Guillain–Barré syndrome: pathophysiology, etiology, causes, and treatment

Rahmathulla S. Rahman, Moayyad S. Bauthman, Amer M. Alanazi, Naif N. Alsillah, Ziyad M. Alanazi, Mahdi I. Almuhaysin, Ruyuf K. Almutairi, Kadhem S. Binobaid, Yousef H. Alharthi, Rime M. Bawareth, Omar N. Alrawili


Guillain–Barré syndrome (GBS) is a polyradiculoneuropathy autoimmune disease that is characterized by significant inflammation that affects the peripheral nervous system in a rapidly progressive pattern that is mainly clinically presented by muscle weakness. The present literature review aims to broadly discuss GBS: etiology, pathophysiology and management in order to gain an understading of the existing studies that are relevant to this literature review. Among the reported antibodies, anti-GM1 and anti-GQ1B have been reported to be responsible for attacking and damaging either the neuromuscular junctions or peripheral nerves. Moreover, it has been found that the anti-GD1a antibodies in patients bind to the neuromuscular junction and also bind to the nodes of Ranvier of the peripheral nerves and the paranodal myelin of the affected nerves. Reports have shown that this disease is identified as special forms of neuropathies that develop in immune-mediated, post-infection sequelae. Furthermore, in another study it was reported that Molecular mimicry has been previously reported to significantly correlate with the development of the disease as it was investigated in animal models. In addition, Campylobacter jejuni, a pathogen that causes gastrointestinal infections has been previously reported to predispose to the development of GBS in humans. However, scientists have found that plasma exchange and intravenous immunoglobulins (IVIG) remain the most significant and efficacious factors in managing the disease. Nevertheless, recent trials have investigated other approaches that are less efficacious and can lead to serious adverse events and complications. 


Guillain–Barré syndrome, Neurology, Management, Epidemiology, Etiology

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Hughes RA, Cornblath DR. Guillain-Barré syndrome. Lancet. 2005;366(9497):1653-66.

Cosi V, Versino M. Guillain-Barré syndrome. Neurol Sci. 2006;27(1):47-51.

Van den Berg B, Walgaard C, Drenthen J, Fokke C, Jacobs BC, van Doorn PA. Guillain-Barré syndrome: pathogenesis, diagnosis, treatment and prognosis. Nat Rev Neurol. 2014;10(8):469-82.

Dornonville de la Cour C, Jakobsen J. Residual neuropathy in long-term population-based follow-up of Guillain-Barré syndrome. Neurology. 2005;64(2):246-53.

Hughes RA, Swan AV, van Doorn PA. Intravenous immunoglobulin for Guillain-Barré syndrome. Cochrane Database Systemat Rev. 2014;9:CD002063.

Raphaël JC, Chevret S, Hughes RA, Annane D. Plasma exchange for Guillain-Barré syndrome. Cochrane Database Systemat Rev. 2002(2):CD001798.

Olivé JM, Castillo C, Castro RG, de Quadros CA. Epidemiologic study of Guillain-Barré syndrome in children <15 years of age in Latin America. J Infect Dis. 1997;175(1):160-4.

Govoni V, Granieri E. Epidemiology of the Guillain-Barré syndrome. Curr Opinion Neurol. 2001;14(5):605-13.

Asbury AK, Cornblath DR. Assessment of current diagnostic criteria for Guillain-Barré syndrome. Ann Neurol. 1990;27:21-4.

Fokke C, van den Berg B, Drenthen J, Walgaard C, van Doorn PA, Jacobs BC. Diagnosis of Guillain-Barré syndrome and validation of Brighton criteria. Brain J Neurol. 2014;137(1):33-43.

Yuki N, Taki T, Inagaki F. A bacterium lipopolysaccharide that elicits Guillain-Barré syndrome has a GM1 ganglioside-like structure. J Exp Med. 1993;178(5):1771-5.

Dirlikov E, Major CG, Medina NA. Clinical Features of Guillain-Barré Syndrome With vs Without Zika Virus Infection, Puerto Rico, 2016. JAMA Neurol. 2018;75(9):1089-97.

Awong IE, Dandurand KR, Keeys CA, Maung-Gyi FA. Drug-associated Guillain-Barré syndrome: a literature review. Ann Pharmacother. 1996;30(2):173-80.

Yuki N, Hartung HP. Guillain-Barré syndrome. N Engl J Med. 2012;366(24):2294-304.

Campbell AM. The aetiology of polyneuritis. Proc Royal Soc Med. 1958;51(3):157-9.

Cao-Lormeau VM, Blake A, Mons S. Guillain-Barré Syndrome outbreak associated with Zika virus infection in French Polynesia: a case-control study. Lancet. 2016;387(10027):1531-9.

Schonberger LB, Bregman DJ, Sullivan-Bolyai JZ. Guillain-Barre syndrome following vaccination in the National Influenza Immunization Program, United States, 1976--1977. Am J Epidemiol. 1979;110(2):105-23.

Lasky T, Terracciano GJ, Magder L. The Guillain-Barré syndrome and the 1992-1993 and 1993-1994 influenza vaccines. N Engl J Med. 1998;339(25):1797-802.

Willison HJ, Jacobs BC, van Doorn PA. Guillain-Barré syndrome. Lancet. 2016;388(10045):717-27.

Sejvar JJ, Baughman AL, Wise M, Morgan OW. Population incidence of Guillain-Barré syndrome: a systematic review and meta-analysis. Neuroepidemiology. 2011;36(2):123-33.

