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目的探讨平山病的临床特征,提高对平山病的诊断和治疗水平。方法回顾性分析自2011年1月~2014年6月徐州医学院附属医院就诊的11例平山病患者的临床资料,同时对相关文献进行回顾分析。结果本组患者中男性10人,女性1人,平均发病年龄为19.5岁(16~23岁),所有患者均表现为上肢远端的肌无力和肌萎缩,5例患者为单侧上肢受累(右上肢4例,左上肢1例),6例患者为双上肢受累,9例患者受累手指出现震颤,2例患者出现肌束颤动。肌电图示所有患者均呈慢性神经源性改变。颈椎MRI自然位9例患者出现脊髓萎缩,所有11例患者均出现颈椎生理曲度变直以及颈髓非对称性扁平,2例患者低位颈髓髓内出现高信号。颈椎MRI屈颈位显示10例患者出现背侧硬脊膜前移、硬脊膜外间隙增宽,9例患者硬膜外出现流空信号,所有11例患者硬膜外均出现新月形信号影,8例患者佩戴颈托,其中6例患者获益。结论平山病罕见,容易误诊,通过患者的临床表现、神经电生理检查、神经影像学检查(屈颈位MRI)可以确诊。平山病是自限性疾病,颈托通过限制颈椎前屈可能阻止疾病的进展,因此早期诊断十分重要。
Objective To explore the clinical features of Pingshan disease and to improve the diagnosis and treatment of Pingshan disease. Methods The clinical data of 11 patients with Pingshan disease treated in Xuzhou Medical College Affiliated Hospital from January 2011 to June 2014 were analyzed retrospectively. The related literatures were retrospectively analyzed. Results The group of 10 males and 1 females, the average age of onset was 19.5 years (16 to 23 years), all patients showed muscle weakness and muscle atrophy in the distal upper limbs, 5 patients were unilateral upper limb involvement Right upper limb in 4 cases, left upper limb in 1 case), 6 patients had double upper extremity involvement, 9 patients had tremor in their fingers, and 2 patients had fibrillation. EMG showed that all patients showed chronic neurogenic changes. Spinal cord atrophy occurred in 9 patients with natural cervical spine MRI. All 11 patients presented with cervical spondylolisthesis straightened and asymmetric flat cervical spinal cord. Two patients showed low signal in cervical spinal cord. Cervical MRI showed that 10 cases showed dorsal dura mater anterior extension and widened epidural space, 9 cases had epidural flow-empty signal, and all 11 cases showed epidural crescent signal Shadow, 8 patients wearing cervical brace, of which 6 patients benefit. Conclusion Pingshan disease is rare and easily misdiagnosed. It can be confirmed by the clinical manifestations, neuroelectrophysiology, neuroimaging (MRI). Pingshan disease is a self-limited disease, neck brace by limiting cervical flexion may prevent the progress of the disease, so early diagnosis is very important.