| 摘要: |
| 目的探讨遗传性压力易感性周围神经病(HNPP)的神经电生理特点,为该病的诊断提供依据。方法对3个家系的8例有症状和无症状疑似HNPP患者进行常规神经电生理检测,包括双侧正中神经、尺神经、桡神经、胫神经和腓总神经的感觉和运动神经传导,并将其结果与同期我院肌电图室30例健康人同年龄组的正常值比较。结果(1)运动神经传导末端潜伏时延长异常率最高,为92.1%,主要出现在正中神经和腓总神经,有症状者和无症状者均延长。(2)运动神经传导速度减慢的异常率为63.2%,以正中神经和腓总神经传导减慢最多见。(3)感觉神经 |
| 关键词: 遗传性压力易感性周围神经病 末端潜伏时 神经传导速度 传导阻滞 |
| DOI：10.3724/SP.J.1008.2011.0730 |
| 投稿时间：2011-04-17修订日期：2011-05-09 |
| 基金项目: |
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| Neurophysiological diagnosis of hereditary neuropathy with liability to pressure palsies |
| DANG Jing-xia*,LIU Jie,WANG Jin,QIN Xing,JIN Jiao-ting,ZHOU Wen-ting,LI Zheng-yi |
| (Department of Neurology, the First Affiliated Hospital of Xi’an Jiaotong University, Xi’an 710061, Shaanxi, China) |
| Abstract: |
| 【Abstract】Objective To characterize the electrodiagnostic features of hereditary neuropathy with liability to pressure palsies（HNPP）. Methods Eight patients from three families with HNPP and their relatives underwent routine nerve conduction studies, and contrasted with 30 healthy subjects. Routine electrodiagnostic tests included median, ulnar, radial, tibial and proneal motor and sensory nerve conduction study. Results 76 motor nerve and 58 sensory nerve conduction velocity of the patients were tested. ①It showed broad and diffuse nerve conduction abnormalities，superimposed by conduction block, ulnar nerves at the elbow were more common than proneal nerves at the fibular head.②Distal motor latencies were significantly prolonged in 92.1% of the nerves, All the median and peroneal nerves with both symptomatic and asymptomatic patients were prolonged.③The abnormal rate of motor nerve conduction slowing was 63.2%, the most common affected nerves were median and peroneal nerve.④The abnormal rate of sensory nerve conduction slowing was 89.6%, with sural and median nerve affected more.Conclusion Neurophysiological examination is crucial to diagnose clinically suspected HNPP patients. It is useful to screen the family members of HNPP patients, as well as early detection, early prevention.②HNPP is characterized electrophysiologically by a generalized neuropathy, superimposed by focal conduction block, prolonged distal motor latency, obvious in median and proenal nerves. The slowing of sensory conduction was more severe than motor conduction. |
| Key words: hereditary neuropathy with liability to pressure palsies distal motor latency nerve conduction study conduction block. |
