平山病8例肌电图与影像特征分析

Electromyologram and neuroradiological characteristic analysis of Hirayama disease

目的探讨平山病临床表现,肌电图及影像学特点。方法对温州医学院附属第一医院2003年6月至2009年6月间确诊的8例平山病患者的临床表现,肌电图及影像学资料进行回顾性分析。结果 8例患者双侧上肢远端肌肉均呈神经源性损害,提示受损节段多在下颈髓前角细胞。8例颈部自然位MRI发现低位颈髓萎缩,屈曲位均发现颈髓前移,硬脊膜后壁前移,硬脊膜外间隙增宽。增强扫描中均可见不同程度的硬膜后间隙内异常强化影。结论根据平山病临床电生理及影像学检查显示平山病可能为下颈段脊髓病变,神经电生理检查与屈颈位MRI在平山病的诊断中有重要作用。

Objective To investigate the clinical manifestations, Eleetromyologram and neuroradiological characteristic of Hirayama Disease. Methods The clinic,electromyoogram information, and neutral neck position and neck flexion MR Iimaging examination were collected from 8 patients final diagnosed as Hirayama Disease between June 2003 to June 2009 in our hospital,They were contrasted and analyzed retrospectively. Results EMG indicated that the impairment of spinal anterior cells were limited to the arm relevant segments and both sides were involved. 8 cases in the natural position MRI scan, the spinal cords were mild atrophy. During neck flexion, the spinal cord was displaced forward and flattened. In the contrast MRI scan, all showed different degrees enhancement in the back of epidural cavity. Conclusion Our data suggested that Hirayama disease may be a special type of cervicalmyel opathy. The neuroelectrophysiological check and the neck flexion MRI check are valuable to the clinical diagnosis and differential diagnosis.