目的 探讨血浆置换治疗儿童急性播散性脑脊髓炎(ADEM)的疗效及安全性。方法 回顾分析2例ADEM 患儿的临床资料,并检索1998年1月至2018年1月万方和PubMed数据库的相关文献进行分析。结果 2例男性患儿,分 别为5岁和2岁,临床有发热伴有抽搐、意识障碍等多种脑病表现;影像学提示白质多发病变,符合ADEM诊断标准。予 大剂量激素、免疫球蛋白治疗后,病情仍进展,肌无力、意识障碍加重,出现呼吸衰竭。予呼吸机辅助呼吸、血浆置换(于 病程2周左右)等,症状快速缓解,神志转清,肌力逐渐恢复,脱离呼吸机。分别随访1年及6个月,均未遗留神经系统症状。 结论 在ADEM患儿对大剂量激素及丙球治疗反应不佳且病情迅速进展时,血浆置换可作为挽救性治疗措施。
Objective To explore the efficacy and safety of plasma exchange in the treatment of acute disseminated encephalomyelitis (ADEM) in children. Method The clinical data of ADEM in 2 children were retrospectively analyzed. The related literature from January 1998 to January 2018 were retrieved from WanFang database and PubMed database and analyzed. Results Two boys aged 5 and 2 years respectively had fever, convulsion, consciousness disturbance and other manifestations of encephalopathy. Imaging showed multiple white matter lesions, which met the diagnostic criteria of ADEM. After the treatment with high dose of hormone and immunoglobulin, the disease still progressed and patients had the increased muscle weakness, deeper coma and respiratory failure. After ventilator-assisted breathing and plasma exchange about 2 weeks after diagnosis, the symptoms were rapidly relieved and the patients had gradual recovery of consciousness and muscle strength, and was successfully weaned from the ventilator. The two boys were followed up for 1 year and 6 months respectively, and no neurological symptoms were found. Conclusion Plasma exchange can be used as a salvage treatment when children with ADEM do not respond well to high-dose steroids and gamma globulin therapy and the disease progressed rapidly.