Desmedt J E, Borenstein S
Arch Neurol. 1976 Sep;33(9):642-50. doi: 10.1001/archneur.1976.00500090048010.
Eight patients with Duchenne muscular dystrophy (DMD) and seven normal children of similar age were studied with a new electromyographic method for coherent displays of potentials. Five hundred motor unit potentials (MUPs) were analyzed in 20 proximal and distal muscles representing a wide spectrum of dystrophy. The progressive MUP disintegration by dropping out of muscle fibers was documented, as well as a high incidence of spontaneous fibrillation. A total of 386 late component (LC) potentials followed the 500 MUPs at consistent latencies. No LC occurred in normal children. The LCs result from motor axon sprouts innervating muscle fibers that are newly formed either by segmentation of existing muscle fibers (focal neucrosis and membrane repair) or by muscle regeneration. Axons in DMD can thus collaterally innervate additional muscle fibers. These processes must delay the onset and progression of clinical weakness in DMD patients.
采用一种用于相干显示电位的新型肌电图方法，对8例杜氏肌营养不良症（DMD）患者和7名年龄相仿的正常儿童进行了研究。在代表广泛肌营养不良范围的20块近端和远端肌肉中分析了500个运动单位电位（MUP）。记录了因肌纤维脱失导致的MUP逐渐解体，以及高发生率的自发纤颤。在500个MUP之后，共有386个迟发成分（LC）电位在一致的潜伏期出现。正常儿童未出现LC。LC是由运动轴突发芽支配新形成的肌纤维所致，这些新形成的肌纤维要么是由现有肌纤维的分割（局灶性坏死和膜修复）形成，要么是由肌肉再生形成。因此，DMD中的轴突可通过侧支支配额外的肌纤维。这些过程必定会延迟DMD患者临床肌无力的发作和进展。
