AUTHOR AND EDITOR INFORMATION

Section 1 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

INTRODUCTION

Section 2 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

Background

Guillain-Barré syndrome (GBS), or acute inflammatory demyelinating polyradiculoneuropathy (AIDP), is characterized by progressive motor weakness and areflexia. Sensory, autonomic, and brainstem abnormalities are also common. These symptoms usually follow a febrile and/or viral illness. 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 AIDP was published by Landry in 1859. Osler provided a more detailed account of what he called acute febrile polyneuritis in 1892. In 1916, Guillain, Barré, 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 AIDP to be distinguished from anterior horn cell diseases such as poliomyelitis and from other neuropathies.

For a CME activity, see Routine Vaccination With Quadrivalent Meningococcal Conjugate Vaccine Not Recommended for Certain Children.

Pathophysiology

Demyelinating and axonal forms of GBS have both been described. In the demyelinating form, segmental demyelination of peripheral nerves is found in association with infiltration of inflammatory cells. GBS with axonal degeneration may occur without demyelination or inflammation.

Many authors believe that the mechanism of disease involves an abnormal T-cell response precipitated by a preceding infection. Some of the pathogenic triggers of GBS include Epstein-Barr virus, cytomegalovirus, hepatitis, varicella, Mycoplasma pneumonia, and Campylobacter jejuni, perhaps 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 and autoimmune response mounted by T-cell lymphocytes and macrophages.

Frequency

United States

Estimates of annual incidence of GBS range from 0.5-1.5 per 100,000 in individuals younger than 18 years. No clear seasonal preponderance of GBS has been noted in the United States although some seasonal variation is reported in neighboring Mexico.

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 involve 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 AIDP, which seemed to be more evenly distributed throughout the year.¹

Mortality/Morbidity

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.

• In general, the outcome of GBS is more favorable in children than in adults. Deaths are relatively rare, especially if recognition of the signs of this disorder are acted upon quickly. The recovery period is longer than the duration of the acute illness, often weeks to months, with a median estimated recovery time of 6-12 months. In one small pediatric series, the median time from onset of symptoms to complete recovery was 73 days.
• The most common serious complications are weakness of the respiratory muscles and autonomic instability. Pneumonia, adult respiratory distress syndrome, septicemia, pressure sores, ileus, constipation, gastritis, dysesthesias, and pulmonary embolus are also important potential complications. During the acute progressive phase of the disease, close attention should be paid to respiratory status.
• • In cooperative children older than 5 years, respiratory function measurements such as vital capacity or maximal inspiratory force (MIF), expressed in units of cc H₂O pressure can be valuable. MIFs are also known as negative inspiratory force (NIF). MIFs less than -20 cc H₂O pressure can be an indication of poor inspiratory ability and respiratory distress. MIFs are normally greater than -40 cc H₂O pressure, thus the more negative, the better MIF.
  • MIFs 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.
  • Blood gases and chest radiographs are also valuable in assessing respiratory parameters.
• Recurrence of GBS occurs in approximately 5% of cases, sometimes many years after the initial bout. Recurrence is generally thought to be relatively uncommon in children but has been reported in one small series to be observed in nearly 12% of cases in the first 2-3 weeks after intravenous immunoglobulin (IVIG) administration.
• Some patients experience a chronic progressive course, known as chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).

Race

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. This increased predilection for GBS has also been reported as a male-to-female ratio of 1.2:1 in a recent review of children with GBS. A similar ratio of 1.26:1 was found in a prospective study of 95 children with GBS in Western Europe.² In a prospective study of 78 children from Mexico, acute inflammatory demyelinative polyneuropathy (AIDP) 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.¹ In Pakistan, a combined adult and pediatric Guillain-Barré study (n=175) reported that 68% of all patients were male.³

Age

Individuals 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. Individuals affected with GBS can be as young as 1 year.

