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Journal of the Korean Neurological Association 2009;27(4): 307-312.
Anti-GQ1b Antibody Syndrome; Is the Another Name of Miller Fisher Syndrome or Advent of a New Syndrome?
김종국, 배희준
고신대학교 의과대학 신경과학교실
항-GQ1b 항체증후군; Miller Fisher 증후군의 다른 이름인가, 새로운 증후군의 출현인가?
Jong Kuk Kim
Department of Neurology, Kosin University College of Medicine, Busan, Korea
The Miller Fisher syndrome (MFS), characterized by ataxia, areflexia, and ophthalmoplegia, is a localized variant of Guillain-Barre syndrome (GBS). Bickerstaff’s brainstem encephalitis (BBE) is a related syndrome in which central nervous system abnormalities accompany the classic triad. The discovery of the anti-GQ1b antibody and localization of GQ1b ganglioside in human nervous system enabled us to understand various kinds of symptoms in MFS and related diseases. Molecular mimicry of antigenic epitope from infective organisms such as Campylobacter jejuni with this ganglioside is likely the predominant pathogenic mechanism. This could explain the unusual conditions such as atypical MFS, GBS with ophthalmoplegia and BBE are various manifestations of post-infectious autoimmune neuropathies. Now, we can speculate them as the anti-GQ1b antibody syndrome in according to their immunological profiles. In addition to this, recent new concept of anti-ganglioside complex antibody will lead us to further understanding of these disorders. Key Words: Guillain-Barre syndrome, Miller Fisher syndrome, Bickerstaff’s brainstem encephalitis, Gangliosides, GQ1b, Ophthalmoplegia, Ataxia
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