Guillain-Barré syndrome

Richard A C Hughes, David Cornblath

Research output: Contribution to journalArticle

Abstract

Guillain-Barré syndrome consists of at least four subtypes of acute peripheral neuropathy. Major advances have been made in understanding the mechanisms of some of the subtypes. The histological appearance of the acute inflammatory demyelinating polyradiculoneuropathy (AIDP) subtype resembles experimental autoimmune neuritis, which is predominantly caused by T cells directed against peptides from the myelin proteins P0, P2, and PMP22. The role of T-cell-mediated immunity in AIDP remains unclear and there is evidence for the involvement of antibodies and complement. Strong evidence now exists that axonal subtypes of Guillain-Barré syndrome, acute motor axonal neuropathy (AMAN), and acute motor and sensory axonal neuropathy (AMSAN), are caused by antibodies to gangliosides on the axolemma that target macrophages to invade the axon at the node of Ranvier. About a quarter of patients with Guillain-Barré syndrome have had a recent Campylobacter jejuni infection, and axonal forms of the disease are especially common in these people. The lipo-oligosaccharide from the C jejuni bacterial wall contains ganglioside-like structures and its injection into rabbits induces a neuropathy that resembles acute motor axonal neuropathy. Antibodies to GM1, GM1b, GD1a, and GalNac-GD1a are in particular implicated in acute motor axonal neuropathy and, with the exception of GalNacGD1a, in acute motor and sensory axonal neuropathy. The Fisher's syndrome subtype is especially associated with antibodies to GQ1b, and similar cross-reactivity with ganglioside structures in the wall of C jejuni has been discovered. Anti-GQ1b antibodies have been shown to damage the motor nerve terminal in vitro by a complement-mediated mechanism. Results of international randomised trials have shown equivalent efficacy of both plasma exchange and intravenous immunoglobulin, but not corticosteroids, in hastening recovery from Guillain-Barré syndrome. Further research is needed to discover treatments to prevent 20% of patients from being left with persistent and significant disability.

Original languageEnglish (US)
Pages (from-to)1653-1666
Number of pages14
JournalThe Lancet
Volume366
Issue number9497
DOIs
StatePublished - Nov 5 2005

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Gangliosides
Guillain-Barre Syndrome
Antibodies
Myelin P2 Protein
Experimental Autoimmune Neuritis
Myelin P0 Protein
Miller Fisher Syndrome
Ranvier's Nodes
Campylobacter Infections
T-Lymphocytes
Campylobacter jejuni
Plasma Exchange
Intravenous Immunoglobulins
Peripheral Nervous System Diseases
Oligosaccharides
Cellular Immunity
Axons
Anti-Idiotypic Antibodies
Adrenal Cortex Hormones
Macrophages

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Guillain-Barré syndrome. / Hughes, Richard A C; Cornblath, David.

In: The Lancet, Vol. 366, No. 9497, 05.11.2005, p. 1653-1666.

Research output: Contribution to journalArticle

Hughes, Richard A C ; Cornblath, David. / Guillain-Barré syndrome. In: The Lancet. 2005 ; Vol. 366, No. 9497. pp. 1653-1666.
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