CLINICAL

Section 3 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

History

• Patients with Guillain-Barré 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.
• Weakness is a hallmark of GBS. The weakness typically starts in the lower extremities and ascends to the upper extremities (hence, the description progressive ascending flaccid paralysis). This progression may occur over hours to days to weeks. The weakness is symmetric, occurring on both sides of the body in most cases. Usually, patients give a history of a preceding illness that involves fever, muscle pains, and upper respiratory illness. Some would claim that vomiting may be more predictive of acute inflammatory demyelinating polyneuropathy (AIDP) while diarrhea may be a harbinger of acute motor axonal neuropathy (AMAN).
• Pain may be the initial manifestation in almost half of affected children. Because of the prominence of pain, some children are misdiagnosed initially.
• Autonomic symptoms (eg, dizziness secondary to orthostatic hypotension) and tachycardia also can occur.
• Unsteadiness is often due to the weakness itself. However, the Miller-Fisher variant, which also can be seen in children, is characterized by ophthalmoplegia, ataxia, and areflexia with relatively little weakness. Such affected patients with Miller-Fischer triad are generally not thought to be at as great a risk for severe respiratory compromise.
• Clinical spectrum of GBS, which will include individual variation and variable severity of presentation.
• • AIDP
  • AMAN
  • Acute motor and sensory axonal neuropathy (AMSAN)
  • Miller-Fischer syndrome (MFS)
  • Polyneuritis cranialis
  • Pharyngo-cervical-brachial syndrome
  • Acute sensory neuropathy of childhood
  • Acute pandysautonomia

Physical

• 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%) are noted. These latter findings are probably more frequent in children than in adults with this syndrome.
• 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.
• 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.
• The autonomic neuropathy involves both the sympathetic and parasympathetic systems. Manifestations include orthostatic hypotension, hypertension, pupillary dysfunction, sweating abnormalities, and sinus tachycardia.

Causes

• GBS is an autoimmune-mediated disease with environmental triggers (eg, pathogenic or stressful exposures).
• 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.
• Many forms of GBS are demyelinating. However, more recently, an axonal form of GBS has been described after a diarrheal illness secondary to C jejuni. 
• Other diseases can present with a GBS-like picture.
• • The differential diagnosis of GBS in childhood is primarily in the spectrum of progressive, symmetric weakness.
• • In infants, botulism should be a consideration. Botulism is characterized not only by weakness but also by involvement of the extraocular muscles and constipation. Pupillary abnormalities can be an important distinguishing feature unique to botulism.
  • When ophthalmoplegia is present, myasthenia gravis should be considered. Occasionally, myasthenia gravis can present with primarily proximal weakness in childhood. Nerve conduction velocity (NCV) and electromyography (EMG) findings, including repetitive stimulation, can help distinguish myasthenia gravis from GBS.
  • GBS-like syndromes can occur in certain infections, such as Lyme disease or HIV. In these cases, lumbar puncture (LP) results typically show a CSF pleocytosis.
  • 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.
  • Other acute neuropathies, caused by lead, heavy metals, or vincristine, can cause a predominantly motor neuropathy.
  • 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. In the Eastern states of United States, the most concerning tick is called Dermacentor variabilis.
  • Occasionally, organophosphate poisoning may present with a GBS-like picture.

DIFFERENTIALS

Section 4 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

Other Problems to be Considered

Major categories

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, HIV, 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

WORKUP

Section 5 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

Lab Studies

• The diagnosis of Guillain-Barré syndrome (GBS) is typically made by the presence of a progressive ascending weakness with areflexia. An LP, electrodiagnostic studies, or occasionally MRI findings can give support for this diagnosis.
• Typically, the LP is suggestive of demyelination (ie, increased protein >45 mg/dL within 3 wk of onset) without evidence of active infection (lack of CSF pleocytosis), as originally noted by Guillain and Barré.
• • The CSF findings may be normal within the first 48 hours of symptoms, and occasionally the protein may not rise for a week. Serial spinal taps are sometimes often warranted if early studies are normal. Usually by 10 days of symptoms, elevated CSF protein findings will be most prominent.
  • 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 casts some doubt on the diagnosis of GBS.

Imaging Studies

• Spine MRI findings: Nearly 2 weeks after presentation of symptoms, lumbosacral MRI can show enhancement of the cauda equina 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

• Temperature, blood pressure, heart rate, respiratory capacity (eg, MIFs), blood gases (if necessary), and urine output of the patient should be monitored.
• • Intubation 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 (MIFs) or negative inspiratory flows (NIFs) are important measures in older children.
  • During the acute phase of the illness, orthostatic hypotension and urinary retention also may cause significant problems.
• Electrodiagnostic studies
• • 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. It is typically prudent to wait at least 7-10 days for electrical studies to be informative. If electrical studies are performed too early, normal results can be falsely reassuring.
  • 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.
• Serum anti-ganglioside antibodies
• • Value as a prognostic marker in children is still under evaluation.
  • Anti-GM1, GM1b, GD1a, and GalNAc-GDIa have been associated in adults with C jejuni infection, acute motor axonal neuropathy, a more severe course, and more residual neurologic deficits. 
  • A recent study of 32 Japanese children diagnosed with Guillain-Barré 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.⁴ 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-Barré syndrome.⁵
  • Other antibodies are associated with specific forms of Guillain-Barré, such as GQ1b with Miller-Fisher syndrome and GT1a with pharyngeal-cervical-brachial variant, and these may be useful in the diagnostic workup of variant clinical presentations.

