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目的探讨遗传性运动感觉性周围神经病I型(CMT I型)患者的临床表现与肌电生理特点之间的关联。方法观察25例CMTI型患者的临床特征,检测其双下肢侧及上肢远端的常规肌电图、周围神经传导速度和波幅改变情况,并探讨CMT l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。结果本组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7例。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。患者病程延长与正中神经、尺神经及胫神经的CMAPs下降趋势有相关关系(r=0.39,P<0.05),MCV也出现类似的变化趋势(r=0.28,P<0.05)。且肌肉萎缩、无力程度与CMAPs正相关而与MCV无相关关系。结论本组CMT I多在青少年时期起病,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异常是诊断CMT 1的依据之一,并与疾病的进展程度相关。
Objective To investigate the association between the clinical manifestations and the electrophysiological characteristics of patients with hereditary motor-sensory peripheral neuropathy type I (CMT I). Methods The clinical features of 25 patients with CMTI were observed. The changes of routine electromyography, peripheral nerve conduction velocity and amplitude in bilateral lower extremities and distal extremities were observed. The relationship between CMT l myofascial weakness, atrophy and tibial nerve, Relationship between nerve and ulnar nerve CMAPs and MCV. Results The main clinical symptoms of this group were progressive lower extremity or limb symmetry of distal muscle weakness and / or muscular atrophy. The symptoms of this group were limited to 7 cases of both lower extremities. Electromyography showed all patients showed neurogenic damage, tibial nerve, peroneal nerve, median nerve and ulnar nerve conduction velocity decreased to some extent or even not lead to lower amplitude. There was a correlation between the prolongation of the course of disease and the decreasing trend of CMAPs in the median nerve, ulnar nerve and tibial nerve (r = 0.39, P <0.05). MCV also showed similar trend (r = 0.28, P <0.05). And muscle atrophy, weakness and positive correlation with CMAPs but no correlation with MCV. Conclusions This group of CMT I mostly in adolescence onset, with chronic progressive lower extremity or distal limb muscle weakness and muscle atrophy as the main features of EMG and peripheral nerve conduction velocity abnormalities is the basis of the diagnosis of CMT 1, and Related to the progress of the disease.