Guillain-Barre Syndrome in Childhood

Introduction
Background
Guillain-Barr syndrome (GBS), or acute inflammatory demyelinating polyradiculoneuropathy (ADP),
describes a heterogeneous condition with a number of different variants. The most common presentation
is characterized by an acute monophasic, non-febrile, post-infectious illness manifesting as ascending
weakness and areflexia. Sensory, autonomic, and brainstem abnormalities may also be seen. With the
eradication of poliomyelitis, GBS is the most common cause of acute motor paralysis in children.
The first modern description of an illness likely to be ADP was published by Landry in 1859. Osler
provided a more detailed account of what he called acute febrile polyneuritis in 1892. n 1916, Guillain,
Barr, and Strohl further enlarged the clinical description and first reported the characteristic
cerebrospinal fluid (CSF) finding, albuminocytologic dissociation (ie, elevation of CSF protein with normal
CSF cell count). The CSF findings, in combination with certain clinical features, allowed ADP to be
distinguished from anterior horn cell diseases such as poliomyelitis, spinal muscular atrophy and from
other neuropathies.
!athophysiology
Demyelinating and axonal forms of GBS have both been described. n the demyelinating form,
segmental demyelination of peripheral nerves is thought to be immune mediated and both humoral and
cell-mediated immune mechanisms have been implicated. GBS with axonal degeneration may occur
without demyelination or inflammation.

Roughly two thirds of patients have a history of an antecedent gastrointestinal or respiratory tract
infection. Many authors believe that the mechanism of disease involves an abnormal T-cell response
precipitated by an infection. Some of the pathogenic triggers of GBS include Epstein-Barr virus,
cytomegalovirus, the enteroviruses, hepatitis A and B, varicella, Mycoplasma pneumonia, and
Campylobacter jejuni, which is perhaps the most common. These pathogens are believed to activate
CD4
+
helper-inducer T cells, which are particularly important mediators of disease.

A variety of specific endogenous antigens including myelin P-2, ganglioside GQ1b, GM1, and GT1a may
be involved in this response. Molecular mimicry of the triggering pathogens resembling antigens on
peripheral nerves leads to an overzealous autoimmune response mounted by T-cell lymphocytes and
macrophages. This interaction then causes the acute demyelinating polyradiculoneuropathy or,
particularly in cases of C jejuni, an acute axonal degeneration. A variant of GBS, Miller Fisher syndrome,
which is characterized by the triad of ophthalmoplegia, ataxia, and areflexia, is also linked to preceding
infection with C jejuni. Most of these patients have antibodies against the GQ1b ganglioside.



Vaccinations

Regarding the concern of antecedent vaccinations, the CDC has published retrospective data of the
1000 reported cases of known GBS from 1990-2005. The highest number of GBS cases was observed
after an influenza vaccination (n=632) and the second highest was after a hepatitis B vaccination
(n=94).
[1 ]
Based on data obtained from the National Health nterview Survey from 1997-2005, an average
of 54 million patients are vaccinated with the influenza vaccine each year. Thus, the incidence of
postinfluenza vaccination GBS is approximately 0.75 per 1 million vaccinations.

The adult and child total mortality of seasonal influenza alone in the United States is estimated to be
more than 36,000 per year according to CDC
[2 ]
so the risk of death from influenza alone would appear to
far outweigh the risk of influenza vaccination-related GBS.
Preliminary surveillance results of GBS after 2009 H1N1 vaccination up to March 2010 revealed an
increased incidence of GBS of 0.8 cases per 1 million people in both adult and child, which is
comparable to other seasonal influenza vaccines (1 extra case per 1 million vaccinations). This is in
contrast to a death rate of 9.7 and hospitalization rate of 222 per 1 million population for H1N1-
associated illness.
[3 ]

Postlicensure surveillance of the quadrivalent HPV (qHPV) vaccination from 2006-2008 reported 12
confirmed cases of GBS resulting in a relative risk of GBS following qHPV vaccination of 0.3 per 100,000
person years, which is no higher than the rate expected in the general population.
[4 ]
Of note, 3 cases of a
rapidly progressive motor neuron disease have also been reported, although a causal relationship has
not been established.
[4 ]


