Kamieniecka Z, Schmalbruch H
Muscle Nerve. 1978 Sep-Oct;1(5):413-5. doi: 10.1002/mus.880010513.
Of 185 patients with myopathy, 22 showed abnormal muscle mitochondria. In 12 of the 22 patients, all of whom had ocular myopathy or the ophthalmoplegia-plus syndrome, muscle biopsies contained 5%-25% "ragged red" fibers. In 4 patients with a facioscapulohumeral distribution of weakness, ragged red fibers were less numerous (3%-8%). In both groups, routine histology showed almost normal muscle. The remaining 6 patients were clinically heterogeneous, all without ptosis or ophthalmoplegia. The biopsies of three of these patients showed severely affected muscle. It is possible that mitochondrial changes in these muscles were nonspecific. Electromyography indicated or suggested a myogenic lesion in 21 of the 22 patients; in 10, the serum creatine kinase was increased.
在185例肌病患者中，22例显示肌肉线粒体异常。在这22例患者中的12例，他们均患有眼肌病或眼肌麻痹加综合征，肌肉活检发现有5% - 25%的“破碎红”纤维。在4例表现为面肩肱型肌无力分布的患者中，破碎红纤维数量较少（3% - 8%）。两组患者的常规组织学检查显示肌肉几乎正常。其余6例患者临床表现各异，均无眼睑下垂或眼肌麻痹。其中3例患者的活检显示肌肉严重受累。这些肌肉中的线粒体变化可能是非特异性的。22例患者中有21例的肌电图显示或提示有肌源性损害；10例患者血清肌酸激酶升高。
