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▼
NEUROMUSCULAR DISEASES
KATHARINE N. CIARROCCA, DMD, MSED
MARTIN S. GREENBERG, DDS
ADI GARFUNKEL, DDS
592
Neuromuscular Disease 593
Diseases that affect both nerve and muscle tissue encompass infarct is usually good, with partial or complete resolution
a broad range of symptoms that often have profound impli- occurring over the following 4 to 6 weeks in many instances.
cations for the successful management of the dental patient.
The diseases discussed in this chapter include only those that CEREBRAL INFARCTION
affect the orofacial region or that have a significant effect on A cerebral infarction occurs when there is ischemia and necro-
dental practice. sis of an area of the brain after a reduction of blood supply to
a level below the level necessary for cell survival. The two major
▼ CEREBROVASCULAR DISEASE causes of cerebral infarction are thrombosis and embolism,
often the result of atherosclerosis. Emboli frequently originate
Cerebrovascular disease includes all disorders that cause dam- in atherosclerotic plaques in any vessel of the neck, such as the
age to the blood vessels supplying the brain, thereby producing carotid artery. The emboli break off, pass through the vascu-
neurologic damage. “Stroke” and “cerebrovascular accident” lature, and ultimately occlude an intracranial vessel, thus caus-
(CVA) are terms used to describe an acute neurologic injury ing a stroke. Thrombosis of an intracranial vessel may also
resulting from a severe interruption in the flow of blood to the lead to stroke.
brain. Complete cessation of the flow may render an irreversible The resulting deficit depends on the particular vessel
cerebral infarct within a period of 3 or 4 minutes. General involved and the extent of any collateral circulation. Carotid
symptoms following stroke include variable motor paralysis, artery atherosclerosis, for example, will most frequently cause
sensory loss, visual difficulties, and speech impairment. infarction in the region of the brain supplied by the middle
Epidemiologic studies have shown that cerebrovascular dis- cerebral artery. Occlusion of this artery results in contralateral
ease is the third leading cause of death in developed countries, signs such as facial weakness, head and eye deviation, flaccid
with a prevalence of 0.8% of the total population affected. It is hemiparesis or hemiplegia, and hemisensory loss.
estimated that more than 400,000 individuals are affected by Thromboembolic cerebral infarction also occurs as a com-
stroke annually in the United States. The majority of patients plication of other diseases. Many of the emboli that occlude
are over 60 years of age. The overall mortality rate following intracranial vessels arise from thrombi that have formed in the
stroke is 25% in the first month and 50% within 5 years. left side of the heart. Emboli originating from the heart are
often the result of thrombus formation after acute myocardial
Cerebrovascular Accident or Stroke infarction, chronic atrial fibrillation, or rheumatic heart dis-
Approximately 80% of strokes are associated with the devel- ease. Hypertension is an important risk factor in the develop-
opment of atherosclerosis leading to cerebral ischemia and ment of thrombosis, particularly at the carotid bifurcation.
infarction. The remaining 20% of cases are caused by cerebral Treatment of severe hypertension is essential for the preven-
hemorrhage.1 Deposition of atheromas in artery walls predis- tion of stroke since it is estimated that the risk of stroke
poses a patient to the development of thrombosis and embo- increases sevenfold in individuals with uncontrolled hyper-
lus formation, which results in infarction of the area of the tension. Septic emboli may result from bacterial endocarditis,
brain supplied by the occluded vessel. A thrombus is a clot in particularly when the mitral valve is involved.
the vasculature that forms from the constituents of blood and Other causes of ischemia and infarction of the brain
may be occlusive or attached to a vessel without obstructing the include decreased blood flow secondary to sudden severe
lumen. An embolus is a clot, composed of a detached throm- hypotension, acute hypertension causing spasm of the cerebral
bus, mass of bacteria, or other foreign body, that originates vessels, and hematologic abnormalities such as thrombocyto-
from a distant site in the body and occludes a vessel. Atheromas sis, anemia, and cavernous sinus thrombosis.
