| Miller Fisher syndrome: brief overview and update with a focus on electrophysiological findings. | |
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MedLine Citation:
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PMID: 21631649 Owner: NLM Status: Publisher |
Abstract/OtherAbstract:
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Miller Fisher syndrome (MFS), a variant of the Guillain-Barré syndrome (GBS), is characterized by ophthalmoplegia, ataxia, and areflexia. The annual incidence is around one patient per one million population. The antiganglioside anti-GQ1b IgG antibody has a role in the pathogenesis of the syndrome, especially of ophthalmoplegia. The presence of this antibody in the serum can be identified in over 80% of the patients, peaking in the first week, whereas albuminocytological dissociation in the cerebrospinal fluid (CSF) appears later. The most consistent electrophysiological findings in MFS are reduced sensory nerve action potentials and absent H reflexes. More variability is seen with F waves and various investigations involving cranial structures. Although there are usually no abnormalities in MFS by routine neuroimaging, in a few cases, contrast enhancement of nerve roots and signs of central nervous system involvement were described supporting the hypothesis of an anti-GQ1b-syndrome, a continuum involving GBS, MFS, and Bickerstaff's brainstem encephalitis. Owing to the lack of randomized trials, treatments used for GBS (intravenous immunoglobulin and plasmapheresis) are usually applied, although from retrospective analyses, the outcome was similar between treated and untreated subjects. The outcome of MFS is usually good with case fatality of < 5%. In the few autopsy cases, macroscopic abnormalities were generally not seen in the nervous system. Microscopic examination of the peripheral nervous system (including cranial nerves) showed segmental demyelination with minimal perivascular infiltration with normal spinal cord and brain stem. |
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Authors:
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Z Arányi; T Kovács; I Sipos; D Bereczki |
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Publication Detail:
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Type: JOURNAL ARTICLE Date: 2011-6-1 |
Journal Detail:
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Title: European journal of neurology : the official journal of the European Federation of Neurological Societies Volume: - ISSN: 1468-1331 ISO Abbreviation: - Publication Date: 2011 Jun |
Date Detail:
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Created Date: 2011-6-2 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 9506311 Medline TA: Eur J Neurol Country: - |
Other Details:
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Languages: ENG Pagination: - Citation Subset: - |
Copyright Information:
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© 2011 The Author(s). European Journal of Neurology © 2011 EFNS. |
Affiliation:
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Department of Neurology, Semmelweis University, Budapest, Hungary. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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