平山病2例报告并文献复习

Hirayama disease: report of two cases

目的探讨平山病的颈髓磁共振(MRI)表现及其诊断价值。方法对2例平山病患者的临床、神经电生理、MRI影像学资料进行分析,并复习相关文献,总结平山病的颈髓MRI特点。结果 2例均为青年男性,主要表现为局限于手和前臂肌萎缩。肌电图均示神经源性损害。自然位MRI扫描,2例均有C4～C7段脊髓轻度萎缩,前后径变短。屈颈位MRI平扫2例均可见下段颈髓前移、变扁平加剧,硬脊膜外间隙增宽,其内可见蚓状异常血管流空信号。增强MRI2例均可见增宽的硬膜外间隙内异常增粗并明显强化的静脉丛。结论平山病的MRI表现有一定的特征性,屈颈位平扫及增强MRI对平山病的诊断有重要的价值。

Objective To analyze the findings and diagnostic values of hirayama disease on magnetic resonance imaging （MRI） . Methods We summarized the feature of MRI in neutral neck position and a fully flexed neck position of hirayama disease by collected the clinical data, nerve Electrophysiology data, findings of Hirayama disease on MRI of 2 patients and also by reviewed the correlation literatures. Results Both two patients were young men, The amyotrophy of forearm and hand was the main clinical symptoms. The findings of electromyogram were both neurogenic impairment. At the neck neu- tral position, the slightly atrophic flatten cervical cord was shown at the lower segment of cervical cord （C4 -C7） in 2 pa- tients. The forwords movement of the lower segment of the flatten spinal cord at same level was demonstrated on MRI in 2 patients at the neck flexion position. Both of them were found the increased width of the posterior epidural space. On the postcontrast scan, abnormal enhanced venous plexus within the increased width posterior epidural space was demonstrated at the flexion position in 2 patients. Conclusion Pre- and post- contrast MRI at neck flexion position could provide some characteristic manifestations in the patient with hirayama disease.