commonly develop in the branching portions of the arterial
system, particularly at the origin of the internal carotid artery. CEREBRAL HEMORRHAGE
Additional sites of thrombus formation associated with stroke Hemorrhage of intracranial vessels may also cause stroke.
include the vertebral, basilar, and middle cerebral arteries. The two most common reasons for hemorrhage are (1) rup-
ture of an aneurysm and (2) an arteriovenous malformation
LACUNAR INFARCTION (AVM) that hemorrhages spontaneously, often secondary
Lacunar infarcts are small lesions (usually < 5 mm in diame- to hypertension or following the administration of antico-
ter) that occur in the distribution of the short penetrating agulant medication.
arterioles in the basal ganglia, circle of Willis, pons, cerebellum, The majority of cases are aneurysmal. Aneurysms are
anterior limb of the internal capsule, and (less commonly) localized dilations of arteries, caused by structural weakness
deep cerebral white matter. Lacunar infarcts are associated of vessel walls. True aneurysms are found in arteries with
with poorly controlled hypertension or diabetes. Symptoms normal wall structures that have been damaged by conditions
usually include unilateral motor or sensory deficit without such as atherosclerosis, mycotic infections, and syphilis. False
visual field deficit or disturbance of consciousness or language. aneurysms occur after the traumatic rupture of arteries and
The neurologic deficit produced by the lacunar infarct may their subsequent repair by fibrous tissue. The size of the
progress over 24 to 36 hours before stabilizing; however, the aneurysm is important in determining its tendency to rup-
prognosis for recovery from the deficit produced by a lacunar ture, a tendency that is aggravated by smoking, alcohol con-
594 Principles of Medicine
position to excessive bleeding. A thorough medical history years apart. The most common symptoms following an acute
with an accurate medication list that includes dosages is essen- exacerbation include impairment of vision, muscular incoor-
tial. In addition, it may be necessary to confer with the dination, and bladder dysfunction. The general histologic fea-
patient’s physician to obtain current coagulation values (ie, tures are multiple disseminated plaques or areas of demyeli-
PT, INR) so as to ensure that the patient is stable for more nation within the central nervous system.
invasive dental treatment.
Xerostomia is a common side effect of the medications Etiology and Epidemiology
used in the management of cerebrovascular disease and related The specific etiology of MS has not been clearly determined.
disease processes. Patients who are thus affected can then be An immunologic basis is strongly suggested by the presence of
susceptible to a higher caries rate. Meticulous oral hygiene, activated T lymphocytes and autoantibodies to glycoproteins
more frequent recalls, saliva substitutes, and fluoride applica- detected in MS lesions. In addition, it is considered probable
tion can aid in the maintenance of the dentition.6 that both genetic and environmental factors are involved, with
Stroke patients can also have physical disabilities, which infection as the major environmental agent. Both viral and
can affect the orofacial area and can alter the provision of bacterial infections can initiate or precipitate attacks of MS.
dental care. Patients with hemiplegia or hemiparesis may Evidence that implicates certain viruses in the initiation of the
need additional help in home care. Patients with weakness in disease has been documented; increased antibody titers against
the muscles of the orofacial area may have poor control of measles virus, rubella virus, mumps virus, Epstein-Barr virus,
oral secretions, a reduced gag reflex, and changes in their herpes simplex viruses 1 and 2, and human herpesvirus 6
ability to masticate, leading to poor nutrition. Patients with (HHV-6) have been found in the cerebrospinal fluid and
apraxia affecting the orofacial region may have impaired vol- serum. To date, none of these viruses has been isolated from
untary movements, such as protruding the tongue, expecto- the lesions of MS, and no specific relationship between MS and
rating, and lip puckering. a specific microorganism has been proven. Further data sup-
In general, dental treatment should not present major portive of an infectious etiology for MS include the observa-
problems for most poststroke patients. Careful history taking, tion that MS has occurred in clusters in specific populations,
checking of blood pressure prior to treatment, avoidance of the prime example being the increased incidence of MS in the
lengthy appointments, and general reassurance are all impor- population of the Faroe Islands following foreign troop occu-
tant factors in the provision of dental treatment for patients pation during World War II.7
with a history of stroke. Genetic influences also appear to play a significant role in
the development of MS. Studies of identical twins have shown
▼ CAVERNOUS SINUS THROMBOSIS that if one twin suffers from MS, there is a 26% chance that the
other twin will also be affected by the disease. A preponderance
Cavernous sinus thrombosis, usually secondary to dental, of specific human leukocyte antigen (HLA) types has also been
nasal, or ocular infections, is a rare but severe complication noted in MS patients.8,9
because of its possible fatal outcome. Infections of the maxil- The most accepted general finding related to the etiology
lary dentition may spread to the cavernous sinus through of MS is the fact that disease prevalence increases with distance
openings in the cranial bones or through emissary veins con- from the equator; for example, MS is most common in north-
necting the extra- and intracranial systems. Venous propaga- ern Europe, Canada, and New Zealand. There are no obvious
tion begins with the facial vein and proceeds through the oph- reasons for this geographic difference in disease prevalence.
