| 遗传性运动感觉性周围神经病Ⅰ型的临床及电生理特点 |
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| 引用本文： | 梁银杏,葛辉,廖松洁,姚晓黎. 遗传性运动感觉性周围神经病Ⅰ型的临床及电生理特点[J]. 中国神经精神疾病杂志, 2010, 36(4) |
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| 作者姓名： | 梁银杏 葛辉 廖松洁 姚晓黎 |
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| 作者单位： | 1. 中山大学附属第一医院神经科,广州,510080
2. 中山大学附属第一医院保健科 |
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| 摘 要： | 目的研究遗传性运动感觉性周围神经病I型(CMT1型)患者的临床特征、肌电生理特点及二者的相关性。方法观察临床确诊的36例CMT1型患者的临床特征,检测双侧上肢远端及下肢常规肌电图及周围神经传导速度及波幅改变情况,并探讨CMT1的肌无力、萎缩程度与胫神经、正中神经及尺神经CMAPs及MCV的关系。结果36例临床确诊的CMT1型患者,男性多于女性,均在青少年期起病,主要临床症状为进行性双下肢或四肢对称性远端肌无力和/或肌萎缩,本组患者症状局限于双下肢者20例(55.56%)。肌电图检查示所有患者均表现为神经源性损害,胫神经、腓神经、正中神经和尺神经均有不同程度的传导速度下降甚至不能引出,波幅降低。随起病年龄增大、病程延长,正中神经、尺神经及胫神经的MCV有下降的趋势并可能测不出(r=0.39,P0.05),CMAPs也出现类似的变化趋势(r=0.28,P0.05)。且肌肉萎缩、无力程度与CMAPs正相关而与MCV无相关关系。结论CMT1多为青少年起病,以慢性进行性双下肢或四肢远端肌无力和肌萎缩为主要特征,肌电图和周围神经传导速度是诊断CMT1的可靠方法,并能预测疾病的进展程度。
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| 关 键 词： | 肌萎缩 肌电生理 运动传导速度 混合肌肉动作电位 |
Clinical and electrophysiologic features of Type I Charcot-Marie-Tooth disease |
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| LIANG Yinxing,GE Hui,LIAO Songjie,YAO Xiaoli.The. Clinical and electrophysiologic features of Type I Charcot-Marie-Tooth disease[J]. Chinese Journal of Nervous and Mental Diseases, 2010, 36(4) |
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| Authors: | LIANG Yinxing GE Hui LIAO Songjie YAO Xiaoli.The |
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| Affiliation: | LIANG Yinxing,GE Hui,LIAO Songjie,YAO Xiaoli.The Department of Neurology,the First Affiliated Hospital of SUN Yat-sen University,Guangzhou 510080,China |
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| Abstract: | Objective To investigate the clinical and electrophysiologic characteristics in Type I Charcot-Marie-Tooth disease (CMT 1).Methods Thirty six patients with CMT 1 were retrospectively studied.The clinical and electrophysiologic features were investigated.Routine electromyograph (EMG) and nerve conduction have been performed in all patients.The correlation between clinical severity and electrophyisiological changes were analyzed.Results All the patients presented symmetrical progressive weakness and numbness ... |
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| Keywords: | CMT1 |
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