Guillain-Barré syndrome: causes, immunopathogenic mechanisms and treatment

Expert Rev Clin Immunol. 2016 Nov;12(11):1175-1189. doi: 10.1080/1744666X.2016.1193006. Epub 2016 Jun 21.

Abstract

Guillain-Barré syndrome is a rare disease representing the most frequent cause of acute flaccid symmetrical weakness of the limbs and areflexia usually reaching its peak within a month. The etiology and pathogenesis remain largely enigmatic and the syndrome results in death or severe disability in 9-17% of cases despite immunotherapy. Areas covered: In terms of etiology, Guillain-Barré syndrome is linked to Campylobacter infection but less than 0.1% of infections result in the syndrome. In terms of pathogenesis, activated macrophages and T cells and serum antibodies against gangliosides are observed but their significance is unclear. Expert commentary: Guillain-Barré syndrome is a heterogeneous condition with numerous subtypes and recent data point towards the role of ganglioside epitopes by immunohistochemical methods. Ultimately, the syndrome results from a permissive genetic background on which environmental factors, including infections, vaccination and the influence of aging, lead to disease.

Keywords: Guillain-Barré; Molecular mimicry; autoimmune neuropathy; immune tolerance; infection and immunity.

Publication types

  • Review

MeSH terms

  • Aging
  • Autoantibodies / metabolism
  • Campylobacter Infections / immunology*
  • Campylobacter Infections / therapy
  • Campylobacter jejuni / immunology*
  • Cross Reactions*
  • Gangliosides / immunology
  • Gene-Environment Interaction
  • Guillain-Barre Syndrome / immunology*
  • Guillain-Barre Syndrome / therapy
  • Humans
  • Immunotherapy / methods*

Substances

  • Autoantibodies
  • Gangliosides