thalmic vein, which is an affluent of the cavernous sinus. In
most cases, patients experience rapid swelling of the face and Clinical Manifestations
eyelids. The classic neurologic signs of acute cavernous sinus The clinical signs and symptoms of MS depend on the site of
thrombosis are exophthalmos, periorbital edema, retinal vein the demyelinating lesion. The lesions may occur almost any-
thrombosis, and involvement of the ophthalmic division of the where in the central nervous system, but they have a predilec-
trigeminal nerve and trochlear and abducent nerves, leading to tion for certain areas. More than 60% of individuals with MS
ptosis, dilated pupils, and lack of corneal reflexes. Treatment have visual disturbances caused by demyelinating lesions of
consists of immediate antibiotic therapy and the removal of the second cranial nerve. The loss of vision usually occurs
the source of infection whenever possible. over a period of several days, with partial recovery within 1
month. Other ophthalmic symptoms include “color blind-
▼ MULTIPLE SCLEROSIS ness” and diplopia caused by involvement of the third, fourth,
and sixth cranial nerves. Uhthoff ’s sign, found in MS, is char-
Multiple sclerosis (MS) is a chronic neurologic disease associ- acterized by rapid vision loss following a body temperature
ated with the demyelination of axons within the central ner- increase that is associated with strenuous exercise. Another
vous system. The disease occurs more frequently among important sign of ocular disturbance associated with MS is
women. The average age of onset is during the fourth decade Marcus Gunn’s pupillary sign, which can be elicited in
of life, but MS may occur at any age. The disease presents in patients with unilateral optic neuritis in the following man-
the form of recurrent attacks; in some cases, the attacks are ner: a bright light is shone into each eye separately; when this
596 Principles of Medicine
light is moved from the normal to the affected eye, the pupil inal neuralgia, sensory neuropathy of the trigeminal nerve,
of the latter dilates rather than constricts. and facial palsy.11
Weakness or paresthesia of the extremities, with an increase Trigeminal neuralgia (TGN) is present in about 2% of
in the deep tendon reflexes, is another common early finding cases of MS and is an initial manifestation in 0.3% of cases.12
in cases of MS. An important feature of motor nerve function In those cases in which MS is associated with TGN, there
in MS patients is the relative fluctuation of symptoms on a appears to be an earlier age of onset, and symptoms are com-
daily basis. These symptoms may remit for long periods and monly bilateral. Pain is normally severe and lancinating, but
then suddenly reverse, leading to paraplegia. Other common trigger zones may be absent. In time, the pain often becomes
signs of the disease include bladder dysfunction, euphoria, less severe but more continuous. Effective drug therapy
ataxia, vertigo, and generalized incoordination. includes the use of carbamazepine, baclofen, gabapentin, or
The majority of cases of MS are chronic and are charac- phenytoin. When medication proves inadequate, thermoco-
terized by exacerbations and remissions over a period of many agulation, surgical sectioning of the nerve, or alcohol injection
years. During acute episodes, severe neurologic involvement is may be considered.
