儿童急性播散性脑脊髓炎24例临床分析

Clinical analysis of 24 children with acute disseminated encephalomyelitis

目的研究儿童急性播散性脑脊髓炎(ADEM)的临床和实验室特点,以提高诊治水平。方法对1998年5月—2008年12月收治的24例ADEM患儿的临床特点、实验室检查结果、治疗方案及预后进行回顾性分析及随访研究。结果 14例有前驱感染病史,2例有疫苗接种史。神经系统症状多样。15/24例(62.5%)脑脊液常规、生化异常。20例行脑脊液寡克隆带及免疫指数检查,其中13例(65%)鞘内IgG合成增加、免疫球蛋白指数增高0.84～2.5;8例(40%)脑脊液寡克隆带(+)。22例行头颅MRI检查,20例(90.91%)异常,以白质病变为主,呈多灶性。3例头颅CT未发现异常而MRI示阳性结果。24例均行脑电图检查,20例(83.33%)异常,其中5例EEG见到棘慢波。24例均予激素治疗,14例同时合用大剂量人血丙种球蛋白,疗效显著,所有患儿均好转。经6个月～9年随访,2例确诊为多发性硬化,15例无复发,7例失随防。结论 ADEM是中枢神经系统常见的急性脱髓鞘性疾病,应及早诊治,脑脊液、MRI检查有助于早期诊断,糖皮质激素及大剂量丙种球蛋白有一定疗效,预后良好。

Objective To identify the clinical and laboratory characteristics of acute disseminated encephalomyelitis（ADEM） in children and improve its diagnosis and treatment.Methods A retrospective and followup study was conducted in 24 children with ADEM to summarize the characteristics of clinical features,laboratory results,treatment and prognosis.Results Prodromal infection and vaccination were found in 14 ADEM patients（62.5%）.Neurological symptoms were various.Results of cereospinal fluid（CSF） routine examination were abnormal in 15 cases（62.5%）.IgG index was performed in 20 cases and increased in 13 cases（65%）.Oligoclonal bands in CSF were positive in 8 cases（40%）.Twenty（20） of 22 cases（90.91%） had abnormal magnetic resonance image and 3 cases with normal computerized tomographic scans.Electroencephalogram was abnormal in 20 of 24 cases（83.33%）.All patients were treated with corticosteroid and 14 who received additional intravenous immunoglobulin had a better result.Twelve cases were followed-up from 6 months to 9 years,with multiple sclerosis in 2 cases and no relapse in 10 cases.Conclusions ADEM was an inflammatory demyelinating disease of the central nervous system.CSF and magnetic resonance image examination were helpful to establish the diagnosis.Good prognosis can be obtained with treatment of steroid and high dose immunoglobulin.