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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)感觉神经传导速度减慢的异常率为89.6%,以正中神经感觉减慢最明显。(4)尺神经在肘部出现传导阻滞明显多于腓总神经在腓骨小头处。结论对临床上高度怀疑HNPP的患者,神经电生理检查是一种无创、快速、简便、准确的检查方法,并且可对患者家属进行筛查,对预防该病有重要意义。
Objective To investigate the neuroelectrophysiological characteristics of hereditary stress-susceptible peripheral neuropathy (HNPP) and provide the basis for the diagnosis of the disease. Methods Eight patients with symptomatic and asymptomatic suspected HNPP in 3 pedigrees were subjected to routine electrophysiological tests including sensory and motor nerve conduction in the bilateral median, ulnar, radial, tibial and common peroneal nerves, The results with the same period in our hospital electromyography room 30 healthy people in the same age group normal value. Results (1) The motor nerve conduction terminal latency prolongation was the highest, 92.1%, mainly in the median nerve and common peroneal nerve, asymptomatic and symptoms were prolonged. (2) The abnormal rate of motor nerve conduction slowed down was 63.2%, the most common was the slowdown of median nerve and common peroneal nerve conduction. (3) The abnormal rate of sensory nerve conduction slowed down was 89.6%, with the most obvious slowing of median nerve sensation. (4) ulnar nerve block appears significantly more in the elbow than the common peroneal fibular head. Conclusion In patients with highly suspected HNPP clinically, neuroelectrophysiological examination is a non-invasive, rapid, simple and accurate method for screening and screening patients’ families, which is of great significance for the prevention of HNPP.