11例平山病临床特征分析

Analysis of clinical features of Hirayama Disease:a review of eleven cases

目的探讨平山病的临床特征,为早期诊断和治疗提供参考。方法对11例确诊的平山病患者的临床表现、神经电生理特征及影像学资料进行回顾性分析。结果平均发病年龄17.0岁,主要表现为局限于手和前臂的肌萎缩,尺侧肌肉萎缩较重,上肢呈斜坡样;肌电图显示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;屈颈MRI均见下段颈髓前移、变平,硬脊膜向前移位,硬脊膜外间隙增宽,硬脊膜外间隙内半月形信号影。结论根据平山病临床神经电生理学及影像学检查显示平山病可能为下颈段脊髓病变,神经电生理及影像学检查有助于其与运动神经元病相鉴别。

Objective To study the clinical characteristic of Hirayama disease as to offering evidences of early diagnosis and treatment. Methods The clinical data, neuronelectrophysiological features and MRI features of 11 patients diagnosed by the criteria of Hirayama Disease were retrospectively analyzed. Results The mean age at onset was 17.0 years old. Hirayama disease occurs predominantly in young males in puberty and characterized by muscular atrophy in the hand and forearm; the brachioradialis is spared （oblique amyotrophy）. EMG indicated that the impairment of spinal anterior cells were limited to the arm relevant segments and both sides were involved. During neck flexion, the spinal cord was displaced forward and flattened. A crescent-shaped epidural mass was noted behind the spinal cord. Conclusion Our data suggested that Hirayama disease may be a special type of cervical myelopathy. The neuroelectrophysiological check and the neck flexion MRI check are valuable to the clinical diagnosis and differential diagnosis.