trol costs between $6,000 and $12,000, depending on $2,500, with an additional $2,000 to $3,000 for a backup
brand and features. system designed to take over, in the event of certain types
Most drivers control the direction of the vehicle of power steering failures.
using the OEM steering wheel. Because most spinal- If contractures or kyphosis position the drivers ster-
num within 10 inches from the steering wheel, the air bag
should be turned off. However, the vehicle must be
equipped with a switch to turn the air bag back on, mak-
ing it available for able-bodied drivers. The consumer
may need a letter from a physician documenting this phys-
ical functional limitation in order to have an air bag
switch installed. The steering column can also be
extended to accommodate wheelchair size and configu-
ration. Steering wheel diameter is a factor if the consumer
has elbow flexion contractures. Although reduced-diam-
eter steering wheels are available, they do not have air
bags. The consumer is no longer required to obtain per-
mission from the National Highway Traffic Safety
Administration (6) for removal of the air bag, but may
need a letter from a physician, documenting this physi-
cal functional limitation, in order to drive without an air
bag. Smaller steering wheels and steering column exten-
sions generally cost under $500.
If the use of the OEM steering wheel is not func-
tionally feasible, remote steering systems can be used. Fig-
ure 53.3 shows a remote steering control system with a
knob-type terminal device. The driver turns a small steer-
ing wheel, attached to a small box, which uses computer
technology to operate the steering system. The OEM
steering wheel is often left in place for the air bag and for
able-bodied drivers. The box can be placed in any loca-
tion in the vehicle and at any orientation necessary for
safe operation by the consumer. Remote steering systems
cost from $10,000 to $15,000.
Some consumers lack the strength in one arm to
operate either the gas/brake system or the steering system.
They need a complete system that can be operated with
FIGURE 53.2 only one hand (2). One-handed control systems generally
require less force to operate than other systems, and allow
Powered hand control. the driver to lay the hand not used for steering on an arm-
DRIVING WITH A SPINAL CORD DISORDER 727
rest, greatly increasing stability and control. There are ception below the shoulder and upper arm find it more
two systems currently available: tri-pin or joystick-con- difficult to control. Both of these one-handed control sys-
trolled. Figure 53.4 shows a one-handed steering system, tems cost $30,000 to $40,000.
with a tri-pin terminal device, in which the driver pushes
for gas, pulls back for brakes, and rotates the forearm
Driver Stability
for turns. Diminished range of motion anywhere in the
elbow or wrist can affect a consumers ability to operate Individuals with diminished upper extremity function as
the system. This system has been successful with relatively a result of a SCI usually also lack trunk stability. They
high-level tetraplegics and has been commercially avail- do not have the musculature to maintain their body
able since 1974 (10). upright (5) or the sensation (except through the inner ear)
The other one-handed operation system uses a joy- to know that they are beginning to fall. Consumers with
stick-type of control, not unlike those on electric wheel- low thoracic paraplegia have trunk control and strong
chairs. This type of joystick control does not provide feed- shoulders and hands to assist in balance. Those with
back to the driver as to the rate of turn of the vehicle. tetraplegia must depend on the wheelchair for stability
Whereas this system has been used with great success for while driving. Having one hand on a steering wheel, with
consumers with weak shoulders and elbows, but good very low forces needed to move the wheel and a powered
hands (e.g., muscular dystrophy, multiple sclerosis, post hand control with very low forces needed to apply the gas
polio, etc.), who are able to make rapid, small, precise and brakes, leaves the driver with tetraplegia floating
inputs to the control lever, consumers with higher-level in her wheelchair. It also leaves this driver entirely depen-
tetraplegia who have little muscular strength or proprio- dent on a chest harness for trunk stability and, therefore,
728 REHABILITATION
vehicle control. If the chest harness is not attached to a functional assessment portion of the evaluation unless
stable wheelchair back, the persons trunk stability may they are registered therapists of some type.
be inadequate for safe driving. Many driver rehabilitation programs operate with a
The driver must also use safety belts. Although the team, which may include an occupational therapist as a
OEM shoulder/lap belt combinations work for able-bod- driving evaluator, a rehabilitation engineer, and a driving
ied drivers, disabled drivers may not be able to use such instructor. The physician is the primary referral source
safety belts. Common practice in the industry has been to and must write a prescription requesting the evaluation.
use a shoulder belt that the consumer can drive under in A proper driving evaluation (4) has three main com-
her wheelchair, and a lap belt attached to the wheelchair. ponents: pre-driving evaluation, vehicle selection and
Although this method has proved effective, the means of evaluation, and behind-the-wheel training. The pre-dri-
attaching the belt ends to the vehicle and wheelchair have ving evaluation is usually performed by an occupational
not been standardized at this time. The vehicle modifica- therapist or driving evaluator and consists of a review
tion industry, SAE(11), and NHTSA(12) are working on of the candidates medical and driving history and a clin-
the problem of standards in this area. ical assessment. The clinical assessment determines the
functional capabilities of the person and obtains infor-
mation in a number of areas. The visual portion assesses
Secondary Controls
acuity, depth perception, field of vision, and other para-
Once it has been determined how the consumer with a meters important for driving. Cognitive functions, such
SCI will access the drivers station and operate the gas, as judgment, problem solving, memory, attention, and
brake and steering, all of the other controls must be con- the recognition of signs and symbols are screened. Range
sidered. Although the alternatives are numerous, some of motion, hand function, mobility skills, strength, bal-
items should be highlighted. Specific knobs and levers can ance, muscle tone, and other physical examination fea-
be modified for the consumers use simply by extending tures are evaluated. A driving simulator may be used to
them or putting wire loops on them (13). Powered shifters assess coordination, brake reaction time, and potential
and powered parking brakes allow these manual func- equipment needs. Simulators are no longer used for all
tions to be controlled by push buttons. The consumer portions of the evaluation because the effects of the
must be able to reach some controls while driving (horn, dynamic aspects of driving (acceleration, deceleration,
signals, etc.) Others can be operated when the vehicle is and lateral acceleration when cornering) on the driver
stopped (doors, windows etc.) (14). If only a few controls and wheelchair are extremely difficult and expensive to
must be moved, it is sometimes possible to add an addi- simulate. The existence of higher technologies in driving
tional switch. Newer vehicles may require specially man- have made an on-the-road portion of the evaluation
ufactured control panels, each of which costs from $2,000 increasingly important (17).
to $7,000. The vehicle selection process involves team mem-
bers forming recommendations about the type of vehi-
cle needed. An equipment evaluation is performed and a
DRIVING PROGRAMS prescription (a specific report of equipment and training
needed) is formulated. A vehicle modifier company must
The scope of the alternatives should suggest the com- be selected, keeping in mind that the modifiers have vast
plexity of selecting modifications for a driver with a SCI. variations in experience, especially with high-technology
Fortunately, a relatively new professional, the driving modifications. Often this decision is made by the third-
evaluator, is coming to prominence throughout the party payer (18). After the vehicle is modified, the driving
nation. There have been driving evaluators since the mid- instructor ensures that the driver can use the controls, as
1970s; however, the criteria required for this role have installed, and assesses the fit of the equipment. A final
only been agreed on in the last 10 to 15 years and are mechanical inspection should be based on local (19) or
still in the process of revision (4). The leading organiza- national standards (11,12).
tion for driving evaluators in the nation is the Association The final component is the behind-the-wheel train-
of Driver Educators for Disabled (ADED) (15), and they ing by a driving instructor. The driver needs a valid license
certify driving evaluation specialists. Despite the avail- or learners permit to undergo this training. The evalua-
ability of a certification system, there is still little infor- tions for higher-level consumers with tetraplegia are made
mation in the literature about the current assessment tech- longer not only because the equipment is more complex,
niques used in these evaluation programs (16). In the but because that complexity requires more training to
ADED model, the driving evaluator is normally an occu- enable instructors to subject the consumer to conditions
pational therapist. Many occupational therapists have (freeways, construction zones, evasive maneuvers, etc.)
also become driving instructors and can fulfill both roles. that cannot be tested immediately. Consumers must learn
Driving instructors are usually unable to perform the to drive the system sufficiently, to cope with situations
DRIVING WITH A SPINAL CORD DISORDER 729
where balance and dynamic forces show their effects on drives and/or potential scrapes while transferring, may
driving (4). To send a consumer on the road with a mod- keep him out of the hospital. Learning safe car transfer
ern sophisticated driving system and only the experience techniques, under a knowledgeable therapists supervision,
of drive in a parking lot is not an adequate way to deter- can prevent injuries. Because driving allows consumers to
mine if she has the strength needed to move the controls be independent, they may be alone when they encounter
through the necessary motions. This can lead to disas- problems. They need to be aware of the effects of heat
trous results. when first entering a closed vehicle on a hot day. They
Some people do not feel a complete evaluation is should be aware of the potential for autonomic dysreflexia
necessary for lower-level SCI and are of the opinion that, and what interventions should be followed. Some con-
if a consumer with paraplegia has no other disabilities, a sumers with tetraplegia carry a note from their physician
vehicle modifier can simply install a hand control. Some or a wallet card describing the most appropriate treatment
vehicle modifiers, however, will not install any driving for their autonomic dysreflexia, as information for emer-
modifications without an evaluation, due to liability con- gency care professionals not familiar with the condition.
siderations. Most people in the industry agree that train- Most phone services have special rates for cell phones for
ing is needed following the purchase of almost any vehi- consumers with disabilities.
cle modification. A consumer with a SCI learning to use Patients whose SCI resulted from trauma should
hand controls may confuse the gas and the brake. Despite have a careful neurologic check for head trauma. Many
significant progress in learning new skills, a person sub- consumers with SCI receive a blow to the head either as
jected to an emergency situation will often revert to long- a cause of, or secondary to, their SCI, and perceptual
established habit patterns. problems that can affect or prevent driving can result
ADED (15) has a Web site (www.driver-ed.org) and from such head trauma (20). Consumers with spina bifida
the American Automobile Association (AAA) keeps a file are also known to have a high incidence of perceptual
of driving evaluation programs and instructors. Many problems that can affect the processing of visual infor-
state departments of vocational rehabilitation have active mation and even the awareness of which side of the road
programs for funding modifications to allow consumers they are on. A major concern with respect to consumers
to return to work and can be a resource for finding local with perceptual problems is that they are usually not
driving evaluation programs and instructors. The aware of the existence of such problems. Even when writ-
National Mobility Equipment Dealers Association ten and on-the-road tests indicate such a problem, there
(NMEDA) (12) can provide information about local vehi- is extensive denial because they cannot sense the problem.
cle modifiers. Many auto manufacturers (e.g., Ford, Gen- Early diagnosis and counseling about such problems may
eral Motors, and Chrysler Benz) have mobility programs reduce the number of consumers attempting to drive who
to provide drivers with disabilities information about should not be driving. With the proper equipment and
their products and about sources of driving evaluations training, however, drivers with SCI who do not have per-
and vehicle modifications. ceptual problems can be safe drivers (21).
The physician and other members of the rehabilitation The wheelchair is the basic support platform for any con-
team can prepare a patient with a SCI for driving. Prepa- sumer with spinal cord tetraplegia who wishes to drive
rations include physical rehabilitation and training, rec- (22). The more stable the platform, the greater the like-
ommending and prescribing durable medical equipment, lihood of success. A reclining wheelchair may seem nec-
and furnishing the patients history. It is important that essary while the consumer is an inpatient and has not yet
the rehabilitation team become familiar with local driving learned other means of pressure relief. The motion
programs, equipment suppliers, and other resources for allowed by the multiple joints of the recliner mechanism
potential drivers. may, however, prevent a consumer from being a driver.
The secondary effects of a spinal cord disorder, such The physician and rehabilitation staff must weigh the
as decubiti and contractures, weigh heavily on driving immediate alternatives of preventing decubiti in that man-
equipment needs or even the ability to drive. Other aspects ner against the later need to be a safe driver. Wheelchair
of normal rehabilitation training take on special signifi- back height is also a frequent cause of driving problems.
cance if the patient with a SCI becomes a driver. Aware- The driver with tetraplegia is dependent on a belt-type
ness of the lack of thermal sensation in the hands can pre- chest harness, which attaches to his or her wheelchair and
vent injury from a metal or plastic part of a driving control passes just under the axilla, for maximum support (5).
system that has been sitting in the sun for hours. The con- The physician must assess the consumers request for a
sumers specific awareness of pressure relief during long lower back for cosmetic appearance. They must consider
730 REHABILITATION
access to back packs or the patients ability to hook her accurate diagnosis is a vital starting point, including
elbow on the back upright for trunk support, against the whether the injury is complete or incomplete. Any sec-
actual need for trunk support in driving. It should be ondary conditions, such as contractures, spasticity, or
noted that lateral trunk supports, attached to wheelchairs, perceptual difficulties, should be described. Medical sta-
are often not effective in driving. They tend not to be high bility, the effects of medications, and any planned surg-
enough, or they tend to interfere with the arm movements eries, such as tendon transfers, are important factors to
needed for driving. Proper wheelchair back support is also communicate as well.
important in the prevention of scoliosis, which can cause
problems in reaching controls and steering. It can also
affect the fitting of a chest harness, which is needed to CONCLUSION
provide trunk stability for cornering.
Physicians should evaluate and describe the patients Many people with spinal cord disorders at the C45 level
spasticity. Spasticity in the upper extremities can be a dif- can drive, as can most people with spinal cord disorders at
ficult obstacle to overcome. The consumers ability to the C56 level. Whereas lack of funding for the high-level
handle the driving controls (normally in a driving evalu- technology needed for safe driving is probably the most
ation) is the final determinant of the effect on driving. Dri- frequent reason for those with higher levels of SCI not dri-
vers may be able to cope with mild spacticity, but severe ving, secondary disabilities such as perceptual problems,
spacticity can prevent them from driving safely. There is contractures, decubiti, and other injuries can also affect
also a difference in the effects of increased tone caused driving potential. For most of these consumers, a driving
by spacticity (which can affect reaction time and force evaluation by a professional evaluator is needed; for the
applied to the controls) and actual spasms (which can higher-level injuries, it is essential. The primary role of the
cause inadvertent control inputs or prevent intended physician and rehabilitation staff, with respect to the dri-
inputs). Unfortunately, many of the drugs prescribed to ving consumer, is to maximize the patients rehabilitation
reduce the effects of spacticity in daily activities adversely progress, prevent complications from the spinal cord dis-
affect the alertness of the driver. order, and identify and treat secondary disabilities. Also of
The drivers destinations are a factor when consid- importance is to ensure that the consumer leaves the hos-
ering the choice of a wheelchair. If the consumer is only pital with a wheelchair that is appropriate for driving, as
marginally able to use a manual wheelchair, but prefers well as for daily living needs. Informing patients about and
the manual wheelchair for exercise, her ability to be truly referring them to evaluation programs can save them
independent in the community (including ramps, etc.) money by avoiding inappropriate modifications.
becomes more critical when driving to unknown desti-
nations. There may be indications (23) that these indi- References
viduals are putting excessive stress on their shoulders and 1. Dip R. COT. An investigation into the employment and occupa-
should consider a powered wheelchair. Driving from the tion of patients with a spinal cord injury. Paraplegia 1994;
wheelchair is often indicated for marginal manual wheel- 32:182187.
2. Koppa R. State of the art in automotive adaptive equipment.
chair users to avoid the extra transfers, but the extra Human Factors 1990; 32(4):439455.
weight of the wheelchair securement device, added to the 3. Pierce S. Drivers needs. TeamRehab Report 1992; Jan/Feb,
wheelchair, can cause additional problems. Manual 2022,38.
4. Pierce S. A roadmap for driver rehabilitation. OT Practice 1996;
wheelchairs having extreme camber in the rear wheels Oct, 3138.
may be too wide to fit on lifts and into lowered floor areas 5. Babirad Juergen MSA. Considerations in seating and positioning
for wheelchair driving. severely disabled drivers. Assist Tech 1989; 1:3137.
6. National Highway Traffic Safety Administration. Exemption from
With regard to driving, important actions the physi- the Make Inoperative Prohibition; Final Rule (2001) Federal
cian can take are to inform the patient about the existence Motor Vehicle Safety Standards, 49CFR, part 595. Washington
of driving evaluation programs and to refer the consumer D.C.7. Murphy, Eugene Ph.D., Reflections on Automotive Adap-
tive Equipment. Bull Prosth Res Fall 1979; 10-32:191207.
to them when appropriate. The physician should also 8. Bray P, Cunningham DM. Vehicles for the severely disabled. Reha-
inform patients of the need for professional driver train- bilitation Literature 1967; April, 28(4):98109.
ing with any adaptive equipment. Many accidents have 9. Reichenberger A. Licensed motor vehicles for handicapped dri-
vers. Report of a Workshop, National Academy of Sciences 1975;
occurred when appropriate equipment has been installed 6975.
but the consumer has not obtained training to use the 10. Gacioch M. Vans, vans and more vans. Mainstream 1989; Dec,
modifications. 717
11. Society of Automotive Engineers, SAE Handbook. 400 Com-
Most driving evaluation programs (especially hos- monwealth Dr., Warrendale, PA 15096-0001.
pital-based programs) need a physicians referral for a dri- 12. National Mobility Equipment Dealers Association Guidelines,
ving evaluation. Although a note on a prescription pad 1999. National Mobility Equipment Dealers Association, Tampa,
FL.
stating Evaluate for driving has been the basis for 13. Sklar J. An introduction to van modifications for the disabled. J
many evaluations, much more information is needed. An Traf Safe Edu 1981; July, 1112,27.
DRIVING WITH A SPINAL CORD DISORDER 731
14. Roush L, Koppa R. A Survey of activation importance of indi- 20. Sivak M, Olson PL, Kewman DG, Hosik W, Henson DL. Driving
vidual secondary controls in modified vehicles. Assist Technol and perceptual/cognitive skills: Behavioral Consequences of Brain
1992; 4:6699. Damage. Arch Phys Med Rehabil 1981; 62:476483.
15. Association of Driver Rehabilitation Specialists (ADED), P.O. Box 21. Katz RT, Golden RS, Tepper D, Rothke S, Holmes J, Sahgal V. Dri-
49, Edgerton, WI 53534. ving safety after brain damage: Follow-up of Twenty-two Patients
16. French D, Hanson C. Survey of driver rehabilitation programs. with Matched Controls. Arch Phys Med Rehabil 1990;
Am J Occup Ther 1999; 53(4): 394397. 71:133137.
17. Britell C. Van Modifications for Wheelchair Accommodation, 22. Simoes N.F. Van modifications for wheelchair accommodation,
Transportation I. A State of the Art Conference on Personal Trans- Transportation I. A State of the Art Conference on Personal Trans-
portation for Persons with Disabilities. NIDRR and University portation for Persons with Disabilities. NIDRR and University
of Virginia, 1991; 13. of Virginia, 1991; 1214.
18. Rogers J. Adaptive devices for automobiles. J Traf Safe Edu 1975; 23. Smith I. Aging with spinal cord injury. Rehab Management 1989;
911. June/July:2835.
19. State of California, Department of Rehabilitation Requirements
for adaptive driving equipment. 1998, part of Master Agreement
with vehicle modifiers.
This Page Intentionally Left Blank
54 Functional Electrical
Stimulation
an electrical circuit), depolarization of the axon below the and the force output of the muscle being stimulated does
cathode, or negative electrode, occurs. As the potential not usually exist. Rather, there is often an S-shaped curve
decreases under the cathode, the membrane becomes describing this strengthduration relationship. Within this
more permeable to sodium (Na+) ions, which in turn curve there exists a portion of the recruitment curve, in
causes further depolarization of the nerve as Na+ moves which there is a very high gain; that is, for very small
into the cell. If this depolarization occurs with sufficient changes in pulse width or amplitude, large changes in
intensity and at a rapid enough rate, the membrane force output occur. This relationship is important when
reaches its threshold and an action potential occurs. Thus, attempting gradual proportional recruitment of motor
the voltage gated Na+ channel is responsible for the gen- strength.
eration of the all-or-none action potential. A separate Pulse frequency determines the rate at which the nerve
voltage gated potassium (K+) channel is in turn respon- fires. Increasing pulse frequency provides for temporal sum-
sible for subsequent repolarization of the nerve. [Further mation of force output. For most applications, it is desir-
description of this topic can be found in standard neuro- able to have a fused or tetanic contraction of muscle. This
physiology textbooks such as that by Kandel, Schwartz, occurs at 20 to 30 Hertz (cycles per second) physiologically,
and Jessell (4).] although some fusion of contraction can be obtained at fre-
Motor units, which consist of axons and the nerve quencies as low as 12 Hz. For FES, the tradeoff as stimu-
fibers that they innervate, are activated electrically by lating frequency is increased (thereby obtaining a smoother
depolarization of the motor axons or the terminal nerve contraction) is more-rapid muscle fatigue.
branches at the neuromuscular junction. Electrical cur- Waveforms available for use in FES applications
rent can directly depolarize muscle fibers, but the amount include monophasic, asymmetric biphasic, or symmetric
of current necessary for this to occur is considerably biphasic. Monophasic stimulation, wherein ion flow is
greater than that for depolarization of nerve axons (5). unidirectional, is generally not appropriate for long-term
Therefore, for practical purposes, FES systems stimulate FES use, because it can cause electrode and tissue break-
nerves, not muscles. Some literature exists on the electri- down (9). A balanced pulse, wherein ion flow occurs in
cal stimulation of denervated muscle, but the clinical both directions sequentially, avoids these problems and
effect of this remains controversial (6). One concern is allows for stimulation at both electrodes. Commonly,
that direct muscle stimulation, although retarding mus- square waves are used for the first of the two pulses, and
cle atrophy, may (at least theoretically and in some ani- then a capacitively coupled discharge is employed as the
mal models) prevent reinnervation through the inhibition balancing second pulse.
of terminal sprouting and the reformation of neuromus- The duty cycle is the percentage of time that the
cular junctions (7). stimulator is on, and is sometimes expressed as a ratio of
Current density, or the amount of current injected on time to off time. The greater the duty cycle, the more
per unit area, is the ultimate determinant as to whether a profound the problem with muscle fatigue, because the
given stimulation will generate action potentials and time of rest is reduced. Duty cycles play a role in devices
therefore recruit motor units. Electrode size helps deter- such as the bladder neuroprosthesis, in which intermit-
mine the current density, because the smaller electrodes tent stimulation is desirable to achieve the desired result
have the greatest current densities under or around them. (see further discussion on this topic below).
For surface electrodes, the electrodeskin interface is also Just like muscles undergoing voluntary exercise, FES-
quite important, because changes in impedance of this stimulated muscles change morphologically and physio-
interface can considerably alter the current density under logically. Type II glycolytic fibers convert to Type I oxida-
the electrode. tive fibers over weeks to months, depending on the
Several important parameters can be adjusted dur- intensity and frequency of stimulation. This phenomenon
ing the application of FES. These include pulse amplitude is associated with changes in vascular supply, and increases
and duration, frequency, waveform, and duty cycle (8). the fatigue resistance of the muscle (10). One question that
Pulse amplitude (in milliamps) and duration (in microsec- still remains incompletely answered is how much FES exer-
onds) are parameters that determine the size of individ- cise is adequate to achieve fiber changes and allow for
ual pulses provided by a stimulator through a set of elec- functional use. This is important clinically as one sets up
trodes. As either is increased above threshold, spatial conditioning protocols for patients using FES devices.
recruitment of additional motor units occurs, thereby
increasing the number of nerve axons activated and, sub-
sequently, the muscle force output. The product of the COMPONENTS OF FES SYSTEMS
amplitude and duration, or the area under the square
wave pulse (if that is the type of pulse being used) repre- FES systems typically consist of multiple components,
sents the net charge injected into the tissue. A direct lin- including an electronic stimulator, a control mechanism,
ear relationship between the amount of charge injected cabling, and electrodes.
FUNCTIONAL ELECTRICAL STIMULATION 735
The electronic stimulator is usually battery powered, engineering literature and describe the mechanism of feed-
although it can be powered from electrically or optically back that is provided. In open-loop control, the electri-
isolated power sources (for safety reasons). Stimulators cal output of the FES system is dependent not on the mus-
range in sophistication and number of channels. These cular force or joint movement produced by the
devices are as complex as the movement they are designed stimulation, but rather by the stimulator itself. In closed-
to produce. Most portable FES neuroprostheses are built loop control, real-time information on muscle force
around a microprocessor-controlled set of stimulators. and/or joint position or movement is fed back into the
The microprocessor may be preprogrammed or may FES system and allows for the modification of its electri-
allow software modification of the stimulator output. The cal output. This allows for a more responsive result that
number of stimulator channels varies with application can accommodate for external variables such as irregu-
from one to twenty-four or more. Various channels are larities in the environment or muscle fatigue. Practical
activated sequentially or in unison to allow micro- problems are associated with closed-loop FES, however,
processor orchestration of complex movements. relating to the need for sensors to measure force and joint
Control systems for therapist-operated FES systems position or angular velocity in a physiologically repro-
consist of dials and switches to control stimulus parame- ducible and cosmetically acceptable way (12).
ters. For subject-controlled FES systems (i.e., neuropros-
theses), control can come in the form of switches, buttons,
joysticks, joint position sensors, EMG electrodes, voice- GENERAL SELECTION CRITERIA
activated controls, sip-and-puff devices, heel switches, FOR FES SYSTEMS IN SCI
and the like.
Electrodes provide the interface between the elec- SCI damages both the long tracts and the spinal-level neu-
trical stimulator and the nervous system. Different types rons, or in terms of the motor system, both upper and
that have been used include surface, percutaneous intra- lower motor neurons. When anterior horn cells or motor
muscular, implantable intramuscular, epimysial, and nerve roots are injured, corresponding muscular dener-
nerve cuff electrodes. In routine physical therapy practice, vation inevitably follows. If this denervation involves a
surface electrodes are exclusively used. Options are avail- significant quantity of musculature, then FES will no
able with regard to material, size, and means of applica- longer be effective. This is principally because of the pro-
tion. The key criteria are that the electrodeskin interface found increase in charge density (by a factor of approxi-
should be of low impedance, the electrode should be flex- mately thirty) required to directly depolarize muscle as
ible enough to accommodate contours, and it should be compared to nerve, as mentioned previously. For specific
easy to don and doff. Percutaneous intramuscular elec- applications, the significant quantity of denervated
trodes travel across the skin to insert into muscle. They musculature necessary to render FES ineffective varies.
are usually inserted through the use of a hypodermic nee- For phrenic nerve pacing, nerve conduction studies (look-
dle, and have a barbed end to ensure stability inside the ing at response latencies) or fluoroscopy of the diaphragm
muscle. Percutaneous electrodes have been used primar- during supramaximal tetanic stimulation can be used to
ily in developmental research protocols or where tempo- screen candidates for diaphragmatic denervation (13). In
rary application of FES is anticipated. Their long-term the case of upper extremity devices, the possibility exists
survival has not been as great as that for implanted elec- for the associated use of tendon transfer surgery (for
trodes (11). Implantable intramuscular electrodes are implanted devices) to replace one or two crucial dener-
more robust than the percutaneous versions, mainly vated muscles with paralyzed but innervated muscles to
because of their thicker lead wires. Epimysial electrodes provide specific aspects of motor movement. For the
are surgically sewn onto the epimysial surface of a mus- lower extremity and in bladder neuroprostheses, dener-
cle. They typically have a conductive core or disc (made vation is less frequently a problem, because the anterior
from metal alloys such as platinum-iridium) and a sur- horn cells often lie below the level of the SCI, and there-
rounding silicone elastomer skirt, which allows for sur- fore are physiologically intact. Individuals with conus
gical stabilization. Nerve cuff electrodes, as their name medullaris or cauda equina injuries, however, are not
implies, directly stimulate nerves by surrounding them cir- good FES candidates because of the considerable extent
cumferentially. Implanted intramuscular, epimysial, and of denervation associated with those injury levels.
nerve cuff electrodes require surgical placement. Contractures can often develop in the hands or the
Implanted cables (or leads) link the stimulator and the legs of individuals post-SCI. If they are severe enough,
stimulating electrode and are complex in design to with- these contractures can be a severe impediment to imple-
stand bending stresses and to remain insulated in the mentation of a neural prosthesis. The prevention of these
bodys saline environment. contractures and tendon shortenings early on in the reha-
Subject control of an FES system can be either open bilitation process is crucial in maintaining later candidacy
loop or closed loop. These terms are borrowed from the for neuroprostheses.
736 REHABILITATION
The time post-injury is not as crucial a criterion for may damage the components (17). In general, therefore,
candidacy as once thought. It has been standard practice it would be prudent to avoid these modalities in patients
in upper extremity reconstructive surgery for tetraplegia with implanted electrical stimulators.
to wait at least 1 year post-injury before performing any
surgical procedures so as to allow time for natural recov-
ery. Recently, this philosophy has been challenged, and FES APPLICATIONS IN SPINAL CORD
individuals as early as 6 months post-injury have had DYSFUNCTION
implant procedures. On the other side of the spectrum,
there is no outer limit as to the time post-injury for neu- Therapeutic FES can be used for muscle strengthening and
roprosthetic consideration. Often, however, individuals cardiac conditioning. Other potential benefits of this type
who are many years post-SCI have become accustomed of electrical exercise are improvement in venous return
to their way of performing tasks, and therefore are less from the legs, reduction of osteoporosis, improvement
receptive to a neuroprosthetic approach, especially if it in bowel function, and psychologic benefits (8).
requires an invasive procedure. Functional uses for FES after SCI include applica-
tions in standing, walking, hand grasp (and release),
bladder, bowel, and sexual function, respiratory assist,
CONTRAINDICATIONS and electroejaculation for fertility. Each of these tech-
nologies has been developed and implemented to vari-
Although no absolute contraindications exist for the use ous degrees over the course of the past few decades, and
of externally applied FES, a patient with a cardiac many continue to be refined further (18,19). These appli-
demand pacemaker or an automatic implanted defibril- cations will be discussed separately. Table 54.1 lists the
lator should be approached with extreme caution. There applications and indicates for each one the current stage
have been some reports that electrical stimulation applied of development.
anywhere on the body has the potential to interfere with
the sensing portion of the demand pacemaker, thereby
Therapeutic Exercise
suppressing activation of the device (14). Some of the rel-
ative contraindications for FES include patients with car- Persons with SCI are generally forced to become more
diac arrhythmias, congestive heart failure, pregnancy, sedentary. Paralysis is compounded by impaired auto-
electrode sensitivity, and patients with healing wound(s) nomic nervous system function, which limits the cardio-
that could be stressed during stimulation (i.e., muscle vascular response to exercise, especially in individuals
stimulation would adversely move healing tissues). with lesions above T5. Muscle bulk, strength, and
For implanted FES systems, the presence of an endurance all decrease after SCI, and muscle fibers have
implanted cardiac pacemaker, active or recurrent sepsis, been shown to convert primarily to anaerobic metabo-
or uncontrolled spasticity would be absolute contraindi- lism after injury. Paralysis of intercostal musculature
cations to the surgery. As with any implant in the body, reduces vital capacity. In addition, there is reduced periph-
individuals with implanted FES systems need to obtain eral circulation, lean body mass, and bone density, and
antibiotic prophylaxis when undergoing invasive proce- an altered endocrine response.
dures such as oral surgery (15). Some FES implants (e.g., The most common system for lower extremity FES
the Vocare bladder and the Freehand hand grasp sys- exercise is the bicycle ergometer. The most common com-
tems as described in later sections) have recently been mercially available ergometer in use is the ERGYS Clin-
shown to be compatible with magnetic resonance imag- ical Rehabilitation System made by Therapeutic Tech-
ing, and the FDA-approved label now reflects this. This nologies, Inc. This computer-controlled FES exercise
had previously been a concern in the SCI population, ergometer uses six channels and surface electrodes to
given the relatively frequent need to use MRI for evalua- sequentially stimulate quadriceps, hamstrings, and glutei
tion in those patients suspected of having a syringomyelia. bilaterally. Some systems also include the capacity for
Recently, the Medtronic Corporation issued a safety simultaneous voluntary arm crank exercise by para-
alert regarding the use of short wave diathermy, plegics, thereby permitting hybrid exercise.
microwave diathermy, or therapeutic ultrasound Cardiac capacity and muscle oxidative capacity have
diathermy for patients implanted with any of its neu- both been shown to improve with FES ergometry. Some
rostimulation systems (16). The concern raised was that subjects can train with FES ergometry up to a similar aer-
the use of diathermy could cause heating at the elec- obic metabolic rate (measured by peak VO2) as that
trodetissue interface that could produce tissue or nerve achieved in the able-bodied population (20). Electrical
damage. NeuroControl Corporation has indicated that exercise also increases peripheral venous return and fib-
these modalities should not be performed over the area rinolysis. Indeed, one study has indicated that FES in con-
of their implanted stimulators or electrodes because it junction with heparin therapy is more effective in pre-
FUNCTIONAL ELECTRICAL STIMULATION 737
TABLE 54.1
Development Stages of FES Systems for Use in Spinal Cord Injury
venting deep venous thrombosis than heparin therapy and the volume of respiratory secretions, improve the
alone (21). There are limits to the cardiovascular bene- level of comfort, and reduce required nursing care in ven-
fits of FES ergometry, however, especially in those with tilator-dependent tetraplegic individuals (26). Unfortu-
lesions above T5. In those patients, there is loss of nately, many individuals with high tetraplegia caused by
supraspinal sympathetic control, which in turn limits the traumatic injury have injury to the anterior horn cells,
bodys ability to increase heart rate, stroke volume, and located at the C3 through C5 spinal levels, that inner-
cardiac output (22). Evidence is mixed as to whether FES vate the diaphragm. Bilateral phrenic nerve conduction
ergometry can retard or reverse the osteoporosis and bone studies are necessary before considering phrenic nerve
demineralization seen in patients with SCI. In one study stimulation and diaphragmatic pacing. In these studies,
of ten spinal cord injured individuals who underwent 12 the conduction velocity is actually a more reliable indi-
months of FES cycling 30 minutes per day, 3 days per cator of phrenic nerve function than is the response
week, bone mineral density of the proximal tibia amplitude (27). Verification of phrenic nerve function
increased 10 percent. Unfortunately, after a further 6 can also be done by observing diaphragm movement
months of exercise, but at a frequency of only one session under fluoroscopy.
per week, the bone mineral density reverted to pre-train- The implanted components of neuroprosthetic sys-
ing levels (23). Also unclear is whether FES ergometry can tems for phrenic nerve pacing consist of nerve electrodes
improve carbohydrate metabolism and insulin sensitiv- placed around or adjacent to the phrenic nerves, radio-
ity in the same way that voluntary exercise does in the frequency receivers, and wiring connecting the two. The
able-bodied. It has been reported that the majority of SCI external components are the radio frequency transmitter
individuals involved in a FES ergometry program had and the antenna. Three companies currently manufacture
improved self-images after participation (22). FES ergom- these devices: Avery, Atrotech, and MedImplant. The
etry has also been associated with increased plasma Avery and Atrotech devices are available in the United
endorphins, normalization of the cortisol level, and States. The Avery device has received premarket approval
improved depression scores (22). One must keep in mind by the FDA, and is the one most widely used. The
the availability and cost of these ergometry devices before Atrotech system is available under an FDA Investigational
prescribing FES exercise either in a hospital/clinic setting Device Exemption. The Atrotech has a more sophisticated
or at home. Consistent use must be demonstrated and a four-pole electrode system, which is thought to reduce the
plan for ongoing regular exercise is required for the main- chance for fatigue. All of these systems allow for changes
tenance of therapeutic effects. in stimulus frequency, amplitude, and train rate.
Surgically, the electrodes are placed on or around the
phrenic nerve in the neck or thorax. The cervical
Breathing Assistance in High Tetraplegia
approach is surgically much less complicated and risky,
Phrenic nerve pacing was developed in the early 1960s but the thoracic approach has advantages with regard to
by Glenn and colleagues (24). This technique has been relative lack of electrode movement once implanted and
used successfully in over 1,000 individuals with respi- the ability to stimulate the more distal contributions to
ratory failure (25). In appropriate candidates, phrenic the nerve. The receiver is placed in a subcutaneous pocket
nerve pacing has the potential to improve mobility, on the anterior chest wall, and the leads are tunneled
speech, and overall health, as well as to reduce anxiety through the third or fourth intercostal space.
738 REHABILITATION
Stimulation is initiated approximately two weeks through the RGO mechanism. As mentioned above,
postoperatively, to allow inflammation and edema of the quadriceps stimulation then provides knee lock (32).
perineurium to subside. Reconditioning is then required SCI patients who are potential candidates for FES
to improve the fatigue resistance of the diaphragm, which standing or walking systems vary greatly with regard to
is usually atrophied in the ventilator-dependent tetra- their injury level. In general, patients with injuries between
plegic. Low-frequency stimulation (712 Hz) is required T4 and T12, with upper body strength and stability as well
to help convert the diaphragm to primarily oxidative, as intact lower motor neurons in the lumbar and upper
slow-twitch type I fibers (28). The respiratory rate usu- sacral myotomes, are appropriate for consideration,
ally lies within 8 to 14 breaths/minute during initiation although some higher-level patients have been involved in
of therapy, and then is reduced somewhat to 6 to 12 some protocols. Individuals with significant joint con-
breaths/minute later on. tractures, dysesthetic pain syndromes, uncontrollable spas-
The potential advantages of phrenic nerve pacing ticity, osteopenia, lack of cardiopulmonary reserve, and
over ventilator support include an improved ability to lack of motivation may be excluded from participation.
speak and the use of less bulky and cosmetically obvious At this writing, one FDA-approved surface FES walk-
equipment. The possible complications of phrenic nerve ing system is available for use in the U.S. Sigmetics Inc.
pacing include infection, mechanical injury to the phrenic makes the Parastep System at an approximate cost
nerve, upper airway obstruction, reduction in ventilation (including training provided by a physical therapist) of
caused by altered respiratory system mechanics, and tech- $11,000. The Parastep System is a four- or six-channel
nical malfunctions leading to failure (26). Most patients surface stimulation device for ambulation with the aid of
using electrophrenic respiration maintain their tra- a walker. Patients considered eligible for use of this device
cheostomy stoma to be used at night and for suctioning, have upper motor neuron injuries at the C5 through C8
although many of them can plug the sites during the day. (incomplete) or T1 through T12 (complete or incomplete)
levels. It is required that the users have adequate trunk con-
trol for stability. The Parastep System uses the triple flex-
FES for Standing and
ion response elicited by peroneal nerve stimulation as well
Walking in Paraplegia
as knee and hip extensor surface stimulation to construct
Over twenty-four centers worldwide have participated the gait cycle. The patient controls the gait with switches
over the years in investigation of the use of FES for lower integrated into a rolling walker (which is also needed for
extremity standing and walking in paraplegia, and at least stability and safety). The electronic controller is housed
twenty-one centers have clinically implemented walking in a cassette-sized box mounted on the patients belt. The
systems (29,30). Standing alone is a goal of some systems. Parastep System and other similar systems have been used
The potential physiologic benefits of standing include a by paraplegics not only for functional standing and walk-
beneficial effect on digestion, bowel, and bladder func- ing but also for aerobic exercise (33).
tion. The functional goals of standing to reach for high Implantable lower extremity FES has also been
objects and face-to-face interaction with other people are developed. The rationales for use of implanted electrodes
also important. The muscles usually stimulated to pro- are the difficulty in activating deep musculature from the
vide for standing function are the quadriceps, which pro- surface, as well as problems with reproducibility of sur-
vide for knee locking in a biomechanical way similar to face electrode placement. Both percutaneous electrodes
that of kneeanklefoot orthoses. The majority of effort and implantable stimulatorreceivers with epimysial or
in lower extremity FES development has focused, how- intramuscular electrodes have been tested, although nei-
ever, on functional ambulation. ther is currently available commercially. The percuta-
Several different approaches have been used in help- neous system developed in Cleveland uses up to forty-
ing paraplegics walk. Orthotic approaches without the eight different electrodes to provide reciprocal gait. The
use of FES have historically included the use of long leg considerable effort required for the maintenance of that
braces and the VanniniRizoli boot (31). The latter is a many percutaneous electrodes has made that type of sys-
thigh-high leather boot that stabilizes the ankle and places tem impractical for widespread use, however. Currently,
the foot in a 15-degree posterior to anterior slope. For a simpler but more invasive approach using sixteen chan-
those with good trunk stability and upper extremity nels of implantable stimulation is being investigated (34).
strength, this has been shown to be a functional ortho- Implantable eight-channel systems, with the more limited
sis, but widespread acceptance has not been achieved. goals of standing and transfer, are also under investiga-
Hybrid approaches, such as the reciprocating gait ortho- tion in the same laboratory.
sis (RGO), originally developed at Louisiana State Uni- In summary, the following statements can be made
versity, and modified at Wright State University, use both concerning lower extremity FES systems and their poten-
mechanical bracing and surface FES. Specifically, FES hip tial for clinical use in the near future. First, all of the pure
extension on one side provides contralateral leg swing FES systems require the use of a walker for stability and
FUNCTIONAL ELECTRICAL STIMULATION 739
safety. (The hybrid systems achieve proximal leg stabil- in functional movement. If one or two muscles are not
ity through bracing.) Second, the energy expenditure electrically available, surgical adjuncts can be employed
required for continuous FES walking is at least twice (if to overcome this problem (in the case of the implanted
not more) that for normal upright ambulation. The speed system). For instance, if the extensor digiti communus
of ambulation achievable, although improving, is not that (EDC) is denervated, the extensor carpi ulnaris can be
of normal ambulation. Third, liability issues limit pro- transferred to the EDC and powered by a FES electrode.
duction and raise price. Future directions for lower Upper extremity passive range of motion must be ade-
extremity FES for paraplegia would be to achieve more quate to allow for functional movements such as bring-
modest goals than originally envisioned, such as stand- ing the hand up to the mouth. Spasticity must be ade-
ing, transfer assistance, and possibly short-distance (e.g., quately controlled. Individuals must be in generally good
home) mobility. It is not anticipated that FES for walk- health, have adequate trunk support, and have intact
ing in paraplegia will replace the wheelchair as the pri- vision to ensure feedback on what the partially or com-
mary mobility aide used by individuals with paraplegia. pletely insensate hand is doing. Best results occur with
motivated people who have good social support systems.
Tetraplegic hand grasp systems have focused on the
FES for Hand Grasp and Release
C5 and C6 level complete SCI patient populations. These
In the past, the main methods employed to address the individuals have adequate voluntary strength in the
problem of motor paralysis in the upper extremities of proximal muscles of the arm (i.e., deltoid, rotator cuff,
tetraplegic SCI individuals have been the use of adaptive biceps) to move their hand in a functional space and
equipment and assistive devices. Surgical interventions, therefore benefit from the provision of hand grasp and
such as tendon transfers, have also been used (35,36). release. Those with C4-level injury (and therefore no del-
Technology for the restoration of hand function in toid or biceps strength) have also participated in limited
tetraplegic individuals has been under development in the laboratory-based investigations of FES systems (41), but
United States, Europe, and Japan for over three decades the results of these studies have been limited. Patients
(37). In addition, neuroprosthetic devices have entered injured at the C7 and lower levels have multiple volun-
the clinical environment (38). The objective of the use of tarily active forearm muscles (e.g., brachioradialis,
FES in the hands of tetraplegic individuals is to restore extensor carpi radialis longus and brevis, pronator teres).
grasp, hold, and release, thereby increasing independence These muscles can be used to motor new functions with-
in the performance of functional tasks and minimizing the out sacrificing current function by means of tendon
use of other adapted equipment. transfer surgery (42).
TABLE 54.2
Available Upper Extremity Neuroprostheses
the system through small movements of either the shoul- is through radio frequency coupling. The system can be
der or wrist. The joint position transducer, which oper- programmed through a personal computer interface by
ates somewhat like a computer joystick, is typically a trained therapist to individualize the grasps as well as
mounted on the skin from sternum to contralateral shoul- the shoulder control for each patient (43).
der or across the ipsilateral wrist, and senses these move- Individuals can choose to use either palmar or lateral
ments. The ECU uses this signal to power proportional prehension grasp patterns at any given time with the Free-
control of hand grasp and release. Communication hand System (Figure 54.2). This is helpful in handling
between the ECU and the implantable stimulator, which either large objects (such as books, telephone receivers,
is located in a surgical pocket created in the upper chest, etc.) or small objects (such as pens and forks), respectively.
The current system provides unilateral hand grasp only.
An evaluation of this system in a multicenter clinical trial
has recently been completed, and in August 1997, the FDA
approved this device for clinical use. Results from the mul-
ticenter trial are presented here (44,45).
A total of sixty-one patients were enrolled in the
study. As of September 2000, there are over 170 patients
in the world using this device. Of the original sixty-one,
69 percent of patients had C5-level injuries and 25 per-
cent had C6-level injuries. All of the patients had one or
more surgical procedures to augment hand function.
These procedures included tendon transfers (e.g., poste-
rior deltoid-to-triceps, brachioradialis-to-wrist extensors)
and joint fusions (e.g., thumb interphalangeal fusion). A
total of 128 cumulative implant years were evaluated dur-
FIGURE 54.1
ing the study (median implant duration 1.6 years). The grasp pattern with the neuroprosthesis.
following adverse events requiring surgical management The GRT was a timed test that evaluated each sub-
and the percent of patients involved in each type of event jects ability to grasp, move, and release six standardized
were reported: receiver required repositioning or replace- objects. Three objects (peg, fork, and paperweight) were
ment (5 percent); skin openings requiring repair (7 per- used with lateral prehension and three objects (block, can,
cent); electrode breakage (5 percent); infection requiring and videotape) were used with palmar prehension. The
electrode removal (5 percent); infection requiring system number of completed moves achieved by subjects in 30
explant (2 percent); and tendon adhesion (2 percent). The seconds increased significantly for the heavier objects
major nonsurgical adverse events included swelling or dis- (paperweight, fork, can, and videotape) with the neuro-
comfort over the implantable components (21 percent); prosthesis. No improvement was seen in manipulation
skin irritation from external products (23 percent); irri- of the lightest objects, namely the peg and the blocks,
tation from incisions or sutures (16 percent); and skin irri- except in the weakest users.
tation from splints or casts (10 percent). The ADL abilities test evaluated whether the neu-
In the evaluation of any assistive technology, two roprosthesis decreased the amount of assistance required
key questions need to be asked: a) what can users do with to perform various ADLs (e.g., eating with utensils,
the device and b) what do users actually do? To answer brushing teeth, shaving, loading a floppy disk in a com-
these questions as they apply to the Freehand System, puter). Furthermore, subjects were asked whether they
three tests were devised. The first two, pinch force mea- preferred to perform the task with or without the neuro-
surement and the grasp release test (GRT), were aimed prosthesis. Across all patients and tasks, 50 percent
at answering the first question. The third set of evalua- showed an improvement in independence score, and 78
tive tools, namely the activities of daily living (ADL) abil- percent preferred to use the neuroprosthesis to perform
ities, assessment, and follow-up tests, were aimed at the activities. The user satisfaction survey consisted of
answering the second question. Finally, a user satisfaction thirty-eight questions with regard to satisfaction, impact
survey was also performed at the time of 6-month or on ADLs, occupation, and need for external assistance.
greater follow-up. Patients reported overall satisfaction with the perfor-
Summary pinch force measurements with and with- mance of the system, indicated that it had a positive
out the neuroprosthesis are shown in Figure 54.3. Clearly, impact on their lives, and provided them with less depen-
pinch force in both lateral and palmar prehension dence on other adaptive equipment.
improved with the neuroprosthesis. The small improve-
ment seen postoperatively with the neuroprosthesis HANDMASTER. Another device available for the
turned off can be attributed to tendon synchronization restoration of hand function in individuals with C5
performed as an adjunct during the implant procedure. tetraplegia is called the Handmaster, which is produced
It should also be noted that all patients realized some by the NESS Corporation in Israel (46). It is composed
improvement in pinch force measurement in at least one of a hinged shell with a spiral splint that stabilizes the
wrist. Surface electrodes are built into the shell and
stimulate the finger flexors and extensors and the thenar
musculature. A trigger button mounted on a separate
control unit allows the user to start the stimulation
sequence. A separate mode button allows the user to
choose different preprogrammed patterns of muscle
activation. The device is designed to be used with C5 and
C6 tetraplegic individuals as well as individuals with
hemiplegia from cerebral injury. The NESS device has
been studied clinically in pilot investigations in Israel (47)
and in the United States (48). Both of these studies
involved individuals with cerebral damage from stroke
or traumatic brain injury. In the latter study, twenty
FIGURE 54.3 subjects with hemiplegia and no functional use of the
Pinch force for lateral and palmar prehension with and with-
hemiparetic hand underwent an accelerated outpatient
out the Freehand neuroprosthesis. The histogram family on stimulation program that ultimately led to an average
the left represents lateral grasp; palmar grasp is on the right. daily device use time of 3.4 hours over an average of 13.1
Force represented in newtons. There is some increase in force weeks. Significant improvements in passive wrist
produced postoperatively even with the neuroprosthesis extension, active elbow flexion, wrist flexion and
turned off. This is probably related to the increase in tone pro- extension, degree of spasticity, and ability to hold a 1-
duced by surgical tightening of the flexor tendons. kg weight were noted after the treatment regimen. With
742 REHABILITATION
Future Directions for Hand-Grasp Neuroprostheses sacral stimulator implant, abolishes uninhibited reflex
bladder contractions and restores bladder compliance.
