PHYSIOLOGICAL ANALYSIS OF INVOLUNTARY MOVEMENTS

本项目的目标是了解 不同不自主的潜在生理机制 运动障碍我们继续描述 Stiffman综合征患者脊髓抑制性反射回路 (SMS)，重点关注选定电池的串行性能， 在治疗试验中的三个点进行反射研究。我们研究了 肌肉诱发的体感诱发电位 III型戈谢病（GD 3）儿童的牵张反射， 年龄匹配的健康对照组。我们的结果表明，SEP在 GD3儿童即使没有证据表明 进行性肌阵挛癫痫我们正在评估 西德纳姆综合征患者的积极和消极运动 舞蹈病我们研究了兴奋后抑制（沉默 期-SP）的儿童与Sydenham舞蹈症经颅 磁刺激我们得出的结论是西德纳姆的孩子 舞蹈病有一个缩短的SP，这似乎是正常的儿童 恢复。我们正在做生理震颤分析，包括 家庭成员肌电图频谱分析和加速度测量 原发性震颤和各种神经病患者。 这些调查应有助于诊断震颤以及 阐明病理生理学。我们研究了 正常对照，并确定27%的研究人群 有证据表明，8 - 12赫兹组成部分的生理性震颤。我们 正在尝试开发生理学方法来评估 心因性震颤一个看起来很有希望的测试是测量 在一个肢体震颤的频谱，同时要求 患者以特定频率进行运动， 另一个极端。在心因性震颤中，"震颤"可能跟随 自主运动频率。进行屈肌反射研究 在正常志愿者中与不宁腿患者进行比较 综合征（RLS）和周期性肢体运动障碍（PLMD）， 检验RLS的PLMD是由增强的 脊髓的兴奋性正常受试者表现出戏剧性的 睡眠相关的屈肌反射反应抑制。而反观 在RLS/PLMD患者中， 睡眠时的屈肌反射反应睡眠相关抑制 患者的单突触H反射保留。EMG模式 的PLMs密切类似的屈肌反射反应。我们 研究表明脊髓机制的异常兴奋性 参与RLS/PLMD患者的屈肌反射反应。 我们的数据表明RLS中的PLM起源于脊髓， 会因为失去脊髓上抑制而变得容易

The goal of this project is to understand the underlying physiological mechanisms of different involuntary movement disorders. We have continued characterization of inhibitory spinal reflex circuits in patients with stiffman syndrome (SMS), with a focus on serial performance of a selected battery of reflex studies at three points in a treatment trial. We have studied the somatosensory evoked potentials (SEP) elicited by muscle stretch reflexes in children with type III Gaucher disease (GD3) and healthy age-matched controls. Our results suggest that the SEP in GD3 children are abnormal even when they have no evidence of progressive myoclonus epilepsy. We are evaluating the nature of the positive and negative movements in patients with Sydenham's chorea. We have studied the post-excitatory inhibition (silent periods - SP) in children with Sydenham chorea with transcranial magnetic stimulation. We concluded that children with Sydenham chorea have a shortened SP which appears to normalize as the child recuperates. We are doing physiological tremor analysis, including spectral analysis of EMG and accelerometry, in persons in families with essential tremor and in persons with various neuropathies. These investigations should help with diagnosis of tremor as well as elucidate the pathophysiology. We studied physiologic tremor in normal controls and determined that 27% of the study population had evidence of the 8-12 HZ component of physiologic tremor. We are trying to develop physiological methods for assessment of psychogenic tremor. One test that looks promising is measuring the frequency spectrum of the tremor in one extremity while asking the patient to make movements with specified frequencies with the another extremity. In psychogenic tremor, the "tremor" may follow the voluntary movement frequency. Flexor reflex studies were done in normal volunteers to compare with patients with Restless legs syndrome (RLS) and Periodic limb movement disorder (PLMD), to test the hypothesis that PLMD of RLS results from enhanced excitability of the spinal cord. Normal subjects showed a dramatic sleep-related inhibition of the flexor reflex response. In contrast, in patients with RLS/PLMD there was significant desinhibition of the flexor reflex response during sleep. Sleep-related inhibition of the monosynaptic H reflex in patients was preserved. The EMG pattern of PLMs closely resembled that of the flexor reflex response. Our study shows abnormal excitability of the spinal cord mechanisms involved in the flexor reflex response in patients with RLS/PLMD. Our data suggest that PLMs in RLS originate in the spinal cord and are facilitated by the loss of supraspinal inhibition.