AbstractThe clinical, electromyographic, and histologic characteristics of a 17‐year‐old girl with reducing body myopathy are described. She is, to our knowledge, the oldest reported case and the only patient described with severe mitral valve prolapse and scoliosis. Electromyography demonstrated spontaneous positive sharp waves and fibrillation potentials with many lowamplitude, short, polyphasic motor unit potentials. The right deltoid muscle was chaacterized by focal areas with large fibers associated with increased endomysial connective tissue and “split” fibers. Purple‐gray sarcoplasmic masses stained with trichrome were PAS‐negative, appeared as “empty” spaces with both ATPase and NADH‐TR, and stained darkly with menadione NBT. The features described expand the clinical presentation of this myopathy, and may lead to a better understandin
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机译：摘要介绍17岁复位体肌病女孩的临床、肌电图和组织学特征。据我们所知，她是报告年龄最大的病例，也是唯一被描述患有严重二尖瓣脱垂和脊柱侧弯的患者。肌电图显示自发性正尖波和纤维性颤动电位，伴有许多低振幅、短、多相运动单位电位。右三角肌被具有大纤维的局灶区域追逐，这些纤维与肌内膜结缔组织和“分裂”纤维增加有关。用三色染色的紫灰色肌浆肿块为 PAS 阴性，在 ATP 酶和 NADH-TR 中表现为“空”空间，并用甲萘醌 NBT 染色为深色。所描述的特征扩展了这种肌病的临床表现，并可能导致更好的理解
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