UCSF Weill Institute for Neurosciences, Department of Neurology, University of California San Francisco, San Francisco, CA, USA.
UC San Diego School of Medicine, University of California San Diego, San Diego, CA, USA.
J Child Neurol. 2020 Aug;35(9):591-599. doi: 10.1177/0883073820923834. Epub 2020 May 27.
Autoimmune encephalitis is the third most common cause of encephalitis in children. We provide a detailed account of presenting symptoms, diagnosis, and response to treatment in pediatric autoimmune encephalitis patients evaluated at University of California San Francisco within a 2.5-year period. Eleven were identified: anti--methyl-d-aspartate receptor (NMDAR) encephalitis (n = 4), antibody-negative autoimmune encephalitis (n = 4), steroid-responsive encephalopathy associated with thyroiditis (SREAT) (n = 2), and glial fibrillary acidic protein (GFAP)-associated encephalitis (n = 1). Most common presenting symptoms included seizures and behavior changes (54%). More than 90% of patients showed improvement following first-line immunotherapy (high-dose corticosteroids, intravenous immunoglobulin, and/or plasma exchange). A total of 64% received second-line treatment with rituximab, cyclophosphamide, or mycophenolate mofetil. One patient with NMDAR encephalitis died despite escalating immunotherapy. None of the patients showed complete recovery after median follow-up of 9 months (range 0.5-66). Children with autoimmune encephalitis have a diverse clinical presentation and may lack an identifiable autoantibody. Majority of patients show a good response to immunotherapy; however, recovery can be delayed.
自身免疫性脑炎是儿童脑炎的第三大常见病因。我们详细介绍了在加利福尼亚大学旧金山分校评估的 2.5 年内患有儿科自身免疫性脑炎患者的发病症状、诊断和治疗反应。共发现 11 例患者：抗 N-甲基-D-天冬氨酸受体（NMDAR）脑炎（n=4）、抗体阴性自身免疫性脑炎（n=4）、伴甲状腺炎的类固醇反应性脑病（SREAT）（n=2）和胶质纤维酸性蛋白（GFAP）相关脑炎（n=1）。最常见的发病症状包括癫痫发作和行为改变（54%）。超过 90%的患者在一线免疫治疗（大剂量皮质类固醇、静脉注射免疫球蛋白和/或血浆置换）后有所改善。共有 64%的患者接受利妥昔单抗、环磷酰胺或霉酚酸酯二线治疗。尽管免疫治疗不断升级，仍有 1 例 NMDAR 脑炎患者死亡。在中位数 9 个月（范围 0.5-66）的随访后，无患者完全康复。自身免疫性脑炎患儿临床表现多样，可能缺乏可识别的自身抗体。大多数患者对免疫治疗反应良好，但恢复可能延迟。
