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A Family Study of Huntington's Chorea with Unusual Manifestations

Ann Intern Med. 1966;64(3):570-574. doi:10.7326/0003-4819-64-3-570
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Huntington's chorea is a relatively rare genetically determined neurological disorder, the incidence of which is about 5 per 100,000 in the United States (1, 2). Classically, it is characterized by: [1] an autosomal dominant mode of inheritance with complete penetrance; [2] onset between the ages of 25 and 45 years; [3] signs and symptoms of choreic movements, hypotonia, intellectual and emotional impairment; and [4] gradual worsening of the disorder. Difficulty establishing the correct diagnosis may be encountered if other neurological signs are present or if some of the typical characteristics are missing. In an early study, Davenport and Muncy (3)


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