Eighteen patients with early Huntington's disease were compared with a
ge- and IQ-matched control volunteers on tests of executive and mnemon
ic function taken from the Cambridge Neuropsychological Test Automated
Battery. Tests of pattern and spatial recognition memory, spatial spa
n, spatial working memory, spatial planning and visual discrimination
learning/attentional set shifting were employed. These tests have prev
iously been found to be sensitive to the later stages of Huntington's
disease. Patients with early Huntington's disease were found to have a
wide range of cognitive impairments encompassing both visuospatial me
mory and executive functions, a pattern distinct from those seen in ot
her basal ganglia disorders. In contrast to patients with more advance
d Huntington's disease, early Huntington's disease patients were not i
mpaired at simple reversal learning, but were impaired at performing a
n extradimensional shift (EDS). The results will be discussed in relat
ion to the hypothesized neuropathological staging of Huntington's dise
ase and to the anatomical connectivity of the striatum.