Cerebral blood flow and cognitive testing correlate in Huntington's disease

Brain atrophy estimated by computed tomographic (CT) scanning and mean hemispheric and regional gray matter cerebral blood flow (CBF) values were measured in patients with mild to moderate Huntington's disease (HD) (N = 16) using the xenon Xe 133 inhalation method and in asymptomatic blood relatives at risk from HD (N = 6) using both the xenon Xe 133 inhalation and the stable xenon CT contrast CBF methods. Results were compared with measurements in two groups of age-matched normal volunteers (N = 48 and N = 42, respectively). Significant brain atrophy in the vicinity of both caudate nuclei was present in patients with HD but not in at-risk individuals. Mean hemispheric xenon Xe 133 CBF values were reduced in patients with HD but seemed to be normal in at-risk individuals. In HD, reductions in CBF were found in both frontotemporal regions. Correlations were found between severity of dementia estimated by reductions of Mini-Mental Status Questionnaire scores and reductions of either mean hemispheric or regional frontotemporal CBF values in HD. The CT estimates of brain atrophy and three-dimensional CBF by stable xenon-contrast measurements were normal in asymptomatic individuals at risk from HD.