平山病的过屈位MRI 表现及其临床价值

目的：探讨平山病的过屈位颈椎MRI特征性影像学表现及其临床诊断价值。方法：总结分析经临床证实的5 例平山病患者的临床及MRI资料，并结合相关文献报道进行回顾性分析。所有患者均行常规生化检查，脑脊液检查，肌电图检查及肌肉活检。结果：5 例均为青少年男性，呈单侧上肢远端无力伴萎缩，其中1 例患者累及另一侧，尺侧肌萎缩明显，上肢呈斜坡样改变，均无感觉障碍和锥体束征；肌电图检查显示神经源性改变，提示受损节段多在下颈髓前角细胞。屈颈MRI检查均可见下颈髓前移、硬脊膜外间隙增宽，可见迂曲条状血管流空影。结论：平山病的过屈位MRI表现具有一定的特征性，对平山病的诊断具有重要价值。

Features and Diagnostic Value of Flexion Position MRI in theHirayama Disease

Objective:To investigate the clinical manifestation and diagnostic value of flexion position MRI in the Hirayama Disease.Methods:Retrospective analysis of 5 clinically proven Hirayama patients MRI data and combined with other reports. The routinebiochemical examination, cerebrospinal fluid examination, electromyogramand muscle biopsy were performed for all the cases.Results:Hirayama disease predominantly in young males in puberty, with muscle weakness and atrophy on one upper limb, and one case with theother side, the brachioradialis was spared (oblique amyotrophy), but without sensory disorder or pyramidal sign. EMG indicated that theimpairment of spinal anterior cells were limited to the arm relevant segments and both sides were involved. During neck flexion, thespinal cord was displaced forward and flattened, in which there were distorting or stripe flow void signals with enhancement.Conclusion:Flexion position MRI of Hirayama Disease had characteristic appearances that had significant values for the diagnosis of Hirayama disease.