BACKGROUND Autoimmune encephalitis (AE) is a heterogeneous class of inflammatory diseases of the brain that can present with a wide spectrum of neuropsychiatric symptoms. Patients may be negative for CSF anti-neuronal antibodies, which can make the diagnosis of AE challenging. Distinguishing features between paediatric and adult patients with AE are not well characterized. OBJECTIVE Describe the clinical presentation, seizure type, EEG and sleep patterns in paediatric and adult patients with AE. METHODS/DESIGN Retrospective review of clinical data from paediatric and adult patients diagnosed with AE from three medical centers between 1/2008-12/2019. RESULTS We included 100 patients with AE, including 65 children. Median age at presentation was 14 years (1-88years). Fifty-five patients had positive CSF autoantibody results (NMDAR 36%, VGKC Ab 10%, anti-GAD65 4%, miscellaneous 3%), and 47 patients were autoantibody-negative. Paediatric patients were more likely to present with psychiatric symptoms, focal seizures and/or status epilepticus, and sleep disturbances compared to adult patients (p < 0.05). There was a higher incidence of NMDA-R encephalitis in children compared to adults. CONCLUSION Paediatric patients with AE were more likely to present with psychiatric symptoms, sleep disturbances, focal seizures, and/or status epilepticus compared to adults (p < 0.05). Insomnia and hypersomnia are common sleep problems associated with AE that should be screened early in the diagnostic evaluation. Further studies can be performed to explore the relationship between sleep disturbances and long-term cognitive effects and the incidence of chronic epilepsy in this subset of patients.

中文翻译：

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儿童和成人自身免疫性脑炎的临床特征：多中心样本

背景自身免疫性脑炎(AE)是一类异质性的脑炎性疾病，可表现为广泛的神经精神症状。患者可能对 CSF 抗神经元抗体呈阴性，这可能使 AE 的诊断具有挑战性。儿童和成人 AE 患者之间的区别特征没有很好地表征。目的 描述儿科和成人 AE 患者的临床表现、癫痫发作类型、脑电图和睡眠模式。方法/设计 对 2008-12/2019 期间来自三个医疗中心的诊断为 AE 的儿科和成人患者的临床数据进行回顾性审查。结果 我们纳入了 100 名 AE 患者，其中包括 65 名儿童。就诊时的中位年龄为 14 岁（1-88 岁）。55 名患者的 CSF 自身抗体结果呈阳性（NMDAR 36%，VGKC Ab 10%，抗 GAD65 4%，杂项 3%），47 名患者自身抗体阴性。与成人患者相比，儿科患者更可能出现精神症状、局灶性癫痫发作和/或癫痫持续状态和睡眠障碍（p < 0.05）。与成人相比，儿童 NMDA-R 脑炎的发病率更高。结论 与成人相比，患有 AE 的儿科患者更可能出现精神症状、睡眠障碍、局灶性癫痫发作和/或癫痫持续状态（p < 0.05）。失眠和嗜睡是与 AE 相关的常见睡眠问题，应在诊断评估的早期进行筛查。可以进行进一步的研究来探索睡眠障碍和长期认知影响以及这部分患者中慢性癫痫发病率之间的关系。