• Clinical science

Guillain-Barré syndrome

Summary

Guillain-Barré syndrome (GBS) is an acute postinfectious polyneuropathy characterized by symmetric and ascending flaccid paralysis. In affected patients, cross‑reactive autoantibodies attack the host's own axonal antigens, resulting in inflammatory and demyelinating polyneuropathy. Albuminocytologic dissociation, characterized by elevated protein levels and normal cell counts in cerebrospinal fluid (CSF), is a hallmark finding of GBS. Additionally, muscle and nerve electrophysiology are used to diagnose demyelinating processes. Symptomatic and supportive treatment results in disease remission in about 70% of cases. In severe cases or patients who do not respond to treatment, intravenous immunoglobulin (IVIG) administration and/or plasmapheresis may be used. Potentially acute life-threatening complications such as respiratory insufficiency, pulmonary embolism, and/or cardiac arrest increase mortality. Although GBS is associated with a good prognosis overall, up to 20% of patients remain severely disabled and approximately 5% of cases are fatal, despite immunotherapy.

Epidemiology

  • Incidence: 1–4 cases per 100,000
  • Sex: > (1.5:1)
  • Adults are affected more frequently than children.

References:[1][2]

Epidemiological data refers to the US, unless otherwise specified.

Etiology

References:[3]

Pathophysiology

References:[4]

Clinical features

  • Initial symptoms: back and limb pain, esp. paresthesias affecting distal extremities
  • Advanced symptoms
    • Ascending paralysis: Bilateral flaccid paralysis spreads from the lower to the upper limbs in a “stocking‑glove” distribution.
      • Cranial nerve involvement: frequently bilateral facial nerve involvement (facial diplegia)
      • Landry paralysis: involvement of the respiratory muscles
    • Reduced or absent muscle reflexes
    • Peripheral, symmetric paresthesias in the hands and feet
    • Neuropathic pain develops in about ⅔ of patients
    • Cardiovascular autonomic dysfunction (arrhythmia), voiding dysfunction, and/or intestinal dysfunction

GBS paralysis affects the muscles of respiration, possibly leading to death due to respiratory failure!

References:[5][6]

Subtypes and variants

Description Etiology Symptoms Treatment
Acute inflammatory demyelinating polyneuropathy
  • Acute variant of Guillain‑Barré syndrome.
  • Predominant sub-type affecting 60–80% of GBS patients in North America and Europe
  • Intravenous immunoglobulin G (IVIg), plasmapheresis
Chronic inflammatory demyelinating polyneuropathy (CIDP)
  • Chronic variant of Guillain‑Barré syndrome.
  • CIDP has not been linked to any infectious agent in particular.
  • Autoantibodies against GM1 gangliosides
  • Similar to those of GBS, but lasting > 2 months
Miller-Fisher syndrome
  • A limited variant of GBS characterized by cranial nerve involvement
  • Intravenous immunoglobulin G (IVIg)
Multifocal motor neuropathy (MMN)
  • Asymmetric paralysis and areflexia
  • Initially involves the distal upper limbs


References:[8][9][4][10][11][12]

Diagnostics

References:[6][4]

Treatment

  • Supportive management
    • Monitor cardiac and respiratory function: in some cases, intensive care unit (ICU) treatment and intubation may be indicated
    • Prevent decubitus and/or thrombosis (esp. pulmonary embolism)
  • High dose of intravenous immunoglobulins
  • Plasmapheresis
    • In adults: equivalent outcome as IV immunoglobulins
    • In children: only recommended in children with rapidly progressing or severe disease

Although GBS is considered an autoimmune disease, glucocorticoids are not recommended for treatment. They have not shown to hasten recovery or affect the long-term outcome.

