临床儿科杂志 ›› 2018, Vol. 36 ›› Issue (8): 602-.doi: 10.3969/j.issn.1000-3606.2018.08.009

• 综合报道 • 上一篇    下一篇

瞬目反射潜伏期在遗传性周围神经病分型中的作用#br#

孙瑞迪 1, 王恒东 2, 江军 1   

  1. 1.华中科技大学同济医学院附属武汉儿童医院(湖北武汉 421000);2.十堰太和医院儿科一病区 (湖北十堰 442008)
  • 收稿日期:2018-08-15 出版日期:2018-08-15 发布日期:2018-08-15
  • 通讯作者: 江军 E-mail:jiangjunzm@163.com
  • 基金资助:
    武汉市科技创新平台-儿童神经疾病临床医学研究中心资助项目(武科计2014-160号)(院内课题支持项目 2017FE002)

Utility of blink reflexes in subtyping of inherited polyneuropathy

 SUN Ruidi1,WANG Hengdong2, JIANG Jun1   

  1. Department of Electrophysiology, 1.Wuhan Children Hospital Wuhan 421000, Hubei, China; 2. Hubei Taihe Hospital, Wuhan 442008, Hubei, China
  • Received:2018-08-15 Online:2018-08-15 Published:2018-08-15

摘要: 目的 探讨瞬目反射R1潜伏期在遗传性周围神经病(CMT)分型中的作用。方法 回顾分析确诊为CMT患 儿的临床资料,收集常规神经传导和瞬目反射的检查结果,记录尺神经运动传导速度,波幅和R1的潜伏期;并根据基因 检测结果将患儿分为脱髓鞘型组和轴突型组,比较两组间R1潜伏期差异。结果 共纳入36例CMT患儿,男20例、女16 例。其中脱髓鞘型24例,男14例、女10例;轴突型12例,男6例、女6例。脱髓鞘型组的R1潜伏期为(14.41±0.73)ms, 明显长于轴突型组的(11.39±0.89)ms,差异有统计学意义(P<0.01);且脱髓鞘型组R1潜伏期均>13 ms,而轴突型组 均≤13 ms。在脱髓鞘型组中,尺神经波幅<0.5 mv患儿的R1可以稳定引出且潜伏期>13 ms。结论 瞬目反射R1潜伏期 > 13 ms为判断CMT脱髓鞘病变的可靠指标,且不受运动传导波幅影响;可指导基因检测。

Abstract:  Objective To investigate the utility of R1 latency of blink reflexes in typing of inherited polyneuropathy: Charcot-Marie-Tooth (CMT). Methods Patients diagnosed with CMT by a genetic test underwent blink reflex testing and extremity NCS were retrospectively analyzed. Blink reflex R1 latency, extremity NCS, and severity were compared between demyelinating subtypes and axonal subtypes. Results We included 24 demyelinating (14 males and 10 females) and 12 axonal (6 males and 6 females) CMT patients. R1-latency in demyelinating CMT group (14.41±0.73 ms) were longer than that in axonal CMT group (11.39±0.89 ms) (P<0.01). R1-latency were all >13 ms in demyelinating CMT group and ≤13 ms in axonal CMT group. Conclusions Blink reflex test is reliable for identification of inherited demyelinating polyneuropathy regardless of severity and can guide genetic testing.