急性播散性脑脊髓炎68例临床特征分析及预后研究

The clinical features and neurologic outcome of acute disseminated encephalomyelitis in children

目的探讨儿童急性播散性脑脊髓炎(ADEM)的临床特征与预后。方法分析2001年2月至2007年3月68例复旦大学附属儿科医院ADEM患儿住院病史资料及近远期疗效随访。结果发病年龄1岁至15岁4个月,中位数6.5岁;住院患儿逐年增加。有前驱感染史者占57.4%;预防接种史者占4.4%;38.2%无明确病因。临床分型:脑型42例(61.8%),脑脊髓型18例(26.4%),脊髓型8例(11.8%);主要症状表现发热、瘫痪、惊厥、精神异常、锥体外系症状、意识障碍、认知障碍、括约肌障碍、共济失调等,部分患儿出现语言功能障碍、颅神经麻痹。脑脊液免疫指标检查57例,44例(77.2%)阳性[IgG指数增加和(或)寡克隆带阳性]。皮质激素近期疗效:起效时间55.9%≤3d、26.5%≤7d、17.6%>1周;好转时间48.5%≤1周;8例自然缓解,死亡1例;远期疗效:病程≥148例;病程<1年20例。随访结果:痊愈38例;后遗症9例(病程1年以上尚未恢复者);7例复发;随访中8例;失访5例。结论儿童ADEM多发生于3～12岁,临床特征复杂多样,病情轻重和病程长短不一;临床有多灶脑病的表现时应重视颅脑MRI检查以早期发现本病;多数远期预后良好,部分严重者完全恢复时间超过6个月,少数患儿留有神经系统后遗症。

Objective To describe the clinical features and neurologic outcome of acute disseminated encephalomyelitis （ADEM） in children.Methods A study on medical record and follow-up information of prospective efficacy was conducted in 68 children with ADEM. Results （ 1 ）The age of patients at onset ranged from 1 to 15.3 years, with a median of 6.5 years, with the inpatients numbers increasing year by year. （2）An infectious event preceded the onset of illness in 57.4% of patients, and a vaccination event in 4.4% of patients, while the others were cryptogenic. （3） Clinical classification： 42 （61.8% ）cases had presentations of the brain, 18 （26.4%）of the brain and spinal cord, 8 （11.8% ）of the spinal cord; the most common presenting symptoms were fever, palsy, seizures, changes in mental status, extrapyramidal syndrome, consciousness impairment, cognitive handicap, sphincter muscle disturbance and ataxia and some patients appeared linguistic functional disturbance and cranial palsy. （4）Increased IgG index and/or oligoclonal band positive were present in the cerebrospinal fluid of 77.2% of cases. （5） prognosis： ①near-future curative effect of corticosteroid treatment： 55.9% of cases showed initiation effective in 3 days, 26.5% in 7 days, 17.6% after 1 week; 48.5% of cases improved in 1 week, and 11.2% improved spontaneously , while one case died. ②prospective efficacy ： 48 cases whose course was longer than one year and 20 cases less than one year course. At the end of follow-up： 38 cases recovered; 9 cases had disability（whose course longer than one year） ; relapse was seen in 7 cases; 8 cases were in follow-up;5 cases lost follow-up. Conclusion ADEM in childhood often occur at the age of 3 to 12 years. The presenting symptoms are various and complicated ; the states of the illness vary from slight to severe and the course varies from short to long. Neuroimaging （especially brain MRI） is extremely important in early diagnosis of ADEM when a patient presenting a polysymptomatic encephalopathy. Most children with ADEM present a good outcome in long-term follow-up; some patients with severe condition recover after 6 months, and a few have disability of nervous system.