A PROSPECTIVE EVALUATION OF COGNITIVE DECLINE IN EARLY HUNTINGTONS-DISEASE - FUNCTIONAL AND RADIOGRAPHIC CORRELATES

We examined prospectively the relationship between progressive disabil ity in Huntington's disease (HD) and concomitant alterations in neurop sychological functioning and brain imaging indices in a cohort of 60 p atients who were enrolled and followed for 30 to 42 months in a contro lled clinical trial. Standardized measures of functional capacity and neuropsychological performance were collected, and CT was performed, a t regular intervals every 6 to 12 months. Psychomotor skills showed th e most significant and consistent decline among the cognitive function s assessed. Memory disturbances were already present at the time of en rollment, but memory did not deteriorate until patients reached advanc ed stages. Other cognitive operations, such as visual construction and semantic knowledge, manifested small and variable changes over time. CT indices of striatal atrophy correlated only with changes in psychom otor function, while the CT index of frontal atrophy weakly predicted memory and semantic knowledge scores at 42 months. These results confi rmed earlier cross-sectional findings and extended our knowledge of th e evolution of cognitive dysfunction in HD.