Twenty-six patients with Huntington's disease (HD) and three subjects at risk for HD were evaluated by computed tomographic, neurologic and neuropsychological examinations. These data were used to delineate the sequence of structural changes in early and intermediate HD, and the relationship of these changes to impairment of neurologic and cognitive function. CT scans documented an early neostriatal-frontal focus of atrophy in HD which spreads caudally over the cerebral cortex during the course of the disease. Chorea was positively correlated with caudate atrophy. Functional and cognitive (especially memory and visuospatial) impairments were strongly related to the degree of atrophy. Multiple regression analyses of CT and neuropsychological data further demonstrated that neostriatal changes make a significant contribution to the cognitive as well as to the motor impairments of HD patients.
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