Future developments and improvements in tetraplegic
Detrusor-sphincter dyssynergia is also reduced, thereby
hand-grasp systems will include implantable controllers,
avoiding the chance of autonomic dysreflexia as well. The
involving both joint angle and myoelectric signal trans-
downside of this procedure, however, is loss of perineal
ducers (52) closed-loop feedback with improved sensor
sensation in sensory incomplete individuals, and loss of
technology, proximal muscle control [i.e., shoulder mus-
reflex erection and ejaculation if present.
culature (53), triceps (54)] control of the prontation/
supination axis addition of finger intrinsics to improve
finger extension (55) and bilateral implementation of Patient Evaluation and Selection
grasp in C6 level individuals. Ultimately, technologies
Potential candidates for the Vocare implant must have
such as the development of injectable electrodes and cor-
intact parasympathetic efferent neurons to the detrusor
tical control of movement may also affect on these neu-
musculature. This can be ascertained by cystometrogra-
roprosthetic devices.
metric recording of bladder pressure, which should be 35
cm water in women and 50 cm water in men. Individuals
will likely have other sacral reflexes intact, such as the bul-
Control of Micturition with FES
bocavernosus and anal wink along with ankle tendon
Electrical stimulation to control bladder function after stretch reflexes. Patients should be neurologically stable
suprasacral SCI has also been under investigation for sev- and be motivated to use this device. Concurrent sepsis or
eral decades. Stimulation at various levels of the neu- pressure sores should be treated prior to implant (57).
rourologic axis has been attempted, including the lumbar
spinal cord, the anterior sacral nerve roots, the pudendal
Results of Bladder Stimulator Implant
nerve, and the bladder/detrusor itself.
In the United Kingdom, Brindley et al. (56) have had
There has been considerable documentation of success
the most experience and success with S2 through S4 ante-
in terms of residual volumes after micturition using this
rior sacral nerve root stimulation in combination with
implanted device. In a study of twenty patients, postvoid
posterior rhizotomy. This system is surgically implanted
through lumbar laminectomy and employs either epidural
or intradural electrodes. These electrodes are connected
via cable to an implanted radio receiver, which couples to
an external stimulator/transmitter. Pulsed stimulation is
used to take advantage of the differences between acti-
vation of the slow-response smooth musculature of the
detrussor (bladder) and activation of the fast-twitch stri-
ated sphincter musculature (Figure 54.4). Bladder pres-
sure gradually increases in a tetanic fashion, while sphinc-
ter pressure rapidly falls after the end of each stimulation
pulse. This produces short spurts of urination, but can
result in nearly complete bladder emptying.
Over 1,500 sacral anterior root stimulators have
been implanted throughout the world. Of those, approx-
imately 90 percent are in regular use 4 to 6 times per day.
In the United States, the clinical trials of this device in
humans started in 1992. As of December 1997, twenty-
six extradural sacral root stimulators had been implanted
in a United States multicenter trial. The FDA approved FIGURE 54.6
the implantation of the device for clinical use in 1998
Physiology of micturition produced by the Vocare sacral
under the trade name Vocare. In the United States, an
nerve stimulator, as identified by cystometrogram recording.
extradural surgical technique is used. X-axis represents time in minutes and seconds with the total
To provide continence of urine with this electrical tracing lasting approximately 3 minutes. A. Intravesicular
stimulation device, one has to consider ways in which to pressure in mm water from 0 to 150. B. Urine flow rate in
abolish the reflex incontinence caused by the activation ml/seconds with the scale from 0 to 40 ml/sec. C. Cumulated
of the sensory reflex pathway. This has been done in most volume of urine voided in ml with the scale from 0 to 600 ml.
cases by performance of a posterior rhizotomy at the D. Approximate timing of the sacral nerve stimulation used
sacral levels. This procedure, done concurrently with the to produce this intermittent voiding pattern.
744 REHABILITATION
residual volumes were reduced on average from 212 cc ity in medicine. The intense scientific research and devel-
106 to 22 cc 14 (58). A significant reduction in the opmental work that has occurred over the last half cen-
number of symptomatic urinary tract infections has also tury has finally borne clinical fruit. During this last
been seen. Continence has been achieved in more than 85 decade, several neuroprosthetic devices have gone
percent of implant recipients, probably because of the through regulatory review and have finally become avail-
concurrent posterior rhizotomy (59). Improved health of able for use with patients. This has been a remarkable
the upper urinary tracts, as demonstrated by a decreased achievement, but is likely just the beginning. Further
incidence of bladder trabeculation, hydronephrosis, and development and refinement of this technology will likely
the like, has been seen in patients post implantation. enhance the lives of a greater number of patients with dis-
Interestingly, the sacral anterior root stimulators ability caused by spinal cord dysfunction, as well as those
have also been shown to improve bowel care (i.e., of other individuals with neurologic impairment.
increased defecation, reduced constipation). About half Although obstacles such as cost, liability, and regulatory
of implant recipients can use the stimulator to defecate. burden will have to be addressed as these devices become
Even in those patients who cannot use the electrical stim- more commonplace, the future remains promising for the
ulation to defecate, the amount of time spent in bowel with spinal cord injury.
evacuation has been shown to be considerably less (60).
A slower stimulation time sequence is used for defecation
than for micturition. Approximately 60 percent of men References
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VIII
RECENT ADVANCES IN
SPINAL CORD RESEARCH
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55 Functional Magnetic
Stimulation
quad cough, and high-frequency chest wall oscillation for determining the central conduction time for upper
(3234). The application of some of these techniques in intercostal muscles have recently been reported (53).
a timely manner has decreased mortality in SCI. In addi- Magnetic stimulation of the phrenic nerve was first
tion to chest physical therapy, the functional exercise of reported by Similowski et al. (54). The MC was posi-
respiratory and accessory muscles has been used effec- tioned above the spinous process of the seventh cervical
tively in the treatment of many SCI patients. Functional vertebra (C7) to stimulate both phrenic nerves with a sin-
exercise includes the use of glossopharyngeal breathing gle stimulus. They were able to obtain reproducible supra-
and breathing against resistance (35,36). maximal CMAP in five of six subjects. Wragg et al. (55)
Historically, functional electric stimulation (FES) of compared magnetic stimulation of the phrenic nerve with
the respiratory muscles has been an active area of research electric stimulation, and showed that mean twitch trans-
in the last several decades. Various types of electric diaphragmatic pressure (Pdi) obtained by magnetic stim-
implants have been employed to generate inspired and ulation was significantly higher than electrical twitch Pdi.
expired pressures. These have consisted of electrodes on The researchers speculated that magnetic stimulation
the phrenic nerve, ventral roots, intercostal nerves, and might have resulted in stimulation of extradiaphragmatic
abdominal muscles (3742). Electric phrenic nerve pac- musculature. Mills et al. (56) also showed that cervical
ing is a technique to assist mechanical ventilation in sub- magnetic stimulation in patients with known diaphrag-
jects with bilateral diaphragm paralysis, such as in cervi- matic paralysis could not generate transdiaphragmatic
cal SCI above C6 (42,43). The criteria for electrical phrenic pressure. The stimulation of the extradiaphragmatic mus-
nerve stimulation include chronic ventilatory insufficiency culature most likely played a role in stiffening the upper
secondary to paralysis that has been stable for at least 1 thoracic cage to allow the diaphragm to act efficiently.
month; viable phrenic nerves; and a diaphragm that Additionally, Laghi et al. (57) also reached the same con-
responds well to a diaphragmatic pacemaker (44). Upper clusion that cervical magnetic stimulation recruited both
intercostal muscles (T1T6) have been demonstrated to act extradiaphragmatic and diaphragmatic muscles, whereas
as inspiratory agonists by lifting and expanding the rib electric stimulation had a more selective action on the
cage (45,46). Intercostal pacing was attempted in diaphragm. They further concluded that magnetic stim-
tetraplegic subjects via ventral root stimulation, and ulation could be an equally effective method for detect-
inspired volumes between 600 and 900 ml were demon- ing diaphragmatic fatigue.
strated (47). FES of the abdominal muscles has been FMS of the respiratory muscles was first demon-
demonstrated to generate significant expiratory pressures. strated in animals (40,58,59). In one study (40), FMS of
After placing percutaneous electrodes on the abdominal the phrenic nerves, the upper intercostal nerves, and the
wall motor points, significant expiratory flow and pres- lower intercostal nerves of dogs was demonstrated (Fig-
sure were measured in patients with SCI (41,42,48). In ure 55.2). The resultant inspired/expired pressures were
1995, Dimarcos group (49) demonstrated significant expi- 20 1.99 cmH2O, 10 1.03 cmH2O, and 11 2.25
ratory pressure when stimulating at T9T10 level in fif- cmH2O pressure respectively. The inspired/expired vol-
teen anesthetized dogs. A quadripolar-stimulating elec- umes were 373 20.5 ml, 219 12.2 ml, and 119
trode was inserted epidurally and on the ventral surface 22.5 ml, respectively. The graphic results are displayed
of the lower thoracic spinal cord. At functional residual in Figure 55.3. The stimulation profile was 30-Hz fre-
capacity (FRC), deflation (10 cm H2O) and inflation quency, 1-sec burst length, and 70-percent intensity. These
(20 cm H2O), the positive airway pressures generated animal studies not only demonstrated the ability of FMS
were 44 4 cm H2O, 28 3 cm H2O, and 82 7 cm to generate inspired or expired volumes and pressures,
H2O, respectively. These results showed that a major por- but also established the foundation for FMS as a poten-
tion of the expiratory muscles can be activated repro- tial technology for inspiratory/expiratory muscle train-
ducibly and in concert via electric stimulation, and that the ing, ventilatory assistance, and cough production.
resulting pressure can be comparable to that of a manu- Expiratory functions produced in humans by FMS
ally assisted cough (49,50). Nevertheless, FES requires were first demonstrated by stimulating the lower thoracic
surgery for electrode implants in most instances. spinal nerve root (11,60). A 12.5-cm round MC was
Magnetic stimulation has been applied to stimulate placed in the lower thoracic region for stimulating the
the cerebral cortex to evaluate the nerve conduction lower intercostal muscles and the abdominal muscles, as
velocity for the respiratory system. Using transcranial shown in Figure 55.4. Maximal expired pressure, volume,
brain stimulation, diaphragm compound muscle action and flow rate generated by FMS were 83.6 16.39 cm
potential (CMAP) had a latency of 16.21 0.33 msec, H2O, 1.54 0.18 L, and 4.75 0.35 L/s, respectively.
an amplitude of 3.52 2.40 mV, and a central motor These values corresponded to 73.2 percent, 100, and 90
conduction time of 8.39 0.41 msec (51). Similarly, percent of the maximal voluntary expired pressure, vol-
nerve conduction studies of the thoracic nerves using ume, and flow, respectively. The optimal coil placement
magnetic stimulation (52) and transcranial stimulation was at T7, and the optimal stimulation parameters were
FUNCTIONAL MAGNETIC STIMULATION 753
FIGURE 55.3
gastrointestinal symptoms also appears to increase with In two recent animal investigations (86,87), it is
time after SCI (68). Bowel management for patients with observed that by applying a smaller figure-8 coil near
SCI includes a balanced diet, judicial use of oral and rec- the thoracolumbar region FMS produced significant
tal medications, and digital stimulation (75). Some SCI improvement in gastric emptying at 1 hour post orogas-
patients with long transit times find bowel care to be com- tric gavage (90.4 percent in the experimental group vs.
plicated, laborious, and difficult to adhere to. These 69.6 percent in the control group). The effect of FMS on
patients often spend several hours a day emptying their gastrointestinal transit time showed a similar pattern. A
colons, contributes to a decreased quality of life and inde- significant decrease on gastrointestinal transit time was
pendence. For patients with very difficult bowel evacua- observed between groups (p 0.05). Post hoc t-tests
tion and/or severe GI dysfunctions, colostomies have sim- showed a significantly increased geometric center in the
plified their care (68,76). group that received stimulation of both anterior cervical
Electric stimulation of visceral organs is not a new and thoracolumbar regions (7.8 0.3), and the stimula-
concept. Hymes et al. (77) used surface electric stimula- tion of the anterior cervical region alone (7.3 0.3), com-
tion of the abdomen and noted a striking decrease in post- pared to the control group (6.1 0.4; p 0.005). This
operative ileus in a prospective, randomized study of 213 information, and the absence of gastric myoelectric activ-
patients. Richardson and Cerullo (78) followed this with ity in chronic SCI, lead to the postulation that the weak-
a study of twenty-one patients with neurogenic bowel dis- ness of the abdominal musculature may be an important
ease from a variety of etiologies, and found a marked factor associated with delayed gastric emptying in
reduction in the incidence of ileus among those patients patients with chronic SCI. The induction of abdominal
treated with abdominal neurostimulation. In addition, muscle contractions may bring about improved gastric
Richardson et al. (79) used transabdominal neurostimu- emptying and gastrointestinal transits, in a manner sim-
lation on forty-four acute SCI patients. None of the ilar to other physical examples of external compressions
patients developed paralytic ileus, whereas 15 percent of that produce compressions of the internal organs, such as
forty-three control subjects with similar injuries did. Frost the compression of the heart caused by chest compres-
et al. (80) used electric stimulation of the sacral der- sions during cardiac pulmonary resuscitation and the
matomes in SCI patients, and concluded that electric stim- compressions of the bladder from abdominal compres-
ulation of the S2 dermatome caused a significant rise in sions to produce more effective bladder emptying.
the number of rectal pressure spikes, but no significant When placing the MC at T9, FMS has been demon-
change in the time required for patients to initiate bowel strated to enhance gastric emptying in both able-bodied
movement. subjects and SCI patients (88) (Figures 55.4 and 55.5).
Advances in ventral sacral root stimulation have also The gastric emptying half-time (GEt1/2) shortened more
demonstrated increased colorectal contractions in than 8 percent and 33 percent for able-bodied and SCI
patients with SCI (8185). Sacral anterior root stimula- subjects, respectively. Figure 55.4 shows an example of
tion has brought significant progress in recent years for gastric emptying curves of a SCI subject at baseline and
both electromicturition and electrodefecation. Varma et with FMS. The GEt1/2 of gastric emptying with FMS was
al. (81) used Brindley stimulators with sequential electri- accelerated by approximately 38 min when compared
cal stimulation of the anterior sacral roots (S2, S3, and with the baseline values. One possible mechanism is that
S4) on five SCI patients. Reproducible results were FMS functions similarly to electrical stimulation to accel-
obtained: S2 stimulation provoked isolated low-pressure erate gastric emptying through the direct activation of the
colorectal contractions; S3 stimulation initiated high- underlying gastric neuromuscular apparatus. Instead of
pressure colorectal motor activity, which appeared peri- stimulating at the stomach level, the thoracic spinal nerves
staltic and was enhanced with repetitive stimuli; and S4 are activated as they exit the neuroforamen (17). FMS
stimulation increased colonic and rectal tone. MacDon- may be acting in a similar manner to gastric pacing, which
agh et al. (82) used BrindleyFinetech stimulators on uses electrodes implanted on the surface of the stomach
twelve SCI patients. At the beginning of stimulation, both muscle to cause rhythmic gastric contractions. Another
the rectosigmoid and the anal sphincter contract. As the postulation is related to the fact that rhythmic abdomi-
anal sphincter relaxes, the rectum is still contracting. This nal muscle contractions bring about improvement in gas-
results in defecation. Of the twelve patients tested, six tric emptying. This is because the abdominal muscles
achieved complete evacuation of feces using the stimula- (major expiratory agonists) were innervated by the lower
tor alone, whereas the remaining six had to perform man- thoracic spinal nerves.
ual evacuation. In 1991, Binnie et al. (83) used the same Placing MC at the lumbosacral region, FMS has
Brindley S2-S4 anterior sacral nerve root stimulator on been shown to activate the colon and improve colonic
ten SCI patients and demonstrated a significant increase transit and gastric emptying in human studies. In one
in the frequency of defecation as compared to ten SCI human study in patients with SCI (16), significant changes
controls without the stimulators. in rectal pressure and colonic transit time caused by FMS
FUNCTIONAL MAGNETIC STIMULATION 755
FIGURE 55.5
0.03 ms and 1.9 0.23 ms) using hook electrodes The effect of the FMS protocol on fibrinolytic
placed along the right S2 sacral root/nerve. This corre- response for individual subjects showed that the mean
sponds with earlier studies that predicted the foci of stim- WBCLT values decreased from 17 1.3 hrs at baseline
ulation to be located at the foramen (17). Moreover, the to 12 1.0 hrs and 11 0.8 hrs at 10 min and 60 min
average amplitude obtained by FMS (1.4 0.26 mv) was post-FMS, respectively. The post-FMS values, 10min and
similar to that obtained from the contact electrode (1.5 60min, were significantly different, p 0.0001 and p
0.43 mv). These amplitudes are more than double those 0.0001, respectively, from the pre-FMS lysis time mea-
obtained by unilateral S2 stimulation. This illustrates that surement. The degree of fibrinolytic enhancement at 10
FMS could stimulate multiple sacral nerves simultane- and 60 minutes post-FMS was not uniform in all indi-
ously, producing similar CMAP as ventral sacral root viduals. This study has demonstrated that FMS of the leg
stimulation. Similar levels of Pves and Pur (change in muscles can produce an enhancement of systemic fibri-
urethral pressure) produced by FMS and FES in this study nolysis that exceeds the increase in fibrinolytic activity
confirmed this result. accounted for by changes in posture and the influence of
the circadian rhythm. This enhancement is apparently
sustained for at least 60 minutes after cessation of the
FMS of the Lower Limb
stimulation protocol. Using a similar protocol on twenty-
for Enhancing Fibrinolysis
two patients with SCI, preliminary results show that the
Thromboembolism is one of the most common compli- WBCLT values at 10 min post-FMS (15 1.6 hrs) and
cations in acute SCI and postoperative patients. Compli- 60 min post-FMS (16 1.5 hrs) were significantly
cations of deep venous thrombosis (DVT) that lead to sig- decreased from pre-FMS (19 1.2 hrs; p 0.001). The
nificant morbidity include post-thrombotic syndrome, values of WBCLT at 10min and 60min post-FMS did not
prolonged edema, and pressure ulcers, and may be a change significantly (p 0.45). These results show that
source of spasticity or autonomic dysreflexia. The impor- FMS provides a more rapid enhancement of systemic fib-
tance of prophylaxis with these patients cannot be under- rinolysis than modalities such as FES and a more sus-
estimated. The widely used prophylactic modalities for tained enhancement than voluntary exercise. It could be
DVT include medications, external pneumatic compres- an excellent tool for use in situations where voluntary
sion, intermittent pneumatic compression, gradient elas- exercise cannot be performed, such as in patients who
tic stocking (99), and FES (100). Application of FES to have SCI, multiple sclerosis, stroke, central nervous sys-
the tibialis anterior and gastrocnemius soleus muscle tem tumors, or in spinal dysraphias.
group has been proposed as a means of preventing DVT
in patients with acute SCI. FES was shown to increase
plasma fibrinolytic activity as well as venous blood flow TRANSCRANIAL MAGNETIC STIMULATION
(100). When FES was combined with low-dose heparin
in one clinical trial, there was a substantial decrease in the Merton and Morton (103) demonstrated that it was pos-
incidence of DVT when compared with placebo or low- sible to electrically stimulate the human motor cortex
dose heparin alone (101). FES has not gained popularity through the intact scalp. A brief, high-voltage electric
as a prophylactic modality because it is cumbersome, shock produced short-latency muscle responses in con-
requires direct contact with the skin, and is painful in tralateral limb muscles, consistent with transmission via
patients with preserved sensation. rapidly conducting corticospinal fibers. Examination of
FMS as a noninvasive and potentially more-effec- the characteristics of these muscle responses, or motor
tive method of stimulating endogenous fibrinolysis, was evoked potentials (MEPs), permitted the study of con-
performed on twenty normal subjects (102). This FMS duction in central motor pathways. The clinical useful-
study was designed to test changes in fibrinolysis in blood ness of this technique, however, was limited by the fact
samples of patients taken before, 15 minutes after, and that it was painful. Barker et al. (3) showed that trans-
1 hour after FMS. The test for fibrinolysis was a modi- cranial magnetic stimulation (TMS) of the human motor
fied dilute whole-blood clot lysis method, and was per- cortex also produced short-latency contractions of con-
formed on site. The subject was placed in the prone posi- tralateral limb muscles, consistent with transmission via
tion, with the MC placed on the popliteal region, rapidly conducting corticospinal fibers. This technique
alternating from side to side every 30 seconds. The fre- has the advantage of being painless. It is now possible to
quency of stimulation, burst length, and burst interval evaluate corticospinal tract function by examining MEP
were 30 Hz, 4 seconds, and 26 seconds, respectively. Fib- characteristics in response to TMS. Clinical studies have
rinolysis was tested by the dilute whole blood clot lysis used TMS to establish the presence, threshold, latency,
time (WBCLT) in the tube by the method of Sirridge, and and size (amplitude, duration, and area) of MEPs in var-
a modified semiautomated method by using a sonoclot ious limb muscles, in both able-bodied individuals
coagulation analyzer. (104,105) and in those with central nervous system
758 RECENT ADVANCES IN SPINAL CORD RESEARCH
pathology (104,106108). Over the past 15 years there tive TMS can induce generalized seizures even in people
have been extensive developments with this methodology, with no known history of epilepsy (137,138). Guidelines
including clarification of mechanisms of activation for the safe use of repetitive TMS have been developed
(109111); safety issues (112116) establishment of nor- (116).
mal values (115,117120); and detection of abnormali-
ties (121,122).
Transcranial Magnetic Stimulation in SCI
The results of TMS are similar to those of transcra-
nial electrical stimulation (TES) (110), except that the Several studies have used TMS to examine the conduc-
latencies of the first responses are shorter with electric tion properties of corticospinal pathways in patients with
stimulation. This is thought to reflect direct activation of SCI (107,129,139,140). Initial safety concerns about the
descending axons (D-waves) with electric stimulation, use of TMS in acute SCI patients who may have metallic
whereas both electrical and magnetic stimulation produce stabilizing devices or unstable fractures were addressed
a series of later indirect waves (I-waves), by synaptic acti- as unfounded by Katz (115). Cortical stimulation typi-
vation of corticospinal neurons (123). The amplitude and cally produces synchronous compound muscle action
latency of the muscle responses elicited by TMS are potentials (MEPs) in muscles innervated above the level
affected by voluntary contraction of the muscle to be acti- of spinal cord lesion, but low-amplitude, prolonged
vated. Contraction facilitates the amplitude of the latency, high-threshold MEPs, or no response in muscles
response and causes a decrease in the latency. The state innervated from roots below the level of the lesion (141).
of activation of the spinal cord alpha motoneurons, even The appearance of MEPs generally tends to be related to
in the absence of contraction, also has an effect on the the clinical status of the patient, with reduced-amplitude
muscle response. If a motoneuron is close to its firing MEPs appearing in patients with incomplete lesions, and
threshold prior to stimulation, a response is more easily absent responses in patients with clinically complete
elicited. For this reason, it is important to control the state lesions. Instances exist in which MEPs may be present but
of activation of the motoneurons when studying MEPs. clinically measurable function is absent (141,142), and
An alternative technique is to record changes in the fir- instances in which MEPs are absent but preserved cor-
ing probability of repetitively discharging single motor ticocospinal function can be revealed through electro-
units in response to the stimulation, using peristimulus physiologic conditioning techniques (143).
time histograms. The area of a peak of increased firing The prolonged latency, caused by a slowing of cen-
can be used to obtain a measure of the amplitude of the tral motor conduction in SCI patients, has been demon-
composite excitatory post-synaptic potential (EPSP) gen- strated by numerous investigators (107,141,128,139,144)
erated in the motoneuron. and attributed to axonal demyelination (107,139). Even
Another development in the use of transcranial mag- in cases where substantial functional recovery has occurred,
netic stimulation over the past 10 years is the rate at central motor conduction abnormality has been found to
which stimuli can be applied. The traditional method of persist. A comparison of the latency changes in MEPs and
stimulating the corticospinal system is with single-pulse in SVCs (the silent period following TMS, known as sup-
TMS. This form of stimulation is arrhythmic, and is pression of voluntary contraction) in SCI patients has been
repeated no faster than once every 2 to 3 seconds. Faster used to explore changes in cortical inhibition, likely reflect-
cycling is prevented in single pulse stimulators by the ing altered function within the brain (145).
charging times of the capacitors, which store the electric Several investigators have proposed that TMS-
charge that generates the current in the coil. Several safety evoked potentials may be useful to predict motor recov-
studies have suggested that single-pulse TMS can be used ery in SCI patients (146,147). Although correlations have
without risk of side effects, such as epileptic seizures or been found between MEPs and ambulatory and hand
transitory memory impairment in all normal subjects function, its primary clinical benefit seems to be in pre-
(124). Although single-pulse TMS has been reported to dicting outcome in patients who are uncooperative or
induce seizures in patients with epilepsy (125,126), it has unable for some other reason to undergo a complete phys-
been safely applied to diverse patient groups, including ical examination. The technique has been found to be of
those with SCI (107,127129); Parkinsons disease use in diagnosing lesions of different neurologic structures
(130132); stroke (108,133); and multiple sclerosis within the spinal cord and in differentiating between con-
(134136). More recently, stimulators capable of gener- tributions toward functional recovery attributable to
ating pulses at up to 60 Hz have been developed. Repet- recovery of spinal motor tracts versus other mechanisms
itive TMS has the potential advantage of disrupting brain of neuroplasticity (e.g., alterations in cortical functions)
activity for the duration of a train of pulses, making it following SCI (147).
much easier to detect processing changes in studies of Focal transcranial magnetic stimulation provides a
cognitive function as well as in studies of sensory and reliable method of mapping the motor cortical represen-
motor function. Several studies have shown that repeti- tations of limb muscles (148), and thus to examine any
FUNCTIONAL MAGNETIC STIMULATION 759
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56
Mechanisms and
Natural History of
Spinal Cord Injury
ur understanding of the mechanisms logic mechanisms that contribute to delayed neuronal and
descending axons within the cord is greatest at the cervi- gists have classified subacute and chronic histopathology
cal level, where the free space around the spinal cord into four or five broad categories (Table 56.1): I. Cords
is minimal. At C5, the most frequent location of cervical with loculated central cysts or cavitations with varying
SCI, the difference between the diameter of the spinal amounts of spared white matter rim tissue; II. Cords with
cord and the bony canal is approximately 0.65 cm (1). severe compression injuries with disruption of the pial
Thus, there is little room for extraneous material such as surface and a predominantly collagenous scar with or
blood or bone fragments and little room to accommodate without some preserved axonal tracts; III. Cords that are
swelling. A key determinant of the degree of injury is the completely severed; IV. Cords with partial lacerations,
energy transfer through this heterogeneous tissue and such as injuries caused by knife or gunshot wounds
fluid system. The energy transferred during injury has (including the very uncommon BrownSequard syn-
been equated to a pressure pulse or wave that travels drome); and V. Cords with a less commonly described
along the spinal compartments, its speed and properties solid cord injury, as identified by Bunge et al. (5). In
modified by the tissue components and physical proper- this latter category, the spinal cord retains its continuity
ties specific to each location (2). The nature of these prop- without apparent central cord gray matter necrosis or cyst
erties favors early gray matter destruction, with relative formation and with preservation of much central gray
sparing of white matter tracts (3). matter. The main defect in this latter category is postu-
The resulting SCI clinical syndromes are also influ- lated as being secondary to demyelination and axon dis-
enced by the peculiarities of the anatomy of the spinal col- ruption, primarily in the lateral white matter tracts.
umn. Hyperflexion injuries result in compression of the It is hard to accurately determine the frequency of
posterior aspect of the vertebral body on the anterior occurrence of the various types and categories of injuries
aspect of the spinal cord. Hyperextension injuries pre- and the various pathologic responses. The data in Table
dominantly cause a central cord syndrome. Ischemic cord 56.1 are merely estimates summarized from data obtained
syndromes depend on the specific vascular territory from two relatively large databases, one in Miami, Florida
involved. The majority of injuries result from compres- and one in Perth, Australia (57,1214). However, many
sion, leaving a bridge of tissue connecting the proximal social and environmental factors affect the etiology and
and distal parts of the spinal cord (4,5). Crush or hyper- the frequency of injury type including local (i.e., urban,
flexion injuries more commonly result in clinically com- suburban, rural) cultural customs; frequency and type of
plete SCI, whereas hyperextension or compression behaviors and activities of increased risk (e.g., skiing, div-
injuries are more commonly associated with clinically ing). The estimates listed in Table 56.1 are provided pri-
incomplete injuries (6). However, as many as 13 to 25 per- marily to offer a range of frequencies and to help stress
cent of individuals surviving traumatic injuries sustain the variety of lesion pathology that can be seen. Not all
complete transections of the spinal cord (7). The veloc- therapeutic options and advances are likely to help indi-
ity and force of the injury are also important. Sudden viduals with each type of injury. To effectively manage
compressive injury is believed to result in more significant each form of injury, clinical and research interventions
injury with hemorrhage, swelling, and greater secondary must address the predominant mechanisms that underlie
damage and scarring. This hypothesis is supported by the the unique pathologic presentation in each case.
observations of Hayes and Kakulas (6), who completed Pathologic lesions with central cysts or cavitations
comparative pathologic studies of sports-related injuries are believed to evolve within a few months following the
and high-velocity injuries caused by motor vehicle acci- clearance of debris that is formed after a contusion injury.
dents. Similarly, in rodent models of SCI, the rate and Microscopic analysis of cross-sections of spinal cord early
magnitude of tissue displacement at the time of injury cor- after injury, from victims dead on arrival may reveal
relates with degree of tissue damage and functional out- little obvious damage or only scattered petechial hemor-
come (2,811). Slowly growing tumors and slowly rhages (15). However, within 12 to 24 hours, examina-
induced compressive injuries, such as those caused by the tion of postmortem tissue often reveals central hemor-
bony encroachment of cervical spondylosis or from pro- rhagic necrosis several segments rostral and caudal to the
gressive ossification of the posterior longitudinal liga- injury site (15). Within hours of injury, polymorphoneu-
ment, result in less scarring and respond better to decom- clear leukocytes (i.e., neutrophils) appear at the site of
pression surgery. injury followed shortly thereafter by macrophages and
other inflammatory cells (4). Eventually, the blood and
necrotic tissue is removed, apoptotic cells are cleared, and
NEUROPATHOLOGY OF HUMAN a residual fluid-filled cavity remains. Cavities may extend
SPINAL CORD INJURIES several segments rostral and caudal to the site of impact
and are often multiloculated. The cavities are surrounded
How the spinal cord is injured determines the nature of by thick gliotic walls and by varying amounts of poten-
the neuropathologic response. In general, neuropatholo- tially dysfunctional, yet anatomically preserved, residual
MECHANISMS AND NATURAL HISTORY OF SPINAL CORD INJURY 767
TABLE 56.1
General Patterns of Neuropathology Following SCI
*These are very rough estimates of the frequency of the various classes of injury derived primarily from the descriptions and sum-
maries of the pathologic findings from the databases from the Royal Perth Hospital at the University of Western Australia and the Miami
Project to Cure Paralysis in Florida (57,1214). Frequency of type of injury is likely to vary between various locales, urban, suburban,
rural, etc. DOA Dead on arrival at the hospital.
ascending and descending fibers. The subarachnoid space had cavities, and most had significant gliosis. White mat-
surrounding the cord at the level of the lesion may be infil- ter tracts accounted for 2.1 to 40.4 percent of the resid-
trated by fibrous tissue. Collagenous and fibrous tissue ual tissue in the injury zone (12). In the remaining three
may also comprise a part of the residual connecting bridge cases, residual central white matter tracts were apparent
of spinal tissue (5,7). but were without corresponding clinical functional cor-
Although a cystic lesion may develop as a subacute relates. This category of anatomically incomplete but clin-
consequence of SCI over weeks or months, some patients ically complete individuals has been termed by some as
may develop new cysts or expand former cysts years after discomplete (19,20). The amount of white matter spar-
their initial injury. This may occur following an addi- ing in ten clinically incomplete patients in the same study
tional, but more subtle injury. For example, slow pro- was not necessarily more than in the discomplete
gressive neurologic deterioration in previously stable SCI group, nor did the amount of spared tissue correlate with
patients may follow a fall or minor car accident or the amount of preservation of clinical function. Clearly
because of vertebral instability or spinal stenois secondary the location and functional nature of the preserved axons
to osteophitic overgrowth at the injury zone. These cysts hold more clinical relevance than the exact amount of
often can be visualized on magnetic resonance imaging white matter spared. In fact, using high-resolution MRI
(MRI) and can sometimes be followed as they expand in images, Metz et al. (21) found that the best functional out-
diameter or in rostral-caudal extent. There have been come was associated with the preservation of the ventral
recent attempts to use fetal tissue transplants to arrest white matter tracts. Aside from the laterally located cor-
expanding cyst formation in chronically injured SCI ticospinal and rubrospinal tracts, the bulk of descending
patients (1618). However, it is too soon to tell if this spinal pathways is located in the ventral funiculi (includ-
approach will yield significant long-term benefits. ing tectospinal, anterior corticospinal, vestibulospinal,
Occasionally, instead of a cavity, lost tissue is and medial reticulospinal tracts).
replaced by a solid collagenous and gliotic scar. In one Both complete transections and partial lacerations
study of eleven clinically complete patients, eight showed involve disruption of the dura and pial-meningeal tissues
no residual white matter at all (12). The majority of the that overlie the spinal cord. The numerous fibroblasts and
residual tissue consisted of connective tissue (13.3 to 100 mesenchymal cells in these structures proliferate profusely
percent); hypertrophied glia (56 to 94.2 percent), and in response to injury, with an exuberant deposition of a
regenerating dorsal nerve roots (5.8 to 44 percent) (12). dense collagenous connective tissue within the spinal
No axons of central origin were clearly identified in these parenchyma. In the case of the complete transection, the
cases. Of the ten clinically incomplete cases studied, seven severed ends of the spinal cord become encapsulated by
768 RECENT ADVANCES IN SPINAL CORD RESEARCH
Through safety education and the implementation of pro- *Adapted from McTigue et al., 2000 (28).
tective devices such as air bags and seat belts, we have
made some progress in reducing the frequency and sever-
ity of SCI. Based on preclinical experimentation in ani-
mal models, acute clinical interventions (i.e., methyl- ies of animal models of SCI. These studies have formed
prednisolone) are currently being studied and used in the the basis of the theory that there is often residual tissue
hope of minimizing the final extent of injury (2527). In that is not directly injured during the initial insult but
human necropsy specimens obtained within 3 months of becomes anatomically or physiologically compromised as
injury, there is still significant swelling, petechial hemor- a result of swelling, excitotoxicity, and perhaps acute
rhage, ongoing liquefaction of tissue, and ongoing inflammation (3234). The area of compromised tissue
macrophage phagocytic activity (6). The potential for expands, circumferentially as a function of time after
intervention immediately after and during the first few injury, but also rostral and caudal to the initial injury zone
months following injury is great. A more complete under- (Figure 56.1). Thus, acute interventions that minimize tis-
standing of the evolving interplay of cells, soluble mole- sue injury over time may have significant implications for
cular agents, and extracelluar matrices is needed to prospective recovery of function given that there is evi-
advance clinical interventions to further decrease sec- dence that considerable functional recovery can be
ondary injury and promote the wound healing process. achieved if even only 10 percent of spinal axons survive
Table 56.2 outlines a few of the processes involved the injury (35).
in the response to SCI. A key emerging concept is that
the very processes that are essential for spinal cord wound Animal Models of SCI
healing also may contribute to secondary injury and cre-
ate a hostile environment for functional recovery (28). For As discussed, various factors are important in deter-
example, inflammatory cells and mediators (e.g., mining the final extent of injury in humans, including
cytokines, chemokines) are critical for the removal of velocity, peak force, and tissue displacement. Several ani-
debris and in triggering the early phases of tissue repair mal models of SCI have been developed that allow for
and preserving structural integrity of the cord (29). How- controlled and reproducible injuries that correlate well
ever, these same cells and mediators can adversely affect with human injuries. In most animal models of contu-
the survival of neurons and glia, thereby leading to sion injury, a thoracic laminectomy is performed and a
increased secondary injury and scar formation (30,31). force transiently impacted on the exposed spinal cord.
Advances in understanding the acute dynamics of In weight drop models, which have been used extensively
the pathologic changes that occur immediately follow- in cats and rats, a small weight is dropped from a pre-
ing injury have been made primarily through careful stud- determined height onto the exposed spinal cord
MECHANISMS AND NATURAL HISTORY OF SPINAL CORD INJURY 769
FIGURE 56.2
Overview of the inflammatory reaction following experimental spinal cord injury. Although the inflammatory reaction is more
complex than depicted in this figure, the primary cellular components of the reaction are illustrated. Cellular reactions are graded
on a continuum from reactions that occur immediately to those observed for months or even years after injury. The intensity of
shading (from weakest to darkest) corresponds with documented changes in the magnitude of the cellular reaction. Note the
acute involvement of microglia and neutrophils followed by monocytes and lymphocytes. Also, note the biphasic response of
lymphocyte infiltration as observed in rat. Similar studies have not been completed for other species or humans after spinal
cord injury.
TNF-
(a pro-inflammatory cytokine) enhances the has been suggested that the timing of the delivery of this
permeability of endothelial cells and promotes the bind- cytokine is critical and that reduction of the early inflam-
ing of leukocytes to the endothelial cell surface thus facil- matory response is neuroprotective, whereas prolonged
itating infiltration of the spinal cord by circulating cells. or delayed suppression may antagonize intrinsic repair
Some evidence exists that TNF-
may be neuroprotective processes in which the immune system plays a pivotal role
in vitro; however, there is also confounding evidence that (51). The importance of the timing of a cytokine-regu-
suggests that when macrophages or microglial are simul- lated inflammatory response was similarly noted when
taneously present in vitro or in vivo, that TNF-
pro- comparing the effects of the injection of a cocktail of pro-
motes neurotoxicity (4648). Elevated levels of TNF-a inflammatory cytokines at 1 day or 4 days following
protein and mRNA have been detected as early as 1 hour experimental SCI. In this paradigm, delayed injections
after SCI, with persisting elevations for at least 7 days resulted in improved tissue preservation, although acute
after injury (45,49). Attenuation of TNF-
is associated administration of these cytokines resulted in greater tis-
with improved SCI outcome (49,50). sue loss (52). The production of cytokines at one time
IL-10 is a potent anti-inflammatory cytokine that point after injury may be neuroprotective and promote
blocks or decreases the production of various cytokines wound healing, whereas the effect of the same cytokines
and chemokines and suppresses monocyte inflammatory at other time points in the sequence of the injury response
responses. An injection of IL-10 shortly after an SCI con- may be destructive. Understanding when components of
tusion injury decreases the induction of TNF-
in the the immune response are beneficial versus when they are
spinal cord and improves functional recovery (49). How- detrimental should ultimately facilitate manipulation of
ever, it appears that whereas early suppression of the the injury response in the clinical setting.
inflammatory response may be beneficial, further alter-
ation of the endogenous response with a second dose of
Macrophages
IL-10 at 3 days after injury leads to no effect on tissue
sparing or recovery (49). Findings of this sort emphasize The importance of timing and specificity is also critical to
again the complicated and multifaceted role of the the positive or negative effects of the macrophage
immune system in the response to injury in the CNS. It response in SCI. Microglia are the resident macrophages
MECHANISMS AND NATURAL HISTORY OF SPINAL CORD INJURY 771
of the CNS and are among the first cells to respond to prednisolone in the clinical situation. It has been postu-
spinal cord trauma. There appears to be significant phe- lated that T-cells become sensitized to peptides of CNS
notypic heterogeneity, as determined by cell surface mark- origin after injury and that an autoimmune response to
ers, among spinal cord microglia (38,53). As these sur- these self-peptides (e.g., fragments of myelin basic protein
face markers may play a role in intracellular signaling, [MBP], or proteolipid protein) may amplify the injury
phenotypic heterogeneity may imply functional hetero- response.
geneity (54). This, in itself, may contribute to some of MBP-reactive T-cells can be isolated from peripheral
the differences seen in the injury responses in white mat- lymph nodes 7 days after SCI in the rat. When these cells
ter (delayed demyelination) versus gray matter (acute are injected into intact animals, the animals develop a par-
necrotization) (30). The heterogeneity of the macrophage alytic disorder similar to the acute neuroinflammatory
response to SCI is further complicated by the fact that res- disorder, experimental allergic encephalomyelitis
ident microglial reactions are bolstered by blood mono- (EAE)(61). Interestingly, T-cells isolated from later time
cytes that are recruited to the site of injury during the first points after SCI (i.e., 7 days) do not induce a similar
few days post-injury. Although there is likely considerable deficit, thus suggesting that regulatory mechanisms sup-
overlap in the functional potential of hematogenous press this potentially injurious autoimmune reaction. The
macrophages and resident CNS microglia, there is suffi- absence of a sustained autoimmune response after trau-
cient evidence to support a divergent role for these two matic SCI in humans suggests that if a similar phenome-
macrophage subsets in the injured spinal cord. In an effort non of T-cell activation against self-antigens occurs, it is
to tease apart the roles of endogenous macrophages suppressed (38,62). Still, it is possible that, during the
(microglia) that are activated at initial injury and the transient phase of T-cell activation, autoreactive T-cells
hematogenous derived macrophages (monocytes) that could contribute to neurodestruction or demeylination.
arrive after several days, anatomic and neurologic out- Just as a controversy surrounds the primary function
come has been evaluated in a rat model of spinal contu- of macrophages in the injured spinal cord, autoreactive
sion injury following selective depletion of peripheral T-cells are garnering similar attention. For example, recent
monocytes (55). In the absence of monocyte-derived evidence suggests that the exogenous administration of
macrophages, reduced necrosis and cavitation occur at MBP-reactive T-cells may lead to increased neural protec-
the lesion epicenter concomitant with increased axon. tion and behavioral improvement (63,64). Because all indi-
This suggests that acute monocyte infiltration of the viduals normally possess a low frequency of myelin-reac-
injury site contributes to secondary neuronal and glial tive T-cells, the manipulation of these cells in human SCI
injury. However, other studies suggest that, under the may be of therapeutic benefit (65).
appropriate conditions, activated macrophages may pro-
mote CNS repair. Macrophages activated with specific
Apoptosis, Demyelination, and Remyelination
proteins and then transplanted into the rodent spinal cord
have been shown to promote axonal regeneration and Immediately following SCI, necrotic cell death occurs
improve functional recovery (56,57). In animal studies, within the primary injury zone. In addition, apoptotic cell
macrophages activated by exposure to peripheral but not death occurs acutely within the lesion epicenter and also
optic nerve supported axonal regeneration (56,58). The extends rostral and caudal from the impact zone over time
key to the success of this paradigm appears to be the spe- (6669). This extension of the post injury cell death
cific molecule that has activated the macrophage. process lasts for more than 6 to 8 weeks and likely for
much longer. It involves primarily oligodendrocytes and
microglia along the axonal pathways undergoing pro-
T-cells and Autoimmunity
gressive Wallerian degeneration (70,71). This process pre-
In addition to discriminating between self and non- sumably contributes to delayed post-SCI demyelination.
self molecules, activated T-cells have many physiologic Eventually, most demyelinated axons become
tasks including modulating macrophage function, main- remyelinated. It appears that expanding populations of
taining microvasular endothelial integrity, and antibody oligodendrocyte progenitor cells and infiltrating Schwann
production by B-cells. The full extent of the role that these cells participate in this process (72,73). Numerous
cells play in the natural history of SCI is not yet clear. observers have identified axons in the injured cord, both
However, the sequential appearance of activated T-cells in human post-mortem tissue and in animal models, that
in the injured spinal cord suggests that they have a defined stain for both central and peripheral myelin, thus sug-
role in the orchestration of the normal wound response. gesting Schwann cell invasion of the cord and Schwann
The activation and response of these cells very much cell remyelination of demyelinated axons (7476).
depends on local cytokine and glucocorticoid levels. Glu- Schwann cells form a basement membrane that can be
cocorticoid levels are modified by SCI (59,60) and clearly stained even in poorly fixed tissue, whereas oligoden-
are affected by the exogenous administration of methyl- drocytes do not form a basement membrane around
772 RECENT ADVANCES IN SPINAL CORD RESEARCH
axons, thus facilitating their distinction. In a study from Recent evidence suggests that extracellular matrix
the Royal Perth Hospital in Australia, out of twenty-seven molecules in the injury zone, even in the absence of a sig-
cases studied at least 6 months post injury, sixteen showed nificant physical scar may halt the growing tips of regen-
remyelination of central axons by peripheral Schwann erating axons (86). Molecules, particularly those upreg-
cells (12). Axons were found coated with Schwann cells ulated by activated astrocytes (including tenascin-C and
near the lesion site and in continuity with oligodendro- several forms of chondroitin sulfate proteoglycan) asso-
cyte myelin further from the lesion site. Recovery of func- ciate with laminins to form a new matrix in the injury
tion has been demonstrated in animal models when zone (87,88). Although the laminins are generally con-
demyelinated axons have been remyelinated by Schwann sidered supportive of axon growth, proteoglycans are
cells, and these axons conduct impulses similar to normal more commonly considered to be inhibitory (89,90).
CNS axons (77,78). However, the situation is actually significantly more com-
Whereas Schwann cell remyelination of denuded plicated. Extracellular matrix molecules are complex mul-
axons occurs via invasion of the injured spinal cord by tidomain structures. Subregions or domains of the mole-
peripheral Schwann cells, oligodendrocyte remyelination cules may be growth inhibitory, whereas other regions on
is likely the result of proliferation and differentiation of the same molecule are stimulatory. For example, laminins
progenitor cells found throughout the spinal cord, which are composed of three chains, but tenascin-C is composed
are stimulated to multiply by factors produced after SCI of six arms with multiple epidermal growth factor (EGF)
(72,79). However, the combined efforts of the Schwann and fibronectin domains (91). It has been hypothesized
cells and oligodendrocytes are, in general, not sufficient that fragments of these molecules may assume new con-
to support clinically significant functional recovery. Stud- formations in the setting of CNS injury and that these
ies have demonstrated that the internodes in remyelinated may have unique effects on neurons (9193). Interven-
sections are not as thick nor as long as they are in nor- tions to alter the composition of the ECM following SCI
mal tissue (80,81). Studies are underway to explore inter- are currently being explored (9496).
ventions, such as the provision of growth factors such as
the neurotrophins Neurotrophin-3 (NT-3) and brain
Stem Cells, Progenitor Cells
derived neurotrophic factor (BDNF) to amplify prolifer-
ation of oligodendrocyte precursors cells in SCI models In amphibian models of successful spinal cord regenera-
to improve myelination (72). tion, a critical source for cells in the reparative and regen-
erative process appears to be a robust proliferative
response from the ependymal cells that line the central
Extracellular Matrix Molecules
spinal canal (97,98). In mammalian injuries, a prolifera-
The participation of the deposition of extracellular matrix tive response also occurs near the central canal that con-
molecules in the SCI wound healing process is perhaps as tributes to the formation of tissue bridges that fill the
problematic as the participation of the other processes injury zone (99101). However, this proliferative
described earlier. On the one hand, the formation of a glial response does not result in epimorphic regeneration of the
scar and deposition of these molecules is part of the wound mammalian cord. There may, nonetheless, be potential
healing process that is essential for responding to the for a more successful regenerative response than we had
destruction imposed by the injury. On the other hand, the suspected in the past. Recent studies in mammals have
exuberance of the response and the types of matrices laid demonstrated that multipotent progenitor cells exist
down may, in the long term, inhibit the regenerative efforts throughout the adult CNS, including the spinal cord, sug-
of injured axons (82). The type of scar tissue found at the gesting prospects for further enhancement of the recov-
injury zone depends in part on the type of injury suffered ery response (102,103). Harvested adult rat spinal cord
and, in the case of animal models, the species under study. progenitor cells can be induced to proliferate in vitro in
As described earlier, penetrating injuries that disrupt the the presence of fibroblast growth factor-2 into cells
duralmeningeal interface tend to result in greater fibrous expressing differentiation markers of astrocytes, oligo-
scarring with significant collagen deposition (4,22). Non- dendrocytes, and occasionally neurons (104). A recent
penetrating injuries tend to result in greater cavitation with study has demonstrated ongoing cell proliferation in the
increased deposition of glial and macrophage-derived intact adult rodent spinal cord, with the majority of pro-
extracelluar matrix molecules (83,84). Interestingly, the rat liferating cells maturing into astrocytes and oligoden-
SCI contusion injury models result in wounds with marked drocytes (103). In the uninjured cord, a division of these
cavitation, whereas the mouse models have wounds filled cells appears to occur throughout the cord rather than
with solid scar tissue (2,11,85). The difference between the as a result of migration from progenitors in the ependy-
SCI wound healing responses in each species may provide mal zone (103). It is not yet clear how progenitor cells
advantages for further exploration of the different contribute to the repair process and whether they can be
responses found in human wounds (2). manipulated in vivo to participate in a more functionally
MECHANISMS AND NATURAL HISTORY OF SPINAL CORD INJURY 773
successful repair of the injury zone. The demonstration Attempts to Alter the Natural History
of multipotent stem cells with proliferative potential of SCI in Humans
throughout the nervous system suggests promise for fur-
ther experimental interventions to augment the endoge- Little pathological data is available to assess the anatomic
nous repair process in SCI. effect of our recent attempts to alter the natural history of
SCI using exogenous substances such as steroids or gan-
gliosides in acute SCI. Bunge (13) reports on one case of
CHRONIC SCI a young man who received methylprednisolone within 2
hours of his injury but eventually died of sepsis on day
Late Pathologic Complications of SCI 16. Compared to the other acute cases reviewed through
the Miami Project, the injury zone in this individual
It is not uncommon to find slow progressive neurologic appeared to be more restricted in rostral-caudal extent. A
deterioration years after an injury. Common causes similar finding of decreased extension of the lesion zone
include the late development of syringomyelia, perhaps following injury has been observed in animal studies of
caused by tethering of the cord by fibrous scarring and methylprednisolone (120). In addition, despite the sub-
arachnoidal pathology. A recent study on spinal thecal sac stantial loss of tissue and necrosis in the center of the injury,
constriction in rodents supports the theory that induced there was preservation of the entire circumference of the
constrictive pressure gradients contribute to cyst forma- outer rim of the spinal cord. Even at the most necrotic core
tion (105). Hypertrophy of injured bony tissues at the old of the epicenter, there was a small number of nerve fibers
injury site can lead to spinal stenosis and cord flattening, traversing the dorsal columns. Although it is intriguing to
thus leading to further neurologic deterioration over time. speculate a causal relationship between steroid treatment
A study of seventy-five SCI patients in France demon- and improved neuroanatomic outcome, the findings
strated a significant correlation of the occurrence of described are based on one case and are within the range
syrinx with spinal canal stenosis and argues for early sur- of neuropathologic injuries described prior to the use of
gical intervention and spinal realignment (106). It has methylprendisolone. The recent detailed research using
been suggested that some of the deficit resulting from the animal models of SCI has provided a plethora of infor-
compression of cervical spondolysis may involve demyeli- mation about cell types, molecules, and timing of expres-
nation of the pathways in the spinal cord (5). A similar sion for the further development of more specific, targeted
phenomenon has been described in animal models, with approaches to intervention following SCI. Hopefully we
additional demonstration of remyelination after decom- will see the benefits of this knowledge in the development
pression (107). Occasionally, one can find a patient who of new treatment strategies in the near future.
does not show significant recovery immediately follow-
ing decompression but who regains some sensory or
motor function many months after decompression. One CONCLUSION
can postulate that this late recovery might be caused by
the subsequent remyelination of intact axons. It has become increasingly clear that the natural evolution
of each spinal cord injury depends on multiple factors.
