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本文对32例神经原性肌萎缩病人的角纤维、同型肌群化、纤维类型比例、肌纤维大小及形状因子变异程度等肌活检病理改变进行了研究。结果提示:Ⅰ型纤维比例和同型肌群化程度较高是少年型脊髓性肌萎缩(SMA Ⅲ)和成人型脊髓性肌萎缩(SMA Ⅳ)的特征性改变,与肌萎缩侧索硬化(ALS)、进行性肌萎缩(PMA)相比,SMA病人有较低的失神经支配和较高的神经再支配能力;角纤维的比例与病程呈负相关,同型肌群化程度与病程呈正相关。
In this paper, 32 cases of neurogenic muscle atrophy patients with angular fibrosis, the same type of muscle, fiber type ratio, muscle fiber size and shape factor variation degree of muscle biopsy and other pathological changes were studied. The results suggest that the higher proportion of type Ⅰ fibers and the higher degree of homozygosity are characteristic features of juvenile spinal muscular atrophy (SMA Ⅲ) and adult type IV spinal cord atrophy (SMA Ⅳ), which are associated with amyotrophic lateral sclerosis (ALS ) And progressive muscular atrophy (PMA). SMA patients had lower denervation and higher neurological distributive ability. The proportion of angle fibers was negatively correlated with the course of disease. The degree of homozygote was positively correlated with the course of disease.