平山病的影像学及神经电生理的研究进展

平山病是一种散发的、青少年上肢远端肌萎缩,为良性自限性疾病,临床特征为隐袭起病的单侧前臂和手的肌肉无力和萎缩。自然位颈磁共振成像(MRI)可见低位颈髓萎缩变扁,屈颈位MRI显示颈髓前移、变扁,脊膜后可见月牙形略长T1和长T2异常信号及其内的流空信号,增强扫描强化明显。肌电图提示颈区肌肉神经源性损害。

Research Advance in Magnetic Resonance Imaging and Electroneurophysiology of Hirayama Disease

Hirayama disease is a benign self-limited juvenile muscular atrophy of the distal upper limbs.Its clinical feature presents as insidious onset,predominantly unilateral upper extremity weakness and atrophy.In natural position cervical MRI images show atrophy of the lower cord.In fully flexed neck position MRI images show forward displacement and flattening of the lower cervical cord,and a crescent-shaped slightly hypointense T1 and hyperintense T2 signal area with flow void signal behind the meninges.The crescent shaped area is enhanced significantly in enhanced MRI.EMG revealed neurogenic damage in muscles of cervical region.