平山病误诊为运动神经元病1例分析并文献复习  

Hirayama Disease Misdiagnosed as Motor Neuron Disease:A Case Report and Literature Review

作　　者：王峥[1] 陈伟贤[1] 曾彦英[1] 段丽敏[1] 

机构地区：[1]南京医科大学第一附属医院老年医学科,江苏南京210029

出　　处：《中国误诊学杂志》2010年第19期4543-4545,共3页Chinese Journal of Misdiagnostics

摘　　要：目的分析少见的平山病(Hirayama disease,HD),就其临床、影像学特点及发病机制进行探讨,提高对该病的认识。方法采用回顾行研究方法,报告1例曾误诊为运动神经元病的平山病的临床表现及检查资料。结果该病例为男性,青春期发病,呈单侧上肢远端无力伴萎缩;肌电图为神经源性改变,屈颈MRI可见下段颈髓前移、硬脊膜向前移位,硬膜外间隙增宽。结论根据平山病的临床及屈颈位MRI表现的特点,可以提高确诊率。Objective To analyze the clinical and radiological features,pathogenetic mechanisms of Hirayama disease and to improve our understanding of the disease.Methods One case with Hirayama disease which had been misdiagnosed was analyzed retrospectively.Results The patient was male,onset at adolescence,with muscle weakness and atrophy on one upper limb;The EMG showed neurogenic abnormalities.Cervical cord MRI in neck flexion position found that the spinal cord was displaced forward and flattened from the C5 to T1 vertebral levels.Conclusion The diagnosis of HD can be made on the basis of detailed clinical manifestations and MRI features in neck flexion position.

关 键 词：肌萎缩/诊断 误诊 病例报告 人类 

分 类 号：R746.4[医药卫生—神经病学与精神病学]