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 |
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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 > School of Infection & Immunity 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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