eMedicine Specialties > Physical Medicine and Rehabilitation > Peripheral Neuropathy

Guillain-Barre Syndrome

Author: Heather Rachel Davids, MD, Physician, Department of Anesthesiology, Interventional Pain Medicine, University of Colorado Health Sciences Center
Coauthor(s): Joyce L Oleszek, MD, Assistant Professor, Department of Physical Medicine and Rehabilitation, University of Colorado at Denver Health Sciences Center, The Children's Hospital of Denver; Angela Cha-Kim, MD, Director of Spinal Cord Injury, Assistant Professor, Department of Physical Medicine and Rehabilitation, Loma Linda University Medical Center
Contributor Information and Disclosures

Updated: Sep 15, 2009

Introduction

Background

Guillain-Barré syndrome (GBS) may be described as a collection of clinical syndromes that manifests as an acute inflammatory polyradiculoneuropathy with resultant weakness and diminished reflexes. With poliomyelitis under control in developed countries, GBS is now the most important cause of acute flaccid paralysis. GBS remains a diagnosis made primarily through the assessment of clinical history and findings.

Although the classic description of GBS is that of a demyelinating neuropathy with ascending weakness, many clinical variants have been well documented in the medical literature. Acute inflammatory demyelinating polyradiculoneuropathy (AIDP) is the most widely recognized form in Western countries, but the variants known as acute motor axonal neuropathy (AMAN) and acute motor-sensory axonal neuropathy (AMSAN) also are well recognized. Based on a clinical spectrum of symptoms and findings, many believe that strictly defined subgroups of GBS, although not easily distinguished, nonetheless exist.

Pathophysiology

GBS is a postinfectious, immune-mediated disease. Cellular and humoral immune mechanisms probably play a role in its development. Most patients report an infectious illness in the weeks prior to the onset of GBS. Many of the identified infectious agents are thought to induce antibody production against specific gangliosides and glycolipids, such as GM1 and GD1b, distributed throughout the myelin in the peripheral nervous system.1

The pathophysiologic mechanism of an antecedent illness and of GBS can be typified by Campylobacter jejuni infections.2,3 The virulence of C jejuni is thought to be based on the presence of specific antigens in its capsule that are shared with nerves. Immune responses directed against the capsular components produce antibodies that cross-react with myelin to cause demyelination. Ganglioside GM1 appears to cross-react with C jejuni lipopolysaccharide antigens, resulting in the immunologic damage to the peripheral nervous system. This process has been termed molecular mimicry.

Pathologic findings in GBS include lymphocytic infiltration of spinal roots and peripheral nerves, followed by macrophage-mediated, multifocal stripping of myelin. This phenomenon results in defects in the propagation of electrical nerve impulses, with eventual conduction block and flaccid paralysis. In some patients with severe disease, a secondary consequence of the severe inflammation is axonal disruption and loss. A subgroup of patients may have a primary immune attack directly against nerve axons, resulting in a similar clinical presentation.

Variants

Several variants of GBS are recognized. These disorders share similar patterns of evolution, recovery, symptom overlap, and probable immune-mediated pathogenesis.

  • The Miller-Fisher syndrome, a common variant of GBS, is observed in about 5% of all GBS cases. The syndrome consists of ataxia, ophthalmoplegia, and areflexia.4 Ataxia is primarily noted during gait and in the trunk, with lesser involvement of the limbs. Motor strength is characteristically spared. The usual course is one of gradual and complete recovery over weeks or months. A close association exists between antiganglioside antibodies and the Fisher variant. Anti-GQ1b antibodies, triggered by certain C jejuni strains, have a relatively high specificity and sensitivity for the disease.5 Dense concentrations of GQ1b ganglioside are found in the oculomotor, trochlear, and abducens nerves, which may explain the relationship between anti-GQ1b antibodies and ophthalmoplegia.
  • The AMAN variant is associated closely with enteric C jejuni infections and high titers of antibodies to gangliosides (ie, GM1, GD1a, GD1b). Patients with AMAN have pure motor symptoms and appear clinically to be very similar to patients with the demyelinating form of GBS with ascending, symmetric paralysis. AMAN is distinguished by electrodiagnostic study results that are consistent with a pure motor axonopathy.6 Biopsies show wallerianlike degeneration without significant lymphocytic inflammation. Many cases have been reported in rural areas of China, especially in children and young adults during the summer months.7 Pure axonal cases may occur more frequently outside of Europe and North America. AMAN cases may also be different from cases of axonal GBS described in the West. Prognosis is often quite favorable. Although recovery for many is rapid, severely disabled patients with AMAN may show improvement over a period of years.
  • The axonal form of GBS, also referred to as acute motor-sensory axonal neuropathy (AMSAN), often presents with rapid and severe paralysis with delayed and poorer recovery (in comparison with recovery from electrophysiologically similar AMAN cases). Like AMAN, axonal GBS also is associated with preceding C jejuni diarrhea. Pathologic findings show severe axonal degeneration of motor and sensory nerve fibers with little demyelination.8
  • A pure sensory variant of GBS has been described in the medical literature, typified by a rapid onset of sensory loss and areflexia in a symmetric and widespread pattern. Lumbar puncture studies show albuminocytologic dissociation in the cerebrospinal fluid (CSF), and electromyography (EMG) results show characteristic signs of a demyelinating process in the peripheral nerves. Prognosis is generally good, but immunotherapies, such as plasma exchange and the administration of intravenous immunoglobulins (IVIGs), can be tried in patients with severe disease or slow recovery.
  • Acute pandysautonomia without significant motor or sensory involvement is a rare presentation of GBS. Dysfunction of the sympathetic and parasympathetic systems results in severe postural hypotension, bowel and bladder retention, anhidrosis, decreased salivation and lacrimation, and pupillary abnormalities.
  • The pharyngeal-cervical-brachial variant is distinguished by isolated facial, oropharyngeal, cervical, and upper limb weakness without lower limb involvement. Other unusual clinical variants with restricted patterns of weakness are observed only in rare cases.