Histologic Findings

Although not typically part of routine GBS diagnostic evaluation in pediatric or adult patients, the following are expected findings in GBS:

• In the demyelinating form, demyelination and mononuclear infiltration by lymphocytes and macrophages are seen in peripheral nerves.
• Lymphocytes and macrophages surround endoneural vessels and cause an adjacent demyelination.
• 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.
• 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. In 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

Section 6 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

Medical Care

To date, treatment for Guillain-Barré syndrome (GBS) has been aimed primarily at immunomodulation. In pediatrics, the most effective form of therapy is generally considered to be intravenous immunoglobulin (IVIG). Each batch of IVIG is made of human plasma derived from pools of 3,000-10,000 donors.

• IVIG has been used in multiple studies to treat the symptoms. It 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 IVIG have not been rigorously determined in childhood GBS. Only one prospective, randomized treatment trial in childhood GBS is published. 
• • One possible regimen includes daily administration of IVIG 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. IVIG can be given by way of a peripheral intravenous route.
  • Some authors use 2 g/kg of IVIG given as a single dose or 1 g/kg/d over 2 days in children who are showing rapid signs of deterioration.
• Plasmapheresis: Studies in children using both historical and case controls indicate that plasmapheresis may decrease the severity and shorten the duration of GBS. 
• • Between 4 and 5 plasmapheresis treatments may be performed over 7-10 days, as described in standard protocols.
  • Potential complications include autonomic instability, hypercalcemia, and bleeding due to depletion of clotting factors.
  • Results of plasmapheresis and IVIG are similar, with possibly fewer side effects seen with IVIG.
  • It stands to reason that plasmapheresis should not typically follow IVIG administration.
  • Plasmapheresis may be offered in some pediatric centers but is limited to larger children. In 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 IVIG, which can be given to smaller children. Also, IVIG can be administered to patients by peripheral IV in specialized ambulatory clinic settings, advanced home nursing programs, and at ward level hospital settings.
• Although steroids previously were used to treat GBS, current data suggest that they provide no to little benefit.

Consultations

• Consultation with a neurologist should be considered to confirm the diagnosis. Intensivists may need to be involved quickly if critical care (cardiorespiratory) issues are suspected.
• Patients who need a prolonged time for recovery may benefit from consultation with a rehabilitation medicine specialist.

Activity

• When stabilized, activity with physical and/or occupational therapy should be encouraged. If motor deficits are profound, prevention of decubitus ulcers is highly important.
• In addition to the weakness, autonomic symptoms (eg, orthostatic hypotension) may also restrict activity and should be monitored.  

MEDICATION

Section 7 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

The goals of pharmacotherapy are to reduce morbidity and prevent complications. Intravenous immunoglobulin (IVIG) is the predominant choice in childhood Guillain-Barré syndrome (GBS). DVT prophylaxis should be targeted and gastritis stress symptoms may benefit from H2 blockers (eg, ranitidine).

Drug Category: Blood products

IVIG is an effective treatment of autoimmune neuropathies in general. It can reduce duration of hospitalization as well as need or duration for mechanical ventilation.

FOLLOW-UP

Section 8 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

Further Inpatient Care

• Careful attention should be paid to multiple issues that may require intervention and specialist consultation. Among the concerns of Guillain-Barré 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.
• In the long term, physical therapy may provide benefit to patients during the recovery phase of the illness.

