儿童抗N-甲基-D-天门冬氨酸受体脑炎的治疗

Management of anti-N-methyl-D-aspartate receptor encephalitis in children

摘要:

抗N-甲基-D-天门冬氨酸受体（NMDAR）脑炎是新认识的一种严重、潜在可治的自身免疫性脑炎，可见于任何年龄，但以儿童更为常见。该病具有显著特征性，病程可分为五期，即病毒感染样症状的前驱期、精神症状期、意识障碍期、异常运动期和恢复期。该病主要治疗包括一线免疫治疗如皮质类固醇激素、静脉丙种球蛋白和血浆交换治疗；二线免疫治疗如利妥昔单抗和环磷酰胺等及肿瘤切除。该文主要介绍抗NMDAR 脑炎各种治疗方法的进展、选择及治疗的局限和不足，以期对该病的临床治疗有所帮助。

Abstract:

Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a new category of severe, potentially treatable autoimmune encephalitis and can appear in patients of all ages, but more frequently in children. It is a highly characteristic syndrome evolving in five stages: the prodromal phase (viral infection-like symptoms), psychotic phase, unresponsive phase, hyperkinetic phase, and gradual recovery phase. The treatment for this disorder includes firstline immunotherapy (steroids, intravenous immunoglobulin, plasmapheresis), second-line immunotherapy (rituximab, cyclophosphamide), and tumor removal. Hereby the progresses, selections and shortcomings of the treatment protocols for this disease are introduced.