Frequency

United States

According to an epidemiologic survey, the average annual incidence of GBS in the United States is 3.0 cases per 100,000 population. In comparing age groups, the annual mean rate of hospitalizations in the United States related to GBS increases with age, being 1.5 cases per 100,000 population in persons aged less than 15 years and peaking at 8.6 cases per 100,000 population in persons aged 70-79 years.9

International

A widespread syndrome, GBS has been reported throughout the world.10 Most studies show annual incidence figures that are similar to those in the United States, without geographical clustering.

Mortality/Morbidity

In epidemiologic surveys, the overall death rate related to GBS ranges from 2-12% of patients. GBS-associated mortality rates increase markedly with age. In the United States, the case-fatality ratio ranges from 0.7% among persons younger than 15 years to 8.6% among individuals older than 65 years. Survey data has shown that in patients aged 60 years or older, the risk of death is 6-fold that of persons aged 40-59 years and is 157-fold that of patients younger than 15 years. Although the death rate increases with age in males and females, after age 40 years males have a death rate that is 1.3 times greater than that of females.

GBS-related deaths usually occur in ventilator-dependent patients, resulting from such complications as pneumonia, sepsis, adult respiratory distress syndrome, and, less frequently, autonomic dysfunction.11 Underlying pulmonary disease and the need for mechanical ventilation increase the risk of death, especially in elderly patients. Length of hospital stays also increases with advancing age, because of disease severity and associated medical complications.

Race

GBS has been reported throughout the international community. In North America, Western Europe, and Australia, most patients with GBS meet electrophysiologic criteria for demyelinating polyneuropathy. In northern China, up to 65% of patients with GBS have axonal pathology.7

Sex

A slight male preponderance is seen in most studies, especially in older patients.

Age

GBS has been reported in all age groups, with the syndrome occurring at any time between infancy and old age. In the United States, the syndrome's age distribution seems to be bimodal, with the incidence of GBS peaking in the elderly population and reaching its second-highest level in young adults. Infants appear to have the lowest risk of developing GBS.

