| 肌原纤维肌病的临床与病理学特征(附1例报告) |
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| 引用本文： | 吴士文,马维娅,沈定国.肌原纤维肌病的临床与病理学特征(附1例报告)[J].临床神经病学杂志,2009,22(2). |
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| 作者姓名： | 吴士文 马维娅 沈定国 |
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| 作者单位： | 1. 武警总医院神经内科,北京,100039
2. 西安高新医院神经内科 |
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| 摘 要： | 目的研究肌原纤维肌病的临床与病理学特征。方法回顾性分析1例肌原纤维肌病患者的临床和病理学资料。结果本例患者中年起病,表现为进行性四肢近端肌无力,有轻度肌萎缩。肌电图示:双下肢神经源性损害。病理检查示:大量肌纤维内含有胞浆体,偶见镶边空泡,含有胞浆体的肌纤维内,出现肌原纤维网结构紊乱及结蛋白异常沉积。结论肌原纤维肌病在临床表现上缺乏特异性,肌纤维内肌原纤维网结构紊乱,出现大量胞浆体及结蛋白异常沉积是其特征性病理改变。
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| 关 键 词： | 肌原纤维肌病 胞浆体 结蛋白 |
Clinical and pathological features of myofibrillar myopathy (report of 1 case) |
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| WU Shi-wen,MA Wei-ya,SHEN Ding-guo.Clinical and pathological features of myofibrillar myopathy (report of 1 case)[J].Journal of Clinical Neurology,2009,22(2). |
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| Authors: | WU Shi-wen MA Wei-ya SHEN Ding-guo |
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| Institution: | WU Shi-wen,MA Wei-ya,SHEN Ding-guo.Department of Neurology,the General Hospital of Chinese Armed Police Force,Beijing 100039,China |
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| Abstract: | Objective To study the clinical and pathological features of myofibrillar myopathy.Methods Clinical and pathological data of a patient with myofibrillar myopathy were anlysised retrospectively.Results The patient showed middle-aged onset,progressively proximal muscle weakness,mildly general muscle atrophy.Neurogenic changers were found in lower extremities on EMG.On mucle biopsy,cytoplasmic bodies presented in many muscle fibers in which disorganized myofibrillar networks and abnormal desmin aggregates were... |
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| Keywords: | myofibrillar myopathy cytoplasmic body desmin |
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