一家系遗传性压力易感性周围神经病四例电生理临床分析  

The clinical analysis of neuroelectrophysiologic diagnosis in bereditary neuropathy with liability to pressurepalsies：four cases of one pedigree

作　　者：支0冬柏 张剑宇[1] 彭岚[1] 陶婷婷[1] 

机构地区：[1]东南大学医学院附属江阴医院神经内科,江苏省江阴214400

出　　处：《中国基层医药》2013年第18期2782-2783,共2页Chinese Journal of Primary Medicine and Pharmacy

摘　　要：目的探讨遗传性压力易感性周围神经病（HNPP）的临床神经电生理改变。方法对1个HNPP家系的先证者及4例家系患者进行神经传导速度检测，并对先证者进行病理组织学检查。结果先证者正中神经、尺神经、腓肠神经感觉神经传导速度（SCV）均减慢；正中神经、尺神经、腓总神经运动神经传导速度（MCV）均减慢。感觉纤维最易累及的神经为腓肠神经，受损程度以腓肠神经最重。运动神经传导检测结果：运动神经诱发电位潜伏期延长者18条异常率75．O％，所查神经远端MCV异常率87．5％。结论HNPP患者可伴有大部分神经传导障碍，可靠的神经电生理检查是诊断HNPP的重要依据，在无症状的HNPP患者及其家属也可出现神经传导障碍。在HNPP患者嵌压处神经更易受损。Objective To investigate the electrophysiological changes of peripheral nerves in both patients with hereditary neuropathy with liability to pressure palsies（HNPP）. Methods The nerve conduction velocities of a family consisting of a proband and 4 members were tested, and the proband was detected by biopsy. Results The proband＇s median nerve~ ulnar nerve, sural sensory nerve conduction velocity （SCV） were decreased. The median nerve, ulnar nerve, common peroneal nerve motor nerve conduction velocity （MCV） of proband were decreased. The sensory fibers of the most frequently involved nerve sural nerve, sural nerve damage to the results ： the motor nerve con- duction motor nerve evoked potential latency and 18 abnormal rate was 75.0%, the nerve distal to the abnormal rate of MCV was 87.5%. Conclusion Prolongation of distal nerve conduction latencies occurs in essentially all individuals whether symptomatic or asymptomatic. Nerves may be more easily damaged at the site of compression.

关 键 词：遗传性运动和感觉性神经病 电生理学 神经传导 

分 类 号：R745[医药卫生—神经病学与精神病学]