5例平山病肌电图及影像学特点分析

Analysis of electromyography and imaging characteristics of 5 cases of Hirayama disease

目的探讨平山病的肌电图及影像学特点,提高临床医师对该病的认识。方法回顾性分析我院收治的5例平山病患者的临床资料,对其临床表现、肌电图和影像学特点进行分析。结果5例患者均为男性,发病年龄18~28岁,单侧受累3例,双侧不对称性受累2例。5例患者均表现为上肢远端肌肉无力伴萎缩,前臂斜坡样改变,伴寒冷麻痹。运动神经传导主要表现为尺神经末端潜伏时延迟,正中神经、尺神经复合肌肉动作电位(CMAP)波幅降低,无运动神经传导阻滞;感觉神经传导均正常。针电极肌电图提示神经源性损害,异常肌肉主要分布在C7-T1节段支配区。5例患者颈椎中立位MRI可见生理曲度变直或脊髓稍变细;2例横断面可见LOA现象;5例屈曲位可见颈髓不同程度受压前移、硬膜外流空信号影,增强扫描可见硬膜外间隙内异常强化影。结论根据平山病的临床表现结合其特征性的肌电图、MRI特点,可对平山病做出早期诊断。

Objective Discuss the electromyography and imaging characteristics of Hirayama disease,and improve clinicians’awareness of the disease.Methods A retrospective analysis of clinical data of five cases of Hirayama disease patients in our hospital,analyze its clinical manifestations,EMG and imaging features.Results 5 patients were male,age 18 to 28 years old,(3 cases)unilateral,bilateral involvement in two cases of asymmetry.Five patients showed the hand and forearm muscle weakness with atrophy,forearm ramp-like changes,with cold paralysis.Motor nerve conduction delay mainly ulnar nerve terminal during latent,median nerve,ulnar nerve compound muscle action potential(the CMAP)amplitude decreased,no motor nerve conduction block;sensory nerve conduction were normal.Needle electrode EMG neurogenic damage,abnormal muscle mainly in the C7-T1 segment dominated area.MRI in the neutral position of the cervical spine in 5 patients showed that the physiological curvature was straightened or the spinal cord was slightly thinned;2 cases showed LOA phenomenon in the cross section;5 cases showed different degrees of compression of the cervical spinal cord forward and the epidural space signal shadow in the flexion position,enhanced scanning Abnormal enhancement in the epidural space can be seen.Conclusion According to the clinical manifestations of Hirayama disease combined with its characteristic electromyography and MRI features,early diagnosis of Hirayama disease can be made.