Ramaekers V T, Disselhorst-Klug C, Schneider J, Silny J, Forst J, Forst R, Kotlarek F, Rau G
Department of Paediatrics, University of Aachen, Germany.
Neuropediatrics. 1993 Jun;24(3):134-8. doi: 10.1055/s-2008-1071530.
Using a recently developed noninvasive EMG recording technique with multi-electrode arrays we investigated the pattern and distribution of motor unit action potentials (MUAP) following maximal voluntary contraction of the musculus abductor pollicis brevis. An additional parameter, i.e. muscular conduction velocity (CV) in single motor units, was calculated from the multi-electrode array EMG recordings. From 63 healthy children of various age the normal EMG pattern and CV were derived and compared to the EMG of diagnosed patients known to suffer from Duchenne muscular dystrophy and from spinal muscular atrophy. In normal individuals the muscular CV in neonates was lowest at 1-2 m/s and gradually reached a plateau of 2.9-4 m/s from the age of 4 years onwards. The EMG in 31 children with Duchenne muscular dystrophy showed an abnormal pattern with low amplitude action potentials. In 30 out of 31 patients a significantly lower muscular CV was found. In 10 children with spinal muscular atrophy the EMG showed action potentials of abnormally large amplitude and a reduced recruitment of firing motor units. The muscular CV remained within the normal range. Compared to classical needle EMG the application of this new noninvasive EMG technique in children is painless and offers an easy-to-handle diagnostic tool to differentiate between neuromuscular diseases of denervating or of myopathic origin.
我们使用一种最近开发的带有多电极阵列的无创肌电图记录技术,研究了拇短展肌最大自主收缩后运动单位动作电位(MUAP)的模式和分布。从多电极阵列肌电图记录中计算出另一个参数,即单个运动单位的肌肉传导速度(CV)。从63名不同年龄段的健康儿童中得出正常的肌电图模式和CV,并与已知患有杜氏肌营养不良症的确诊患者以及脊髓性肌萎缩症患者的肌电图进行比较。在正常个体中,新生儿的肌肉CV最低,为1-2米/秒,从4岁起逐渐达到2.9-4米/秒的平稳状态。31名杜氏肌营养不良症患儿的肌电图显示出异常模式,动作电位幅度较低。在31名患者中的30名中发现肌肉CV明显较低。10名脊髓性肌萎缩症患儿的肌电图显示动作电位幅度异常大,且发放运动单位的募集减少。肌肉CV仍在正常范围内。与传统的针电极肌电图相比,这种新的无创肌电图技术应用于儿童时无痛,且提供了一种易于操作的诊断工具,可用于区分神经源性或肌源性神经肌肉疾病。