平山病的神经电生理学研究  

Study of electroneurophysiology of Hirayama disease

作　　者：徐迎胜[1] 郑菊阳[1] 张朔[1] 张俊[1] 康德宣[1] 樊东升[1] 

机构地区：[1]北京大学第三医院神经内科,100083

出　　处：《中国现代神经疾病杂志》2008年第4期314-317,共4页Chinese Journal of Contemporary Neurology and Neurosurgery

摘　　要：目的探讨平山病的神经电生理学特点及其与肌萎缩侧索硬化、多灶性运动神经病的鉴别诊断。方法分别对平山病(26例)、肌萎缩侧索硬化(30例)和多灶性运动神经病(16例)患者进行运动和感觉传导速度、肌电图及交感皮肤反应等神经电生理学检查。运动传导速度采用由远端至近端分段刺激,记录复合肌肉动作电位的波幅、时限、面积及波形的变化,并判断是否存在神经传导阻滞;肌电图检查记录脑区肌肉(双侧胸锁乳突肌),颈区肌肉(拇短展肌、小指展肌、第一骨间肌、肱二头肌),胸区肌肉(T10椎旁肌、腹直肌)和腰骶区肌肉(胫骨前肌)的肌电活动。比较3组患者神经电生理学特点的差异性。结果平山病组患者均无神经传导阻滞,肌电图检查显示颈区肌肉呈神经源性损害;肌萎缩侧索硬化组患者亦无神经传导阻滞,肌电图检查显示脑区、颈区、胸区和腰骶区肌肉均呈神经源性损害;多灶性运动神经病组患者均存在神经传导阻滞,肌电图检查颈区和腰骶区肌肉呈神经源性损害。平山病组患者神经传导阻滞的发生率与多灶性运动神经病组比较,差异有统计学意义(χ2=42.000,P=0.000);平山病组患者神经源性损害的发生率与肌萎缩侧索硬化组比较,差异亦有统计学意义(χ2=56.000,P=0.000)。结论平山病组患者运动和感觉传导速度均无异常,无神经传导阻滞,但肌电图检查显示颈区肌肉呈神经源性损害。Objective To explore the electroneurophysiological features of Hirayama disease （HD） and its differential diagnosis with amyotrophic lateral sclerosis （ALS） and multifocal motor neuropathy （MMN）. Methods Electroneurophysiological studies were performed in 26 patients with HD, 30 patients with ALS and 16 patients with MMN, including motor conduction velocity （MCV）, sensory conduction velocity （SCV）, electromyography （EMG） and sympathetic skin response （SSR）. MCV were done segmentally by stimulating the nerve distally as well as proximally. The amplitude, duration, area and waveform changes of compound muscle action potential （CMAP） were recorded and analysed. The values obtained from the distal and proximal parts were compared to ascertain the presence of conduction block （CB）. EMG was performed in muscles of different regions： bulbar region （bilateral stemocleidomastoid muscles）, cervical region （abductor pollicis brevis muscle, abductor digiti minimi muscle, first dorsal intercostals muscle, biceps brachii muscle）, thoracic region （lower thoracic paraspinal muscle, rectus abdominis muscle） and lumbosacral region （tibialis anterior muscle）. Compared the electroneurophysiological features of patients in these 3 groups. Results In patients with HD, no CB was found, there were neurogenic changes of EMG in muscles of cervical region. While in patients with ALS, there were neurogenic changes of EMG in muscles of bulbar region, cervical region, thoracic region and lumbosacral region, and no CB was found in these patients either. There were CB in all MMN patients, neurogenic changes of EMG in muscles of cervical region and lumbosacral region were inves- tigated. As concerned with the incidence of CB, there was significant difference between HD patients and MMN patients （χ^2= 42.000, P = 0.000）. Regarding neurogenie changes of EMG, there was significant difference between HD patients and ALS patients （χ^2= 56.000, P= 0.000）. Conclusion In patients with HD, t

关 键 词：肌萎缩 肌萎缩侧索硬化 运动神经元病 诊断 鉴别 神经传导阻滞 肌电描记术 

分 类 号：R746[医药卫生—神经病学与精神病学]