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眼咽型肌营养不良症:与原发性肌病不同的肌肉超微结构

Oculopharyngeal dystrophy: ultrastructure of muscles distinct from the primary myopathy.

作者信息

Kozachek J W, Wilson F J

出版信息

Acta Neuropathol. 1982;57(1):7-12. doi: 10.1007/BF00688872.

Abstract

The pectoral and psoas muscles from a 72-year-old man afflicted with oculopharyngeal muscular dystrophy were processed at autopsy for electron microscopy. The ultrastructural analyses of the pectoral muscle showed myofibrils which exhibited Z line streaming and a general breakdown in the organization of the sarcomere. In addition, some of the myofibrils displayed sites of degeneration at the center of the A band. The changes in the psoas muscles which are distant from the primary myopathic loci of oculopharyngeal muscular dystrophy were more extensive than that seen in the pectoral muscle. Control tissues showed intact myofibrils ad little postmortem alteration.

摘要

对一名患有眼咽型肌营养不良症的72岁男性的胸肌和腰大肌进行尸检,用于电子显微镜检查。胸肌的超微结构分析显示,肌原纤维出现Z线流,肌节组织普遍破坏。此外,一些肌原纤维在A带中央显示出变性部位。腰大肌远离眼咽型肌营养不良症原发性肌病病灶的变化比胸肌更为广泛。对照组织显示肌原纤维完整,死后改变很小。

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