Nguyen TP, Taylor RS. Guillain Barre Syndrome. In: StatPearls. Treasure Island (FL): StatPearls Publishing. StatPearls Publishing LLC. 2021.

Jacobs BC, Rothbarth PH, van der Meché FG. The spectrum of antecedent infections in Guillain-Barré syndrome: a case-control study. Neurology. 1998;51(4):1110-5.

Susuki K, Nishimoto Y, Yamada M. Acute motor axonal neuropathy rabbit model: immune attack on nerve root axons. Ann Neurol. 2003;54(3):383-8.

Yuki N, Yamada M, Koga M. Animal model of axonal Guillain-Barré syndrome induced by sensitization with GM1 ganglioside. Ann Neurol. 2001;49(6):712-20.

Willison HJ, O'Hanlon G, Paterson G, et al. Mechanisms of action of anti-GM1 and anti-GQ1b ganglioside antibodies in Guillain-Barré syndrome. J Infect Dis. 1997;176(2):144-9.

Greenshields KN, Halstead SK, Zitman FM. The neuropathic potential of anti-GM1 autoantibodies is regulated by the local glycolipid environment in mice. J Clin Investig. 2009;119(3):595-610.

Chiba A, Kusunoki S, Obata H, Machinami R, Kanazawa I. Serum anti-GQ1b IgG antibody is associated with ophthalmoplegia in Miller Fisher syndrome and Guillain-Barré syndrome: clinical and immunohistochemical studies. Neurology. 1993;43(10):1911-7.

Goodfellow JA, Bowes T, Sheikh K. Overexpression of GD1a ganglioside sensitizes motor nerve terminals to anti-GD1a antibody-mediated injury in a model of acute motor axonal neuropathy. J Neurosci. 2005;25(7):1620-8.

Susuki K, Rasband MN, Tohyama K. Anti-GM1 antibodies cause complement-mediated disruption of sodium channel clusters in peripheral motor nerve fibers. J Neurosci. 2007;27(15):3956-67.

Chiba A, Kusunoki S, Shimizu T, Kanazawa I. Serum IgG antibody to ganglioside GQ1b is a possible marker of Miller Fisher syndrome. Ann Neurol. 1992;31(6):677-9.

Gregson NA, Jones D, Thomas PK, Willison HJ. Acute motor neuropathy with antibodies to GM1 ganglioside. J Neurol. 1991;238(8):447-51.

O'Leary CP, Veitch J, Durward WF, Thomas AM, Rees JH, Willison HJ. Acute oropharyngeal palsy is associated with antibodies to GQ1b and GT1a gangliosides. J Neurol Neurosurg Psychiatry. 1996;61(6):649-51.

Ortiz-Salas P, Velez-Van-Meerbeke A, Galvis-Gomez CA, Rodriguez QJ. Human Immunoglobulin Versus Plasmapheresis in Guillain-Barre Syndrome and Myasthenia Gravis: A Meta-Analysis. J Clin Neuromuscular Dis. 2016;18(1):1-11.

Van der Meché FG, Schmitz PI. A randomized trial comparing intravenous immune globulin and plasma exchange in Guillain-Barré syndrome. Dutch Guillain-Barré Study Group. N Engl J Med. 1992;326(17):1123-9.

Raphaël JC, Chevret S, Hughes RA, Annane D. Plasma exchange for Guillain-Barré syndrome. The Cochrane Database Systemat Rev. 2012(7):CD001798.

Plasmapheresis and acute Guillain-Barré syndrome. The Guillain-Barré syndrome Study Group. Neurology. 1985;35(8):1096-104.

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

Hughes RA. Plasma exchange versus intravenous immunoglobulin for Guillain-Barré syndrome. Therapeutic Apheresis. 1997;1(2):129-30.

Hughes RA, Brassington R, Gunn AA, van Doorn PA. Corticosteroids for Guillain-Barré syndrome. Cochrane Database Systemat Revi. 2016;10(10):CD001446.

McKhann GM, Griffin JW, Cornblath DR, Mellits ED, Fisher RS, Quaskey SA. Plasmapheresis and Guillain-Barré syndrome: analysis of prognostic factors and the effect of plasmapheresis. Ann Neurol. 1988;23(4):347-53.

Van Koningsveld R, Schmitz PI, Meché FG, Visser LH, Meulstee J, van Doorn PA. Effect of methylprednisolone when added to standard treatment with intravenous immunoglobulin for Guillain-Barré syndrome: randomised trial. Lancet. 2004;363(9404):192-6.

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

Walgaard C, Jacobs BC, Lingsma HF, et al. Second intravenous immunoglobulin dose in patients with Guillain-Barré syndrome with poor prognosis (SID-GBS): a double-blind, randomised, placebo-controlled trial. Lancet Neurol. 2021;20(4):275-83.

Verboon C, van den Berg B, Cornblath DR. Original research: Second IVIg course in Guillain-Barré syndrome with poor prognosis: the non-randomised ISID study. J Neurol Neurosurg Psychiatr. 2020;91(2):113-21.

Doets AY, Hughes RA, Brassington R, Hadden RD, Pritchard J. Pharmacological treatment other than corticosteroids, intravenous immunoglobulin and plasma exchange for Guillain-Barré syndrome. Cochrane Database Systemat Revi. 2020;1(1):CD008630.