Review of the Menactra meningococcal conjugate vaccine (MCV) reveals a slight increase in the risk of
GBS, with a rate of 0.20 cases of GBS per 100,000 personmonths compared to a background incidence
of 0.11 GBS cases per 100,000 personmonths in unvaccinated individuals.
[5 ]
But, similar to the data on
influenza, the risk of meningococcal-related morbidity and mortality far outweighs the risk of vaccination-
related GBS. Case-cohort control analysis is needed to fully define the association of vaccines and GBS,
especially in children, and to explore the risk factors of why some rare individuals may be most
vulnerable to vaccine-related GBS.
requency
United States
Estimates of annual incidence of GBS range from 0.5-1.5 per 100,000 in individuals younger than 18
years. Only rarely does it occur in children under the age of two. There is a slight male predominance.

No clear seasonal preponderance of GBS has been noted in the United States although some seasonal
variation is reported in neighboring Mexico and Central America.
[6 ]

International
Risk of occurrence is similar throughout the world, in all climates, and among all races, except for reports
of seasonal predilections noted in some countries for Campylobacter- related GBS in the summer and
upper respiratory illness-related GBS in the winter. Recently, epidemics of an illness closely resembling
GBS were noted to occur annually in the rural areas of North China, particularly during the summer
months. These epidemics have been associated with C jejuni infection, and many of these patients are
found to have antiglycolipid antibodies. Because these cases involved degeneration of peripheral motor
axons without much inflammation, the syndrome has been termed acute motor axonal neuropathy
(AMAN).

Recently, other region-specific demographic studies have shown discrete preponderance of AMAN. For
example, in a prospective pediatric study (n=78) from Mexico, AMAN seemed to exhibit a seasonal peak
from July-September, unlike ADP, which seemed to be more evenly distributed throughout the year.
[7 ]


An ndian case-control study reports that 27.7% of childhood GBS cases were associated with C jejuni
infection.
[8 ]

ortality/orbidity
Overall mortality rate in childhood GBS is estimated to be less than 5%; mortality rates are higher in
medically underserved areas. Deaths are usually caused by respiratory failure, often in association with
cardiac arrhythmias and dysautonomia. Full recovery within 3-12 months is experienced by 90-95% of
pediatric patients with GBS. Between 5% and 10% of individuals have significant permanent disability.
O n general, the outcome of GBS is more favorable in children than in adults. Deaths are relatively
rare, especially if diagnosed and treated early. The recovery period is long, often weeks to
months, with a median estimated recovery time of 6-12 months. n one small pediatric series,
the median time from onset of symptoms to complete recovery was 73 days.
O The most common serious complications are weakness of the respiratory muscles and
autonomic instability. Pneumonia, adult respiratory distress syndrome, septicemia, pressure
sores, pulmonary embolus, ileus, constipation, gastritis and dysesthesias are also important
potential complications. During the acute phase of the disease, close attention should be paid to
respiratory status and signs of dysautonomia.
4 n cooperative children older than 5 years, respiratory function measurements, such as
vital capacity or maximal inspiratory force (MF), expressed in units of cc H
2
O pressure
can be valuable. MFs are also known as negative inspiratory force (NF). MFs less
than -20 cc H
2
O pressure can be an indication of poor inspiratory ability and respiratory
distress. MFs are normally greater than -40 cc H
2
O pressure, thus the more negative,
the better MF.
4 MFs provide objective data to follow and compare. This measure is unfortunately
difficult to monitor in young (<5 y) and any uncooperative child. Experienced pediatric
respiratory therapists can be very valuable in these measures.
4 Experienced pulmonary care is vital if neuromuscular weakness is affecting pulmonary
function. Possible interventions include CPAP, BPAP, mechanical ventilation, or cough-
assist devices.
4 Blood gases are not helpful in assessing neuromuscular respiratory failure and only
become abnormal when there is no longer any respiratory reserve, so that other means
of assessing respiratory function, such as respiratory rate and dyspnea on lying supine
are far more valuable early indictors of respiratory dysfunction.
4 Chest radiographs can be obtained to look for signs of infection.
4 Cardiac monitoring is essential to detect any signs of cardiovascular instability and treat
any arrhythmia.
4 The diagnosis of childhood GBS reportedly can be delayed if respiratory involvement is
the primary clinical finding upon presentation. Mistaken suspicion of a respiratory illness
may delay the diagnosis of GBS in children.
[9 ]

