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目的提高临床医生对平山病的认识,减少误诊。方法收集和分析3例平山病患者的临床资料,总结平山病的临床特点、神经电生理改变磁共振成像(MRI)表现、诊断和治疗方法。结果本组3病例均为青少年起病,男性2例,女性1例。临床均表现为右侧上肢远端肌无力、肌萎缩,伴寒冷麻痹、震颤。电生理表现为运动、感觉神经传导速度正常,病变侧颈7至胸1支配区肌肉呈神经源性损害。颈椎MRI表现为生理曲度变直,下段颈髓萎缩,髓内异常信号。结论凡遇青少年出现上肢远端不对称肌无力、肌萎缩时,应行肌电图、颈髓MRI检查排除平山病。
Objective To improve clinicians understanding of Pingshan disease and reduce misdiagnosis. Methods The clinical data of 3 patients with Pingshan disease were collected and analyzed. The clinical features of Pingshan disease were summarized. The MRI findings, diagnosis and treatment of neuropsychological changes were summarized. Results The 3 cases of this group were adolescent onset, 2 males and 1 female. Clinical manifestations of right upper extremity muscle weakness, muscle atrophy, with cold paralysis, tremor. Electrophysiological manifestations of exercise, sensory nerve conduction velocity is normal, the lesion side of the neck 7 to the chest 1 dominated zone neurogenic muscle damage. Cervical MRI manifestations of physiological curvature straight, the lower cervical spinal cord atrophy, intramedullary abnormalities signal. Conclusion Where young adolescents appeared distal asymmetric muscle weakness, muscle atrophy, electromyography should be performed, cervical spinal cord MRI exclusion Pingdingshan.