Clinical and neuropathologic assessment of severity in Huntington's disease

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 produc...

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Bibliographic Details
Published inNeurology Vol. 38; no. 3; p. 341
Main Authors Myers, R H, Vonsattel, J P, Stevens, T J, Cupples, L A, Richardson, E P, Martin, J B, Bird, E D
Format Journal Article
LanguageEnglish
Published United States 01.03.1988
Subjects
Aging - physiology
Brain - pathology
Cell Count
Diagnosis-Related Groups
Female
Humans
Huntington Disease - mortality
Huntington Disease - pathology
Huntington Disease - physiopathology
Male
Middle Aged
Neurons - pathology
Severity of Illness Index
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Summary: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.
ISSN:0028-3878
DOI:10.1212/WNL.38.3.341