References:[1][6][13]

Prognosis

  • Up to 70% of patients with GBS have a good prognosis: Disease progression peaks 2–4 weeks after the onset of symptoms. Symptoms then recede in reverse order of their development, i.e., the last symptoms to appear resolve first, as Schwann cells remyelinate peripheral nerve axons.
  • 3–7% of patients with GBS die due to acute complications such as respiratory paralysis (apnea), pulmonary infection, pulmonary embolism, or cardiac dysfunction.

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

References:[14]

  • 1. Hughes RAC, Wijdicks EFM, Barohn R, Benson E, et al. Practice parameter: Immunotherapy for Guillain-Barré Syndrome. Neurology. 2003; 61(6). doi: 10.1212/WNL.61.6.736.
  • 2. Yuki N, Hartung HP. Guillain–Barré Syndrome. N Engl J Med. 2012; 366: pp. 2294–2304. doi: 10.1056/NEJMra1114525.
  • 3. Hartung HP. Infections and the Guillain-Barré Syndrome. J Neurol Neurosurg Psychiatry. 1999; 66: p. 277. doi: 10.1136/jnnp.66.3.277.
  • 4. 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: pp. 469–482. doi: 10.1038/nrneurol.2014.121.
  • 5. Le T, Bhushan V, Chen V, King M. First Aid for the USMLE Step 2 CK. McGraw-Hill Education; 2015.
  • 6. Guillain-Barre Syndrome: Diagnostic Criteria. http://bestpractice.bmj.com/best-practice/monograph/176/diagnosis/criteria.html. Updated March 8, 2017. Accessed April 2, 2017.
  • 7. Dimachkie MM, Barohn RJ, Katz J. Multifocal Motor Neuropathy, Multifocal Acquired Demyelinating Sensory and Motor Neuropathy, and Other Chronic Acquired Demyelinating Polyneuropathy Variants. Neurol Clin. 2013; 31(2): pp. 533–555. doi: 10.1016/j.ncl.2013.01.001.
  • 8. Mathey EK, Park SB, Hughes RAC et al. Chronic inflammatory demyelinating polyradiculoneuropathy: from pathology to phenotype . http://jnnp.bmj.com/content/early/2015/02/12/jnnp-2014-309697.full. Updated February 12, 2015. Accessed April 2, 2017.
  • 9. Lawson VH, Arnold DW. Multifocal motor neuropathy: a review of pathogenesis, diagnosis, and treatment. Neuropsychiatr Dis Treat. 2014; 10: pp. 567–576. doi: 10.2147/NDT.S39592.
  • 10. Ramachandran TS, Lorenzo N. Acute Inflammatory Demyelinating Polyradiculoneuropathy. In: Acute Inflammatory Demyelinating Polyradiculoneuropathy. New York, NY: WebMD. http://emedicine.medscape.com/article/1169959-overview. Updated June 8, 2017. Accessed June 13, 2017.
  • 11. Mathey EK, Park SB, Hughes RAC, et al. Chronic inflammatory demyelinating polyradiculoneuropathy: from pathology to phenotype. J Neurol Neurosurg Psychiatry. 2015; 86(9): pp. 973–985. doi: 10.1136/jnnp-2014-309697.
  • 12. Simon JI, Armstrong J. AIDP/CIDP Part 2: Treatment. https://now.aapmr.org/aidpcidp-part-2-treatment/. Updated September 20, 2014. Accessed April 4, 2018.
  • 13. Andary MT. Guillain-Barre Syndrome. In: Guillain-Barre Syndrome. New York, NY: WebMD. http://emedicine.medscape.com/article/315632-overview. Updated January 8, 2017. Accessed April 6, 2017.
  • 14. Willison HJ, Jacobs BC, van Doorn PA. Guillain-Barré syndrome. Lancet. 2016; 388: pp. 717–727. doi: 10.1016/ S0140-6736(16)00339-1.
  • CPK isoenzymes test. https://medlineplus.gov/ency/article/003504.htm. Updated September 1, 2015. Accessed April 2, 2017.
last updated 11/13/2020
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