急性播散性脑脊髓炎患儿的临床特征及复发因素

Clinical Feature and Prognostic Factors for Relapse of Acute Disseminated Encephalomyelitis in Children

目的探讨儿童急性播散性脑脊髓炎（ADEM）的临床特征及复发因素。方法 2003年2月-2009年12月,本院神经内科共收治符合ADEM诊断标准并完成随访的48例患儿,分析其临床资料、实验室检查、神经影像学特征及随访结果,总结其临床特征及复发因素。结果 48例ADEM患儿中,男22例,女26例;发病年龄1岁1个月~12岁6个月,中位年龄6.3岁。发病前驱期有感染症状31例（占65%）,疫苗接种史4例（占8%）,无明显诱因13例（占27%）。临床主要以运动障碍（46例）、精神障碍（21例）、头痛头晕（21例）及意识障碍（15例）为主要表现。MRI以皮质下白质、半卵圆中心、侧脑室周围、基底核、脑干及脊髓损害为主,呈点片状或稍长T1、长T2信号。8例为第2次复发或多次复发。其复发与视神经症状、头颅MRI上显示幕上和幕下病灶同时存在、脊髓病灶及视觉诱发电位异常有关。随访结果：34例完全缓解,无任何神经系统后遗症;8例复发;14例遗留神经功能缺损,主要以运动障碍为主。结论儿童ADEM临床表现多样,视神经损害及磁共振成像显示病灶的部位对复发的判断有一定的临床价值。ADEM长期预后大多良好,少数患儿遗留神经功能缺损。

Objective To explore the clinical feature and prognostic factors for relapse of acute disseminated encephalomyelitis（ADEM） in children.Methods Forty-eight patients with ADEM in the Department of Neurology of Anhui Provincial Children′s Hospital from Feb.2003 to Dec.2009 were selected,their clinical data,laboratory examination and neuroimaging character were analyzed,and the cli-nical feature and prognostic factors for relapse were summarized.Results All the 48 cases included 22 boys and 26 girls,the onset age was from 1 year and 1 month to 12 years and 6 months,median age was 6.3 years old.Among the 48 ADEM children,31 cases（65%） showed infection symptoms in the prodromal period,4 cases（8%） had received vaccination and 13 cases（27%） had no apparent cause.The clinical manifestations included movement disorders（46 cases）,mental disorders（21 cases）,headache and dizziness（21 cases）,and consciousness disorders（15 cases）.MRI presents mainly was subcortical white lesions,centrum ovale majus,basal ganglia,lateral periventricular area,the brain stem and spinal cord,revealing punctiform and lamellar enhancing T1 lesion or T2 lesions.Eight cases in the 48 patients relapsed or repeated relapsed.The optic nerve abnormal appearance,simultaneous supratentorial and infratentorial lesions under MRI,spinal cord lesion and abnormal vision-evoked potential were significantly related to the relapse.The long-term data showed that 34 cases reached complete remission with no neurologic sequela,while 8 cases relapsed,and 14 cases remained neural function defects,it mainly was dyskinesia.Conclusions ADEM in children may show varied clinical manifestations.Optic nerve lesion and the location of lesion under MRI may keep a certain degree of diagnostic value on the relapse.Most ADEM cases have benign prognosis,while a small number of the patients remain neurologic impairment.