Clinical and neuropathologic assessment of severity in huntington’s disease

Academic Article


  • Clinical records were evaluated for 163 Huntington’s disease patients in whom postmortem brain specimens had been graded for degree of neuropathologic involvement in the striatum. Juvenile/adolescent onset (4 to 19 years of age) was associated with very severe neuropathologic involvement produced by an apparent rapid degenerative process. Cases of early (20 to 34 years) and midlife (35 to 49 years) onset had respectively less severe striatal involvement, suggesting a slower degenerative progression. High correlations among the grade of neuropathologic involvement, cell counts of neurons, and a rating of physical disability suggest that each represents a common underlying degenerative process of the disease. The relationship between the age at onset and the extent of neuropathologic involvement suggests that a single mechanism may determine both onset and rate of degenerative disease progression. © 1988 American Academy of Neurology.
  • Published In

  • Neurology  Journal
  • Digital Object Identifier (doi)

    Author List

  • Myers RH; Vonsattel JP; Stevens TJ; Cupples LA; Richardson EP; Martin JB; Bird ED
  • Start Page

  • 341
  • End Page

  • 347
  • Volume

  • 38
  • Issue

  • 3