Stimulus-sensitive myoclonus in akinetic-rigid syndromes

Abstract

The cutaneous reflexes of upper limb muscles were studied in five patients with Parkinson's disease and 10 patients with stimulus-sensitive myoclonus associated with akinetic-rigid syndromes. The middle finger was stimulated with ring electrodes and rectified electromyographs were averaged from seven upper limb muscles and orbicularis oculi. Responses from subjects with Parkinson's disease without stimulus-sensitive myoclonus were similar to those of normal subjects. The responses from patients with stimulus-sensitive myoclonus associated with Parkinson's disease or multiple system atrophy had the normal pattern except that a long latency facilitation, which is present in normal subjects and known as E2, was greatly exaggerated. Patients with stimulus-sensitive myoclonus associated with cortical-basal ganglionic degeneration had a completely different pattern of responses. There was synchronous activation of all recorded upper limb muscles with latencies substantially shorter than those of the long latency facilitation (E2) in normal subjects. Cutaneous reflex testing may therefore be useful in the differentiation of akinetic-rigid syndromes.