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Extremely rare form of familial choreoathetosis with infantile onset.

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Infantile Choreoathetosis of Fisher.

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Described in only one family, affecting four generations. Autosomal dominant inheritance.

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Distinguishing features were infantile onset with exclusive neurologic impairment including progressive choreoathetosis predominantly affecting the legs and consequently impairing gait. Occasionally, pyramidal tract signs can occur. Patients do not show any signs of dementia, seizures, or rigidity.

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No specific anesthetic considerations are expected to arise.

Fisher M, Sargent, J, Drachman D: Familial inverted choreoathetosis. Neurology 29:1627, 1979.  [PubMed: 159419]

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