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目的探讨平山病的临床特征及影像学特点。方法回顾性分析我院近年来诊断的2例平山病病例的临床表现、肌电图及磁共振成像(MRI)影像资料,并复习相关文献。结果 2例均为青少年男性,呈单侧上肢远端无力伴萎缩,1例患者累及另一侧,尺侧肌萎缩明显,上肢呈斜坡样改变,无感觉障碍和锥体束征,肌电图检查显示神经源性改变。屈颈MRI检查均可见下颈髓前移、硬脊膜外间隙增宽,1例患者颈5-6水平脊髓内可见T2高信号。结论平山病临床少见且易误诊,当青少年出现上肢远端不对称肌无力伴肌萎缩时,需进一步行肌电图和屈颈位MRI检查以提高平山病的确诊率。
Objective To explore the clinical features and imaging features of Pingshan disease. Methods The clinical manifestations, electromyography and magnetic resonance imaging (MRI) images of 2 cases of piedmont disease diagnosed in our hospital were retrospectively reviewed. The related literatures were reviewed. Results Both of the two cases were adolescent males. The unilateral upper extremity was with weakness accompanied with atrophy. One patient involved the other side, the ulnar muscle showed obvious atrophy, the upper extremity had a slope-like change, no sensory disturbances and pyramidal signs, Check shows neurogenic changes. Fetal cervical MRI examination showed lower cervical spinal cord adduction, epidural space widened, 1 case of cervical 5-6 spinal cord visible high signal. Conclusions Pingshan disease is rare and misdiagnosed. When the adolescent appears the distal asymmetric muscle weakness accompanied by muscle atrophy, further examination by electromyography and kyphosis should be performed to improve the diagnosis of Pingshan disease.