目的探讨急性播散性脑脊髓炎(ADEM)的临床特征、影像学表现及与临床的关系。方法分析2006年1月-2009年8月94例ADEM患儿的流行病学、临床表现、实验室检查、影像学特点、治疗及预后转归情况。将患儿分为昏迷组和无昏迷组,分别统计2组的病变部位及例数,采用2χ检验比较2组病变部位之间有无统计学差异。结果患儿发病年龄1岁1个月~16岁,平均7.0岁。男53例,女41例,脑病是突出症状,发生率为87.2%。重症出现昏迷,从发病至昏迷平均3.1 d。主要症状有发热、头痛/呕吐、抽搐、肢体瘫痪、颅神经麻痹、视力受累等。常出现锥体束征和脑膜刺激征。61例行脑脊液检查,其中34例(55.7%)脑脊液细胞数升高[(10~385)×106L-1)],主要为淋巴细胞升高。颅内多个部位可受累,其中受累频率高者有多个脑叶、双侧背侧丘脑、双侧基底核、侧脑室旁、中脑、脑桥及小脑。昏迷组双侧丘脑受累的发生率为56.5%,无昏迷组为25.4%,差异有统计学意义(2χ=7.152,P<0.01)。其他部位受累的发生率2组比较差异无统计学意义。绝大部分患者(88例)接受甲泼尼龙冲击治疗及口服泼尼松维持治疗,自起病至接受治疗平均15.1 d。66%的患儿出院时恢复较好,行走无需帮助。结论儿童ADEM男童略多于女童,临床特征复杂多样,病情轻重不一;颅脑MR I检查有助于及时明确诊断;双侧丘脑同时受累者常出现昏迷。及时恰当的治疗对预后极为重要。 Objective To investigate the clinical features, imaging findings and clinical features of acute disseminated encephalomyelitis (ADEM). Methods The epidemiology, clinical manifestations, laboratory tests, imaging features, treatment and prognosis of 94 ADEM children from January 2006 to August 2009 were analyzed. The children were divided into coma group and non-coma group, respectively, the two groups of lesions and cases were counted, 2χ test was used to compare the two groups of lesions with or without statistical differences. Results The incidence of children aged 1 year 1 month to 16 years old, with an average of 7.0 years old. 53 males and 41 females, encephalopathy is a prominent symptom, the incidence was 87.2%. Severe coma, from onset to coma average 3.1 d. The main symptoms are fever, headache / vomiting, convulsions, limb paralysis, cranial nerve palsy, visual acuity involvement. Cone beam signs and meningeal irritation often appear. Cerebrospinal fluid was detected in 61 patients, of which 34 (55.7%) had an elevated number of cerebrospinal fluid [(10 ~ 385 × 106L-1)], mainly lymphocytosis. Multiple parts of the brain may be involved, of which the higher frequency of involvement of multiple lobes, dorsal thalamus, bilateral basal nuclei, lateral ventricle, midbrain, pons and cerebellum. The incidence of bilateral thalamic involvement was 56.5% in the coma group and 25.4% in the non-coma group, with a significant difference (2χ = 7.152, P <0.01). The incidence of other parts of the two groups was no significant difference. The vast majority of patients (88 patients) were treated with methylprednisolone and oral prednisone, with an average of 15.1 days from onset to treatment. 66% of children recovered better at discharge, walking without help. Conclusions There are more children with ADEM than girls. The clinical features are complex and diverse, and the severity of the disease is different. MR I test of brain can help diagnose in time and the patients with bilateral thalamus often coma. Proper and timely treatment of the prognosis is extremely important.