O Recurrence of GBS occurs in approximately 5% of cases, sometimes many years after the initial
bout. Treatment-related fluctuation (deterioration after VG treatment) in one small series was
observed in nearly 12% of cases in the first 2-3 weeks after intravenous immunoglobulin (VG)
administration.
O Some patients experience a chronic progressive course, known as chronic inflammatory
demyelinating polyradiculoneuropathy (CDP). Time is currently the main divider between CDP
and ADP in that CDP can only be diagnosed if the patient has been symptomatic for 8 weeks
or more.
#ace
Although major histocompatibility locus genes may play a role in susceptibility to GBS, no evidence
exists for any racial predilection.
Sex
Males appear to be at greater risk for GBS than females.
O This increased predilection for GBS has also been reported as a male-to-female ratio of 1.2:1 in
a review of children with GBS.
O A similar ratio of 1.26:1 was found in a prospective study of 95 children with GBS in Western
Europe.
[10 ]

O n a prospective study of 78 children from Mexico, acute inflammatory demyelinative
polyneuropathy (ADP) was 3 times more common in male patients than in female patients,
while acute motor axonal neuropathy (AMAN) was slightly more common in males than in
females.
[7 ]

O n Pakistan, a combined adult and pediatric Guillain-Barr study (n=175) reported that 68% of all
patients were male.
[11 ]

O n a study of 52 ndian children (median age, 5 y) with GBS, 75.4% were male.
[12 ]

O n a retrospective analysis of 10,486 cases of GBS in those younger than 15 years in Latin
America and the Caribbean, 58.2% were male.
[6 ]

