线粒体肌病的超微结构观察

线粒体肌病为一少见疾病。本文报告７例，其临床表现可分为两种类型；以四肢骨骼受受侵为主６例；以眼外肌受侵为主１例。７例均进行肌细胞活检，电镜观察显示肌膜下和肌原纤维内线粒体数量明显增多，形态也有改变，线粒体嵴异常，基质内含有晶格状或类晶格状包含体；肌纤维内糖原颗粒及脂滴增加。本文还结合文献对线粒体肌病的诊断标准及病因和发病机制进行了探讨。

Ultrastructural observations in mitochondrial myopathy

Seven cases of mitochondrial myopathy are reported. Their clinical manifestations can be separated into two groups:syndromes showing predominantly skeletal muscles affected(6 cases); syndromes presenting with chronic progressive external ophthalmoplegia.Muscle biopsies were performed in all the cases.Electron microscope shows that the myofibers possess an excessive number of mitochondria,mainly located in the subsarcolemmal region and between myofibrils.Many mitochondria are enlarged and present structural abnormalities such as abnormal cristae or contained paracrystalline or crystalline inclusions of various shapes.Affected fibers also contain increased number of glycogen granules and lipid droplets.