MORPHOLOGICAL-CHANGES OF MOTOR UNITS IN DUCHENNES MUSCULAR-DYSTROPHY

MORPHOLOGICAL-CHANGES OF MOTOR UNITS IN DUCHENNES MUSCULAR-DYSTROPHY
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DOI:
10.1001/archneur.1977.00500190030004
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发表时间:
1977-01-01
影响因子:
--
通讯作者:
TELERMANTOPPET, N
TELERMANTOPPET, N
中科院分区:
其他
文献类型:
--
作者:
COERS, C;TELERMANTOPPET, N

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IM。对19例Duchenne‘S肌营养不良症患儿的肌肉活检标本进行了运动神经支配分析,并对其中9例标本进行了纤维类型分析。运动神经支配的主要改变是运动终板纵向移位达10 mm,并存在大量无用的轴突,在结缔组织内自由终止。末梢下轴突侧支未见增加,末梢神经支配比率正常。纤维类型的特征是纤维直径的随机变化,I型纤维略占优势,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神经再支配的可能性,这与Duchenne‘’S肌营养不良症的晚成分电位有关。
The i.m. motor innervation in 19 muscle biopsy specimens from boys with Duchenne''s muscular dystrophy and the fiber-type pattern was analyzed in 9 biopsy specimens. The main change in motor innervation was a longitudinal displacement of motor endplates that reached 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 I fibers, and an impaired type differentiation in many fibers. The morphological data did not support the possibility of denervation and collateral reinnervation that was related to the late component potentials found in Duchenne''s muscular dystrophy.