神经原性肌萎缩的肌肉组织病理定量研究

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。

Quantitative Histopathologic Study of Biopsied Muscle Specimens in Patients with Neurogenic Amyotrophy

In 32 cases of neurogenic amyotrophy and 15 normal subjects the quantitative comparison between different types and durations of these patients were made for the alterations of muscle fibers which included angular fibers, the percentage of muscle fiber types, muscle fiber type grouping, and the variability coefficients of muscle fiber size and form factor. All patients were divided into 4 groups including 8 cases of ALS, 9 cases of PMA, 8 cases of SMA type I and 7 cases of SMA type IN. The results suggested that the higher percentage of type I fiber and degree of fiber type grouping were characteristic in the SMA type I and SMA type IV groups. With the duration prolonged, the percentage of angular fibers became lower and the degree of fiber type grouping became higher. The variation degree of the muscle fiber size and form factor were non-specific.