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October 1966

Huntington's Chorea: A Multidisciplinary Study of Affected Parents and First Generation Offspring

Author Affiliations

From the Department of Medicine, Division of Neurology, Department of Psychiatry, the College of Medicine, and the Department of Speech and Hearing Science, the College of Arts and Sciences, Ohio State University, Columbus, Ohio.

Arch Neurol. 1966;15(4):345-355. doi:10.1001/archneur.1966.00470160011002

HUNTINGTON'S chorea is a well-recognized, genetically determined disease of autosomal dominant inheritance. Though usually considered to be a disorder having its onset between the ages of 20 and 40 years, there are many well-documented cases of this disease occurring in the teens and early childhood.1,2 The presence of choreiform movements in a patient with progressive mental deterioration and a heredofamilial background of a similar nature makes the diagnosis of Huntington's chorea obvious in most cases. However, this disease may manifest itself in varying forms; without the proper family history, it can be confused with other diseases of the central nervous system.

A recent study3 of a patient and members of his family with unusual features of Huntington's chorea prompted us to further investigate the first generation offspring of those parents affected with the disorder. In the initial report only two children of affected parents were evaluated: a

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