evident. This slowly resolves, but some permanent neurologic Sensory neuropathy secondary to MS can be progressive
involvement remains after each episode. The extent and sever- and difficult to diagnosis. It most often affects the second and
ity of the permanent involvement varies considerably from third divisions of the trigeminal nerve, has a sudden onset, and
patient to patient. In mild cases, little permanent effect is is painful. Neuropathy to the mental nerve can cause numb-
noted, and patients may have a normal life span. In severe ness of the lower lip and chin.
acute cases, total paralysis may occur within months. Overall, Facial paralysis appears later in the course of the disease. It
it has been found that approximately 70% of patients with may be difficult to distinguish between the paralysis caused by
MS live for more than 25 years after the onset of the disease. MS and that due to Bell’s palsy, but up to 24% of MS sufferers
may experience facial paralysis.11
Diagnosis
The diagnosis of MS is clinical and is based on the age of the ▼ AMYOTROPHIC LATERAL
patient, the presence of neurologic signs that cannot be
explained by a single lesion, the progressive nature of the dis-
SCLEROSIS
ease, and a history of exacerbations and remissions. There are Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig
no definitive laboratory tests for MS, but demyelinating disease and motor neuron disease, belongs to a group of
changes can be seen on magnetic resonance imaging (MRI) in degenerative diseases affecting both the upper and the lower
more than 90% of cases.4 The presence of increased motor neurons of the central nervous system. The principal
immunoglobulins (specifically immunoglobulin G [IgG]) in symptoms are weakness and wasting, which normally begin in
the cerebrospinal fluid without infection is another diagnos- the upper extremities and are invariably unilateral at onset.
tic indicator of the disease. The estimated prevalence of ALS in the United States is
reported as 2 to 7 per 100,000 persons, with slightly more
Treatment males than females affected. The average age of onset is
Evidence suggests that high doses of intravenous cortico- between the fourth and sixth decades of life. The etiology is
steroids may arrest the progress of MS; about 85% of patients unknown, although the disease is familial in 10% of cases
with relapsing-remitting MS show objective signs of neuro- when it is inherited as an autosomal dominant or autosomal
logic improvement during treatment with intravenous corti- recessive trait.1 The degenerative changes of ALS can be seen
costeroids. Long-term treatment with immunosuppressants in the corticospinal tracts (upper motor neurons), the motor
may reduce the frequency of relapse in patients with MS. cells of the brainstem, and the anterior horn cells of the spinal
Azathioprine is probably the safest drug in this category and cord (lower motor neurons).
has reduced relapse to 70% of study patients in 3 years, com-
pared to 80% of patients in the placebo group. Administration Clinical Manifestations
of methotrexate appears to be the best therapy for slowing In the most typical form of ALS, stiffness of the fingers, awk-
deterioration in patients with chronic progressive MS. The use wardness in tasks requiring fine finger movements, and slight
of interferon-γ-1b and -1a has shown promise; both have been weakness or wasting of the hand muscles are the first indica-
shown to reduce clinical attacks and lesions detectable by con- tions of the disease. Cramping and fasciculation of the mus-
trast-enhanced MRI by approximately 30% when compared to cles of the forearm, upper arm, and shoulder girdle also appear.
