Guillain-Barre syndrome

Guillain-Barre syndrome (GBS) is an acute inflammatory polyneuropathy that is classified according to symptoms and divided into axonal and demyelinating forms.

Two-thirds of patients have a history of gastroenteritis or influenza-like illness weeks before onset of neurologic symptoms. The acute infectious illness is usually viral, but sometimes bacterial.

Approximately 20% to 30% of patients with GBS will develop respiratory muscle weakness requiring ventilation.

Neurophysiology is confirmatory and is abnormal in most patients, even early in the disease.

Lumbar puncture is useful, and the classic finding is elevated protein with normal cell count (albuminocytologic dissociation).

Treatment combines supportive and disease-modifying therapy (high-dose immune globulin or plasma exchange).

GBS is an acute inflammatory neuropathy.[1]Hadden RD, Hughes RA. Management of inflammatory neuropathies. J Neurol Neurosurg Psychiatry. 2003 Jun;74(suppl 2):ii9-14. https://jnnp.bmj.com/content/74/suppl_2/ii9.long http://www.ncbi.nlm.nih.gov/pubmed/12754323?tool=bestpractice.com [2]Hiraga A, Mori M, Ogawara K, et al. Recovery patterns and long term prognosis for axonal Guillain-Barré syndrome. J Neurol Neurosurg Psychiatry. 2005 May;76(5):719-22. https://jnnp.bmj.com/content/76/5/719.long http://www.ncbi.nlm.nih.gov/pubmed/15834034?tool=bestpractice.com ​​​​​ It is a clinically defined syndrome characterized by motor difficulty, absence of deep tendon reflexes, paresthesias without objective sensory loss, and increased cerebrospinal fluid albumin with a normal cell count (albuminocytologic dissociation).[1]Hadden RD, Hughes RA. Management of inflammatory neuropathies. J Neurol Neurosurg Psychiatry. 2003 Jun;74(suppl 2):ii9-14. https://jnnp.bmj.com/content/74/suppl_2/ii9.long http://www.ncbi.nlm.nih.gov/pubmed/12754323?tool=bestpractice.com [3]Guillain G, Barré JA, Strohl A. Radiculoneuritis syndrome with hyperalbuminosis of cerebrospinal fluid without cellular reaction. Notes on clinical features and graphs of tendon reflexes. 1916 [in French]. Ann Med Interne (Paris). 1999 Jan;150(1):24-32. http://www.ncbi.nlm.nih.gov/pubmed/10400560?tool=bestpractice.com ​​​​​ Acute inflammatory demyelinating polyradiculoneuropathy is the most commonly encountered variant.[4]Asbury AK, Arnason BG, Adams RD. The inflammatory lesion in idiopathic polyneuritis. Its role in pathogenesis. Medicine (Baltimore). 1969 May;48(3):173-215. http://www.ncbi.nlm.nih.gov/pubmed/5769741?tool=bestpractice.com ​ The acute infectious illness is usually viral (e.g., cytomegalovirus, Epstein-Barr virus, hepatitis E), but sometimes bacterial (e.g., Campylobacter jejuni, Mycoplasma species). Several arthropod-borne viral infections have been linked to GBS including Zika, dengue, chikungunya, Japanese encephalitis and Oropouche fever.[5]Counotte MJ, Meili KW, Taghavi K, et al. Zika virus infection as a cause of congenital brain abnormalities and Guillain-Barré syndrome: a living systematic review. F1000Res. 2019 Aug 14;8:1433. https://pmc.ncbi.nlm.nih.gov/articles/PMC6852328 http://www.ncbi.nlm.nih.gov/pubmed/31754425?tool=bestpractice.com [6]Ralapanawa DM, Kularatne SA, Jayalath WA. Guillain-Barre syndrome following dengue fever and literature review. BMC Res Notes. 2015 Nov 27;8:729. https://bmcresnotes.biomedcentral.com/articles/10.1186/s13104-015-1672-0 http://www.ncbi.nlm.nih.gov/pubmed/26613722?tool=bestpractice.com ​​​​​[7]Oehler E, Fournier E, Leparc-Goffart I, et al. Increase in cases of Guillain-Barré syndrome during a Chikungunya outbreak, French Polynesia, 2014 to 2015. Euro Surveill. 2015;20(48):30079. http://www.ncbi.nlm.nih.gov/pubmed/26690898?tool=bestpractice.com [8]Simon O, Billot S, Guyon D, et al. Early Guillain-Barré syndrome associated with acute dengue fever. J Clin Virol. 2016 Apr;77:29-31. http://www.ncbi.nlm.nih.gov/pubmed/26895226?tool=bestpractice.com ​​​​​[9]Cerny T, Schwarz M, Schwarz U, et al. The range of neurological complications in chikungunya fever. Neurocrit Care. 2017 Dec;27(3):447-57. https://link.springer.com/article/10.1007/s12028-017-0413-8 http://www.ncbi.nlm.nih.gov/pubmed/28741102?tool=bestpractice.com [10]Martos-Benítez FD, Betancourt-Plaza I, Osorio-Carmenates I, et al. Neurological performance and clinical outcomes related to patients with oropouche-associated Guillain-Barré syndrome. J Peripher Nerv Syst. 2025 Mar;30(1):e12683. http://www.ncbi.nlm.nih.gov/pubmed/39853692?tool=bestpractice.com ​​​​​ This topic focuses on diagnosis and management of GBS in adults.