Synaptic Plasticity Numerous simultaneous and synchronous processes orches-
trate the wound healing response, some beneficial and some,
The loss of projections to distal targets following SCI perhaps, harmful in their exuberance. To effect more suc-
affords the adjacent circuitry and terminals the opportu- cessful functional recovery, it will be necessary to anticipate
nity to sprout and form new synapses and make changes the type of ongoing injury response and consider the appro-
in synaptic strength at other sites. Several studies have priate timing of the targeted intervention or interventions.
demonstrated reorganization within the brain following
SCI (108,109). Recent studies suggest that reorganization
of cortical and subcortical circuitry may be responsible Acknowledgments
for the phenomena of phantom sensations frequently This work was supported in part by funds supplied by the
observed following SCI (110112). Evidence also suggests National Institutes of Health (NS-37321, P50 DE13749).
reorganization of intraspinal circuitry after SCI with the
potential for further synaptic changes following behav-
ioral interventions (113119). Some of these changes pre-
sumably occur shortly after injury, others over months.
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57 Acute Treatment Strategies
for Spinal Cord Injury
FIGURE 57.1
ondary injury to neurons and axons. Knowledge of this neuropathology compared to rats fed a normal vitamin
mechanism prompted the development of neuroprotec- E diet (2 I.U./100g diet) (7).
tive pharmacologic strategies aimed at antagonizing oxy-
gen radicalinduced LP in a safe and effective manner.
HIGH-DOSE METHYLPREDNISOLONE
THERAPY IN EXPERIMENTAL SCI:
PROTECTIVE EFFECTS OF EFFICACY AND MECHANISMS
THE LIPID PEROXIDATION
INHIBITOR ALPHA TOCOPHEROL Methylprednisolone (sodium salt of the 21-succinate
IN EXPERIMENTAL SCI ester, MP, Figure 57.2) is a synthetic glucocorticoid steroid
that was initially developed for the purpose of enhanced
Alpha tocopherol (i.e., vitamin E, Figure 57.2), which is antiinflammatory activity together with lessened miner-
a well-known membrane-targeted scavenger of lipid per- alocorticoid activity compared to the prototypical glu-
oxyl radicals, has been examined in models of SCI. How- cocorticoid cortisol (hydrocortisone). Several years ago,
ever, each of the available studies has had to utilize inten- we began a series of studies in which we systematically
sive pretreatment, based on the slow uptake of tocopherol examined the experimental efficacy of glucocorticoid
by the central nervous system (CNS). Accordingly, in cats steroids in experimental CNS trauma using MP as a pro-
pretreated with 1,000 I.U. of alpha tocopherol orally once totype. Our primary mechanistic focus has been on the
daily for 5 days and subjected to a severe spinal cord con- hypothesis that the principal molecular basis for sec-
tusion injury, there is a significant attenuation of pro- ondary posttraumatic neuronal degeneration is LP
gressive posttraumatic spinal cord ischemia development, induced by oxygen free radicals, and that glucocorticoid
measured in white matter of the injured spinal segment steroids might be well suited to inhibit LP, in view of their
(4). This dosing regimen had previously been shown to affinity for cell membranes and known membrane stabi-
reduce lipid peroxidative damage to cat spinal cord (5) lizing properties (8).
induced by microinjection of ferrous chloride. Subsequent An initial in vivo study demonstrated that pretreat-
studies have demonstrated that identical vitamin E pre- ment of cats with a single large i.v. dose of MP served to
treatment can enhance the chronic recovery of cats sub- protect homogenates of uninjured spinal cord, removed
jected to spinal cord compression injury (6) compared to 1-hour after steroid administration, from in vitro lipid
nonsupplemented animals. peroxidation. Most striking was the demonstrated need
On the other hand, vitamin E deficiency acts to for a very large unconventional dose of 30 mg/kg; pre-
increase posttraumatic pathophysiology. For instance, in treatment with lower doses was ineffective. On the other
rats subjected to spinal cord compression injury, vitamin hand, the doseresponse curve for this antioxidant
E deficiency ( 0.1 I.U./100 g diet chronically) resulted effect was biphasic. Doubling the dose to 60 mg/kg caused
in greater posttraumatic ischemia, lipid peroxidation, and a loss of effect and tripling it to 90 mg/kg actually pro-
ACUTE TREATMENT STRATEGIES FOR SPINAL CORD INJURY 779
FIGURE 57.2
moted subsequent in vitro lipid peroxidation (9). In a par- at concentrations as high as 1 mM. Thus, the LP-inhibit-
allel study, in which the spinal tissue levels of MP from a ing structureactivity relationships deviated significantly
single large dose were followed over time, it was observed from that for glucocorticoid receptormediated actions.
that the time-course of the antioxidant effect followed the Following these earlier pilot experiments, it was dis-
tissue pharmacokinetics quite closely, indicative of a non- closed that a 15 to 30 mg/kg i.v. dose of MP, given to
glucocorticoid receptor mechanism of action (10). In cats soon after blunt SCI, could also attenuate posttrau-
other words, the steroid had to be present in the spinal matic LP, as measured by various biochemical indices
tissue in a critical concentration for the protection against (1214). In addition to the characterization of the abil-
LP to occur. Subsequent to these initial studies, MP was ity of high doses of MP to inhibit LP, it was demonstrated
shown to directly inhibit peroxidation in rat brain synap- that a high dose can also exert a number of other actions
tosomal membranes when present in a 100
M concen- on the injured spinal cord that clearly contribute to an
tration. Lower and higher concentrations produced less attenuation of posttraumatic neuronal degeneration.
effect (11). In that study, the antioxidant properties of Because all of these actions require high doses of MP, they
other glucocorticoids were also examined. The widely are likely to be secondary to the inhibition of peroxida-
used ultrapotent steroid dexamethasone was also more tion-related processes. The list includes the support of
potent than MP in protecting synaptosomes from oxygen energy (1517); prevention of progressive posttraumatic
radical damage, but its maximal efficacy was slightly less ischemia development (18,19); reversal of intracellular
than MP. Prednisolone was equally effective, but less calcium accumulation (18); prevention of calcium over-
potent than MP. The prototype glucocorticoid steroid load-induced, calpain-mediated neurofilament degrada-
hydrocortisone was ineffective as an antioxidant, even tion (17); and attenuation of glutamate release (20). Prob-
780 RECENT ADVANCES IN SPINAL CORD RESEARCH
ably related to these beneficial biochemical and physio- tantly, however, LP-mediated tissue degeneration evolves
logical actions, a 30 mg/kg i.v. dose has been shown to rapidly after injury and is predominantly irreversible.
enhance the acute recovery of somatosensory evoked A fourth finding is that the time-course of MPs pro-
potentials (18). However, the central effect in this pro- tective effects parallels the tissue uptake and elimination
tective scenario is inhibition of posttraumatic LP, as illus- of the steroid. Because the half-life of MP in cat spinal tis-
trated in Figure 57.3. Indeed, further work has shown sue is only 2 to 6 hours, frequent doses are necessary to
that although pretreatment with MP can inhibit mem- maintain blood flow, improve tissue preservation, and
brane lipid hydrolysis (e.g., arachidonic acid release) and maximize the potential for recovery (16,17). To demon-
consequent vasoactive prostaglandin F2
and thrombox- strate the importance of taking this issue seriously, the
ane A2 formation (14), post-injury treatment with high comparative effects of a single versus a double dose reg-
doses of MP does not attenuate prostaglandin F2
and imen of MP were examined on spinal tissue lactic acido-
thromboxane A2 levels. This suggests that the anti- sis, ATP levels, and neurofilament preservation in con-
inflammatory glucocorticoid receptor-mediated inhibi- tused cat spinal cord at 4 hours post-injury (17). Cats that
tion of arachidonic acid release is not operative when MP received a 30 mg/kg i.v dose of MP at 30 mins post-injury
is administered after injury has occurred (21). Further- showed elevated lactate levels, ATP depletion, and
more, spinal cord protective doses of MP do not appear reduced energy charge and neurofilament loss essentially
to significantly inhibit posttraumatic neutrophil invasion equivalent to that seen in vehicle treated cats. However,
into the injured cord parenchyma (22). cats that received the 30 mg/kg i.v. dose at 30 minutes
Four observations describe the action of MP in SCI. post-injury plus a second 15 mg/kg i.v. dose 2 hours later
First is the necessity for large intravenous doses, as showed significantly better energy metabolism and neu-
described above. rofilament preservation than the vehicle-treated cats.
Second is the complex, biphasic doseresponse curve Moreover, the double-dose treated spinal cords were not
seen for many beneficial effects of MP; although an intra- significantly different from uninjured cords in terms of
venous 30-mg/kg dose is required to inhibit LP, doubling lactate, ATP, and neurofilament content.
the dose causes a loss of that action. The most logical Subsequent to this study, a chronic recovery trial was
explanation for such a pattern is that the high-dose phar- undertaken in which an intravenous dosing regimen was
macology of MP is mediated by a direct membrane tested in cats subjected to moderately severe spinal injury.
action. To illustrate these characteristics, Figure 57.4 dis- The regimen was designed to maintain effective antioxi-
plays the doseresponse correlation for the effect of MP dant concentrations in the injured spinal cord for a 48-
in inhibiting LP in spinal cord tissue with the effect of sup- hour period after injury (24). An initial 30 mg/kg bolus
porting aerobic energy metabolism (i.e., decreased lactate was given 30 minutes after injury, followed with 15 mg/kg
accumulation) and maintenance of normal blood flow. doses 2 and 6 hours later, and then a continuous infu-
The third observation is that treatment must be ini- sion of 2.5 mg/kg/hour for the remainder of the first 48
tiated early to achieve a therapeutic effect on the injured hours after injury. Cats were blindly evaluated over 4
spinal cord. The uptake of MP by spinal tissue decreases weeks for their ability to walk, run, and climb stairs, after
rapidly with time after injury (23), probably because of which they were sacrificed; a histologic analysis of the
secondary posttraumatic tissue loss and a progressive injury site was then conducted. Vehicle-treated cats typ-
decrease in blood flow to the injury site (19). More impor- ically remained paraplegic at the 4-week follow-up. In
FIGURE 57.3
HIGH-DOSE METHYLPREDNISOLONE
EFFICACY IN NASCIS II
potential for glucocorticoid receptormediated steroid III trial, three groups of patients were evaluated. The first
side effects (such as diabetic problems, immuno- (control) group was treated with the 24-hour MP dosing
suppression and infection, impaired wound healing, gas- regimen that had previously been shown to be effective in
tric ulceration, and negative nitrogen balance). NASCIS II. The second group was also treated with MP,
To test this hypothesis, a number of MP analogs were except that the duration of MP infusion was prolonged
prepared that lacked the 11-beta hydroxyl functionality to 48 hours. The rationale for this regimen was to deter-
that is known to be essential for glucocorticoid receptor mine whether extension of the MP infusion from 24 to
binding. The majority of these nonglucocorticoid steroids 48 hours resulted in greater improvement in neurologic
were either delta-9, 11, or 11-alpha substituted com- recovery. The third group of patients was treated with a
pounds. They were initially screened for their ability to single 30 mg/kg IV bolus of MP plus 48-hour adminis-
inhibit CNS tissue LP in terms of a protection of 14C- tration of tirilazad. No placebo group was included
labeled gamma aminobutyric acid (14C-GABA) uptake by because it was deemed ethically inappropriate to with-
rat brain synaptosomes from LP-induced impairment. The hold the initial large bolus of MP. Another objective of
more active antioxidants in the series were then secon- the study was to determine whether treatment initiation
darily screened in a severe head-injury model in mice for within 3 hours following injury was more effective than
promotion of early neurologic recovery. The most impres- therapy initiated from 3 to 8 hours post-SCI.
sive compound in these assays was U-72099E (Figure 57.2; Upon completion of the NASCIS III trial, it was
34). This compound was demonstrated to be devoid of glu- found that all three treatment arms produced comparable
cocorticoid activity in mice (i.e., lack of weight gain sup- degrees of recovery when treatment was begun within the
pression, lack of suppression of thymic involution, and fail- 3-hour window. When the 24-hour dosing of MP was
ure to inhibit ACTH release from cultured pituitary tumor begun more than 3 hours post-SCI, recovery was poorer
cells). However, despite its essentially complete lack of glu- in comparison to the cohort treated sooner than 3 hours
cocorticoid receptormediated activities, U-72099E was following SCI. However, in the 3- to 8-hour post-SCI
equally effective, yet more potent than MP at protecting cohort, when MP dosing was extended to 48 hours, sig-
rat brain synaptosomes from LP-induced inhibition of nificantly better recovery was observed. In the compara-
synaptosomal 14C-GABA uptake. In addition, it equaled ble tirilazad cohort (38 hours post-SCI), recovery was bet-
the ability of MP to enhance the early neurologic recov- ter than in the 24-hour MP group, and poorer than in the
ery of head-injured mice. These results confirmed the 48-hour MP group. These results showed that: a) initia-
hypothesis that the neuroprotective effects of MP were tion of treatment within the first 3 hours is optimal; b) the
independent of glucocorticoid receptor mechanisms, and non-glucocorticoid tirilazad is as effective as 24-hour MP
that a nonglucocorticoid steroid could duplicate the therapy; and c) if treatment is initiated more than 3 hours
antioxidant efficacy of MP in models of neuronal mem- post-SCI, extension of the MP dosing regimen is indicated,
brane damage and acute CNS trauma. from 24 hours to 48 hours. However, in comparison with
Subsequently, it was found that the substitution of the 24-hour dosing regimen, significantly more glucocor-
a complex amine on the nonglucocorticoid steroid tioid-related immunosuppressive side effects were seen
nucleus in place of the 21 hydroxyl functionality results with more prolonged dosing; that is, the incidence of severe
in a dramatic enhancement of the lipid antioxidant activ- sepsis and pneumonia significantly increased. In contrast,
ity. Many of these 21-aminosteroid compounds tirilazad showed no evidence of steroid-related side effects,
(Lazaroids) effectively inhibit iron-catalyzed LP in rat suggesting that this nonglucocorticoid 21-aminosteroid
brain tissue homogenates under assay conditions in which would be safer for extension of dosing beyond the 48-hour
the glucocorticoid steroid MP and the nonglucocorticoid limit used in NASCIS III.
analog U-72099E are completely ineffective. Of these, U-
74006F (tirilazad mesylate, Figure 57.2) showed excel-
lent activity in experimental models of spinal cord injury
FUTURE APPROACHES TO ACUTE
(30,3537) and was entered into clinical development.
PHARMACOTHERAPY OF SCI
Possibility of Extending
COMPARISON OF 24- AND 48-HOUR
Tirilazad Administration
METHYLPREDNISOLONE AND
TIRILAZAD IN NASCIS III The next logical step in pharmacologic development is
to build on the successful, but partial, neuroprotection
The demonstrated efficacy of a 24-hour dosing regimen achieved with MP and tirilazad. One obvious approach
of MP in human SCI in NASCIS II (3133), and the dis- would be to extend the duration of treatment with tiri-
covery of tirilazad (36-37), led to another trial of these lazad based on its apparent clinical efficacy and its greater
agents in human SCI, NASCIS III (39). In the NASCIS safety compared with MP. Theoretically, this would offer
ACUTE TREATMENT STRATEGIES FOR SPINAL CORD INJURY 783
additional protection from prolonged posttraumatic LP axonal sprouting; inhibitors of the myelin-derived NOGO
reactions within the injured spinal cord with little added protein, which acts to inhibit axonal growth (51); and, per-
risk to the patient. On the other hand, the side effect pro- haps, monosialoganglioside (GM 1) (52). The value in
file of 48-hour MP therapy in NASCIS III indicates that combining MP with a neurorestorative compound (basic
48 hours of intensive dosing is the upper limit of tolera- fibroblast growth factor) has in fact been demonstrated
bility and improved functional recovery. (53). Neuroprotective and neurorestorative agents also
have potential applications in cell transplantation, where
they could serve to protect transplanted cells and promote
Novel Scavengers of Reactive Oxygen Species their differentiation and growth.
In view of the clear role of reactive oxygen or oxygen rad-
icalinduced LP in the pathophysiology of posttraumatic CONCLUSION
spinal cord degeneration, and the demonstrated benefits
of antioxidant compounds with neuroprotective activity Methylprednisolone and tirilazad represent the first and sec-
like MP and tirilazad, it is logical to pursue the develop- ond generations of neuroprotective agents for acute SCI.
ment of novel antioxidant compounds. Recent work sug- They form the foundation for an exciting continuum toward
gests that the most critical reactive oxygen species in acute pharmacologically restoring maximal function to individ-
SCI may be peroxynitrite, which is formed from the com- uals with SCI. Future interventions are likely to include a
bination of superoxide and nitric oxide radicals (40). Per- variety of neuroprotective agents with different mechanisms
oxynitrite is capable of causing widespread damage to of action, as well as neurorestorative agents that promise
lipids, proteins, and nucleic acids. Prototypical scavengers even greater potential for functional recovery. By building
of peroxynitrite include penicillamine and Tempol, both on our past success, we will further interfere with the mech-
of which are neuroprotective in cell culture and in vivo anisms of the secondary injury process, and thus enhance
models of acute CNS injury (41,42). the potential efficacy of cellular renewal approaches involv-
ing the transplantation of stem cells and gene therapy.
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58 Recent Advances in
Neural Regeneration
785
786 RECENT ADVANCES IN SPINAL CORD RESEARCH
review focuses on the situation following spinal cord injuryparalysis and the loss of somatic sensation.
injury (SCI), although many of the principles are applic- Accordingly, restoration of voluntary motor control and
able after injury to other parts of the nervous system. sensory loss requires the re-establishment of pathways
In terms of the final degree of functional recovery, that are capable of long distance communication.
preventing secondary degeneration is likely to be of crit- That injured axons are capable of long distance
ical importance. The importance of such strategies can be regeneration was shown almost 100 years ago, with the
easily seen by considering the impact of the concept that demonstration that severed rabbit axons could grow up
stroke is a brain attack, and is important to treat emer- to 1cm along sciatic nerve grafts (8). This was confirmed
gently in the same way as a heart attack. This approach more recently using modern investigative techniques, the
to treatment has greatly improved functional outcome fol- key discoveries coming from Albert Aguayos laboratory
lowing stroke. Neuroprotective strategies for the acutely in the early 1980s (9). These investigators demonstrated
injured CNS are dealt with in another chapter of this text. convincingly that grafts of peripheral nerves could stim-
Here, we focus on growth and repair responses that may ulate the long distance regeneration of amputated cen-
begin soon after the injury, but that occur primarily after tral axons in both the optic nerve and the spinal cord.
degeneration has run its course. These processes have the These landmark studies are recognized as being the key
potential of mediating the recovery of function in the factors leading to the reversal of what had previously been
chronic post-injury period. a generally pessimistic attitude regarding the possibility
The most dramatic effects of SCI are caused by the of CNS regeneration. Clearly, these studies changed the
interruption of long tracts that carry information to and course of nervous system injury research.
from the brain and distal regions of the spinal cord. Although peripheral nerve grafts promoted axonal
Accordingly, when thinking about SCI, we tend to focus regrowth through the graft, axons usually failed to re-
on long-distance regeneration of long tracts. At the same enter the CNS parenchyma. In the case of the optic nerve,
time, however, it is important to recall that virtually all peripheral nerve grafts did enable axonal regeneration to
SCIs also cause gray matter damage, leading to the loss the superior colliculus and some degree of synapse for-
of local circuitry. Indeed, most contusive injuries cause mation, but the number of new synapses was small (10).
selective injury to the gray matter in the core of the spinal Nevertheless, these studies demonstrated that adult CNS
cord, leaving a variable rim of white matter. Hence, the axons have the inherent capability to regenerate, and sug-
problems associated with spinal cord injury are caused by gested that the inability of the adult CNS to regenerate
both axotomy (damage to axons) and direct neuronal loss can be attributed to an inhospitable CNS environment.
in the injured segment, which includes second-order sen- There are two general possibilities to explain this
sory neurons, interneurons, and the lower motorneurons nonsupportive environment: a) that the CNS contained
that supply musculature. active inhibitors of axonal outgrowth; and b) that the
Following axotomy, the surviving proximal region CNS tissue environment lacked important neurotrophic
of a severed axon initially dies back, but then often molecules that were necessary for successful regeneration.
exhibits a regenerative response visible as clubs of As it turns out, both of these concepts had considerable
growth, or growth cones in the current nomenclature merit, and in this chapter we review recent therapeutic
(6). These regenerative sprouts usually fail to extend interventions involving blockade or removal of neurite
beyond 1mm, and may be retracted and resorbed (7). This growth inhibitors, or implantation of growth permissive
failure to regenerate has led to the misconception that the tissue. We also review the pharmacologic and genetic
regenerative response to CNS injury is in all instances methods of altering intracellular mechanisms of growth
abortive. As we will discuss later, neurons actually do cone advance. These manipulations allow axons to
exhibit a number of regenerative growth responses that advance over environments that are otherwise associated
lead to the formation of novel connections, and these con- with growth inhibition.
nections may contribute importantly to recovery of func- The studies reviewed in this chapter demonstrate
tion. Still, the sine qua non is bona fide restoration of con- that incremental restoration of function is achievable by
nections lost as a result of injury. In the case of the injured targeting elements of the multifactorial cause of regener-
spinal cord, this requires long-tract regeneration, because ative failure in the adult CNS. Thus, combined treatments
voluntary motor function is mediated by long pathways that integrate two or more of these approaches hold
that originate from the cortex, midbrain, and brainstem promise for optimizing success. These recent and promis-
and terminate at segmental levels in the spinal cord. Sim- ing advances call for increased collaboration between
ilarly, somatic sensation is mediated by long pathways researchers, and increased dialog between researchers and
that originate in the dorsal root ganglia and relay through clinical trial specialists, to hasten the ethical translation
second-order neurons in the spinal cord and brainstem. of these advances to the human condition.
Spinal cord injuries interrupt these long pathways, and We begin by considering growth processes that do
cause the principal symptoms associated with spinal occur in the injured nervous system, even without inter-
RECENT ADVANCES IN NEURAL REGENERATION 787
vention. Then we consider those novel, currently exper- retrained to mediate useful function. An understand-
imental, interventions that offer the potential to promote ing of post-lesion reorganization will yield insights into
true long-tract regeneration that would otherwise not neural mechanisms that influence post-lesion functional
occur. capacity. Moreover, the development of manipulations
that alter regenerative plasticity offer the potential to sig-
nificantly modify the outcome of CNS trauma. In the fol-
REGENERATIVE PLASTICITY lowing section, we review recent developments in the field
of regenerative plasticity, including the exciting demon-
Regenerative plasticity is the term applied to neuronal stration that motor ability following injury to the human
growth responses that occur after injury that do not spinal cord can be defined, to a large degree, by training.
involve long-distance axonal regeneration. Clinicians and
patients have long suspected that the injured CNS has
A Brief History of the Key Findings
some capacity to recover, primarily because of the obser-
Related to Regenerative Plasticity
vation that CNS-injured patients often show significant
functional recovery without intervention. This recovery Today, the idea of axonal and synaptic growth in the
is also seen in SCI patients with severe motor and sen- mature nervous system is completely accepted. This was
sory deficits immediately following the injury. The initial, not the case, however, during the first half of the twenti-
and often most extensive, phase of functional recovery eth century. Until the mid-1950s, the view of the scien-
takes place during the first days to weeks after injury. This tific community regarding inherent regenerative plastic-
early recovery is likely caused by a reversal of edema, ity was dominated by the experiments of Tello and
restoration of blood flow and tissue homeostasis, and Ramon y Cajal at the turn of the twentieth century, which
metabolic changes at the lesion site that allow neurons suggested that the injured adult CNS was capable of only
to return to their normal functional activity. Following abortive growth (6). Ramon y Cajals famous quote set
SCI, this recovery period corresponds to the resolution of this tone of pessimism:
spinal shock, which is the clinical term that refers to the
general depression of spinal cord function after injury. Everything may die, nothing may be regenerated.
However, recovery can continue for several years after the
injury, raising the possibility that inherent repair processes This pessimistic view began to change in the late
take place involving neuronal growth. 1950s, with the demonstration by Liu and Chambers that
Several types of post-lesion neuronal growth have the CNS (indeed, the injured spinal cord) could respond to
been well documented. One involves the formation of injury by the formation of regenerative sprouts from adja-
novel circuits through sprouting and anatomic reorgani- cent undamaged axons (12). This was the first example
zation. This type of growth involves axonal branching, of the sprouting phenomenon, later documented in a vari-
synaptogenesis, and dendritic growth and reorganization. ety of brain regions. Further progress was initially remark-
Such reorganization may be especially important follow- ably slow, although a group at the University of Pennsyl-
ing incomplete SCI; in which cortical, subcortical, and vania, following up on the findings of Liu and Chambers,
local spinal cord circuitry remains wholly or partially continued to explore the sprouting phenomenon. Over the
intact and interconnected by unlesioned fibers. subsequent decade, papers appeared here and there docu-
Another form of regenerative plasticity in the spinal menting sprouting phenomena in other parts of the ner-
cord involves the aberrant growth of axons that have been vous system [especially the visual system, see (13)]. These
damaged. These abortively regenerating axons can studies reported the growth of axonal sprouts into new ter-
grow to form new synaptic connections. For example, ritory, but it remained to be determined whether these
damaged axons in white matter that fail to grow along sprouts actually formed synaptic connections, or whether
their normal trajectory often grow into nearby gray mat- the sprouting was abortive, with axons ending blindly.
ter, where they form synaptic contacts. These lesion- Indeed, probably the majority in the field assumed the lat-
induced connections are likely to be of functional signif- ter because of the still prevailing views of Cajal, and the
icance. For example, these aberrant connections may general acceptance of the view that all growth in the
contribute to abnormal reflex responses, and also to the mature nervous system was abortive.
highly unusual motor responses that can be elicited by Within this historical context, the 1969 report of
tactile stimulation (11). G. Raisman took on a special significance (14). Raisman
There is also reason to believe that these new con- provided the first definitive evidence that new synapses
nections could mediate novel, adaptive functions that help could form after injury. Specifically, Raisman presented
to compensate for the injury. Moreover, recent evidence convincing electron microscopic evidence that neurons in
strongly suggests that connections in the spinal cord, the septal nucleus were reinnervated after their normal
including aberrant ones that form after injury, can be connections had been destroyed by a lesion.
788 RECENT ADVANCES IN SPINAL CORD RESEARCH
Raismans landmark paper motivated a wave of lished through detailed quantitative electron micro-
experiments in the early 1970s involving other brain scopic studies of synapse replacement on neurons
regions, especially the hippocampus, which conclusively in the hippocampal dentate gyrus (20,21). This brain
demonstrated lesion-induced reorganization of circuitry region offers particular advantages for such studies
following injury. These studies established that regener- because of its very precise anatomic organization,
ative plasticity was ubiquitous, and could lead to the for- and the fact that afferent pathways can be selectively
mation of new physiologically operational synaptic con- destroyed. This fact suggests that denervated neu-
nections with neurons that had lost their normal rons provide a strong stimulus for local synapse
connections following lesion (15). growth, perhaps by releasing target-derived growth
At about the same time that these anatomic studies factors that act on nearby axons.
were going on, another line of research documented Reinnervation does not usually involve long-dis-
mechanisms for modifying the strength of existing tance axon growth. Reinnervation is accomplished
synapses. The lead discovery was of the phenomenon of as a result of the local sprouting of axons already
long-term synaptic potentiation [LTP, see (16)], which in place in the denervated territory, in which the
was followed shortly thereafter by the discovery of long- maximal growth of an axon is a few tens of microm-
term synaptic depression (LTD). These findings are highly eters. This fact places an important limit on the pos-
significant in the context of rehabilitation research sible sources of reinnervating fibers. Nevertheless,
because they indicate that existing circuits (including the lesion-induced growth may lead to the expansion
aberrant circuits that form after injury) can be modi- of a projection that is so small as to be virtually
fied by delivering appropriate patterns of activity. For the undetectable in the normal situation. Thus, a pro-
clinician and patient, this means that it will eventually jection that is almost exclusively unilateral in the
be possible to design rehabilitation strategies to take normal situation may expand to provide bilateral
advantage of these inherent plastic capabilities of neural input.
circuits. In this way, it should be possible to develop treat- Reinnervation leads to the formation of physiolog-
ments and functional interventions that can profoundly ically functional synaptic connections. In all cases in
influence the outcome of CNS injury. which it has been studied, new synapses that form
In the 10-year period following Raismans initial on denervated neurons are capable of synaptic trans-
paper, there were literally dozens of studies that reported mission. Moreover, reinnervating fibers can restore
lesion-induced synaptic reorganization in different brain a degree of control over the firing properties of the
regions. The hippocampus was a favorite site for such postsynaptic neurons. For example, in the hip-
studies, because its precise laminar organization permit- pocampal dentate gyrus, denervation leads to a sub-
ted easy detection of new connections [for a review, see stantial decrease in the synaptic drive of the affected
(17, 18)]. It has not been definitively established that the neurons, thus decreasing their ongoing firing rates.
rules defined by studies in the hippocampus apply to With reinnervation, normal levels of neuronal activ-
other structures, but it is thought that they do, especially ity are restored (22). This restoration of overall lev-
to regions of the cortex where the basic cell biologic prop- els of excitatory input may help the formerly den-
erties of neurons are generally comparable (19). ervated neurons to re-engage in their normal
physiologic functions.
Denervated neurons are often reinnervated. Cer- Following unilateral lesions, reinnervation is often
tainly one key conclusion that came from this era is accomplished by the contralateral homolog of the
that following injury, denervated neurons rarely damaged structure. Reinnervation by the contralat-
remain in the denervated state. They either die eral homolog is of considerable potential importance
(undergo transneuronal degeneration); withdraw because it provides a potential substrate for restor-
their dendrites, thus reducing their available post- ing input; this could function analogously with the
synaptic space; or are reinnervated. It is the latter normal pathways that had been destroyed (23).
process that is especially important for recovery,
because it provides the opportunity for integrating
Functional Reorganization in the Injured CNS
previously denervated neurons into novel functional
circuits. Cortical structures exhibit especially dramatic examples
Reinnervation is usually accomplished by a local of what is typically called functional reorganization. The
growth process now called reactive synaptogenesis. best examples of these involve reorganization in sensory
Reinnervation is accomplished by the local prolif- and motor cortices, which display a precise topographic
eration of synaptic terminals in the denervated area, organization that facilitates the detection of injury-
which then establish new synaptic contacts with induced or training-induced changes. In addition to the
nearby denervated neurons. This concept was estab- well-studied examples of reorganization in the visual cor-
RECENT ADVANCES IN NEURAL REGENERATION 789
tex (24), somatosensory and motor cortices exhibit an In the case of injuries to the cortex itself, reorgani-
extensive reorganization that dramatically alters their zation probably involves the same type of lesion-induced
function. growth described, in which denervated neurons are rein-
Following loss or disconnection from a body part, nervated by nearby afferent systems. Following a unilat-
regions of the somatosensory or motor cortex controlling eral lesion of the CST, the motor cortex develops a bilat-
that body part are invaded by adjacent cortical territories eral somatotopic projection to the red nucleus; in the
that control intact body parts. Thus, cortical territories normal animals, the motor cortex projects primarily to
controlling intact body parts expand into unused cortical the ipsilateral red nucleus (39). This is another example
territory. This phenomenon has most convincingly been of reinnervation by the contralateral homolog of a dam-
demonstrated following limb amputation in adult mon- aged structure after a unilateral lesion.
keys (25,26). In monkeys with long-standing therapeutic The ability of spinal cord circuitry to reorganize
amputation of a forelimb, electrophysiologic stimulation after injury became widely accepted after the identifica-
of the cortical region normally associated with movement tion and characterization of central pattern generators
of the amputated forelimb resulted in movement of the (CPGs), which have a remarkable ability to adapt to a
orofacial, shoulder, and trunk regions. Likewise, in mon- changing environment. Historically, CPGs were most con-
keys with long-standing therapeutic amputation of a vincingly demonstrated by Lundberg and colleagues, who
hindlimb, stimulation of the cortical region normally asso- completely isolated the spinal cords of adult cats by sev-
ciated with movement of the amputated hindlimb resulted ering the link to the brain and paralyzing all muscles (40).
in movement of the trunk, hip stump, and tail. Reorgani- These researchers then demonstrated the existence of
zation of the primary motor cortex has also been demon- CPGs in this preparation by administering DOPA, which
strated following ischemic infarcts that led to interruption initiated alternating patterns of activity from motor neu-
of CNS motor tracts in adult squirrel monkeys (27). rons controlling flexion and motor neurons controlling
In humans, functional magnetic resonance imaging extension of leg muscles. The ability of CPGs to reorga-
(MRI), transcranial magnetic stimulation, and positron nize following injury was demonstrated by placing cats
emission tomography (PET) have convincingly demon- with a complete cervical spinal cord transection on a
strated a similar reorganization of the motor cortex after treadmill. During the first week of training, spinal ani-
SCI (2830); limb amputation (31); and ischemic nerve mals were incapable of supporting their hindquarters and
damage (32,33). could not initiate rhythmic movement. However, with one
Reorganization of subcortical regions in the brain to two weeks of training, spinal cats displayed partial
has been most often observed in studies of the red nucleus, bodyweight support and occasional plantar placement of
concerning the control of locomotion. Lesion-induced their paws on the treadmill. By 1 month after injury,
reorganization in the red nucleus can be elicited by injury spinal cats demonstrated improved bodyweight support
to the corticospinal tract (CST), perhaps because of the and stance length. Most impressively, these animals
fact that these two motor pathways cooperate in pro- responded to the movement of the treadmill by initiation
ducing skilled movements. Following unilateral lesion of of locomotion. These studies demonstrated an astonish-
the corticospinal tract (CST), loss of voluntary control ing plasticity of spinal cord circuitry and, most impor-
of arm and foot movements partially recovers over time tantly, indicated that the activity of the spinal pattern gen-
(34,35). Concurrent with this partial behavioral recovery, erator can be modified by training.
the output of the red nucleus to motorneurons control-
ling movement below the lesion reorganizes, so that its
Training Potentiates Functional
control of extensors and flexors is dramatically altered
Reorganization in the Injured CNS
(36). In the normal animal, the rubrospinal system pref-
erentially activates extensor muscles, whereas in the par- One of the greatest advances in the field of regenerative
tially recovered CST-injured animals the rubrospinal sys- plasticity is the recent demonstration that plasticity can
tem almost equally activates extensors and flexors. be dramatically enhanced by training, which may lead to
The anatomic basis of functional reorganization is the functional recovery of movement. This phenomenon
in most cases not known. For example, in the case of the is most convincingly demonstrated following SCI, in
reorganization that occurs in the somatosensory cortex which sensory and motor deficits and improvements are
following peripheral nerve injury, there is some evidence clearly discernable, and has been shown to take place in
that reorganization involves synaptic remodeling at the rats, cats, and humans [for review, see (41)].
level of the subcortical input pathways [dorsal column Spinal cats display an increased efficacy of stepping
nuclei or thalamus, see (37)]. At the same time, however, with as little as 30 minutes per day of step training
there is also evidence for reorganization of intracortical (4244). Spinal animals trained to stand but not step,
circuitry (38). Indeed, both may occur, and contribute to learn to stand but show little or no ability to step. Fur-
cortical remapping. thermore, such training evokes memory. Spinal cats
790 RECENT ADVANCES IN SPINAL CORD RESEARCH
trained to step for 12 weeks, then withdrawn from step AXONAL REGENERATION
training for 12 weeks, have a decreased capacity for step-
ping (44). If these animals are then retrained to step, they We return now to the question of long-tract regeneration,
relearn much faster than during their initial training (44). and the reasons that it does not normally occur after CNS
A similar phenomenon has been observed in rats injury, especially SCI. In this regard, it is again impor-
with chronic complete SCI, using robotic arms attached tant to recall the two key hypotheses: a) that the injured
to the hindlimbs to introduce periodic perturbations in CNS contains factors that inhibit or block regeneration;
the step cycle (45). These rats were allowed to step on a and b) that the absence of regeneration is caused by the
treadmill with their upper bodies supported. After 25 absence of agents that stimulate or allow regenerative
steps, the robotic arms were used to introduce an upward growth.
force during the step cycle to disturb the step cycle, and
this perturbation continued for 25 steps. With repeated
Growth Inhibition
cycles of perturbed and unperturbed stepping, the rats
learned to adapt to the varying stimulus, and generate a The idea that the injury site represented a physical bar-
consistently normal kinematic pattern. rier to regeneration has a long history. One of the oldest
Similar motor learning has been observed in human ideas is that regeneration is impeded by the dense
subjects with neurologically complete mid- to low-thoracic astroglial scar that forms at the sites of injury, and recent
SCI (46,47). Treadmill training has been shown to lead to studies still support the view that the proliferation of reac-
the return of rhythmic locomotor muscle activation pat- tive astrocytes within and around the injury site con-
terns and decreased spasticity. Using patient harnesses that tributes to the establishment of a dense glial scar that can
permit adjustment of the degree of weight support, it was inhibit axonal extension (49). As discussed later, however,
demonstrated that the activation of leg muscles, measured it is now thought that the blockade of regeneration is
by electomyographic amplitude, increases in proportion to caused by the fact that astrocytes express key molecules
the level of the subjects weight bearing (47). Similar results that are growth inhibitory, and not to the fact that the
have been observed in incomplete SCI humans (41). scar is a purely physical barrier.
Patients who undergo training show a gradual increase in Astrocytes are not the only cells that express mole-
their ability to support their body weight, and consistently cules that inhibit axon growth. For example, oligoden-
demonstrate improved locomotion. These improvements drocytes within the region of injury and within myeli-
persist for up to 6 years after discharge from the rehabili- nated tracts beyond the region of injury express several
tation center when activity was maintained in a domestic inhibitory factors that cause collapse of the growth cone
setting (48). and a cessation of axonal elongation (50). Many of these
The neurologic basis of training-induced functional inhibitory molecules are components of the myelin sheath
reorganization remains to be defined. It is reasonable to that oligodendrocytes elaborate.
think, however, that training may induce the same sorts
of changes in circuitry that have been documented fol-
Lack of Growth-Promoting Molecules
lowing defined patterns of activity (LTP, LTD, and related
processes). Also, the patterned activation of neural cir- It is also thought that an important reason for regenera-
cuits that is induced by training may upregulate the tive failure is the lack of appropriate growth factor sup-
expression of key growth and trophic factors, which have port, as well as a lack of substrate support for growing
the potential of improving overall neuronal health and axons. The list of molecules that are capable of promot-
inducing the formation of additional synaptic connec- ing axon growth is impressive. An important issue is to
tions. A challenge for the future will be to test these determine which molecules are required to stimulate the
hypotheses, and develop training regimens that are most regeneration of the axons that are damaged in particular
effective for circuit modifications. situations.
Because regenerative plasticity occurs ubiquitously The fact that regenerative failure is caused by both
and has functional consequences that can be modified by negative and positive factors means that widespread pro-
training, a better understanding of these processes will motion of long-distance axonal regeneration through gli-
lead to improvements in the efficacy of rehabilitative reg- otic or cavitated environments will likely require multi-
imens. Furthermore, a greater understanding of the ple strategies. These will likely involve treatments that
underlying basic scientific mechanisms governing inher- remove inhibitors, together with treatments that replace
ent regenerative plasticity will hasten the development of neurotrophic support and provide appropriate surface
more effective therapeutic interventions, in the same man- molecules that stimulate, or at least support, neurite out-
ner that our current understanding of basic neurobiol- growth.
ogy has culminated in the therapeutic interventions In this section, we review modern studies that
described in the following section. demonstrate that individual or combined treatments are
RECENT ADVANCES IN NEURAL REGENERATION 791
capable of eliciting long-distance axonal regeneration, axons in the spinal cord (6570) and elicit partial func-
which, in some instances, is correlated with functional tional recovery (71,72). Fetal cell transplants into sites
recovery. These regenerative strategies fall into three of adult hemisection injuries have been shown to result in
major categories; transplantation technologies, sometimes limited ingrowth of host serotonergic axons and coeru-
in combination with the engineered expression of neu- lospinal axons from host tissue, and limited reciprocal
rotrophic support; blockade or removal of neurite growth innervation of the host spinal cord by transplanted cells
inhibitors; and genetic and pharmacologic methods of (66,72). The ability of axons to cross the hostgraft inter-
altering the intracellular mechanisms of growth cone face may be enhanced by the ability of fetal grafts to
advance. reduce the glial reaction that normally occurs at the
hostgraft interface (66,68,73). Furthermore, following
fetal transplants to sites of contusion injury in adult cats,
Transplantation Technologies
an improvement in locomotor behavior is accompanied
by synaptogenesis of ascending host projections onto
Fetal Tissue
donor neurons, as determined by neurophysiologic test-
The cell transplantation field has seen its greatest successes ing (68).
in the treatment of Parkinsons disease (PD) and Hunt- It has also been shown that the density and extent
ingtons disease (HD), both of which are characterized by of host axon ingrowth to fetal transplants can be
progressive neural cell loss. The transplant populations enhanced by the administration of neurotrophic factors
used in both of these instances have been fetal cells. In the (74,75). Hemisected animals treated with the combina-
case of PD, cells were derived from the ventral midbrain tion therapy of fetal grafts plus BDNF or NT-3, demon-
at that time during development when nigral dopaminer- strated ingrowth of corticospinal axons in addition to
gic neurons are undergoing terminal differentiation. In the serotonergic and coerulospinal axons, as well as an
case of HD, cells were derived from the striatal pri- increase in the distance and density of ingrowth to the
mordium of the developing forebrain. In vivo analyses graft in all three axonal populations (74). The influence
have demonstrated that the grafted fetal tissue differenti- of neurotrophins on the ingrowth of host axons was not
ated into mature process-bearing neurons and established a general effect of growth factors on the environment per
functional connections that released the appropriate neu- se, as administration of CNTF had no effect on any of the
rotransmitter. Most impressively, this anatomic and bio- descending axonal populations tested. The administra-
chemical repair was accompanied by significant amelio- tion of BDNF in combination with a fetal spinal cord
ration of behavioral deficits, as seen in rodents, monkeys, transplant was shown to promote axonal regrowth in the
and humans (5158). Despite generally promising early adult spinal cord (74), and prevent the atrophy of neu-
results, recent long-term follow-up studies have raised con- rons damaged by the injury (62). Furthermore, combined
cerns about negative side-effects of fetal transplants for the fetal tissue transplantation plus growth factor adminis-
treatment of PD (59), and so the future of this type of tration has been shown to result in the upregulation of
transplantation therapy is uncertain. the inducible transcription factor c-Jun, which is associ-
A long history of research exists on the possible use ated with the regeneration of axotomized CNS axons
of fetal tissue in the treatment of SCI. Transplanted fetal (75). Taken together, these studies provide motivation for
tissue has been shown to survive within the spinal cords the continued evaluation of combination therapies involv-
of both acutely and chronically injured adult animals ing transplants and growth factor treatment.
(60,61). The rationale is that such tissue may provide neu- Despite the encouraging results of fetal cell trans-
rotrophic support, a substrate through which severed plantation studies, the widespread clinical use of fetal cells
axons can regenerate, and perhaps act as a replacement is limited by the availability of donor tissue, concerns
synaptic relay. The idea here is that the axons that were regarding the purity and viability of the transplant pop-
interrupted by the lesion might grow into the graft and ulation, as well as ethical and political issues. Nonethe-
form synapses with neurons in the graft, which would less, the regenerative success of this therapy in both the
then project back into distal segments to form synaptic injured brain and spinal cord has validated the approach
connections with host neurons below the graft [for review of transplant-mediated repair strategies, and hastened the
see (62)]. search for equally effective transplant preparations that
In terms of providing trophic support, fetal tissue do not have the limitations of fetal tissue.
transplants have been shown to prevent the retrograde
death of red nucleus neurons following acute thoracic
Stem Cells
transection of the rubrospinal tract (63), presumably as
a result of their ability to secrete neurotrophic factors The rapidly growing field of stem cell biology offers a
(64). In addition, several experiments have demonstrated promising future for cell replacement and neural regen-
their ability to support the regeneration of severed host eration therapies. Neural stem cells have been identified
792 RECENT ADVANCES IN SPINAL CORD RESEARCH
in both the developing and adult nervous systems of all the hippocampi of other animals, while these same cells
mammalian organisms, including humans. They can also generate olfactory bulb neurons expressing appropriate
be derived from more primitive embryonic stem cells, neurotransmitter phenotypes following transplantation
which offer several advantages over their more mature into the rostral migratory stream, or glial cells following
counterparts, including their capability of indefinite repli- transplantation into regions of the CNS that do not nor-
cation and their flexibility for genetic manipulation. mally generate neurons in the adult (85,86). These stud-
Recently developed methods for stem cell purification and ies demonstrate that the environment of the CNS can crit-
amplification have provided neuroscientists with suffi- ically affect the differentiation potential of transplanted
cient quantities of purified stem cells, which will greatly stem cells.
facilitate in vitro studies of cell survival, control of pro- These influences present formidable challenges to
liferation, control of differentiation, and differentiation cell replacement strategies aimed at promoting stem
potential, as well as in vivo studies of their regenerative cellmediated repair of neurodegenerative disease or
potential. injury, because of the complex and reactive environment
Transplantation experiments have demonstrated of such lesions. Where cell transplantmediated repair of
that the differentiation potential of stem cells is far greater the injured CNS has been demonstrated most successfully
than anticipated. Remarkably, stem cells and even more the transplant environments have been simplistic, such as
mature progenitor populations are capable of generating regions of demyelination (8789). Transplantation of
differentiated cells of a completely different organ or com- multipotential cell populations into adult CNS injury sites
partment than that from which they were isolated. Thus, results in the generation of large numbers of glial cells,
stem cells isolated from the adult forebrain can generate usually astrocytes, as seen in an abundant literature doc-
blood cells (76), hematopoietic stem cells can give rise to umenting stem cell transplantation into animal models of
myocytes (77), muscle progenitor cells can generate blood PD and HD (90,91). In a recent study directed at spinal
cells (78), and marrow stromal cells can give rise to neu- cord regeneration, embryonic stem cells were trans-
rons following in vitro manipulation (79), or astrocytes planted into spinal cord contusion lesions in the adult rat
following transplantation into the brain (80). In a very and shown to differentiate primarily into oligodendro-
exemplary study of this kind, Jonas Frisen and colleagues cytes and astrocytes, with a small amount of neuronal dif-
have recently demonstrated that adult mouse brain neural ferentiation (92). Although this report documented
stem cells transplanted into the amniotic cavity of early- improved locomotor function after early engraftment, no
stage chick embryos integrated into the developing studies were carried out to define exactly what role the
embryo and gave rise to embryonic cells of various fates, transplanted cells may have played in the recovery. A crit-
eventually contributing to the generation of tissues and ical need exists for replication of this study by an inde-
organs of all germ layers (81). pendent laboratory, especially because a clinical trial
Although such transplantation studies have pro- using a similar transplant paradigm has already been
vided remarkable examples of the multipotential capac- launched.
ity of stem cells, they have also highlighted our lack of A promising strategy for circumventing the difficulty
understanding of the control of stem cell differentiation of controlling differentiation following transplantation is
in vivo. The factors affecting the differentiation of trans- to predifferentiate expanded stem cell populations prior
planted stem cells remain largely unknown. Thus, stem to transplantation. The ability to direct the developmen-
cell transplantation strategies must contend with the fact tal progression of stem cells in vitro is currently emerg-
that the differentiation of multipotent cells is strongly ing, as seen in the generation of large numbers of neurons
influenced by the environmental signals and cellular defi- from human bone marrow stromal cells (79), and the gen-
ciencies operating at the site of implantation. Transplan- eration of dopaminergic and serotonergic neuronal pop-
tation of human stem cells into the non-neurogenic envi- ulations from embryonic stem cells (93). These tech-
ronment of the adult rat striatum and substantia nigra nologies will make possible the generation of large
results in differentiation of transplanted stem cells pri- numbers of predifferentiated cell populations for testing
marily into astrocytes (82). Transplantation of adult- in cell replacement and regenerative paradigms, as well
derived stem cells into the neurogenic environment of the as provide neuroscientists with the raw materials to dis-
olfactory bulb results in the generation of large numbers sect the factors affecting progression along a lineage and
of neurons (83). Likewise, transplantation of human mul- the key points of control in lineage commitment.
tipotential progenitor cells into neurogenic regions of the
adult rat brain results in migration along routes normally
Schwann Cells and Olfactory Ensheathing Cells
taken by endogenous neuronal precursors, followed by
site-specific neuronal differentiation (84). Stem cells iso- The growth permissiveness of the peripheral nerve is
lated from the adult hippocampus generate new hip- largely because of the presence of Schwann cells and their
pocampal neurons and glia following transplantation into basal lamina (94,95). Indeed, Schwann cells isolated from
RECENT ADVANCES IN NEURAL REGENERATION 793
peripheral nerve have been shown to promote neurite which was correlated with a marked improvement in
growth in the injured spinal cord and brain (96101). forelimb use (125). A subsequent report demonstrated a
Schwann cells express a variety of cell adhesion mole- significant extension of regenerating axons into regions
cules, including N-CAM, L1, and N-cadherin, and they of the spinal cord distal to the injury following OEC
synthesize extracellular matrix molecules as well as transplantaion (121). Importantly, this study also docu-
trophic factors, including the neurotrophins neural mented the migration of OECs away from the implanta-
growth factor (NGF), BDNF, and NT3 (102,103). These tion site into more distal regions of the spinal cord.
factors provide a favorable substrate, as well as trophic Ramon-Cueto and colleagues reported a remarkable
support, for axon regeneration. Genetically modified degree of axonal regeneration following OEC transplan-
Schwann cells engineered to secrete trophic factors have tation into complete transection injuries in adult rats; this
been shown to enhance their regenerative responses (104). was accompanied by a partial recovery of function (115).
