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全身性或多灶性反射性交感神经营养不良性强直性肌张力障碍患者的神经生理学方面

Neurophysiologic aspects of patients with generalized or multifocal tonic dystonia of reflex sympathetic dystrophy.

作者信息

van de Beek Willem-Johan T, Vein Alla, Hilgevoord Anthony A J, van Dijk J Gert, van Hilten Bob J

机构信息

Department of Neurology, Leiden University Medical Center, 2300 RC Leiden, The Netherlands.

出版信息

J Clin Neurophysiol. 2002 Jan;19(1):77-83. doi: 10.1097/00004691-200201000-00011.

Abstract

Reflex sympathetic dystrophy (RSD) is a syndrome dominated by sensory, autonomic, and motor features of the extremities. In this study, 10 severely affected RSD patients who progressed to multifocal or generalized tonic dystonia underwent H-reflex evaluation, needle electromyography (EMG), polysomnography, somatosensory evoked potentials, and transcranial magnetic stimulation. H-reflex evaluation revealed an impaired vibratory inhibition of the H-reflex and a higher facilitation peak in the recovery curve between 200 to 350 msec. Needle EMG revealed an impaired reciprocal inhibition, and many patients were unable to alter the amount of muscle activity voluntarily. Evaluations of the stretch reflex showed a markedly decreased threshold and abnormal responses to tonic and phasic changes. Polysomnography performed in five patients revealed no abnormal EMG activity during nonrapid eye movement and rapid eye movement sleep, but EEG arousal phenomena provoked abnormally high and brief bursts of surface EMG activity in all registered muscle groups. Somatosensory evoked potentials and transcranial magnetic stimulation were normal. Taken together, the findings in these patients with tonic dystonia of RSD are in accordance with an impairment of inhibitory interneuronal circuits at the level of the brainstem or spinal cord.

摘要

反射性交感神经营养不良(RSD)是一种以四肢感觉、自主神经和运动特征为主的综合征。在本研究中,对10例进展为多灶性或全身性强直性肌张力障碍的重度RSD患者进行了H反射评估、针极肌电图(EMG)、多导睡眠图、体感诱发电位和经颅磁刺激检查。H反射评估显示,H反射的振动抑制受损,在200至350毫秒之间的恢复曲线中促进峰值更高。针极肌电图显示交互抑制受损,许多患者无法自主改变肌肉活动量。牵张反射评估显示阈值明显降低,对强直性和相位性变化的反应异常。对5例患者进行的多导睡眠图检查显示,在非快速眼动和快速眼动睡眠期间没有异常肌电图活动,但脑电图觉醒现象在所有记录的肌肉群中引发了异常高且短暂的表面肌电图活动爆发。体感诱发电位和经颅磁刺激均正常。综上所述,这些RSD强直性肌张力障碍患者的研究结果与脑干或脊髓水平的抑制性中间神经元回路受损一致。

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