Coërs C, Telerman-Toppet N
Arch Neurol. 1977 Jul;34(7):396-402. doi: 10.1001/archneur.1977.00500190030004.
We studied the intramuscular motor innervation in 19 muscle biopsy specimens from boys with Duchenne's muscular dystrophy and the fiber-type pattern was analyzed in nine biopsy specimens. The main change in motor innervation was a longitudinal displacement of motor end plates that could reach 10 mm and the presence of numerous unemployed axons ending freely within connective tissue. There was no increased collateral ramification of subterminal axons and the terminal innervation ratio was normal. The fiber-type pattern was characterized by a random variation of fiber diameters, a slight predominance of type 1 fibers, and an impared type differentiation in many fibers. The morphological data do not support the possibility of denervation and collateral reinnervation that could be related to the late component potentials found in Duchenne's muscular dystrophy.
我们研究了19例杜兴氏肌营养不良男孩肌肉活检标本中的肌内运动神经支配情况，并对9例活检标本的纤维类型模式进行了分析。运动神经支配的主要变化是运动终板的纵向移位，可达10毫米，以及大量无髓轴突在结缔组织内自由终止。终末下轴突的侧支分支没有增加，终末神经支配率正常。纤维类型模式的特征是纤维直径随机变化，1型纤维略有优势，许多纤维的类型分化受损。形态学数据不支持去神经和侧支再支配的可能性，而去神经和侧支再支配可能与杜兴氏肌营养不良中发现的晚期成分电位有关。
