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目的：探讨儿童急性脱髓鞘脑病的临床特征和诊治过程以提高确诊率。方法回顾性分析近5年收治的50例儿童急性脱髓鞘脑病的临床资料。结果50例患儿中以头痛、呕吐起病20例，以视觉障碍起病12例，以情绪障碍起病15例，全身无力、精神差起病8例，惊厥发作起病6例，行走困难、感觉障碍起病5例。经治疗急性期明显好转45例，无效5例、治疗中断8例。缓解再复发7例，死亡2例。结论儿童急性脱髓鞘脑病临床表现复杂多变，容易误诊漏诊。要及时诊断，尽早治疗、坚持用药。“,”Objective To discuss the clinic characteristics and diagnosis, treatment of the children with acute demyelinating encephalopathy, so as to increase diagnosis rate. Methods We reviewed retrospectively the clinic data of acute demyelinating encephalopathy in the 50 children, who were admitted to our hospital in the past ifve years. Results There were 20 cases of headache, vomiting onset. The onset of visual impairment in 12 cases and mood disorders in 15 cases occurred. General weakness, poor mental onset was in 8 cases. 6 cases of seizure on-set occurred. Dififcult walking, sensory disturbance onset were in 5 cases. 45 cases signiifcantly improved by the treatment, 5 cases were ineffective, 8 cases were of treatment interruption. 7 cases were ease recurrence, 2 cases died. Conclusion Clinical manifestations in children with acute demyelinating encephalopathy were complicated and easy to misdiagnose. Timely diagnosis, early treatment and being adhere to medication are of great impor-tance.