Clinical

History

  • Antecedent illness
    • Up to two thirds of patients with GBS report an antecedent illness or event 1-3 weeks prior to the onset of weakness. Upper respiratory and gastrointestinal illnesses are the most commonly reported conditions.12,13 Symptoms generally have resolved by the time of medical presentation for the neurologic condition.
    • C jejuni is the major causative organism that is identified in most studies and is responsible for AIDP and AMAN cases. In one major study, previous diarrheal illness had occurred in 60% of patients with axonal GBS (by neurophysiologic testing).
    • Vaccinations, surgical procedures, and trauma have been reported to trigger the development of GBS.14 Much of this information is anecdotal, although vaccination with the swine flu vaccine (administered in 1976) was shown to increase the risk of contracting GBS to a small, but definable, degree. Rabies vaccine prepared from infected brain tissue also was found to have an association with GBS. Studies of other vaccines, however, have not shown a significant relationship between these drugs and GBS.
  • Weakness
    • The classic clinical picture of weakness is ascending and symmetrical in nature. The lower limbs are usually involved before the upper limbs. Proximal muscles may be involved earlier than the more distal ones. Trunk, bulbar, and respiratory muscles can be affected as well.
    • Weakness develops acutely and progresses over days to weeks. Severity may range from mild weakness to complete tetraplegia with ventilatory failure. Peak deficits are reached by 4 weeks after the initial development of symptoms.15 The progression of symptoms beyond that point brings the diagnosis under question. Recovery usually begins 2-4 weeks after the progression ceases.16
  • Sensory changes
    • Most patients complain of paresthesias, numbness, or similar sensory changes. Sensory symptoms often precede the weakness. They are frequently ascending in nature and are more pronounced in a distal distribution.
    • Sensory symptoms are usually mild. In most cases, objective findings of sensory loss tend to be minimal and variable.
    • On nerve conduction studies (NCS), 58-76% of patients exhibit sensory abnormalities.
  • Cranial nerve involvement
    • Cranial nerve involvement is observed in 45-75% of patients with GBS. Common complaints may include the following:
      • Facial droop
      • Diplopias
      • Dysarthria
      • Dysphagia
    • Facial and oropharyngeal weakness usually appears after the trunk and limbs are affected.
  • Pain
    • In a prospective, longitudinal study of pain in patients with GBS, 89% of patients reported pain that was attributable to GBS at some time during their illness. On initial presentation, almost 50% of patients described the pain as severe and distressing.
    • The mechanism of pain is uncertain and may be a product of several factors. Pain can result from direct nerve injury or from the paralysis and prolonged immobilization.
    • Most patients complain of back and leg pain, often described as aching or throbbing in nature. The mechanism of pain is thought to be inflamed nerve roots. Dysesthetic symptoms are observed in approximately 50% of patients during the course of their illness. Dysesthesias frequently are described as burning, tingling, or shocklike sensations and are often more prevalent in the lower extremities than in the upper extremities. Dysesthesias may persist indefinitely in 5-10% of patients. Other pain syndromes in GBS include the following:
      • Myalgic complaints, with cramping and local muscle tenderness
      • Visceral pain
      • Pain associated with conditions of immobility (eg, pressure nerve palsies, decubitus ulcers)
    • The intensity of pain on admission correlates poorly with neurologic disability on admission and with the end outcome.
  • Autonomic changes
    • Autonomic nervous system involvement with dysfunction in the sympathetic and parasympathetic systems can be observed in patients with GBS.
    • Autonomic changes can include the following:
      • Tachycardia
      • Bradycardia
      • Facial flushing
      • Paroxysmal hypertension
      • Orthostatic hypotension
      • Anhidrosis and/or diaphoresis
    • Urinary retention and paralytic ileus also can be observed. Bowel and bladder dysfunction rarely presents as an early symptom or persists for a significant period of time.
    • Dysautonomia is more frequent in patients with severe weakness and respiratory failure.
    • Autonomic changes rarely persist in a patient with GBS.
  • Respiratory involvement
    • Upon presentation, 40% of patients have respiratory or oropharyngeal weakness.
    • Typical complaints include the following:
      • Dyspnea on exertion
      • Shortness of breath
      • Difficulty swallowing
      • Slurred speech
    • Ventilatory failure with required respiratory support is observed in up to one third of patients at some time during the course of their disease.

Physical

  • Vital signs
    • Cardiac arrhythmias, including tachycardias and bradycardias, can be observed as a result of autonomic nervous system involvement.
  • Tachypnea may be a sign of ongoing dyspnea and progressive respiratory failure.
  • Blood pressure lability is another common feature with alterations between hypertension and hypotension.
  • Cranial nerves
    • Facial weakness (cranial nerve VII) is observed most frequently, followed by symptoms associated with cranial nerves VI, III, XII, V, IX, and X. Involvement of facial, oropharyngeal, and ocular muscles results in facial droop, dysphagia, dysarthria, and findings associated with disorders of the eye.
    • Ophthalmoparesis may be observed in up to 25% of patients with GBS. Limitation of eye movement most commonly results from a symmetric palsy associated with cranial nerve VI. Ptosis from cranial nerve III (oculomotor) palsy also is often associated with limited eye movements. Pupillary abnormalities, especially those accompanying ophthalmoparesis, are relatively common as well.
  • Motor examination
    • Lower extremity weakness usually begins first and ascends symmetrically and progressively over the first several days.
    • Upper extremity, trunk, facial, and oropharyngeal weakness is observed to a variable extent.
    • Marked asymmetric weakness calls the diagnosis of GBS into question.
  • Sensory examination
    • Despite frequent complaints of paresthesias, objective sensory changes are minimal.
    • A well-demarcated sensory level should not be observed in patients with GBS; such a finding calls the diagnosis of GBS into question.
  • Reflex changes
    • Reflexes are absent or hyporeflexic early in the disease course and represent a major clinical finding on examination of the patient with GBS.
    • Pathologic reflexes, such as the Babinski sign, are absent.
    • Hypotonia can be observed with significant weakness.

