| 伴有周围神经损害的强直性肌营养不良 |
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| 引用本文： | 笪宇威,李彦,徐敏,刘尖尖,贾建平.伴有周围神经损害的强直性肌营养不良[J].卒中与神经疾病,2006,13(6):345-347,350. |
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| 作者姓名： | 笪宇威 李彦 徐敏 刘尖尖 贾建平 |
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| 作者单位： | 100053,北京,首都医科大学宣武医院神经内科 |
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| 摘 要： | 目的探讨强直性肌营养不良(DM)的临床表现和病理改变。方法通过1例确诊的DM病例的临床表现和神经肌肉的病理改变来系统回顾本病的发病机理、分类、临床表现、病理改变、诊断和治疗。结果该例患者的临床表型和肌肉病理改变符合DM1型,腓肠神经主要病理改变为轴索变性,伴有轻度脱髓鞘。结论DM是一组遗传性疾病,临床表现为骨骼肌萎缩、力弱、强直和多种骨骼肌以外的症状,可伴有以轴索变性为主的周围神经损害。
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| 关 键 词： | 强直性肌营养不良 病理 周围神经 |
| 文章编号： | 1007-0478（2006）06-0345-03 |
| 收稿时间： | 2006-03-31 |
| 修稿时间： | 2006-03-312006-08-23 |
Myotonic dystrophy with peripheral damage |
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| Da Yuwei , Li Yan , Xu Min ,et al..Myotonic dystrophy with peripheral damage[J].Stroke and Nervous Diseases,2006,13(6):345-347,350. |
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| Authors: | Da Yuwei Li Yan Xu Min |
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| Institution: | Department of Neurology, Xun wu Hospital ,Capital University of Medical Sciences ,Beijing 100053 |
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| Abstract: | Objective To study the clinical and pathological features of myotonic dystrophy(DM).Methods One case of definite DM was studied to analyze the clinical and pathological features.Pathogenesis,classification,clinical manifestations,pathological features,diagnosis and therapy of DM were reviewed in detail.Results The clinical and muscle pathological features of this patient were consistent with DM type 1.Sural nerve biopsy showed axonal degeneration with mild demyelination.Conclusions DM is a group of inherited disease that results in skeletal muscle weakness,wasting,myotonia and numerous nonmuscular manifestations with axonal degeneration as the main change in peripheral nerve. |
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| Keywords: | Myotonic dystrophy Pathology Peripheral nerve |
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