Hardy Duriel
Department of Neurology, Dell Medical School, University of Texas at Austin, Austin, Texas.
Pediatr Neurol. 2022 Jul;132:56-66. doi: 10.1016/j.pediatrneurol.2022.05.004. Epub 2022 May 13.
Autoimmune encephalitis is a group of central nervous system (CNS) inflammatory disorders that most commonly affect young adults and children. These disorders are closely associated with antibodies against neuronal cell-surface proteins, receptors, and ion channels; however, some forms of the disorder have no known antibody at this time. In children, neurological manifestations such as seizure, movement disorders, and focal neurological deficits are more prominent at initial presentation than psychiatric or behavioral symptoms. When psychiatric symptoms do occur, they often manifest as temper tantrums, aggression, agitation, and rarely psychosis. Prompt diagnosis and early treatment can lead to improved outcomes and decreased relapses. First-line therapies include intravenous steroids, intravenous immunoglobulin, and plasmapheresis, whereas rituximab and cyclophosphamide are utilized for refractory or relapsing disease. This review highlights the different forms of this disorder, discusses approach to diagnosis and treatment, and reviews the outcome and prognosis of children diagnosed with different forms of autoimmune encephalitis.
自身免疫性脑炎是一组中枢神经系统（CNS）炎症性疾病，最常影响年轻人和儿童。这些疾病与针对神经元细胞表面蛋白、受体和离子通道的抗体密切相关；然而，目前某些形式的该疾病尚无已知抗体。在儿童中，癫痫发作、运动障碍和局灶性神经功能缺损等神经学表现，在初次就诊时比精神或行为症状更为突出。当精神症状确实出现时，它们通常表现为发脾气、攻击行为、烦躁不安，很少表现为精神病。及时诊断和早期治疗可改善预后并减少复发。一线治疗包括静脉注射类固醇、静脉注射免疫球蛋白和血浆置换，而利妥昔单抗和环磷酰胺则用于难治性或复发性疾病。本综述重点介绍了该疾病的不同形式，讨论了诊断和治疗方法，并回顾了被诊断为不同形式自身免疫性脑炎的儿童的预后和转归。
