REDUCED BASAL GANGLIA VOLUME ASSOCIATED WITH THE GENE FOR HUNTINGTONS-DISEASE IN ASYMPTOMATIC AT-RISK PERSONS

Previous investigations using linear CT measures found no evidence of caudate atrophy in asymptomatic persons who have the DNA haplotype lin ked to the Huntington's disease (HD) gene. We measured volumes of the caudate, putamen, and globus pallidus on MRIs of 10 gene marker-positi ve and 18 gene marker-negative asymptomatic at-risk persons. The volum es of all basal ganglia structures were significantly reduced in the m arker-positive group, even after controlling for age, total brain volu me, and minor neurologic signs. Discriminant function analysis using b asal ganglia volumes and age as predictor variables correctly identifi ed genetic status in 86% of subjects. These results indicate that basa l ganglia volume is reduced before individuals become symptomatic with HD.