Epilepsy is seen historically as a disease of aberrant neuronal signaling manifesting as seizures. With the discovery of numerous auto-antibodies and the subsequent growth in understanding of autoimmune encephalitis, there has been an increasing emphasis on the contribution of the innate and adaptive immune system to ictogenesis and epileptogenesis. Pathogenic antibodies, complement activation, CD8+ cytotoxic T cells, and microglial activation are seen, to various degrees, in different seizure-associated neuroinflammatory and autoimmune conditions. These aberrant immune responses are thought to cause disruptions in neuronal signaling, generation of acute symptomatic seizures, and, in some cases, the development of long-term autoimmune epilepsy. Although early treatment with immunomodulatory therapies improves outcomes in autoimmune encephalitides and autoimmune epilepsies, patient identification and treatment selection are not always clear-cut. This review examines the role of the different components of the immune system in various forms of seizure disorders including autoimmune encephalitis, autoimmune epilepsy, Rasmussen encephalitis, febrile infection-related epilepsy syndrome (FIRES), and new-onset refractory status epilepticus (NORSE). In particular, the pathophysiology and unique cytokine profiles seen in these disorders and their links with diagnosis, prognosis, and treatment decision-making are discussed.

中文翻译：

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炎症、发作和癫痫发生：对人类疾病的探索

癫痫在历史上被视为一种表现为癫痫发作的异常神经元信号传导疾病。随着大量自身抗体的发现以及随后对自身免疫性脑炎的理解的增长，人们越来越重视先天性和适应性免疫系统对 ictogenesis 和癫痫发生的贡献。致病性抗体、补体激活、CD8+ 细胞毒性 T 细胞和小胶质细胞激活在不同程度的癫痫相关神经炎症和自身免疫疾病中可见。这些异常的免疫反应被认为会导致神经元信号中断、急性症状性癫痫发作的产​​生，并且在某些情况下会导致长期自身免疫性癫痫的发展。尽管免疫调节疗法的早期治疗可改善自身免疫性脑炎和自身免疫性癫痫的预后，但患者的识别和治疗选择并不总是明确的。本综述研究了免疫系统的不同组成部分在各种形式的癫痫发作中的作用，包括自身免疫性脑炎、自身免疫性癫痫、拉斯穆森脑炎、发热性感染相关癫痫综合征 (FIRES) 和新发难治性癫痫持续状态 (NORSE)。特别讨论了在这些疾病中看到的病理生理学和独特的细胞因子谱，以及它们与诊断、预后和治疗决策的联系。本综述研究了免疫系统的不同组成部分在各种形式的癫痫发作中的作用，包括自身免疫性脑炎、自身免疫性癫痫、拉斯穆森脑炎、发热性感染相关癫痫综合征 (FIRES) 和新发难治性癫痫持续状态 (NORSE)。特别讨论了在这些疾病中看到的病理生理学和独特的细胞因子谱，以及它们与诊断、预后和治疗决策的联系。本综述研究了免疫系统的不同组成部分在各种形式的癫痫发作中的作用，包括自身免疫性脑炎、自身免疫性癫痫、拉斯穆森脑炎、发热性感染相关癫痫综合征 (FIRES) 和新发难治性癫痫持续状态 (NORSE)。特别讨论了在这些疾病中看到的病理生理学和独特的细胞因子谱，以及它们与诊断、预后和治疗决策的联系。