Patient Education

• For excellent patient education resources, visit eMedicine's Brain and Nervous System Center. Also, see eMedicine's patient education article Guillain-Barré Syndrome.
• Family counseling and education can be very important early in the illness to set the tone for potential of prolonged and critical illness course.
• Other patient and family-oriented Web sites include the following:
• • NINDS Guillain-Barré Syndrome Information Page
  • GBS/CIDP Foundation International
  • MayoClinic.com, Guillain-Barré syndrome

MISCELLANEOUS

Section 9 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

Medical/Legal Pitfalls

• Respiratory compromise is the most concerning and life-threatening aspect of GBS in childhood. Recognition of early respiratory distress signs is absolutely vital. In the absence of respiratory distress signs in an outpatient scenario, giving appropriate instructions to patient families for "return criteria" to the emergency department is paramount.
• The challenge of GBS is the potential for progression from mild gait disturbance to the need for intubation for respiratory support. Many cases of childhood GBS will not require intubation but one cannot be certain without close observation and time. There are not clear early markers to stratify a child's risk of respiratory failure.
• As an inpatient, care should be to taken to monitor respiratory and cardiac function, especially in the acute, progressive stage of the disease.

ACKNOWLEDGMENTS

Section 10 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author, Kenneth J Mack, MD, PhD, to the development and writing of this article.