Causes

GBS is considered to be a postinfectious, immune-mediated disease targeting peripheral nerves. Up to two thirds of patients report an antecedent illness prior to the onset of neurologic symptoms.12,17 Respiratory infections are most frequently reported, followed by gastrointestinal infections.13

In several studies, C jejuni was the most commonly isolated pathogen. Serology studies in a Dutch GBS trial identified 32% of patients as having had a recent C jejuni infection, while studies in northern China documented infection rates as high as 60%.7,18 Gastrointestinal and upper respiratory tract symptoms can be observed with C jejuni infections. C jejuni infections can also have a subclinical course, resulting in patients with no reported infectious symptoms prior to development of GBS. Patients who develop GBS following an antecedent C jejuni infection often have a more severe course, with rapid progression and a prolonged, incomplete recovery. A strong clinical association has been noted between C jejuni infections and the pure motor and axonal forms of GBS.

The virulence of C jejuni is thought to be based on the presence of specific antigens in its capsule that are shared with nerves. Immune responses directed against capsular lipopolysaccharides produce antibodies that cross-react with myelin to cause demyelination. C jejuni infections demonstrate significant association with antibodies against gangliosides GM1 and GD1b. Although GM1 antibodies can be found with demyelinating GBS, GM1 antibodies are more common in the axonal and inexcitable groups. Even in the subgroup of patients with GM1 antibodies, however, the clinical manifestations vary. Host susceptibility is probably one determinant in the development of GBS after infectious illness.18

Cytomegalovirus (CMV) infections are the second most commonly found infections preceding GBS; they account for the most common viral triggers of GBS. The aforementioned Dutch GBS study found CMV to be present in 13% of patients.19 CMV infections present as upper respiratory tract infections, pneumonias, and nonspecific, flulike illnesses. GBS patients with preceding CMV infections often have prominent involvement of the sensory and cranial nerves. CMV infections are significantly associated with antibodies against the ganglioside GM2.

Other significant, although less frequently identified, infectious agents in GBS patients include Epstein-Barr virus (EBV), Mycoplasma pneumoniae, and Varicella-Zoster virus. An association between GBS and human immunodeficiency virus (HIV) also is well recognized. Infections with Haemophilus influenzae, para-influenza virus type 1, influenza A virus, influenza B virus, adenovirus, and herpes simplex virus have been demonstrated in patients with GBS, although not more frequently than they have in controls.20

Various events, such as surgery, trauma, and pregnancy, have been reported as possible triggers of GBS, but these associations remain mostly anecdotal in the medical literature. Vaccinations also have been linked to GBS,14 by temporal association. In most cases, however, no definite causal relation has been established between vaccines and GBS (with the exception of rabies vaccine prepared from infected brain tissue and the aforementioned swine flu vaccine, which was administered in 1976). Subsequent surveys have found no significantly increased incidence of GBS after vaccination programs.20

More on Guillain-Barre Syndrome

Overview: Guillain-Barre Syndrome
Differential Diagnoses & Workup: Guillain-Barre Syndrome
Treatment & Medication: Guillain-Barre Syndrome
Follow-up: Guillain-Barre Syndrome
References
Further Reading
[ CLOSE WINDOW ]

References

  1. Jacobs BC, Koga M, van Rijs W, et al. Subclass IgG to motor gangliosides related to infection and clinical course in Guillain-Barré syndrome. J Neuroimmunol. Jan 4 2008;[Medline].

  2. Jacobs BC, van Doorn PA, Schmitz PI, et al. Campylobacter jejuni infections and anti-GM1 antibodies in Guillain-Barré syndrome. Ann Neurol. Aug 1996;40(2):181-7. [Medline].

  3. Koga M, Takahashi M, Masuda M, et al. Campylobacter gene polymorphism as a determinant of clinical features of Guillain-Barre syndrome. Neurology. Nov 8 2005;65(9):1376-81.

  4. Fisher M. An unusual variant of acute immune polyneuritis (syndrome of ophthalmoplegia, ataxia, and areflexia). N Engl J Med. 1956;255:57-65.

