Kennedy病患者感觉神经检测 ; Test of sensory nerve in patients with Kennedy disease

目的 对Kennedy病患者进行神经电生理和病理研究,了解其感觉神经功能及结构状况.方法 对14例Kennedy病患者进行肌电图和神经传导速度、三叉神经-颈反射(TCR)、接触性热痛诱发电位(CHEP)检测及腓肠神经活检.对照组进行相应研究.结果 Kennedy病患者感觉神经传导速度正常或轻度减慢(减慢者占7.2%).感觉神经动作电位波幅明显降低,为(0.65～2.85)μV;三叉神经-颈反射潜伏期延长[初始峰潜伏期为(38.9±7.0)ms]、波形双侧不对称;痛觉诱发电位表现为CHEP潜伏期延长[手背、前臂的掌侧面和C7部位刺激时,CHEP初始峰潜伏期分别为(613±57)ms、(595±32)ms、(489±37)ms]或波形消失;腓肠神经活检示大的有髓鞘纤维减少.结论 Kennedy病累及感觉系统,大、小感觉神经纤维均可受累. ; Objective To study the electrophysiological and pathological features of Kennedydisease (KD) and to make sure the functional and anatomical change of the sensory nerve of KDpatients. Methods Electrophysiological and pathological tests were performed in 14 KD patients, includingelectromyogram, conduction velocities of median, ulnar, peroneal, tibial, and sural nerves, trigemino-cervical reflex(TCR), contact heat evoked potential(CHEP) , and biopsy of the sural nerves. During TCR,the patients lay on the back, holding the heads slightly raised to make the sternocleidomastoid musclescontract slightly. Electrical stimuli were applied to the infraorbital nerves and recording electrodes wereplaced in the sternocleidomastoid muscles. For CHEP, contact heat was delivered via a circular thermode toexcite selectively nociceptors with a rapid rising time at 70℃/s to elicit pain and CHEP. Thermal stimuliwere sent at 54.5℃ to three body sites: skin of back of hand, proximal volar forarm, and C7. The CHEPswere recorded from Cz and Pz. Results The sensory conduction velocity and latency were almost normal,while the amplitude of the sensory nerve action potential(SNAP) declined to O. 65 -2. 85 μV. The latency oftrigemino-cervical reflex was longer than normal with the onset peak latency of (38.9±7.0 ) ms. Theconfiguration was asymmetric. The onset peak latencies when the skin of the back of hand, volar surface offorearm, and C7 were stimulated were (613±57), (595±32), and (489±37) ms respectively. Biopsy ofthe sural nerves showed that the large myelinated nerve fiber was decreased. Conclusion Sensory nerve isinvolved in the patients with Kennedy disease, ...