Guillain-Barre syndrome (GBS) is identified throughout the western hemisphere without geographic clustering, but with minor seasonal variations.[10]Webb AJ, Brain SA, Wood R, et al. Seasonal variation in Guillain-Barré syndrome: a systematic review, meta-analysis and Oxfordshire cohort study. J Neurol Neurosurg Psychiatry. 2015 Nov;86(11):1196-201.
https://jnnp.bmj.com/content/86/11/1196.long
http://www.ncbi.nlm.nih.gov/pubmed/25540247?tool=bestpractice.com
Population-based studies give crude mean annual incidence rates varying from 0.6 to 1.9 per 100,000 population. A few outbreaks have been reported, including the 1976 outbreak in the US after the swine influenza program (although the link between the influenza immunization and incidence of GBS is unclear).[11]Leonhard SE, Mandarakas MR, Gondim FAA, et al. Diagnosis and management of Guillain-Barré syndrome in ten steps. Nat Rev Neurol. 2019 Nov;15(11):671-83.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6821638
http://www.ncbi.nlm.nih.gov/pubmed/31541214?tool=bestpractice.com
[5]Yuki N, Hartung HP. Guillain-Barré syndrome. N Engl J Med. 2012 Jun 14;366(24):2294-304.
http://www.ncbi.nlm.nih.gov/pubmed/22694000?tool=bestpractice.com
GBS is slightly more common in men than women, with an estimated male-female ratio of 1.78.[5]Yuki N, Hartung HP. Guillain-Barré syndrome. N Engl J Med. 2012 Jun 14;366(24):2294-304.
http://www.ncbi.nlm.nih.gov/pubmed/22694000?tool=bestpractice.com
[12]Willison HJ, Jacobs BC, van Doorn PA. Guillain-Barré syndrome. Lancet. 2016 Aug 13;388(10045):717-27.
https://www.doi.org/10.1016/S0140-6736(16)00339-1
http://www.ncbi.nlm.nih.gov/pubmed/26948435?tool=bestpractice.com
Acute inflammatory demyelinating polyradiculoneuropathy (AIDP) is the most common form in North America and Europe and accounts for 85% to 90% of cases.[13]Poropatich KO, Walker CL, Black RE. Quantifying the association between Campylobacter infection and Guillain-Barré syndrome: a systematic review. J Health Popul Nutr. 2010 Dec;28(6):545-52.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2995022
http://www.ncbi.nlm.nih.gov/pubmed/21261199?tool=bestpractice.com
[14]The French Cooperative Group on Plasma Exchange in Guillain-Barré Syndrome. Appropriate number of plasma exchanges in Guillain-Barré syndrome. Ann Neurol. 1997 Mar;41(3):298-306.
http://www.ncbi.nlm.nih.gov/pubmed/9066350?tool=bestpractice.com
AIDP can occur at any age, although rarely in infancy, and incidence increases with age; mean age of onset is approximately 40 years.[12]Willison HJ, Jacobs BC, van Doorn PA. Guillain-Barré syndrome. Lancet. 2016 Aug 13;388(10045):717-27.
https://www.doi.org/10.1016/S0140-6736(16)00339-1
http://www.ncbi.nlm.nih.gov/pubmed/26948435?tool=bestpractice.com
[15]Winer JB, Hughes RA, Anderson MJ, et al. A prospective study of acute idiopathic neuropathy. II. Antecedent events. J Neurol Neurosurg Psychiatry. 1988 May;51(5):613-8.
https://jnnp.bmj.com/content/jnnp/51/5/613.full.pdf
http://www.ncbi.nlm.nih.gov/pubmed/3404161?tool=bestpractice.com
GBS is the most common cause of acute flaccid paralysis in children.[16]Jones HR Jr. Guillain-Barré syndrome in children. Curr Opin Pediatr. 1995 Dec;7(6):663-8.
http://www.ncbi.nlm.nih.gov/pubmed/8776016?tool=bestpractice.com
The acute motor axonal neuropathy (AMAN) subtype of GBS is more common in Japan and China, particularly in young people. It occurs more frequently during summer.[17]Ho TW, Mishu B, Li CY, et al. Guillain-Barré syndrome in northern China. Relationship to Campylobacter jejuni infection and anti-glycolipid antibodies. Brain. 1995 Jun;118 ( Pt 3):597-605.
http://www.ncbi.nlm.nih.gov/pubmed/7600081?tool=bestpractice.com
Sporadic AMAN worldwide affects 10% to 20% of patients with GBS.[18]Jacobs BC, van Doorn PA, Schmitz PI, et al. Campylobacter jejuni infections and anti-GM1 antibodies in GBS. Ann Neurol. 1996 Aug;40(2):181-7.
http://www.ncbi.nlm.nih.gov/pubmed/8773599?tool=bestpractice.com
Miller-Fisher syndrome affects between 5% and 10% of patients with GBS in western countries, but is more common in eastern Asia, affecting 25% of patients with GBS in Japan and 19% in Taiwan.[19]Lyu RK, Tang LM, Cheng SY, et al. Guillain-Barré syndrome in Taiwan: a clinical study of 167 patients. J Neurol Neurosurg Psychiatry. 1997 Oct;63(4):494-500.