REFERENCES

Section 11 of 11

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

1. Nachamkin I, Barbosa PA, Ung H, Lobato C, Rivera AG, Rodriguez P. Patterns of Guillain-Barre syndrome in children: results from a Mexican population. Neurology. Oct 23 2007;69(17):1665-71. [Medline].
2. Korinthenberg R, Schessl J, Kirschner J. Clinical presentation and course of childhood Guillain-Barré syndrome: a prospective multicentre study. Neuropediatrics. Feb 2007;38(1):10-7. [Medline].
3. Shafqat S, Khealani BA, Awan F, Abedin SE. Guillain-Barré syndrome in Pakistan: similarity of demyelinating and axonal variants. Eur J Neurol. Jun 2006;13(6):662-5. [Medline].
4. Nishimoto Y, Susuki K, Yuki N. Serologic marker of acute motor axonal neuropathy in childhood. Pediatr Neurol. Jul 2008;39(1):67-70. [Medline].
5. Schessl J, Koga M, Funakoshi K, Kirschner J, Muellges W, Weishaupt A, et al. Prospective study on anti-ganglioside antibodies in childhood Guillain-Barré syndrome. Arch Dis Child. Jan 2007;92(1):48-52. [Medline].
6. Abd-Allah SA, Jansen PW, Ashwal S, Perkin RM. Intravenous immunoglobulin as therapy for pediatric Guillain-Barre syndrome. J Child Neurol. Sep 1997;12(6):376-80. [Medline].
7. Bradshaw DY, Jones HR. Guillain-Barre syndrome in children: clinical course, electrodiagnosis, and prognosis. Muscle Nerve. Apr 1992;15(4):500-6. [Medline].
8. Bril V, Ilse WK, Pearce R, et al. Pilot trial of immunoglobulin versus plasma exchange in patients with Guillain-Barre syndrome. Neurology. Jan 1996;46(1):100-3. [Medline].
9. Delanoe C, Sebire G, Landrieu P, et al. Acute inflammatory demyelinating polyradiculopathy in children: clinical and electrodiagnostic studies. Ann Neurol. Sep 1998;44(3):350-6. [Medline].
10. DiMario FJ. Intravenous immunoglobulin in the treatment of childhood Guillain-Barre syndrome: a randomized trial. Pediatrics. Jul 2005;116(1):226-8. [Medline].
11. England JD. Guillain-Barré syndrome. Annu Rev Med. 1990;41:1-6. [Medline].
12. Epstein MA, Sladky JT. The role of plasmapheresis in childhood Guillain-Barre syndrome. Ann Neurol. Jul 1990;28(1):65-9. [Medline].
13. Gorson KC, Ropper AH, Muriello MA, Blair R. Prospective evaluation of MRI lumbosacral nerve root enhancement in acute Guillain-Barre syndrome. Neurology. Sep 1996;47(3):813-7. [Medline].
14. Griffin JW, Li CY, Ho TW, et al. Guillain-Barre syndrome in northern China. The spectrum of neuropathological changes in clinically defined cases. Brain. Jun 1995;118 (Pt 3):577-95. [Medline].
15. Griffin JW, Li CY, Ho TW, et al. Pathology of the motor-sensory axonal Guillain-Barre syndrome. Ann Neurol. Jan 1996;39(1):17-28. [Medline].
16. Ho TW, Li CY, Cornblath DR. Patterns of recovery in the Guillain-Barre syndromes. Neurology. 1997;48:695-700. [Medline].
17. Ho TW, Mishu B, Li CY. Guillain-Barre syndrome in northern China. Relationship to Campylobacter jejuni infection and anti-glycolipid antibodies. Brain. Jun 1995;118 (Pt 3):597-605. [Medline].
18. Hughes RA. Ineffectiveness of high-dose intravenous methylprednisolone in Guillain-Barre syndrome. Lancet. Nov 2 1991;338(8775):1142. [Medline].
19. Hughes RA, Rees JH. Clinical and epidemiological features of Guillain-Barre syndrome. J Infect Dis. 1997;176:S92-8. [Medline].
20. Hung PL, Chang WN, Huang LT, et al. A clinical and electrophysiologic survey of childhood Guillain-Barre syndrome. Pediatr Neurol. Feb 2004;30(2):86-91. [Medline].
21. Jones HR. Childhood Guillain-Barre syndrome: clinical presentation, diagnosis and therapy. J Child Neurol. 1996;11:4-12. [Medline].
22. Korinthenberg R, Monting JS. Natural history and treatment effects in Guillain-Barre syndrome: a multicentre study. Arch Dis Child. Apr 1996;74(4):281-7. [Medline].
23. Korinthenberg R, Schessl J, Kirschner J, Monting JS. Intravenously administered immunoglobulin in the treatment of childhood Guillain-Barre syndrome: a randomized trial. Pediatrics. Jul 2005;116(1):8-14. [Medline].
24. Koul R, Chacko A, Ahmed R, et al. Ten-year prospective study (clinical spectrum) of childhood Guillain-Barre syndrome in the Arabian peninsula: comparison of outcome in patients in the pre- and post-intravenous immunoglobulin eras. J Child Neurol. Nov 2003;18(11):767-71. [Medline].
25. Lamont PJ, Johnston HM, Berdoukas VA. Plasmapheresis in children with Guillain-Barre syndrome. Neurology. Dec 1991;41(12):1928-31. [Medline].
26. Marks HG, Augustyn P, Allen RJ. Fisher's syndrome in children. Pediatrics. Nov 1977;60(5):726-9. [Medline].
27. McCarthy N, Andersson Y, Jormanainen V. The risk of Guillain-Barre syndrome following infection with Campylobacter jejuni. Epidemiol Infect. Feb 1999;122(1):15-7. [Medline].
28. Nagasawa K, Kuwabara S, Misawa S, et al. Electrophysiological subtypes and prognosis of childhood Guillain-Barre syndrome in Japan. Muscle Nerve. Feb 27 2006;[Medline].
29. Prevots DR, Sutter RW. Assessment of Guillain-Barre syndrome mortality and morbidity in the United States: implications for acute flaccid paralysis surveillance. J Infect Dis. Feb 1997;175 Suppl 1:S151-5. [Medline].
30. Ropper AH. The Guillain-Barre syndrome. N Engl J Med. Apr 23 1992;326(17):1130-6. [Medline].
31. Ropper AH, Kehne SM. Guillain-Barre syndrome: management of respiratory failure. Neurology. 1985;35:1662-5. [Medline].
32. Rostami AM. Pathogenesis of immune-mediated neuropathies. Pediatr Res. Jan 1993;33(1 Suppl):S90-4. [Medline].
33. Ryan MM. Guillain-Barre syndrome in childhood. J Paediatr Child Health. May-Jun 2005;41(5-6):237-41. [Medline].
34. Shanbag P, Amirtharaj C, Pathak A. Intravenous immunoglobulins in severe Guillian-Barre syndrome in childhood. Indian J Pediatr. 2003;70(7):541-3. [Medline].
35. Sladky JT. Guillain-Barre syndrome in children. J Child Neurol. Mar 2004;19(3):191-200. [Medline].
36. Stratton KR, Howe CJ, Johnston RB. Adverse events associated with childhood vaccines other than pertussis and rubella. Summary of a report from the Institute of Medicine. JAMA. May 25 1994;271(20):1602-5. [Medline].
37. van der Meche FG, Schmitz PI. A randomized trial comparing intravenous immune globulin and plasma exchange in Guillain-Barre syndrome. Dutch Guillain-Barre Study Group. N Engl J Med. Apr 23 1992;326(17):1123-9. [Medline].
38. Zafeiriou DI, Kontopoulos EE, Katzos GS, et al. Single dose immunoglobulin therapy for childhood Guillain-Barre syndrome. Brain Dev. Jul 1997;19(5):323-5. [Medline].

Article Last Updated: Sep 18, 2008