CMT1A型与遗传性压迫易感性周围神经病的神经电生理对比研究  

The comparative study on electrophysiological changes in patients with hereditary neuropathy with liability to pressure palsy and patients with Charcot-Marie-Tooth 1A type disease

作　　者：宋春莉[1] 王哲[1] 刘丽波[2] 周丽娜[1] 唐攀[1] 梁战华[1] 韩杰[1] 孙大勇[1] 

机构地区：[1]大连医科大学附属第一医院神经内科,辽宁大连116011 [2]大连医科大学附属第一医院检验科,辽宁大连116011

出　　处：《中风与神经疾病杂志》2013年第3期215-217,共3页Journal of Apoplexy and Nervous Diseases

基　　金：辽宁省科技厅社会发展攻关计划资助项目(2011225013);大连市科技局资助项目(2011E15SF123)

摘　　要：目的探讨腓骨肌萎缩症1A型(CMT1A)与遗传性压力易感性神经病(HNPP)在神经电生理检测的不同特点。方法记录9例CMT1A型和12例HNPP患者的临床特点,对两组患者进行了正中神经、尺神经、胫神经、腓总神经运动神经传导速度检测和正中神经、尺神经、胫神经、腓浅神经、腓肠神经感觉神经传导速度检测。结果 CMT1A型患者存在广泛的电生理异常,四肢周围神经NCV都明显减慢或消失,而且感觉和运动减慢程度一致,并且对任何节段周围神经的影响程度相同;HNPP患者的电生理特点是广泛的SCV不同程度减慢,而MCV减慢相对较轻且不同节段程度不同,主要是末端潜伏期值延长,以及明显的运动神经易卡压部位传导阻滞。结论神经电生理检测是该两种疾病诊断及鉴别诊断的重要手段,短节段电位检测可证实HNPP的嵌压部位。Objective To investigate different feature of electrophysiology in patients with hereditary neuropathy with liability to pressure palsy（HNPP） and patients with Charcot-Marie-Tooth disease. Methods Record clinical features from 9 patients of CMT1A and 12 patients of HNPP, compare two groups of patients with motor nerve conduction velocity testing in median nerve, ulnar nerve, tibial nerve, peroneal nerve, as well as the sensory nerve conduction velocity in median nerve, ulnar nerve, tibial nerve, superficial peroneal nerve and sural nerve. Result Limb peripheral nerve NCV of CMT1A type patients are slowed down or disappeared, degree in SCV and MCV also decreased, suggest a wide range of electrophysi- ological abnormalities impacting on every segment. HNPP patients display extensive SCV decreases with different degree. At the same time MCV slow down relatively mild and vary different degrees in different segments, distal latency values dis- play prolonged as well as motor nerve conduction block in easily entrapment site. Conclusion Nerve electrophysiological testing is important means for diagnosis and differential diagnosis of the two diseases, the detection of short-segment may po- tentialy demonstrate the HNPP＇ s entrapment parts.

关 键 词：腓骨肌萎缩症1A型 遗传性压力易感性神经病 神经电生理 

分 类 号：R745[医药卫生—神经病学与精神病学]