平山病一例报道及文献复习

Hirayama Disease:A Case Report and Literatures Review

目的探讨平山病的临床特点。方法分析2010年11月收治的1例平山病患者的临床表现及辅助检查,并复习该病相关文献。结果该例患者为青年男性,主要症状为双手握力差,伴肌萎缩,手指伸展时可见肌束颤动。肌电图示双上肢大鱼际肌及第一骨间肌神经源性损害。脑MRI平扫未见异常。颈椎过屈位MRI显示颈5~颈7平面颈髓变细,椎管变窄,颈4-胸1平面脊髓后方硬膜外脂肪间隙增宽,增强见索样改变,斑片状强化及血管流空信号,符合平山病的诊断。结论当青少年出现上肢远端不对称肌无力伴肌萎缩时,诊断需考虑平山病,结合神经电生理检查和屈颈位MRI检查可明确诊断,佩戴颈托可能有助于延缓病情进展。

Objective To investigate the clinical features of Hirayama disease（HD）.Methods We analyzed the clinical manifestations and assistant examination results of one patient with HD diagnosed in November,2010.In addition,we reviewed the related literatures.Results The patient was a young man,and the main symptoms were bilateral hand weakness with muscular atrophy.The muscle bundles trembled when he stretched his fingers.The electromyography showed neurogenic damage of both the thenar muscles and the first interosseous muscles.Brain magnetic resonance imaging（MRI） showed normal;cervical spine flexion MRI showed thinning cervical spinal cord with spinal canal narrowing at C_5-C_7 plane.In addition,epidural fat gap widened at C_4-T_1 plane,and enhanced scan showed cord-like changes,patchy strengthening and blood flow void shadow.Conclusions When asymmetric distal upper limb muscle weakness with muscle atrophy appears in adolescents,HD should be considered.The combination of neuroelectrophysiological examination and cervical spine flexion MRI scan is helpful for diagnosis.Wearing cervical collar may slow disease progression.