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Miller Fisher syndrome: an updated narrative review
Ciro Maria Noioso1, Liliana Bevilacqua1, Gabriella Maria Acerra1
1Neurology Unit, University Hospital "San Giovanni di Dio e Ruggi d'Aragona", University of Salerno, Salerno, Italy.
Introduction:
Miller Fisher syndrome (MFS) is considered a rare variant of Guillain-Barré syndrome (GBS), a group of acute-onset immune-mediated neuropathies characterized by the classic triad of ataxia, areflexia, and ophthalmoparesis. The present review aimed to provide a detailed and updated profile of all aspects of the syndrome through a collection of published articles on the subject, ranging from the initial description to recent developments related to COVID-19.
Methods:
We searched PubMed, Scopus, EMBASE, and Web of Science databases and gray literature, including references from the identified studies, review studies, and conference abstracts on this topic. We used all MeSH terms pertaining to "Miller Fisher syndrome," "Miller Fisher," "Fisher syndrome," and "anti-GQ1b antibody."
Results:
An extensive bibliography was researched and summarized in the review from an initial profile of MFS since its description to the recent accounts of diagnosis in COVID-19 patients. MFS is an immune-mediated disease with onset most frequently following infection. Anti-ganglioside GQ1b antibodies, detected in ~85% of patients, play a role in the pathogenesis of the syndrome. There are usually no abnormalities in MFS through routine neuroimaging. In rare cases, neuroimaging shows nerve root enhancement and signs of the involvement of the central nervous system. The most consistent electrophysiological findings in MFS are reduced sensory nerve action potentials and absent H reflexes. Although MFS is generally self-limited and has excellent prognosis, rare recurrent forms have been documented.
Conclusion:
This article gives an updated narrative review of MFS with special emphasis on clinical characteristics, neurophysiology, treatment, and prognosis of MFS patients.
Insights
Miller Fisher syndrome (MFS), a rare variant of Guillain-Barré syndrome (GBS), is an immune-mediated neuropathy. This review details MFS clinical features, diagnosis, and prognosis, including recent COVID-19 connections.
Area of Science:
- Neurology
- Immunology
- Rare Diseases
Background:
- Miller Fisher syndrome (MFS) is a rare variant of Guillain-Barré syndrome (GBS).
- It is characterized by ataxia, areflexia, and ophthalmoparesis.
- MFS is an immune-mediated neuropathy often triggered by infection.
Purpose of the Study:
- To provide a comprehensive and updated review of Miller Fisher syndrome.
- To cover MFS from its initial description to recent findings, including COVID-19 associations.
- To emphasize clinical characteristics, neurophysiology, treatment, and prognosis.
Main Methods:
- Systematic literature search of PubMed, Scopus, EMBASE, and Web of Science.
- Inclusion of gray literature, reference lists, and conference abstracts.
- Use of MeSH terms related to Miller Fisher syndrome and anti-GQ1b antibodies.
Main Results:
- Anti-ganglioside GQ1b antibodies are implicated in pathogenesis, present in ~85% of patients.
- Neuroimaging is typically normal, but rare cases show nerve enhancement or CNS involvement.
- Electrophysiology reveals reduced sensory nerve action potentials and absent H reflexes.
Conclusions:
- Miller Fisher syndrome is generally self-limited with an excellent prognosis.
- Recurrent forms of MFS are rare but documented.
- The review offers an updated perspective on MFS management and outcomes.