placebo. Other nonpharmacologic measures, such as total lym- Before long, the triad of atrophic weakness of the hands and
phoid irradiation, plasmapheresis, and immunoglobulin ther- forearms, light spasticity of the arms and legs, and generalized
apy, have had marginal benefit.10 hyperreflexia (in the absence of sensory changes) leaves little
doubt as to the diagnosis. After some time, the disease affects
Oral Health Considerations all regions, including the muscles of mastication, facial expres-
Certain clinical manifestations of MS affect the orofacial sion, and tongue, leading to difficulties in mastication and
region; three are of particular interest to the dentist: trigem- speech. Dysfunction of the temporomandibular joint and the
Neuromuscular Diseases 597
development of malocclusion may also occur as the disease striatum. Experiments to reduce dopamine levels in animals
progresses. Aspiration pneumonia is the cause of death in most have clearly shown that the classic symptoms of Parkinson’s
patients with ALS. disease can be observed following this procedure. Thus, a low
dopamine level will result in hypokinesia whereas a high level
Treatment will lead to hyperkinesia. Symptoms similar to parkinsonism
There is no effective treatment for this disease, and the course may also be induced by drugs that cause a reduction of
is one of progressive deterioration, with a mean survival of 3 dopamine in the brain, the most common of the drugs being
years. Ceftriaxone, gabapentin, guanidine hydrochloride, gan- phenothiazine derivatives. When drugs such as these are ter-
gliosides, interferons, and cyclophosphamide are a few of the minated, symptoms also quickly subside. Although a definite
long list of agents that have all proven to be ineffective. Only etiology has not been established, the most likely explanation
supportive measures can be used.13 is that the disease results from a combination of accelerated
aging, genetic predisposition, exposure to toxins, and an
Oral Health Considerations abnormality in oxidative mechanisms.15,16
Muscles of the orofacial area can be affected by the disease
process of ALS. The most striking physical effect of ALS relates Clinical Manifestations
to the declining function of the muscles used for breathing. As Tremor, rigidity, bradykinesia, and postural instability are the
the patient may not be able to cough or clear the throat, a cardinal features of parkinsonism and may be present in any
reclining position for dental treatment is contraindicated due combination. The tremor is most conspicuous at rest, is
to the increased risk of aspiration.14 enhanced by emotional stress, and tends to be less severe dur-
In addition, a hyperactive gag reflex can pose a problem. ing voluntary activity. Rigidity (an increase in resistance to
Although it provides assurance that no dental debris will be passive movement) is responsible for the characteristically
aspirated, every effort must be made not to induce gagging or flexed posture seen in many patients. The most disabling
vomiting. Topical anesthetics should be avoided, patients symptoms are due to bradykinesia, manifested as a slowness of
should be nil per os (NPO) for 12 hours prior to treatment, voluntary movement and a reduction in automatic movements
and the dentist should avoid contact with soft-tissue areas that such as the swinging of the arms while walking.
may induce gagging. Topical fluoride may also induce nausea. The onset of the disease is generally insidious. Mild stiff-
Finally, patients with ALS may have difficulty with oral ness of the muscles of the extremities and tremor of the hands
hygiene. Not only will these patients be challenged with phys- are frequently early signs. The typical hand tremor is often
ical disabilities that limit their effectiveness in maintaining called a “pill-rolling” movement, characterized by the rubbing
oral hygiene, they are also faced with a loss of the natural clear- of the thumb against the fingers, and is particularly pro-
ing ability of the oral cavity because of decreased muscular nounced when the patient is otherwise at rest. The general
activity of the tongue and the perioral musculature.14 stiffness progresses slowly until significant disability is noted
Mechanical toothbrushes, fluoride rinses, and more frequent by the patient. Walking becomes more difficult, and the
periodontal recall may benefit these patients greatly. patient develops a slow shuffling gait with a stooped position.
As the ability to perform voluntary movements decreases,
▼ PARKINSONISM: PARKINSON’S DIS- patients usually experience an inability to coordinate separate
EASE (PARALYSIS AGITANS) or independent movements.
Many of the signs of Parkinson’s disease are found in the
Parkinsonism is a neurodegenerative disorder characterized by head and neck. The typical “masklike” facial appearance with
rigidity, tremors, bradykinesis, and impaired postural reflexes. infrequent blinking and lack of expression is caused by
The most common form of parkinsonism is Parksinson’s dis- bradykinesis. The muscle rigidity also causes difficulty in swal-
ease (paralysis agitans), but parkinsonism is seen in a variety lowing, resulting in drooling. Speech becomes labored because
of disorders such as postencephalitic parkinsonism, arte- of the lack of muscle control, and mandibular tremor results
riosclerotic parkinsonism, and post-traumatic parkinsonism in masticatory difficulties, especially in those with removable
following closed head injury. Parkinson’s disease was first dental appliances. Abnormalities in oral behavior, such as pur-
described by James Parkinson in 1817. It is an idiopathic dis- poseless chewing, grinding, and sucking movements, are also
ease that mainly affects adults in middle or late life. The well recognized in patients with Parkinson’s disease and make
reported prevalence rate in the general population is 130 in dental treatment especially difficult.
