Guillain-Barré syndrome

Last updated: November 1, 2023

Summarytoggle arrow icon

Guillain-Barré syndrome (GBS) is an acute immune-mediated polyneuropathy that typically manifests with bilateral ascending flaccid paralysis and sensory involvement, e.g., paresthesia. The pathogenesis of GBS involves autoantibodies against antigens in the myelin sheath, other Schwann-cell antigens, and axon membranes. Approximately 65% of patients have an upper respiratory tract or gastrointestinal infection before the onset of GBS symptoms. Diagnosis is primarily clinical; alternative diagnoses should be considered especially in patients with atypical presentations. Cerebrospinal fluid (CSF) analysis showing albuminocytologic dissociation and electrodiagnostic study findings help support the diagnosis. Medical treatment depends on the severity of symptoms; available treatment options are intravenous immunoglobulin (IVIg) and plasmapheresis. Close monitoring is required for all patients. Complications such as respiratory failure, pulmonary embolism, and/or cardiac arrest increase mortality. Up to 20% of patients remain severely disabled and approximately 5% of patients die despite medical treatment.

Epidemiologytoggle arrow icon

  • Incidence [1]
    • ∼ 1 case per 100,000 individuals
    • Adults are affected more frequently than children.
  • Sex: > (1.5:1) [2]

Epidemiological data refers to the US, unless otherwise specified.

Etiologytoggle arrow icon

Pathophysiologytoggle arrow icon

Clinical featurestoggle arrow icon

Disease extent and severity in GBS vary widely, from mild muscle weakness to quadriplegia and respiratory failure.

Typical disease progression

  • Onset is acute or subacute, and symptoms progress rapidly.
  • Symptoms peak at ∼ 2–4 weeks and then plateau over months or years, followed by partial or complete resolution. [2][3]

Typical presentation

Classic sensorimotor features are observed in most patients with GBS (see also “Variants of GBS”). [3]

GBS can affect respiratory muscle function; life-threatening respiratory failure occurs in approx. 20% of patients. [8][9]

Subtypes and variantstoggle arrow icon

Subtypes of Guillain-Barré syndrome

Classification into subtypes is based on electrodiagnostic study findings; each subtype is associated with specific autoantibodies.

Overview of subtypes of Guillain-Barré syndrome
Subtype Pathophysiology Epidemiology Characteristic features
Acute inflammatory demyelinating polyneuropathy (AIDP)
  • Most common subtype (up to 90% of cases) in the US, Canada, and Europe [2]
Axonal loss Acute motor axonal neuropathy (AMAN)
  • Most common subtype (30–65% of cases) in some Asian countries (e.g., China and Japan) and Mexico [2]
Acute motor and sensory axonal neuropathy (AMSAN)

Classification into subtypes does not affect treatment decisions but may inform prognosis. [2][3]

Variants of Guillain-Barré syndrome

Variants of the classic sensorimotor presentation are characterized by the involvement of a specific group of muscles or nerves; overlap between variants is common.

Diagnosticstoggle arrow icon

General principles [9][10]

A complete neurological examination is required for all patients.

CSF analysis [2][7]

  • Obtained to support diagnosis and rule out infectious and malignant causes
  • Findings
    • May be normal in the first 1–2 weeks of the disease [2]
    • Typical: albuminocytologic dissociation (i.e., increased protein levels with normal leukocyte count < 10 cells/mcL in CSF)

CSF cell count is typically normal in patients with GBS; consider other diagnoses in patients with a count > 50 cells/mcL. [2][3]

Electrodiagnostic studies

Nerve conduction studies [7][8]

  • Obtained to support diagnosis and/or to classify GBS into subtypes
  • Findings vary according to the subtype of GBS and may be normal during the early stages of disease.

Nerve conduction study findings may be normal in the week following symptom onset or in patients with certain GBS clinical variants, e.g., Miller-Fisher syndrome. [9]


Additional studies [7][9]

Differential diagnosestoggle arrow icon

Differential diagnoses of Guillain-Barré syndrome [3][9]

See “Etiology of polyneuropathy” and “Differential diagnoses of polyneuropathy” for further details.

Clinical features of Guillain-Barré syndrome vs. transverse myelitis

Clinical features of Guillain-Barré syndrome vs. transverse myelitis
Guillain-Barré syndrome Transverse myelitis
Symptom onset
  • Acute or subacute
    (4 hours to 21 days)
Motor findings
  • Ascending paralysis
  • Involvement of respiratory muscles
  • Reduced or absent
Sensory findings
Autonomic dysfunction
Cranial nerve involvement
  • Not affected
CSF examination
Imaging and findings
  • MRI: focal, enhanced lesion in the spinal cord
Electrophysiological findings
  • Usually normal

The differential diagnoses listed here are not exhaustive.

Managementtoggle arrow icon

Approach [9]

Even though many patients recover without treatment, hospital admission and multidisciplinary care are necessary because of the unpredictable course of the disease and risk of death.

Medical treatment [3][8][9]

Glucocorticoids are not recommended for the treatment of GBS, as they have not been proven to hasten recovery or affect the prognosis. [9]

Monitoring [9][11]

The frequency of reassessment should be based on clinical severity; follow local protocols when available.

ICU-level care and intubation may be indicated.