ge
ndividuals older than 40 years have a steadily increasing risk, peaking at age 70-80 years, compared
with younger individuals. Children are at lower risk than adults, with incidence ranging from 0.5-1.5 per
100,000 children.
Recent retrospective reviews of childhood GBS reported the average age to be in the range of 4-8 years.
ndividuals affected with GBS can be as young as 1 year.
Clinical
History
O Patients with Guillain-Barr syndrome (GBS) present with complaints of weakness and/or
unsteadiness (ataxia). Pain and dysesthesias also are noted, particularly in children. Often,
onset of these symptoms is within 2-4 weeks of an illness or immunization. Urinary retention is
also noted early in the course of 10-15% of children with GBS.
O Weakness is a hallmark of GBS. The weakness typically starts in the legs and ascends to the
arms (hence, the description progressive ascending flaccid paralysis). This progression may
occur over hours to days to weeks. The weakness is usually symmetric. Patients often give a
history of a preceding illness that involves fever, muscle pains, diarrhea or an upper respiratory
illness.
O Pain may be the initial manifestation in almost half of affected children and may distract from the
actual diagnosis.
O Autonomic symptoms (eg, dizziness secondary to orthostatic hypotension) and tachycardia also
can occur.
O Clinical spectrum of GBS, which will include individual variation and variable severity of
presentation.
4 ADP
4 AMAN
4 Acute motor and sensory axonal neuropathy (AMSAN)
4 Miller-Fischer syndrome (MFS)
4 Polyneuritis cranialis
4 Pharyngo-cervical-brachial syndrome
4 Acute sensory neuropathy of childhood
4 Acute pandysautonomia
!hysical
O On physical examination, an ascending motor weakness is noted along with areflexia in the
classic form. Occasionally, autonomic instability (26%), ataxia (23%), dysesthesias (20%), and
cranial nerve findings (35-50%), predominantly facial palsy, are noted. These latter findings are
probably more frequent in children than in adults with this syndrome.
O Leg weakness (ie, foot drop) is usually noticed first and weakness eventually involves the calves
and thighs. Later, respiratory muscles and upper extremities show involvement. Some children
may become non-ambulatory. Weakness also may involve the respiratory muscles, and some
children need respiratory support during the course of the disease. Mechanical ventilation is
used until respiratory muscle function returns.
O Areflexia is a hallmark of GBS. Occasionally, some of the more proximal reflexes still may be
elicited during the early phase of the disease. Of clinical value is documenting reflexes in serial
exams; the progression from normoreflexia/hyporeflexia to areflexia is consistent with acute
features of GBS.
O The autonomic neuropathy involves both the sympathetic and parasympathetic systems.
Manifestations include orthostatic hypotension, hypertension, pupillary dysfunction, sweating
abnormalities, and sinus tachycardia.
Causes
O GBS is an autoimmune-mediated disease with environmental triggers (eg, pathogenic or
stressful exposures).
O Several infections (eg, Epstein-Barr virus, cytomegalovirus, hepatitis, varicella, other herpes
viruses, Mycoplasma pneumoniae, C jejuni) as well as immunizations have been known to
precede or to be associated with the illness. C jejuni seems to be the most commonly described
pathogen associated with GBS. Occasionally, surgery has been noted to be a precipitating
factor.
O Many forms of GBS are demyelinating. However, more recently, an axonal form of GBS has
been described after a diarrheal illness associated with C. jejuni.
O The differential diagnosis of GBS in childhood is primarily in the spectrum of progressive,
symmetric weakness.
4 n infants, botulism should be considered. Botulism is characterized not only by
(descending) weakness but also by involvement of the extraocular muscles
(ophthalmoplegia), miosis of the pupil and constipation. Pupillary abnormalities can be
an important distinguishing feature unique to botulism.
4 When ophthalmoplegia is present, myasthenia gravis should be considered.
Occasionally, myasthenia gravis can present with primarily proximal weakness in
childhood. A good history, testing for acetylcholine receptor antibodies and
electrophysiologic studies with nerve conduction studies (NCS) and electromyography
(EMG), including repetitive stimulation, can help to distinguish myasthenia gravis from
GBS.
4 GBS-like syndromes can occur in certain infections, such as Lyme disease or HV. n
these cases, lumbar puncture (LP) results typically show a CSF pleocytosis.
4 Myelopathies also can present sometimes with progressive weakness, and the physical
examination should help differentiate a spinal cord syndrome from a diffuse neuropathy.
Transverse myelitis can also produce a rapidly progressive paralysis, hyporeflexia, and
back pain. Poliomyelitis and other enteroviral infections of the anterior horn cell cause
acute focal, asymmetric limb weakness, usually in association with fever and pain.
4 Other acute neuropathies, caused by lead, heavy metals, or vincristine, can cause a
predominantly motor neuropathy.
4 Tick infestation can cause an ascending paralysis, and children should be searched for
ticks if they present with these symptoms. Often, the clinical syndrome improves
dramatically after removal of ticks. n the Eastern states of United States, the most
concerning tick is called Dermacentor variabilis.
4 Occasionally, organophosphate poisoning may present with a GBS-like picture.
ifferential iagnoses
Acute nflammatory Demyelinating Polyradiculoneuropathy HV-1 Associated Progressive
Polyradiculopathy
Cauda Equina and Conus Medullaris Syndromes HV-1 Associated Vacuolar
Myelopathy
Chronic nflammatory Demyelinating
Polyradiculoneuropathy
Lyme Disease
HV-1 Associated Acute/Chronic nflammatory
Demyelinating Polyneuropathy
Myasthenia Gravis
HV-1 Associated Distal Painful Sensorimotor
Polyneuropathy
Organophosphates
HV-1 Associated Multiple Mononeuropathies Toxic Neuropathy
HV-1 Associated Neuromuscular Complications
(Overview)

