PHYSIOLOGICAL ANALYSIS OF INVOLUNTARY MOVEMENTS

不自主运动的生理分析

• 批准号: 5203921
• 负责人: M HALLETT
• 金额: --
• 依托单位: NATIONAL INSTITUTE OF NEUROLOGICAL DISORDERS AND STROKE
• 依托单位国家: 美国
• 资助国家: 美国
• 起止时间: 至
• 项目状态: 未结题
• 来源: https://reporter.nih.gov/project-details/5203921
• 关键词: Gaucher's disease; abnormal involuntary movement; botulinum toxins; disease /disorder classification; evoked potentials; gamma aminobutyrate; glycine receptors; human subject; myoclonus; neuromuscular disorder diagnosis; neurophysiology; partial seizure; pathology; positron emission tomography; sleep; spinal cord

Involuntary movements have been classified primarily on descriptive basis. The use of physiological criteria for the classification and study of patients with movement disorders has lead to new insights onto pathophysiology and treatment of these disorders. We continue our efforts to characterize the physiological mechanisms responsible for positive and negative myoclonus. We have expanded the physiological features of the opsoclonus-myoclonus syndrome. The myoclonus in patients with Gaucher's disease has been studied physiologically. We have studied physiologically patients with epilepsia partialis continua (EPC) and these findings have been correlated with PET studies these same patients. Using EEG dsynchronization to voluntary movements and sensory evoked potentials, we have identified differences in the patterns of cortical activation in patients with hand dystonia. These findings support findings from parallel PET and transcranial magnetic stimulation studies. In some patients with tic disorders, we have found patterns of cortical activation similar to those accompanying normal voluntary movements. We have found abnormalities of spinal cord mechanisms for vibratory inhibition of the H-reflex in patients with stiff-man syndrome (SMS) which implicates a dysfunction of GABAergic spinal cord mechanisms. Patients with hereditary hyperekplexia, a genetic disorder affecting glycine receptors, show abnormalities in reflex pathways thought to be mediated by glycinergic Ia interneurons. Oligosynaptic spinal cord reflexes including vibratory inhibition, flexor reflexes and cutaneous silent period in patients with severe dystonia before and after intrathecal baclofen are being studied. The physiological properties of tremor to various inertial loads and during writing were studied in patients with writing tremor before treatment with botulinum toxin.

非自愿运动主要根据描述性分类， 基础 使用生理学标准进行分类， 对运动障碍患者的研究导致了对以下问题的新见解： 病理生理学和这些疾病的治疗。 我们继续 努力表征负责的生理机制 阳性和阴性肌阵挛。我们已经扩大了生理 眼阵挛-肌阵挛综合征的特征。 患者的肌阵挛 已经从生理学上研究过了。 我们研究了 部分性持续性癫痫（epilepsia partialis continua，EPC）患者， 这些发现与这些患者的PET研究相关。 应用脑电双同步技术研究随意运动和感觉诱发 电位，我们已经确定了不同的模式，皮层 手肌张力障碍患者的激活。 这些发现支持 并行PET和经颅磁刺激研究的结果。 在一些抽动障碍患者中，我们发现了皮质神经元的模式， 类似于伴随正常自愿运动的那些激活。 我们已经发现脊髓的振动机制异常 僵硬人综合征（SMS）患者的H反射抑制 这暗示了GABA能脊髓机制的功能障碍。 患有遗传性过度兴奋的患者， 甘氨酸受体，显示异常的反射途径被认为是 由甘氨酸能Ia中间神经元介导。 少突触脊髓 反射，包括振动抑制、屈肌反射和皮肤反射 重度肌张力障碍患者治疗前后的静默期 鞘内巴氯芬正在研究中。 的生理特性 震颤的各种惯性负载和在写作过程中进行了研究， 肉毒杆菌毒素治疗前书写震颤的患者。