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University of Michigan (Drs. Young, Penney, and Starosta-Rubinstein), Ann Arbor, MI; the University of Rochester (Dr. Shoulson), Rochester, NY; the Hereditary Disease Foundation (Ms. Gomez and Dr. Wexler), Santa Monica, CA; the Mailman Research Center (Ms. Travers), Miami, FL; Indiana University (Dr. Ramos), Indianapolis. IN; the University of Southern California (Dr. Snodgrass), Los Angeles. CA; and the University of Zulia (Drs. Bonilla and Moreno), Maracaibo, Venezuela.
We studied 65 Huntington's disease patients and 225 at-risk individuals over the past 4 years. The rate of decline of these untreated patients from Venezuela was similar to that seen in US patients who had received neuroleptic drugs. Chorea, oculomotor dysfunction, and dysdiadochokinesis were early symptoms; parkinsonian features and dystonia came later. Juvenile patients declined nearly twice as fast as adult-onset patients. No distinctive neurologic phenotypes were seen in children of two affected parents.
Address correspondence and reprint requests to Dr. Young, University of Michigan, Neuroscience Laboratory Bldg., 1103 East Huron, Ann Arbor, MI 48104.
This investigation resulted from a contract (N01-NS-0–0452) between the National Institutes of Health, NINCDS (N. Wexler, principal investigator) and the University of Zulia, Maracaibo, Venezuela (E. Bonilla, principal investigator). Primary funding for this study was provided by the National Institutes of Health (USPHS grant NS22031, N. Wexler, Project Officer), the Hereditary Disease Foundation (Santa Monica, CA), the W.M. Keck Foundation (Los Angeles, CA), the Vollmer Foundation (New York, NY), and the Regenstein Foundation (Chicago, IL). Additional support for this study was provided by USPHS grants NS 00420, NS 00464, and NS 15655 for A.B.Y. and J.B.P., and NS 17978, NS 00397, and RR 00044 for I.S., and by the Lena Marcus Trust.
Presented in part at the thirty-fifth annual meeting of the American Academy of Neurology, San Diego, CA, April 1983.
Accepted for publication June 19, 1985.
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