BioDrugs

, Volume 14, Issue 1, pp 1–11

A Role for Interferon- β in Guillain-Barré Syndrome?

Current Opinion

DOI: 10.2165/00063030-200014010-00001

Cite this article as:
Créange, A. BioDrugs (2000) 14: 1. doi:10.2165/00063030-200014010-00001

Abstract

Guillain-Barré syndrome (GBS) is an acute inflammatory demyelinating neuropathy that is associated with long-lasting morbidity and a substantial risk of mortality. The 2 reference treatments, plasma exchange and intravenous immunoglobulins (IVIg), do not change the functional prognosis for the most severely ill patients.

The pathogenesis of GBS involves humoral and cellular immune dysfunctions that have only recently been characterised. Antibodies to nerve antigens may participate in complement activation, antibody-dependent macrophage cytotoxicity and reversible conduction failure. The cellular immune reaction is associated with increases in pro-inflammatory cytokines [such as tumour necrosis factor-α (TNFα)] and matrix metalloproteinases (MMPs; e.g. MMP-9), and a decrease in anti-inflammatory cytokines [such as transforming growth factor-β1 (TGFβ1)]. All the changes favour adhesion to and transmigration across the endothelium of immune cells, a key phenomenon associated with GBS. Recovery from GBS is characterised by the normalisation of these changes. Experimental allergic neuritis (EAN), the experimental model of GBS, has strikingly similar immunological characteristics.

The usual treatment options for patients with GBS (plasma exchange and IVIg) mainly target the humoral component of the immune response. Interferon-β (IFNβ) is a cellular immunomodulator that inhibits antigen presentation and TNga production and binding, and modulates macrophage properties. IFNβ increases anti-inflammatory T cell functions and the production of anti-inflammatory cytokines, such as TGFβ1. IFNβ has important effects on leukodiapedesis, caused by modulating the expression of cell adhesion molecules and the MMP-9 proteinases. It has been used with success in EAN, in some patients with acute exacerbation of chronic inflammatory demyelinating polyneuropathy, and in 1 patient with GBS. The pathophysiology of patients with GBS, an understanding of IFNβ properties and results of experimental studies support the investigation of IFNβ in trials of patients with GBS.

Copyright information

© Adis International Limited 2000

Authors and Affiliations

  1. 1.Service de NeurologieCentre Hospitalier Universitaire Henri MondorCréteil CedexFrance

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