Despite their regenerative properties for injured CNS This group had previously demonstrated the ability of
axons, transplanted Schwann cells do not provide an ade- transplanted OECs to migrate in the CNS and facilitate
quate interface for growing axons to leave the graft and the reinnervation of the dorsal root entry zone following
re-enter host tissue (96,99,101). rhizotomy (126). OECs have also recently been used in
The desire for a more effective glial cell population conjunction with Schwann cell transplantation to
capable of promoting axonal ingrowth to the graft, as enhance regeneration (115). Thus, OECs seem to have the
well as a more growth permissive grafthost interface, unique ability to migrate within the injured and normal
may be met with the discovery of the olfactory ensheath- CNS, integrate with host tissue, and promote the long-
ing cell (OEC). The olfactory system is unique in that it distance regeneration of axotomized tracts that is accom-
supports the regeneration of axons from the olfactory panied by partial functional recovery. Again, there is crit-
epithelium into the CNS throughout the life of the organ- ical need for independent replication of these findings.
ism. The success of the olfactory system in supporting Despite these promising results, substantial barri-
regeneration has been attributed to the OEC. These cells ers prevent the immediate translation of this therapeutic
secrete a number of neurotrophic factors including BDNF, intervention to the clinical setting. Significant gaps exist
NGF, NT-3, and NT-4 (105107), and express a variety in our understanding of the phenotypes of the different
of adhesion molecules including laminin, L1, fibronectins, cell populations that exist within growth-supportive OEC
S100, glial-derived nexin, and NCAM. All of these mol- preparations, as well as their individual contributions to
ecules have been shown to support axonal elongation the regenerative effect of their transplantation. Although
(108115). transplanted OEC preparations are themselves growth
Like transplanted Schwann cells (116,117), OECs supportive, they also modify the behavior of host glial
have the ability to remyelinate axons (118) and enhance cells, and induce rapid and intense vascularization; a basic
action potential conduction (119). Both cell types have understanding of the contribution of these independent
also been shown to share the ability to reduce posttrau- effects on host tissue to the regenerative potential of the
matic cystic cavitation and astrocytic scar formation treatment is currently lacking. Furthermore, procedures
when injected into acute compression injuries. However, for obtaining human OECs by acceptable methods and
OECs differ from Schwann cells in that they have the abil- generating large numbers of cell preparations have yet to
ity to penetrate the surface of the CNS, as seen in their be developed.
normal relationship with axons, wherein they accompany
olfactory nerves all the way from the olfactory nerve fiber
Gene Therapy for SCI
layer to their synaptic terminals in the glomeruli of the
olfactory bulb (120). In addition, following transplanta- One approach that has the potential of allowing the deliv-
tion to the injured CNS, OECs have been observed to ery of missing molecules involves a gene therapeutic strat-
readily migrate from the lesion and intermingle with reac- egy. This takes two forms. In one approach, genes are
tive host glia (115,121). In vitro, OECs have been shown introduced into cells ex vivo, and the genetically modified
to support the increased survival of cocultured primary cells are then introduced into the host. In the other
neurons (111), and enhance the neurite extension of var- approach, vectors of various types (usually viruses) are
ious neuronal populations (122124). used to introduce novel genes into cells in vivo by trans-
Recent in vivo studies have reported a remarkable fection. Of these, the ex-vivo approach is furthest along,
axonal regeneration and functional recovery following and the greatest progress has involved the use of geneti-
the transplantation of OECs into transection injuries of cally modified fibroblasts.
the spinal cord. Following transection of the corticospinal
tract in adult rats, Raisman and colleagues demonstrated FIBROBLASTS AS A GENE THERAPY VEHICLE. The
that suspensions of OECs injected into the lesion site pro- transplantation of fibroblasts that have been genetically
moted the long-distance regeneration of severed axons, engineered to express particular molecules of interest has
794 RECENT ADVANCES IN SPINAL CORD RESEARCH
been shown to promote cell survival, neuronal growth towards the highest concentration of NGF, and
innervation, axonal regeneration, and remyelination suggests that neurotrophin expression may be required at
(127129). Autologous fibroblasts are readily attainable, sites distant from the injury.
and can be transplanted without provocation of adverse Axonal regeneration is also elicited following trans-
immunologic reactions in the host. This strategy offers plantation of NT-3 secreting fibroblasts into a similar
the ability to select cell populations with the maximal mid-thoracic spinal cord hemisection injury to adult rats
expression of a gene product of interest prior to (137). Recipients of NT-3 secreting grafts showed signif-
transplantation, to ensure that all cells within the icant regeneration of corticospinal projections, as well as
transplant population express the transgene at the time a significant amelioration of locomotor deficit. Unlike the
of grafting, and to apply the gene product to specific NGF-recipients, regenerating axons did not penetrate the
regions of the CNS, using stereologic implantation. graft, but grew along the spared ventral bridge of gray
Regenerative strategies using fibroblasts have most matter, penetrating up to 8 mm caudal to the injury site.
often involved genetically engineered overexpression of Axonal regeneration well below an injury site was also
neurotrophic factors. Neurotrophic factors are central to noted following transplantation of BDNF-secreting
several biologic processes, including cell survival, synap- fibroblasts into cervical partial hemisection injuries in
togenesis, dendritic remodeling, neurotransmitter release, adult rats. BDNF expression in these animals promoted
and axonal elongation (130,131). Impressively, recent long-distance regrowth of rubrospinal axons, which was
studies indicate that the expression of neurotrophic fac- accompanied by partial behavioral recovery. Long-dis-
tors by engineered fibroblasts can be regulated after graft- tance regeneration of rubrospinal axons was noted within
ing (132). Fibroblasts transfected with a retrovirus con- both gray and white matter below the injury site, an inter-
taining a tetracycline responsive promoter for the esting finding in light of the growth inhibitory proper-
expression of the neurotrophin nerve growth factor ties of CNS myelin. The mechanism by which exposure
(NGF) can be regulated in their expression of neuro- to neurotrophins may block inhibition of axonal regen-
trophin by the addition or removal of the tetracycline eration by myelin-associated proteins is discussed in the
modulator doxycycline in the animals drinking water. following sections.
This study indicated that expression could be turned on These studies motivate the continued evaluation of
and off for up to 3 months after grafting, the longest time genetically engineered fibroblasts as vehicles for neu-
examined. rotrophic factor delivery. The data also indicate that dis-
NGF-secreting fibroblasts have been shown to alter tinct classes of axons possess differing affinities to simi-
cholinergic innervation following implantation into pri- lar extracellular environments, a feature that will be
mate brains (129). Primate and rodent aging is associated important to keep in mind as future combined therapeu-
with a significant reduction of cortical cholinergic inner- tic strategies are designed.
vation (133135). The implantation of NGF-secreting
fibroblasts into the basal forebrain reversed age-related
Blockade or Removal of
degeneration of cortical cholinergic innervation, to a level
Neurite Growth Inhibitors
that was equivalent to or greater than that seen in intact
young monkeys. Interestingly, the effect of NGF delivery As noted, the two principal hypotheses pertain to growth-
was exerted at a distance, because the growth factor was inhibiting molecules. One focuses on myelin; the other on
presented to the cholinergic cell bodies, yet influenced ter- inhibitory molecules expressed by reactive astrocytes.
minal axon density in the distant cortex.
NGF-secreting fibroblasts have also been shown to
Growth Inhibition by Myelin
elicit axonal elongation following transplantation into
sites of mid-thoracic spinal cord hemisection injury in The idea that myelin is inhibitory came initially from a
adult rats (136). Primary nociceptive (pain-mediating) collection of observations in both the developing and
fibers densely penetrated the NGF-secreting graft by 2 adult systems. For example, a number of studies in devel-
weeks post injury, and coerulospinal fibers extensively oping systems reveal that myelination does not begin until
penetrated the graft by 3 to 6 months after implantation. axon growth is fairly complete. In the developing ham-
The demonstration that coerulospinal fibers penetrated ster visual system, myelination of the optic tract and supe-
the graft indicates that brainstemspinal projections are rior colliculus begins only after the retinotectal axons
capable of robust growth after injury. Nonetheless, these have innervated the superior colliculus (138). In the devel-
animals did not show evidence of locomotor recovery. oping chick spinal cord, axonal projections from the
Although these axonal populations penetrated the graft brainstem to even the most caudal targets within the lum-
robustly, they did not display growth and elongation out- bosacral spinal cord are complete by embryonic day 11,
side of the grafts. This finding is interesting in light of whereas myelination of the spinal cord begins on embry-
the chemotropic properties of NGF; that is, NGF elicits onic day 13 (139). Furthermore, the ability of the devel-
RECENT ADVANCES IN NEURAL REGENERATION 795
oping chick spinal cord to functionally recovery from SCI structure (140,156158). This immunologic treatment
is lost at a critical point in development that corresponds involves the administration of serum complement in com-
to the onset of myelination in the spinal cord (140). Thus, bination with antibodies to the major myelin sphingolipid
the transition from a permissive to a restrictive period galactocerebroside (anti-GalC), which bind to myelin
for CNS repair after injury correlates with the develop- membranes and activate the complement. In vitro stud-
mental onset of myelination. ies suggest that the mechanism of anti-GalC-induced
Another line of circumstantial evidence came from demyelination involves the microtubule disassembly of
studies in the mature nervous system. For example, oligodendrocyte processes mediated by an influx of extra-
axonal sprouting in the adult CNS is inversely correlated cellular calcium (159). Intraspinal injection of this com-
with the degree of myelination (141). Sprouting occurs position results in a well-defined region of complete
more readily in lightly myelinated regions of the CNS, demyelination that can be precisely controlled with
including the hippocampus (15); the septum (142); the regards to size and positioning. Myelin debris is quickly
cerebellum (143); the olfactory system (144); and the sub- cleared from the region of demyelination, and axonal
stantia gelatinosa of the spinal cord (145). damage does not occur. Remyelination begins approxi-
Direct evidence that components of myelin are mately 14 days after the immunologic protocol is dis-
inhibitory to axon growth came from biochemical stud- continued, and is carried out by endogenous oligoden-
ies. Initial biochemical investigations revealed that CNS drocyte progenitors in the adult white matter (160,161),
myelin contains several proteins that inhibit neurite out- despite the fact that mature oligodendrocytes survive
growth. Later studies further defined the nature of these within the region of demyelination (160).
molecules, revealing the inhibitory nature of Nogo-A Immunologic demyelination was first developed in
(146); myelin-associated glycoprotein [MAG (147)]; chick embryos, where it was used to delay the develop-
chondroitin sulfate proteoglycan [CSPG (148)]; brevican mental onset of myelination (140). As discussed earlier,
(148); and the brain-specific versican V2 splice variant the ability of the developing chick spinal cord to func-
(148). tionally recovery from SCI is lost at a critical point in
These basic findings motivated attempts to develop development that corresponds to the onset of myelination
function-blocking antibodies against the various in the spinal cord (140). Delaying the developmental
inhibitory molecules; for example, an antibody that binds onset of myelination with immunologic demyelination
to two of the several myelin proteins associated with neu- extended the permissive period for axonal regeneration
rite growth inhibition, termed NI35, and the antigenically and functional recovery to later stages of development.
related NI250, also called Nogo-A (149). Using partial Immunologic demyelination was then applied to adult
protein sequence data derived from a proteolytic digest birds, which resulted in transient disruption of compact
of bovine Nogo-A, three different laboratories were able myelin; this procedure facilitated axonal regeneration of
to identify the cDNA for this major inhibitory protein, 6 to 19 percent of axotomized avian brainstemspinal
called Nogo (150152). Three different transcripts orig- neurons, and physiologic recovery (156).
inate from the Nogo gene share a common carboxy-ter- More recently, immunologic demyelination has been
minal domain. A portion of this domain has been shown shown to promote axonal regeneration in rodents.
to have an inhibitory activity, and is expressed on the Immunologic demyelination of adult rat spinal cord, pre-
extracellular surface of both Nogo-transfected cells and viously subjected to a partial injury of the dorsal column,
oligodendrocytes (150,153). Animals treated with anti- resulted in robust axonal regeneration at a distance from
bodies against Nogo-A, delivered by implanting the injury site (157,158). In a combination therapy,
hybridoma cells into the brain, exhibited long-distance regions of immunologic demyelination were placed on
axonal regeneration (154) and enhanced functional either side of a contusion site in adult rats, to remove
recovery (155). Functional recovery in this study was myelin-associated inhibitors of growth either side of the
attributed to the regeneration of corticospinal axons, lesion, and to promote the spread of Schwann cells trans-
because ablation of the sensorimotor cortex reversed the planted into the contusion site. This combination therapy
behavioral recovery, although enhanced regrowth of was shown to elicit robust axonal regeneration, as well
raphaespinal and coerulospinal axons was also noted. as a significant functional improvement in overground
Identification of the Nogo receptor and of the intracellu- locomotion (162).
lar pathways that mediate Nogo signaling will undoubt- Another method of addressing the entire myelin
edly lead to new strategies to overcome Nogo-mediated structure, as opposed to particular inhibitory proteins
inhibition. within myelin, is a vaccination approach that is designed
Because myelin contains many proteins that are to coat CNS myelin, presumably rendering its growth-
inhibitory to neurite outgrowth, an alternative strategy to inhibitory components inaccessible to growing neurites
overcome myelin-associated growth inhibition has been (163). Treatment of cerebellar neuronal cultures with
the selective and transient removal of the entire myelin antisera from mice immunized with CNS myelin prepa-
796 RECENT ADVANCES IN SPINAL CORD RESEARCH
rations enhanced the growth of the cerebellar neurons lar signaling may overcome multiple inhibitory influences.
over CNS myelin substrates that normally inhibit neu- Poo and colleagues have demonstrated that the level of
rite outgrowth. In vivo, extensive regenerative growth of cytosolic cyclic nucleotides can modulate the response of
CST fibers was demonstrated in mice immunized with growth cones to a number of factors involved in neurite
CNS myelin. Polyclonal antibodies were presumably gen- attraction and repulsion (167169). Directly addressing
erated against multiple inhibitory components of CNS myelin-associated inhibition, Filbin and colleagues have
myelin. The presence of growth-inhibitory CSPG within recently demonstrated that MAG/myelin-dependent inhi-
CNS myelin raises the possibility that CNS myelin vacci- bition can be overcome by priming neurons with neu-
nation resulted in the generation of antibodies that func- rotrophins, and that this effect was mediated by the
tionally blocked components of both CNS myelin and the cAMP-PKA pathway (170). This study indicated that
glial scar at the injury site. MAG/myelin acted through heterotrimeric G-proteins to
Thus, methods of blocking or removing the growth inhibit the neurotrophic stimulation of the cAMP-PKA
inhibitory effects of CNS myelin have been shown to elicit pathway, and that elevating cAMP and activation of PKA
robust axonal regeneration, and in some instances, par- in neurons or growth cones permitted neurites to over-
tial functional recovery. Because of the presence of come MAG/myelin-dependent inhibition. It is reasonable
myelin-associated growth inhibitors within CNS regions to speculate that multiple inhibitors in the extraneuronal
beyond the region of injury, it is likely that a method of environment can stimulate signaling cascades within
overcoming myelin inhibition will have to be combined growth cones, which then converge at a common down-
with methods to bridge lesion sites in severe injuries, in stream target to control growth cone advance.
order to maximize axonal regeneration. That is, axons A possible target is the Rho family of GTPases,
must be stimulated in such a way that they become insen- which is known to be phosphorylated and inactivated by
sitive to inhibitors of axonal outgrowth. Such strategies PKA and play a role in the dynamics of the actin
are discussed in the following section. cytoskeleton and growth cone advance (171). Inhibition
of Rho blocks CNS-myelin induced growth cone collapse
and inhibition of DRG axonal outgrowth (172). Inhibi-
Altering Intracellular Signaling
tion of Rho has also been shown to promote the growth
Contradicting the notion that CNS myelin is inhibitory of primary retinal neurons and PC12 cells over myelin
to axonal regeneration are a handful of reports that and MAG substrates in vitro, as well as regeneration of
demonstrate extensive axonal growth into white matter retinal ganglion cell axons following optic nerve crush
tracts of the spinal cord. Silver and colleagues have in vivo (173). Thus, the state of intracellular signaling
demonstrated that microtransplanted adult dorsal root proteins or second messengers when an axon is exposed
ganglion neurons extend axons over intact or degener- to an inhibitory influence may dictate its response. The
ated adult CNS myelin for long distances in vivo possibility that intracellular signaling is altered in embry-
(164,165). Berry and colleagues have demonstrated that onic neurons, adult neurons primed by exposure to neu-
if an explant of peripheral nerve was placed in the retina rotrophins, or adult neurons that have been isolated and
after an optic nerve lesion, optic nerve axons grew exten- cultured prior to transplant, may explain the ability of
sively and for long distances over CNS myelin within the these preparations to elongate axons over CNS white
optic nerve (166). And Bregman and colleagues have matter. The identification of common intracellular sub-
demonstrated that when embryonic fetal tissue and strates for different growth inhibitors, and of methods
BDNF were supplied to an injury site in the adult rat of altering them in vivo, may lead to the development of
spinal cord, axons not only grew into the embryonic tis- a means to overcome multiple inhibitory influences with
sue grafts but also extended for long distances through a single treatment.
white matter beyond the lesion (62). These studies sug-
gest that certain manipulations can render growing axons
insensitive to, or able to overcome, the growth-inhibitory CONCLUSION
properties within the adult CNS.
Altering intracellular signaling represents a novel The studies outlined in this chapter indicate that the
strategy in the effort to overcome growth inhibition in the injured adult CNS has a significant capability for regen-
adult CNS. This may be done by pharmacologically or erative plasticity and long-distance regeneration. Perhaps
genetically targeting injured cells. This may represent a more surprising than its ability for anatomic reorganiza-
more accessible target than the multiple contributors to tion is the ability of the injured CNS to function using a
regeneration failure in the injured adult CNS, which man- neuronal configuration that differs so significantly from
ifest at various points after injury. Multiple inhibitory the pre-injured state. It follows that the regenerating CNS
molecules may share common intracellular substrates. is not predisposed to a template circuitry, so long as
Thus, it is possible that a single antagonist of intracellu- functionality can be attained.
RECENT ADVANCES IN NEURAL REGENERATION 797
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59 Spinal Cord
Repair Strategies
pinal cord injury (SCI) is one of the Treatment that promotes functional regeneration
regarding growth factors and neurotrophic factors and cellular and molecular programs that lead to regeneration
their receptors in the CNS, allow us to develop a slightly in the injured peripheral neurons after axotomy. How-
more positive view regarding the possibilities of reach- ever, similar cellular programs are either not upregulated,
ing the ultimate goal of repairing the damaged spinal or are upregulated only transiently, after axotomy of CNS
cord. In this chapter, we focus on the unfavorable envi- neurons. Degeneration and retrograde cell death occur
ronment in the injured spinal cord and three strategies in these neurons after axotomy.
to rescue neurons from death. These strategies include the After SCI, the interrupted ascending and descending
replacement of nerve cells after neuronal death, and pathways dramatically alter the activity of the neuron
reconnection and recovery of normal function in the pool. For example, during locomotion of the lower limbs,
neural pathways. the interruption of descending input from brain and brain
stem leads to the loss of initiation, which takes place in the
central pattern generator (an intrinsic circuitry underly-
PATHOLOGIC CHANGES OF ing rhythmic alternating stepping movements within the
SPINAL CORD AFTER INJURY lumbar spinal cord), and results in the consequent paral-
ysis of the lower limbs (613). At the injury site, the tis-
After SCI, neurons and pathways located within the sue undergoes complicated changes and the environment
injury site undergo several changes. First of all, the itself is altered dramatically. For example, the bloodbrain
ascending and descending pathways are disrupted. barrier is disrupted, there is activation of microglia, inva-
Although there is still a chance for these pathways to sion of macrophages, and astrocytes become reactive and
remain undisrupted, they may remain dysfunctional encapsulate the injured area. This secondary injury cas-
because of spinal shock or other energy failure states. cade can lead to increased cell loss at the lesion site, cyst
Also, any functional ascending or descending pathways formation, and cavitation. Even if axons at the injury site
may be characterized by abnormal plasticity. For exam- are spared from direct damage, conduction across the
ple, aberrant conduction, which generates spasticity and injury site is often abolished. Caudal to the lesion, the
neuropathic pain, may be present. Despite this, there still spinal cord circuitry is still present, but the supraspinal
may be a presence of functional ascending or descending input is decreased or abolished and local inhibitory cir-
pathways with normal plasticity. Other changes may cuitry is increased.
include local neuron pool loss caused by necrosis or apop- The role of oligodendrocytes in blocking regenera-
tosis. This can lead to a dysfunctional local neuron pool. tion has been well established in recent years. It is likely
These changes, in turn, contribute to obstacles that these inhibitory influences restrict axonal growth
against regeneration and functional recovery, caused by both at the lesion site and within the host nervous sys-
pathway disruption, dysfunction, and malfunction. Neu- tem distal to the lesion. This inhibitory mechanism is cru-
ronal loss caused by necrosis and apoptosis can also cial for the stabilization of axon sprouting (i.e., preven-
impede regeneration. More importantly, apoptosis of the tion of subsequent collateral branching) after functional
oligodendroglia, a myelin-forming cell, can occur. Other axonal connections in CNS myelination during develop-
problems that prevent recovery may be caused by the ment (14). This is suggested by the temporal correlation
changed synaptic connection in the upper and lower neu- between the developmental onset of myelination and the
rons, which can contribute to defective regenerative completion of primary axonal growth (15,16). Axonal
potential in the CNS neurons. Furthermore, damage to sprouting within the adult CNS has been reported to
the CNS causes a hostile environment that contains free occur more readily in lightly myelinated regions such as
radicals, edema, hemorrhage, ischemia, inflammation, the substantia gelatinosa of the spinal cord (17); the olfac-
disturbed cerebrospinal fluid (CSF) dynamics, reactive tory system (18); the hippocampus (19); the septum (20);
gliosis, cysts, metalloprotein deposition, myelin debris and the cerebellum (21). This indicates that neuronal plas-
deposition, growth inhibitors, and repulsive guidance ticity involving axonal sprouting is inversely related to the
molecules. extent of CNS myelination. Consequently, the corti-
cospinal tract of the spinal cord becomes severely
restricted in its ability to sprout after myelination. The
UNFAVORABLE ASPECTS FOR inhibitory nature of myelin toward axonal regeneration
REGENERATION IN THE INJURED in the CNS has been further explored by studies of
MAMMALIAN SPINAL CORD inhibitory molecules.
Myelin-associated glycoprotein (MAG) is a potent
Both intrinsic neuronal and extrinsic environmental fac- inhibitor of axonal regeneration (22,23). Soluble MAG
tors contribute to the lack of regeneration after trauma in is released from myelin and potently inhibits axonal
the mammalian CNS. In comparison, the peripheral ner- regeneration. A comparison of neurite growth in con-
vous system (PNS) produces an upregulation of specific tact with CNS or PNS constituents has revealed that
SPINAL CORD REPAIR STRATEGIES 803
PNS myelin is a positive substrate for neurite growth, released at the injury site. All of these have trophic effects
whereas CNS myelin has strong inhibitory properties on many of the surrounding glial cells (5052). The sub-
(2426). In Aguayos bridging studies, regenerating sequent proliferation and hypertrophy of astrocytes
fibers could elongate throughout the whole distance of around the injury site is denoted by increased immunore-
a PNS graft. However, the fibers usually fail to enter the activity to glial fibrillary acidic protein (GFAP) (49,53).
spinal cord for any significant distance beyond the dis- The increased number of astrocytes may be caused by cell
tal nervecord interface (27). Pioneering work by migration division (54). The expression of GFAP has been
Schwab and associates has demonstrated that oligo- suggested to be related to neuronglia interactions, and
dendroglial cells produce proteins, such as neurite in particular, induced secondarily to the microglial
growth inhibitors NI-35 and NI-250, components of response (5557).
central myelin, which inhibit axon regeneration Whether astrocytes play a totally inhibitory role, or
(2830). Recently, the neurite inhibitors were isolated in some aspect a supportive role, their use for regeneration
and characterized as Nogo proteins and subgrouped to is still controversial. Some evidence suggests that embry-
A, B, and C. A single Nogo gene has been elucidated onic astrocytes can act as conductive substrates for grow-
after differential splicing and gives rise to these three dif- ing axons, whereas adult astrocytes or reactive astrocytes
ferent proteins. Although there appear to be several are inhibitory to axonal growth (44,55,5860). Astrocytes
interesting ways in which this inhibition might be over- can potentially express molecules that are both growth per-
come (such as using the monclonal antibody IN-1) missive and growth inhibitory. Some molecules can be
(31,32), other alternatives to avoid the nonpermissive growth permissive at one time, but growth inhibitory at
microenviroment of CNS white matter are worth another time. Throughout development, astrocyte-related
exploring. One possibility is to reroute pathways from molecules are present. They include tenascin, phosphacan,
nonpermissive white to permissive grey matter (33). In heparin sulphate proteoglycan (HSPg); chondroitin
the grey matter of the spinal cord, which contains nerve sulphate proteoglycan (CSPG); and dermatan/keratan
cell bodies and many nonmyelinated fibers, less Nogo sulphate proteoglycan (KSPG) (44,6163). These mole-
activity occurs. Long distance 5-HT regeneration in the cules have different expression patterns at various stages.
spinal cord has been demonstrated to occur to over 95 Recent findings suggest that in the adult CNS, tenascin,
percent in grey matter after either chemical (34) or sur- CSPG, and KSPG may be inhibitory, whereas KSPG
gical lesioning (35). Moreover, in using in vitro explant is a supportive substrate for neuronal differentiation
coculture of postnatal sensorimotor cortex and spinal (42,4547,63). Although tenascin is a permissive molecule
cord, the corticospinal fibers were noted to arborize for axonal outgrowth during the early stages of develop-
within the grey matter of spinal cord explants (36). ment, it may have a nonpermissive or repulsive role later
Reimplantation of the severed dorsal roots in the adult (64). A high ratio of HSPG to CSPG expression appears
rat spinal cord has revealed axonal regeneration and ter- during the early stage of spinal cord development, which
minal arborization when the roots were reinserted into is more permissive for axonal growth; however, this is
the grey matter, whereas regeneration was limited when reversed during later stages of development (63). Many
insertion was into the white matter (37). aspects relating to the expression of cell adhesion mole-
Regeneration of damaged axons within the CNS has cules at different developmental stages remain to be clar-
often been suggested to be inhibited by reactive astro- ified. For example, the role of families of glial receptors for
cytes, which activate a physiologic stop pathway that axon adhesion and the actual glial cell adhesion systems
is built into the axon and normally operates when axons that function in the spinal cord are still unknown.
from stable connections interact with target cells (3846). Although it is known that astrocytes can synthesize sev-
Astrocytes not only present a physical barrier to growth, eral growth factors in vitro and/or express mRNA in vivo
but also a chemical barrier; after injury, they also express (6570), it is still unknown whether or not they secrete
a boundary molecule that is able to restrict axonal growth neurotrophic factors in vivo to facilitate regeneration, or
(42,4549). Through their production of proteoglycans act as mediators of neurotrophic factor transport.
within the extracellular matrix, astrocytes are directly
associated with failure of axon regrowth (48). Certain
serum factors and/or inflammatory cytokines may play FUNCTIONAL RECOVERY
a role in the molecular cascade that produces the extra- IN A PATIENT WITH SCI
cellular matrix. The breakdown of the bloodbrain bar-
rier and the infiltration of macrophages at the lesion site The clinical treatment for a CNS lesion has been based
are associated with the deposition of chondroitin sulfate on the principle of decompression and bone stabilization.
proteoglycans close to the so-called glial scar (49). Rapid For the damaged CNS per se, no definite mode of ther-
local inflammation and edema occur after spinal cord apy exists, except general supportive methods. However,
trauma, with a series of cytokines and chemokines being by applying the knowledge of neural regeneration that
804 RECENT ADVANCES IN SPINAL CORD RESEARCH
has developed quickly in the last decade, we are now of neuronal survival, or reactivate neuronal function. In
standing on a new platform looking toward an era of the strategy to replace cells after neuronal death, cellu-
novel CNS repair and functional recovery. The challenges lar transplantation is necessary to restore neural net-
include learning the mechanisms for SCI and repair, devel- works and/or chemical function. Sources for cellular
oping a therapy in this sensitive and multifactorial envi- replacement may be from embryonic tissues, adult
ronment, and clinically documenting it. Most of spinal neural progenitor cells, adult cells transformed by cer-
cord research today is performed on lower animals; tain factors, adult ensheathing cells, and transgenic cells
researchers must first take the leap to higher animals, and with specific function. In an effort to reconnect and
then to humans, facing substantially increased complex- recover normal function in the neural pathway, a repair
ity along the way. strategy using neural grafts together with trophic fac-
Most researchers agree it is unlikely that any single tors has been shown to result in meaningful functional
therapeutic intervention will facilitate complete func- recovery in rodent spinal cord transection models (33).
tional repair, whether it promotes neuron survival, stim- Functional reactivation of the dysfunctional pathways
ulates axonal outgrowth, or transiently blocks inhibitory in the spinal cord was noted after the application of
signals. Furthermore, delicately altering the balance trophic factor. Studies in this strategy cover neu-
between those factors which promote regeneration with rotrophic factor delivery, axon guidance, removal of
those endogenous mechanisms inhibiting adult CNS growth inhibition, bridging and artificial substrates, and
repair may be sufficient to drive recovery in a function- modulation of the immune response. Results from these
ally positive direction. Finally, determining the spatial and studies indicate that functional recovery can be attained
temporal organization of experimental therapies is criti- to some extent in the discontinuous CNS by either
cal to designing an integrated approach to neurotrauma increasing the permissive cues or decreasing the non-
recovery. The rapidly expanding information regarding permissive cues of the existing environment around the
CNS development will continue to provide valuable infor- sprouting nerves. However, many mechanisms may
mation for creating the necessary conditions within the account for observed functional recovery that do not
injured adult CNS that are more conducive to functional include regeneration. Functional recovery may be
repair, perhaps in part by approximating the favorable attained from reactivation of the dysfunctional path-
growth state of initial development. The minimal death ways, increased sprouting or synaptogenesis of the
of the injured neuron from retrograde degeneration will remaining survival pathways, or regeneration and cor-
be helpful in the function recovery. The cell body in the rect path finding from the disrupted pathways (Figure
cerebral cortex (as in the descending corticospinal tract); 59.1). For better functional recovery, these mechanisms
in the brainstem (as in the descending rubrospinal tract); are all important, although some are not authentic
or in the lower spinal cord level (as in the ascending dor- regenerative mechanisms.
sal column) should be prevented from dying back. The
damaged axon must then extend its disrupted processes
to its proper neuronal targets for functional recovery. STRATEGY TO RESCUE
After synaptic contact is made, remyelination of the path- NEURONS FROM DEATH
way and plasticity changes in accordance to the functional
connection will follow. The major goal of neuroprotective therapy is to prevent
According to the above scenario, functional recov- secondary cell death from occurring within hours, days,
ery for a patient with SCI can be classified into three or even weeks following injury. Cell death after SCI is
strategies. Strategy A involves rescuing neurons from caused by different mechanisms. These include:
death. This includes methods to prevent local neuron pool
loss, as well as retrograde and anterograde neuronal Excitotoxicityoverprotection of and stimulation
degeneration above and below the disrupted pathways. by normal physiologic neurochemicals, such as
Strategy B focuses on the replacement of cells after neu- glutamate
ronal death. Strategy C strives to reconnect and recover Cell destructioncaused by release of high levels
normal function in the neural pathway, and involves reac- of free radicals
tivation of dysfunctional pathways, re-establishment of Inflammationintervention by the immune system
normal connections, and regrowth of pathways with sur- Swellingmass effect caused by cytotoxic and vaso-
viving upper and lower neurons. genic edema (71)
In the strategy to rescue neurons from death, neu-
roprotective methods, such as the use of antioxidants, Neuroprotective effects can be provided in several
corticosteroids, trophic factors, and antiangiogenic ways. For example, the application of corticosteroids,
agents, might be helpful to produce individual effects to antioxidative agents, intracellular signaling manipulators,
manipulate intracellular signaling, increase the chance and trophic factors are beneficial.
SPINAL CORD REPAIR STRATEGIES 805
FIGURE 59.1
Corticosteroids Antioxidants
In general, neuroprotection research has progressed much Oxidative stress plays a key role both in primary dam-
further than research focusing on transplantation. How- age following acute traumatic SCI and in secondary dele-
ever, despite all the research being conducted, the only terious processes associated with inflammatory mediators
real advance that has been made is the North American and neutrophil-mediated inflammation (7274). Reactive
Spinal Cord Injury Study (NASCI) on glucocortico- oxygen species (ROS) and reactive nitrogen species (RNS)
steroids. Over ten clinical trials on neuroprotective agents, are believed to contribute to CNS injury through oxida-
not including the NASCI study, failed in last decade. In tive damage to membrane lipids, proteins, and DNA.
1979, Dr. Wise Young discovered, in a cat model, that ROS includes superoxide anions, hydrogen peroxide, and
methylprednisolone in high doses resulted in neurophys- hydroxyl radicals, whereas RNS includes nitric oxide and
iologic recovery. This discovery has led to more than ten peroxinitrite, potent derivatives of nitric oxide (NO) and
years of research and the mobilization of the drug from superoxide.
the laboratory to clinical application. Currently, high Nitric oxide (NO), a radical synthesized from L-
doses of methylprednisolone have become standard neu- arginine by NO synthase (NOS), can be protective or
roprotective therapy for SCI. Methylprednisolone prob- destructive depending on its redox state and cellular
ably acts as a free radical scavenger as well as an anti- source (75,76). Studies suggest that the activities of both
inflammatory agent. Provided that the patient is available neuronal NOS (nNOS) and inducible NOS (iNOS) are
within 24 hours after injury, it is given in an initial IV detrimental to the ischemic brain or spinal cord and that
bolus dose, followed by infusion over 24 to 48 hours. endothelial NOS (eNOS) activity may be protective
During the 1990s, researchers conducted considerable (7678). Furthermore, intraspinal injection of L-NAME
experiments to find a drug that was superior to methyl- induced a dose-dependent loss of neurons in the spinal
prednisolone. Many drugs have been tested, but none has cord, further supporting the protective effect of consti-
so far proven superior to methylprednisolone. Examples tutive NO (79).
include GM 1 ganglioside (Syngen), and 21 aminosterois Superoxide is produced under physiologic condi-
from J7J (Triclozad), both of which were found to be tions as a by-product of mitochondrial respiration; other
less efficacious than glucocorticoids. such by-products are xanthine oxidase, NADPH-
806 RECENT ADVANCES IN SPINAL CORD RESEARCH
oxidase, and cyclooxygenase (80,81). Superoxide dis- ways (102). Dehydroascorbate, the oxidized form of vit-
mutase (SOD) converts superoxide into H2O2; H2O2 is amin C, increases glutathione levels through stimulation
reduced to H2O by catalase and selenium-dependent glu- of the pentose phosphate pathway (103). A carboxy-
tathione peroxidase. A disruption of the balance between fullerent having antioxidant properties, continuously
oxidative damage and antioxidant defense can produce infused, has prolonged the survival of mice having a high-
excess superoxide and H2O2, thereby producing .OH. In expressing strain of ALS by around 8 days, and no effects
cases of familial amyotrophic lateral sclerosis (ALS), a on tissue oxidative stress were demonstrated (104). Sto-
dysfunction of copper/zinc SOD (Cu/Zn-SOD) is badine is an effective scavenger of hydroxyl, peroxyl, and
thought to be a causative agent for death of motoneu- alkoxyl radicals and an effective quencher of a singlet of
rons (82,83). It was demonstrated that superoxide, oxygen. The antioxidant effect of stobadine has been
hydroxyl, and peroxinitrite radicals increase in the CNS demonstrated in spinal cord homogenates (105). The
following traumatic brain injury or SCI (8486). Apop- nitrone-based antioxidant phenyl-N-tert-butylnitrone
tosis in rat motor neurons, induced by the withdrawal (PBN) has been found efficacious in preventing ischemia
of a trophic factor, also involve the combined effects of and reperfusion injury, septic shock, and other trauma.
NO and superoxide (87). An increased level of nitroty- The tripeptide glutathione (GSH) is known to scav-
rosine, a product of peroxinitrite, was recently found enge both inorganic and organic peroxides. In addition,
present in sections of injured spinal cord and ischemic it regenerates vitamin E from the vitamin E radical
brain (85,86). Induction of magnesium-containing SOD (106,107). Astrocytic GSH peroxidase activity is 3.5-
has been reported for a variety of oxidative stress con- fold higher than its neuronal counterpart (108). It sug-
ditions. Increased expression of Mn-SOD could limit the gests that the cytoplasm of astrocytes is better able than
formation of peroxinitrite (88). SOD prevents the seque- neurons to metabolize hydrogen peroxide at the expense
lae of SCI and improves neurologic recovery and infarc- of GSH. In normal cells, the availability of cysteine is a
tion in rabbits following ischemia and reperfusion injury limiting factor in GSH production. By providing this
(89,90). Two synthetic SOD/catalase mimetics, EUK-8 amino acid, N-acetylcysteine (NAC) can substantially
and EUK-134, were recently shown to reduce oxidative increase intracellular GSH levels (109). The promotion
stress and prolong survival in an ALS mouse model (91). of de novo GSH synthesis is critical, because the glu-
Mn-SOD was recently shown to be a major protective tathione (GSSG) that is oxidized after SCI forms glu-
factor in NO-mediated neurotoxicity (92). On the other tathiyl-protein adducts which are no longer available to
hand, several reports showed that constitutive overex- be reduced back to GSH. Evidence supporting this is
pression of Cu/Zn SOD or Mn-SOD in cells and animals reported by Kamencic et al. (110), who state that pro-
causes oxidative damage (9396). When activity of moting GSH synthesis after SCI decreases secondary
Cu/Zn SOD increases and accumulates, its reaction with damage. In the case of SCI, spinal cord neurons can be
transition metals (Fentons reaction) is facilitated. This exposed to high concentrations of arachidonic acid (AA)
may occur when H2O2 comes into contact with the because of the release of a free fatty acid, 4-hydroxy-
reduced form of copper (Cu). nonenal (HNE). HNE is one of the major biologically
Although various experimental treatments have active products of AA peroxidation (111). Malecki
been tried, the only clinically proven treatment now avail- reported that pretreating spinal cord neurons with NAC
able for spinal cord trauma is the administration of or ebselen, a molecule with GSH peroxidaselike activ-
methylprednisolone (MP). MP sodium succinate (MPSS), ity, reduced the cytotoxic effect of HNE. Ebselen shows
a water-soluble derivative of MP, is usually used in ani- protection in clinical stroke and subarachnoid hemor-
mal experiments and in humans. MPSS may improve neu- rhage (112). It can consume hydrogen peroxide and is
rologic outcome through its anti-inflammatory effect regenerated by GSH. GSH peroxidase, thioredoxin
while activating glucocorticoid receptors (97). The appli- reductase, and selenoprotein P have been shown to play
cation of MPSS reduces lipid hydrolysis and peroxidation a potential role in protection against peroxynitrite (113).
in response to experimental SCI (86,98,99); it was further Overexpression of GSH peroxidase has been shown to
revealed that MPSS blocked the pathways for producing increase the resistance of cortical neurons to amyloid -
toxic prostaglandin F2
(PGF2
) and PGF2
-induced peptide-mediated neurotoxicity (114).
hydroxyl radical formation during spinal cord impact. Previous studies indicate that impaired mitochon-
In previous studies, other antioxidants showed a drial function, ROS, and lipid peroxidation occur soon
modest effect. Vitamin E delayed the onset, but did not after SCI, whereas the compensatory activation of cata-
extend the survival of ALS mouse models (100). Recent lase and GSH levels occurs at later times (115). Therefore,
studies with vitamin E indicate that high doses can slow therapeutic strategies aimed at facilitating the actions of
the progression of Alzheimers disease (101). The brain antioxidant enzymes or inhibiting ROS formation and
possesses a very effective system of vitamin E recycling lipid peroxidation in the CNS may prove beneficial in
through ascorbate and ascorbate/GSH-dependent path- treating SCI.
SPINAL CORD REPAIR STRATEGIES 807
is the possibility of neutralizing those neurochemical fac- development, which occurs during the time of initial
tors present in the injured tissue that inhibit regeneration. axonal elongation and before the build-up of inhibitory
The regenerated nerve fibers may migrate across the mechanisms in the spinal cord (144147). After complete
injured area and grow into the spinal cord distal to the transection in the newborn rat or kitten spinal cord,
injury, after the inhibitory environment has been neu- supraspinal projections to the spinal cord caudal to the
tralized. The other benefit of cellular transplantation is to lesion are abolished. Transplantation of fetal spinal cord
remyelinate those axons that have lost their myelin tissue into the lesion can restore some supraspinal pro-
sheaths, and have consequently worsened or lost signal jections and result in improved locomotor function
transmission. (148,149). Fetal spinal cord tissue transplantation in the
Although one therapy might remediate more than lesioned neonatal spinal cord has been shown to increase
one function, a successful strategy toward functional axonal growth and improve both skilled forelimb reach-
recovery after SCI probably needs multiple therapies. Cur- ing and posture, as well as locomotor function (149151).
rently, the most promising research directions in trans- In the adult spinal cord, changes in the regenerative
plantation for SCI include several techniques. One includes potential of the neurons and the cellular and molecular
peripheral nerve transplantation, either in the form of environments limit the capacity of the mature cord to
peripheral nerves, Schwann, or olfactory ensheathing cells. regrow after injury. The amount of axonal growth and
Another technique involves the transplantation or modi- the extent of recovery of function decrease drastically in
fication of the immune environment, either through adult animals after fetal cell transplantation. After SCI,
macrophage transplantation or immunomodulatory sub- the descending axons from the adults supraspinal centers
stances. Other techniques involve the use of antibodies that project into a fetal spinal cord tissue transplant, but ter-
inhibit inhibitory proteins, as well as fetal cell transplan- minate near the hosttransplant border, despite the fetal
tation and stem cell technology. cord environment within the transplant (152153). Very
Fetal cell or stem cell transplantation involves replac- little evidence has shown that nerve fibers emit from the
ing neurons and is discussed first. Additional areas that transplant to the host tissue. Application of an exogenous
have the potential of supporting transplantation therapy trophic factor may increase the regrowth of nerve fibers
include the use of gene therapy for stimulating neurons. in the fetal tissue transplant. Administration of neu-
In most cases of SCI, both neurons and glia are lost. rotrophins (BDNF, neurotrophin-3 [NT-3], and neu-
Nerve cell replacement is very critical in certain SCI rotrophin 4/5 [NT-4/5]) increases the amount of
lesions, especially when the spared systems cannot sup- supraspinal axonal growth into the transplant and the
plant the function of lost cells. For example, the motor extent of branching within the transplant after spinal cord
neuron pools in the cervical or lumbar enlargement are hemisection and fetal spinal cord transplantation (153).
degraded to such a degree that they are inadequate to sup-
port essential lower motor neuronal functions. No mat-
Stem Cell Technology
ter how well the lesion is bridged or reinnervated, the tar-
get motor neurons that project to the muscles are Stem cells hold great promise as a source for introducing
irrevocably lost. new neurons or glial cells to the damaged spinal cord. Stem
cell transplantation involves three stages. First, the stem
cells are purified. Next, they are grown in cell cultures.
Fetal Cell Transplantation
Then, they are transplanted back into the spinal cord. Stem
Recognizing the need for cell replacement, several labo- cells are genetically unmodified cells with a multipotent
ratories have taken advantage of fetal tissue grafts to character that adapts itself to its environment. Thus, after
replace cells in the CNS after a variety of insults (143). transplantation, the graft hopefully takes and the cells
This is perhaps the only transplantation technique that subsequently differentiate into the appropriate neuronal
has already been tested on humans with CNS disorders, and glial subpopulations (154157). Previous studies uti-
as well as patients with Parkinsons disease and lized stem cells that had been isolated and cultured from
syringomyelia. Ideally, a cell replacement source should diverse regions of the developing and adult rodent brain
be homogeneous, readily obtainable, and synergetic. as well as other tissues. Neural cells for replacement may
However, fetal tissue grafting usually requires multiple transdifferentiate from adult non-neuronal tissue, such as
donor sources in a very short period of time for collec- bone marrow stromal cells, using an appropriate growth
tion. Hence, the mechanical, physical, and ethical hurdles factor (158). Stem cells can be propagated and genetically
of using this therapy limit its large-scale use. Neverthe- tagged with markers or therapeutic genes in vitro before
less, knowledge from the studies on fetal cell transplan- grafting in vivo (159161). Several studies have reported
tation provides valuable insight on neural regeneration. that stem-cell grafts can lead to neurogenesis in animal
The capacity of axonal regrowth across spinal cord models of neural degeneration (162). Despite these encour-
transections exists during a small time window early in aging data, however, the functional implication for this
SPINAL CORD REPAIR STRATEGIES 809
approach is still dim. A recent report by McDonald has restrictive period for CNS repair correlates with the devel-
shown transplantation with embryonic stem (ES) cells into opmental onset of myelination in the embryonic chick
the contused spinal cord of a rat (163) with improved loco- spinal cord (178). Similar findings were observed in mam-
motion ability. Whether the improvement was derived mals. Recovery after complete lesions has been shown in
from the surviving ES cells that contributed to the re-estab- neonates before myelination (179).
lishment of neural network, or only from their chemical Researchers have used many different animal mod-
effects, is uncertain. els of SCI. Spinal cords are typically not transected: they
The existence of neural stem cells has recently been are bruised or crushed. Whereas incomplete spinal cord
shown in nonhuman primates and humans (164,165). The lesions have generated valuable information about reac-
adult brain (158) and spinal cord (166) have been shown tive and compensatory changes, lesions such as hemisec-
to contain stem cells that continuously divide throughout tions, weight-dropping contusion models, compressions,
adult life. Neural progenitor cells proliferate and migrate and different chemical or mechanical partial lesions are less
in the spinal cord after trauma or other stress (167169). suitable for the studies of functional recovery through
In an acute demyelination model, progenitor cells are able regeneration of cut axons (31,32,34,180). Many examples
to proliferate and become oligodendrocytes, thus remyli- in previous studies show recovery data that has been dif-
nating the lesioned area (170172). In another model of ficult to interpret because the exact degree of axonal injury
pyramidal cell apoptosis of brain, the progenitor cells are cannot be determined. To avoid all ambiguities of inter-
capable of replacing the dead cortical neurons and extend- pretation and to model the worst possible clinical scenario,
ing their axons through the intact CNS (173). These find- one with complete discontinuity in the lesion area, some
ings suggest that an endogenous self-repair potential, albeit researchers have chosen to study only animals in which a
trivial, exists in the adult spinal cord. However, it is clear complete surgical transection of the spinal cord has been
that endogenous stem cells do not produce complete recov- carried out (33,181). The thoracic cord between T89 has
ery in cases of severe trauma. To determine whether a strat- been frequently chosen as the major transection site in
egy that uses endogenous or exogenous stem cell replace- these studies because the descending pathways to the
ment can be successfully applied to repair the injured spinal hindlimbs are definitely interrupted, the innervation to the
cord, further studies must be conducted. The molecules forelimbs is not disturbed, the rib cage can assist the sta-
and genes that govern cell proliferation, migration, and dif- bility of the spinal column in this condition, and the dis-
ferentiation; the methods of increasing the survival of pro- tance to the lumbar enlargement is long enough to allow
liferated stem cells in the lesioned area; and how these cell retrograde tracing studies. Although animal studies aim
can be functionally integrated into the remaining circuitry at the regeneration and functional recovery under the con-
must be clarified. dition of complete pathway interruption, most SCI patients
have incomplete anatomic interruption. To pursue a com-
prehensive functional recovery for a SCI patient, all mech-
STRATEGY TO RECONNECT AND RECOVER anisms involved in the functional improvement are impor-
NORMAL FUNCTION IN NEURAL PATHWAYS tant, even if some are not authentic regenerative processes.
