29例腓骨肌萎缩症患者的神经电生理检测特征性分析

Neuroelectrophysiological characteristic analysis on 29 patients with Charcot-Marie-Tooth disease

目的探讨腓骨肌萎缩症(Charot-Marie-Tooth disease,CMT)患者的神经电生理改变特点。方法应用美国Nicolet公司的Viking IV肌电图/诱发电位仪对29例腓骨肌萎缩症患者进行周围神经传导速度及肌电图检测,对所得结果进行回顾性总结和分析。结果100%患者被检的运动或感觉神经传导速度存在不同程度的减慢或消失,且感觉神经病变重于运动神经,下肢受累程度重于上肢;70块被检肌肉中,43块(61.4%)呈神经源性损害,5块(7.2%)呈可疑神经源性损害,以远端肌肉病变严重;患者年龄越大,肌肉受累程度越严重,相反,年龄偏小的患者则以神经传导速度减慢为主。结论不同CMT患者的肌肉受累程度和神经传导速度减慢程度与其年龄大小、病程长短有关,而对于同一患者来说,其下肢受累程度要重于上肢。

Objective To evaluate the neuroelectrophysiological characteristics of patients with Charot-Marie- Tooth disease （CMT）. Methods Peripheral nerve conduction velocity and electromyography were performed on 29 patients with CMT using American Viking Ⅳ machine. Retrospctive analysis was used to analyze the data. Results The nerve conduction velocities significantly slowed or disappeared in all of the patients, The sensory nerve damage was more severe than the motor nerve damage and lower extremity nerve damage was more severe than the upper extremity nerve damage. Of 70 muscles, 43 （61.4%） exhibited neurogenie damage, and 5 （7.2%） suspicious neurogenic damage. The elder patients had severe muscle damage whereas the youngers mainly presented with the slower never conduction velocties. Conclusions Our data suggest that the severity of the muscle damage and the nerve conduction abnormalities in patients with Charcot-Marie-Tooth disease is related to the age of onset and the length of course and that nerve damage is more severe in lower than in upper extremities.