100,000 persons; however, among those who are older than 60
years of age, the rate is considerably higher.15,16 Treatment
Etiology and Pathogenesis MEDICAL MEASURES
In idiopathic parkinsonism, dopamine depletion due to degen- Drug treatment is often not required early in the course of
eration of the dopaminergic nigrostriatal system in the brain- parkinsonism. Patients with mild symptoms but no disabil-
stem leads to an imbalance of dopamine and acetylcholine, ity may be helped by amantadine. This drug improves all of
neurotransmitters that are normally present in the corpus the clinical features of parkinsonism, but its mode of action
598 Principles of Medicine
cases of CP, the right side is more often involved. Sometimes, perception on the anterior two-thirds of the tongue and
there are seizures associated with mental retardation. The dys- reduced salivary secretion.
kinetic type is characterized by athetotic purposeless move-
ment, involving both agonist and antagonist muscles, that is Treatment
increased by voluntary activity. During either natural or The only medical treatment that may influence the outcome
induced sleep, the movements cease. Head movements and is the administration of systemic corticosteroids within the
facial grimacing are characteristic. One should not be misled first few days after the onset of paralysis, but this therapy
by the appearance of the patient since most CP patients are should be avoided if Lyme disease is suspected. Combining
intellectually normal. steroids with antiherpetic drugs such as acyclovir may decrease
the severity and length of paralysis.28
Treatment It is also helpful to protect the eye with lubricating drops
With improved perinatal care, both anoxia and perinatal infec- or ointment and a patch if eye closure is not possible. When
tions have been markedly reduced, thus leading to a reduction paralysis-induced eye opening is permanent, intrapalpebral
in the incidence of CP. The key to success in the management gold weights are inserted, thus closing the upper eyelid. Facial
of CP is teamwork and a planned approach to the individual plastic surgery and the creation of an anastomosis between
child’s problem. Many children with CP have normal intelli- the facial and hypoglossal nerves can occasionally restore
gence and should not be penalized because of dysarthria or partial function and improve appearance for patients with
involuntary movements. Physiotherapy should be instituted as permanent damage.
early as possible in order to prevent contractures, and ortho-
pedic surgery has been used occasionally with some degree of ▼ GUILLAIN-BARRÉ SYNDROME
success.21 If the patient suffers from seizures, appropriate drug
therapy is instituted (see “Epilepsy,” later in this chapter). Guillain-Barré syndrome (acute idiopathic polyneuropathy)is
an acute symmetrical ascending polyneuropathy, often occur-
Oral Health Considerations ring 1 to 3 weeks (and occasionally up to 8 weeks) after an acute
Children with CP show an increased incidence of enamel infection. The Guillain-Barré syndrome often follows a non-
defects, the cause of which is not clear. A much more both- specific respiratory or gastrointestinal illness but has also been
ersome finding is the sialorrhea and drooling experienced by described after a few specific infections (such as with
CP patients. This develops in the absence of orofacial and Cytomegalovirus, Epstein-Barr virus, Enterovirus, Campylobacter
neck muscle coordination. One treatment, sialodocholo- jejuni, or Mycoplasma) and after immunization. There is a
plasty (the relocation of salivary gland ducts into the tonsil- worldwide incidence of 1.6 to 1.9 cases per 100,000 population
lar fossa and the removal of the sublingual salivary glands), per year.29 The disorder probably has an immunologic basis, but
has been shown to be effective.22 Less invasive management the precise mechanism is unclear.
