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Clinical and neuropathologic assessment of severity in Huntington's disease

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 of Public Health, Boston University (Dr. Cupples), 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. Richardson), Boston, MA; the Department of Neurology, Massachusetts General Hospital, Harvard Medical School (Dr. Martin), Boston, MA; and Ralph Lowell Laboratories, Mailman Research Center, McLean Hospital and Harvard Medical School (Dr. Bird), Belmont MA.

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.

Address correspondence and reprint requests to Dr. Myers, Neurological Epidemiology & Genetics, Boston University Medical Center, 720 Harrison Avenue, Suite 1105, Boston, MA 02118.

Supported in part by NINCDS Grant 16367 (Huntington's Disease Center Without Walls), NIMH/NINCDS Grant 31862 (Brain Bank), Hereditary Disease Foundation, The Fonds National Suisse de la Recherche Scientifique (Switzerland), and the Massachusetts Huntington Disease Society of America.

Received March 18, 1987. Accepted for publication in final form June 15, 1987.

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