遗传性压力敏感性周围神经病10例临床及电生理分析.DOCVIP

作者：周夏俊，上海长海路168号，长海医院神经内科，200433，aseren@通讯作者：管阳太，上海长海路168号，长海医院神经内科，200433，yangtaiguan@ 遗传性压力敏感性周围神经病20例临床及电生理分析 周夏俊1 张敏敏1 刘黔云2 管阳太1* 1上海市第二军医大学附属长海医院 200433 2 上海市同济大学附属同济医院 200065 【目的】通过分段电生理方法，研究遗传性压力敏感性周围神经病（HNPP）的临床和电生理特征。【方法】收集符合HNPP诊断标准的发病者和无临床症状者共20例，慢性炎性脱髓鞘性周围神经病（CIDP）组16例，腓骨肌萎缩症1A型（CMT 1A）组12例，腕管综合征（CaTS）组23例，肘管综合征（CuTS）组18例，正常对照（NC）组25例，测定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波比率（MFR P/M）。【结果】发病组和无症状组有着一致的临床和电生理特征；HNPP组TLI明显降低；CaTS组正中神经TLI较HNPP组降低；HNPP组尺神经MFR（P/M）较CuTS组无显著差别。【结论】HNPP不仅是以卡压、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，这种损害可能并不是由卡压继发的；HNPP的腕部损害存在自愈倾向。 【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波比率 A Clinical and Electrophysiology Study of 20 Cases Hereditary Neuropathy with liability to Pressure Palsies Zhou xia-jun1 Zhang min-min1 Liu qian-yun2 Guan yang-tai1* 1Shanghai Changhai Hospital 2Shanghai Tongji Hospital affiliated to Tongji [Objects]To study the clinical and electrophysiological characteristic of Hereditary neuropathy with liability to pressure palsies (HNPP) by segmental electrophysiological methods. [Methods]We collected total 20 symptomatic and asymptomatic patients according to HNPP diagnostic criteria, 16 cases of Chronic inflammatory demyelinating polyneuropathy (CIDP), 12 cases of Charcot-Marie-Tooth 1A(CMT1A), 23 cases of Carpal tunnel syndrome(CaTS), 18 cases of Cubital tunnel syndrome (CuTS) and 23 cases of Normal control(NC). Regular median and ulnar nerve conduction was tested to figure their Terminal latency index (TLI) and Proximal/Middle Modified F ratio (MFR P/M). [Results]There was no clinical and electrophysiological difference between symptomatic or asymptomatic HNPP group. HNPP group showed a lower trend of TLI by other groups. But decrease of median nerve TLI of CaTS group was more dominant. There was no significant difference between CuTS group and HNPP group by ulnar nerve MFR(P/M). [Conclusion]HNPP is not only featured by nerve compression and peripheral neuropathy background, but also prominent distal lesion,