肯尼迪病患者的神经电生理分析

目的:研究肯尼迪病患者的神经电生理特点,提高对此病电生理的认识。方法:对经基因确诊的3例肯尼迪患者的神经电生理进行总结分析。3例患者均行神经传导和肌电图(EMG)检测。神经传导检查包括:正中神经、尺神经、腓总神经、胫神经和腓肠神经。检测项目包括运动神经潜伏期(Lat)、复合肌肉动作电位波幅(CMAP)、运动神经传导速度(MCV);感觉神经潜伏期(lat)、感觉神经动作电位波幅(SNAP)、感觉神经传导速度(SCV)。EMG:检查双侧舌肌、胸锁乳突肌、三角肌、第一骨间肌、胸椎旁肌、股四头肌、胫前肌。检测项目包括静止时的自发电位,轻收缩时运动单位动作电位(MUP)的时限、波幅,重收缩时的募集相。结果:第1例患者运动神经传导正常,感觉神经SNAP波幅低和SCV正常。第2例患者双正中神经运动Lat延长、CMAP下降,NCV减慢,双正中神经感觉未测及SNAP波幅,合并腕管综合征;其余运动神经传导正常,感觉神经SNAP波幅和SCV均低。第3例运动神经传导正常,感觉SNAP波幅低和SCV下降。本组3例患者EMG多块肌肉静息时出现2处以上的纤颤波、正尖波,轻收缩时MUP时限增宽,波幅增高,部分呈巨大电位,重收缩时呈单纯相或单纯混合相。结论:KD患者EMG呈广泛神经源性改变,可累及感觉。

THE ANALYSIS ON ELECTRONEUROPHYSIOLOGY OF PATIENTS WITH KENNEDY DISEASE

Objective： To stdudy the electroneurophysiologic features of the patients with Kennedy disease （ KD ） , so that deep the understanding of the electrophysiologic change of KD. Methods： Three patients with KD diagnosed by gene analysis accepted nerve conduction study and EMG detection. The nerves included median nerve, ulnar nerve, nervus peroneus communis, tibial nerve and sural nerve. The parameters were motor nerve latency （ Lat ） , compound muscle action potential （ CMAP ） , motor nerve conduction velocity （ MCV ） , sensory nerve latency （ lat ）, sensory nerve action potential （ SNAP ） and sensory nerve conduction velocity （ SCV ）. The EMG was recorded at bilateral linguales, sternocleidomastoid muscles, deltoid muscles, first dorsal interossei muscles, thoracic paravertebral muscles, quadriceps femoris muscles and tibialis anterior muscles. Parameters included spontaneous activity, amplitude of polyphasic wave of motor unit action potential （MUAP） ; pattern of recruitment. Results： In case 1, the MCVs and SCVs were normal and the amplitudes of SNAPs were decreased. In case 2 combined with carpal tunnel syndrome, the Lats of bilateral median moter nerves were prolonged, CMAPs decreased and MCVs reduced, and the SNAPs of bilateral median sensory nerves disappeared The MCVs were normal and the amplitudes of SNAPs case 2 and in case 3. There were above 2 spontaneous and SCVs were both decreased except for median nerves in activities in multiple muscles of the three patients with KD The duration and amplitude of MUAPs were increased and some were huge potentials. Simple phase or simple-mix phase were showed at strong contraction. Conclusions ： The EMGs of patients with KD showed wide Changes derived from lower motor neuron. Sensation abnormality may be involved in the manifestation.