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Late onset of Huntington's disease.
  1. R H Myers,
  2. D S Sax,
  3. M Schoenfeld,
  4. E D Bird,
  5. P A Wolf,
  6. J P Vonsattel,
  7. R F White,
  8. J B Martin


    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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