Late onset of Huntington's disease

Date
1985
DOI
Authors
Myers, R. H.
Sax, D. S.
Schoenfeld, M.
Bird, E. D.
Wolf, P. A.
Vonsattel, J. P.
Martin, J. B.
Version
OA Version
Citation
Myers, R. H., Sax, D. S., Schoenfeld, M., Bird, E. D., Wolf, P. A., Vonsattel, J. P., White, R. F., & Martin, J. B. (1985). Late onset of huntington's disease. Journal of Neurology, Neurosurgery, and Psychiatry, 48(6), 530-534.
Abstract
Twenty-five patients with late-onset Huntington's disease were studied; motor impairment appeared at age 50 years or later. The average age at onset of chorea was 57.5 years, with an average age at diagnosis of 63.1 years. Approximately 25% of persons affected by Huntington's disease exhibit late onset. A preponderance of maternal transmission was noted in late-onset Huntington's disease. The clinical features resembled those of mid-life onset Huntington's disease but progressed more slowly. Neuropathological evaluation of two cases reveal less severe neuronal atrophy than for mid-life onset disease.
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