In terms of functional recovery in with incomplete path-
Reestablishing neural function after SCI requires trans- way interruption SCI patients, neuroprotective measures
plantation techniques to set up a cellular bridge at the and the reactivation of the dysfunctional spinal cord may
point of injury, thus allowing damaged axons to regen- be more or equally important than regeneration of the
erate over the gap created by the injury and reestablish interrupted nerve fibers.
connection of the appropriate nerve circuits. It is clear In the 1980s, Aguayo and colleagues showed that
that an inhibitory environment in the spinal cord must be axotomized spinal cord neurons, brain-stem neurons, and
overcome in some way. Ways now exist to identify those retinal ganglion cells of adult rats were capable of grow-
inhibitors and the methods by which they can be blocked ing axons into the regions of the regenerating axon and
(169). The main problem is for the regenerated nerve its microenvironment (27,182184). In the peripheral ner-
fibers to migrate across the point where they must grow vous system (PNS), Schwann cells are the critical elements
into the spinal cord distal to the injury. that provide several factors beneficial to regeneration
Studies of developing chick embryos have indicated (24,25). Schwann cells undergo a series of changes in
that their regenerating ability after SCI disappears later response to axotomy, which include not only the initiation
in the developmental period. Thoracic cord transection, of myelin breakdown, but also a massive upregulation of
prior to E13, results in relatively complete neuroantomic the synthesis of NGF, BDNF, CNTF, and several pre-
axonal regeneration and functional recovery (174176). sumptive growth-promoting components (N-CAM,
On the other hand, transection after E13 results in sparse tenascin, L1, etc.) (185). Comparing neurite growth in con-
axonal regrowth and little or no functional recovery tact with CNS or PNS constituents has revealed that PNS
(174177). Thus, the transition from a permissive to a myelin is an acceptable substrate for neurite growth,
810 RECENT ADVANCES IN SPINAL CORD RESEARCH
whereas CNS myelin has strong inhibitory properties (26). trolled order and patterns similar to those found in nor-
In Aguayos bridging studies, regenerating fibers could mal rats. Although the repaired animals remain func-
elongate throughout the whole distance of a PNS graft. tionally inferior to normal animals, the data demonstrate
However, the fibers usually failed to enter the spinal cord that it is possible to regain some hindlimb function after
for any significant distance beyond the distal nervecord complete SCI (33, 188). The above research demon-
interface (27). Pioneering work by Schwab and associates strated for the first time that this grafting strategy could
has demonstrated that oligodendroglial cells produce pro- result in limited but definitive functional improvement in
teins, such as Nogo generelated proteins NI-35 and NI- locomotor activity on rats with a transected spinal cord.
250, that inhibit axon regeneration (2830). Although
there appear to be several interesting ways in which this
Predegenerative Nerve Graft
inhibition might be overcome, such as the use of monclonal
antibody IN-1 (31,32), other alternatives to avoid the non- The difficulty of regeneration within the CNS can be
permissive microenviroment of CNS white matter should caused by a lack of growth-promoting substances. The
also be explored. activities of these substances decrease in the mature CNS.
However, for regeneration, it is necessary to have these
substances, which act as natural neurite growth stimula-
White Matter to Gray Matter Rerouting in
tors. In addition to growth-promoting substances, it is
Peripheral Nerve Bridges
necessary to have a sufficient level of trophic factors and
In the grey matter of the spinal cord, which contains an extracellular matrix, such as laminine and collagen, to
nerve cell bodies and many nonmyelinated fibers, less NI support axonal regeneration. Previous studies have
activity occurs. Long-distance 5-HT regeneration in the shown that predegenerated peripheral nerve grafts facil-
spinal cord has been demonstrated to occur in over 95 itate neurite outgrowth from the injured hippocampus
percent of grey matter after either chemical (34) or sur- in a more effective way than freshly taken grafts. This
gical lesioning (35). Moreover, using in vitro explant co- effect was particularly distinct when grafts that were pre-
culture of postnatal sensorimotor cortex and spinal cord, degenerated for 7 days and 35 days were used (189,190).
the corticospinal fibers were noted to arborize within the In the predegenerative nerve, there are no neuronal ele-
gray matter of spinal cord explants (36). Reimplantation ments or myelin. Also, a larger number of Schwann cells
of the severed dorsal roots in the adult rat spinal cord are present, when compared to the fresh nerve. Schwann
revealed axonal regeneration and terminal arborization cells are active and able to produce a range of known
when the roots were reinserted into the gray matter, growth factors such as NGF, BDNF, and CNTF, as well
whereas regeneration was limited when insertion was as unknown growth factors (191193). The role of pre-
into the white matter (37). Based on these findings, degenerative nerve grafts is not restricted to the previously
rerouting pathways from nonpermissive white to per- mentioned uses. The predegenerative nerve may still have
missive gray matter was chosen as a major strategy in valuable substances that may not have been identified.
Chengs repair strategy to evade those oligodendroglial These substances also secrete numerous extracellular
proteins that inhibit axon regeneration (33). The periph- matrix components that can build pathways for regener-
eral nerve bridges thus redirected descending motor path- ating axons and are able to bind adhesive molecules in the
ways from proximal white to distal gray matter, and basement membrane and on the surface of the Schwann
ascending pathways from distal white to proximal gray cells (192,194).
matter, taking into account the specific anatomy of rat Previous studies indicate that nerve grafts that have
descending and ascending pathways (187). Grafting been destroyed by freezethaw treatments contain
intercostal nerve segments to the rat spinal cord was car- Schwann cell basal lamina tubes, which are similar to
ried out after removal of 5 mm of the cord at T8. Para- Schwann cells and are apparently favorable scaffolds
plegic rats that were subjected to the full repair strategy (195). The empty basal lamina tubes are in the shape of
(eighteen white-to-gray bridges, fibrin glue with aFGF, a tunnel. These tunnels can be favorable for axonal
compressive wiring) demonstrated a return of hindlimb growth. The inner surfaces of these tunnels contain adhe-
positioning, followed by return of hindlimb movements sion molecules on which the axons can securely adhere.
and partial weight bearing. None of these changes was Also, the acellularbasal lamina allografts are immuno-
noted in any of several different types of paraplegic con- logically advantageous (196). The infiltration of
trol animals. Structural analysis using anterograde and macrophages after nerve injury, and subsequently in vitro,
retrograde wheat-germ agglutinin horseradish peroxi- as seen in the predegenerated cultures, seems to be a fur-
clase (WGA-HRP) axonal tracing revealed many regen- ther promotor of Schwann cell proliferation, because
erated descending fiber tracts, including the corticospinal macrophages participate in the production of mitogenic
tract. Detailed analysis of gait demonstrated that the factors for Schwann cells (197) and induce NGF expres-
repaired animals were able to use their forelimbs in con- sion through interleukin-1 stimulation (198).
SPINAL CORD REPAIR STRATEGIES 811
Schwann Cell, Olfactory Ensheathing Glia, and less be worthwhile. For example, long-distance regener-
Activated Macrophages Transplantation ation of certain descending tracts (NA and 5-HT) do
indeed occur after chemical lesion, and appear to take
Much work has been done with Schwann cells, which are place in gray matter. Also, many types of CNS neurons
another type of neural support cell. For instance, can be stimulated to extend processes through a free graft
researchers at the Miami Project have developed methods of peripheral nerve. In addition, growth and trophic fac-
to cultivate human Schwann cells and to produce millions tors have potent stimulatory effects. Finally, refined sur-
of cells for transplantation from biopsies of peripheral gical protocols could be implemented, using fibrin glue as
nerves in adults. Besides transplants using Schwann cells as a stabilization component. Furthermore, it is important
the supporting cells, olfactory ensheathing glia (OEG) have to study whether the repair strategy can be applied to ani-
been tested in injury sites to observe regeneration in CNS. mals with chronic SCI. Special additional measures might
OEGs are neurosensory cells of the olfactory epithelium, be necessary to achieve functional effects in a chronic
which are continuously replaced in the adult; their newly paraplegia situation. The final goal is obviously to
formed axons grow into the olfactory bulb. OEG have develop a method worthy of clinical trials.
many growth-promoting properties for neurons and are
able to migrate extensively within the CNS. OEGs could
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60 Retraining the Human
Spinal Cord
GENERAL CONCEPTS: CONTROL OF lie this view and some of the evidence on the underly-
STANDING AND STEPPING ing concepts. Two of these concepts are that the spinal
cord is an important information-processing and deci-
t is well documented that the sion-making center, and its information processing abil-
FIGURE 60.1
A general conceptual model of the control of locomotion and adaptive events that may occur following an incomplete (B) and
a complete (C) disruption of ascending and descending signals to and from the brain compared to the uninjured (A). The bolder
lines imply pathways that are probably at least functionally adapted. In the incomplete model, both supraspinal and spinal com-
ponents probably adapt in ways that can improve function. However, significant adaptations have been shown to occur in the
spinal cord without any input to the spinal cord from the brain.
and they can be depressed depending on the pattern of SENSORY INFORMATION MONITORED AND
activity that predominates. Following an injury in which PROCESSED BY THE SPINAL CORD
little voluntary control of stepping patterns remain, effec-
tive stepping patterns can be generated by the remaining All modes of sensory input, such as from muscle spin-
spinal networks. These networks also seem to be able to dles, Golgi tendon organs, free nerve ending in muscle,
acquire the ability to function more independently of joint receptors, and skin receptors probably provide
supraspinal control than normally occurs with practice of information that can be used to recognize the specific
a given motor task (Figure 60.1B). For example, severe temporal events associated with a step cycle or a given
but incomplete SCI subjects can continue to improve their standing posture. This sensory information seems to be
stepping ability without any improvement in voluntary sufficient for the spinal cord to anticipate what should
control of movement when tested clinically (7,8). Impor- happen next, based on the excitatory and inhibitory
tant changes occur supraspinally in the incompletely events that have immediately preceded a given time
injured individual. It appears that a relatively small point. It seems reasonable to assume that the spinal cord
amount of remaining descending functional input can has evolved so that its neural pathways can readily rec-
become effective, probably as a result of concomitant ognize, for example, when one foot is beginning the
adaptation at the spinal and supraspinal level, and result swing phase and the other is in the early phase of a
in new synergistic actions. Similar changes seem to occur stance. We propose that the spinal cord interprets a
in the spinal cord below the lesion following complete complete or gestalt pattern of afferent input from all
spinal injuries (Figure 60.1C). peripheral sensors in a dynamically appropriate pattern.
Muscle atrophy and a concomitant reduction in In essence, this enables the spinal cord to make context-
force-generating potential also occur following spinal dependent decisions.
injury (9). Interestingly, the amount of atrophy that In interpreting the sensory information associated
occurs differs substantially across SCI subjects, with no with stepping or standing, one can consider the alpha-
obvious explanation apparent for the variability. Some of bet, words, phrases, and sentences analogous to individ-
this variability may be the result of differing degrees of ual sensory receptors, combinations of receptors in a mus-
spasticity, but this has not been studied carefully. Changes cle and among synergists and agonists, and finally, among
in the potential of muscles to generate forces also define muscles controlling all joints of both legs. Different com-
the ability to control movements. For any level of neural binations of sources and modes of receptor information
function remaining, the larger the muscle fibers that are formulate meaningful words, phrases, and sentences for
recruited, the more force will be generated (10). the spinal cord to interpret. In turn, the sequence of motor
RETRAINING THE HUMAN SPINAL CORD 819
FIGURE 60.2
Data from multiple series of steps taken at differing levels of CAN THE SPINAL CORD LEARN?
loading, as regulated by a body weight support system. In gen-
eral, extensor and flexor EMG activity increased with higher
peak limb loading conditions independent of the level of
In adult laboratory animals whose spinal cords were sur-
available supraspinal input. Exemplerary data from a non- gically transected at the low thoracic level, the ability to
disabled (ND) and SCI subjects are shown. Averaged soleus step or stand is a function of whether these motor tasks
(SOL, top), medial gastrocnemius (MG, middle), and tibialis are practiced (2,1417). If adult spinalized cats are trained
anterior (TA, bottom) EMG mean amplitude per burst (V; daily to step with full load bearing on a treadmill over a
rectified, high-pass filtered at 32 Hz) versus limb peak load period of 3 to 12 weeks, their stepping ability improves.
(percent of body weight load, % BWL) from nondisabled If they are trained daily to stand, likewise, their standing
(ND), clinically incomplete (SCIC), and clinically complete ability is improved. The specificity of this training or
(SCIA) subjects. Each point represents the average of SOL experience is illustrated by the fact that the animals
EMG mean amplitudes ( SE) within a 10 percent interval trained to stand will learn to stand, but their stepping abil-
of the BWL range (i.e., 010%, 1020%, etc.) Data from the
ity remains poor. It is not known whether a complete
right limb (open symbols) and left limb (closed symbols) of
each subject are shown separately. Data were not available
spinalized animal can be trained simultaneously to step
for every BWS interval. The regression line was based on indi- and stand over the same period of weeks. Two other fea-
vidual data points from each step cycle and drawn for each tures of learning are present in these trained spinal ani-
muscle when there was a significant relationship (p0.05). mals. If step training is stopped, their ability to step
declines, as if they forget. But if these animals are
trained for the second time after they have forgotten,
IS THE SPINAL CORD SMART? they learn to step much more quickly than when trained
the first time after the spinal injury. This response is
What do we mean when we say the spinal cord is smart? another learning-related phenomenonrelearning a task
We use the term here to emphasize that the spinal cord occurs faster than during the initial training period (for
can process sensory information in the context of the review see references 16,18).
RETRAINING THE HUMAN SPINAL CORD 821
Similar studies have been and are being performed can be disruptive, should be considered in the pharma-
using humans with SCI (4,5,7,8,19). Although the results cologic management of spasticity.
are not absolutely conclusive, a number of results seem Little progress has been made in developing a solu-
to illustrate that the essential learning features for labo- tion to the spasticity function dichotomy, largely because
ratory animals also apply to humans. the etiology of spasticity is so poorly understood. A work-
Following SCI, greater gains in motor function are ing hypothesis is that spontaneous, uncoordinated con-
found by practicing the motor task. Assuming that the tractions following SCI are manifestations of a learned
spinal control of movement can be improved with prac- chronic dysfunction of the sensory-motor pathways of the
tice, it seems that tissue repair approaches that may be on spinal cord. Perhaps, inappropriate activation patterns
the horizon should be used in concert with motor train- might be expected if the spinal cord injured person does
ing. If the spinal cord pathways have not learned to step, not step or stand, which would generate more normal
new axonal growth or the incorporation of new or mod- activation patterns. Conversely, if locomotor-like activa-
ified cells may not become functional if the pathways have tion patterns can be generated by step training by using
not been trained to recognize postural and locomotor cues weight support on a treadmill (BWST), perhaps more
and to generate the motor patterns that correspond to a appropriate activation patterns can be learned (2,16).
given set of sensory inputs. In general, it seems likely that In conjunction with this concept, fewer pharmacologic
greater recovery will occur by combining the interactive interventions may be needed to minimize spasticity if
effects of multiple interventions. appropriate sensory-motor patterns are learned. No care-
fully documented, quantitative data have been obtained
to date to stringently address these possibilities.
PLASTICITY OF INTERACTIONS
BETWEEN THE SPINAL AND SUPRASPINAL
Enhancing Information Processing
CONTROL SYSTEMS
Within the Spinal Cord
In some spinally injured subjects who have some resid- Another approach on the horizon is the administration of
ual descending input, remarkable levels of recovery have adrenergic and serotonergic agonists directly to the lum-
been reported (7,8). A high level of motor control seems bosacral spinal segments to facilitate locomotion. Both of
possible with relatively few descending axons extending these neurotransmitter systems can generate fictive loco-
below the lesion level. As suggested earlier, these obser- motion in laboratory animals. In the normal lumbosacral
vations probably reflect a synergistic effect of adaptive spinal cord, the only effective source of these transmitters
responses in the spinal cord and supraspinal motor con- is from the synapses of axons descending from supra-
trol centers. The ability of motor control centers in the spinal neurons. Following complete spinal transection, lit-
brain to adapt following lesions is dramatic. It appears tle or no norepinephrine (NE) or 5-hydroxytryptamine
that the brain has the ability to reorganize itself so that (5-HT) persists in the spinal cord. However, when chron-
novel pathways can control motion as a result of the ically spinalized animals are given an agonist of these neu-
combined and concomitant adjustments occurring at rotransmitters, locomotion is improved in some, but
supraspinal and spinal levels (20). depressed in others (1). Although the explanation for this
variability is not fully understood, it probably reflects the
highly dynamic features of the responsiveness, often
PHARMACOLOGIC INTERVENTIONS referred to as pharmacologic states, of the spinal cord
FOLLOWING SCI that can change over time.
From a clinical viewpoint, the pharmacologic facil-
Reducing Motor Output itation of stepping may provide little insight into a prac-
tical solution for improving mobility in the individual
Pharmacologic interventions are frequently used to reduce with SCI, because of the challenge of pharmacologically
muscle spasms in SCI subjects. For example, baclofen, a manipulating locomotion with the necessary level of con-
gamma aminobutyric acid (GABA) B receptor blocker, trol. It is neither feasible nor functional to administer
and the alpha-2 agonists, clonidine and tizandine, can 5-HT each time the subject chooses to walk. The facili-
reduce the incidence and magnitude of these involuntary tative effect needed could not be obtained in a timely
contractions. However, one possible negative effect is a manner in everyday living. An acceptable level of consis-
reduction in the sensory information that can be tent pharmacologic control of locomotion has not been
processed by the spinal cord to control posture and loco- achieved even in laboratory animals using any combina-
motion when using these pharmacologic interventions. tion of pharmacologic interventions.
The tradeoffs in maintaining more motor control versus We have little understanding of how NE or 5-HT
less frequent and severe spontaneous contractions, which modulates sensory processing within the spinal cord. If
822 RECENT ADVANCES IN SPINAL CORD RESEARCH
the effectiveness of sensory processing to generate pat- Although only a small proportion of a muscles force
terns of effective motor output could be enhanced, then potential is needed when walking, muscle atrophy can
pharmacologic interventions may be helpful. This appears preclude effective mobility. Indeed, one normally only
to be the case at least when the level of inhibition is lim- activates a small proportion of the motor units within a
ited by blocking glycine receptors with strychnine, [dis- given motor pool to walk at a comfortable speed, but if
inhibiting and thus facilitating (21,22)]. However, if the there is a generalized, 50 percent atrophy of all fibers of
drug itself must induce the rhythmic pattern, then a phar- a muscle, those motor units that are recruited will gener-
macologic approach has less promise. ate half the normal force that would have been generated
Although improvements in locomotion have been without atrophy. This effect alone may be sufficient to
achieved using pharmacologic interventions in laboratory prevent a subject with SCI from executing a motor task,
animals, in virtually all cases there also is evidence of dys- such as stepping or standing.
function. This variation in response may reflect different To walk normally after 50 percent muscle atro-
pharmacologic sites of the spinal cord as well as the crit- phy, more motor units must be recruited to generate the
icality of the dosage administered. In all cases, the side same kinematics of a movement (Figure 60.4A). For
effects of drugs that manipulate the transmitter systems example, a subject with 50 percent muscle atrophy might
can make their use clinically prohibitive. This might be have to recruit 40 percent of his motor units, rather than
expected for 5-HT and NE, given the ubiquitous nature 20 percent to generate the force needed to step (Figure
of their distribution throughout the nervous system. 60.4A). This recruitment of additional motor units in
As noted previously, another potential pharmaco- each of the contributing motor pools also has fatigue
logic strategy for improving motor function is to manage effects, because the higher threshold units are more fati-
the excitability of spinal pathways so that step- and stand- gable (Figure 60.4B). When a greater than normal num-
associated afferent input can excite the appropriate motor ber of units are recruited, as may be needed to compen-
pathways. One such drug that seems to facilitate sensory sate for muscle atrophy, fatigue onset occurs more rapidly
processing is strychnine, a specific blocker of glycine recep- than normal because of the fact that the higher thresh-
tors. Glycine is a major inhibitory neurotransmitter in the old units are inherently more fatigable. The units with the
spinal cord, playing a role in the reciprocal inhibition of higher threshold for excitation have lower levels of meta-
antagonist motor pools via interneurons. Experiments in bolic support via oxidative phosphorylation and have a
the spinal transected cat suggest that modulation of lower potential to sustain metabolic homeostasis than the
inhibitory pathways can be used to improve locomotion. lower threshold units. Thus, in a rehabilitative strategy to
In animals that were trained to stand and could not step, maximize motor performance involving muscles con-
strychnine induced stepping when placed on a treadmill. trolled by neurons distal to the spinal lesion, it will be an
Strychnine seemed to facilitate information processing advantage to minimize the level of muscle atrophy.
rather than directly inducing steplike oscillations. Perhaps How can we prevent muscle atrophy? Some combi-
analogous drugs can be developed so that this approach nation of electrical stimulation, treadmill training, diet,
can be used in humans (21). and the use of anabolic growth factors could contribute
to the preservation of muscle mass following SCI. Only
modest, and in some cases no, gains in muscle mass have
REHABILITATION GOALS IN TREATING been observed with electrical stimulation of muscle. Sub-
SKELETAL MUSCLES jects being trained on a treadmill using a BWS system did
show an increase in muscle mass, as indicated by magnetic
The motor output of any given muscle at any given time resonance imaging (MRI)-derived muscle volume mea-
is largely a function of how many motor units within a surements (unpublished observations) from step training.
motor pool are activated and the total cross sectional area The development of an effective way to avoid or to mini-
of all of the muscle fibers of those units. Within a few mize the level of muscle atrophy in SCI could have a very
weeks after SCI, muscles are likely to have atrophied significant effect on the motor tasks that can be performed.
rapidly. Interestingly, however, the severity of the atrophy It is also clear that muscle phenotypes change fol-
varies among individuals and among muscles within an lowing SCI, with more muscle fibers expressing fast
individual. The reasons for this varied response are poorly myosin phenotypes (see 9 for review) in laboratory ani-
understood. mals and in human subjects. The functional consequences
Muscle atrophy can be a limiting factor in regain- of this are not clear, but theoretically this could make the
ing mobility simply because there is a reduction in force muscles more fatigable and less efficient in generating
output that is proportional to the loss in muscle mass. For force per unit of ATP. Locomotor training and muscle
example, if there is a 50 percent loss in muscle mass and stimulation can reduce the magnitude of the transition
muscle fiber cross-sectional area, there will be a 50 per- of more fast myosin isoforms and less slow myosin
cent loss in the potential force that can be generated. expression.
RETRAINING THE HUMAN SPINAL CORD 823
FIGURE 60.4
REHABILITATIVE STRATEGIES FOR the axial load that must be placed on assistive devices.
AMBULATION A study from Walters and colleagues found that if lower
extremity strength at 1-month post-SCI was 10 or more
Procedures typically used in conventional therapy fol- on the ASIA scale of 0 to 50 in an incomplete paraplegic,
lowing SCI vary depending on the severity and level of 100 percent of subjects could ambulate in the commu-
injury. Following SCI, ASIA A and B patients are gener- nity 1-year post-injury (2426). On the other hand, 100
ally not provided with any form of step training. Tradi- percent of incomplete quadriplegic patients could
tionally the most functionally important skills needed to achieve community ambulation if the score was 20 or
compensate using the unaffected muscle groups take greater.
precedence in therapy. For example, inpatient and out- However, the motor scores derived from standardly
patient therapies for paraplegia may include work on administered voluntary efforts are not reflective of the
trunk support, upper extremity strengthening, transfers, stepping ability when the individuals step using body
and wheelchair mobility. For many ASIA C and D weight support on a treadmill (Figure 60.5). For exam-
patients, those skills may also be important. However, ple, the level of excitation of lower limbs from individu-
trainingneuromotor function below the lesion for ambu- als with SCI during stepping using BWST exceeds that
lation can also accompany these efforts. This may include when they voluntarily attempt to activate the same mus-
resistance exercises for muscle strengthening, selective use cles (20). Furthermore, after the step training, the ambu-
of muscles for isolated flexor and extensor movements latory ability often improves without any change in the
and for truncal control and balance, conditioning exer- lower extremity motor score (7).
cises to lessen muscle fatigability and improve endurance, Locomotor training using manual assistance and
and the management of hypertonicity, which can inter- BWST may have a number of advantages over more con-
fere with standing and walking. For the disabled person ventional approaches for the recovery of walking. The
with adequate upper extremity strength, it is conventional BWST system allows step training to proceed without any
for step retraining to begin with practice in arising from dependence on upper extremity loading on the parallel
the seated position to standing in the parallel bars . bars or an assistive device (Figure 60.5). No orthotics are
Devices that can reduce the weightbearing and facilitate needed. The harness assists maintenance of an upright
reciprocal stepping can be helpful. As gait training con- trunk, and the early practice of standing and stepping
tinues over a period of weeks, overground ambulation appears to activate the trunk musculature to rapidly enable
and the level of weightbearing may improve. independence in holding an upright posture. The level of
Historically, patients who achieve overground lower extremity unloading permits step training when sub-
ambulation usually can generate at least 3/5 strength at jects offer less than 3/5 strength at the knee extensors and
the hip flexors and knee extensors. However, when using hip flexors. Repetitive practice of the segmental sensory-
traditional rehabilitative approaches to SCI, the most motor pathways generated during the normal gait cycle
critical components for the ability to ambulate are not seems to improve the probability of generating more effec-
always reflected in the lower extremity motor score (30). tive stepping over a period of weeks (Figure 60.6). Based
The greater the strength in the lower extremities, the less on numerous EMG recordings as shown in Figure 60.6, it
824 RECENT ADVANCES IN SPINAL CORD RESEARCH
FIGURE 60.6
Acknowledgments
We are grateful for the support of the National Institute of Neu-
rological Disorders and Stroke (NINDS); the National Institute
of Child Health and Development (NICHD); the National Aero-
FIGURE 60.7 nautics and Space Administration (NASA); the Kent Waldrep
National Paralysis Foundation; and the Christopher Reeves
Locomotive capabilities of all chronic patients (n 44) before Paralysis Foundation.
(left histogram) and after (right histogram) Laufband (train-
ing locomotion on a treadmill) therapy. Six functional groups
(05, defined in Wernig et al.) contain wheelchair-bound
(classes 02; black columns) and nonwheelchair-bound
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7. Wernig A, Muller S, Nanassy A, Cagol E. Laufband therapy based
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826 RECENT ADVANCES IN SPINAL CORD RESEARCH
14. de Leon RD, Hodgson JA, Roy RR. Edgerton VR. Locomotor (GAD67) in feline lumbar spinal cord after complete thoracic
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Recruitment of spinal motor pools in humans during voluntary 28. Dobkin B, Apple D, Barbeau H, Saulino M, Fugate L, Scott M.
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Tobin AJ. Increased expression of glutamate decarboxylase
IX
SPECIAL TOPICS IN
SPINAL CORD MEDICINE
This Page Intentionally Left Blank
61 Spinal Cord Injury
and Aging
pinal cord injury (SCI) can change a theories overlap substantially; however, the underlying
SCI, these lower urinary tract changes may occur at ear- Systems show that the incidence of abnormal renal func-
lier ages than typically seen with the aging process, as the tion testing increases with both age and duration of injury,
result of years of trauma to the urethra through catheter- and that removal of urinary tract stones, most common
ization, bladder hypertrophy caused by chronic sphinc- in persons using indwelling catheters, increased from 3.1
terdetrusor dyssynergia, and chronic infections. These percent at 5 years to 10.8 percent at 20 years post-injury.
factors, in addition to aging, may necessitate modifica- The method of bladder management appears to be
tions to a previously effective program of urinary man- associated with certain urinary complications. Typically,
agement. In another example, in people using wheel- studies have documented higher rates of bladder stones,
chairs, the upper extremities are used in a manner that is UTIs, and bladder cancer associated with the use of
unusual, and can, with time, result in shoulder deterio- indwelling catheters. For individuals who are managed
ration and pain. These examples typify one of the main with an indwelling catheter, anticholinergic medication
issues with aging in SCI: that is, the actual wear and tear used routinely may improve health outcomes (11). Indi-
on an aging body structure may result in significant func- viduals using long-term intermittent catheterization may
tional consequences for the person with SCI. be at an increased risk for developing urethral stricture
In the following sections, many of the aging-related and epididymitis (12).
changes experienced by people with SCI are described. Cancer of the bladder appears to be one of the few
The extent to which these changes have an impact on neoplasms for which the incidence is unequivocally
both physical and psychosocial function and health is increased by the presence of SCI (1316). Risk factors for
also discussed. the development of bladder cancer that are related to SCI
include recurrent UTI as well as the use of indwelling
catheters (17,18). It appears that malignant degeneration
THE GENITOURINARY SYSTEM requires the cumulative effects of exposure to various risk
factors (such as recurrent infections, indwelling catheter
Diminished bladder capacity and urethral compliance, an management, urinary tract stones, cigarette smoking, etc.)
increase in uninhibited detrusor contractions and resid- over a long period of time. A recent study showed that
ual bladder volumes, as well as a gradual decline in kid- indwelling catheter management resulted in a four-fold
ney function are some of the findings associated with nor- higher risk for the development of bladder cancer than
mal human aging in the general population (7,8). In nonindwelling catheter methods of management (17).
addition, age-related changes in the diurnal output of People with SCI who develop bladder carcinoma typically
urine result in an increase in nocturia. Another finding is present with hematuria. Unfortunately, hematuria is not
that elderly individuals appear to be at increased risk for a reliable indicator of bladder cancer in people with SCI,
urinary tract infections (UTIs), presumably related to the because it also commonly occurs with UTI, bladder
decline in immune function, postmenopausal changes, stones, and catheter changes.
and the effects of prostatism (9). These tumors are commonly metastatic and invasive
Following SCI, the physiologic disruption of geni- at the time of diagnosis in people with SCI, highlighting
tourinary function is characterized by the loss of voli- the importance of developing effective screening methods.
tional control over micturition as well as the loss of coor- Urine cytology and biochemical markers of urinary tract
dination of detrusor and sphincter reflexes. Once these malignancy do not appear to be appropriate screening
reflexes have recovered, there is a tendency to sphinc- tools at present, because of their high false-positive rate,
terdetrusor dyssynergia and elevated lower urinary tract which can be caused by concomitant UTI and related
pressure over time. This can lead, ultimately, to hyper- hematuria. Although the effectiveness of screening cys-
trophy of the detrusor muscle and decreased bladder com- toscopy to detect these tumors in chronically catheterized
pliance. The cumulative effect of these changes can also spinal cord individuals has been questioned by some,
result in the development of hydronephrosis and upper most clinicians feel that this method remains the best
tract deterioration (9). option for early detection of bladder cancer in persons
Although these SCI-related alterations in urinary with SCI (18,19). In addition, because of the risk of
tract physiology pose significant risks to health, urinary chronic prostatitis related to recurrent UTI, it is reason-
tract complications have been declining as a cause of able to speculate that there may be some added risk of
death in SCI over the past 25 years, now accounting for prostate cancer in males with chronic SCI, although there
only 2.3 percent of deaths (10). The improved urinary is no evidence to date that such an association exists. In
tractrelated mortality is likely due to clinical advances fact, one recent study found a lower incidence of carci-
in urologic management and modern antibiotic treat- noma of the prostate in those spinal cord injured indi-
ment. It is important, however, to note that urologic com- viduals who were more disabled, suggesting that, at the
plications continue to be common among people living very least, there is no added risk of this cancer associated
with SCI. Data from the U.S. Spinal Cord Injury Model with SCI (20). Nonetheless, males aging with SCI should
SPINAL CORD INJURY AND AGING 831
be considered at risk and be provided with the age-spe- an individualized bowel evacuation regimen that incor-
cific prostate cancer screening that is recommended for porates a variety of reflex stimulation maneuvers, laxa-
their general population counterparts (21). tives, and dietary interventions. It is believed that con-
Guided by an awareness of these issues, the long-term stipation manifested by difficulties in producing reflex
follow-up for people with SCI should include attention to bowel evacuation is commonly the result of the anorec-
the potential for functional deterioration and the devel- tal dyssynergia or inadequate rectal expulsive force
opment of urinary tract cancers. The clinical approach is caused by SCI gut motility impairment (27). The primary
based on prevention and early detection. The person with treatment approach is based on an assessment of the
SCI should be educated regarding the fundamentals of existing bowel routine, with suggestions for alterations
bladder management in an effort to reduce the risk of based on common sense. Many people with SCI have
recurrent UTI. This includes education regarding adequate chosen excessively long intervals between bowel pro-
hydration, hygienic bladder management technique, and grams for their convenience. They should be encouraged
regular urologic follow-up. Individuals who elect to use to maintain a bowel program frequency of daily or every
indwelling catheter methods of bladder management other day. The use of laxatives or enemas should be
should receive adequate iinformation regarding the accom- avoided or kept to a minimum. Suppository use is con-
panying risk of bladder cancer. Cigarette smoking has also sidered supplemental to digital stimulation and evacua-
been identified as a significant risk factor for bladder can- tion when necessary. For some individuals with refrac-
cer, therefore, smoking cessation programs should also be tory bowel dysfunction characterized by excessively long
encouraged for those who smoke. bowel programs or frequent fecal incontinence, opting
for an elective colostomy may significantly improve qual-
ity of life (30,31).
THE GASTROINTESTINAL SYSTEM Hemorrhoids and periodic rectal bleeding are also
common accompaniments to chronic SCI, with a major-
Several studies have described the consequences of aging ity of individuals reporting these conditions (32). For
on gastrointestinal physiology in the general population minor symptomatic lesions, topical therapy may be suf-
(2224), showing that a generalized decline in gut motil- ficient. For more severe hemorrhoids, banding is com-
ity and diminished acid secretion accompanies the aging monly required. In the most severe refractory cases pre-
process. Acid secretion in the stomach is also diminished senting with abundant hemorrhoid tissue and recurrent
with increasing years, and the stomach exhibits diminished significant bleeding, operative hemorrhoidectomy may be
emptying of fluid meals but relatively unimpaired empty- necessary.
ing of solid food. The small bowel shows little, if any, spe- No evidence exists to date to suggest that persons
cific change related to aging; however, the colon and rec- with SCI are at added risk of colon cancer. However, it is
tum exhibit diminished motility and an increase in safe to assume that this population is at risk equal to the
diverticular disease (25). Surveys of people with SCI have general population for this common cancer. For that rea-
documented a variety of gastrointestinal complications and son, periodic SCI follow-up should include screening for
functional changes that accompany the aging process. colorectal cancer (33). Fecal occult blood may not be a
In the gastrointestinal system, colorectal function is reliable screening tool because of the frequent presence of
significantly altered by SCI and would be expected to be hemorrhoids, rectal prolapse, and other distal rectal
a substantial source of problems in the person aging with pathology in the SCI population. Therefore, people with
SCI. It is known that colonic transit time is prolonged in SCI in the at-risk age group should be endoscopically
persons with spinal cord injury, especially in the left colon screened periodically. The endoscopist should be familiar
and rectum (26,27). This finding correlates with the com- with the risk of autonomic dysreflexia and be prepared
mon report of constipation in this population. Specifically, to treat blood pressure elevation or other sequelae during
a British study of persons more than 20 years post-injury the procedure.
showed that 42 percent of the subjects had difficulties with There has been speculation about an increased inci-
constipation, whereas 27 percent reported problems with dence of gastroesophageal reflux disease (GERD) in peo-
fecal incontinence, and 35 percent had gastrointestinal ple with SCI. However, a recent report showed no sig-
pain (28,29). Those with tetraplegia were more likely to nificant difference in the overall incidence of reflux
report fecal incontinence, whereas constipation was more disease but a higher prevalence of more severe esophagi-
likely to be reported by people with paraplegia and those tis in the SCI subjects (34). It has been suggested that
who used digital stimulation, manual evacuation, or val- changes in gastric motility may be implicated in chronic
salva for their bowel routines. abdominal distention in long-term SCI, but the underly-
The most profound alteration in gastrointestinal ing cause of gastric dilatation remains poorly understood
physiology resulting from SCI is the loss of volitional con- (33). Recent evidence suggests that gallstone disease is
trol over bowel emptying. This requires the adoption of approximately seven times more prevalent in the SCI pop-
832 SPECIAL TOPICS IN SPINAL CORD MEDICINE
ulation than in the general population, having been found Chronic open skin sores of long duration have been
at autopsy in 29 percent of a group of spinal cord injured associated with the development of Marjolins ulcer, and
subjects compared to a 4 percent incidence in nondisabled the development of squamous carcinoma in the sore (39).
matched controls (35). It does not appear that the for- Because of the high frequency of skin sore occurrence in
mation of gallstones in SCI is specifically related to aging, the chronic SCI population, periodic assessment should
as the added risk of gallstone disease appears to be include a thorough evaluation of the integument and a
restricted to individuals with lesions above T10, with the reinforcement of skin sore prevention education. The clin-
increased incidence of stones generally occurring within ical approach to managing skin sores in SCI is primarily
the first year post-injury. Nonetheless, clinicians should prevention through patient education. This involves
be aware of the increased incidence of this condition with instruction on skin protection, pressure relief, hygiene,
age in general when evaluating abdominal complaints in and routine surveillance. If initiated promptly and per-
the long-term follow-up population. In addition, although formed diligently, conservative treatment of pressure sores
research has demonstrated an increased risk for the devel- is commonly effective.
opment of gallstones in people with SCI, the risk of bil-
iary complications was not of sufficient magnitude in this
group to warrant prophylactic cholecystectomy (36). THE NERVOUS SYSTEM
Because of the high frequency of gastrointestinal
problems in the aging SCI population, specific attention Changes related to the nervous system in the aging gen-
to bowel symptoms should be incorporated into routine eral population have been reported to include loss of
follow-up procedures. Regular assessment of the bowel vibratory sensation, muscle mass, and strength; slower
program should be done, and ongoing education regard- reaction time, decreased fine coordination and agility,
ing bowel program performance, ways to manage and/or decreased deep tendon reflexes, and deteriorating stabil-
modify the bowel routine, and a bowel-friendly diet ity in station and gait (40). The aging spinal cord histo-
should be emphasized. logically shows loss of myelinated tracts as well as a loss
of anterior horn cells. However, significant changes may
not occur until after the fifth decade (41). A study of per-
THE INTEGUMENT sons aging with SCI of more than 20 years duration
showed that 12 percent reported some sensory loss, and
Multiple factors result in the aging of human skin. Normal more than one in five individuals reported increasing
aging results in atrophy and changes in the histologic struc- motor deficits over the years (42). Without further study,
tures that comprise the dermis (37). Elasticity and vascu- it is not possible to state with certainty that an age-related
larity of the dermis, and collagen content are diminished, loss of myelinated tracts and dropout of anterior horn
predisposing aging skin to injury. Flattening of the der- cells may contribute to these reported symptoms.
malepidermal junction and thinning of the epidermis result In studies of people with long-term SCI, a high inci-
in a decreased tolerance to shear and a greater likelihood dence of upper extremity entrapment neuropathies has
of epidermal detachment and blister formation. Diminished been reported, with up to 63 percent of people with para-
vascularity and sweating also may heighten the risk of ther- plegia showing evidence of these, both on electrodiagnos-
mal injury. People with SCI are known to be at risk for skin tic testing and on symptom surveys (43,44). The most fre-
trauma resulting in pressure ulcers, commonly related to quent site of involvement is the median nerve at the wrist,
immobility, lack of sensory protection, and spasticity. Data but ulnar nerve entrapments at the elbow and wrist are
from the United States Model Spinal Cord Injury Systems also common. Because of repeated hand contact with
showed the incidence of pressure ulcers increasing from 15 wheelchair rims, as well as positioning of the wrist during
percent at 1-year following SCI to nearly 30 percent at 20 transfer activities and pressure relief, individuals with SCI
years post-injury (38). The risk is highest in those with com- are clearly at risk for nerve entrapments in the upper
plete tetraplegia, who demonstrated a 40 percent prevalence extremities. It is suspected that the incidence of significant
of pressure ulcers at the 20-year follow-up. The basic prin- entrapment increases with duration of injury, although this
ciples of pressure relief, debridement, and asepsis are still has not been conclusively proven. Treatment for this con-
the foundation of successful conservative management (39). dition should include assessing the mechanics of mobility
Large and deep skin sores will commonly require myocu- and daily living activities to determine any underlying
taneous flap closure (see Chapter 43). Local infection neces- sources of repetitive trauma. For some individuals, the res-
sitates treatment with appropriate antibiotics, and deep olution of symptoms may necessitate a modification of
wounds should raise the suspicion of contiguous activities. In addition, education regarding techniques to
osteomyelitis. In these instances, a bone biopsy should be conserve and protect wrist function may be beneficial for
performed for the purposes of diagnosis and identification some individuals. Other modalities include wrist splint-
of the causative organisms, to guide antibiotic therapy. ing to reduce repetitive trauma at the extremes of wrist
SPINAL CORD INJURY AND AGING 833
flexion and extension, which are known to contribute to THE MUSCULOSKELETAL SYSTEM
carpal tunnel symptomatology. Although corticosteroid
injection therapy has been tried as a conservative measure In the general population, musculoskeletal system aging
for people with carpal tunnel syndrome, the benefit may is characterized by deterioration of articular cartilage
be only temporary. Although ulnar entrapments at the function. This ultimately leads to degenerative arthritic
wrist may prompt consideration of surgical treatment, SCI changes, both in the spine and in joints of the appendic-
survivors with these neuropathies are usually successfully ular skeleton (48). Because of the unique physical stresses
treated with activity and equipment modification and required in people with SCI during mobility activities, it
rarely require surgical intervention. However, when con- is not surprising that overuse syndromes of the upper
servative measures fail to provide a relief of symptoms in extremities are common in this population.
people with significant entrapments, surgical release of the More than 50 percent of SCI survivors have been
transverse carpal ligament is often recommended. Indi- shown to report upper extremity pain (49,50), with shoul-
viduals undergoing this surgery should anticipate a period der discomfort being the most frequent complaint, fol-
of restricted activity after the surgery, which may tem- lowed by pain at the wrist. Transfer activities, wheelchair
porarily necessitate an increased need for assistance from propulsion, and pressure relief maneuvers most com-
others. Postoperative activity restrictions have been monly produce upper extremity discomfort. Although
reduced with some recent advances in surgical technique, acromioclavicular degenerative changes may be seen on
including the percutaneous endoscopic approach to trans- X-ray, plain radiographs commonly are of limited value
verse carpal ligament section. in assessing shoulder pain in these individuals. In symp-
When individuals with chronic SCI experience neu- tomatic individuals with SCI who report shoulder pain,
rologic deterioration, it is most commonly the result of arthrography and MRI imaging have better diagnostic
progressive posttraumatic cystic myelopathy (45). This yield, commonly showing impingement syndrome and
condition is also referred to as posttraumatic syringo- rotator cuff tears (51,52). Most studies demonstrate that
myelia, and is characterized by the progressive enlarge- the prevalence and severity of upper extremity overuse
ment of a cystic cavity originating at the site of injury and problems are correlated with both age and duration of
extending in either a cephalad or caudal direction in the injury. These problems can be managed conservatively,
spinal cord. More recently, the concept of progressive cys- including a periodic review of daily activities and mobil-
tic myelopathy has been broadened to include progres- ity mechanics, which may result in suggestions for activ-
sive noncystic or myelomalacic myelopathies. These con- ity modification in an effort to avoid pain-causing maneu-
ditions are thought to be a part of a pathophysiologic vers. Individuals with overuse syndrome at the shoulders
continuum. The onset of this neurologic complication may have a muscular imbalance across the glenohumeral
may vary from several months to several decades after joint, with anterior musculature development signifi-
injury, but most commonly occurs within the first 5 to cantly greater than that posterior to the shoulder. Mus-
10 years post-injury. Signs and symptoms of late pro- cular balance across the joint to restore optimal gleno-
gressive neurologic deterioration include losses of sensory humeral geometric relationships may be facilitated with
and/or motor function, increasing spasticity, neurologic an exercise regimen specifically designed to address the
pain, increasing autonomic dysreflexia, increasing sweat- posterior shoulder girdle (53). Surgery for impingement
ing, and the development of a variable, positional or rotator cuff tears has been suggested when conserva-
Horners syndrome. Confirmation of this diagnosis tive measures are unsuccessful. Individuals with SCI who
includes a combination of the typical history and physi- contemplate operative treatment for impingement or rota-
cal findings and magnetic resonance imaging (MRI) of an tor cuff tears should anticipate a prolonged and possibly
abnormality of an enlarging syrinx cavity or myeloma- difficult postoperative rehabilitation period as well as a
lacic spinal cord. Arachnoid scarring that interferes with temporary impact on independence, with additional per-
spinal fluid flow and spinal cord mobility appears to be sonal care assistance commonly required (54,55).
the underlying mechanism of progressive spinal cord Osteoporosis is a common accompaniment to the
pathology. When neurologic deterioration is progressive, aging process, most typically associated with post-
surgical treatment, including untethering of the arachnoid menopausal elderly women but also occurring in aging
scar and, in some cases, the shunting of cyst cavity fluid, men (56). Osteoporosis caused by paralysis and disuse
is warranted (46). All individuals with SCI have the is commonly felt to be the underlying risk factor for
potential for late neurologic change; therefore, the assess- pathologic fractures following SCI. Lower extremity
ment of motor and sensory function, as well as a neuro- osteoporosis develops rapidly in the first year post-injury,
logic review of systems, should be included in periodic with about one-third of the original bone mass being lost
follow-up. Appropriate electrodiagnostic and imaging by 16 months post-injury before relative stability is
studies are indicated in the presence of signs or symptoms achieved (57). An extremity fracture rate of over 30 per-
of neurologic deterioration (46,47). cent for individuals followed for several decades has been
834 SPECIAL TOPICS IN SPINAL CORD MEDICINE
reported in recent surveys (58). In addition, data from the some may actually improve. When potential difficul-
U.S. Model Spinal Cord Injury Systems indicate that ties in these areas do occur, early identification and
women are more likely to develop long bone fractures intervention can delay, minimize, or even eliminate the
in the lower extremities as the time post-injury increases negative consequences of age-related changes. Unfor-
(38). Various interventions have been proposed, includ- tunately, such interventions may be difficult to imple-
ing standing, functional electrical stimulation (FES), and ment with any degree of certainty as to effectiveness,
treatment with biphosphonates. However, no treatment and efforts in identifying effective strategies may be
has yet been shown to provide long-term prevention of hampered by misperceptions of older individuals and
osteoporosis and protection from fracture risk. Prelimi- their quality of life. It has been asserted, in fact, that
nary trials of pamidronate have shown some promise, ageism still has a detrimental effect on healthy aging
and it is hoped that continued investigation in this area (67). A further consideration is that the same interven-
will lead to effective treatment in the future (59). SCI clin- tions that are found to be effective in the general pop-
icians should incorporate a thorough symptom review ulation may be inappropriate or ineffective for individ-
and examination as a part of periodic reassessment to uals aging with SCI.
identify musculoskeletal complaints at their earliest onset.
In addition, equipment and seating system interventions
Independence
may improve the suboptimal posture that often results
in chronic back pain (48). Aging individuals may experience a loss of muscle
strength, slowed reflexes, decreased coordination, lower
energy levels, chronic pain, arthritis, and osteoporosis,
THE IMMUNE SYSTEM which can result in declining physical independence
(68,69). Data from the 1996 National Health Interview
The normal immune system declines with age, and the Survey showed that more than 22 percent of the civilian
risk of infection increases, according to research (6062). community-dwelling population of the United States,
In addition, the function of the immune system is known aged 45 to 64 years and more than 36 percent of those
to be influenced by factors such as depression, deteriora- aged 65 and older reported some degree of activity limi-
tion of social support systems, chronic pain, neuroen- tation (70).
docrine changes, and the influence of medications (63). In SCI, some form of activity limitation typically is
There is evidence of a diminished immune function present from the onset of the injury. Aging may further
in people with SCI above the T10 level, which is manifest magnify dependency issues as the individuals needs, abil-
by impaired bacterial phagocytosis (64). A longitudinal ities, and limitations change over time. A recent longitu-
study of people with SCI of more than 20 years duration dinal study of individuals with long-term SCI found
showed a dramatic increase in urinary tract infections advancing age to be a significant predictor of functional
(UTIs) among those aged 60 and over, and a slight increase decline. The average age when additional functional
in the frequency of infection between the tenth and thirti- assistance was first needed was 49 years for those with
eth post-injury year (28). Because factors such as depres- tetraplegia or tetraparesis and 54 years for those with
sion, diminished psychosocial support, polypharmacy, and paraplegia or paraparesis (68). Functional decline or
chronic pain may coexist in people with SCI, it would decreasing physical independence has been identified as
appear safe to assume that those aging individuals will an adverse outcome of long-term SCI. Research has
have an increased likelihood of immune impairment when shown that 22 percent of participants report a decline
compared to their nondisabled counterparts. over a 3-year time span (71), as well as both cross-sec-
Several recent studies have suggested that exercise tional and longitudinal significant increases in the need
and rehabilitation therapies are associated with improved for assistance among older individuals (72).
cellular immunity in persons with SCI (65,66). It is hoped It is encouraging that the possibilities of preserving
that further research in immunology, immunologic assess- functional ability and maintaining independence are
ment, and treatment will result in interventions designed areas amenable to intervention. These interventions
to improve immune defenses in people with chronic SCI. include changing the mechanics which certain activities
(such as transfers) are accomplished by using new equip-
ment and devices, or modifying existing equipment. In
PSYCHOSOCIAL ASPECTS situations where assistance from others does increase or
OF SCI AND AGING become necessary, efforts should be made to incorporate
this help into an individuals life in a way that still enables
Unlike many of the physical changes associated with the person with SCI to maintain maximal independence,
aging, it has not been the case that some psychosocial such as having the power to hire, train, and even fire
aspects of a persons life necessarily decline. In fact, helpers.
SPINAL CORD INJURY AND AGING 835
Community Integration people with SCI, when compared to the general popula-
tion (79). A longitudinal study of aging in SCI found no
For various reasons, activity levels can be expected to significant differences in perceived stress by age, duration
decline with advancing age (73), and it is an all-too-com- of injury, gender, or severity of impairment among a
mon belief that disengagement from society and declining group of British individuals whose injuries were at least
involvement in social activities is a normal adjustment 20 years in duration (89). In fact, there was a tendency
to aging. Studies have shown variable results, with some toward less stress in older individuals. In addition, results
findings indicating that decreases in community integra- from the Baylor College of Medicine Life Status Study
tion are not always the case, and in some cases, the extent showed no relationship between age and stress in a sam-
of social participation does not decline with age (74). ple of community-dwelling people with SCI (90).
