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Clinical and neuropathologic assessment of severity in Huntington's disease
Abstract
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.
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“To avoid confounding the analysis of these clinical features by the known, strong relationship between HTT CAG repeat size and disease pathology and onset [4,28,31,32], CAG-adjusted residuals were calculated for all continuous clinical traits”
From miR-10b-5p expression in Huntington’s disease brain relates to age of onset and the extent of striatal involvement (2015) · cites this paper as [32]
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Metadata
- Authors
- Myers, R. H., Vonsattel, J. P., Stevens, T. J., Cupples, L. A., Richardson, E. P., Martin, J. B., & Bird, E. D.
- Affiliations
- From the Department of Neurology, Boston University Medical School and Massachusetts General Hospital, Harvard Medical School (Dr. Myers), Boston, MA; The C. S. Kubik Laboratory for Neuropathology, James Homer Wright Pathology Laboratories, Massachusetts General Hospital, and the Department of Neurology-Neuropathology, Harvard Medical School (Dr. Vonsattel), Boston, MA; Ralph Lowell Laboratories, Mailman Research Center, McLean Hospital and Harvard Medical School (Mr. Stevens), Belmont, MA; School...
- Venue
- Neurology
- Published
- 1988-03
- Volume
- 38
- Issue
- 3
- Pages
- 341-341
- ISSN
- 0028-3878
- 1526-632X
- Cited by
- 178
- References
- 0
- Language
- en
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