Other !roblems to Be Considered
Spinal cord lesions - Transverse myelitis, epidural abscess, tumors, poliomyelitis, enteroviral infections of
the anterior horn cells, Hopkins syndrome, vascular malformations, cord infarctions, cord compression,
lumbosacral disk syndromes, trauma
Peripheral neuropathies - Vincristine, glue sniffing, heavy metals, organophosphate pesticides, HV,
diphtheria, Lyme disease, inborn errors of metabolism, Leigh disease, Tangier disease, porphyria, critical
illness polyneuropathy
Neuromuscular junction disorders - Tick paralysis, myasthenia gravis, botulism, hypercalcemia
Myopathies - Periodic paralysis, dermatomyositis, critical illness myopathy, benign acute childhood
myositis
[13 ]

orkup
Laboratory Studies
O The diagnosis of Guillain-Barr syndrome (GBS) is typically made by the presence of a
progressive ascending weakness with areflexia. An LP, electrodiagnostic studies, or
occasionally MR findings can give support for this diagnosis.
O Typically, the LP is suggestive of demyelination (ie, increased protein >45 mg/dL within 3 weeks
of onset) without evidence of active infection (lack of CSF pleocytosis), as originally noted by
Guillain, Barr and Strohl.
4 The CSF findings may be normal within the first 48 hours of symptoms, and
occasionally the protein may not rise for a week. Usually by 10 days of symptoms,
elevated CSF protein findings will be most prominent.
4 Most patients have fewer than 10 leukocytes per milliliter, but occasionally a mild
elevation (ie, 10-50 cells/mL) is seen. Greater than 50 mononuclear cells/mL of CSF
makes the diagnosis of GBS doubtful.
Imaging Studies
Spine MR findings: Nearly 2 weeks after presentation of symptoms, lumbosacral MR can show
enhancement of the nerve roots with gadolinium. This imaging study has been described to be 83%
sensitive for acute GBS and present in 95% of typical cases.
Other Tests
O Temperature, blood pressure, heart rate, respiratory capacity (eg MFs) and urine output of the
patient should be monitored.
4 ntubation and mechanical ventilation should be considered when vital capacity falls
below 15 mL/kg body weight or arterial pressure of oxygen falls below 70 mm Hg (or the
patient has significant fatigue). Maximal inspiratory flows (MFs) or negative inspiratory
flows (NFs) are important measures in older children.
4 During the acute phase of the illness, orthostatic hypotension and urinary retention also
may cause significant problems.
O Electrodiagnostic studies
4 Within the first week of the onset of symptoms, electrodiagnostic studies in at least two
limbs reveal a dispersed, impersistent, prolonged, or absent F response (88%),
increased distal latencies (75%), conduction block (58%) or temporal dispersion of
compound muscle action potential (CMAP), and reduced conduction velocity (50%) of
motor and sensory nerves. Criteria for axonal forms include lack of neurophysiologic
evidence of demyelination, with loss of amplitude of CMAP or sensory nerve action
potentials to at least <80% of lower limit of normal values for age. t is typically prudent
to wait at least 7-10 days for electrical studies to be informative. f electrical studies are
performed too early, normal results can be falsely reassuring.
4 By the second week of illness, reduced compound muscle action potential (CMAP,
100%), prolonged distal latencies (92%), and reduced motor conduction velocities
(84%) are prominent.
O Serum anti-ganglioside antibodies
4 Value as a prognostic marker in children is still under evaluation.
4 Anti-GM1, GM1b, GD1a, and GalNAc-GDa have been associated in adults with C
jejuni infection, acute motor axonal neuropathy, a more severe course, and more
residual neurologic deficits.
4 A recent study of 32 Japanese children diagnosed with Guillain-Barr syndrome
identified one or more of these antibodies in 44% and in 64% of those who met the
electrodiagnostic criteria for acute motor axonal neuropathy. Those with positive
antibodies had a more prolonged recovery with more residual symptoms at the end of
the study.
[14 ]
However, another study in Western Europe did not find any difference in
clinical course or outcome in the 4 patients with positive antibodies out of 63 total
children with Guillain-Barr syndrome.
[15 ]