The idea of community integration is a concept that Compared to the relatively limited information on
goes beyond physical functioning, and includes an indi- long-term stress and depression related to aging with SCI,
viduals role as an active, productive member of society the study of quality of life in SCI literature has provided
who is well integrated into the family and community more insight into long-term psychosocial outcomes. Not
(75,76). Specific studies of individuals with SCI have sum- surprisingly, it has been suggested that quality of life
marized the finding that individuals with less severe neu- among people with SCI is generally lower than among
rologic injuries, of younger age, of Caucasian ethnicity, and those without disabilities (91,92). The research findings,
more education will achieve greater community integra- however, tend to be contradictory. Whereas some stud-
tion (77). Although such demographic factors (with the ies indicate that life satisfaction for people with SCI is not
exception of education) cannot be changed, these variables necessarily negatively affected by aging (72,93,94), oth-
accounted for less than 10 percent of the variance in social ers find significant differences in self-perceived quality of
integration scores, as measured by the Craig Handicap life, with younger individuals and those injured shorter
Assessment and Reporting Technique (CHART) (78). periods of time rating their quality of life better than older
Clearly, a variety of other factors influences successful individuals did (28,95,96). This variation in findings may
community integration for individuals aging with SCI. be a consequence of the differences in how older and
younger individuals assess quality of life. It is clear, how-
ever, that aging is only one aspect of quality of life, and
Stress, Depression, and
life satisfaction following SCI can also be related to over-
Perceived Quality of Life
all general health, social support, community integration,
Depression and life satisfaction may be related to how and personal factors, as well as level of injury and expec-
well a person copes with the changes that occur as a result tations about functional abilities (94,97101).
of aging (79). Evidence also shows that chronic stress can It becomes the responsibility of both healthcare
be detrimental to health (80), although it may not be com- providers and people with SCI and their families to work
pletely accurate to assume that older people experience cooperatively in an attempt to identify the underlying fac-
more stress than younger individuals (81). Findings, how- tors that influence quality of life, increasing stress, or
ever, link stress and poor health with depression in older depression. This involves a careful look at the person with
individuals (82). Because it is difficult to separate mental SCI, and his or her living situation, resources, and envi-
and physical health in older individuals, the consequences ronment. Successful aging is the result of a well-integrated
of depression in later life can be quite serious (83). In fact, assessment of these multiple areas and making adjust-
studies have demonstrated that psychosocial factors such ments or recommendations to benefit as effectively as pos-
as depression are independent, etiologic and prognostic sible the person aging with SCI.
factors for coronary heart disease (8486).
In light of the above evidence, some findings indi-
cate that perceived quality of life is not necessarily worse LOOKING TO THE FUTURE
for older individuals, even for those with chronic illnesses
(87). Often called life satisfaction, happiness, and well- Mortality and Life Expectancy
being, quality of life is a multidimensional concept, incor-
porating a combination of biologic, psychologic, inter- Numerous studies attest to the finding that survival fol-
personal, social, economic, and cultural dimensions (88). lowing SCI has improved dramatically, not only in the
As a result, aging, in and of itself, may not be specifically early stages but over the longer term as well (102), with
associated with a lower perceived quality of life. life expectancies often extending into the sixth and sev-
Research regarding stress, depression, and quality of enth decades of life. In spite of these improvements, how-
life in people aging with SCI generally supports findings ever, life expectancy for the person with SCI is still much
in the general aging literature, although there is a ten- lower than that of the general population. Survival fol-
dency for baseline stress and depression to be greater for lowing SCI is affected not only by level and severity of
836 SPECIAL TOPICS IN SPINAL CORD MEDICINE
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62
Womens Health
Challenges after
Spinal Cord Injury
839
840 SPECIAL TOPICS IN SPINAL CORD MEDICINE
FIGURE 62.1
nerves, carry motor and sensory fibers to the perineum. These modulators stimulate ovarian follicular growth and
Sensory feedback is also provided by the anterior branch steroidogenesis, respectively. The phase of follicular
of the ilioinguinal (L1) nerve, which supplies the mons growth occurs before ovulation. The endometrial wall
pubis and labia majora. The genitofemoral nerve (L1,L2) hypertrophies at this time. At the point of ovulation FSH
supplies the rest of the labia majora, and the posterior and LH rapidly increase (LH/FSH surge). Following
femoral cutaneous nerve supplies the posterior vulva. release of the egg from the ovary (ovulation), the uterus
The menstrual cycle occurs in a controlled sequence begins its luteal phase. Specific positive and negative feed-
of events to ensure precise hormonal regulation of the back mechanisms also direct other end-organ functions,
hypothalamus, pituitary, and ovary. Gonadotropin releas- such as endometrial lining composition, cervical mucous
ing hormone (GNRH) is secreted in a pulselike release secretions, and vaginal cell properties; all of which are
from the hypothalamus (2). It exerts positive expression important for successful fertilization (3).
on the pituitary to release two gonadotropins: follicle sim- The vertebral thoracic nerves 3, 4, and 5 supply the
ulating hormone (FSH) and lutenizing hormone (LH). breasts. Their stimulation is important for prolactin secre-
WOMENS HEALTH CHALLENGES AFTER SPINAL CORD INJURY 841
tion and breast milk production. The neurotransmitter sequelae of SCI. In a self-reported multicenter study of
dopamine suppresses prolactin secretion and GNRH 472 women (14) with SCI, when asked about perimen-
release (4). Many other neuromodulators (5) that are also strual problems, 25 percent experienced increased auto-
found in the spinal cord have been found to contribute nomic symptoms (sweating, flushing, headaches, or goose
to the hypothalamic pituitary feedback control system. flesh); 18 percent had more frequent bladder spasms, and
Examples of these are norepinephrine, serotonin, endoge- 22.5 percent said they had worsening of muscle spastic-
nous opioids, prostaglandins, and various growth factors. ity. These symptoms are analogous to the dysmenorrhea
or cramping some able-bodied women describe at the
time of menstruation. Studies of able-bodied women link
GYNECOLOGICAL ISSUES AFTER SCI these premenstrual symptoms to elevated prostaglandin
F2 alpha in the endometrium (15). Often anti-inflamma-
At the time of SCI, the spinal cord enters a state of spinal tory agents such as naproxen, ibuprofen, or mefenamic
shock. It has been hypothesized that the disruption of the acid can alleviate these SCI symptoms.
internal neurobiologic communication that ensues also Return of menstruation and its long-term manage-
has repercussions on the feedback systems of the hypo- ment is a significant concern for women with SCI. The
thalamic pituitary axis (6,7). In fact, following SCI, the previously mentioned self-reported study reported no sig-
menstrual cycle is also disrupted and secondary amenor- nificant changes in menstrual cycle length (25.1 days vs.
rhea, menorrhagia, metrorrhagia (8), and neurogenic pro- 27.9 days) or length of menstruation (5.1 days and 5.0
lactinemia with and without galactorrhea (7,9) may days) in pre- and post-injury experiences, respectively. Of
occur. Other conditions following a traumatic SCI may interest were problems in menstrual management and
have an influence on gynecologic endocrine balance. bladder continence during menses, in which tampons and
Nutrition, stress, serum mineral and electrolyte distur- pads cause catheter occlusion or interference with its
bances, and medications (especially steroids) (10) have placement. Labial pressure sores from catheters more
been shown to affect hypothalamic pituitary communi- commonly occur during this time. Furthermore, mobil-
cation. In a pilot study of twenty-five women post-injury ity limitations affect menstrual hygiene, which places
(11) almost two-thirds had mild to greatly elevated pro- these women at high risk for toxic shock syndrome if tam-
lactin levels at some point after their injury. Twelve per- pons are not changed frequently. Higher levels of SCI pre-
cent developed galactorrhea. Other endocrine abnormal- vent independent self-care for many women during this
ities, such as hypoestrogenemia, were also documented. time of the month. It is not surprising for some women
Interestingly, those women with injuries involving tho- to seek a solution by undergoing a hysterectomy, endome-
racic levels T3 through T5 tended to have a greater fre- trial ablation procedure, or by taking hormones for sup-
quency of prolactinemia with galactorrhea. This has also pression of menses (13).
been shown to occur in chest wall injuries (12). Neuro- Birth control practices are important for those
genic prolactinemia-galactorrhea syndrome mimics lac- women sexually active and not wishing pregnancy. The
tation, wherein the thoracic nerves are stimulated and a multicenter womens health study shows that 70.3 percent
disinhibition of dopamine control occurs, thereby pro- of those women sexually active after SCI practice birth
ducing breast milk. control. Following SCI there was a preference for part-
Endocrine function should be assessed post-injury ners using condoms (38.9 percent); followed by permanent
in all women. Hormone alterations can interfere with sterilization (26.1 percent); then oral contraceptives (OCP)
bone metabolism, vasomotor regulation, skin integrity, (22.1 percent). Oral contraceptive usage (57.2 percent)
and emotional well-being. If after 6 months, menstrua- was the birth control of choice before injury.
tion has not returned to pre-injury patterns, then check- Some combinations of oral contraceptives with
ing FSH, LH, estradiol, thyroid stimulating hormone, and higher estrogen concentrations have risks for deep venous
prolactin levels will identify the underlying imbalance. thrombosis (DVT) or myocardial infarction in some able-
Standard interventions (10,13) include oral contracep- bodied women, especially if they are older than 35 years
tives to regulate menstrual cycling or bromocryptine to of age and smoke cigarettes (16). Although the immedi-
correct prolactinemia or galactorrhea. One must keep in ate immobility in women with SCI predisposes them to
mind, however, that the potential for increasing vascular DVT (17) acutely post-injury, there are no data that prove
thrombotic events with these agents in this patient pop- that risks are any greater with OCPs in women with
ulation has not been well studied. chronic SCI than in the able-bodied population. OCPs
With the resumption of regular menstruation (usu- or any form of hormonal manipulation for birth control,
ally 3 to 6 months after injury) women return to pre- such as depot-medroxyprogesterone acetate (DPMA)
injury fertility status. Birth control is thus advisable if injections or subdermal hormonal implants, should be
pregnancy is not sought during this time. The resumption prescribed with caution and avoided in most women
of a regular menstrual cycle may also exacerbate the within 1 year of injury, smokers, and anyone with past
842 SPECIAL TOPICS IN SPINAL CORD MEDICINE
history of DVT (pre- or post-injury) or cardiovascular also exerts pressure on the inferior vena cava and com-
conditions. Monthly or tri-monthly DPMA injections are mon femoral veins, which will worsen edema and possi-
alternatives for birth control and can produce amen- bly predispose these women more to DVT (2123).
norhea. The mechanism of action of these injections is via As transfers become difficult and weight alterations
hypoestrogenemia, which, with long-term use, may lead change seating contours, pressure ulcers may develop. Fur-
to osteoporosis and cardiovascular sequalae (18). thermore, changing nutritional demands and the anemia
Other birth control options must also be carefully of pregnancy impairs skin healing once a decubitus devel-
considered before prescription. The intrauterine device ops. Bowel motility decreases during pregnancy as a result
(IUD) (19) is associated with increasing the risk of pelvic of increased progestin and iron supplementation. Bowel
inflammatory disease in some women whose sexual activ- programs must be modified to adjust to this dramatic
ity includes multiple partners. This becomes more impor- change. Again, the temporary loss of mobility makes main-
tant in women with SCI who have chronic bacteria and taining independence with bowel management difficult.
urinary tract infections (UTIs). Another complication of Other pregnancy difficulties described by women
the IUD is displacement or perforation of the uterus. with SCI include frequent autonomic dysreflexia, wors-
Because many women with SCI will not be able to per- ening spasticity, and loss of prepregnancy independence
ceive pain, the presenting symptoms of pelvic inflamma- (14). As many individuals with SCI are on multiple med-
tory disease or uterus perforation will be missed. Before ications, each should be examined for its appropriate use
IUD placement in these women, a complete sexual and during each trimester of pregnancy or lactation. The U.S.
urologic history should be obtained and appropriate Food and Drug Administration (FDA) classifies drugs by
counseling given. a use-in-pregnancy rating system based on risks and ben-
Diaphragms, cervical caps, or vaginal sponges are efits to the fetus and mother. The ratings are as follows:
other options. Women with higher levels of injury, how-
ever, cannot place them independently. A diaphragm must Category AControlled studies show no risk. Ade-
remain in place at least 6 hours after intercourse to be quate, well-controlled studies in pregnant women
effective, and prolonged pressure against the vaginal wall have failed to demonstrate a risk to the fetus in any
may create mucosal breakdown. trimester of pregnancy.
Sexually transmitted diseases (STDs) have been Category BNo evidence of risk in humans. Ade-
reported as being less frequent following injury (14), at quate, well-controlled studies in pregnant women
about 2.5 percent of women who are sexually active. More have not shown increased risk of fetal abnormalities
study is needed to explore this health issue in this patient despite adverse findings in animals, or, in the absence
population. Concern exists that STDs may not be recog- of adequate human studies, animal studies show no
nized and are thus underdiagnosed and undertreated. fetal risk. The chance of fetal harm is remote, but
remains a possibility.
Category CRisk cannot be ruled out. Adequate,
PREGNANCY well-controlled human studies are lacking, and ani-
mal studies have shown a risk to the fetus or are
Although some data suggest (14) that women with SCI lacking as well. There is a chance of fetal harm if the
become pregnant for the first time at an older age than drug is administered during pregnancy, but the
able-bodied women, social issues may play a major role. potential benefits may outweigh the potential risk.
In a cross-sectional study, 66 (13.9 percent) out of 472 Category DPositive evidence of risk. Studies in
women with SCI, average age 40, reported having had a humans, or investigational or postmarketing data,
total of 101 pregnancies post-injury. The relatively lower have demonstrated fetal risk. Nevertheless, poten-
pregnancy rate makes prospective studies about obstet- tial benefits from the use of the drug may outweigh
ric complications difficult. Unique medical problems, the potential risk. For example, the drug may be
however, appear to occur in these women during preg- acceptable if needed in a life-threatening situation or
nancy, labor, and delivery. serious disease for which safer drugs cannot be used
Neurogenic bladder dysfunction and pregnancy or are ineffective.
increase the incidence of UTIs. Chronic antibiotic sup- Category XContraindicated in pregnancy. Studies
pression may be warranted. The growth of the fetus and in animals or humans, or investigational or post-
abdomen places pressure on the bladder, thus predispos- marketing reports have demonstrated positive evi-
ing it to urine leakage either around the catheter or dence of fetal abnormalities or risk that clearly out-
between catheterizations (20). Interference of diaphragm weighs any possible benefit to the patient.
movement will diminish respiratory capacity and increase
the risk of pneumonia or respiratory compromise, espe- Table 62.1 includes commonly prescribed medicines
cially in women with tetraplegia. The growth of the fetus for SCI sequelae and potential maternal fetal contraindi-
WOMENS HEALTH CHALLENGES AFTER SPINAL CORD INJURY 843
TABLE 62.1
Common Medications Used by Women with SCI and Precautions for Use in Pregnancy and Lactation
FDA USE-IN-
PREGNANCY SECRETED IN
TYPE EXAMPLES RATING CATEGORY BREAST MILK COMMENTS
TABLE 62.1
Common Medications Used by Women with SCI and Precautions for Use in Pregnancy and Lactation
(continued)
FDA USE-IN-
PREGNANCY SECRETED IN
TYPE EXAMPLES RATING CATEGORY BREAST MILK COMMENTS
cations. To avoid withdrawal symptoms, careful weaning lesions above T6 and a small percentage below T6 (27)
is advisable. will experience autonomic dysreflexia during uterine con-
The level of SCI has an affect on the perception of tractions during labor and delivery. The sequelae of fetal
labor. It has been recommended that after 28 weeks of distress, maternal intracranial hemorrhage, coma,
gestation, the woman should be frequently checked for seizures, and even death have been described (2830) if
effacement and dilation (21,24). Tocodynamometry or this condition goes unrecognized and untreated. Treat-
early hospitalization may be necessary. Labor is mediated ment is prompt delivery of the baby and placenta or
by neurohumeral factors (22). The change in estrogen, regional epidural or general anesthesia (31). Occasionally
in part, influences parasympathetic-mediated uterine con- parenteral antihypertensives (32) are required. It is impor-
tractions. Progesterone indirectly exerts its effect on sym- tant to distinguish autonomic dysreflexia from pre-
pathetic control to adjust the strength of contractions. eclampsia (33), which occurs with the same frequency in
In able-bodied women, pain during the first stage of labor both able-bodied women and women with disabilities.
is primarily from uterine contractions via the T10 The cardiovascular symptoms of dysreflexia are hyper-
through L1 spinal cord levels. This is followed by dila- tension, severe headache, bradycardia or tachycardia, and
tion of the cervix and perineal stretching, which produces occurrence of these symptoms during uterine contractions
pain via the pudendal nerve and its branches (S2S4) dur- only. The hypertension, tachycardia, and protenuria of
ing the end of the first stage and throughout the second eclampsia occur throughout labor.
stage of labor. Delivery may be further complicated by the muscu-
Unrecognized conventional labor symptoms and loskeletal sequelae of SCI, which may include hip disar-
delivery can occur especially in women whose levels are ticulation, contractures, heterotopic ossification, previ-
T10 and above. However, women often experience atyp- ous femur fractures, scoliosis, and severe spasticity.
ical labor symptomatology. Pain above the level of injury, Positioning on the delivery table may be problematic.
referred pain to the shoulders or upper back, abnormal These conditions may contribute to more frequent use of
nonspecific pain, increased spasticity, autonomic dysre- forceps or vacuum or cesarean section delivery (14,21).
flexia, and increased bladder spasms may indicate labor Early reports (22,34,35) of pregnancy outcomes for
(23). Prenatal education should include an explanation of women with SCI suggest a higher incidence of cesarean
these possible symptoms. section, episiotomy dehiscence, failure to progress in
By far the most critical complication of labor and labor, breech presentation, and low birth weight. Other
delivery is autonomic dysreflexia. It has been reported studies, however, support that women with SCI have out-
that 60 to 80 percent (25,26) of women with SCI with comes similar to able-bodied women (14,33). Good
WOMENS HEALTH CHALLENGES AFTER SPINAL CORD INJURY 845
prospective studies are needed to confirm the risks of fear of being a mother with a disability, and social avoid-
these delivery complications. ance should be addressed for sexual well-being.
SCI has a significant impact on the sexual behavior and In the past, urologic management has been unimaginative
function of women (3638). In reports of women with (at best) for the woman with SCI. The lack of an exter-
SCI, the percentage who had sexual intercourse before nal collection device has for the most part confined her to
injury (87 percent) was comparable to national surveys indwelling catheters or incontinence unless she has an are-
of able-bodied women (14). This study showed that par- flexive bladder and can perform intermittent self-catheter-
ticipation in sexual intercourse decreased after SCI and izations. Today, however, the knowledge of this aspect
did not reach pre-injury level, although it did increase sig- of womens health has virtually exploded, giving women
nificantly over time. The physiologic changes following with SCI many options (4648). Because SCI urologic
SCI, as they affect the sexual response cycle (39), are neu- management is addressed in Chapter 23, details are not
rologically level dependent. The excitation phase of lubri- provided here. One must remember, however, that the
cation and clitoral enlargement is primarily mediated by ultimate goal of satisfactory bladder management is to
the parasympathetics (S2S4). The components of decrease urologic complications and to preserve long-
orgasm, which includes the rhythmic contractions of fal- term renal function (4951). Bladder management meth-
lopian tubes, uterus, and paraurethral muscles are pre- ods are successful only when individualized to a womans
dominantly controlled by the sympathetics (T10L1). neurologic level, completeness of injury, hand function,
The final stage of orgasm (which is analogous to ejacu- caretaker support, employment activities, and desire to
lation in males) is directed by parasympathetic and perform one type over the other.
somatic afferents (40). Further studies by Sipski (41,42)
showed that women with incomplete upper motor neu-
ron SCI or complete SCI could have reflex lubrication. MENOPAUSAL ISSUES
Although they were significantly less likely than able-
bodied women to achieve orgasm, there was no signifi- It is known that individuals with SCI experience various
cant difference among different levels and completeness pathophysiologic changes as a result of their neurologic
of injury to achieve orgasm. Another phenomenon deficits. Aging can further add new disability (52) from
unique to women with SCI is the ability to experience advancing osteoporosis (53); cardiovascular disorders
paraorgasms or intensely pleasurable feelings above the (54); osteoarthritic conditions (55); spine deformity, joint
injury level (43). In addition, sensitive areas, such as the pain, respiratory decline, and impingement neuropathies
breasts or neck, may become focal erogenous zones for (56,57). Individuals who achieve a specific level of func-
sexual fulfillment (44). tion post-injury may find that they are not able to main-
New studies (14) relate those problems encountered tain that level and will require wheelchair adaptation,
during sexual activity that are injury dependent after SCI. new assistive devices, pain therapy intervention, or even
Among these are difficulties with positioning for inter- increased or new attendant care.
course, problems with Foley catheter, occurrence of blad- Women who undergo menopause experience the
der incontinence and unwanted bowel reflexes, and auto- physiologic effects of estrogen deficiency, which can alter
nomic dysreflexia. To aid the woman in dealing with these bone metabolism nd result in osteoporosis. Estrogen
physiologic changes as they affect her sexual function, depletion further contributes to the decline of dermato-
practitioners must openly discuss sexual issues with these logic, urologic (58), and cardiovascular (59) systems.
women. Books such as that by Duchan and Gill (45) are Emotional disturbances are also often associated with
helpful consumer references. If lubrication does not occur, estrogen withdrawal. Thus, as women who have had
prescribing a commercial lubricant is a simple interven- chronic SCI transition through menopause, the physio-
tion. Prophylactic antispasticity or autonomic dysreflexia logic consequences of hormone alterations result in com-
medications before intercourse may be helpful. Emptying pounded deficits, which can create significant functional
the bowel or bladder and taping or removing the and psychologic impairments.
indwelling catheter are helpful hints for unreliable bowel Immobility is a consequence of paralysis, contrac-
and bladder reflexes during intercourse. Deflating the tures, sensorimotor deficits, or other neurologic disorders.
intravesical balloon of the catheter diminishes its interfer- Years of diminished weight-bearing activities predispose
ence. Creative changes in positioning are helpful in both women and men with disabilities to disuse osteo-
addressing musculoskeletal changes. Finally, psychologic porosis (53,60). For women, however, the menopausal
parameters such as poor body image, fear of pregnancy, transition heralds estrogen deficiency that further com-
846 SPECIAL TOPICS IN SPINAL CORD MEDICINE
pounds loss of bone mass. Unfortunately, the quantifica- cal disorder (72). These manifest in wide blood pressure
tion of bone loss in women with SCI has not been stan- swings, bradycardia, tachycardia, flushing, sweating, and
dardized. No indicators exist for determining typical bone chills. The menopausal transition and its hormonal alter-
loss from either the disuse or menopausal state. It is ations can exaggerate these conditions. Furthermore,
unknown what therapies are efficacious or when the autonomic dysreflexia, which is a condition that occurs
appropriate interventions could be initiated to delay the in high paraplegia and tetraplegia, has been a reported
progression of osteoporosis (61). symptom of hormonal changes during the menstrual cycle
A concern exists that the osteoporosis that occurs (73). It presents as symptoms of hypertension, headache,
following SCI may lead to further compression fractures, flushing, and bradycardia. Although studies are lacking
scoliosis, and kyphosis. Severe spinal curvature changes to confirm the prevalence of autonomic dysreflexia in
are associated with predisposing individuals with SCI to menopausal women with SCI, its symptoms and appro-
spondylitic, posttraumatic tethering and syringomyelia priate intervention may not be recognized. On the other
(62), which can produce new neurologic loss. Curvature hand, symptoms of vasomotor disturbances and hypo-
and posture changes can lead to pain, radiculopathies (63) thalamic thermoregulation are pathognomonic for
above the injury level; decrease in respiratory function decreasing estrogens during menopause. Hot flashes
(64) with predisposition to pneumonia; and difficulty in are in part controlled by the central nervous system (74),
swallowing (65). but have never been studied in women with SCI.
Lower extremity fractures are a consequence of Hormone replacement therapy (HRT), using com-
osteoporosis in able-bodied, postmenopausal women binations of estrogen and progesterone, is advocated for
(66). Recently, an analysis of the National SCI database the amelioration of symptoms such as hot flashes, sleep
revealed a trend for women with SCI to more likely disturbances, vaginal changes, and mood swings. It has
develop lower extremity long bone fracture as time post- been effective in reducing postmenopausal heart disease,
injury increased (67). In fact, for women seen 20 years osteoporosis, and Alzheimers disease (75). Consideration
post-injury, with an average age of 52 years (average age must be made, however, for the recent study by Schairer
of menopause for able-bodied is 51) (5), there was a sta- (76) relating HRT to increased risk of breast cancer. It has
tistically higher reported incidence of lower extremity also been related to uterine cancer (77).
long bone fractures than in males during that follow up. The treatment of menopause sequelae in women
Estrogen deficiency has also been postulated to with disabilities has been controversial in the past (55).
worsen arteriosclerosis and cardiovascular disease (68) Concern exists however, that these women have an
by altering lipoprotein metabolism (69). Women with SCI increased risk of delayed diagnosis and treatment of all
are also prone to atherosclerosis because they participate reproductive health problems, including gynecologic can-
less in aerobic activities and have a greater tendency to cers. A survey conducted at the Baylor Center of Research
lead sedentary lifestyles. Premature heart disease, hyper- on Women with Disabilities (72) indicated that women
tension, or stroke becomes problematic, especially in light with mobility impairments were significantly more likely
of the fact that these women may undergo menopause at than women without disabilities to be concerned about
an earlier age than able-bodied women (14,70). Estro- whether HRT is safe for them. There has been little evi-
gen deficiency also contributes to skin changes such as dence to support that women with disabilities are at an
dermal thinning, loss of elasticity, and diminished sub- increased risk of HRT side effects, such as increased
cutaneous blood flow (58). The physically challenged are hypercoagulability. Some women with SCI may have rel-
at higher risk for developing pressure sores, especially if ative contraindications to conventional HRT or may not
there is a sensorimotor impairment (71). Postmenopausal wish to take HRT or other menopausal supplements like
women with SCI have an increased risk of developing bisphosphonates, calcitonin, or selective estrogen recep-
pressure sores when compared to premenopausal women tor modulators (SERMSs). Natural hormone treatments
with disabilities. Furthermore, wound healing may be and alternative therapies, such as phytoestrogens, may
delayed in comparison to able-bodied women. Related relieve menopausal symptoms and be safer alternatives
skin changes in the vaginal and urethral mucosa lead to (7880). Additional research is needed to establish the
atrophy of the vaginal epithelium. Conditions such as efficacy of these alternatives, especially in this patient
vaginal and urethral drying, vaginitis, and patulous ure- population.
thra may affect bladder management and continence in Evidence proving the safety and effectiveness of both
those women who have had stable and previously satis- conventional and alternative menopausal treatments in
factory neurogenic bladder function. women with a variety of disabling, chronic conditions is
Vasomotor instability is another menopausal con- surfacing in the literature. These interventions may reduce
sequence that may be exaggerated in women with SCI. It the risk of osteoporosis, early coronary heart disease,
has been reported that women with SCI often experience incontinence exacerbation, and skin breakdown in the
labile vasomotor responses from the primary neurologi- mature woman with a disability.
WOMENS HEALTH CHALLENGES AFTER SPINAL CORD INJURY 847
PSYCHOSOCIAL ISSUES AND WELLNESS ing abuse by spouses (37 percent); strangers (28 percent);
parents (15 percent); service providers (10 percent); and
Many important psychosocial issues face women with dates (7 percent). Of these, 12 percent were raped. How-
disabilities. The impact of SCI is just as pronounced on ever, less than half of these women reported their abuse.
the woman with standard as well as nontraditional As with child protection laws, many states have manda-
roles in life. Studies in the past have compared the short- tory reporting for healthcare clinicians who suspect abuse
term impact on the marital status of both men and women or neglect in any individual with a disability.
who have sustained a SCI. DeVivo and Fine (81) looked Preventive health care is another social issue often
at divorce rates and marriage rates in individuals married neglected in women with disabilities. A study by Nosek
at the time of injury and compared them with the gen- (87) reported that women with disabilities were less likely
eral population. Although they were unable to predict to receive regular pelvic examinations than women with-
which gender would be least likely to marry post-injury, out disabilities. Lack of facility accessibility, trained per-
it was interesting to find that for those married at the time sonnel, and clinician knowledge of a disability set up
of injury, being female, of younger age at injury, African- physical and attitudinal barriers. In addition, women
American, without children, and having higher levels of with a disability may not know what preventive health
injury were all associated with the greater likelihood of practices are recommended. Current recommendations
divorce. Later, a study examining the divorce rate of indi- by The American College of Obstetrics and Gynecololgy
viduals who married after their injury showed the oppo- are for Papanicolaou (Pap) tests and other screening pro-
site effect: males and individuals previously divorced were cedures to be performed annually depending on the age
2.2 times more likely to get divorced than females or indi- of the women (88). Pap smears are to be performed at
viduals married for the first time (82). onset of sexual activity with follow-up examinations at
Parenting issues are paramount for mothers or moth- least every 3 years or annually if the woman has multi-
ers-to-be with SCI. Ensuring the safety, care, and discipline ple or new sexual partners. Mammography for breast
of their children (83) requires creative support systems and cancer detection should be initiated between the ages of
adaptations to manage with physical challenges. Accep- 40 and 45, and every 1 to 2 years for women to age 50
tance of extra support can free the mother to focus on and annually after 50. For women with a family history
those critical parenting skills based on love, bonding, and of breast cancer or who have never had a baby, annual
leadership that define her as the childs mother (84). screening should begin during the early 40s. Education
Abuse against women is a significant social problem by the healthcare practioner for monthly breast self-
(85). Awareness of this problem in women with disabil- examinations should be addressed with all women. Table
ities has just begun to surface (86). In one study, 40 per- 62.2 illustrates other recommended preventive health
cent of 245 women with disabilities reported experienc- screenings for women. HIV testing, TB skin tests, hema-
TABLE 62.2
Recommended Preventive Health Screening for Women (88)
globin, lipid profiles, stool guiac, colonoscopy, and other 17. Brach BB, Moser KM, Cedar L, Minteer M, Convery R. Venous
thrombosis in acute spinal cord paralysis. J Trauma 1977;
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19. Farley TM, Rosenberg MJ, Rowe PJ, Chen JH. Meirik. Intra-
unterine devices and pelvic inflammatory disease: An international
CONCLUSION perspective. Lancet 1992; 339:785788.
20. Verduyn WH. Spinal cord injured women, pregnancy and deliv-
ery. Paraplegia 1986; 24:231240.
The medical management of women following an SCI 21. Cross LL, Meythaler JM, Tuel SM, Cross AL. Pregnancy follow-
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health. Unfortunately, our understanding of the full effect to Years (special issue). West J Med 1991; May 157:607611.
22. Ohry A, Peleg D, Goldman J, David A, Rozin R. Sexual function,
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ulation grows, a collaborative approach between physi- Obstet Invest 1978; 9:281291.
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63 Spinal Cord Disorders in
Children and Adolescents
his chapter reviews spinal cord child-life and recreation therapy. In contrast, older ado-
satisfaction. Adults with childhood-onset SCI or those younger than 20 years of age (2129). As in adults with
with myelomeningocele are employed less frequently than SCI, males are more commonly affected than females dur-
the general population. This is particularly important, ing adolescence. However, as the age that children sustain
because employment is significantly associated with life a SCI decreases, the preponderance of males becomes less
satisfaction. Compared to able-bodied peers, children marked, and by 3 years of age the number of females with
and adolescents with SCI participate in significantly fewer SCIs equals that of males (25,26,30,31). The life
prevocational activities (9). Children with spinal cord dis- expectancy of children and adolescents with SCIs varies
orders should be expected to have age-appropriate with the neurologic level and the degree of completeness
chores, and as they grow up, they must be involved in of the SCI (30,32). The less severe the neurologic deficit,
developmentally appropriate prevocational and voca- with respect to both level of injury as well as category, the
tional activities that will adequately prepare them for longer the expected survival. In comparison to the gen-
adult employment (911). eral population, life expectancy ranges from 59 to 64 per-
Comprehensive primary care for children and ado- cent for individuals with C1C4 injuries, 70 to 73 per-
lescents with spinal cord disorders is frequently neglected cent for those with C5C8 lesions, 80 to 84 percent for
and overshadowed by tertiary care needs (1215). In those with paraplegia, and 88 to 89 percent for those with
addition to standard childhood immunizations, children Frankel D lesions (32).
and adolescents with spinal cord disorders should be The neurologic level and degree of completeness
immunized with the pneumococcal vaccine when they are varies with age, with younger children more likely to be
2 years of age or older and yearly influenza vaccination paraplegic and have complete lesions (30,32). Among
should begin at 6 months of age (16). Children who are children who sustain their SCI when they are 8 years old
12 years of age and younger should receive two doses of or younger, 70 percent are paraplegic and approximately
the split virus preparation of the influenza vaccine 1 two-thirds have complete lesions. In contrast, approxi-
month apart the first time they are immunized against mately 50 percent of older children and adolescents are
influenza (16). paraplegic, and approximately 50 percent have complete
lesions. Younger children are more likely to have upper
cervical injuries and less likely to have C4C6 injuries,
SPINAL CORD INJURIES which is the more common level for tetraplegia in older
children and adolescents (25,3032). Infants and young
The distinctive anatomic and physiologic features of chil- children are more susceptible to upper cervical injuries
dren and adolescents, along with growth and develop- because of their proportionately larger heads and under-
ment, are responsible for the unique manifestations and developed neck musculature.
complications of SCI in the pediatric population (1720). The most common cause of SCI in children and ado-
SCI without radiologic abnormalities (SCIWORA), lap- lescents is motor vehicle crashes, with violence and sports
belt injuries, birth injuries, upper cervical injuries, and the being the next most common etiologies (30,32,33). Vio-
delayed onset of neurologic deficits are relatively unique lence causes SCI in children of all ages, but is an especially
to pediatric SCI and are a result of sustaining a SCI at a common cause in adolescents, particularly among His-
young age. As a result of growth, children who sustain panics and African-Americans (30,32). Although violence
their SCI before 8 to 10 years experience a higher inci- remains a major cause of SCI in the pediatric population,
dence of scoliosis and hip dislocation. Patient compliance there has recently been a decrease in the percentage of
varies with development and is responsible for the SCIs attributed to violence done to children and adoles-
untrainable toddler becoming a model patient during the cents as also has been observed in adults (24,34). Those
early years of school and then a noncompliant adolescent. etiologies of SCI that are unique to the pediatric popula-
In children injured prior to puberty, SCI can affect tion include lap-belt injuries, child abuse (3540), birth
growth, an example being the failure of paralyzed limbs injuries, Down syndrome, skeletal dysplasias, juvenile
to grow normally. Impaired mobility associated with a rheumatoid arthritis, C1C2 subluxation caused by ton-
SCI limits the ability of children and adolescents to sillophayrngitis (41), and transverse myelitis (42,43).
explore their environment in a developmentally appro- Lap-belt injuries most commonly affect children
priate fashion, thus impairing psychosocial, educational, weighing between 40 and 60 pounds, because the lap belt
and vocational development. rises above the pelvic brim and acts as an anterior ful-
crum, resulting in flexion/distraction forces in the mid-
lumbar spine (4447). The three major components of
Epidemiology
lap-belt injuries are abdominal wall bruising, intra-
Approximately 3 to 5 percent of the SCIs that occur each abdominal injuries, and spinal cord damage. The abdom-
year in the United States occur in individuals younger inal wall bruising is caused by trauma from the lap belt
than 15 years of age and about 20 percent occur in those and ranges from abrasions to full-thickness skin loss. The
SPINAL CORD DISORDERS IN CHILDREN AND ADOLESCENTS 853
most frequent abdominal injuries are perforations or tears Neonatal SCIs occur in approximately one per
of the small or large intestines, with injuries occurring less 60,000 births, and most commonly result in upper cervi-
frequently to the kidneys, liver, spleen, pancreas, blad- cal lesions related to torsional forces during delivery
der, or uterus. Although the injury forces of a lap-belt (5062). In contrast, SCIs associated with breech deliv-
injury are concentrated at the mid-lumbar spine, the neu- eries are related to traction forces and most commonly
rologic level varies from mid-thoracic to the conus or involve the lower cervical or upper thoracic spine. The
cauda equina. The most common location for vertebral least-common types of neonatal SCIs are thoracic or lum-
damage is between L2 and L4 (Figure 63.1): however, 23 bar lesions, resulting from vascular occlusion associated
to 30 percent of children with lap-belt injuries have SCI- with umbilical artery catheters or paradoxical air
WORA. In order to decrease the risk of lap-belt injuries embolism through transitional cardiovascular shunts.
in children who weigh more than 40 pounds and who are Associated findings include hypoxic encephalopathy and
4 to 8 years of age, approved belt-positioning booster brachial plexus or phrenic nerve injuries. Affected
seats should be utilized (48,49). neonates generally present with a flaccid type of paraly-
sis; the differential diagnosis includes spinal muscular
atrophy, congenital myotonic dystrophy, amyotonia con-
genita, and neural tube defects.
Approximately 15 to 20 percent of individuals with
Down syndrome demonstrate atlantoaxial instability,
which is related to ligamentous laxity (6366). An
atlantodens interval (ADI) greater than 4.5 mm is con-
sidered abnormal. The majority of children with
atlantoaxial instability are asymptomatic. In general, only
symptomatic patients require surgical intervention with
a C1C2 fusion. Restricting high-risk activities in asymp-
tomatic patients with an increased ADI is controversial.
Additionally, children with Down syndrome commonly
demonstrate occipitalatlanto instability, which must be
carefully assessed (64).
Children and adolescents with polyarticular juvenile
rheumatoid arthritis (JRA) may experience fusion of the
cervical vertebra, particularly C2C3. This may progress
to fusion of a significant portion of the cervical spine,
placing the patient at risk for a cervical fracture and pos-
sible tetraplegic SCI (67). In addition, individuals with
JRA may develop instability at C1C2 because of syn-
ovitis of the facet and synovial joints surrounding the
odontoid process or from destruction of the odontoid
process as a result of the inflammatory process (68).
Skeletal dysplasias, such as achondroplasia and
Morquio syndrome, may be associated with a cervical
myelopathy (6971). Infants with achondroplasia may
have a small foramen magnum, resulting in compression
of the upper cervical cord and the caudal medulla (70).
Individuals with achondroplasia, primarily males, are also
at risk of developing spastic paraplegia because of spinal
stenosis (69). Children with dwarfing syndromes with
odontoid dysplasia, such as Morquios mucopolysaccha-
ridosis IV, may develop atlantoaxial instability. Myelopa-
thy may develop in more than 50 percent of those with
instability (69,71).
FIGURE 63.1
Pathophysiology
Spine radiographs of a 16-year-old male who sustained a
complete T7 spinal cord injury as a result of a lap-belt injury. Anatomic and physiologic characteristics unique to pre-
The radiographs demonstrate L3L4 fracture-dislocation. pubertal children 10 years of age and younger are respon-
854 SPECIAL TOPICS IN SPINAL CORD MEDICINE
units/kg over 10 minutes (5,000 to 10,000 units in this series, five of the patients with hypercalcemia had a
adults), followed by continuous infusion of 28 clinical presentation consistent with an acute abdomen,
units/kg/hour for infants or 20 units/kg/hour for children and two of them underwent exploratory laparotomies.
over 1 year (20,000 to 40,000 units/day in adults) Patients with hypercalcemia may also be asymptomatic.
(88,89). The dose of heparin is adjusted to maintain the Serum calcium is elevated above the normal age-
activated partial thromboplastin time between 60 and 85 adjusted range, which is 10.8 mg/dl in children and 10.2
seconds. Oral anticoagulation with warfarin sodium, 0.2 mg/dl in adolescents. In addition, ionized calcium is ele-
mg/kg, is started concurrently with heparinization. vated above its upper limit of 1.23 mmole/L. Serum phos-
Dosage adjustments are made to maintain a prothrombin phorus is normal, and alkaline phosphatase is either nor-
time of 2 to 3 international normalized ratio (INR). An mal or slightly elevated above age-appropriate norms.
alternative approach to managing DVT is low molecular Parathyroid hormone is usually depressed because of the
weight heparin (1 mg/kg q12 hours subcutaneously). hypercalcemia.
DVT prophylaxis includes anticoagulation and The management of hypercalcemia includes hydra-
graduated elastic stockings for older children and ado- tion, which may require intravenous normal saline, and
lescents. For younger children who cannot wear com- furosemide (Lasix 0.52 mg/kg/day Q612H) to facili-
mercially available graduated elastic stockings, custom- tate renal excretion of calcium (96). Pamidronate is effi-
made lower extremity garments, such as Jobst stockings, cacious in managing hypercalcemia (18,97100). It is
may be a consideration. Elastic wraps to wrap the legs administered intravenously at a dose of 1mg/kg, with a
should not be used because the unevenness of wrapping usual adult dose of 60 mg administered over 4 hours. A
may result in constrictions with venous obstruction, thus single dose of pamidronate is usually effective in resolv-
increasing the risk of DVT. In addition, some elastic ing the hypercalcemia.
wraps contain latex, which is contraindicated because of The complications of hypercalcemia include nephro-
the risk of latex allergy in the SCI population. Alterna- calcinosis, urolithiasis, and renal failure. In the series
tives for prophylactic anticoagulation are the same as for reported by Tori and Hill, ten of their eighteen (55 per-
adults, except that low molecular weight heparin is not cent) pediatric patients with hypercalcemia experienced
specifically approved for use in children. However, it is urinary stones, compared to an 18 percent incidence of
common practice to utilize low molecular weight heparin stones in patients without hypercalcemia (94). Addition-
in this situation because of the ease of administration and ally, two of their eighteen patients developed renal failure
the fact that laboratory monitoring is not needed. The and nephrocalcinosis.
dosage of low molecular heparin is 0.5 mg/kg adminis-
tered subcutaneously every 12 hours (0.75 mg/kg for
Autonomic Dysreflexia
infants younger than 2 months) (90,91).
The pathophysiology, clinical manifestations, and man-
agement of autonomic dysreflexia in children and ado-
Hypercalcemia
lescents with SCI is comparable to the adult SCI popula-
Hypercalcemia most commonly involves adolescent and tion (101,102). Differences between the pediatric and
young adult males, usually during the first 3 months after adult SCI population include developmental variations of
injury (9295). Hypercalcemia affects 10 to 23 percent of blood pressure in children and adolescents, different
individuals with SCI. Hypercalcemia presumably occurs blood pressure cuff sizes for children, and the ability of
as a result of increased bone resorption as a consequence children to communicate.
of the immobilization associated with SCI. The increased For children and adolescents, blood pressure varies
incidence of hypercalcemia in the pediatric SCI popula- with age and body size. Normal blood pressure increases
tion is caused by the increased bone turnover in growing as children grow older, with older adolescents reaching
children and adolescents and their large and active bone adult norms (103). For children and adolescents without
mass, particularly in adolescent males. Because hyper- SCIs, median systolic blood pressure can be estimated by
calcemia depresses renal function, the excessive calcium the formula: 90 mm Hg (2 age in years) (104). Sim-
load is not adequately excreted by the kidneys, resulting ilar to adults with SCI, children and adolescents with cer-
in decreased calcium excretion and an impairment of vical and upper thoracic SCI have lower baseline blood
renal concentrating ability. pressures compared to individuals without SCIs. In view
Patients with hypercalcemia typically present with of the lower blood pressures in children and adolescents
the insidious onset of abdominal pain, nausea, vomiting, with SCIs, as a consequence of both age and neurolgical
malaise, lethargy, polyuria, polydipsia, and dehydration. level, it is important that baseline blood pressures be
Patients may also exhibit behavioral changes or an acute determined. Blood pressure elevations of 20 to 40 mm Hg
psychosis. In a series of 87 individuals younger than 16 above baseline should be considered a sign of autonomic
years, 18 (24 percent) experienced hypercalcemia (94). In dysreflexia.
856 SPECIAL TOPICS IN SPINAL CORD MEDICINE
Blood pressure measurement in the pediatric popu- thetic output is a response to noxious stimuli below the
lation is complicated by the need to use appropriately zone of the SCI. Stimuli that may incite hyperhidrosis
sized blood pressure cuffs and the anxiety that children include UTI; urolithiasis (107); post-injury myelopathic
experience with healthcare professionals (105). Anxiety changes, including posttraumatic syringomyelia (110113);
associated with obtaining blood pressures may make it and tethering of the spinal cord at the injury site (114); or
difficult to obtain accurate measurements during baseline it may be unexplained.
determinations, as well as during an episode of autonomic The sympathetics that innervate the sweat glands of
dysreflexia. A calm and reassuring environment for the the face and neck originate from T17, those for the trunk
child or the adolescent and the presence of parents may from T412, and those for the legs from T9L2 (108).
be helpful. This pattern of sympathetic innervation of the sweat
In view of the varying cognitive and verbal com- glands is responsible for excessive sweating in the face and
munication abilities of children as they progress from neck, which are above the zone of injury, as a result of
infancy through adolescence, the symptoms of autonomic noxious stimuli occurring below the zone of injury.
dysreflexia may not be expressed at all or may be com- The treatment of hyperhidrosis should be initiated
municated less clearly compared to adults. As an exam- if it is embarrassing, impairs function, or increases the risk
ple, in preschool-aged children, even though they are ver- of developing pressure ulcers. The management of hyper-
bal, autonomic dysreflexia may present with vague hidrosis should begin with avoidance and alleviation of
symptoms rather than expressed complaints of a pound- precipitating factors. Medications that may be beneficial
ing headache. Medical alert identification should be uti- are those that inhibit sympathetic overactivity, such as
lized and appropriate education must be provided for propantheline (115) or transdermal scopolamine (108).
those adults who are significantly involved in the lives of
children with SCI, such as teachers, school nurses,
Temperature Regulation
coaches, and community-based healthcare providers.
Education about autonomic dysreflexia should include The severity of defects in temperature regulation is related
symptom recognition as well as emergency management. to the level and completeness of the SCI (101,106,116).
Children and adolescents must take increasing respon- Lesions at T6 or above produce a poikilothermic state
sibility for the prevention, diagnosis, and treatment of auto- because the SCI interferes with central control of the
nomic dysreflexia. This should include the consistent wear- major splanchnic sympathetics and voluntary muscles of
ing of medical alert identification, maintaining of an the lower body. The patient is unable to decrease core
information sheet or card about autonomic dysreflexia, and body temperature by vasodilatation and sweating below
taking responsibility for educating healthcare providers or the zone of injury. Similarly, the patient is unable to
other significant adults about its diagnosis and management. increase core temperature by vasoconstriction and shiv-
The management of autonomic dysreflexia in chil- ering below the zone of the SCI. Therefore, this group of
dren and adolescents should be conducted efficiently in patients is at risk of hypothermia or hyperthermia as a
a calm and reassuring atmosphere. Symptomatic mea- result of environmental temperatures or endogenous fac-
sures are generally successful in managing the majority of tors, such as exercise (117). Infants and young children
episodes of autonomic dysreflexia. For those not respon- are especially vulnerable to environmental temperature
sive to conservative measures, nifedipine (Procardia, extremes because of their relatively large surface area and
Adalat, 0.25 mg/kg or 10 mg in adolescents weighing 40 their variable communication, cognitive, and problem-
or more kg) should be administered by chew and swal- solving abilities. In contrast, adolescents with SCI may be
low for those who can follow directions or sublingually susceptible to hypothermia or hyperthermia because of
for younger children and infants. Patients with recurrent their erratic judgment and behavior.
autonomic dysreflexia may be managed with prazosin Rarely, children with SCI are seen who cannot
(Minipress 25150 mcg/kg/24 hours every 6 hours) engage in summertime outdoor activities because their
or terazosin (Hytrin 15 mg daily). inability to sweat causes heat exhaustion. Functional
cooling suits are commercially available for children with
this problem (1).
Hyperhidrosis
Hyperhidrosis is seen primarily in individuals with tetraple-
Fever
gia or upper thoracic paraplegia (106109). In a series of
154 individuals with SCI, 27 percent experienced hyper- Fever is a common occurrence during the first few months
hidrosis (109). The pathogenesis of hyperhidrosis proba- after a SCI, and may pose a diagnostic challenge because
bly involves sympathetic overactivity of the cephalad por- of multiple etiologies and loss of sensation (101,118,119).
tion of the spinal cord immediately below the zone of injury Although fever in the acute SCI period is not unique to chil-
(109). Similar to autonomic dysreflexia, increased sympa- dren with SCIs when compared to adults with SCIs, chil-
SPINAL CORD DISORDERS IN CHILDREN AND ADOLESCENTS 857
dren are more prone to becoming febrile and experienc- modalities, psychologic interventions, and medications
ing higher temperature elevations. The most common (129,130). Physical modalities may include physical ther-
cause of fever is a UTI. Other common causes include apy, hydrotherapy, and transcutaneous electrical neural
DVT, heterotopic ossification, pathologic fractures, pres- stimulation (TENS). Children and parents should be reas-
sure ulcers, surgical site infections, pulmonary disorders, sured that dysesthesia generally resolves within 3 to 6
and epididymitis. Patients with intraabdominal disorders months. Medications primarily used in the pediatric SCI
frequently present with subtle signs and symptoms, which population for dysesthesia include amitriptyline (Elavil
demand a high index of suspicion when a patient presents 0.1 mg/kg/dose at night), carbamazepine (Tegretol
with fever, anorexia, nausea, vomiting, and abdominal dis- 1020 mg/kg/day BID or TID, QID if suspension is
tension (120). Multiple sources of fever are seen in approx- used), and gabapentin (Neurontin), which can be used
imately 15 percent of febrile individuals, whereas no eti- in children older than 12 years of age (9001,800 mg/day
ology is found for 8 to 11 percent of febrile episodes and TID) (125,131,132). Other medications that may be
may reflect thermoregulatory abnormalities (118,119). beneficial include clonidine (Catapres 5-7 mcg/kg/day
The evaluation of a febrile child with a SCI should Q 612 hours) and phenytoin (Dilantin 35 mg/day
begin with a thorough history and physical examination. QD or BID).
