EVALUATION OF NEUROMUSCULAR DISEASES

神经肌肉疾病的评估

• 批准号: 3922578
• 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/3922578
• 关键词: action potentials; atrophy; axon; biopsy; carpal tunnel syndrome; chronic fatigue syndrome; degenerative motor system disease; electromyography; hereditary motor and sensory neuropathy; histopathology; human subject; neural transmission; neuromuscular disorder diagnosis; poliomyelitis; single cell analysis

Understanding of neuromuscular disease is founded on careful clinical observation, electrodiagnostic studies and pathology. This protocol has been carried out to learn more about established diseases, to characterize new disease, to assess current methodologies and technologies and technologies and to refine old methods and develop new ones. We have started a study of patients with Charcot-Marie-Tooth disease, with the object of discovering whether the affected peripheral nerve fibers are more severely damaged in their distal parts (near the muscles) or in their proximal parts (near the spinal cord). This information should help us to understand the nature of the underlying pathological defect. We also set up study of the refractory period of transmission in pathological nerve fibers at entrapment sites, using carpal tunnel syndrome patients as subjects. This enable us to assess the severity of entrapment by criteria different from those used in conventional conduction studies. We are continuing to study patients who have previously had poliomyelitis and who notice increasing weakness many years after the acute attack. While these patients show abnormalities of their surviving motor units, such changes are difficult to quantitate; nor is it clear whether or not the number of surviving motor units gradually decreases in such patients. We have set up a study of refractory period in the surviving motor axons of ALS patients as there is some evidence that this becomes increasingly abnormal in any one axon as it approaches the muscle. There are considerable technical problems in recording the refractionary period of single motor axons in man, which we are slowly surmounting.

对神经肌肉疾病的理解是建立在仔细 临床观察、电诊断研究和病理学。 本协议已进行，以了解更多关于建立 疾病，描述新疾病，评估当前 方法和技术，并改进旧的 方法，开发新的。 我们已经开始对腓骨肌萎缩症患者进行研究 疾病，目的是发现受影响的 周围神经纤维在其远端受损更严重， 或在其近端部分（靠近 脊髓）。 这些信息应该有助于我们了解 潜在病理缺陷的性质。 我们还设立了研究 病理性神经传导不应期 卡压部位的纤维，使用腕管综合征患者 作为实验对象 这使我们能够评估诱捕的严重程度 用不同于常规传导中所用的标准 问题研究 我们正在继续研究那些以前有过 小儿麻痹症和谁注意到越来越弱多年后， 急性发作。 虽然这些病人的大脑出现了异常， 存活的运动单位，这种变化很难量化; 也不清楚幸存的运动单位数量 在这些患者中逐渐减少。 我们已经建立了一个研究不应期在幸存的运动 ALS患者的轴突，因为有证据表明， 在任何一个轴突中，当它接近肌肉时，都会变得越来越不正常。 记录过程中存在相当大的技术问题 在人的单运动轴突的折射期，我们是 慢慢超越。