EVALUATION OF NEUROMUSCULAR DISEASES

该方案的制定是为了表征新的 神经肌肉疾病，了解更多关于已建立的疾病， 评估当前的方法和技术并改进旧方法 以及开发新的。使用不同的电诊断 技术，我们进行了一些不同的研究： a. 肌病的表面肌电图（EMG）功率谱： 表面记录的肌肉，肌电图可能提供一个无痛的选择 神经肌肉疾病的针肌电图。 分析 活检证实的肌病患者的表面肌电图表明， 在低频和高频的功率可以增加灵敏度， 考试 B. Fabry病的自主神经研究：一组基础自主神经功能检查 可以在EMG实验室进行的测试，包括交感神经 皮肤反应、Valsalva比率和心率变异性 呼吸，已被用于研究这些患者的自主神经功能。 初步结果显示了一些异常，主要是心脏 副交感神经功能 C. 定量感觉研究的再现性：我们已经进行了 在一组健康志愿者中进行了一系列定量感觉研究， 定义不同的试验间和个体内变异性 测试方式。 本试验用于评价 周围神经病变

This protocol was developed as an effort to characterize new neuromuscular diseases, to learn more about established diseases, to assess current methodologies and technologies and to refine old methods as well as to develop new ones. Using different electrodiagnostic techniques, we have carried on a number of different studies: a. Power spectrum of surface electromyography (EMG) in myopathy: Surface recording of the muscle, EMG may provide a painless alternative to needle electromyography in neuromuscular diseases. Analysis of surface EMG in patients with biopsy-proven myopathy suggests that ratios of the power at low and high frequencies may increase the sensitivity of the test. b. Autonomic studies in Fabry's disease: A basic battery of autonomic testing that can be done in the EMG laboratory, including sympathetic skin responses, Valsalva ratio and heart rate variability with respiration, has been used to study autonomic function in these patients. Preliminary results have shown some abnormalities, primarily with cardiac parasympathetic function. c. Reproducibility of quantitative sensory studies: We have performed serial quantitative sensory studies in a group of healthy volunteers to define the intertrial and intraindividual variability for the different modalities of testing. This test is being used for the evaluation of peripheral neuropathy.