PHYSIOLOGICAL ANALYSIS OF INVOLUNTARY MOVEMENTS

这个项目的目标是了解潜在的生理 不同的不自主运动障碍的机制。 我们有 抑制性脊髓反射回路的持续表征 stiffman综合征（SMS）的患者，重点是推定的 GABA能通路。 在一个额外的实验中， 8例SMS患者，均具有抗GABA能抗体反应性 神经元 正如前几年研究的患者中发现的那样， 由肌腱振动引起的H反射似乎是最一致的 异常 这些患者也参加了一项治疗， 协议，具有所选反射电池的串行性能 在治疗过程中的三个方面进行研究。 我们继续招募 临床症状提示获得性惊吓的患者 疾病，寻找可能的自身免疫攻击的候选人， 甘氨酸途径。 四名患者接受了生理学研究， 表征脊髓和脑干反射通路的异常。 我们 继续描述高雪氏病类型中的肌阵挛 3;体感诱发电位振幅与分期相关 疾病。 我们正在评估积极的性质， 西德纳姆舞蹈病患者的负面运动 我们正在做 生理震颤分析，包括EMG的频谱分析， 加速度计，在人的家庭与特发性震颤和 患有各种神经病的人。 这些调查应该有助于 诊断震颤以及阐明病理生理学。 我们已经开始对睡眠中的周期性运动进行更深入的研究， 不宁腿综合征患者的情况

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 presumptive GABAergic pathways. A battery of reflexes were studied in an additional 8 SMS patients, all of whom had antibodies reactive against GABAergic neurons. As found in patients studied in previous years, inhibition of H-reflexes by tendon vibration seems to be the most consistent abnormality. These patients have also been enrolled in a treatment protocol, with serial performance of a selected battery of reflex studies at three points in their treatment. We continue to recruit patients with clinical symptoms suggestive of an acquired startle disease, looking for candidates with possible autoimmune attack on glycinergic pathways. Four patients underwent physiological studies to characterize abnormalities in spinal and brainstem reflex pathways. We have continued to characterize the myoclonus in Gaucher's disease type 3; somatosensory-evoked potential amplitude correlates with the stage of the disease. We are evaluating the nature of the positive and negative movements in patients with Sydenham's chorea. 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 the diagnosis of tremor as well as elucidate the pathophysiology. We have begun more intensive studies of periodic movements in sleep seen in patients with restless legs syndrome.