EVALUATION OF NEUROMUSCULAR DISEASES

这项协议的开发是为了刻画新的 神经肌肉疾病，为了更多地了解已有的疾病， 评估当前的方法和技术并改进旧的方法 以及开发新的。使用不同的电子诊断 技术方面，我们进行了许多不同的研究： A.肌病患者的表面肌电信号功率谱： 表面记录肌肉，肌电可提供一种无痛替代 目的：探讨神经肌肉疾病的针状肌电图术。分析 经活检证实的肌病患者的表面肌电信号提示 在低频和高频下的功率的变化可以增加对 这个测试。 B.Fabry病的自主神经研究：自主神经的基本单元 可以在肌电实验室进行的测试，包括交感神经 皮肤反应、Valsalva比率和心率变异性 呼吸，已经被用来研究这些患者的自主神经功能。 初步结果显示有一些异常，主要是心脏 副交感神经功能。 C.定量感官研究的重现性：我们已经进行了 在一组健康志愿者中进行的一系列定量感觉研究 定义不同药物的试验间和个体内可变性 测试的方式。这项测试被用来评估 周围神经病。

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.