平山病误诊运动神经元病二例临床分析

目的探讨平山病诊断要点,提高临床医生对该病认识,降低误诊率。方法报道我院近两年被误诊为运动神经元病的2例平山病病例,结合文献复习就平山病与运动神经元病的临床特点、神经电生理特性以及影像学诊断要点进行总结分析和鉴别。结果 :结合文献报道分析,平山病平均起病年龄16.91±2.47岁,男女比18.6:1,均表现以上肢远端受累为主的肌无力和肌萎缩,其中寒冷麻痹72.3%,手伸展震颤74.3%,肌电图示神经源性异常肌电改变,屈颈位颈椎MR有特征性改变:硬膜外腔见月牙形异常信号或流空血管影。结论 "寒冷麻痹及手平举震颤",是平山病临床表现,也是避免误诊的重要鉴别要点。颈椎MR特征性改变对确诊平山病具有重要价值。

Two hirayama disease cases misdiagnosis of motor neuron disease and the review

Objective To analyze the characteristic of diagnosis in Hirayama disease for decreasing the rate of misdiagnosis.Methods The clinic,electromyologram and MRI imaging examination were analysed from 212 patients reported in chineses articles and two patients diagnosised in our department.Results The moyen age of Hirayama disease patients was 16.91±2.47,the ratio male/female was 18.6:1,its characteristic manifestation was myasthenia of limited distal of limb,finger and wrist accompanied with distal amyotrophy.T...