of sialorrhea includes a systemic medication such as gly-
copyrolate and anticholinergic agents.23 Clinical Manifestations
The syndrome often begins with myalgia or paresthesias of the
▼ BELL’S PALSY lower limbs, followed by weakness, which often ascends to
involve abdominal, thoracic, and upper-limb muscles. In
Bell’s palsy is recognized as a unilateral paresis of the facial severe cases, respiration is compromised. Involvement of the
nerve. The dysfunction has been attributed to an inflammatory autonomic nervous system by the disease process may induce
reaction involving the facial nerve. A relationship has been changes in blood pressure and pulse rate. From an oral medi-
demonstrated between Bell’s palsy and the isolation of herpes cine perspective, the interesting feature of this disease is the fact
simplex virus 1 from nerve tissues.24–26 Bell’s palsy must be dif- that impaired swallowing or paresthesias of the mouth and
ferentiated from other causes of facial nerve palsy, including face may be early signs of the disease. The seventh cranial
Lyme disease, herpes zoster of the geniculate ganglion (Ramsay nerve is frequently involved, and bilateral facial weakness is
Hunt syndrome), and tumors such as acoustic neuromas.27 common. Involvement of other cranial nerves may result in
ptosis or facial myokymia. Dysarthria, dysphagia and diplopia
Clinical Manifestations may develop in severe cases.
Bell’s palsy begins with slight pain around one ear, followed by
an abrupt paralysis of the muscles on that side of the face. The Treatment and Prognosis
eye on the affected side stays open, the corner of the mouth The paralysis in Guillain-Barré syndrome may progress for
drops, and there is drooling. As a result of masseter weakness, about 10 days and may then remain relatively unchanged for
food is retained in both the upper and lower buccal and labial about 2 weeks. The recovery phase is much slower and may
folds. The facial expression changes remarkably, and the take from 6 months to 2 years for completion. Permanent signs
creases of the forehead are flattened. Due to impaired blink- of neurologic damage can persist in some patients.
ing, corneal ulcerations from foreign bodies can occur. Treatment with prednisone is ineffective and may actually
Involvement of the chorda tympani nerve leads to loss of taste affect the outcome adversely by prolonging recovery time.
600 Principles of Medicine
stage of life, the age of the patient at the onset of seizures is alized seizure by a few seconds or minutes and that is itself a part
closely associated with the various causes of epilepsy. Infants of the attack, arising locally from a restricted region of the brain.
are much more likely to suffer from epilepsy after complica- The most common type of seizure is the tonic-clonic or
tions at birth, such as anoxia, intracranial injury, metabolic dis- grand mal seizure; 90% of epileptics experience it alone or in
orders, and congenital malformations. Predominant causes in combination with another type of seizure. A grand mal seizure
children and adolescents include trauma and acute or febrile characteristically begins with an aura. The aura may be expe-
infections whereas young adults who have engaged in alcohol rienced as epigastric discomfort, as an emotion, or as an hallu-
or drug abuse commonly suffer from generalized seizures after cination of hearing, vision, or smell. The aura is followed sec-
periods of severe abuse. Epilepsy in older adults may occur as onds to minutes later by unconsciousness, a cry, and tonic
a complication of any of the previously mentioned causes but muscle spasms; this rigid phase lasts about 30 seconds. Because
is more often associated with cerebrovascular diseases such as of the spasm of the respiratory muscles, the patient does not
stroke and tumor. breathe and becomes cyanotic during this period. The tonic
phase is followed by a clonic phase composed of convulsive jerky
Classification of Seizures movements, incontinence, and tongue biting (Figure 23-1). The
Seizures can be categorized in various ways, but the descrip- patient may injure him- or herself seriously if he or she is near
tive classification proposed by the International League Against hard or sharp objects.A postictal state characterized by headache,
Epilepsy is clinically the most useful. Seizures are divided into confusion, lethargy, occasional temporary neurologic deficit, and
those that are generalized and those that affect only part of the deep sleep usually follows a grand mal seizure.
