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腓骨肌萎缩症又名遗传性运动感觉周围神经病(Charcot Marie Tooth病),现将一家族6例发病者(附图)报告如下。典型病例女,52岁。渐进性双下肢无力10年.10年前无何诱因感觉双下肢无力,行走缓慢,嗣后逐渐加重,经常摔倒,双下肢变细。近5年来上述症状明显加重。近1年来下蹲后不能站立,行走则足趾碰地。神经系统检查:双小腿部肌肉萎缩,大腿部则不明显,双足下垂。双下肢近端肌力Ⅳ级,远端肌力Ⅱ级~+.双跟腱反射减弱,而膝反射正常.巴氏征阴性.感觉正常.肌电图检查:胫后、正中神经感觉传导速度分别减慢56%和59%,波幅分别下降83%和90%。末端运动潜伏期延长118%
Charcot Marie-Milman disease (Charcot Marie Tooth disease), also known as Charcot Marie Tooth disease, is now reported as follows in six patients with one family affiliation (with photos). Typical case female, 52 years old. Progressive weakness of both lower extremities 10 years 10 years ago, no incentive to feel both lower extremities weakness, walking slowly, then gradually increased, often fall, lower extremities thinning. The past five years, the symptoms were significantly worse. Almost a year after squatting can not stand, walking is toe touch. Nervous system examination: double lower leg muscle atrophy, thigh is not obvious, feet drooping. Lower extremity proximal muscle strength grade IV, distal muscle strength grade ~ + .Double Achilles tendon reflexes, and knee reflexes normal .Bas sign is negative .Feel normal .EMG examination: tibial, median nerve sensory conduction velocity Slowed 56% and 59% respectively, with the volatility dropping by 83% and 90% respectively. End exercise latency of 118%