Recent studies have detected basal ganglia atrophy
in clinically asymptomatic persons with the genetic mutation
that causes Huntington's disease (HD). Whether reductions
in caudate and putamen volume on MRI scans are associated
with changes in cognitive and neurologic functioning was
examined in 13 healthy adults with the IT-15 mutation.
Reduced striatal volume was found to correlate with greater
neurologic (largely motor) impairment, slower mental processing
speed, and poorer verbal learning, although none of the
participants met even liberal criteria for clinical diagnosis
of HD. These correlations are strikingly similar to those
observed in symptomatic HD patients, possibly reflecting
the earliest manifestations of disease. (JINS,
1998, 4, 467–473.)
