线粒体肌病的病理分析

目的:探讨线粒体肌病患者的临床特点及其组织化学病理、电镜超微结构病理特征,提高对本病的确诊率。方法:对4例患者肌活检组织进行光镜和电镜超微病理观察。结果:3例患者肌活检组织MGT染色光镜检查发现破碎红纤维,4例患者肌活检组织电镜下发现线粒体堆积于肌原纤维间及肌膜下,形态变异,均可见晶状包涵体。结论:MGT染色发现破碎红纤维,电镜下线粒体堆积、形态异常,特别是线粒体内晶状包涵体,对本病的诊断有重要价值。

Pathological characteristic of mitochondrialmyopathies

Objective：To investigate the clinical features,histochemical characters and microstructure under electronic microscope of muscle in patients with mitochondrial myopathy,and to improve the diagnosis accuracy.Methods：The muscle biopsy specimens from 4 patients were examined with lightmicroscope and transmission electron microscope.Results：The histochemical staining light findings showed ragged red fibers（RRF）in 3 cases.All cases had diffusive proliferation of mitochondria and paracrystaline inclusion bodies between the myofibrils and beneath the sarcolemma and morphologic abnormalities.Conclusion：Histochemical MGT staining light shows RRF,accumulation of abnormal mitochondrials,especially the paracrystaline inclusion which plays an important role in diagnosis.