brain (partial seizures)36 (Table 23-1). Simple partial seizures The number, severity, and duration of grand mal seizures
originate from one localized area of the brain and do not fea- vary considerably from one patient to another. Status epilepticus,
ture loss of consciousness. In contrast, complex partial a severe form of the disorder, occurs when a series of seizures fol-
seizures, often referred to as temporal lobe or psychomotor low each other before the patient is able to regain consciousness.
seizures, are associated with an impairment of consciousness. Absence or petit mal seizure is the second most common
The majority of generalized seizures are called either absence type of seizure, and it occurs without an aura and with few or
(petit mal) seizures or tonic-clonic seizures (grand mal). The no clonic or tonic movements. Absence seizures present almost
remaining generalized seizure types described in the classifi- exclusively in children and frequently disappear during the
cation (ie, myoclonic or infantile seizures and clonic, tonic, and second decade of life. A single seizure lasts just seconds. The
atonic seizures) are usually found in childhood and carry a patient loses consciousness and appears to stare into space. He
poorer prognosis for normal childhood development. “Status or she continues normal activity immediately after the seizure
epilepticus” refers to a period of recurrent seizure attacks with- is over. Petit mal seizures may occur several times each day, and
out recovery between each attack. All forms of seizure may severe cases may interfere with school and social activities.
progress to a period of status epilepticus. Around puberty, approximately 50% of persons who experi-
Clinical Manifestations ence petit mal seizures will develop tonic-clonic seizures.
A B
FIGURE 23-1 A, Bilateral human bites of the upper lip, received during an epileptic seizure. B, Traumatic injury (bite) to tongue occurred during an
epileptic seizure.
these drugs produce significant but different side effects, includ- connective tissues.40 Gingival overgrowth may occur at any age
ing blood dyscrasias, anemia, and alteration of hepatic function. but seems to affect younger patients to a greater degree than
adults. Men and women are equally affected. There does not
Oral Health Considerations appear to be a correlation between dosage and the incidence
Patients with epilepsy may be treated in the private dental set- of gingival overgrowth. There is strong clinical evidence of a
ting. A thorough medical history should indicate what type of correlation between poor oral hygiene and the amount of tis-
seizures the patient has, how well the seizures are controlled, sue enlargement.
the frequency and duration of seizures, the potential triggers Clinically, phenytoin gingival overgrowth starts in the inter-
for seizures, and what to expect if the patient has a seizure. dental papillae and occurs only where teeth are present. The
Treatment planning may be altered, however, depending on the papillae enlarge buccally and lingually. The enlarged areas are
status of the seizure disorder. As a general rule, it is better to firm, pink, and covered with normal mucosa. The severity of the
place a fixed prosthesis rather than a removable appliance hyperplasia varies. In some patients, the enlarged gingivae may
because of the potential for removable appliances to become involve just one or two papillae; in other cases, the crowns of the
dislodged during a seizure.39 teeth are completely covered with gingival tissue. The best treat-
Patients who are taking anticonvulsant drugs are subject to ment of phenytoin gingival overgrowth begins with prevention.
gingival overgrowth. This overgrowth is most often associated Little doubt remains that careful oral hygiene can prevent or at
with patients who are taking phenytoin (Figure 23-2). About least minimize the gingival enlargement. Soon after being placed
half of the patients placed on phenytoin will show evidence of on anticonvulsant therapy, each patient should be referred to a
gingival enlargement, usually within 2 to 18 months after start- dentist for oral hygiene instruction and gingival curettage.
ing the medication. The etiology is still unknown, but there Patients who have not been properly managed and who develop
appears to be an increase in the number of fibroblasts in the gross gingival enlargement will require gingivectomy. Curettage
A B
FIGURE 23-2 A, Phenytoin gingival overgrowth in a 17-year-old girl. The enlarged tissues are light pink and fibrous, and they show no evidence
of edema, inflammation, or ulceration. B, Patient with phenytoin gingival overgrowth following electrocautery of enlarged gingival tissues. The excess
maxillary gingival tissue had been removed 10 days previously. The excess mandibular gingival tissue had just been removed. Fractures of the mesioincisal
angles of the maxillary first incisors occurred during a seizure.
604 Principles of Medicine
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