遗传性压力敏感性周围神经病20例临床和神经电生理特征  

Clinical and electrophysiology study on 20 cases of hereditary neuropathy with liability to pressure palsies

作　　者：周夏俊[1] 张敏敏[1] 刘黔云[2] 管阳太[1] 

机构地区：[1]上海长海医院神经内科,上海200433 [2]同济大学附属同济医院神经内科

出　　处：《上海医学》2013年第11期924-927,共4页Shanghai Medical Journal

摘　　要：目的通过分段神经电生理检查,研究遗传性压力敏感性周围神经病(HNPP)的临床和神经电生理特征。方法收集符合HNPP诊断标准的发病者(发病组)和无临床症状者(无症状组)各10例(合并为HNPP组),慢性炎性脱髓鞘性周围神经病(CIDP)患者(CIDP组)16例,腓骨肌萎缩症(CMT)1A型患者(CMT1A组)12例,腕管综合征(CaTS)患者(CaTS组)23例,肘管综合征(CuTS)患者(CuTS组)18例,健康志愿者(正常对照组)25例,检测所有受试者的正中神经、尺神经的常规神经传导,计算远端潜伏期指数(TLI)和近端/中段改良F波比值[MFR(P/M)]。结果发病组与无症状组的临床表现和神经电生理特征相似。正常对照组中正中神经与尺神经间TLI值的差异无统计学意义(P>0.05),而腓总神经的TLI值显著高于同组其他神经(P值均<0.01)。发病组和无症状组的正中神经和尺神经的TLI值分别显著低于正常对照组相同神经(P值均<0.05),发病组与无症状组间各神经TLI值的差异均无统计学意义(P值均>0.05)。HNPP组和CaTS组正中神经的TLI值均显著低于其他组的相同神经(P值均<0.01),且CaTS组显著低于HNPP组(P=0.04)。HNPP组尺神经的TLI值显著低于其他组的相同神经(P值均<0.05),其他组间尺神经TLI值的差异无统计学意义(P值均>0.05)。HNPP组与CuTS组间尺神经MFR(P/M)的差异无统计意义(P>0.05)。结论 HNPP不仅以神经卡压、周围神经病背景为特征,更是以远端损害突出为表现的脱髓鞘性周围神经病,这种损害可能不是由神经卡压继发的,HNPP的腕部损害存在自愈倾向。Objective To study the clinical and electrophysiological characteristics of hereditary neuropathy with liability to pressure palsies （HNPP） by segmental electrophysiological methods. Methods We collected 10 symptomatic and 10 asymptomatic patients according to HNPP diagnostic criteria, 16 patients with chronic inflammatory demyelinating polyneuropathy （CIDP）, 12 patients with Charcot-Marie-Tooth 1A （CMT1A）, 23 patients with carpal tunnel syndrome （CATS）, 18 patients with cubital tunnel syndrome （CUTS） and 25 healthy volunteers （normal control group）. Regular median and ulnar nerve conduction was tested to figure their terminal latency index （TLI） and proximal/middle modified F ratio EMFR （P/M）~. Results Similar clinical and electrophysiological characteristics were found in symptomatic and asymptomatic patients. In normal control group, there was no significant difference in TLI between median nerve and ulnar nerve （P~〉0.05）, while TLI of peroneal nerve was significantly higher than that of other nerves （both P〈0.01）. TLI values of median and ulnar nerve in symptomatic and asymptomatic groups were significantly lower than that of the normal groups （all P〈0.05）, but there was no significant difference in TLI between symptomatic and asymptomatic groups （all P〈0.05）. HNPP and CaTS group had lower TLI of median nerve than other groups （all P〈0.01）; TLI of median nerve in CaTS group was significantly lower than that in HNPP group （P = 0. 04）. TLI of ulnar nerve in HNPP group wassignificantly lower than that in other groups （all P〈0.05）, but there was no significant difference in TLI of ulnar nerve between the other groups （all P〉0.05）. There was no significant difference in ulnar nerve MFR （P/M） between HNPP group and CuTS group （P〉0.05）. Conclusion HNPP is featured not only by nerve compression and peripheral neuropathy, but also by demyelinated distal lesion of peripheral nerve, which may not be followed by nerve compression. Nat

关 键 词：遗传性压力敏感性周围神经病 远端潜伏期指数 改良F波比值 

分 类 号：R745[医药卫生—神经病学与精神病学]