伴有肌原纤维聚集体的非典型性肌病

Atypical myopathy with myofibrillar aggregates.

作者信息

Kinoshita M, Satoyoshi E, Suzuki Y

出版信息

Arch Neurol. 1975 Jun;32(6):417-20. doi: 10.1001/archneur.1975.00490480083010.

DOI:10.1001/archneur.1975.00490480083010

Abstract

An autopsy of a 23-year-old woman with progressive muscular weakness and wasting showed a unique muscle abnormality with segmental involvement of individual fibers by peculiar inclusions. Electron microscopically, these inclusions resembled cytoplasmic bodies, being formed of two concentric zones of filamentous materials. They seemed to arise from filaments of myofibrils that were fragmented and highly disorganized in affected areas.

摘要

对一名患有进行性肌肉无力和消瘦的23岁女性进行的尸检显示，其肌肉存在独特异常，个别纤维呈节段性受累，并伴有特殊包涵体。电子显微镜下，这些包涵体类似胞质体，由丝状物质的两个同心区域构成。它们似乎源自肌原纤维的细丝，在受影响区域这些细丝破碎且高度紊乱。