Fisher Syndrome and Anti-GQ1b Antibody

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  • Chiba Atsuro
    Department of Neurology, School of Medicine, Kyorin University

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  • Fisher症候群と抗GQ1b抗体

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Description

Fisher syndrome (FS) is a regional variant of Guillain-Barré syndrome (GBS) and is characterized by three clinical symptoms, i.e., ophthalmoplegia, ataxia, and areflexia, which are known as the FS triad. In 1956, Miller Fisher was the first to report a detailed clinical description of the characteristics of this syndrome. The pathological mechanisms and nosology of the syndrome have been discussed for several decades, but the arguments lacked a disease-specific biomarker. Further research on anti-glycolipid antibodies related to GBS that began in the late 1980s led to the discovery of a disease-specific biomarker, an immunoglobulin G (IgG) antibody against ganglioside GQ1b, in FS. An anti-GQ1b antibody-related animal model of FS has not yet been established; however, the pathological significance of the anti-GQ1b antibody has been strongly suggested based on the following findings: high incidence and disease-specificity of the antibody; increase in the antibody titers before neurological onset; unique distribution of the GQ1b-antigen consistent with the clinical characteristics of FS in human peripheral nervous tissues; and the presence of GQ1b-mimicking oligosaccharide structures in causative organisms of preceding infection. Serum IgG antibodies against ganglioside GQ1b have also been detected in other conditions with clinical characteristics similar to FS, such as GBS with ophthalmoplegia, acute ophthalmoplegia, ataxia following an infection, and atypical cases with central nervous system manifestations. Therefore, these antibodies are considered to be important diagnostic biomarkers for FS and other related conditions.

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