PHYSIOLOGICAL ANALYSIS OF INVOLUNTARY MOVEMENTS

本项目的目标是了解 不同非自愿行为的潜在生理机制 运动障碍。我们一直在描述 Stadman综合征患者的抑制性脊髓反射回路 (短信)，重点是选定的电池的系列性能 在一项治疗试验中对三个点进行反射研究。我们已经研究过 肌肉诱发的体感诱发电位 III型高谢病(GD3)和 年龄匹配的健康对照组。我们的结果表明 GD3儿童即使没有证据也是不正常的 进行性肌阵挛癫痫。我们正在评估它的性质 Sydenham‘s病患者的正负向运动 舞蹈病。我们研究了兴奋性后抑制(Silent 经颅性Sydenham舞蹈症患儿的经期 磁刺激。我们得出结论，患有西德纳姆病的儿童 舞蹈症有一个缩短的SP，在儿童时期看起来是正常的 休养生息。我们正在进行生理性震颤分析，包括 家庭成员肌电和加速度计的频谱分析 有特发性震颤和有各种神经疾病的人。 这些检查应该有助于震颤的诊断以及 阐明其病理生理机制。我们研究了生理性震颤 并确定27%的研究人群 有生理性震颤的8-12赫兹分量的证据。我们 正在尝试开发生理学方法来评估 心因性震颤。一种看起来很有希望的测试是测量 询问患者一侧肢体震颤的频谱 病人以指定的频率用 另一个极端。在心因性震颤中，“震颤”可能随之而来。 自主运动的频率。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是增强的结果的假设 脊髓的兴奋性。正常受试者表现出戏剧性的 与睡眠相关的屈肌反射反应抑制。相比之下，在 RLS/PLMD患者存在明显的去抑制作用 睡眠时的屈肌反射反应。睡眠相关的抑制 保留了患者的单突触H反射。肌电模式 与屈肌反射反应非常相似。我们的 研究表明脊髓机制的异常兴奋性 参与了RLS/PLMD患者的屈肌反射反应。 我们的数据表明，RLS中的PLM起源于脊髓和 是由于失去了脊髓上的抑制而变得容易。

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.