中文摘要：

目的 分析44例急性播散性脑脊髓炎（ADEM）患者的临床特征.方法 收集2009年8月至2014年7月在中山大学附属第三医院诊断为ADEM患者的临床资料,并进行回顾性分析.结果 44例ADEM纳入分析：男23例,女21例;儿童期发病9例,少年期11例,成人期24例.临床病程分为单相型（23/44,52％）或多时相型（21/44,48％）.14例（31.8％）发病前2周内有明确诱因;最常见临床症状包括发热（20/44,45％）、精神症状（18/44,41％）、意识障碍（17/44,39％）、抽搐（12/44,27％）;首次发病EDSS评分（4.3±1.3）分,mRS评分（2.7±0.9）分.10例出现单一或多种自身免疫抗体指标异常.44例中血清NMO-IgG阳性2例,脑脊液寡克隆区带阳性3例.头颅MRI最常见表现为散在型（18/44,41％）、基底节型（12/44,27％）、巨块型（10/44,23％）,满天星型（3/44,7％）及出血型（1/44,2％）相对少见.糖皮质激素（40/44,90.9％）和丙种球蛋白（13/44,29.5％）是首次发病的常用治疗药物.29例（65.9％）完成了规律随访,随访时间（4.2±2.3）年,其中单时相型10例,多时相型19例;多时相型患者首次复发时间（2.5±2.3）年,总复发次数（3.3±1.4）次;最后一次复发平均EDSS评分（3.9±2.2）分,mRS评分（2.2±1.3）分.MRI随访时间（5.3±1.9）年,其中18例（62.1％）部分缓解,6例（20.7％）病灶持续存在,5例（17.2％）出现新发病灶.19例多时相型患者中13例规律治疗,激素（13/13,100.0％）和丙种球蛋白（7/13,53.8％）仍是复发急性期的主要药物.缓解期,多时相型患者多选择小剂量激素口服治疗预防复发（12/13,92.3％）,并联合使用免疫抑制剂如硫唑嘌呤、他克莫司、环孢素、利妥昔单抗.结论 ADEM成人起病并非少见,可以为多时相病程,全脑症状明显,MRI表现常见为散在型及基底节型,随访后部分缓解.由于ADEM临床表现多样,并可转化为MS及NMO,因此ADEM可能作为一组“临床综合?

英文摘要：

Objective To investigate the clinical features in 44 patients with acute disseminated encephalomyelitis （ADEM）.Methods Consecutive ADEM patients admitted to Neurology Department of the Third Affiliated Hospital of Sun yat-sen University during August 2009 to July 2014 were enrolled.Clinical and laboratory data of the patients were reviewed and analyzed.Results Forty-four patients with ADEM based on the 2012 criteria were recruited,including 23 male and 21 female;9 children,11 teenagers and 24 adults.There were 23 monophasic ADEM （23/44,52%） and 21 multiphasic ADEM （21/44,48%）.Fourteen patients （31.8%） had definite incentive factors within 2 weeks preceding the disease onset.The commonest presenting symptoms were fever （20/44,45%）,mental disorder （18/44,41%）,disturbance of consciousness （17/44,39%） and seizure （12/44,27%）.The average EDSS score was （4.3 ± 1.3）,and the average mRS score was （2.7 ± 0.9）.Abnormal autoimmune antibodies were detected in 10 patients.Two patients were positive for NMO-IgG,and three patients were positive for oligoclonal bands.On MRI scanning,small lesions were observed in 18 of 44 patients （18/44,41%）;large confluent white matter lesions in 10 patients （10/44,23%）;symmetric bithalamic involvement in 12 patients （12/44,27%）.Patients were mainly treated with intravenous corticosteroids （40/44,90.9%）and immunoglobulin G （13/44,29.5%） in acute phase.Regular follow-up performed in 29 patients （65.9%）,and the average follow-up time was （4.2±2.3） year.A monophasic course was found in 10 patients,and multiphasic course in 19 patients.After （2.5 ±2.3） years,patients with multiphasic ADEM experienced their first clinical relapse,and the relapse frequency was （3.3 ± 1.4）.The average EDSS score was （3.9 ±2.2）,and the mRS score was （2.2 ± 1.3） in their latest relapse.In follow-up MRI for （5.3 ±1.9） years,lesions in 18 patients （62.1%） were partially ameliorated,while 6 patients （20.7%） persisted,an