Increased false alarms in a subset of persons at-risk for Huntington's disease

The accuracy and decision criterion of 51 persons at-risk (AR) for Huntington's disease (HD) along with 36 age-matched, healthy controls were evaluated using a word recognition memory test. Analyses revealed that the AR group was less accurate than the controls at recognizing a previously learned word list. Within the AR group, 13 AR persons were identified who committed significantly more false alarms than the remaining AR and control people. The 13 AR persons also showed evidence of lower cerebral metabolic ratios in frontal, caudate, and insula regions, as evaluated by positron emission tomography using I8F-Fluoro-deoxyglucose, and were considered to be at high risk for HD. One of these 13 AR persons has become symptomatic since testing, confirming the high risk status. These results suggest that certain tests of cognition and decision-making together with measures of regional brain metabolism might show the subtle cognitive changes taking place in potential HD gene carriers years before the clinical symptoms are observed.