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Miller fisher syndrome: a hospital-based retrospective study
1Neurology, The Neurological Institute, Veterans General Hospital, Taipei, Taiwan, ROC.
Abstract:
Miller Fisher syndrome (MFS), characterized as ataxia, areflexia and ophthalmoplegia, is generally considered as a variant of Guillain-Barré syndrome (GBS). However, some investigators believed that the syndrome could be explained by a central origin. To obtain more information about MFS for comparison with GBS, we conducted a retrospective study by analyzing the clinical data of MFS patients admitted to our hospital over a period of 11 years. The calibrated male/female ratio was 1.65. A seasonal clustering in winter was noted. The percentage of MFS among GBS was especially high (18%, 11/60) in Taiwan when compared with other series. Involvement of limb muscle strength, autonomic function and cranial nerves, except ocular motor nerves, was rarely found in our patients. When MFS is accompanied by limb weakness, it might represent a transitional form between MFS and GBS. Bulbar palsy and dysautonomia might predict a relatively poor prognosis. To obtain more reliable information, lumbar puncture should be done 1 week after disease onset, and electrophysiological tests should be done serially in every MFS patient. Eighty percent (80%, 4/5) of our patients were positive for IgG anti-GQ(1b) antibody activity. In our study, there is more evidence indicating that MFS is a peripheral nervous system disorder; however, no definite conclusion could be made as to whether MFS is exclusively a peripheral or central nervous system disorder. We think MFS is an immune-mediated clinical entity which mainly involves the peripheral nervous system with rare involvement of other parts of the central nervous system.
Insights
Miller Fisher syndrome (MFS) is an immune-mediated disorder primarily affecting the peripheral nervous system. This study found MFS is a distinct variant of Guillain-Barré syndrome (GBS), with specific clinical features and high anti-GQ1b antibody activity.
Area of Science:
- Neurology
- Immunology
- Clinical Medicine
Background:
- Miller Fisher syndrome (MFS) is often considered a variant of Guillain-Barré syndrome (GBS).
- The exact origin of MFS, whether central or peripheral nervous system, remains debated.
- Understanding MFS characteristics is crucial for differentiating it from GBS.
Purpose of the Study:
- To analyze the clinical features of MFS patients.
- To compare MFS with Guillain-Barré syndrome (GBS).
- To investigate the potential central or peripheral nervous system origin of MFS.
Main Methods:
- Retrospective analysis of clinical data from MFS patients over 11 years.
- Examination of patient demographics, seasonal occurrence, and clinical manifestations.
- Serological testing for IgG anti-GQ1b antibody activity.
Main Results:
- MFS showed a male predominance (1.65:1) and seasonal clustering in winter.
- Taiwan reported a high MFS prevalence among GBS cases (18%).
- Limb weakness, autonomic dysfunction, and cranial nerve involvement were rare; 80% tested positive for anti-GQ1b antibodies.
Conclusions:
- MFS presents distinct clinical features compared to typical GBS.
- Evidence suggests MFS is primarily a peripheral nervous system disorder, though a central component cannot be entirely excluded.
- MFS is an immune-mediated entity, often associated with anti-GQ1b antibodies.