  5. Chiba A, Kusunoki S, Obata H, et al. Serum anti-GQ1b IgG antibody is associated with ophthalmoplegia in Miller Fisher syndrome and Guillain-Barre syndrome: clinical and immunohistochemical studies. Neurology. Oct 1993;43(10):1911-7. [Medline].

  6. Asbury AK, Cornblath DR. Assessment of current diagnostic criteria for Guillain-Barre syndrome. Ann Neurol. 1990;27 Suppl:S21-4. [Medline].

  7. Ho TW, Mishu B, Li CY, et al. Guillain-Barre syndrome in northern China. Relationship to Campylobacter jejuni infection and anti-glycolipid antibodies. Brain. Jun 1995;118 ( Pt 3):597-605. [Medline].

  8. Brown WF, Feasby TE, Hahn AF. Electrophysiological changes in the acute "axonal" form of Guillain-Barre syndrome. Muscle Nerve. Feb 1993;16(2):200-5. [Medline].

  9. 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].

  10. Kushnir M, Klein C, Pollak L, et al. Evolving pattern of Guillain-Barre syndrome in a community hospital in Israel. Acta Neurol Scand. Nov 8 2007;[Medline].

  11. Fletcher DD, Lawn ND, Wolter TD, et al. Long-term outcome in patients with Guillain-Barré syndrome requiring mechanical ventilation. Neurology. Jun 27 2000;54(12):2311-5. [Medline].

  12. Baravelli M, Fantoni C, Rossi A, et al. Guillain-Barré syndrome as a neurological complication of infective endocarditis. Is it really so rare and how often do we recognise it?. Int J Cardiol. Jan 10 2008;[Medline].

  13. Nelson L, Gormley R, Riddle MS, et al. The epidemiology of Guillain-Barré Syndrome in U.S. military personnel: a case-control study. BMC Res Notes. Aug 26 2009;2:171. [Medline]. [Full Text].

  14. Souayah N, Nasar A, Suri MF, et al. Guillain-Barré syndrome after vaccination in United States: data from the Centers for Disease Control and Prevention/Food and Drug Administration Vaccine Adverse Event Reporting System (1990-2005). J Clin Neuromuscul Dis. Sep 2009;11(1):1-6. [Medline].

  15. Hughes RA, Rees JH. Clinical and epidemiologic features of Guillain-Barre syndrome. J Infect Dis. Dec 1997;176 Suppl 2:S92-8. [Medline].

  16. El Mhandi L, Calmels P, Camdessanché JP, et al. Muscle strength recovery in treated Guillain-Barré syndrome: a prospective study for the first 18 months after onset. Am J Phys Med Rehabil. Sep 2007;86(9):716-24. [Medline].

  17. Jacobs BC, Rothbarth PH, van der Meche FG, et al. The spectrum of antecedent infections in Guillain-Barre syndrome: a case-control study. Neurology. Oct 1998;51(4):1110-5. [Medline].

  18. van der Meche FG, Visser LH, Jacobs BC, et al. Guillain-Barre syndrome: multifactorial mechanisms versus defined subgroups. J Infect Dis. Dec 1997;176 Suppl 2:S99-102. [Medline].

  19. Visser LH, van der Meche FG, Meulstee J, et al. Cytomegalovirus infection and Guillain-Barre syndrome: the clinical, electrophysiologic, and prognostic features. Dutch Guillain-Barre Study Group. Neurology. Sep 1996;47(3):668-73. [Medline].

  20. Winer JB, Hughes RA, Anderson MJ, et al. A prospective study of acute idiopathic neuropathy. II. Antecedent events. J Neurol Neurosurg Psychiatry. May 1988;51(5):613-8. [Medline].

  21. Kusunoki S. Antiglycolipid antibodies in Guillain-Barre syndrome and autoimmune neuropathies. Am J Med Sci. Apr 2000;319(4):234-9. [Medline].

  22. Albers JW, Kelly JJ Jr. Acquired inflammatory demyelinating polyneuropathies: clinical and electrodiagnostic features. Muscle Nerve. Jun 1989;12(6):435-51. [Medline].

  23. Hughes RA, Wijdicks EF, Benson E, et al. Supportive care for patients with Guillain-Barre syndrome. Arch Neurol. Aug 2005;62(8):1194-8. [Medline]. [Full Text].

  24. Dalakas MC. Intravenous immunoglobulin in the treatment of autoimmune neuromuscular diseases: present status and practical therapeutic guidelines. Muscle Nerve. Nov 1999;22(11):1479-97. [Medline].

  25. Hadden RD, Cornblath DR, Hughes RA, et al. Electrophysiological classification of Guillain-Barre syndrome: clinical associations and outcome. Plasma Exchange/Sandoglobulin Guillain-Barre Syndrome Trial Group. Ann Neurol. Nov 1998;44(5):780-8. [Medline].

