길입성 진전의 병태생리: 다증 증례 연구

Pathophysiology of orthostatic tremor: a multiple case study

Introduction : Orthostatic tremor develops in the legs while standing up with no weakness, pain or imbalance in the leg and the tremor is characteristically not observed when walking. However there have been some confusions about orthostatic tremor in several aspects. For the past ten years, we have observed 4 patients with orthostatic tremor. In each case tests were performed to investigate the following three important areas of inquiry about orthostatic tremor. Firstly, whether this disorder is an independent diagnostic entity or a variant of essential tremor. Secondly, whether the progress of this disorder is specifically related with standing posture. Lastly, the nature of the pathophysiologic mechanism behind the appearance of the tremor when standing after the lapse of a certain latent period and its disappearance upon the commencement of walking. Methods : Our 4 cases of orthostatic tremor were studied clinically, electrophysiologically, and pharmacologically. Electrophysiological tests included tremor spectrum test and electromyography. Results : We observed the presence of this tremor in several other tonic postures, as well as its absence, in a vertically lifted position from all our cases. Our cases registered a variable tremor frequency between 5 and 12 Hz according to the tremor spectrum test and EMG. Furthermore all our 4 cases demonstrated patterns of both synchronous EMG activity and alternating EMG activity at various times in homologous muscles of both legs. Orthostatic tremor was improved significantly with propranolol as well as clonazepam. Conclusions : From the results of our study we drew the following conclusions. It is probable that orthostatic tremor is simply a variant of essential tremor rather than being an independent diagnostic entity and that in most cases its development is specifically related with muscle contraction rather than merely with the act of standing. Furthermore we discovered a clue in the previously described neural control mechanism that the nuclear bag fibers in the muscle spindle have lag time of several seconds in their response to muscle strength and that their baseline does not reset fully in rapidly moving muscle. This neural control mechanism could offer sufficient explanation for the phenomena of tremor appearance when standing and disappearance when walking in orthostatic tremor.