Huntington's disease (HD) is a neurodegenerative disorder
diagnosed clinically with the development of choreiform movements.
However, neuropsychological studies have demonstrated cognitive and
psychiatric changes during the preclinical phase (pre-HD) prior to formal
diagnosis. Previous studies have demonstrated the sensitivity of time
reproduction tasks to basal ganglia pathology, as seen in clinical HD and
Parkinson's disease. In this study, 29 pre-HD participants, ranging
from 3 to 39 years from estimated onset (YEO) of HD based on genetic
testing and chronological age, were administered the paced finger-tapping
task using target intervals of 600 and 1200 ms. Mean inter-response
interval, a measure of timing accuracy, did not systematically deviate
from the target interval as a function of YEO. In contrast, timing
variability increased curvilinearly as a function of YEO, but not with
chronological age alone. Motor timing variability, but not accuracy, may
serve as a marker to define the earliest behavioral changes in HD. The
present study is among the first to examine the relationship between
behavioral measures and YEO in pre-HD. (JINS, 2007, 13,
539–543.)