摘要
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）uV；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 Kennedy disease （KD） and to make sure the functional and anatomical change of the sensory nerve of KD patients. Methods Electrophysiological and pathological tests were performed in 14 KD patients, including electromyogram, conduction velocities of median, ulnar, peroneal, tibial, and sural nerves, trigeminocervical 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 muscles contract slightly. Electrical stimuli were applied to the infraorbital nerves and recording electrodes were placed in the sternocleidomastoid muscles. For CHEP, contact heat was delivered via a circular thermode to excite selectively nociceptors with a rapid rising time at 70℃/s to elicit pain and CHEP. Thermal stimuli were sent at 54. 5℃ to three body sites： skin of back of hand, proximal volar forarm, and C7. The CHEPs were 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 0. 65 -2. 85 pN. The latency of trigemino-cervical reflex was longer than normal with the onset peak latency of （ 38.9 ± 7. 0 ） ms. The configuration was asymmetric. The onset peak latencies when the skin of the back of hand, volar surface of forearm, and C7 were stimulated were （613 ±57）, （595 ±32）, and （489 ±37） ms respectively. Biopsy of the sural nerves showed that the large myelinated nerve fiber was decreased. Conclusion Sensory nerve is involved in the patients with Kennedy disease, including the large and small fibers.
出处
《中华医学杂志》
CAS
CSCD
北大核心
2008年第39期2771-2774,共4页
National Medical Journal of China
