Late onset of huntington's disease
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CitationMyers, 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.
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.