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Miller Fisher syndrome: axonal, demyelinating or both?
1Department of Neurology, Beth Israel Medical Center, New York, New York, USA. SScelsa@BethIsraelNY.Org
Electromyography and Clinical Neurophysiology
|January 13, 2001
Summary
Miller Fisher syndrome (MFS) typically involves axonal polyneuropathy, while ataxic Guillain-Barré syndrome (atxGBS) presents as demyelinating polyneuropathy. Electrophysiology helps differentiate these neurological conditions.
Area of Science:
- Neurology
- Neurophysiology
Background:
- Miller Fisher syndrome (MFS) pathophysiology is debated, with uncertainty regarding its axonal versus demyelinating nature.
- The distinct pathophysiology of ataxic Guillain-Barré syndrome (atxGBS), a variant of GBS, is also under investigation.
Purpose of the Study:
- To investigate the electrophysiologic characteristics of MFS and atxGBS.
- To clarify whether MFS is primarily axonal or demyelinating and to determine the pathophysiology of atxGBS.
Main Methods:
- Review of electrophysiologic findings in 6 patients with MFS and 2 patients with atxGBS.
- Categorization of electrophysiologic findings as axonal or demyelinating neuropathy based on established criteria.
Main Results:
- Five out of six MFS patients showed electrophysiologic evidence of axonal, predominantly sensory polyneuropathy; one patient met criteria for demyelinating polyneuropathy.
- Both atxGBS patients exhibited demyelinating sensorimotor polyneuropathy.
- MFS findings suggest a predominantly axonal, sensory polyneuropathy, though demyelinating forms may be underdiagnosed.
Conclusions:
- Electrophysiologic abnormalities in MFS typically indicate a predominantly axonal, sensory polyneuropathy.
- Demyelinating forms of MFS may occur and potentially be under-diagnosed.
- AtxGBS, based on this cohort, appears to be a predominantly demyelinating polyneuropathy.