title: Guillain-Barré syndrome: a century of progress.
abstract: In 1916, Guillain, Barré and Strohl reported on two cases of acute flaccid paralysis with high cerebrospinal fluid protein levels and normal cell counts - novel findings that identified the disease we now know as Guillain-Barré syndrome (GBS). 100 years on, we have made great progress with the clinical and pathological characterization of GBS. Early clinicopathological and animal studies indicated that GBS was an immune-mediated demyelinating disorder, and that severe GBS could result in secondary axonal injury; the current treatments of plasma exchange and intravenous immunoglobulin, which were developed in the 1980s, are based on this premise. Subsequent work has, however, shown that primary axonal injury can be the underlying disease. The association of Campylobacter jejuni strains has led to confirmation that anti-ganglioside antibodies are pathogenic and that axonal GBS involves an antibody and complement-mediated disruption of nodes of Ranvier, neuromuscular junctions and other neuronal and glial membranes. Now, ongoing clinical trials of the complement inhibitor eculizumab are the first targeted immunotherapy in GBS.
authors.affiliation: Neuroimmunology Laboratory, Laboratory Building, Queen Elizabeth University Hospital, Glasgow, G51 4TF, Scotland, UK.
authors.forename: John A
authors.initials: JA
authors.lastname: Goodfellow
authors.affiliation: Institute of Infection, Immunity and Inflammation, University of Glasgow, University Place, Glasgow, G12 8TA, Scotland, UK.
authors.forename: Hugh J
authors.initials: HJ
authors.lastname: Willison
mesh_terms: D000818:Animals; D001369:Axons / Q000473:pathology*; D016123:Campylobacter jejuni / Q000472:pathogenicity*; D005732:Gangliosides / Q000276:immunology*; D020275:Guillain-Barre Syndrome / Q000209:etiology* / Q000266:history / Q000276:immunology* / Q000628:therapy; D049673:History, 20th Century; D049674:History, 21st Century; D006801:Humans; D007167:Immunotherapy / Q000379:methods*
chemical_list: D005732:Gangliosides