Miller Fisher syndrome

Brief overview and update with a focus on electrophysiological findings

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31 Citations (Scopus)

Abstract

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

Original languageEnglish
JournalEuropean Journal of Neurology
Volume19
Issue number1
DOIs
Publication statusPublished - Jan 2012

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Miller Fisher Syndrome
Ophthalmoplegia
H-Reflex
Abnormal Reflexes
Plasmapheresis
Antibodies
Intravenous Immunoglobulins
Encephalitis
Ataxia
Neuroimaging
Action Potentials
Brain Stem
Cerebrospinal Fluid
Central Nervous System
Incidence
Serum
Population

Keywords

  • Cerebrospinal fluid
  • Clinical signs
  • Electrophysiology
  • Miller Fisher syndrome
  • Pathology
  • Prognosis

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology

Cite this

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title = "Miller Fisher syndrome: Brief overview and update with a focus on electrophysiological findings",
abstract = "Miller Fisher syndrome (MFS), a variant of the Guillain-Barr{\'e} 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",
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