Correlation between triplet repeat expansion and computed tomography measures of caudate nuclei atrophy in Huntington's disease

Huntington's disease (HD) is an autosomal dominant, progressive disorder ch aracterized by choreic movements, cognitive de, dine, and psychiatric manif estations. Eleven patients with HD were retrospectively selected from a lar ger group of 42 patients based on the similar, early onset of the disease ( between 21 and 30 years) and the same duration of HD at the moment of compu ted tomography (CT) examination (5 years). A significant correlation betwee n the number of CAG trinucleotides and the bicaudate index or the frontal h orn index, two indices of caudate atrophy, was found in this group of patie nts. Our results, although in a small number of patients, suggest that the striatal degeneration, assessed by CT measures, is primarily regulated by t he size of expanded CAG repeats.