Late onset of Huntington's disease

Academic Article


  • 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.
  • Digital Object Identifier (doi)

    Author List

  • Myers RH; Sax DS; Schoenfeld MS; Bird ED; Wolf PA; Vonsattel JP; White RF; Martin JB
  • Start Page

  • 530
  • End Page

  • 534
  • Volume

  • 48
  • Issue

  • 6