72例儿童急性播散性脑脊髓炎临床特征及治疗转归

Acute disseminated encephalomyelitis in 72 children: clinical characteristics,treatment and outcome

目的:探讨儿童急性播散性脑脊髓炎(ADEM)临床特征及治疗转归。方法:收集我院2013年1月至2016年12月确诊为ADEM的患儿72例,回顾性分析其临床表现、辅助检查、治疗转归的特点。结果:72例ADEM患儿平均年龄3. 86岁,3岁以内发病者并不少见,四季均有发病,病前1～2个月内有感染及疫苗接种史的共45例(62. 5%)。主要临床表现为脑病症状、颅神经受累、肢体运动障碍、共济失调、脑膜刺激征、锥体束征等。辅助检查:脑脊液检查异常率62. 4%,头颅CT异常率46. 0%,头颅MRI异常率95. 8%,脊髓MRI异常率46. 9%。治疗:63例给予甲基强的松龙冲击治疗,59例给予丙种球蛋白治疗,17例继发癫痫患者给予抗癫痫治疗及抗病毒、降颅压、营养神经等治疗。转归:出院时56. 0%患者痊愈,随访6～48个月,85. 94%患者恢复良好,2例复发,6例遗留后遗症。结论:ADEM多有前驱史,MRI常为多发异常信号,主要治疗为激素及丙种球蛋白,激素停药宜慢,积极治疗大多预后较好,需长期随访,警惕复发或转变为MS。

Objective： To investigate the clinical characteristics, treatment and outcome of acute disseminated encephalomyelitis（ ADEM ） in children.. Methods ：72 children confirmed as ADEM in our hospital fl＇om Janual-y-2013 to December 2016 were collected and their characteristics of clinical manifestations, auxiliary examinations, treatment and outcome were retrospectively analyzed. Results ：The average age of 72 children with ADEM was 3.86 years old. The incidence of ADEM was not uncommon in children under 3 years old. The incidence of ADEM was found in all seasons. There were 45 cases （62. 5% ） with infection and vaccination history within 1-2 months before the onset of ADEM. The main clinical manifestations were encephalopathy, cranial nel-ve involvement, limb dyskinesia, ataxia, meningeal irritation, pyramidal tract sign, etc.. Auxiliary examinations showed the abnormal rates of cerebrospinal fluid examination, cranial CT, cranial MRI and spinal MRI were 62.4% , 46.0% , 95.8% and 46. 9%, respectively. 63 cases were treated with methylprednisolone pulse therapy; 59 cases were treated with gamma globulin therapy; 17 cases with secondary epilepsy received antiepileptic therapy and other therapies such as antiviral treatment, reducing intracranial pressure, neurotrophic therapy. 56. 0% of the patients were cured at discharge. Follow-up for 6-48 months showed that 85.94% of the patients recovered well, 2 cases relapsed and 6 cases remained sequelae. Conclusion： Most ADEM has a predecessor history; MRI often demonstrates multiple abnormal signals; using hormones and gamma globulin are the main treatment for ADEM, and hormone withdrawal should be slow. Although active treatment can mostly achieve good prognosis, ADEM need long-term follow-up in order to be alert for its recmxence or change to MS.