MRI诊断平山病1例并文献复习

The MRI Features of One Case of Hirayama Disease and Literature Review

目的分析平山病磁共振成像(MRI)特点及本病临床表现,复习相关文献,加深对该病的认识.方法回顾分析经昆明医科大学第一附属医院临床证实的1例平山病患者的病历及MRI影像资料,探讨平山病MRI特征性表现,影像学解剖基础与该病的发病机制.结果平山病发病以青年男性多见,临床表现为一侧上肢远端肌无力及肌萎缩.受累下段颈髓局限受压变细,脊膜囊后壁前移,脊膜后新月型长T2信号及其内流空信号影,增强扫描硬膜外间隙明显强化为典型MRI影像学表现.颈椎过曲功能位MRI扫描技术能更好显示其特征表现.本病例与相关文献相吻合.结论 MRI常规位和屈颈功能位检查结合患者临床资料可以对平山病作出诊断.

Objective Through analyzing Hirayama disease＇s MRI characteristics and reviewing its literature, to enhance its diagnosis level. Methods One case of Hirayama disease which had been clinically confirmed was reported, and the literatures were reviewed. The MRI findings, the related pathogenesis and the pathological anatomy foundation were discussed. Result By summarizing the relevant literatures, we found that Hirayama disease always occurred in young men. Progressive muscle weakness and wasting were characteristic clinical manifestations and occurred predominantly in the distal part of the right upper limb. In natural position cervical MRI images showed atrophy of the lower cord. In fully flexed neck position MRI images showed forward displacement and flattening of the lower cervical cord, and a crescent-shaped slightly hyperintense T2 signal area with flow void signal behind the meninges. The crescent shaped area was enhanced significantly in enhanced MRI. Conclusion Combining clinic data with the conventional position and the function position of the neck condition on MRI scan can make diagnosis of Hirayama disease.