目的 探讨急性脱髓鞘脑病的临床表现和诊治特点.方法 对26例急性脱髓鞘脑病患者一般临床资料、脑脊液、脑电图及影像学检查结果 进行分析,给予标准剂量激素(地塞米松10 mg·d-1),或大剂量甲基泼尼松龙静脉冲击治疗(20~30 mg·kg-1·d-1),联合丙种球蛋白(400 mg··-1)和神经营养药物治疗并观察疗效.结果 14例患者脑脊液异常,所有患者脑电图均异常,MRI检查脱髓鞘病灶呈T1低信号或等信号、T2高信号改变、形状多不规则.总有效率88.5%,治疗结果 好转23例,无效3例.结论 急性脱髓鞘脑病临床表现多样,影像学有助于明确诊断,EEG有助于评价疗效,激素和丙种球蛋白联用疗效较好.%Objective To evaluate the clinical fealures and diagnostic characleristics of palienls with acute demyelinaling encephalopathy( ADE). Methods Totally 26 ADE patients were enrolled in the study. Hormone therapy, standard -dose dexamelhasone (10 mg · d-1) or high -dose melhylprednisolone injection ( 20~ 30 mg · kg-1 · d-1 ) , combined wilh immunoglobulin (400 mg · kg-1 · d-1 ) and neurolrophic drugs were given Lo these palienls. Clinical materials, cerebrospinal fluid (CSF) , electroencephalogram (EEG) , radiological oulcomes were analyzed and the effectiveness of trealmenl were evaluated. Results CSF abnormalities were found in fourteen patients and abnormal waves in electroencephalogram were found in all 26 patients. Magnetic resonance imaging (MRI) showed low or equal intensity signal on T1 -weighted images and high intensity signal on T2 -weighted images, with irregular shape. The effective rate was 88. 5% , 23 patients improved while 3 patient showed no effects al all. Conclusion The clinical features of ADE patients are complex. Imaging examination could be helpful in diagnosis of ADE and EEG results could be helpful to assess the effectiveness of treatment. Hormone therapy combined with immunoglobulin has a satisfactory effect.
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