Guillain-Barré syndrome: a century of progress

Goodfellow, J. A. and Willison, H. J. (2016) Guillain-Barré syndrome: a century of progress. Nature Reviews Neurology, 12(12), pp. 723-731. (doi:10.1038/nrneurol.2016.172) (PMID:27857121)

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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.

Item Type:Articles
Status:Published
Refereed:Yes
Glasgow Author(s) Enlighten ID:Willison, Professor Hugh and Goodfellow, Mr John
Authors: Goodfellow, J. A., and Willison, H. J.
College/School:College of Medical Veterinary and Life Sciences > Institute of Infection Immunity and Inflammation
College of Medical Veterinary and Life Sciences > School of Medicine, Dentistry & Nursing
Journal Name:Nature Reviews Neurology
Publisher:Nature Publishing Group
ISSN:1759-4758
ISSN (Online):1759-4766
Published Online:18 November 2016
Copyright Holders:Copyright © 2016 Macmillan Publishers Limited
First Published:First published in Nature Reviews Neurology 12(12): 723-731
Publisher Policy:Reproduced in accordance with the publisher copyright policy

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