  26. Randomised trial of plasma exchange, intravenous immunoglobulin, and combined treatments in Guillain-Barré syndrome. Plasma Exchange/Sandoglobulin Guillain-Barré Syndrome Trial Group. Lancet. Jan 25 1997;349(9047):225-30. [Medline].

  27. 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].

  28. Visser LH, van der Meché FG, Meulstee J, et al. Risk factors for treatment related clinical fluctuations in Guillain-Barré syndrome. Dutch Guillain-Barré study group. J Neurol Neurosurg Psychiatry. Feb 1998;64(2):242-4. [Medline].

  29. Brannagan TH 3rd, Nagle KJ, Lange DJ, et al. Complications of intravenous immune globulin treatment in neurologic disease. Neurology. Sep 1996;47(3):674-7. [Medline].

  30. Paran D, Herishanu Y, Elkayam O, et al. Venous and arterial thrombosis following administration of intravenous immunoglobulins. Blood Coagul Fibrinolysis. Jul 2005;16(5):313-8. [Medline].

  31. van Doorn PA. What's new in Guillain-Barré syndrome in 2007-2008?. J Peripher Nerv Syst. Jun 2009;14(2):72-4. [Medline].

  32. Garssen MP, Blok JH, van Doorn PA, et al. Conduction velocity distribution in neurologically well-recovered but fatigued Guillain-Barré syndrome patients. Muscle Nerve. Feb 2006;33(2):177-82. [Medline].

  33. Garssen MP, Schmitz PI, Merkies IS, et al. Amantadine for treatment of fatigue in Guillain-Barre syndrome: a randomised, double blind, placebo controlled, crossover trial. J Neurol Neurosurg Psychiatry. Jan 2006;77(1):61-5. [Medline].

  34. Bernsen RA, de Jager AE, Schmitz PI, et al. Residual physical outcome and daily living 3 to 6 years after Guillain-Barré syndrome. Neurology. Jul 22 1999;53(2):409-10. [Medline].

  35. Bernsen RA, de Jager AE, van der Meché FG, et al. How Guillain-Barre patients experience their functioning after 1 year. Acta Neurol Scand. Jul 2005;112(1):51-6. [Medline].

  36. Bernsen RA, Jacobs HM, de Jager AE, et al. Residual health status after Guillain-Barré syndrome. J Neurol Neurosurg Psychiatry. Jun 1997;62(6):637-40. [Medline].

  37. Romano JG, Rotta FT, Potter P, et al. Relapses in the Guillain-Barre syndrome after treatment with intravenous immune globulin or plasma exchange. Muscle Nerve. Oct 1998;21(10):1327-30. [Medline].

  38. Appropriate number of plasma exchanges in Guillain-Barré syndrome. The French Cooperative Group on Plasma Exchange in Guillain-Barré Syndrome. Ann Neurol. Mar 1997;41(3):298-306. [Medline].

  39. Winer JB, Hughes RA, Osmond C. A prospective study of acute idiopathic neuropathy. I. Clinical features and their prognostic value. J Neurol Neurosurg Psychiatry. May 1988;51(5):605-12. [Medline].

  40. Petzold A, Brettschneider J, Jin K, et al. CSF protein biomarkers for proximal axonal damage improve prognostic accuracy in the acute phase of Guillain-Barré syndrome. Muscle Nerve. Jul 2009;40(1):42-9. [Medline].

  41. Visser LH, Schmitz PI, Meulstee J, et al. Prognostic factors of Guillain-Barre syndrome after intravenous immunoglobulin or plasma exchange. Dutch Guillain-Barre Study Group. Neurology. Aug 11 1999;53(3):598-604. [Medline].

  42. Agrawal S, Peake D, Whitehouse WP. Management of children with Guillain-Barré syndrome. Arch Dis Child Educ Pract Ed. Dec 2007;92(6):161-8. [Medline].

  43. Cochen V, Arnulf I, Demeret S, et al. Vivid dreams, hallucinations, psychosis and REM sleep in Guillain-Barré syndrome. Brain. Nov 2005;128(Pt 11):2535-45. [Medline]. [Full Text].

  44. Cornblath DR. Electrophysiology in Guillain-Barre syndrome. Ann Neurol. 1990;27 Suppl:S17-20. [Medline].

  45. Criteria for diagnosis of Guillain-Barré syndrome. Ann Neurol. Jun 1978;3(6):565-6. [Medline].

  46. Cruz J. Neurologic and Neurosurgical Emergencies. Philadelphia, Pa: WB Saunders; 1998.

  47. Guillain-Barre Syndrome Steroid Trial Group. Double-blind trial of intravenous methylprednisolone in Guillain-Barre syndrome. Lancet. Mar 6 1993;341(8845):586-90. [Medline].

