Impairment of Rapid Movement in Huntington's Disease

Patients with Huntington's disease (HD) and relatives at risk were examined with respect to their capacity to produce rapid voluntary motor activity. For this purpose, the fastest possible self-paced single isometric forefinger extensions and the fastest alternating forefinger movements were tested. In addition to these fastest voluntary performances, the time course of spontaneous hyperkinetic finger movements and the peak frequency of finger and hand tremor were analysed as a measure of the temporal characteristics of involuntary movements. Comparison of these parameters in HD patients and individuals at risk with age and sex-matched normal controls revealed a significant slowing of all types of contractions or movements in the majority (up to 95%) of the patients and in up to 40% of the relatives at risk. Reaction times were only slightly prolonged, and the abnormalities of the movement parameters showed no correlation with detailed psychometric data. Hence it is unlikely that the disturbance in the execution of rapid motor acts is due to dementia. Tremor was also slower than normal and the hyperkinesias were still slower than the fastest voluntary contractions. It appears from this study that slowness of motor performance is not only evident in Parkinson's disease but may represent a more general dysfunction in basal ganglia disease.