Appropriate laboratory and imaging studies should be
guided by the clinical evaluation. The physical examina-
Latex Allergy
tion must encompass a general evaluation to identify
problems such as otitis media, sinusitis, or pneumonia. Immediate-type allergic reactions to latex have been iden-
The evaluation must also be tailored to SCI-specific prob- tified with increasing frequency over the past decade
lems, such as the identification of a swollen scrotum (133135). Those populations at greatest risk of latex
caused by epididymitis or a swollen extremity with lim- allergy include children with myelomenigocele, SCI, and
ited range, consistent with heterotopic ossification or a congenital genitourinary anomalies, and healthcare work-
pathologic fracture. ers (101,136,137). Approximately 6 to 18 percent of chil-
Laboratory and imaging studies should be guided by dren and adolescents with SCIs are allergic to latex (137).
clinical findings, but generally include a urine analysis and In one report of adults with SCIs who had chronic
culture, a complete blood count with differential, ery- indwelling catheters, seven of fifteen (47 percent) had evi-
throcyte sedimentation rate, and C-reactive protein. Liver dence of latex allergy (138).
function tests, serum amylase and lipase, plain abdomi- Latex allergy probably results from frequent and
nal radiographs, abdominal and pelvic ultrasound, gal- extensive contact with latex-containing products, espe-
lium scan, and CT may be helpful in evaluating the cially medical supplies and equipment. Additional risk
patient for potential abdominal disorders. factors include young age at initial exposure and longer
duration of exposure to latex-containing products. Aller-
gic reactions may be elicited by direct contact with latex
Pain
via cutaneous, mucosal, serosal, or intravenous routes or
Chronic pain is a significant problem among children and by airborne dissemination of latex antigens that adhere
adolescents with SCI (121,122). Pain may be very dis- to glove powder.
abling and negatively affect school, work, and social Latex allergic reactions may manifest as localized or
interactions. Pain may be radicular and originate from the generalized urticaria, wheezing, angioedema, or anaphy-
area of trauma because of the compression of a nerve root laxis. Allergic reactions to latex that occur intraopera-
at the level of injury. Pain may also result from mechan- tively may be life threatening, and may be difficult to diag-
ical instability of an unhealed fracture or may represent nose because of surgical drapes covering the patients skin,
central pain or dysesthesia (122125). The evaluation of which may mask the presence of urticaria.
pain in infants and younger children is complicated by The diagnosis of latex allergy is made by a history
their variable communication abilities. consistent with an immediate-type allergic reaction or
Self-abusive behavior, or self-mutilation, is occa- with in vitro assays or skin tests. Children are considered
sionally seen in individuals of all ages with SCIs, and may to be allergic to latex if they have a consistent history of
be a manifestation of dysesthesia (126128). The most reacting to latex or a positive laboratory or skin test. Clin-
common presentation of self-abusive behavior is bitten ical manifestations are frequently subtle, such as the child
fingertips, which can result in finger amputations. One of who develops a blotchy rash on his face when he blows
the authors has cared for a young boy with C8 tetraple- up a balloon. Latex allergy should be suspected in indi-
gia who presented with chronic trauma and destruction viduals who have unexplained intraoperative allergic
of his nipples, resulting from constant picking. reactions, or in individuals allergic to kiwi, bananas, avo-
The management of dysesthesia, including the fac- cados, or chestnuts (139). Although skin tests are prob-
tors of self-mutilation, should incorporate physical ably the most sensitive method of identifying latex allergy,
858 SPECIAL TOPICS IN SPINAL CORD MEDICINE
the routine use of skin tests is limited by the lack of avail- muscle strength and endurance should be key objectives
ability of standardized preparations and the potential for of an exercise program (152). Exercise programs should
initiating a severe allergic reaction (135). be developmentally based, compatible with age-appro-
In view of the potential severity of latex allergy, indi- priate and contemporary activities, consistent with pre-
viduals at risk, such as those with SCIs or myelomeningo- injury interests, and incorporated into family and com-
cele, should be cared for in a latex-free environment. This munity activities (149,150). Exercise programs should
should minimize the risk of sensitizing patients, in addi- promote independence, be integrated into the childs or
tion to preventing allergic reactions both in patients with adolescents lifestyle and routine, and, most important,
known, as well as in those with undiagnosed, latex aller- be fun.
gies. Patients, their families, and caregivers should be edu- Children and adolescents with SCIs should be
cated about the potential for latex allergy and the neces- assessed for their risk of cardiovascular disorders, which
sity to avoid all latex-containing products. Individuals includes factors such as obesity, smoking, sedentary
allergic to latex should wear a medical alert identification lifestyle, hyperlipidemia, hypertension, and family history.
and carry autoinjectable epinephrine. Screening for hyperlipidemia after 2 years of age should
be pursued in children with a high-risk family history.
Cardiovascular
Pulmonary
Cardiovascular disorders, including hypertension and
coronary artery and cerebrovascular diseases are major Pulmonary complications are important problems during
causes of morbidity and mortality in adults with SCIs both the acute and chronic phase of SCI for children with
(140). The increased risk of cardiovascular disease in indi- SCIs (18,140,153155). Children with high cervical
viduals with SCIs may be a result of their sedentary injuries generally require lifelong ventilatory support,
lifestyles, which is related to volitional motor impair- phrenic nerve pacing, or both (156158). Children who
ments (141). Individuals with SCI should be encouraged are candidates for phrenic nerve pacing are those with C3
to adopt a lifestyle and pursue preventive measures that or higher lesions (159,160). Bilateral phrenic nerve stim-
reduce their risk of cardiovascular complications. Because ulation is usually performed in children to avoid exces-
of their relatively long life span, it becomes particularly sive mediastinal shifts. In addition, tracheostomies are
important for children and adolescents with SCIs to make needed because of the upper airway obstructions that
adaptations to normally practiced preventive measures, occur in young children during phrenic nerve pacing.
such as exercise, diet, stress reduction, and avoidance of Children may experience failure to thrive if they are
tobacco. entirely dependent on phrenic nerve pacing, so that sup-
Exercise is a major element in preventing cardio- plemental nighttime ventilation via a tracheostomy may
vascular complications. A regular exercise program be required (160). Noninvasive ventilation, using systems
becomes a significant challenge in children and adoles- such as biphasic positive airway pressure (BiPAP) and air-
cents with SCIs because of their motor limitations, com- way secretion management, may be applicable in the pedi-
pounded by varying preferences and compliance, which atric SCI population (161,162).
may be limited by their developmental stage (142,143). Infants and young children with tetraplegic SCI may
Because of motivation and size, younger children with be at risk of incipient respiratory failure, which may be
paraplegia may be physically active by crawling and manifested by a sleep-disordered breathing disturbance
ambulating with a variety of orthotics. With this excep- with sleep apnea, snoring, restless sleep, morning confu-
tion, the pediatric SCI population shares significant lim- sion, daytime sleeping, headache, and mental dullness
itations of exercise options with the adult SCI population. (155,163165). A high index of suspicion must be main-
Children and adolescents with SCIs, especially those with tained in these young children with tetraplegia, and sleep
cervical and upper thoracic lesions, may exhibit decreased studies should be ordered if the child demonstrates any
cardiovascular adaptions to exercise, which may be man- symptoms of a sleep-disordered breathing disturbance.
ifested by decreased cardiac output, exertional hypoten- Risk factors for sleep-induced respiratory failure include
sion, and hyperthermia (117,144-147). In additional, diaphragmatic and intercostal muscle paralysis, obesity,
children and adolescents must take special precautions and use of medications such as baclofen or diazepam
when exercising; one of the authors patients, with a T1 (164).
level injury, died of a crush injury and suffocation at home
during unsupervised weight lifting.
Urology
Various exercise programs are available for children
and adolescents with SCIs, such as adapted physical edu- Intermittent catheterization is the standard management
cation and therapeutic recreational activities (148151). of the neurogenic bladder in children and adolescents
Cardiovascular fitness and increased aerobic capacity, with SCIs (12,166173). Clean intermittent catheteriza-
SPINAL CORD DISORDERS IN CHILDREN AND ADOLESCENTS 859
tion is initiated when the child is approximately 3 years who are incontinent despite medications (190194). The
old, or earlier if the child is experiencing recurrent UTIs Vocare system is most appropriate for patients who can
or exhibiting renal impairment. Self-catheterization is ini- independently transfer to a toilet and pull their pants
tiated in children with adequate hand function when they down. Candidates for the Vocare system must have a
are developmentally 5 to 7 years old (171). spastic bladder.
The utilization of prophylactic antibiotics and the
treatment of asymptomatic bacteriuria in the pediatric
Bowel Management
SCI population are similar to that of adults with SCIs
(174). Prophylactic antibiotics should not be routinely The crucial issues for bowel management in children and
used. They should be limited to patients who experience adolescents with SCIs include complete and regular emp-
recurrent and severe UTIs and in those with obstructive tying, continence, short duration of the bowel program,
uropathy or compromised renal function, including aesthetics, and prevention of complications (173,195200).
hydronephrosis and vesicoureteral reflux. Asymptomatic The necessity for regularity in performing bowel programs
bacteriuria is generally not treated unless the patient has often conflicts with the lack of conformity of children and
compromised renal function. Treatment should be lim- adolescents. However, the anxiety associated with the
ited to those with symptomatic UTIs, as manifested by potential for fecal incontinence is a strong incentive for
systemic toxicity (fever, chills, dysreflexia, or exacerba- compliance with bowel program scheduling. Bowel pro-
tion of spasticity), incontinence, or cloudy and foul- grams are begun when children are 2 to 4 years of age,
smelling urine. Usage of fluoroquinolones should be lim- which is a developmentally appropriate age, or earlier if
ited in children younger than 18 years of age because of they are experiencing diarrhea or constipation (201).
the potential of cartilage damage (175). However, fluo- The fundamental components of a bowel program
roquinolones may be used in children and young adoles- include independence, privacy, and regularity with respect
cents with SCIs if alternative safe and effective antimi- to frequency and time of day. The bowel program should
crobials are not available (176). take place on a toilet or a commode. A sitting position
Continence and independence are crucial aspects of facilitates defecation, and if neurologically capable, the
bladder management of children and adolescents with child should be taught and encouraged to increase intra-
SCIs. The management of incontinence may include anti- abdominal pressure. It is important that these key prin-
cholinergics, modification of fluid intake and catheteri- ciples be instituted when a bowel program is initiated,
zation schedule, and treatment of urologic complications, irrespective of the age of the child or adolescent.
such as UTIs and urolithiasis. Urodynamics should be per- For children and adolescents who do not respond to
formed in patients with persistent incontinence (170,177). standard bowel program interventions, options include
Children and adolescents with limited bladder capacity the antegrade continence enema (ACE procedure); enema
unresponsive to anticholinergics may be candidates for a continence catheters, pulsed irrigation enemas (202); and
bladder augmentation (172,178182). the Vocare system (203). With the antegrade continence
Continent catheterizable conduits and functional enema procedure, antegrade evacuation of the bowel is
electrical stimulation (FES) are alternatives for individu- stimulated by administering an enema directly into the
als who are not independent in performing intermittent cecum. This is delivered into the cecum via the appen-
catheterization (172,182,183). A continent catheterizable dix, which is accessible through an abdominal wall stoma
conduit, known as the Mitrofanoff procedure, consists (204). The ACE procedure has been reported to be use-
of creating a catheterizable conduit using the appendix ful in improving continence and decreasing constipation
or a segment of small bowel, which is used to connect the (205).
bladder to a stoma, either on the lower abdominal wall
or in the umbilicus (184,185). Continent catheterizable
Spasticity
conduits allow individuals with limited upper extremity
function, such as those with C6 or C7 injuries, to Children with SCIs are less likely to demonstrate spas-
catheterize themselves (186189). Additionally, continent ticity compared to the adult SCI population. This may
catheterizable conduits may promote independence in be a reflection of the higher incidence of parpaplegia in
bladder management for individuals who have difficulty children with SCIs compared to adults (206). Neverthe-
accessing their urethra, such as females who have diffi- less, spasticity is an important problem for a significant
culty transferring to a commode or toilet or those who number of children with SCIs (207209). General man-
cannot actively abduct their legs. agement principles of spasticity in children with SCIs are
The Vocare system, a FES system that assists in no different from those in the adult SCI population (206).
both bladder and bowel management, may be beneficial Evaluation should include a thorough history and phys-
for individuals with SCIs who have recurrent UTIs or ical examination with attention directed to potential incit-
abnormal sensation related to catheterization, and those ing factors. Factors that perpetuate or exacerbate spas-
860 SPECIAL TOPICS IN SPINAL CORD MEDICINE
ticity include noxious stimuli below the zone of injury, For spasticity that does not respond to standard
which are frequently clinically inapparent. Hence, a high management, options include intrathecal baclofen , selec-
index of suspicion and a thorough evaluation are neces- tive dorsal root rhizotomies, epidural spinal cord stimu-
sary, particularly in view of the age-dependent variabil- lation, and localized injection of botulinum toxin
ity in the ability to communicate. Hip subluxation or dis- (217222). Baclofen can be administered intrathecally by
location is an example of a noxious stimulus that may an implanted pump, and has been utilized increasingly
exacerbate spasticity and that is more common in the in children and adolescents with SCIs, with encouraging
pediatric SCI population. results (223). Experience with intrathecal baclofen in the
The goals of spasticity management are to improve pediatric population also includes children and adoles-
function, prevent complications, and alleviate pain and cents with cerebral palsy (224). Disadvantages of
embarrassment (206,210). Both the advantages and dis- intrathecal baclofen are cost, for both the initial implan-
advantages of spasticity must be considered when treat- tation and pump refills, and the rare occurrence of seri-
ing spasticity. The major aspects of spasticity management ous adverse reactions (225,226).
include prevention, nonpharmacologic interventions,
medications, and invasive procedures. Prevention is the
Surgical
foundation of any therapeutic program for spasticity, and
comprises the avoidance of precipitating factors and the
Halo Fixators
establishment of good bladder, bowel, and skin programs.
Nonpharmacologic interventions include relief of incit- Proper halo ring application on children is crucial in pre-
ing factors and a program of stretching and range-of- venting pin loosening and pin tract infections. For infants,
motion exercises and positioning. multiple pins (ten in comparison to four in adults) with
Medications should be considered in patients with low torque (2 inch-pounds) have been shown to be safe
spasticity that affects the childs functioning and that is (227). Torque should range from 4 to 6 inch-pounds for
unresponsive to conservative treatment. Baclofen (Liore- children between 2 and 12 years of age and to 8 inch-
sal) administered orally is the initial medication of choice pounds for children older than 12 years of age. CT scan-
and is initiated at 0.125 mg/kg/dose BID to TID (5 mg ning of the skull is recommended for pin placement for
BID to TID in children 12 years and older) (211). Doses children under 6 years of age, because of the variability
are then increased every 3 to 5 days by increments of in skull thickness (228,229). If halo fixation fails, a Min-
0.125 mg/kg/dose (5 mg/dose in children 12 years and erva-type cervicothoracolumbosacral orthosis is an alter-
older). The usual maximum daily dose is 1 to 2 mg/kg/day native. Because use of Crutchfield tongs in patients
administered QID (80 mg/day in children 12 years and younger than 12 years old may result in skull penetra-
older). Although baclofen remains the drug of choice for tion and dural fluid leaks, halo traction is the preferred
managing spasticity in adolescents, the potential for illicit method for this age group.
drug experimentation must also be considered (212).
Other medications that may be beneficial in the man-
Spine Boards
agement of spasticity include diazepam, clonidine, dantro-
lene, gabapentin, and tizanidine. The second drug of choice Because the head is proportionately larger than the rest
is probably diazepam (Valium 0.1 mg/kg/dose QHS to of the body in children younger than 8 to 10 years of age,
QID), which may be used in combination with baclofen or their necks will be inadvertently flexed if they are immo-
as a single drug for patients who do not tolerate baclofen. bilized on a standard spine board (Figure 63.3 A,B).
Clonidine (57 mcg/kg/day Q 612 hours) may be effec- Therefore, when spinal stabilization is needed, younger
tive as a single agent or in combination with other drugs children should be immobilized on child-specific spine
(213). Transdermal administration of clonidine (0.10.3 boards (Figure 63.3C) (230). However, if a standard spine
mg patch weekly) may be used in older children or ado- board must be used for a younger child, excessive cervi-
lescents (214,215). Dantrolene (Dantrium) is generally cal flexion can be avoided by raising the torso 2 to 4 cm,
not used to manage spasticity in children and adolescents leaving the head at the board level (Figure 63.3D).
with SCIs. Although not approved for use for spasticity,
gabapentin (Neurontin 9001,800 mg/day TID for
Spine Deformities
children older than 12 years of age) may assist in control-
ling spasticity. Although tizanidine (Zanaflex) is not Spine deformities are an extremely common problem in
approved for use in pediatrics, it is used by some clini- pediatric SCI, especially if the injury is sustained prior to
cians and demonstrates efficacy similar to that in adults skeletal maturity (231234). (Figure 63.4) For children
with SCIs (216). Because of the potential for hepatotoxi- injured prior to skeletal maturity, 98 percent will develop
city, liver function studies should be performed, especially scoliosis, with 67 percent requiring surgery (233). In con-
during the first 6 months of therapy. trast, for children and adolescents whose injuries occurred
SPINAL CORD DISORDERS IN CHILDREN AND ADOLESCENTS 861
A C
FIGURE 63.3
Figure A depicts an adult immobilized on a standard backboard. Figure B shows a young child on a standard backboard; the rel-
atively large head results in a kyphotic position of the neck. In Figure C, the child is on a modified backboard with a cut-out to
recess the occiput, which provides for safe cervical positioning. In Figure D a double mattress pad raises the chest, providing
safe cervical positioning. Herzenberg JE, Hensinger RN, Dedrick DK, et al. Emergency transport and positioning of young chil-
dren who have an injury of the cervical spine. The standard backboard may be hazardous. J Bone Joint Surg, 1989; 71A:1522.
A B C
FIGURE 63.4
Spine radiographs of a 13-year-old female who sustained an incomplete C4 spinal cord injury (ASIA Impairment Scale score of
C) when she was 8 years old. Figure A demonstrates a 65 degree thoracolumbar curve. Figures B and C are radiographs obtained
after she underwent a posterior spine fusion with instrumentation.
862 SPECIAL TOPICS IN SPINAL CORD MEDICINE
Hip Deformities
Hip subluxation, dislocation, and contractures are fre-
quent complications in children with SCIs, especially if
they were injured at younger ages (Figure 63.5)
(1,235,238,239). In one series, hip instability was
observed in 100 percent of children who were injured
when they were less than 5 years of age and in 83 per-
cent of those injured when they were younger than 10
years of age (1). In another report, hip instability was
found in 60 percent of children injured when they were FIGURE 63.5
8 years of age or younger (240). Hip instability occurs in Pelvic radiographs of a female with incomplete C4 tetraple-
patients regardless of their neurologic level, presence or gia. Radiographs obtained at 10 years of age (Figure A)
absence of spasticity, or their gender (241). demonstrate both hips to be located, with subluxation of the
The indications for surgical management of hip right hip when she was 11 years of age (Figure B). Figure C is
instability are not clear-cut. An aggressive approach to a radiograph obtained after surgical correction of the hip dis-
managing hip instability should be entertained in view location.
SPINAL CORD DISORDERS IN CHILDREN AND ADOLESCENTS 863
of the future applications of the FES systems for upright pathologic fractures, with the etiology of the remaining
mobility and the future possibility for spinal cord regen- fractures not being identified. Children and adolescents
eration (1,242). The management of hip instability may with SCI who develop a pathologic fracture present with
include the surgical release of hip contractures, a capsu- fever and a swollen extremity. The supracondylar region
lorrhaphy, varus osteotomies, and anterior or posterior of the femur and the proximal tibia are the most common
acetabular augmentations (235,243). sites of a pathologic fracture. Radiographic abnormali-
Because of the high incidence of hip instability and ties may initially be subtle, and the diagnosis of a patho-
contractures in children injured when they are younger logic fracture in growing children requires a high index
than 5 years old, bracing from the time of sustaining the of suspicion (235).
SCI should be considered. Prophylactic bracing could The treatment of pathologic fractures in children
involve an abduction-flexion orthosis with free, unre- with SCIs should consist of removable splints
stricted flexion, maintaining 20 degrees of abduction (1,235,253). If casts are necessary, they must be well
bilaterally. padded over all bony prominences and bivalved to enable
inspection to prevent pressure ulcers. Because of osteo-
porosis, internal or external fixation generally may not
Heterotopic Ossification
hold very well. Fortunately, exuberant callus usually
An accurate incidence of heterotopic ossification (HO) in develops within 3 to 4 weeks, at which time splinting or
pediatric SCI is not known but may be approximately 3 casting can be discontinued with resumption of range-
percent, in contrast to a 20 percent incidence in adults of-motion exercises. However, ambulation should be
with SCI (235,244). The hip is most commonly involved postponed for 6 to 8 weeks after sustaining a fracture.
in children and adolescents with SCIs. In pediatric SCI, Prevention is critical, but is especially challenging
HO begins on average 14 months after injury, in contrast in the pediatric SCI population because of the risk-tak-
to adult onset within 1 to 4 months (245). Etidronate dis- ing behaviors characteristic of children and adolescents.
odium (Didronel) to prevent HO is not routinely used Prevention must focus on safety in risky activities. In addi-
in the pediatric SCI population because of the relatively tion, bone mineral loss should be minimized by encour-
low risk of HO (246). In addition, the use of etidronate aging weight bearing with orthotics or FES. Good nutri-
disodium should be limited in children prior to puberty tion and adequate sunlight are also essential. Appropriate
because of the possible development of a rachitic-like syn- training and adequate equipment for transfers are essen-
drome (247249). Surgery is indicated if there are signif- tial components of pediatric SCI rehabilitation. In the
icant functional deficits. Resection of HO should be future, bisphosphonates such as alendronate may have a
undertaken 1 to 1.5 years after its onset, to avoid the pos- role in preventing pathologic fractures.
sible progression of femoral neck osteoporosis and
intraarticular fibrosis if surgery is postponed until the
Pressure Ulcers
bone scan and alkaline phosphatase are normal
(244,250). The postoperative use of radiation therapy Pressure ulcers are one of the most common complica-
may be contraindicated in younger children because of tions for children and adolescents with SCI (254,255).
the long-term consequences of radiation. However, The incidence of pressure ulcers in the pediatric SCI pop-
indomethacin (Indocin 13 mg/kg/day TID or QID, ulations is not known. In a retrospective study of people
maximum dose of 200 mg/day) is used in the postopera- who sustained their SCI when they were less than 13 years
tive period (251). of age, 55 percent developed at least one pressure ulcer
during a mean follow-up period of 10.3 years (254). The
peak age for pressure ulcer development was 8 years of
Osteopenia and Pathologic Fractures
age. Issues unique to the pediatric population include
The onset of osteopenia begins soon after sustaining a SCI variable compliance in both preventive and therapeutic
and reaches a plateau 6 to 12 months after sustaining a endeavors because of the different developmental stages
SCI. Children and adolescents with SCI have bone den- of children and adolescents (254,256). Toddlers and
sities that are 60 percent of normal age- and sex-matched younger children may be at risk of developing pressure
controls (252). A combination of standing, stepping, and ulcers because of inadvertent trauma from the careless
FES may increase bone mineral density by approximately activities and play typical in these age groups. Younger
25 percent. children have limited cognitive abilities to comply with
Pathologic long bone fractures, as a consequence of the usual preventive measures that older individuals fol-
loss of bone mineral density, occur in approximately 14 low, such as pressure relief. Preventive measures may
percent of children and adolescents with SCI (1,235). Gait include wristwatches with automatic resetting timers to
training, range-of-motion exercises, and minor trauma remind children to perform pressure relief. Additionally,
are responsible for approximately 40 percent of the preventive interventions should be developmentally
864 SPECIAL TOPICS IN SPINAL CORD MEDICINE
based, with responsibility gradually shifted from the par- or weak C6 tetraplegia, who would otherwise not be can-
ents to the children as they grow up. As children grow didates for reconstructive surgeries (270,271). Similar to
up and become physically larger, new equipment must adults with SCI, the Freehand System in adolescents
be matched to their size. Properly fitting wheelchairs and results in increased independence and improved satisfac-
adequate cushions must be prescribed, and pressure map- tion (267,272). Findings in animal studies, and now FDA
ping should be performed to reduce the risk of pressure clinical trials in humans, indicate that children as young
ulcers. as 6 years of age may benefit from this technology (273).
Rehabilitation Ambulation
Pediatric SCI rehabilitation must be developmentally The ability to ambulate depends on a variety of factors,
based, and goals must be responsive to the dynamic including age, body size, neurologic level and degree of
changes that occur as children and adolescents grow up completeness, compliance, and preferences. Children who
(1,257264). The objectives of rehabilitation are to main- are most likely to be community ambulators are those
tain health and restore productivity, with the ultimate who are young, have L3 or lower lesions, or have an
goal that the patient experiences a satisfying life. A major American Spinal Injury Association (ASIA) impairment
challenge in providing care for children and adolescents scale score of D (274,275). Compared to adolescents and
with SCI is to address the changing needs at each devel- adults, children tend to be more active ambulators
opmental stage, with the ultimate goal being that the because of their size, increased energy level, and reduced
patient becomes an adult with a high quality of life (265). concern for cosmesis (275-280).
Standard interventions should encompass mobility; Parapodia permit children with SCIs to stand with-
activities of daily living; bowel, bladder, and skin pro- out the need for upper extremity weight bearing, and
grams; recreation; social services; and psychologic and allow them to perform activities with both hands
vocational counseling. Conventional rehabilitation must (275,281). Children are also able to ambulate with para-
be broadened to incorporate effective mobility and access podia. The basic requirements to utilize parapodia include
in the community and educational, vocational, and recre- head control and the absence of significant contractures
ational interventions that promote a productive and sat- of the lower extremities. In general, patients who utilize
isfying life. As children and adolescents grow up, they parapodia are either therapeutic or household ambula-
need new equipment because of increasing size and chang- tors. However, parapodia provide children with some
ing needs. Using mobility as an example, infants and independence in mobility and an opportunity to be
young toddlers may crawl, stand, and ambulate in para- upright and face their peers at eye level.
podia, and use strollers for wheeled mobility. Although Children can begin using parapodia as young as 9 to
young school-aged children may crawl at home, they use 12 months of age, which is a developmentally appropriate
a variety of orthotics for ambulation or standing in time to start standing and walking. Parapodia may also be
school, and they should become independent users of helpful in giving the young child an opportunity to be
their wheelchairs. Older children and adolescents pri- upright, prior to initiating other orthotics. Another advan-
marily utilize wheelchairs for community mobility, with tage of parapodia is that they do not require intensive ther-
older adolescents needing access to motor vehicles for apy. Parapodia are generally well accepted by preschool-
community mobility. ers and early school-aged children, but children tend to stop
using parapodia by the time they are 7 to 10 years of age.
Rochester parapodia and swivel walkers are two of
Upper Extremity Function
the more commonly used parapodia. Rochester parapo-
Although hand function in children with tetraplegia may dia have hip and knee joints that facilitate donning and
be improved with a variety of static and dynamic doffing, allow the patient to sit while wearing the brace,
orthotics, they generally stop using these braces because and may facilitate transferring from the floor or chair to
of cosmesis, the added stigma, and the burden of carry- standing (Figure 63.6) (281,282). Children can ambulate
ing additional equipment (266,267). In contrast, surgi- with the Rochester parapodia by swiveling, produced by
cal reconstruction, including tendon transfers of the upper twisting their upper trunks and swinging their arms. They
extremities to restore hand function, is beneficial for chil- may also choose to perform a swing-to or a swing-
dren and adolescents with SCI (268,269). The usual through gait utilizing an assistive device, such as a walker
objectives of surgical reconstruction of the upper extrem- or forearm crutches.
ities are to restore elbow extension, wrist extension, fin- The Orthotic Research and Locomotion Assessment
ger flexion, and thumb pinch. Unit (ORLAU) swivel walker permits a child or adoles-
Implantable FES systems have been successfully uti- cent with a SCI to ambulate with a reciprocal-like move-
lized to restore grasp and release in adolescents with C5 ment of paired foot plates (Figure 63.7) (283). The main
SPINAL CORD DISORDERS IN CHILDREN AND ADOLESCENTS 865
A B
FIGURE 63.6
advantages of the ORLAU swivel walker are the recip- Standing may have extensive functional and psy-
rocal-like gait, the plastic chute that facilitates donning, chologic benefits for children with SCI (280,285). In addi-
and the sturdy construction that allows use by adoles- tion to parapodia, there are a number of static and mobile
cents. However, an individual wearing an ORLAU swivel standing devices that are suitable for children, including
walker cannot sit because it does not have any joints. standing wheelchairs, standing frames, and mobile stand-
A third orthosis that is more commonly used in chil- ing devices (280). Because of their size, standing wheel-
dren or adolescents with SCI is the reciprocating gait chairs are only appropriate for older children and ado-
orthosis (RGO), which is generally utilized by individu- lescents. The Standing Danny (Figure 63.9) and the Rifton
als with upper lumbar or thoracic paraplegia (Figure standing walker (Figure 63.10) are two examples of
63.8) (275,280,281). In comparison to hipkneeankle mobile standing devices. Mobile standing devices are
foot orthoses (HKAFO) or kneeanklefoot orthoses most appropriate for preschool-aged to pre-adolescent
(KAFO), which may also be utilized in these types of children, and they are primarily used for household or
patients, RGOs provide a reciprocating gait and are more school activities.
energy efficient (284). Children as young as 15 to 18 FES systems are another option for upright mobil-
months old can begin to use RGOs. Children with active ity in children, and these have been demonstrated to be
hip flexors, or who are young and motivated, are most feasible and practical (1,286). Using an implanted FES
likely to be community ambulators, although the major- system, adolescents were able to stand at home 2 to 4
ity of RGO users will primarily be therapeutic or house- times a week (287). They were able to perform common
hold ambulators (280,281). activities while standing, which included reaching high
866 SPECIAL TOPICS IN SPINAL CORD MEDICINE
A B
FIGURE 63.7
The ORLAU (Orthotic Research and Locomotion Assessment Unit) swivel walker; front (A) and back (B) views.
places and exercising. In another clinical trial, an dimensions of sexuality must be addressed with children
implanted FES system was compared to KAFOs (288). and adolescents with SCIs and their families. These
Compared to KAFOs, the FES system was equal or bet- aspects of sexuality include the general sexuality issues
ter in promoting independence and was preferred for the common to all children and adolescents, sexuality issues
majority of activities. Hip dislocation, lower extremity common to children with special needs, and the SCI-spe-
contractures, severe scoliosis, and myocutaneous flaps cific sexuality issues (289291). From the onset of the
that have been performed for pressure ulcers are con- SCI, patients and parents must be educated in a prospec-
traindications to FES for upright mobility. It is therefore tive and optimistic manner about future sexuality issues,
critical to prevent these complications in children and including fertility. As children and adolescents grow, sex-
adolescents with SCIs, who may benefit one day from uality education and counseling must be provided in a
innovative treatments such as FES. developmentally based fashion. Additionally, sexuality
counseling must be progressively addressed directly to
older children and adolescents without parents being pre-
Psychosocial Issues and Sexuality
sent. Females who have sustained SCI and their parents
Sexuality is an often-overlooked aspect of caring for chil- should be reassured that the SCI will result in minor or
dren with special needs. This is particularly critical no abnormalities or delays in onset or resumption of men-
because children and adolescents with disabilities tend struation (292).
to be infantalized or treated as asexual beings. Several
SPINAL CORD DISORDERS IN CHILDREN AND ADOLESCENTS 867
A B
FIGURE 63.8
Reciprocating gait orthosis (RGO); front (A) and back (B) views.
Attendant Care
Although family members may be available to provide
daily care, attendant care should be given consideration
for children with tetraplegia. Attendant care assists par-
ents to maintain their parental role, which includes limit-
setting and support and guidance for their children. To
perform the roles of both parent and caregiver is a major
challenge for most parents. To avoid burning out par-
ents, respite care is essential, particularly if their children
have high tetraplegia with complicated and intense
needs.
Recreation
Play and recreation should be an integral aspect of the
rehabilitation program for children and adolescents with
SCIs (149151). The pre-injury interests and experiences
FIGURE 63.9 of the child or adolescent should provide direction to play
and recreation-based therapy. Play is essential for young
The standing Danny mobile standing device. children during rehabilitation because play is their pri-
mary activity of daily living. Play techniques must be
incorporated into the rehabilitation program. Recreation
and play afford older children and adolescents the nec-
essary break from more traditional rehabilitation. Older
children and adolescents must be provided appropriate
outlets and access to typical age-appropriate activities,
such as sports, television, movies, music, and talk. Simi-
larly, they need knowledge and exposure to community
activities and wheelchair sports.
Substance Abuse
As with the adult SCI population, substance abuse and
psychologic issues are major problems for children and
adolescents with SCIs (296). Adolescents with SCI may
be at greater risk of suicide because of the tremendous
impact of a SCI, superimposed on the usual turbulence
of adolescence.
NEURAL-TUBE DEFECTS
defect, damage to the neural plate during its surgical repair, present with decreased upper extremity function, pro-
toxicity of amniotic fluid, and mechanical trauma from gressive scoliosis, neck pain, and depressed respiratory
the uterine wall during gestation, labor, and delivery function. Shunting of the hydrocephalus usually results
(327,328). in the resolution of the symptoms related to a Chiari II
malformation; however, some patients may require a pos-
terior fossa decompression (324,344).
Clinical Manifestations and Management
Hydrosyringomyelia in association with the Chiari
Surgical repair of the myelomeningocele should be under- II malformation is a relatively common occurrence in
taken shortly after birth to reduce the risk of infection and individuals with myelomeningocele (324,344). The clin-
ventriculitis (329). Closure should be undertaken within ical manifestations are similar to those of development
the first 24 hours of life, if the myelomeningocele sac is hydrosyringomyelia.
leaking; otherwise, the closure can be performed within Motor paralysis; sensory loss; spasticity; and blad-
the first 2 to 3 days of life (326,329,330). Goals of the der, bowel, and sexual dysfunction are the major mani-
initial closure are to cover the defect with skin, untether festations of spinal cord dysfunction. The extent of the
the spinal cord, and reconstruct the neural tube and dura. neurologic deficit is determined by the location of the
Because of the potential development of hydrocephalus, myelomeningocele. In contrast to spinal cord injuries, in
patients need close monitoring after closure of the which the degree of motor deficits either approximate or
myelomeningocele defect. Hydrocephalus develops in exceed the sensory deficits, children with myelomeningo-
approximately 80 to 90 percent of cases and requires ven- cele generally have sensory deficits that are more severe
triculoperitoneal shunting (299,300,331,332). than the motor deficits. In myelomeningocele, the spinal
The primary manifestations of myelomeningocele cord deficits are more severe on the dorsal aspect of the
are a consequence of the brain and spinal cord abnor- cord, including the posterior spinal nerve roots, with rel-
malities. Individuals with myelomeningocele demonstrate ative sparing of the anterior spinal nerve roots. In the
a variety of cognitive defects (333). Children who have absence of normal sensation, the function of muscles
experienced meningitis or ventriculitis are more likely to under voluntary control is limited. Most patients with
have cognitive abnormalities (334). Approximately 30 myelomeningocele have a flaccid type of paralysis, with
percent of children with myelomeningocele have subnor- approximately 10 to 30 percent of individuals exhibit-
mal intelligence, primarily perceptual motor abnormali- ing spasticity (345,346).
ties with normal verbal skills. Individuals with Various classification systems are used to categorize
myelomeningocele frequently exhibit disorders of spinal cord dysfunction in children with myelomeningo-
visualspatial organization, and may have learning dis- cele, and they are generally used to predict ambulation
abilities, hearing and visual impairments, and seizures (345,347,348). It is imperative that accurate serial docu-
(335). Children with myelomeningocele commonly mentation be performed of motor and sensory levels and
demonstrate defects in coordination and dexterity of hand the presence and degree of spasticity. This is particularly
function (336338). Children with myelomeningocele important for early recognition of complications such as
may manifest cocktail chatter, characterized by exces- hydrocephalus, hydrosyringomyelia, and retethering of
sive talking and superficiality of content (339). the spinal cord.
Because most individuals with myelomeningocele Tethering of the spinal cord at the site of the defect
have hydrocephalus and require ventriculoperitoneal is a major component of the myelomeningocele abnor-
shunts, they are at risk for shunt infections and shunt mal- mality at birth. One of the primary goals of the initial sur-
function. Shunt malfunction in younger children is man- gical correction in the newborn is to untether the spinal
ifested by signs and symptoms of increased intracranial cord. However, there is a tendency for retethering, in
pressure, including nausea, vomiting, and severe which the spinal cord becomes adherent to the
headache. In contrast, symptoms of shunt malfunction myelomeningocele repair site (326). The majority of indi-
in adolescents and young adults may be more subtle. They viduals with myelomeningocele demonstrate retethering
may manifest indolent symptoms of shortened attention of the spinal cord with a low-lying spinal cord (300,349).
span, poor school performance, irritability, intermittent With growth, the retethered spinal cord cannot migrate
headaches, weakness, or worsening scoliosis. cephalad as it normally should. Patients with a retethered
In infants and younger children, symptoms of hind- spinal cord may experience impairment of the remaining
brain dysfunction resulting from the Chiari II malforma- spinal cord function, which may be manifested by the
tion may include feeding and swallowing abnormalities, onset or worsening of weakness, spasticity, sensory loss,
stridor, vocal cord paralysis, weak cry, apnea, sleep-dis- pain, progressive scoliosis, orthopedic deformities in the
ordered breathing (340343); nystagmus, opisthotonus, lower extremities, or changes in bowel or bladder func-
and weakness and spasticity of the upper extremities tion. Because the majority of patients with low-lying
(324). Older children and adolescents more commonly spinal cords are asymptomatic, the decision to surgically
SPINAL CORD DISORDERS IN CHILDREN AND ADOLESCENTS 871
repair the retethered spinal cord must be based on well- Orthopedic Issues
documented clinical changes.
Clinical manifestations and management of the Individuals with myelomeningocele experience a variety
neurogenic bladder and bowel in children with of orthopedic complications, especially disorders of the
myelomeningocele is not significantly different from chil- lower extremities and spine (325,365). Orthopedic defor-
dren and adolescents with SCI. However, individuals with mities may be a consequence of several factors, includ-
myelomeningocele may have congenital anomalies of the ing muscle paralysis, unopposed muscle function, spas-
genitourinary system that require additional monitoring ticity, and congenital malformations (365). Orthopedic
or treatment (350). Males with myelomenigocele are at deformities may be present at birth and be a consequence
increased risk of cryptorchidism (351,352). of paralysis present in utero, affecting the position of the
Once the infant has been stabilized after the fetus. Examples of this include clubfeet, dislocated hips,
myelomeningocele defect has been repaired, urodynamic or extension contractures of the knees (325). Other skele-
testing should be performed during the neonatal period tal deformities, such as congenital vertebral and rib anom-
(353357). Neonates with high bladder pressures (leak alies, present in as many as 15 percent of patients are
point pressure 40 cm H2O) or detrussorsphincter primary defects associated with myelomeningocele. Post-
dyssynergia are at risk of developing urinary tract dete- natally acquired orthopedic complications, such as dis-
rioration, and they should be managed with intermittent located hips, hip and knee contractures, and pathologic
catheterization and anticholinergics. In contrast to indi- fractures are a consequence of the neuromuscular defects
viduals with SCI, those with myelomeningocele have a associated with myelomeningocele. Spine deformities,
neurogenic bladder from conception. This may signifi- such as scoliosis, kyphosis, and lordosis, may be a result
cantly retard bladder growth, particularly in children with of both congenital vertebral anomalies and neuromuscu-
high-pressure, low-volume bladders. Bladder augmenta- lar defects (366). In general, the management of these
tion may be indicated in individuals with myelomeningo- orthopedic complications is complicated, and should be
cele who have inadequate bladder capacity despite anti- provided by clinicians experienced in caring for individ-
cholinergic therapy (357). The standard bladder uals with myelomeningocele. Depending on the neuro-
management of individuals with myelomeningocele is logic level, many children and adolescents with
intermittent catheterization (166,171,167,354,357,358). myelomeningocele will ambulate to varying degrees;
The ability to perform self-catheterization may be limited hence, management of lower extremity deformities
by the visualmotor defects that are frequently present should take this into account.
in patients with myelomeningocele and developmental Hip contractures and dislocation are very common
delays that may also be present (171,359). in children with myelomeningocele, especially those
Bowel incontinence is a major problem for children with thoracic and upper- to mid-lumbar lesions, where
with myelomenigocele, especially those without bulbo- up to 90 percent of patients may be affected (300,367).
cavernosus or anocutaneous reflexes (360). Education, In those with thoracic lesions, hip dislocation occurs
along with regular and consistently timed reflex-triggered because all of the hip musculature is denervated (365).
bowel evacuations, are essential elements of a bowel pro- In contrast, hip dysplasia in individuals with lumbar-
gram (360). For children with myelomenigocele with level lesions results from muscle imbalance, with active
bowel program complications unresponsive to more con- hip flexors and adductors unopposed by hip extensors
servative measures, antegrade continence enemas (204), and abductors. The treatment of hip dysplasia in the
pulsed irrigation enemas (206), enema continence myelomeningocele population is somewhat controver-
catheters (205,361), or biofeedback (362364) are poten- sial, particularly because hip dysplasia is usually not
tial alternatives. painful and may not significantly affect the ability to
Secondary manifestations or complications may be ambulate (368). For patients with thoracic and upper-
apparent at birth and would be considered congenital to mid-lumbar level lesions, treatment of the hip dys-
defects, or they may be acquired postnatally. Examples of plasia is usually limited to those with pelvic obliquity,
congenital defects include clubfeet, dislocated hips, or placing them at a higher risk of developing pressure
extension knee contractures; these are secondary com- ulcers. Children with lower lumbar lesions have good
plications of the primary spinal cord deficit, with result- ambulation potential. Their dislocated hips should be
ing in-utero paralysis. Postnatally acquired secondary surgically corrected by 4 years of age, including both
complications result in significant morbidity and occa- skeletal corrections and muscle transfers, to prevent
sionally mortality. The prevention and early management recurrent dislocation.
of secondary complications are an integral aspect of the Hip contractures are common complications in chil-
overall management of children and adolescents with dren and adolescents with myelomeningocele. Hip con-
myelomeningocele. Meningitis, ventriculitis, tethered tractures exceeding 30 to 40 degrees can hinder ambula-
cord, and shunt malfunction have already been discussed. tion. Treatment of hip contractures must be individualized
872 SPECIAL TOPICS IN SPINAL CORD MEDICINE
and based on the neurologic level, ambulation status, and metaphyseal location, and they may exhibit exuberant
presence of hip dysplasia. For individuals with thoracic callus formation, raising concerns of a tumor. Fractures
and upper lumbar levels, hip contractures are generally usually present with a warm, swollen, and erythematous
managed by surgical releases and aggressive bracing and extremity in a febrile child. The fractures should be man-
physical therapy, postoperatively. For those with mid- to aged with splinting, and weight bearing can be initiated
lower-lumbar lesions, a combination of surgical releases within 2 to 3 weeks to prevent further osteoporosis and
and appropriate tendon transfers is performed. Unfortu- reduce the risk of further fractures (365).
nately, surgical releases are frequently complicated by Scoliosis affects the majority of patients with
recurrences of the hip contractures (300). myelomeningocele and results from a variety of factors,
Extension and flexion contractures and valgus rota- including congenital vertebral anomalies, neuromuscular
tion deformities are the major abnormalities of the knee weakness, pelvic obliquity, and hip contractures
in children with myelomeningocele (300,365). Extension (300,332,366,369). Scoliosis affects almost all patients
contractures of the knee are relatively uncommon and with thoracic-level lesions, with a decreased incidence of
generally are the result of breech deliveries or are seen in scoliosis in patients with lower neurologic levels
patients with mid-lumbar level lesions who have strong (366,370). Progressive scoliosis may be a manifestation
quadriceps without strong knee flexors. Physical therapy of decompensated hydrocephalus, retethered spinal cord,
and splinting are usually successful in managing knee or hydrosyringomyelia (371,372). The correction of
extension contractures; however for those that are resis- hydrocephalus, a retethered spinal cord, or hydrosy-
tant to conservative management, a modified V-Y quadri- ringomyelia may slow or halt progression of scoliosis in
cepsplasty should be performed. patients with spinal curvatures less than 40 degrees, but
Knee flexion contractures greater than 20 degrees generally does not change the course of more severe
may limit ambulation. Children under 2 years of age hav- curves (371373).
ing flexion contractures of their knees may be success- The management of scoliosis must include accurate
fully managed with physical therapy and splinting; oth- monitoring of the motor and sensory examination, deep
erwise surgical correction is indicated. Valgus rotation tendon reflexes of both the upper and lower extremities,
deformities of the knee are a result of iliotibial band tight- and degree of spasticity. Radiographs of the spine are per-
ness and the forces of ambulation. Management includes formed at least annually. Imaging studies to exclude hydo-
muscle transfers, distal iliotibial band sectioning, and cephalus, retethered cord, and hydrosyringomyelia
kneeanklefoot orthoses (KAFOs); and distal femoral should be performed on patients who are experiencing
osteotomies may be necessary if the deformity becomes progressive scoliosis, particularly if accompanied by pro-
fixed. gressive weakness or spasticity. Individuals with curves
Ankle and foot deformities are common in children that are greater than 25 degrees or those with unbalanced
and adolescents with myelomeningocele, and they are curves should wear bivalved molded body jackets when
frequently resistant to conservative measures and usu- they are awake and up sitting, standing, or walking. Par-
ally require surgical correction (300,365). Approxi- ticular care must be taken to prevent the development of
mately 50 percent of patients with myelomeningocele pressure ulcers under the body jacket. The timing of the
have clubfeet, as a result of muscle paralysis and in utero surgical repair and the extent and type of spinal surgery
positioning. These clubfoot deformities tend to be rigid, depends on the childs age (preferably after age 10);
are resistant to casting, and usually require surgical neurologic level, ambulation status, and location and flex-
repair (365). Calcaneovalgus deformities result from ibility of the curve. For children who are ambulators, the
unopposed contraction of the anterior tibial muscle, per- lumbosacral joint generally should not be fused. In addi-
oneal muscles, or the toe extensors, and are most com- tion, hip contractures should be corrected prior to spine
monly seen in patients with low lumbar lesions. The fusions to avoid excessive torque to the postoperative
deformity is usually progressive, resulting in a crouch spine fusion (300).
gait, and predisposing to pressure ulcers from shoe or Eight to 15 percent of patients with myelomeningo-
brace wear. Surgical correction consists of a anterior tib- cele develop kyphosis, particularly those patients with
ial tendon transfer to the os calcis (365). Individuals with thoracic lesions (325,326,369), (Figure 63.11). Kypho-
sacral-level lesions may present with cavus deformities sis can interfere with sitting or wearing orthotics, and may
of the foot, which are frequently accompanied by claw cause a pressure ulcer over the kyphus. Progression of the
toe deformities, predisposing to pressure ulcers under the kyphotic deformity can compromise ventilation, because
toes or metatarsal heads. abdominal contents are pushed up into the thorax. Sur-
Pathologic fractures most commonly involve chil- gical correction of kyphosis is complex and technically
dren 3 to 7 years of age, and they usually occur after cast demanding, and may be associated with significant mor-
immobilization or during skeletal traction (300). These bidity and mortality (326,369). Preoperative evaluation
pathologic fractures generally are in an epiphyseal or of ventriculoperitoneal shunt functioning is critical.
SPINAL CORD DISORDERS IN CHILDREN AND ADOLESCENTS 873
A B
FIGURE 63.13
C
Radiographics of a child with sacral agenesis demonstrating
abrupt loss of the vertebral column at T12-L1 (A and B) and
foot deformities (C).
876 SPECIAL TOPICS IN SPINAL CORD MEDICINE
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64 The Prevention of
Spinal Cord Injury
Of the many forms of disability which can beset to comprehensive SCI management. Advanced by the
mankind, a severe injury or disease of the spinal cord British Medical Research Council and the visionary lead-
undoubtedly constitutes one of the most devastating ership of Sir Ludwig Guttman, this new system of care
calamities in human life. changed forever the outlook and prognosis for the spinal
Sir Ludwig Guttman
cord injured. Beginning with tightly focused initial med-
ical and surgical management, Guttmans continuum of
pinal cord injury (SCI), with its resul- services moved seamlessly into intense, comprehensive
sponsored Model Systems was 144.8 days. Over the next All injuries are divisible into two major groupings:
15 years (1978 to 1992) the average LOS decreased to unintentional and intentional. The former account for
77.5 days, including an average LOS for acute rehabili- approximately two-thirds of the approximately 150,000
tation of 63 days (6). injury-related deaths that occur in this country each year
Although it is appealing to suggest that current out- and are commonly (albeit inappropriately) referred to as
comes are comparable to those of the 1970s and 1980s, accidents. Unintentional injury producing events or eti-
and that the reduction in ALOS is reflective of scientific ologies include motor vehicle crashes, falls, fires and
advancements leading to shorter, less-expensive periods burns, poisonings, drownings, diving, and other sports-
of inpatient hospitalization, many professionals believe related incidents. Motor vehicle crashes are the major
the reductions in ALOS are financially driven. Based on cause of spinal cord injuries.