Patients with bulbar palsy, involvement of the upper extremities, or autonomic dysfunction have an increased risk of respiratory failure. [3]

Respiratory support

Identify predictors of respiratory failure in GBS early to guide decisions about intubation, mechanical ventilation, and ICU admission. Manage respiratory failure urgently.

Classic signs of respiratory distress and signs of impending respiratory failure typically occur late in GBS. Identify predictors of respiratory failure in GBS as early as possible. [12][13]

Succinylcholine is contraindicated in patients with GBS as it can cause life-threatening hyperkalemia and cardiac arrest. [15]

Supportive management [9]

Disposition [9][11]

Prognosistoggle arrow icon

  • Approx. 80% of patients with GBS regain the ability to walk unaided 6 months after symptom onset. [9]
  • Consider calculating the modified Erasmus GBS Outcome Score (mEGOS) to predict the patient's ability to walk unaided.
  • 3–7% of patients with GBS die as a result of complications, e.g.: [2][8]
  • Patient factors associated with an increased risk of long-term disability include: [3]
    • Rapid symptom progression
    • Severe symptoms at peak of the disease
    • Older age
    • Axonal damage (e.g., in AMAN)
  • It may take > 5 years for symptoms to improve and/or resolve. [9]

Death can occur as many as > 30 days after onset of symptoms, during the recovery phase.

Referencestoggle arrow icon

  1. Leonhard SE, Mandarakas MR, Gondim FAA, et al. Diagnosis and management of Guillain–Barré syndrome in ten steps. Nat Rev Neur. 2019; 15 (11): p.671-683.doi: 10.1038/s41582-019-0250-9 . | Open in Read by QxMD
  2. Willison HJ, Jacobs BC, van Doorn PA. Guillain-Barré syndrome. Lancet. 2016; 388: p.717-727.doi: 10.1016/ S0140-6736(16)00339-1 . | Open in Read by QxMD
  3. Yuki N, Hartung HP. Guillain–Barré Syndrome. N Engl J Med. 2012; 366: p.2294-2304.doi: 10.1056/NEJMra1114525 . | Open in Read by QxMD
  4. Walling AD, Dickson G. Guillain-Barré syndrome. Am Fam Physician. 2013; 87 (3): p.191-7.
  5. Matthew Harms. Inpatient Management of Guillain-Barré Syndrome. The Neurohospitalist. 2011; 1 (2): p.78-84.doi: 10.1177/1941875210396379 . | Open in Read by QxMD
  6. Umer SR, Nisa Q, Kumari M, Abbas S, Mahesar SA, Shahbaz NN. Clinical Features Indicating the Need for Mechanical Ventilation in Patients with Guillain Barre Syndrome. Cureus. 2019.doi: 10.7759/cureus.5520 . | Open in Read by QxMD
  7. Lawn ND, Fletcher DD, Henderson RD, Wolter TD, Wijdicks EFM. Anticipating Mechanical Ventilation in Guillain-Barré Syndrome. Arch Neurol. 2001; 58 (6): p.893.doi: 10.1001/archneur.58.6.893 . | Open in Read by QxMD
  8. Doets AY, Walgaard C, Lingsma HF, et al. International Validation of the Erasmus Guillain–Barré Syndrome Respiratory Insufficiency Score. Ann Neurol. 2022; 91 (4): p.521-531.doi: 10.1002/ana.26312 . | Open in Read by QxMD
  9. Martyn JAJ, Richtsfeld M, Warner DO. Succinylcholine-induced Hyperkalemia in Acquired Pathologic States. Anesthesiology. 2006; 104 (1): p.158-169.doi: 10.1097/00000542-200601000-00022 . | Open in Read by QxMD
  10. Guillain-Barré Syndrome. Updated: December 20, 2019. Accessed: December 23, 2020.
  11. Uncini A, Vallat J-M, Jacobs BC. Guillain-Barré syndrome in SARS-CoV-2 infection: an instant systematic review of the first six months of pandemic. Journal of Neurology, Neurosurgery & Psychiatry. 2020; 91 (10): p.1105-1110.doi: 10.1136/jnnp-2020-324491 . | Open in Read by QxMD
  12. Hartung HP. Infections and the Guillain-Barré Syndrome. J Neurol Neurosurg Psychiatry. 1999; 66: p.277.doi: 10.1136/jnnp.66.3.277 . | Open in Read by QxMD
  13. Nelson KE. Invited Commentary: Influenza Vaccine and Guillain-Barre Syndrome--Is There a Risk?. Am J Epidemiol. 2012; 175 (11): p.1129-1132.doi: 10.1093/aje/kws194 . | Open in Read by QxMD
  14. Van Den Berg B, Walgaard C, Drenthen J, Fokke C, Jacobs BC, Van Doorn PA. Guillain–Barré syndrome: pathogenesis, diagnosis, treatment and prognosis. Nature Reviews Neurology. 2014; 10: p.469–482.doi: 10.1038/nrneurol.2014.121 . | Open in Read by QxMD
  15. Asbury AK, Cornblath DR. Assessment of current diagnostic criteria for Guillain-Barré syndrome. Ann Neurol. 1990; 27 (S1): p.S21-S24.doi: 10.1002/ana.410270707 . | Open in Read by QxMD

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