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自身免疫性脑炎。

Autoimmune Encephalitis.

机构信息

Division of Neurology, Department of Pediatrics, Children's Hospital of Los Angles, Los Angeles, CA.

Department of Neurology, Keck School of Medicine, University of Southern California, Los Angeles, CA.

出版信息

Pediatr Rev. 2022 Apr 1;43(4):198-211. doi: 10.1542/pir.2021-005096.

Abstract

Autoimmune encephalitis is a common and treatable cause of encephalitis in children and adults. Individuals present with a variety of symptoms, including altered mental status, behavioral changes, irritability, insomnia, developmental regression, seizures, dyskinetic movements, and autonomic instability. Evaluation includes electroencephalography, magnetic resonance imaging, and lumbar puncture. Once infectious and other causes are reasonably ruled out, treatment should be started empirically without waiting for antibody confirmation. Early clinical suspicion is key, as the outcome depends on early initiation of immunotherapy, including corticosteroids, intravenous immunoglobulin, and/or plasmapheresis. Severe or refractory cases require other treatments, such as rituximab, cyclophosphamide, or other immunotherapies using novel monoclonal antibodies. Psychiatry should be involved early for the management of behavioral issues. Additional considerations include management of seizures and dyskinesias. ICU admission may be required for management of hypoventilation necessitating mechanical ventilation (either intrinsic or iatrogenic, eg, from sedatives), refractory seizures, and dysautonomia. Anti-N-methyl-d-aspartate receptor and other forms of autoimmune encephalitis are less often associated with neoplasia (such as ovarian teratoma) in children compared with adults, but screening and removal of tumor if present should be performed.

摘要

自身免疫性脑炎是儿童和成人脑炎的常见且可治疗的病因。个体表现出多种症状,包括精神状态改变、行为变化、易怒、失眠、发育倒退、癫痫发作、不自主运动和自主神经不稳定。评估包括脑电图、磁共振成像和腰椎穿刺。一旦合理排除了感染和其他原因,就应在等待抗体确认之前,凭经验开始治疗。早期临床怀疑是关键,因为结果取决于早期开始免疫治疗,包括皮质类固醇、静脉注射免疫球蛋白和/或血浆置换。严重或难治性病例需要其他治疗,如利妥昔单抗、环磷酰胺或其他使用新型单克隆抗体的免疫疗法。应尽早让精神科医生参与行为问题的治疗。其他需要考虑的因素包括癫痫发作和运动障碍的管理。可能需要入住 ICU 来管理需要机械通气的通气不足(无论是内在的还是医源性的,例如,由于镇静剂)、难治性癫痫发作和自主神经功能障碍。与成人相比,抗 N-甲基-D-天冬氨酸受体和其他形式的自身免疫性脑炎较少与儿童的肿瘤(如卵巢畸胎瘤)相关,但如果存在肿瘤,应进行筛查和切除。

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