SCI rehospitalization rates, which have been increasing, Intentional injuries are those resulting from acts of
it appears that the maximal point of efficiency may violence and are subdivided further into the categories
have been exceeded. Thus, initial lengths of stay may be of interpersonal and intrapersonal violence. As a group,
shorter, but as a result, sicker patients are being dis- intentional injuries account for the remaining one-third
charged, and the unintended consequence is their even- (approximately 50,000) of all injury-related deaths occur-
tual if not predictable rehospitalization. ring each year in the United States.
With regard to the prevention of SCI, or for that
matter prevention of any injury resulting from any cause,
INJURY PREVENTION AND CONTROL: it is absolutely mandatory to embrace the axiom that
A FRAME OF REFERENCE injuries are not accidents. This fundamental distinction
FOR SCI WORKERS between phenomena is critical because accidents, by def-
inition are unforeseen, unplanned, and unexpected events.
To increase the probability of preventing SCI in the future, In the case of an accident, the implication is that there
substantial evidence demonstrates the importance of was no fault or misconduct on the part of those injured
understanding prevention in a generic context, and then that which occurred could not have been predicted or pre-
applying that new knowledge to all aspects of SCI pre- vented. However, because it has been shown that injuries
vention. As fundamental as it seems, it is essential to can be studied in much the same way as infectious and
establisheven if we cannot agree upona common chronic diseases using time-honored epidemiologic prin-
injury prevention and control nomenclature. This is ciples and techniques, we have learned that unlike acci-
needed to assure that multidisciplinary workers with dents, injuries are predictable and, to a measurable
markedly different experience and training are talking extent, preventable (810).
about the same thing. Although this may appear overly
simplistic, it is worth noting that for many years there
were substantial controversy and spirited disagreement THE SCIENCE OF PREVENTION
among ASIAs founding fathers about two seemingly
straightforward concepts: a) neurologic level of spinal Injury prevention and control consists of three distinct
cord lesion; and, b) neurologic extent of injuryhow to but related phases: primary, secondary, and tertiary
precisely define the anatomic location of the lesion and prevention.
how to characterize the extent of neurologic deficit. Primary prevention focuses on the sum total of efforts
After what seemed to many an interminable debate, intended to stop the occurrence of an event with injury-pro-
the American Spinal Injury Association (ASIA) adopted def- ducing potential, in this case, SCI. For example, having
initions and criteria that are used today as the common established that motor vehicle crashes are the leading cause
frame of semantic reference for SCI workers (7). Thus, it of SCI and that a disproportionate number of motor vehi-
makes sense to use that experience and resultant model as cle crashes are caused by and/or involve drunk drivers,
the basis for this current discussion of SCI prevention. antidrunk driving legislation and its associated enforce-
ment is an example of the primary prevention of SCI. In
this case, the intent is to keep motor vehicle crashes caused
Injuries and Injury Categories
by intoxicated drivers from ever occurring.
As a universe, injuries are the physical expression of dam- Secondary prevention acknowledges that, despite
age to living tissue resulting from energy transfer. The our best efforts, it is not possible to prevent the occur-
exchange may be sudden and of very short duration, such rence of all injury-producing events. Thus, secondary pre-
as that occurring in a motor vehicle crash, or protracted, vention encompasses the sum total of efforts intended to
as in the case of carbon monoxide poisoning, where death reduce the severity of injuries.
typically occurs only after several hours of exposure to Returning to the etiology responsible for most SCI
the lethal gas. motor vehicle crashesthe classic example of a secondary
THE PREVENTION OF SPINAL CORD INJURY 887
preventive intervention is the seat belt. Here is the ally poor prognoses. Further, because of significant
premise: despite everything that is attempted, many motor advancements in emergency and acute care, many people
vehicle crashes still occur. However, if a preventive inter- now survive who, in the past, would have died from their
vention such as a seat belt is used correctly, it is likely the injuries. As a consequence, the number of injured people
injury will be substantially less severe than it might have potentially requiring rehabilitationrestoration of their
been. Although secondary in character, the seat belt is physiologic, psychologic, and social functioningis
an example of an active intervention that is implemented rapidly increasing. Moreover, because various healthcare
when the motor vehicle occupant makes a conscious deci- reform proposals promote the idea of some type of uni-
sion to use it and does so. Secondary interventions can versal coverage, the number of persons who could be eli-
also be passive, meaning that the intervention requires no gible for rehabilitation may increase dramatically in the
conscious act on the part of the person to be protected, future. If this happens, costs will likely increase, not
as is the case of supplemental restraint systems or air bags. decline, caused by a predictably huge growth in utilization.
Another example of secondary prevention can be In this instance, there will be no economy of scale (11).
demonstrated from an emergency medical service per- On the other hand, the proliferation of managed
spective. Specifically, the manner in which an injured per- care organizations (with their inherent service deliv-
son is immobilized and extricated from a motor vehicle eryrelated restrictions) is forcing providers to demon-
crash (even one with minimal or modest accompanying strate the efficacy of various diagnostic and therapeutic
property damage) by first-responders canand often practices, procedures, and technologies or risk being
doesinfluence the eventual extent of neurologic seque- denied reimbursement for providing them. Whereas some
lae if an SCI has occurred. In such cases, the underlying may question the reasonableness of such policies, propo-
assumption is always that there has been neurologic nents would argue (we think, correctly) that third par-
trauma to the spinal cord, even if there is no apparent clin- ties should be expected to pay only for goods and services
ical indication of cord insult. Accordingly, state-of-the-art of proven benefit, value, and cost effectiveness. The impli-
EMS protocols call routinely for use of the soft cervical cation of this position relative to rehabilitation is that we
collar and back board to immobilize motor crash victims must identifythrough rigorously conducted, intellectu-
as they are removed from vehicles and transported to ally honest researchthose diagnostic and therapeutic
trauma centers. The operative axiom is better safe than practices, procedures, and technologies that really work
sorry. And, in the case of the obvious SCI, appropriate and eliminate those of questionable or unproven merit
immobilization and extrication can greatly reduce the from traditional rehabilitation practice (11).
probability of a neurologically incomplete spinal cord Suffice it to say, a substantial amount of research
lesion from worsening or even becoming a much more firmly establishes the clinical benefits and economic sav-
devastating, neurologically complete injury through mis- ings associated with the early, aggressive, and highly
handling. expert application of rehabilitation technology to SCI
Preservation of life through emergency and acute patients. In fact, most injury-related cost estimates
medical care is the remaining aspect of secondary pre- described in the injury literature use SCI and TBI as mod-
vention. It encompasses a spectrum of finely honed med- els, because their documentation is superior to cost esti-
ical and surgical services provided to trauma patients for mates for virtually all other injuries (11).
the purposes of resuscitation, stabilization, treatment, and By far, primary prevention is the most cost-effective
correction of life-threatening defects or insults. Under this form of injury control, but in many respects it is also the
rubric, secondary prevention for persons with SCI also most abstract and elusive. It is difficult to measure pre-
addresses the prevention of common, costly, and debili- cisely that which does not happen, in the absence of his-
tating secondary medical conditions or complications torically accurate surveillance data. And, causeeffect
such as pressure ulcers, respiratory infections, pneumo- relationships are notoriously difficult to prove, especially
nia and atelectasis, autonomic dysreflexia, urinary tract when attempting to ascribe change to something as com-
infections, deep vein thrombosis, pulmonary embolism, plex as human behavior (12).
heterotopic ossification, as well as a wide range of behav- For example, while it is true that tough, new anti-
ioral and psychosocial pathologies. drunk driving laws have been adopted by state legisla-
Tertiary prevention or rehabilitation, is the third tures across the country, it cannot be proven, with any
component of injury prevention and control. It encom- measure of certainty, that such laws result directly in a
passes the sum total of efforts used to restore functional significant reduction in motor vehicle crash mortality and
capacity after an injury has occurred. In fact, we have morbidity statistics. Clearly, a variety of other factors
arrived at a point in medical achievement where rehabil- must be considered. For example, improved roadway
itation is not optional. characteristics, vehicle design that is inherently safer
Society no longer refuses to treat injured people, even today than in the past, a documented reduction in per
those with catastrophic conditions who have exception- capita alcohol consumption and the like, are all factors
888 SPECIAL TOPICS IN SPINAL CORD MEDICINE
capable of influencing a measured reduction in alcohol- increasing accountability, such data are not merely desir-
related motor vehicle crash rates, and hence, morbidity able, they are mandatory. Simply put, in todays highly
and mortality statistics (12). competitive health promotion arena, primary prevention
Another example of the difficulty associated with resources are much too scarce to squander on programs
attempting to link cause to effect is reflected in the out- that sound good on paper, feel good during execution,
come of Congressional action, taken in 1996, abolishing but do not work in practice.
the twenty-year-old federally imposed 55 mph speed limit.
Despite dire predictions by highway safety advocates of
enormous increases in traffic-related fatalities, lawmak- COSTS OF SPINAL CORD INJURY
ers returned the authority to set speed limits to the sev-
eral states. It is of more than casual interest that early sta- Harvey and coworkers estimated the direct costs of SCI,
tistics suggest the dire projections did not materialize. in 1988 dollars, to be $95,000 for initial hospitalization;
Admittedly, the data are provisional; however, they sug- $8,000 for environmental modifications; $8,000 per year
gest that todays motor vehicle crash environment is inher- for medical services, supplies, and adaptive equipment;
ently safer than it was two decades ago (12). and $6,000 for personal assistance and institutional care.
Moreover, by its very nature, primary prevention has These average figures varied by severity, patient age and
the potential to be the most cost-effective component of certain behavioral characteristics (22).
the injury control continuum. However, because primary By comparison, the results of work conducted at the
prevention programs are rarely subject to rigorous eval- University of Alabama at Birmingham by the National
uation, it has become increasingly clear that it is also an Spinal Cord Statistical Center suggest that the average
area having great potential for yielding little if any return yearly healthcare and living expenses in 1992 dollars var-
on investment. One classic example of this is driver edu- ied from $417,000 for the first post-injury year and
cation for high school students. Although supporters $75,000 for each subsequent year for persons who sus-
argue that driver education programs are responsible for tained severe injuries, to $123,000 for the first year and
lower motor vehiclerelated mortality statistics, rather $9,000 for each subsequent year for persons who sus-
convincing evidence exists that driver education programs tained less-severe injuries.
are responsible for increasing death and injury because The average cost for all groups was $198,000 for
such programs encourage more younger driversdrivers the first year and $24,000 for each subsequent year. These
in the highest risk age groupsto be on the highways figures do not include indirect costs, such as those result-
(1316). Other critics suggest that given the unappreci- ing from lost wages and productivity, which could aver-
ated complexity of the driving task and the inherent lim- age as much as $38,000 per year, but which vary sub-
itations of driver education programs, there is little rea- stantially on the basis of education, severity of injury, and
son such programs might work at all since no current pre-injury employment history (23).
driver education program can effectively perform those
tasks required of it (1721).
Potential Cost Savings
Another classic example is the Drug Abuse Resis-
tance Education Program (DARE), a primary interven- Although SCI does not occur nearly as often as many
tion that spread to schools across the country following other injuries, its associated costs are typically enormous.
its inception in 1983 and has been shown to be as inef- Moreover, given that life expectancies are today markedly
fective as high school driver training. Specifically, a three- increased for persons with SCI and that the preponder-
year study, commissioned by the National Institute of Jus- ance of new injuries occurs among younger persons, the
tice in the early 1990s, determined that whereas indirect costs of SCI are disproportionately high, often
DAREa high-profile programincreased childrens exceeding direct costs (23).
self-esteem, helped polish social skills, and improved atti- It is difficult, if not impossible, to estimate the poten-
tudes toward police, it did not and does not have a mea- tial savings that might accrue from all possible preventive
surable effect on drug abuse, the fundamental purpose interventions over the lifetime of an average spinal cord
of the intervention program. injured person. Too many variables would need to be fac-
The point is that in the absence of rigorous evalua- tored into an equation that would still be speculative at
tion criteria and practice, even the most promising, logi- best, because of many exacerbating issues. For example,
cal sounding, and appealing interventions may fall far how can we predict who will develop a significant sec-
short of their intended purpose. ondary condition or complication? Clearly, there are risk
Thus, before adopting a primary prevention pro- factors, and we know that some complications occur
gram for SCI, the litmus test should be rigorously docu- more frequently than others. Yet, there are many patients
mented evidence of the interventions effectiveness. Such at high risk for complications who, for whatever reason,
data are often difficult to acquire, but in this era of never develop them.
THE PREVENTION OF SPINAL CORD INJURY 889
However, to illustrate the cost effectiveness of pri- sonable; b) that the targeted interventions are appropri-
mary preventionpreventing the SCI from occurring ate and effective; and c) that $50 million would be
we can draw on some simple assumptions in a very basic appropriated by a combination of government and pri-
model that helps underscore the importance of preven- vate sector sources and used, successfully, for targeted
tion. To accomplish this, we draw on incidence estimates SCI prevention campaigns. Although caveats a and b are
for new SCIs occurring annually in the United States and well within the realm of possibility, the likelihood of a
then extrapolate using projected first-year and lifetime comprehensive, national SCI prevention campaign bud-
direct costs. geted at $50 million per year becoming a reality is
DeVivo and Fine (24) estimated the overall preva- remote. In fact, it is beyond remoteit is virtually
lence of SCI to be 906 new cases per million, based on the nonexistent.
mathematical relationship between incidence and preva- Clearly, the potential overall savings to the system far
lence. For this projection, they used an incidence figure exceeds the projected $150 million. The reasons are quite
of 30.2 new SCI per million persons at risk. Other inci- simple: among other limitations, this illustration is con-
dence estimates vary according to source, but it is gener- fined to direct costs. Moreover, it did not consider poten-
ally agreed that incidence ranges somewhere between 30 tial cost savings associated with preventing new SCIs in
to 40 new cases of SCI per million persons at risk each persons in other age groups. Also, the illustration ignores
year (25). direct savings associated with the costs of medical com-
plications requiring outpatient services and physician fees;
costs associated with required rehospitalizations, nursing
Projected Savings
home care, durable equipment, environmental modifica-
DeVivo, Whiteneck, and Charles (23) used a 4 percent tions, medications, and supplies; attendant care, household
discount rate to estimate the present value of average life- assistance, vocational rehabilitation, and a lengthy list of
time direct costs for persons sustaining SCI at 25 and 50 miscellaneous expenses for items such as transportation to
years of age. For the purpose of this illustration, we con- and from medical providers; related long distance tele-
fine our discussion of their projections to the 25-year-old phone charges; care and maintenance of helper animals;
SCI victim. and more. Suffice it to say, if such costs were figured in and
DeVivo and coworkers estimated average, direct life- added to indirect costs (including lost opportunity costs
time costs ranged from $287,018 for persons with the for the SCI patient and the caregiver (s) overall costs are
least severe injuries (Frankel Grade D) to $1,349,029 for much greater. In turn, the potential overall savings increase
persons with the most severe injuries (Frankel Group 1, far beyond the $150 million figure.
C1C4).
Coupling the estimated 8,314 new SCIs occurring
each year (24) with the reported statistic that 58.5 per- ETIOLOGICALLY DRIVEN
cent of all new SCIs occur in the 16- to 30-year age range, PREVENTION PROGRAMS
then using DeVivos model 25-year-old SCI patient as rep-
resentative of persons in the 16- to 30-year age range, we Motor Vehicle CrashRelated
project approximately 4,864 new SCIs annually (25). Morbidity and Mortality
Extrapolating from their figures, we derive an aver-
age lifetime direct cost for a person sustaining a SCI at 25 In the U.S. today, motor vehicle crashes are the leading
to be $818,023. If there are approximately 4,864 new SCIs cause of death for persons between 1 and 34 years of age.
in this age range each year, and if the average lifetime direct For persons between 5 and 29 years of age, more than
cost of each is $818,023, the projected direct cost of all 20 percent of deaths from all causes (including nonin-
4,864 SCIs is $3.97 billion dollars in 1992 dollars, which jury related deaths) are motor vehiclerelated, and motor
translates to $4.88 billion in June, 2000 dollars. vehicle crashes account for more than 40 percent of all
Having established a reasonable reference figure, deaths (again, including noninjury related deaths) for per-
preventing a modest 5 percent of those SCIs projected to sons in their late teens. In fact, motor vehicle crashes
occur in this age group in just 1 year would result in account for more deaths of people between 1 and 75 years
potential future savings of nearly $200 million. If a per of age than any other injury-producing event (26,27).
capita average of $1 million was spent on targeted SCI As reported by various workers, motor vehicle
prevention activities in each of the 50 states in a given crashes are responsible for over 0.5 million hospitaliza-
year, the overhead would be $50 million, resulting in tions, and approximately 5 million injuries not requiring
a net, lifetime savings to the health and medical care hospitalization each year. Given their importance as the
delivery system of $150 million for a mere 243 SCIs that leading cause of unintentional injuries and death, it is no
did not happen. Of course, the caveats are a) that our surprise that motor vehicle crashes are the major cause of
several assumptions based on the work of others are rea- catastrophic SCI (60 percent).
890 SPECIAL TOPICS IN SPINAL CORD MEDICINE
Clearly, driving under the influence of alcohol is a Prevention-Oriented Programs for SCIs
major risk factor for motor vehicle crashes. Prevention Resulting from Motor Vehicle Crashes (28)
programs resulted in a significant reduction of alcohol-
related deaths, as reflected in a statistic revealing that The NCIPC analyzed data collected from a national tele-
between 1987 and 1997, the alcohol-related motor vehi- phone survey (the Behavioral Risk Factor Surveillance Sys-
cle fatality rate dropped nearly 40 percent. Even so, tem) and Mothers Against Drunk Driving (MADD) to see
impaired driving remains a major threat to all road users, if residents of states with relatively low grades on the 1995
drivers, passengers, and others (28). MADD Rating the States survey were more likely to report
Older drivers have a crash rate of 3.0 per million drinking and driving. MADDs grades were used as an
vehicle miles traveled, which is approximately three times indicator of each states DUI prevention and enforcement
higher than that of drivers between 30 and 60 years of climate. Men from states with relatively low MADD
age. This is important for a number of reasons, not the grades were more likely to report drinking and driving.
least of which is our aging population. In fact, in the past However, there was no association between MADD grades
decade, the number of active older drivers has increased and self-reported drinking and driving among women.
50 percent. As the NCIPC points out, this number will The NCIPC is working with the North Carolina
continue to increase as the population continues to age. Department of Environment, Health, and Natural
Presently, it is estimated there are approximately 35 mil- Resources to evaluate whether mandatory substance-abuse
lion Americans (or approximately 13 percent of the U.S. assessment is effective in reducing repeat DUI arrests. The
population) who are at least 65 years of age. Demogra- findings will provide North Carolina and other states with
phers predict that the number of people who are at least recommendations for improving substance-abuse assess-
65 years of age will likely double by the year 2030. ment programs for drivers convicted of DUI.
Finally, with regard to older drivers and older passengers, Researchers reviewed studies on the impact of ran-
in the decade between 1987 and 1997, traffic deaths for dom alcohol screening on motor vehiclerelated injuries.
those over 70 increased 22 percent, with almost 6,000 Their findings indicated that screening reduces the inci-
people over the age of 70 dying of injuries sustained in a dence of deaths and nonfatal injuries in the U.S. and Aus-
motor-vehicle crash (28). tralia. The studies they reviewed also raised questions about
As reported by the NCIPC (28) in the year 1997, how long the effects of random alcohol screening last and
383,000 children under 16 years of age were injured in the degree of enforcement necessary to achieve them.
traffic incidents and more than 3,000 were killed. Of those A systematic review was conducted of research on
killed, 70 percent were motor vehicle passengers. In the 21 low-blood alcohol laws targeting young and inexperi-
years between 1975 and 1996, the child passenger death enced drivers. Researchers concluded that such laws pre-
rate dropped by approximately 11 percent, but traffic vent many crashes, injuries, and deaths.
safety experts believe the reduction could have been greater In Washington State, for the years 1989 to 1993, a
had the use of child restraint systems been more widely study was conducted of drivers injured in motor-vehicle
adopted. For example, during 1997, 63 percent of children crashes reported by the police. Study results showed that
between 0 and 14 years of age who were killed in motor alcohol was involved in 43 percent of such crashes and
vehicle crashes were not restrained. Of those between 5 in 47 percent of crash-related hospitalizations. For drink-
and 9 years of age, 46 percent were unrestrained. A recent ing drivers, the average hospital bill per crash was higher
study revealed that 75 percent of children weighing than for nondrinking drivers ($18,258 versus $14,181).
between 40 and 60 pounds were improperly restrained and A study conducted by Harvard University found
19 percent were unrestrained. A 1996 study found that that, although most alcohol-impaired drivers who were
85 percent of infants, 60 percent of those between 1 and fatally injured were not accompanied by adult passengers,
4 and 65 percent of those between 5 and 15 were about 5 to 10 percent of fatally injured drivers were
restrained. It is important to underscore that whether a accompanied in the vehicle by relatively unimpaired pas-
child wears a restraint often depends on the adult driver. sengers who might have intervened to prevent alcohol-
It is not surprising that when riding with an unrestrained impaired driving.
adult, only 1 in 4 children was restrained. Moreover, in The NCIPC is participating in a collaborative effort,
traffic incidents involving alcohol, there was an inverse led by the National Highway Traffic Safety Administra-
relationship between the blood alcohol concentration tion, to update the 1988 report, Transportation in an
(BAC) of the adult driver and the likelihood of a child pas- Aging Society, and to host an international conference
senger being restrained. Finally, with regard to infants and with a diverse group of experts to develop a national
children, it should be emphasized that airbags are haz- agenda and strategic plan for the safety and mobility of
ardousin fact, potentially lethalfor children 12 and older adults. The anticipated outcome of the conference
under. For this reason, children in that age group should will be a plan of action to reduce motor-vehicle deaths
not ride in the front seat of air bagequipped vehicles. and injuries among older people.
THE PREVENTION OF SPINAL CORD INJURY 891
A study is being conducted to determine the char- Researchers are studying the effectiveness of parents
acteristics of older adults who die while driving a motor influence, either through persuasive communication or
vehicle. Traits under study include demographic charac- driving restrictions, in reducing motor-vehicle crashes
teristics (e.g., age, education, medical condition); driving among teenagers.
behaviors (e.g., miles driven, driving after dark); and A biomechanics project at the University of Pitts-
physical functioning (e.g., activities of daily living). burgh will evaluate wheelchair seating systems in motor
A study is underway to determine if older drivers vehicles to improve occupant protection for people who
pose an excess risk of death or injury to other people. use wheelchairs while riding in motor vehicles.
Researchers are using a statewide database containing 3 The University of Pittsburgh will determine from
years worth of hospital discharge data and police acci- fetal death certificates what external events and associ-
dent reports for Wisconsin. ated factors accompany fetal deaths caused directly or
A study of older drivers found that those with lower indirectly by an injury suffered while in the womb. This
levels of cognitive and visual function drove less and project is cofunded with the National Highway Traffic
avoided high-risk driving situations. Safety Administration.
Researchers analyzing age-related driver ability and The University of California, Berkeley, has designed
performance problems used a computerized test as well and is testing a method of gathering reliable information
as a road test. They found significant correlations about the costs of head injury (helmeted and not hel-
between unsafe driving incidents and deficiencies in atten- meted) related to motorcycle crashes. This system is being
tion and in perceptual, cognitive, and psychomotor developed to provide states with a tool to assess the costs
abilities. of motorcycle-related head injuries when considering pol-
A systematic review of the published literature was icy concerning helmet use.
conducted to assess the effectiveness of clinical or com- Researchers at Harvard are analyzing crash data to
munity-based interventions designed to increase the use determine whether the thresholds at which air bags
of child-restraint devices among children younger than 5 deploy should be modified to ensure maximum protec-
years old. Such programs appear to be moderately effec- tion at higher-speed impacts, while not deploying at lower
tive in the short term, but their effects diminish substan- speeds, which can have an injurious effect on drivers,
tially 1 or more months after the intervention. especially women. Raising the deployment threshold of
A project based at Harvard University will evaluate airbags should be considered to increase safety.
efforts to increase the proportion of properly restrained Researchers at the University of Iowa are develop-
children, age 12 and under, seated in the rear of motor ing a battery of behavioral tests that will indicate the dri-
vehicles. Investigators will organize a community coali- ving capabilities of people with head injuries. These tests,
tion, design risk communications, and provide incentives conducted both on- and off-road, will measure attention,
or rewards to motivate parents and children to adopt memory, and driving function.
these safety behaviors. Researchers at Colorado State University are evalu-
Researchers are developing and evaluating the pro- ating interventions to lessen anger among young people
totype of a child booster safety system for children from while driving. Interventions focus on reducing anger and
30 to 60 pounds that will provide head protection, will its expression while driving, minimizing risky and aggres-
be easily positioned, and will incorporate a five-point sive driving, and reducing the number of crashes.
restraint system. If perfected, it will provide the same level Researchers at the University of California at Los
of protection as currently provided to children under 40 Angeles are investigating the effects of legislation in Cali-
pounds in child safety seats. fornia that restrict the time of day when teens can drive
Researchers at the University of North Carolina at and the age of passengers allowed in a minor drivers vehi-
Chapel Hill examined data related to childhood deaths cle. By comparing the incidence of and circumstances asso-
and injuries in motor vehicle crashes, following the imple- ciated with crashes of 16- and 17-year-old drivers before
mentation of a 1985 law mandating that child passengers and after the law was put into place, researchers will esti-
ages 4 to 15 years in the front seat use a safety belt. They mate the effects of the law in reducing crashes and injuries.
found that children in this age range experienced a 42 per- A project at the University of Washington will study
cent decline in deaths and serious injuries after the law the association between passenger airbags and passenger
was implemented. death in a vehicle crash and whether the risk of death
A project at the University of California at Irvine will varies by age of the passenger.
study factors associated with the use or nonuse of child Researchers at the University of Washington will
safety seats and will evaluate the effectiveness of pro- develop and test a device that can be retrofitted in a stan-
grams that provide education in addition to fining par- dard motor vehicle seat for reducing the whiplash
ents who do not use safety seats, compared with programs motions of the cervical spine in an occupant involved in
that only issue fines to parents. a rear-end collision.
892 SPECIAL TOPICS IN SPINAL CORD MEDICINE
A project at the Johns Hopkins School of Public by visiting the UAB-ICRCs Web site http://www.uab.edu/
Health will examine the feasibility of evaluating the pro- icrc/ready.htm.
tective effects of devices that automatically take up slack
in a seatbelt prior to crash forces being transmitted to
Fall-Related Morbidity and Mortality
the occupant of the vehicle.
One NCIPC-supported project will result in an inter- Falls are the leading cause of injury deaths among per-
active driving simulator for driver education and training sons 65 years of age and older and the second overall
for teenagers. The simulator will be designed for both the leading cause of unintentional injury-related deaths for
novice driver and advanced driver training for new auto- all age groups combined (28,29). Although falls and fall-
motive technology, adverse weather conditions, and emer- related injuries happen in all age groups, older persons
gency maneuvering. Using high school students, the pro- are more vulnerable and hence more likely to die from
gram will compare advanced simulator training with falls than any other segment of our population. In fact,
conventional driver training. each year more than 10,000 deaths among older Ameri-
cans are attributed to falls and fall-related injuries (30).
Further, the actual number of deaths in which a fall is a
UABs Project READY
contributing factor is probably much higher than sug-
In 2000, the University of Alabama at Birmingham gested by data as currently reported on death certificates.
(UAB)-based Injury Control Research Center launched a For example, although a primary cause of death might be
new intervention initiative targeting teens who drink alco- listed as pneumonia, it is possible the death might not
hol or use marijuana. Known by the acronym READY, have occurred had the injured senior citizen not been bed-
which stands for Realistic Education on Alcohol and fast as the result of an earlier fall (31).
Drugs for Youth, the intervention is designed to demon- Additionally, falls are the most common cause of
strate the consequences of substance abuse to teenagers injuries and of trauma-related hospital admissions (28).
who have been arrested and faced a local Family Court They are the source of virtually all fractures among the
judge (who is working with the project). elderly (32) and the second or third leading cause of brain
The program features activities that organizers hope injury and SCI, accounting for approximately 20 percent
will dramatically alter teens perceptions about drug and of all reported neurotrauma in a given year. Falls in
alcohol use. Teens see a graphic slide show prepared by younger age groups account for far fewer deaths, but an
the Jefferson County Alabama Coroner/Medical Exam- enormous amount of nonfatal injury (3336).
iners Office demonstrating the potential consequences of Hip fractures are the most serious fall-related injury.
trauma and violence resulting from drug and alcohol use. Likewise, because of our aging population, the hip fracture
The teens also spend a night in the emergency department problem in the U.S. is predicted to increase substantially
of either of the areas adult level-one trauma centers, The over the next 30 to 40 years. To illustrate, although it is esti-
UAB Hospital, or Carraway Methodist Medical Center, mated by the CDC that 300,000 fall-related hip fractures
for a first-hand view of the possible effects of drug and will occur in the year 2000, that number is expected to
alcohol abuse. exceed 500,000 fall-related hip fractures by 2040.
The program is limited to teens aged 14 to 17 years, White females are known to be at highest risk for falls
who appear in the Jefferson County Family Court for a and consequent hip fractures. Other factors known to
first offense for alcohol or marijuana use. The program increase the risk of falls and related injuries include: phys-
has four components: orientation, the coroner/medical ical conditions that limit ones ability to perform routine
examiner slide show, the night in the trauma center emer- activities of daily living; having weak muscles and balance
gency department, and a counseling session with Family problems; taking several medications or using psychoac-
Court personnel. All participants are also required to tive medications; environmental hazards such as cluttered
write an essay recounting their experiences. The goal is floors and stairs, slippery surfaces, and poor lighting; wear-
to reach young offenders after the first offense, thereby ing soft, thick-soled shoes; and vision problems.
changing their future behavior.
READY is a collaborative effort involving the Injury
Control Research Center; UAB Hospital; UAB depart- PREVENTION-ORIENTED PROGRAMS FOR
ment of emergency medicine and the UAB division of SCIS RESULTING FROM FALLS
trauma/burns and surgical critical care; Carraway
Methodist Medical Center; Childrens Hospital; Jefferson Several strategies have been shown to reduce the risk of
County Family Court; the Jefferson County Coroner/ fall-related injuries and deaths. These include: regular
Medical Examiners Office; the Jefferson County Sheriffs exercise to improve strength and balance; review and
Department and the Birmingham Police Department. adjustment of medications by a healthcare professional
Additional information about READY can be acquired to avoid side effects such as dizziness, drowsiness, or dis-
THE PREVENTION OF SPINAL CORD INJURY 893
orientation; and home modifications such as installing Biomechanics researchers developed a dual-stiffness
grab bars, removing tripping hazards, increasing lighting, floor to prevent hip fractures among the elderly. Previ-
and installing rails on both sides of stairs. ous CDC-sponsored research found that hip fractures
Simple clinical screening tests can accurately iden- occurred when the person fell directly on the hip, sug-
tify seniors who are most likely to fall. gesting that the energy absorbed during a fall, and not
In 1998, NCIPC began funding the Center on Aging bone strength, was the main cause of hip fracture.
at San Diego State University to establish the National Researchers used this information, along with computer
Resource Center on Aging and Injury. The Center is a and plastic models of hips and floors, to identify an opti-
joint effort between the Center on Aging, San Diego State mal new flooring material that provides a firm walking
University, and the American Society on Aging in San surface but yields to sudden impact.
Francisco. The Resource Center is using cutting-edge Researchers analyzed data from the Study to Assess
technology to collect, evaluate, and disseminate infor- Falls among the Elderly (SAFE) in South Miami Beach,
mation about preventing unintentional injuries among Florida, to determine whether certain kinds of medications
older adults. Fact sheets, formal publications, and the or drug combinations increased the risk of a hip fracture.
Web site (www.olderadultinjury.org) will educate health- They found no association between hip fractures and the
care professionals, caregivers, and other individuals con- use of antihypertensives, beta blockers, and vasodilators.
cerned about reducing injuries among older adults. A retrospective cohort study of Tennessee nursing-home
Since 1996, the NCIPC has been collaborating with residents found that residents were more likely to fall if
the National Fire Protection Association (NFPA), U.S. they used tricyclic and other heterocyclic antidepressants
Consumer Product Safety Commission (CPSC), and other and selective serotonin-reuptake inhibitors.
organizations to develop an educational program to reduce Analysis of the SAFE data found that for healthy
the incidence of fire- and fall-related injuries among older older persons, vigorous exercise decreases the risk of fall-
adults. In 1999, Remembering When: A Fire & Falls Pre- related fractures.
vention Program for Older Adults was completed and Analysis of the SAFE data also showed that older
pilot tested in Mississippi, Arkansas, and Alaska and in adults current calcium intake (from their diet or supple-
Atlanta and Cleveland. The revised program can be ments) does not appear to be associated with their risk
accessed through NFPAs Web site (www.nfpa.org). of sustaining a hip fracture.
Since 1996, NCIPC has funded a project of the Uni- CDC funded a randomized clinical trial of seven
versity of California at Los Angeles and the Roybal Insti- pairs of nursing homes to evaluate an intervention that
tute of Gerontology that is developing and testing an used structured, individual assessments of nursing-home
intervention to prevent falls and fall-related injuries residents. These comprehensive assessments were paired
among older Hispanics living in East Los Angeles. with recommendations on environmental and personal
NCIPC developed a fall-risk assessment checklist, safety, wheelchair use, psychotropic drug use, and walk-
Falls at Home, that uses reliable questions to identify ing safety. Residents who took part in the intervention
home hazards for falls and produce a fall-risk score. This were less likely to fall and be injured than other nursing-
tool can help public health organizations identify older home residents. These results were particularly significant
persons who are most likely to fall and better target inter- for those who had three or more falls in the previous year.
ventions. This instrument was field tested by the
Allegheny County (Pennsylvania) Health Department as
Children Cant Fly
part of their fire- and fall-prevention program. The valid-
ity of the fall-risk score is currently being assessed, based In the early 1970s, in response to an epidemic of falls
on the field-test data. among children from the windows of high-rise apartment
NCIPC developed A Tool Kit to Prevent Senior buildings, the New York City Department of Health
Falls, a comprehensive packet of materials that provides launched a pilot program known as Children Cant Fly.
health professionals with current technical information This program illustrates the potential effectiveness of
and resources about falls and fall-related injuries. Mate- injury prevention programs combining environmental,
rials can be incorporated into new or existing programs behavioral, and regulatory components.
to reduce falls among older adults. The tool kit contains Intervention strategies included free distribution
fact sheets, graphs and charts, current statistics, research (and in some cases installation) of window guards, mass
findings, and a science-based fall prevention brochure and media campaigns, community outreach activities, edu-
home safety checklist. Approximately 4,000 tool kits cation about the hazards of unguarded windows for at-
were distributed in late 1999 to state and local health risk families, and follow-up visits to families of fall vic-
departments and agencies on aging, health maintenance tims to provide referrals, and window guards, and to
organizations, and advocacy groups such as the Ameri- collect demographic and sociologic data. All of these
can Association of Retired Persons (AARP). strategies, combined, contributed to a 35 percent decline
894 SPECIAL TOPICS IN SPINAL CORD MEDICINE
in childhood mortality related to falls from heights population characteristics such as age, sex, and race
between 1973 and 1975. (with younger males being at greatest risk because their
In 1976, the New York City public health code was coefficient exposure is higher than other groups). Thus,
amended to include a requirement that landlords provide these important determinants of injury must be factored
window guards in apartment buildings inhabited by chil- into targeted prevention programs if such efforts are to
dren under 10 years of age. The educational interventions succeed.
were subsequently revised to reflect this new mandate.
Property owners were advised regarding compliance with
Prevention-Oriented Programs for SCIs
the regulation, and families with children under 10 years
Resulting from Diving, Sports, and
of age were informed about their right to window guard
Recreation-Related Events
installation. This third component of the intervention
proved effective and culminated in an overall reduction
THINK FIRST
of 50 percent in childhood mortality stemming from falls
from high-rise apartments (34). THINK FIRST, the National Brain and SCI Prevention
It is important to note that when this program Program (formerly known as The National Head and SCI
became a casualty of budget cuts in 1981, children living Prevention Program, which succeeded an earlier program
in New York City suffered a resurgence of falls from win- known as Feet First) is an award-winning public edu-
dows. In 1986, the city renewed its commitment to cation effort targeting teenagers and young adults, the age
enforcing the window guard mandate following several groups most at risk. This upbeat program educates young
highly publicized tragedies. As one might predict, fall- people about personal vulnerability and risk taking. The
related injuries among children began to decline. Char- message is that you can have a fun-filled, exciting life,
lotte Spiegel, program director of Children Cant Fly, without hurting yourself if you THINK FIRST and use
astutely observed that there is an ongoing need for con- your mind to protect your body. The mission of THINK
tinuedintervention to control and effectuate a decline FIRST is to prevent brain, spinal cord, and other trau-
in [falls] (37). matic injuries through the education of individuals, com-
munity leaders, and public policy makers. THINK FIRST
programs are underway in communities throughout the
Diving, Sports, and Recreation-Related
United States, Canada, Chile, and Mexico. Since the pro-
Morbidity and Mortality
grams inception in 1986, more than 6 million young peo-
Although there is an inherent risk of being injured in any ple have heard the THINK FIRST message.
sporting activity, the risk of sustaining a SCI is many First developed and implemented by the American
orders of magnitude greater for diving than for other Association of Neurological Surgeons (AANS) of the Con-
sports such as football, snowskiing, horseback riding, gress of Neurological Surgeons (CNS) in 1986, the name
surfing, gymnastics, trampoline, wrestling, hang gliding, THINK FIRST was adopted in May 1990. The catalysts
waterskiing, baseball, basketball, track and field. and for the establishment of the original national effort were
skateboarding. In fact, Go, DeVivo and Richards neurosurgeons E. Fletcher Eyster, M.D., of Pensacola,
reported that almost two-thirds of all sports-related SCIs Florida and Clark Watts, M.D., of Columbia, Missouri.
are caused by diving incidents (25). Moreover, some These two physicians had been involved in injury preven-
interesting trends in the causes of SCI have been noted tion programming in their communities for many years.
since the federally sponsored Model Systems Program Their early efforts were born out of frustration at not being
began admitting patients in the early 1970s. To illustrate, able to cure or fix spinal cord injured patients.
in the Model Systems database, the proportion of SCIs According to information appearing on their Web
caused by sports activities has declined from nearly 15 site, the efficacy of THINK FIRST has been demonstrated
percent before 1978 to only 8.8 percent since 1990. Cer- by its increasing acceptance by junior and senior high
tainly, this change cannot be interpreted to reflect either school educators, student essays, letters from parents and
the absolute or relative risk of SCI due to diving or other public officials, adoption by professional organizations,
sports, because the actual incidence rate of diving-related the measurement of attitude changes toward injury by stu-
SCI (and SCIs caused by other sports-related causes) is dents, and increased usage of safety belts by students. New
not known. It may be that the location of the several programs are encouraged to build an evaluation compo-
Model Systems may bias the number and types of cases nent into their efforts from the outset to continue docu-
that are captured by the participating Centermany of menting the efficacy of this approach to injury prevention.
which have changed over the years. Also, the demo- Although founded by neurosurgeons, the THINK
graphics of those being entered into the Model Systems FIRST Foundation recognizes that, by virtue of their work
have changed over time. Suffice it to say, diving and in treating patients who have sustained traumatic injuries,
sports-related SCIs are without question influenced by physicians and allied health professionals are natural
THE PREVENTION OF SPINAL CORD INJURY 895
spokespeople for prevention. Recently, the Foundation 110, Rolling Meadows, IL 60008 or via email: think-
has expanded its efforts to encourage orthopedic, trauma, first@aans.org. THINK FIRSTs telephone number is
pediatric, and emergency medicine physicians to get (847) 290-8600; toll free (800) Think56 (844-6556) and
involved, as well as nurses, physical and occupational the fax number is: (847) 290-9005 (38).
therapists, orthopedists, physician assistants, educators,
and others. THINK FIRST provides these professionals
HeadSmart
with the impetus and some of the tools to assist in spread-
ing the prevention message in their communities, espe- This program is the leading prevention initiative of the
cially to young people. Brain Injury Association, formerly known as the National
The THINK FIRST for KIDS curriculum was Head Injury Foundation. Established in 1992, HeadSmart
designed to enhance childrens interest in, learning about, provides elementary school teachers with innovative brain
and acceptance of safety messages. The curriculum is injury prevention materials, including a lesson book and
based on applied-learning and behavioral theories that reproducible handouts that can be tailored and integrated
suggest that varied messages, introduced over time, into lessons in every subject from math to art. The les-
increase understanding, knowledge retention, and sus- son book is flexible, user friendly, and contains easy-to-
tained behavior. Accordingly, the curriculum has been follow procedures; it is organized by grade level and
organized into six safety behavior units taught over a six- includes a section dedicated to violence prevention that
week period and integrated into teachers regular math, addresses conflict resolution, anger management, coop-
science, reading, spelling, and health curricula. The six erative problem solving, and self-esteem enhancement.
units cover the following: a) general function and struc- HeadSmart lessons address multiple causes of brain
ture of the brain and spinal cord; b) motor vehicle and injury by introducing and reinforcing pedestrian, play-
pedestrian safety; c) bicycle safety; d) conflict resolution ground, bicycle, and motor vehicle safety, as well as the
and weapons safety; e) water safety; and f) playground, proper use of protective equipment during sports activi-
recreation, and sports safety. The safety topics are pre- ties. Each lesson is designed to address one or more of the
sented not only in a range of subject contexts, but by following concepts: a) the brain is vital and unique; b) liv-
means of different teaching strategies, activities, and ing in modern society involves conditions that have make
media, which include role playing, storytelling, reading, the brain vulnerable beyond the bodys natural defenses;
hands-on demonstrations, and visual aids, such as class- c) injuries are not accidents (i.e., people are responsible
room posters, a comic book series, and an animated for their personal behavior and should take measures to
video, and, in Oregon, free bike helmets. The curriculum protect themselves against unwarranted injury); and d)
also includes unit-specific parent information letters. violence is never the answer to a problem (i.e., people are
THINK FIRST works with several other national responsible for their behavior toward others).
groups active in injury prevention. Relationships are An essential element of the HeadSmart program is
maintained with the National Coalition to Prevent Child- repeated exposure to brain function and brain injury pre-
hood Injury (the SAFE KIDS campaign), the National SCI vention messages. This elevates prevention from a one-
Association, the National Brain Injury Foundation, the time, special activity to an integrated segment of the
National Coordinating Council on SCI, the American school day. The Brain Injury Association believes that by
Medical Associations National Adolescent Health selecting elementary schoolaged children as their target
Forum, Mothers Against Drunk Driving, National High- audience, educational strategies have the short- and long-
way Traffic Safety Administration, and others. term potential for reducing the incidence of brain injury.
In several states, groups of local THINK FIRST This is partially because young people comprise a high-
programs have joined together in statewide coalitions to risk population for brain injury, and is also related to the
share common concerns and programming efforts, tendency for behavior modification to be more easily
address state public policy issues, and undertake joint achieved at earlier ages (39). At this time, we are not
fund-raising activities. Each such coalition has a desig- aware of any evaluation studies addressing the efficacy of
nated local program coordinator to work in close con- the HeadSmart curriculum.
junction with the national THINK FIRST office. Sev-
eral of these designated local programs also serve as
Injuries Resulting from Acts of
model training centers for the national THINK FIRST
Interpersonal Violence
program. A future goal of THINK FIRST is to have a
designated coordinator and training center for every According to the National Center for Health Statistics,
state. more than 6,000 people are physically injured by acts of
Additional information about the program can be interpersonal violence on an average day in the United
acquired by contacting the national headquarters: States (40,41). This translates to over 2 million nonfatal
THINK FIRST Foundation, Meadowbrook Drive, Suite injuries each year.
896 SPECIAL TOPICS IN SPINAL CORD MEDICINE
Intentional injury and its analogous term, interper- recreating the villagedrawing neighbors together so
sonal violence, have been defined as the use of physical they can tackle other problems, too (45). As is the case
force with the intent to inflict injury or death upon one- with Think First, we are not aware of any independent
self or another (42). With so broad a definition, and con- evaluation studies confirming the effectiveness of the Safe
sidering the enormous costs to society from violence, a Streets Now! initiative.
wide array of academic disciplines and professions have
approached the challenge of violence prevention. It is gen-
PeaceBuilders
erally acknowledged that a single approach to alleviat-
ing violence does not exist; therefore a multidisciplinary PeaceBuilders is a violence-prevention program target-
strategy is warranted (43). ing elementary school children (K-5). The program
Criminal justice generally views violence as it relates endeavors to change the school climate in a positive man-
to violations of the law. On the other hand, behavioral ner, and to promote prosocial behavior among students
science views violence as variations of aggressive human and staff. The main objective of this comprehensive pro-
behavior that can harm individuals, their families, and gram consists of instilling in children the following five
their communities. principles: a) praise people; b) avoid put-downs; c) seek
Finally, public health views violent acts as precursors wise people as advisors and friends; d) notice and cor-
to physical injury and, as such, a detriment to health (42). rect hurts we cause; and e) right wrongs. PeaceBuilders
Theoretically, each disciplines area of experience and view- activities are deliberately woven into the schools every-
points of violence partially overlap. In practice, the clear day routine to make them a way of life, rather than an
distinctions between these disciplines begin to blur, result- isolated exercise without meaningful application. This
ing in larger areas of common perspectives (44). way of life strategy reflects the educational, psycho-
Professionals such as police officers, social work- logic, and criminology research on which the program is
ers, judges, state public health professionals, probation based and is designed to be echoed and reinforced at
officers, nurses, mental health professionals, teachers, and home, in the community, and in the mass media.
physicians are just a few examples of the groups involved A consortium of the Pima County, Arizona Com-
in intentional injury control (42). Moreover, with the munity Services Department, the University of Arizona,
widely held belief that violence is a community problem, and the Tucson-based company Heartsprings, Inc. is cur-
more and more individuals are taking a proactive stance rently conducting a formal evaluation of ten schools con-
and claiming a role in violence prevention. sidered to be at risk. The schools in which PeaceBuilders
has been implemented are located in the Tucson Unified
and Sunnyside School Districts. The underlying theory of
Prevention-Oriented Programs
the program is that youth violence can be ameliorated
Resulting from Acts of Interpersonal Violence
through the initiation of prevention tactics early in child-
hood, improvement of resilience, and reinforcement of
Safe Streets Now!
positive behaviors. In addition, aggressive behavior can be
When arson began to seem like a reasonable stratagem curtailed by modifying the school environment to reward
for closing down a neighborhood drug house, a single and praise pro-social behavior among students, while min-
mother in Berkeley, California was struck by a novel idea. imizing factors that can trigger aggressive, hostile behav-
Rather than rely on the police to arrest drug dealers, ior. PeaceBuilders strives to increase the daily frequency
Molly Wetzel and eighteen of her neighbors sued the land- and salience of tangible and symbolic pro-social models,
lord of the drug house in Small Claims Court for allow- augment social competence, decrease the severity and fre-
ing his property to be used for purposes that prevented quency of aggressive behaviors, and provide strategies to
the enjoyment of life and property. The suit was pre- curb inadvertent reinforcement of negative behaviors.
cipitated by the robbery of Wetzels children at gunpoint, In many respects, PeaceBuilders appears to be the
a rash of burglaries in the vicinity, and the gunfire that embodiment of the African proverb it takes a village to
erupted in the area with alarming regularity. raise a child. PeaceBuilders recognizes that violent behav-
The band of neighbors won their case, each was ior neither evolves nor flourishes in a vacuum, but rather
awarded $2,000, and the drug house was shut down. This in a broad social context of harmful or protective factors
triumph led Wetzel to begin Safe Streets Now!, a national including neighborhoods, communities, and media. For
nonprofit organization that supports citizens who utilize this reason, the program includes four components: a) par-
small claims courts to address community blight. Since ent education; b) family marketing; c) collateral training
1992, the grassroots organization has closed 800 drug (for community volunteers); and d) mass media tie-ins.
houses in several states and has won over $1 million in Prior to implementation of PeaceBuilders, faculty at each
California court settlements. Were doing more than study site attended a pre-intervention orientation session
shutting down drug houses, explains Wetzel. Were and participated in a training workshop. Once the pro-
THE PREVENTION OF SPINAL CORD INJURY 897
gram was underway, faculty received site coaching dur- school-based program designed to help seventh graders
ing the first 8 to 12 weeks of the study, attended study ses- resist peer pressure to join gangs and to resolve conflicts
sions that addressed difficult issues peculiar to individual without resorting to violence. The program is imple-
schools, and attended periodic forums that allowed staff mented in areas where gang activity already exists or
to learn about the successes of their peers and solutions shows signs of emerging. Participants learn to set goals,
to common problems. Occasionally, one-day institutes develop self-esteem, make sound choices, appreciate cul-
were offered, which focused on applying and developing tural diversity, and learn about the harmful effects of
new materials and intervention strategies. drug use and its ramifications in relation to self, family,
The intervention structure of PeaceBuilders entails and friends.
nine broad behavior change techniques: a) common lan- Uniformed police officers and Federal agents teach
guage for community norms; b) story and live models the curriculum, which is designed to be taught 1 period per
for positive behavior; c) environmental cues to signal week for 9 consecutive weeks. A scaled-down 4-week ver-
desired behaviors; d) role plays to increase range of sion is also available for third and fourth graders. A fol-
responses; e) rehearsals of positive solutions after nega- low-up summer project is an integral part of GREAT, pro-
tive events (new way replays); f) group and individual viding an opportunity for reinforcement of lessons learned
rewards to strengthen positive behavior; g) threat reduc- in the classroom, as well as a springboard for community
tion to reduce reactivity; h) self- and peer-monitoring for service. The summer component is also beneficial in that
positive behavior; and i) generalization promotion to it provides some structure for youth who might otherwise
increase maintenance of change across time, places, and remain unsupervised over the summer months (47). At this
people. A typical school day could begin with a teacher time, we are not aware of any evaluation studies con-
greeting a child with a cue to th