Miller-Fisher Syndrome: Are Anti-GAD Antibodies Implicated in Its Pathophysiology?

Ioannis E Dagklis,Sotirios Papagiannopoulos,Ourania Argyropoulou,Varvara Theodoridou,Dimitrios Kazis,Sevasti Bostantjopoulou

doi:10.1155/2016/3431849

Ioannis E Dagklis, Sotirios Papagiannopoulos + Show 4 more

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https://doi.org/10.1155/2016/3431849

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Abstract

Miller-Fisher syndrome (MFS) is considered as a variant of the Guillain-Barre syndrome (GBS) and its characteristic clinical features are ophthalmoplegia, ataxia, and areflexia. Typically, it is associated with anti-GQ1b antibodies; however, a significant percentage (>10%) of these patients are seronegative. Here, we report a 67-year-old female patient who presented with the typical clinical features of MFS. Workup revealed antibodies against glutamic acid decarboxylase (GAD) in relatively high titers while GQ1b antibodies were negative. Neurological improvement was observed after intravenous gamma globulin and follow-up examinations showed a continuous clinical amelioration with simultaneous decline of anti-GAD levels which finally returned to normal values. This case indicates that anti-GAD antibodies may be associated with a broader clinical spectrum and future studies in GQ1b-seronegative patients could determine ultimately their clinical and pathogenetic significance in this syndrome.

Highlights

Miller-Fisher syndrome (MFS) is a variant of the GuillainBarre syndrome (GBS) and its classical clinical triad consists of ophthalmoplegia, ataxia, and areflexia [1]
MFS is a disease of immunological origin involving an immune-mediated process triggered by an antecedent infection [2]
The association of MFS with IgG autoantibodies against GQ1b ganglioside was first described in the early 1990s

Summary

Introduction

Miller-Fisher syndrome (MFS) is a variant of the GuillainBarre syndrome (GBS) and its classical clinical triad consists of ophthalmoplegia, ataxia, and areflexia [1]. It is a relatively rare neurological disorder, accounting for approximately 5% of patients with GBS [1]. MFS is an immune-mediated condition and specific anti-ganglioside antibodies, especially IgG anti-GQ1b antibodies, are found in over 80% of the patients [2]. In a small proportion of GQ1b-seronegative MFS patients no known antibodies are detected. We present a patient with clinically definite MFS with relatively high serum titers of anti-glutamic acid decarboxylase (GAD) antibodies and no anti-GQ1b antibodies or antibodies against other gangliosides

Case Presentation

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Discussion