4 Other antibodies are associated with specific forms of Guillain-Barr , such as GQ1b
with Miller-Fisher syndrome, GD1b with acute sensory neuronopathy, and GT1a with
pharyngeal-cervical-brachial variant, and these may be useful in the diagnostic workup
of variant clinical presentations.
Histologic indings
Although not typically part of routine GBS diagnostic evaluation in pediatric or adult patients, the
following are expected findings in GBS:
O n the demyelinating form, demyelination and mononuclear infiltration by lymphocytes and
macrophages are seen in peripheral nerves.
O Lymphocytes and macrophages surround endoneural vessels and cause an adjacent
demyelination.
O These lesions can be discrete and are scattered throughout the peripheral nervous system,
although they may have a predilection for inflammation of the nerve roots.
O The conduction block and demyelination of the motor nerves result in the progressive weakness
that is characteristic of this syndrome. Similarly, the involvement of the sensory nerves leads to
pain and paresthesias.
Many authors believe that the mechanism of the disease involves an abnormal T-cell response
precipitated by a preceding infection. This is thought to give rise to an abnormal immune stimulation. A
variety of specific endogenous antigens may be involved in this response, including myelin P-2 and
ganglioside GM1, GQ1b, and GT1a.
Recently, epidemics of GBS were noted to occur annually in the rural areas of North China, particularly
during the summer months. This has been associated with C jejuni infection, and many of these patients
have antiglycolipid antibodies. n this axonal form of GBS, biopsy specimens reveal Wallerianlike
degeneration of fibers in the ventral and dorsal nerve roots, with only minimal demyelination or
lymphocytic infiltration. These axonal lesions affect both the sensory fibers and the motor fibers.
Although this form of GBS has been associated with Campylobacter infection, it appears to be a rare
complication of such infection.
Treatment
edical Care
To date, treatment for Guillain-Barr syndrome (GBS) has been aimed primarily at immunomodulation. n
pediatrics, the most effective form of therapy is generally considered to be intravenous immunoglobulin
(VG). Each batch of VG is made of human plasma derived from pools of 3,000-10,000 donors.
O VG has been used in multiple studies to treat the symptoms. t seems helpful in reducing the
severity of the disease as well as the duration of symptoms. However, the long-term outcome
may not be affected. Several regimens have been used. The optimal dose and dosage
schedules for VG have not been rigorously determined in childhood GBS. Only one
prospective, randomized treatment trial in childhood GBS is published.
4 One possible regimen includes daily administration of VG for 5 days at a dose of 0.4
g/kg/d, which can lead to improvements 2-3 days after the start of therapy. VG can be
given by way of a peripheral intravenous route.
4 Some authors use 2 g/kg of VG given as a single dose or 1 g/kg/d over 2 days in
children who are showing rapid signs of deterioration. Although, in a small, randomized
trial, the outcomes between the 2 treatment regimens was equivalent, and treatment-
related fluctuation (deterioration after receiving VG) occurred more often in children
who received the 2-day course of VG.
[16 ]

O Plasmapheresis: Studies in children using both historical and case controls indicate that
plasmapheresis may decrease the severity and shorten the duration of GBS.
4 Between 4 and 5 plasmapheresis treatments may be performed over 7-10 days, as
described in standard protocols.
4 Potential complications include autonomic instability, hypercalcemia, and bleeding due
to depletion of clotting factors.
4 Results of plasmapheresis and VG are similar, with possibly fewer side effects seen
with VG.
4 t stands to reason that plasmapheresis should not typically follow VG administration.
4 Plasmapheresis may be offered in some pediatric centers but is limited to larger
children. n most institutions, children weighing less than 10-15 kg may not be
considered for volume exchange therapy and central line vascular access dictates
intensive care hospitalization. These features distinguish plasmapheresis from VG,
which can be given to smaller children. Also, VG can be administered to patients by
peripheral V in specialized ambulatory clinic settings, advanced home nursing
programs, and at ward level hospital settings.
O Although corticosteroids were previously used to treat GBS, current data suggest that they
provide little benefit.
Consultations
O Consultation with a neurologist should be considered to confirm the diagnosis. ntensivists may
need to be involved quickly if critical care (cardiorespiratory) issues are suspected.
O Patients who need a prolonged time for recovery may benefit from consultation with a
rehabilitation medicine specialist including physical therapy, occupational therapy, and orthotist.
ctivity
O When stabilized, activity with physical and/or occupational therapy should be encouraged. f
motor deficits are profound, prevention of decubitus ulcers is highly important.
O n addition to the weakness, autonomic symptoms (eg, orthostatic hypotension) may also restrict
activity and should be monitored.
edication
The goals of pharmacotherapy are to reduce morbidity and prevent complications. ntravenous
immunoglobulin (VG) is the predominant choice in childhood Guillain-Barr syndrome (GBS). DVT
prophylaxis should be targeted and gastritis stress symptoms may benefit from H2 blockers (eg,
ranitidine). A bowel routine should also be instituted, as gastroparesis secondary to autonomic
dysfunction and/or extended bed rest is not uncommon.
Blood products
VG is an effective treatment of autoimmune neuropathies in general. t can reduce duration of
hospitalization as well as need or duration for mechanical ventilation.


