Detection and prevalence of alpha-latrotoxin-like effects of serum from patients with Guillain-Barré syndrome

Muscle & Nerve
B C JacobsJaap J Plomp

Abstract

Anti-GQ1b antibodies are associated with the Miller Fisher syndrome (MFS), a variant of the Guillain-Barré syndrome (GBS). In the ex vivo mouse diaphragm, anti-GQ1b-positive MFS serum induces muscle fiber twitching, a temporary dramatic increase of spontaneous quantal acetylcholine release, and transmission blockade at neuromuscular junctions (NMJs). These effects resemble those of alpha-latrotoxin (alpha-LTx) and are induced by antibody-mediated activation of complement. We developed an assay for detection of the alpha-LTx-like effect, using muscle fiber twitching as indicator. We tested 89 serum samples from GBS, MFS, and control subjects, and studied correlations with clinical signs, anti-ganglioside antibodies, micro-electrode physiology, and complement deposition at NMJs. Twitching was observed with 76% of the MFS and 10% of the GBS samples. It was associated with ophthalmoplegia and anti-GQ1b antibodies in patients, and with increased spontaneous acetylcholine release and C3c-deposition at mouse NMJs. This study strongly suggests that antibodies to GQ1b (with cross-reactivity to related gangliosides) are responsible for the alpha-LTx-like activity. The twitching assay is an efficient test for detection of this effect, and...Continue Reading

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Citations

Mar 1, 2006·Expert Review of Clinical Immunology·Bethan Lang, Nick Willcox
Oct 11, 2008·Journal of the Peripheral Nervous System : JPNS·Susan K HalsteadHugh J Willison
Dec 13, 2005·Clinical Neurology and Neurosurgery·Yee-Cheun ChanBernard Poon Lap Chan
Sep 27, 2008·Journal of Neuroimmunology·Manel M SantaféJosep Tomàs
Oct 14, 2008·Lancet Neurology·Pieter A van DoornBart C Jacobs
Jan 11, 2008·Brain : a Journal of Neurology·Susan K HalsteadHugh J Willison
Sep 28, 2002·Current Opinion in Neurology·Pieter A Van Doorn, Marcel P J Garssen
Sep 28, 2002·Current Opinion in Neurology·Hans-Peter HartungBernd C Kieseier
Oct 16, 2004·Expert Review of Anti-infective Therapy·Raymond Sw Tsang, Alfonso Valdivieso-Garcia
Jan 7, 2022·Journal of Anatomy·Samuele NegroMichela Rigoni

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