急性播散性脑脊髓炎12例临床研究

Clinical study of acute disseminated encephalomyelitis in 12 children

目的 研究急性播散性脑脊髓炎 (ADEM )的临床特点 ,以提高其诊治水平。方法 对 12例临床初诊为ADEM患儿进行回顾及随访研究 ,并对临床特点、治疗方案及预后进行分析。结果 1.临床表现 :75 %病前有前驱感染或疫苗接种史。 83%为单相性 ,17%为多相性病程。神经系统症状多样。 2 .辅助检查 :18%脑脊液常规、生化异常 ,5 6 %鞘内IgG合成率增高 ;头颅MRI均异常 ,以白质病变为主。电生理检查中以脑电图异常多见。 3.治疗及预后 :11例予甲泼尼龙冲击 ,5例同时合用大剂量人血丙种球蛋白 ,所有患儿均好转。 11例经3个月～ 6年随访 ,1例确诊为多发性硬化 ,10例无复发 ,其中 6例 (6 0 % )遗留后遗症。结论 ADEM具有区别于中枢神经系统感染的临床特点 ,致残率高 ,应及早诊治 ,糖皮质激素及大剂量丙种球蛋白有一定疗效。ADEM与多发性硬化的关系、如何改进治疗方法。

Objective To identify the clinical features of acute disseminated encephalomyelitis(ADEM) in childhood and to improve the skill of diagnosis and therapy.Methods A retrospective and follow-up study was conducted in 12 children with the initial diagnosis of ADEM to summarize the characteristics of clinical features, treatment and prognosis.Results 1. Clinical manifestations:the prodromal infection and vaccination were found in 75 %. The process was monophasic in 83 % and multiphasic in the other 17 % of cases. Various symptoms of central nervous system occurred one by one or simultaneously in the illness process. 2. Laboratory investigations: 18 % of cases had abnormal CSF( pleocytosis and raised protein). Raised intrathecal synthesis of IgG was found in half of the cases. All MRI brain scan demonstrated lesions predominantly affecting white matter . Abnormal EEG was the predominant findings in the electrophysiologic tests. 3. Treatment and prognosis:high dose methylprednisolone was administ in 11 cases. Five cases of them received intravenous immunoglobulin. All the cases showed good response. Eleven cases were taken follow-up from 3 months to 6 years, the final diagnosis turned to multiple sclerosis in 1 case. No recurrence occurred in the other 10 cases. Six cases of them had long-term neurologic sequelae.Conclusions ADEM is a disease with high incidence of disability, which has different features from infectious encephalitis. Early diagnosis and treatment are important. Steroid and high dose immunoglobulin have great effects on it. The relationship between ADEM and multiple sclerosis and how to improve the prognosis needs further investigation.