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Disease Profile

Miller-Fisher syndrome

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.
1-9 / 1 000 000

331 - 2,979

US Estimated

1-9 / 1 000 000

514 - 4,622

Europe Estimated

Age of onset

All ages

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ICD-10

G61.0

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

Cranial variant of Guillain-Barré syndrome; Cranial variant of GBS

Categories

Nervous System Diseases

Summary

Miller Fisher syndrome is a rare acquired nerve disease considered to be a variant of Guillain-Barré syndrome.[1] The main features are lack of muscle coordination (ataxia), eye muscle weakness resulting in the inability to move the eyes in several directions (ophthalmoplegia), and the absence of tendon reflexes. Symptoms often start several days after a viral illness.[1][2][3] Other symptoms include generalized muscle weakness and respiratory failure. The cause is not known, but it is thought to be an autoimmune disease in which there are autoantibodies that attack the nerves. In most people with Miller Fisher syndrome an antibody (anti-GQ1b) is identified. The presence of these autoantibodies helps confirm the diagnosis of the syndrome.[3] Treatment includes intravenous immunoglobulin (IVIG), plasmapheresis (a plasma exchange procedure in which the antibodies are removed from the blood) and supportive care. The prognosis is usually good, and in most cases, there is almost complete recovery within 6 months.[3][4] In rare cases, the syndrome may progress and permanent neurological deficits may be present.[3][4][5]

Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

    Learn more

    These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

    Where to Start

      In-Depth Information

      • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
      • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
      • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
      • PubMed is a searchable database of medical literature and lists journal articles that discuss Miller-Fisher syndrome. Click on the link to view a sample search on this topic.

        References

        1. NINDS Miller Fisher Syndrome Information Page. NINDS. August 2011; https://www.ninds.nih.gov/disorders/miller_fisher/miller_fisher.htm. Accessed 10/21/2011.
        2. Miller-Fisher syndrome. Radiopaedia. https://radiopaedia.org/articles/miller-fisher-syndrome.
        3. Miller-Fisher syndrome. GBS/CIDP Foundation International. https://www.gbs-cidp.org/variants/miller-fisher-syndrome/.
        4. Verboon C, van Berghem H, van Doorn PA, Ruts L & Jacobs BC. Prediction of disease progression in Miller Fisher and overlap syndromes. J Peripher Nerv Syst. October 24, 2017; https://www.ncbi.nlm.nih.gov/pubmed/29065229.
        5. Barbato F, Di Paolantonio A, Distefano M, Mastrorosa A, Sabatelli M, Servidei S & Luigetti M. Recurrent miller fisher: a new case report and a literature review. Clin Ter. May-June, 2017; 168(3)::208-213. https://www.ncbi.nlm.nih.gov/pubmed/28612899.