IVIG (Gammagard, Gamimune)
Features relevant to efficacy may include neutralization of circulating myelin antibodies through anti-
idiotypic antibodies; down-regulation of proinflammatory cytokines, including FN-gamma; blockade of Fc
receptors on macrophages; suppression of inducer T and B cells and augmentation of suppressor T
cells; blockade of complement cascade; promotion of remyelination; 10% increase in CSF gG.
osing
dult
2 g/kg V over 2-5 d
!ediatric
Possible regimen includes 0.4 g/kg/d V for 5 d; other authors use 2 g/kg once or 1 g/kg/d over 2 d
Interactions
None reported
Contraindications
Documented hypersensitivity to product; severe systemic response to immune globulin (human); gA
deficiency; anti-gE/gG antibodies
!recautions
!regnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if
benefits outweigh risk to fetus
!recautions
Screen patients before VG administration; gA deficiency occurs in about 1 out of 1000 people; exercise
extreme caution in patients with congestive heart failure or renal insufficiency; rare reports exist of
noncardiogenic pulmonary edema, acute renal failure, aseptic meningitis, hemolytic anemia, and
thrombotic events
Adverse reactions are usually minor and occur in no more than 10% of patients, which include mild-to-
moderate headache, chills, chest discomfort, fatigue, fever, nausea, wheezing, dizziness, rashes, pains,
and tenderness at injection site
Consider checking serum gA before VG and using gA-depleted VG (G-Gard-SD) if indicated; VG
may increase serum viscosity and thromboembolic events
Adverse effects include migraine attacks, 10% increased risk of aseptic meningitis; increased risk of
urticaria, pruritus, or petechiae 2-5 d postinfusion and lasting as long as 1 mo; increased risk of renal
tubular necrosis in older patients, diabetic patients, volume-depleted patients, and patients with
preexisting kidney disease
Can lead to changes in lab values including elevated antiviral or antibacterial antibody titers for 1 mo; 6-
fold increased ESR for 2-3 wk; apparent hyponatremia
ollow-up
urther Inpatient Care
O Careful attention should be paid to multiple issues that may require intervention and specialist
consultation. Among the concerns of Guillain-Barr syndrome (GBS) comorbidities are
cardiorespiratory function, nutrition, urinary retention, decubitus ulcers, constipation, gastritis,
dysesthesias/pain, mood and anxiety issues, iatrogenic infectious complications, and
contractures in patients who are severely ill or who have a particularly prolonged course.
O n the long term, physical therapy may provide benefit to patients during the recovery phase of
the illness.
!atient Education
O For excellent patient education resources, visit eMedicine's Brain and Nervous System Center.
Also, see eMedicine's patient education article Guillain-Barr Syndrome.
O Family counseling and education can be very important early in the illness to set the tone for
potential of prolonged and critical illness course.
O Other patient and family-oriented Web sites include the following:
4 NNDS Guillain-Barr Syndrome nformation Page
4 GBS/CDP Foundation nternational
4 MayoClinic.com, Guillain-Barr syndrome