Heuss D, Claus D, Neundörfer B
Department of Neurology, Friedrich Alexander University of Erlangen-Nürnberg, Germany.
Clin Neuropathol. 1996 Jul-Aug;15(4):200-8.
In the presented study we attempted to demonstrate a correlation between muscle regeneration and fibrillations in electromyography in dystrophic myopathies. Especially in Emery-Dreifuss muscular dystrophy there is much abnormal spontaneous activity, and NCAM (neural cell adhesion molecule)and cytoskeletal protein vimentin expressing myocytes are predominantly seen. Therefore, definitely regenerating fibers are identified apart from only a few remnants of previous necrosis. Moreover, in the other biopsies of dystrophic myopathies there are also scattered and clustered NCAM and vimentin expressing regenerating myofibers. Here, regressive fiber changes, like necrosis, are more prominent. Furthermore, most regenerating fibers show pseudo-cholinesterase activity indicating innervation. Interestingly, motor end-plate changes in regeneration and in disuse atrophy are very similar. They predominantly consist of terminal sprouting and pseudo-cholinesterase spread. However, in disuse atrophy there is no abnormal spontaneous spread in electromyography. Therefore, in regenerating muscle not innervation, but regeneration itself is likely to be the cause of fibrillations. In conclusion, a correlation is evident between regenerating muscle and fibrillations in electromyography.
在本研究中，我们试图证明营养不良性肌病中肌肉再生与肌电图中纤维颤动之间的相关性。特别是在Emery-Dreifuss肌营养不良症中，存在大量异常的自发活动，并且主要可见表达神经细胞粘附分子（NCAM）和细胞骨架蛋白波形蛋白的肌细胞。因此，除了先前坏死的少数残余物外，还可识别出明确正在再生的纤维。此外，在营养不良性肌病的其他活检组织中，也存在散在和成簇的表达NCAM和波形蛋白的再生肌纤维。在这里，诸如坏死等退行性纤维变化更为突出。此外，大多数再生纤维显示假性胆碱酯酶活性，表明有神经支配。有趣的是，再生和废用性萎缩中的运动终板变化非常相似。它们主要由终末芽生和假性胆碱酯酶扩散组成。然而，在废用性萎缩中，肌电图没有异常的自发扩散。因此，在再生肌肉中，肌电图中的纤维颤动的原因可能不是神经支配，而是再生本身。总之，再生肌肉与肌电图中的纤维颤动之间存在明显的相关性。
