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中央核性肌病一例罕见病例的细胞病理学。光镜和电镜研究。

Cytopathology of an unusual case of centronuclear myopathy. Light- and electron-microscopic investigations.

作者信息

Hülsmann N, Gullotta F, Okur H

出版信息

J Neurol Sci. 1981 Jun;50(3):311-33. doi: 10.1016/0022-510x(81)90146-5.

Abstract

The results of enzyme-histochemical and electron-microscopic investigations of a patient with centronuclear myopathy combined with targets, cores and prevalence of type-I fibers are presented. The patient had suffered from perinatal hypoxic brain damage, causing enlargement of the ventricular system, slight frontal atrophy and right-sided hemiparesis. Morphologic investigation of muscle fibers demonstrated dynamic activity of an autophagic system represented by a distinct increase of Golgi components adhering to the nuclear surface. By analysing the lytic events ad their sequences, a postnatal secondary migration of subsarcolemmal nuclei to the central regions of muscle fibers can be postulated.

摘要

本文呈现了对一名患有中央核性肌病患者的酶组织化学和电子显微镜检查结果,该患者伴有靶形、核心以及I型纤维优势。患者曾遭受围产期缺氧性脑损伤,导致脑室系统扩大、轻度额叶萎缩和右侧偏瘫。对肌纤维的形态学研究显示,自噬系统呈现动态活动,表现为附着于核表面的高尔基体成分明显增加。通过分析溶解事件及其顺序,可以推测出生后肌膜下核向肌纤维中央区域的继发性迁移。

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