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Prediction of disease progression in Miller Fisher and overlap syndromes
Christine Verboon1, Heleen van Berghem1, Pieter A van Doorn1
1Department of Neurology, Erasmus MC, Rotterdam, The Netherlands.
Abstract:
Patients with Miller Fisher syndrome (MFS) may have a relatively mild clinical course or progress to Guillain-Barré syndrome (GBS) with limb weakness (MFS-GBS overlap syndrome). Other variants in this spectrum are GBS with ophthalmoparesis and Bickerstaff's Brainstem encephalitis (BBE). To compare the clinical course of MFS and overlap syndromes and to identify predictors of disease progression. In a prospective study of 170 patients with GBS and variant forms, 37 (22%) had a MFS, MFS-GBS overlap syndrome, ophthalmoplegic GBS or BBE. The clinical, serological, and electrophysiological features were compared. Twenty-three patients presented with MFS, of which 10 (43%) developed limb weakness (MFS-GBS overlap syndrome). All these transitions occurred in the first week after onset of symptoms. There were no differences in the clinical, electrophysiological and serological features at entry between MFS and MFS-GBS. Twelve patients had ophthalmoplegic GBS and the disease severity at nadir and outcome was worse than in the patients with a MFS-GBS overlap syndrome. No early predictors for progression from MFS to MFS-GBS overlap syndrome were found. All transitions occurred in the first week. This finding implicates that all patients with MFS need careful monitoring for at least 1 week.
Insights
Miller Fisher syndrome (MFS) can progress to Guillain-Barré syndrome (GBS) with limb weakness. Early predictors for this MFS-GBS overlap were not found, necessitating close monitoring in the first week.
Area of Science:
- Neurology
- Neuroimmunology
- Clinical Neuroscience
Background:
- Miller Fisher syndrome (MFS) is a rare variant of Guillain-Barré syndrome (GBS).
- MFS can present with ophthalmoplegia, ataxia, and areflexia, and may progress to MFS-GBS overlap syndrome with limb weakness.
- Other related disorders include GBS with ophthalmoparesis and Bickerstaff's Brainstem encephalitis (BBE).
Purpose of the Study:
- To compare the clinical course of MFS and overlap syndromes.
- To identify predictors of disease progression in MFS.
- To differentiate clinical features among MFS, MFS-GBS overlap syndrome, ophthalmoplegic GBS, and BBE.
Main Methods:
- Prospective study of 170 patients with GBS and its variants.
- Analysis of clinical, serological, and electrophysiological features.
- Comparison of disease progression and outcomes between different MFS spectrum disorders.
Main Results:
- Of 37 patients with MFS spectrum disorders, 23 had MFS, and 10 (43%) progressed to MFS-GBS overlap syndrome.
- All transitions from MFS to MFS-GBS overlap occurred within the first week of symptom onset.
- Ophthalmoplegic GBS showed worse severity and outcome compared to MFS-GBS overlap syndrome; no early predictors for MFS progression were identified.
Conclusions:
- Patients with MFS require vigilant monitoring for at least one week due to the potential for progression to MFS-GBS overlap syndrome.
- No early clinical, electrophysiological, or serological predictors distinguish MFS patients who will progress.
- Ophthalmoplegic GBS represents a more severe clinical course than MFS-GBS overlap syndrome.
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