Abstract
This paper compares leg muscle electromyogram (EMG) responses to sudden toe-up tilts of a moveable platform in patients with Huntington's disease (HD), clinically normal offspring at risk of developing HD (HD risks) and healthy controls. The EMG pattern in standing subjects and patients consisted of short- and middle-latency responses (SL and ML) in the stretched triceps surae muscles and long-latency responses (LL) in the shortened tibialis anterior muscles. The SL response could be further divided into two distinct subcomponents termed SL1 and SL2. An ML response was identified in only 50% of normal subjects and patients. HD patients differed from normal subjects by showing delayed onset latencies and prolonged durations for the LL response, and smaller amplitudes for the ML response. The subjects at risk also showed diminished ML amplitudes and prolonged LL durations, but normal LL onset latencies. In the sitting condition, the EMG responses of the HD patients and of the HD risks did not differ from those of controls: in all groups SL1 was reduced and delayed, SL2 slightly enhanced, while ML and LL were absent. Because both afferent and efferent conduction times are normal in HD, the delayed LL onset reflects abnormal supraspinal organisation of postural control in HD, and indicates that basal ganglia may have a modulatory effect on the LL responses. The normal EMG responses in the sitting patients suggest appropriate regulation of these responses according to postural set in HD.