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[伴有包涵体的肌炎:一种鲜为人知的特发性炎性肌病类型]

[Myositis with inclusion bodies: a little-known variety of idiopathic inflammatory myopathy].

作者信息

Casademont Pou J, Grau Junyent J M, Pou Serradell A, Urbano-Márquez A

出版信息

Med Clin (Barc). 1989 Jun 24;93(4):121-4.

PMID:2552235
Abstract

The authors report two male patients with inclusion body myositis (IBM) and review the features of this condition in the literature. This is an uncommon type of idiopathic myositis which involves males more often than females; it usually develops in elderly patients; its course is very slow; it is usually associated with distal weakness; neurophysiological studies show a mixed "myogenic" and "neurogenic" pattern; and it is usually unresponsive to corticosteroids. The diagnosis is basically made on the basis of the histological features, mainly consisting of vacuoles surrounded by a basophilic haze in histochemical stains of frozen tissue and, particularly, by the presence of characteristic microfilaments in ultrastructural studies.

摘要

作者报告了两名患有包涵体肌炎(IBM)的男性患者,并回顾了文献中该疾病的特征。这是一种罕见的特发性肌炎,男性比女性更常受累;通常发生于老年患者;病程非常缓慢;通常伴有远端肌无力;神经生理学研究显示为混合性“肌源性”和“神经源性”模式;并且通常对皮质类固醇无反应。诊断主要基于组织学特征,主要包括在冷冻组织的组织化学染色中出现嗜碱性晕圈包围的空泡,特别是在超微结构研究中存在特征性微丝。

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