G Riche1, C Caudie, C Vial, C Bourrat. 1. Service de neurologie A, hôpital neurologique et neurochirurgical Pierre-Wertheimer, Lyon, France.
Abstract
INTRODUCTION: Miller-Fisher syndrome is defined by the triad: ophthalmoplegia, ataxia and areflexia. This rare entity is generally regarded as a variant of the Guillain-Barré syndrome, although neurophysiological patterns differ. In the acute phase of the disease, sera of affected patients contain high titers of antiganglioside anti-GQ1b, which is a specific marker. Recurrences are exceptional. EXEGESE: We report the case of a man with three recurrences of Miller-Fisher syndrome within 16 years. Anti-GQ1b antibody titers were elevated during an episode, decreasing but not completely and vanishing 2 years later. Intravenous human immunoglobulin treatment probably accelerated improvement at the two last episodes. CONCLUSIONS: Some experimental and immunohistochemical data from the literature argue for a probable direct pathogenic role of antibodies against GQ1b ganglioside in this syndrome. This should be a rationale for the use of immunomodulating treatments.
INTRODUCTION:Miller-Fisher syndrome is defined by the triad: ophthalmoplegia, ataxia and areflexia. This rare entity is generally regarded as a variant of the Guillain-Barré syndrome, although neurophysiological patterns differ. In the acute phase of the disease, sera of affected patients contain high titers of antiganglioside anti-GQ1b, which is a specific marker. Recurrences are exceptional. EXEGESE: We report the case of a man with three recurrences of Miller-Fisher syndrome within 16 years. Anti-GQ1b antibody titers were elevated during an episode, decreasing but not completely and vanishing 2 years later. Intravenous human immunoglobulin treatment probably accelerated improvement at the two last episodes. CONCLUSIONS: Some experimental and immunohistochemical data from the literature argue for a probable direct pathogenic role of antibodies against GQ1b ganglioside in this syndrome. This should be a rationale for the use of immunomodulating treatments.