This study examined whether neuropsychological changes emerge over time in asymptomatic adults who have the

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.

There have been few studies of psychogenic amnesia based on a cognitive or neuropsychological framework. In the present study, a patient with acute onset of profound psychogenic retrograde amnesia was examined. Although her performance on neuropsychological tasks revealed intact anterograde memory, language functioning, visuospatial and constructional skills, and mental speed and flexibility, she displayed severe impairments on a variety of retrograde memory tasks. Furthermore, initial observations revealed inconsistencies between the patient’s recall of semantic knowledge on direct questioning and her ability to demonstrate the use of this knowledge on indirect tasks. To test this formally, we devised an indirect remote knowledge task to examine a possible dissociation between explicit and implicit memory. Two healthy subjects matched for age, gender, education, occupation, and estimated IQ were also tested. As predicted, the findings demonstrate implicit knowledge despite impaired explicit recall for the same material. (JINS, 1996, 2, 146–158.)