  48. Hadden RD, Hughes RA. Treatment of immune-mediated inflammatory neuropathies. Curr Opin Neurol. Oct 1999;12(5):573-9. [Medline].

  49. Hafer-Macko C, Hsieh ST, Li CY, et al. Acute motor axonal neuropathy: an antibody-mediated attack on axolemma. Ann Neurol. Oct 1996;40(4):635-44. [Medline].

  50. Hahn AF. Guillain-Barre syndrome. Lancet. Aug 22 1998;352(9128):635-41. [Medline].

  51. Hiraga A, Mori M, Ogawara K, et al. Recovery patterns and long term prognosis for axonal Guillain-Barré syndrome. J Neurol Neurosurg Psychiatry. May 2005;76(5):719-22. [Medline].

  52. Ho T, Griffin J. Guillain-Barre syndrome. Curr Opin Neurol. Aug 1999;12(4):389-94. [Medline].

  53. Lawn ND, Wijdicks EF. Fatal Guillain-Barre syndrome. Neurology. Feb 1999;52(3):635-8. [Medline].

  54. Lawn ND, Wijdicks EF. Post-intubation pulmonary function test in Guillain-Barre syndrome. Muscle Nerve. Apr 2000;23(4):613-6. [Medline].

  55. Lu JL, Sheikh KA, Wu HS, et al. Physiologic-pathologic correlation in Guillain-Barre syndrome in children. Neurology. Jan 11 2000;54(1):33-9. [Medline].

  56. Matthews PM, Arnold DL. Diagnostic Tests in Neurology. New York, NY: Churchill Livingston; 1991.

  57. McKhann GM. Guillain-Barre syndrome: clinical and therapeutic observations. Ann Neurol. 1990;27 Suppl:S13-6. [Medline].

  58. McKhann GM, Cornblath DR, Griffin JW, et al. Acute motor axonal neuropathy: a frequent cause of acute flaccid paralysis in China. Ann Neurol. Apr 1993;33(4):333-42. [Medline].

  59. McKhann GM, Griffin JW, Cornblath DR, et al. Plasmapheresis and Guillain-Barre syndrome: analysis of prognostic factors and the effect of plasmapheresis. Ann Neurol. Apr 1988;23(4):347-53. [Medline].

  60. Meulstee J, van der Meche FG. Electrodiagnostic criteria for polyneuropathy and demyelination: application in 135 patients with Guillain-Barre syndrome. Dutch Guillain-Barre Study Group. J Neurol Neurosurg Psychiatry. Nov 1995;59(5):482-6. [Medline].

  61. Meythaler JM. Rehabilitation of Guillain-Barre syndrome. Arch Phys Med Rehabil. Aug 1997;78(8):872-9. [Medline].

  62. Meythaler JM, DeVivo MJ, Braswell WC. Rehabilitation outcomes of patients who have developed Guillain-Barre syndrome. Am J Phys Med Rehabil. Sep-Oct 1997;76(5):411-9. [Medline].

  63. Mokrzycki MH, Kaplan AA. Therapeutic plasma exchange: complications and management. Am J Kidney Dis. Jun 1994;23(6):817-27. [Medline].

  64. Moulin DE. Pain in central and peripheral demyelinating disorders. Neurol Clin. Nov 1998;16(4):889-98. [Medline].

  65. Moulin DE, Hagen N, Feasby TE, et al. Pain in Guillain-Barre syndrome. Neurology. Feb 1997;48(2):328-31. [Medline].

  66. Bradley WG, Daroff RB, Fenichel GM, et al, eds. Neurology in Clinical Practice. 3rd ed. Boston, Mass: Butterworth-Heinemann; 2000.

  67. Oh SJ, LaGanke C, Claussen GC. Sensory Guillain-Barre syndrome. Neurology. Jan 9 2001;56(1):82-6. [Medline].

  68. Perry JR, Fung A, Poon P, et al. Magnetic resonance imaging of nerve root inflammation in the Guillain-Barré syndrome. Neuroradiology. 1994;36(2):139-40. [Medline].

  69. Braddom RL, ed. Physical Medicine and Rehabilitation. Philadelphia, Pa: WB Saunders; 1996.

  70. Rees JH, Gregson NA, Hughes RA. Anti-ganglioside GM1 antibodies in Guillain-Barre syndrome and their relationship to Campylobacter jejuni infection. Ann Neurol. Nov 1995;38(5):809-16. [Medline].

