肯尼迪病患者自动递增刺激法运动单位估数的测定

The evaluation of motor unit number estimation in patients with Kennedy's disease

目的研究肯尼迪病(KD)患者运动单位估数(MUNE)的变化并探讨其临床意义。方法收集KD患者20例和健康对照者30名,分别用自动递增磁刺激法检测受试者大、小鱼际肌MUNE数值及正中神经、尺神经最大复合肌肉动作电位(CMAP)波幅。比较KD组和对照组大小鱼际肌MUNE的差异;比较KD组患者大小鱼际肌MUNE异常率与正中神经、尺神经CMAP异常率的差异。同时,对KD患者病程与MUNE的相关性进行分析。结果与对照组比较,KD患者大、小鱼际肌MUNE数值降低(82.16±49.37比251.12±68.89,94.26±44.56比235.63±63.02,均P<0.01)。KD患者大、小鱼际肌MUNE异常率分别为85.0%和80.0%,而正中神经、尺神经CMAP异常率均为15.0%,KD患者大小鱼际肌MUNE异常率与正中神经、尺神经CMAP异常率比较差异均有统计学意义(P<0.01)。KD患者MUNE数值与病程呈负相关(P<0.05)。结论 MUNE作为一种无创的电生理学技术,可能对早期及精确地判断KD患者下运动神经元受损情况有所帮助。

Objective To evaluate the changes and clinical significance of motor unit number estimation（MUNE）in patients with Kennedy＇s disease（KD）.Methods An automated incremental stimulating technique was used to estimate the motor unit number of thenar and hypothenar muscles from 30 healthy subjects without nerve and muscle disease and 20 patients definitely diagnosed as KD.At the same time,compound muscle action potentials（CMAPs）of median nerves and ulnar nerves were detected in all subjects.MUNEs of the thenar and hypothenar muscles were compared between the two groups.The abnormal rates were compared between MUNEs and CMAPs in KD patients.At the same time,the correlation of MUNEs and course of disease were analyzed.Results The MUNE value of 60 normal thenar muscles in 30 normal controls was 251.12±68.89,and the value of the hypothenar muscles was 235.63±63.02,respectively.The MUNE values of thenar and hypothenar muscles in 20 cases with KD were 82.16±49.37 and 94.26±44.56,respectively.The differences of MUNE values of both thenar and hypothenar muscles between normal subjects and KD patients were significant（P〈0.01）.The abnormal ratio of MUNE of thenar and hypothenar muscles in KD patients was 85.0% and80.0%,respectively;compared with the abnormal ratio of CMAPs of median nerves and ulnar nerves in KD patients（15.0%）,the differences were significant（P〈0.01）.The MUNE value was negatively correlated with duration of the disease（P〈0.05）.Conclusions As an atraumatic electrophysiological technique,MUNE may be helpful to early and accurately judge the lower motor neuron damage in KD patients.