https://jnnp.bmj.com/content/jnnp/63/4/494.full.pdf
http://www.ncbi.nlm.nih.gov/pubmed/9343130?tool=bestpractice.com
GBS is associated with Zika virus outbreaks. One meta-analysis estimated that 1.23% of people with Zika virus develop GBS, and another estimated that incidence of GBS in Latin America and the Caribbean rose 2.6-fold during Zika virus outbreaks.[20]Barbi L, Coelho AVC, Alencar LCA, et al. Prevalence of Guillain-Barré syndrome among Zika virus infected cases: a systematic review and meta-analysis. Braz J Infect Dis. 2018 Mar-Apr;22(2):137-41.
https://www.sciencedirect.com/science/article/pii/S1413867017309340?via%3Dihub
http://www.ncbi.nlm.nih.gov/pubmed/29545017?tool=bestpractice.com
[21]Capasso A, Ompad DC, Vieira DL, et al. Incidence of Guillain-Barré Syndrome (GBS) in Latin America and the Caribbean before and during the 2015-2016 Zika virus epidemic: A systematic review and meta-analysis. PLoS Negl Trop Dis. 2019 Aug;13(8):e0007622.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6730933
http://www.ncbi.nlm.nih.gov/pubmed/31449532?tool=bestpractice.com
One study in the Americas found that just under half of 51 deaths associated with Zika virus were related to GBS.[22]Cardona-Ospina JA, Henao-SanMartin V, Acevedo-Mendoza WF, et al. Fatal Zika virus infection in the Americas: A systematic review. Int J Infect Dis. 2019 Nov;88:49-59.
https://www.doi.org/10.1016/j.ijid.2019.08.033
http://www.ncbi.nlm.nih.gov/pubmed/31499212?tool=bestpractice.com
There have been multiple reports from a number of countries of patients with confirmed coronavirus disease 2019 (COVID-19) infection developing GBS.[23]Uncini A, Vallat JM, Jacobs BC. Guillain-Barré syndrome in SARS-CoV-2 infection: an instant systematic review of the first six months of pandemic. J Neurol Neurosurg Psychiatry. 2020 Oct;91(10):1105-10.
https://www.doi.org/10.1136/jnnp-2020-324491
http://www.ncbi.nlm.nih.gov/pubmed/32855289?tool=bestpractice.com
[24]Caress JB, Castoro RJ, Simmons Z, et al. COVID-19-associated Guillain-Barré syndrome: The early pandemic experience. Muscle Nerve. 2020 Oct;62(4):485-91.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7405390
http://www.ncbi.nlm.nih.gov/pubmed/32678460?tool=bestpractice.com
[25]Hasan I, Saif-Ur-Rahman KM, Hayat S, et al. Guillain-Barré syndrome associated with SARS-CoV-2 infection: A systematic review and individual participant data meta-analysis. J Peripher Nerv Syst. 2020 Dec;25(4):335-43.
https://www.doi.org/10.1111/jns.12419
http://www.ncbi.nlm.nih.gov/pubmed/33112450?tool=bestpractice.com
The reported mean age (55 to 59 years) and male predominance of patients developing GBS appear to be typical of hospitalized COVID-19 patients.[23]Uncini A, Vallat JM, Jacobs BC. Guillain-Barré syndrome in SARS-CoV-2 infection: an instant systematic review of the first six months of pandemic. J Neurol Neurosurg Psychiatry. 2020 Oct;91(10):1105-10.
https://www.doi.org/10.1136/jnnp-2020-324491
http://www.ncbi.nlm.nih.gov/pubmed/32855289?tool=bestpractice.com
[24]Caress JB, Castoro RJ, Simmons Z, et al. COVID-19-associated Guillain-Barré syndrome: The early pandemic experience. Muscle Nerve. 2020 Oct;62(4):485-91.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7405390
http://www.ncbi.nlm.nih.gov/pubmed/32678460?tool=bestpractice.com
[25]Hasan I, Saif-Ur-Rahman KM, Hayat S, et al. Guillain-Barré syndrome associated with SARS-CoV-2 infection: A systematic review and individual participant data meta-analysis. J Peripher Nerv Syst. 2020 Dec;25(4):335-43.
https://www.doi.org/10.1111/jns.12419
http://www.ncbi.nlm.nih.gov/pubmed/33112450?tool=bestpractice.com
More evidence is needed about whether the incidence of GBS in patients with COVID-19 is higher than that in the general population.[24]Caress JB, Castoro RJ, Simmons Z, et al. COVID-19-associated Guillain-Barré syndrome: The early pandemic experience. Muscle Nerve. 2020 Oct;62(4):485-91.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7405390
http://www.ncbi.nlm.nih.gov/pubmed/32678460?tool=bestpractice.com
There have also been reports of GBS after COVID-19 vaccination.[26]European Medicines Agency. Meeting highlights from the Pharmacovigilance Risk Assessment Committee (PRAC) 3-6 May 2021. May 2021 [internet publication].
https://www.ema.europa.eu/en/news/meeting-highlights-pharmacovigilance-risk-assessment-committee-prac-3-6-may-2021