  71. Rees JH, Soudain SE, Gregson NA, et al. Campylobacter jejuni infection and Guillain-Barré syndrome. N Engl J Med. Nov 23 1995;333(21):1374-9. [Medline].

  72. Ropper AH. The Guillain-Barre syndrome. N Engl J Med. Apr 23 1992;326(17):1130-6. [Medline].

  73. Sater RA, Rostami A. Treatment of Guillain-Barre syndrome with intravenous immunoglobulin. Neurology. Dec 1998;51(6 Suppl 5):S9-15. [Medline].

  74. Sheikh KA, Nachamkin I, Ho TW, et al. Campylobacter jejuni lipopolysaccharides in Guillain-Barre syndrome: molecular mimicry and host susceptibility. Neurology. Aug 1998;51(2):371-8. [Medline].

  75. Weinberg DH. AAEM case report 4: Guillain-Barre syndrome. American Association of Electrodiagnostic Medicine. Muscle Nerve. Feb 1999;22(2):271-81. [Medline].

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Keywords

Guillain-Barré syndrome, Guillain-Barre syndrome, Guillain-Barre, demyelination, Campylobacter jejuni, demyelinating neuropathy, polyradiculoneuropathy, GBS, C jejuni, acute inflammatory demyelinating polyradiculoneuropathy, Landry-Guillain-Barre syndrome, Landry-Guillain-Barre-Strohl syndrome, acute demyelinating neuropathy, infectious polyneuritis, acute polyradiculoneuritis, axonal Guillain-Barre syndrome, acute motor axonal neuropathy, acute motor-sensory axonal neuropathy, Miller-Fisher syndrome, pharyngeal-cervical-brachial GBS

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Contributor Information and Disclosures

Author

Heather Rachel Davids, MD, Physician, Department of Anesthesiology, Interventional Pain Medicine, University of Colorado Health Sciences Center
Heather Rachel Davids, MD is a member of the following medical societies: American Academy of Pain Medicine, American Academy of Physical Medicine and Rehabilitation, and Association of Academic Physiatrists
Disclosure: Nothing to disclose.

Coauthor(s)

Joyce L Oleszek, MD, Assistant Professor, Department of Physical Medicine and Rehabilitation, University of Colorado at Denver Health Sciences Center, The Children's Hospital of Denver
Joyce L Oleszek, MD is a member of the following medical societies: American Academy of Physical Medicine and Rehabilitation
Disclosure: Nothing to disclose.

Angela Cha-Kim, MD, Director of Spinal Cord Injury, Assistant Professor, Department of Physical Medicine and Rehabilitation, Loma Linda University Medical Center
Angela Cha-Kim, MD is a member of the following medical societies: American Academy of Physical Medicine and Rehabilitation and American Paraplegia Society
Disclosure: Nothing to disclose.

Medical Editor

Daniel D Scott, MD, MA, Associate Professor, Department of Physical Medicine and Rehabilitation, University of Colorado at Denver and Health Sciences Center
Daniel D Scott, MD, MA is a member of the following medical societies: Alpha Omega Alpha, American Academy of Physical Medicine and Rehabilitation, American Association of Neuromuscular and Electrodiagnostic Medicine, American Paraplegia Society, Association of Academic Physiatrists, National Multiple Sclerosis Society, and Physiatric Association of Spine, Sports and Occupational Rehabilitation
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Michael T Andary, MD, MS, Residency Program Director, Professor, Department of Physical Medicine and Rehabilitation, Michigan State University College of Osteopathic Medicine
Michael T Andary, MD, MS is a member of the following medical societies: American Academy of Physical Medicine and Rehabilitation, American Association of Neuromuscular and Electrodiagnostic Medicine, American Medical Association, and Association of Academic Physiatrists
Disclosure: allergan Honoraria Speaking and teaching; Pfizer Honoraria Speaking and teaching

CME Editor

Kelly L Allen, MD, Regional Medical Director, IMX-Medical Management Services
Disclosure: Nothing to disclose.

Chief Editor

Robert H Meier III, MD, Director, Amputee Services of America; Active Medical Staff, Presbyterian/St Luke's Hospital, Spalding Rehabilitation Hospital, Select Specialty Hospital; Consulting Staff, Kindred Hospital
Robert H Meier III, MD is a member of the following medical societies: American Academy of Physical Medicine and Rehabilitation and Association of Academic Physiatrists
Disclosure: Nothing to disclose.

 
 
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