16例线粒体肌病和线粒体脑肌病电镜观察分析  

Electron microscopy findings in a series of 16 cases of mitochondrial myopathy and encephalomyopathy

作　　者：许蕾[1] 刘振红[2] 张彤[1] 刘威[1] 薛平[3] 

机构地区：[1]河北医科大学第二医院神经内科,石家庄050000 [2]河北医科大学第二医院精神科,石家庄050000 [3]河北医科大学第二医院外科实验室,石家庄050000

出　　处：《脑与神经疾病杂志》2009年第4期253-256,共4页Journal of Brain and Nervous Diseases

基　　金：河北省卫生厅医学科学研究重点课题项目(08086)

摘　　要：目的探讨线粒体肌病与脑肌病患者肌肉的超微结构特征,分析该病的病因和可能的发病机制。方法对16例线粒体肌病与脑肌病患者的肌活检组织进行光镜和电镜超微病理观察。结果电镜观察16例,在肌原纤维间和肌膜下可见弥漫的线粒体数目增多,13例表现为形态异常,可见巨大线粒体,嵴结构不清,排列紊乱,呈同心圆样,均可见线粒体内类结晶状包涵体,有的同时伴有糖原颗粒的异常增多、脂滴沉积及溶酶体异常,有的线粒体只能靠双层膜结构及残存的嵴被识别。3例仅线粒体数量增多,未见其他异常。结论电镜观察肌膜下和肌原纤维间线粒体异常增多且形态异常,特别是线粒体内类结晶状包涵体,对本病的诊断有重要价值。Objective To investigate the ultrastructural features of muscle in patients with mitoehondrial myopathy and encephalomyopathy and the possible etiological factors and pathogenesis . Methods The muscle biopsy specimens taken from 16 patients were examined with light microscop and transmission electron microscope . Results There was a diffuse proliferation of mitochondria between the myofibrils and beneath the sarcolemma. In 13 cases , the uhrastructural findings showed morphologic abnormalities such as giant mitochondria, round mitochondria with concentric cristae and electron-dense substances, and paracrystalline inclusions in the mitochondria. In some cases there were associated with accumulations of glycogen granules, lipid droplets and phagolysosome. In some mitochondrias, the contour of the cristae was so disrupted that they could only be identified by their characteristic double membranes and a few residual normal eristaes. In 3 cases the increase in mitochondrial number is the only alteration. Conclusion Electron microscopy can identify structurally abnormal mitochondrias. The accumulation of abnormal mitochondrials between the myofibrils and beneath sarcolemal, especially the paracrystaline inclusion bodies,play important roles in making a diagnosis of this disease.

关 键 词：线粒体肌病 线粒体脑肌病 超微结构 类结晶状包涵体 

分 类 号：R746[医药卫生—神经病学与精神病学]