Melzer Nico, Rosenow Felix
Department of Neurology, Medical Faculty and University Hospital, Heinrich Heine University Düsseldorf, Moorenstraße 5, Düsseldorf 40225, Germany.
Goethe University Frankfurt, Epilepsy Center Frankfurt Rhine-Main, Department of Neurology, University Hospital Frankfurt, Frankfurt, Germany; Goethe University Frankfurt, LOEWE Center for Personalized Translational Epilepsy Research (CePTER), Frankfurt, Germany.
Seizure. 2025 May;128:20-23. doi: 10.1016/j.seizure.2024.05.017. Epub 2024 May 27.
The current International League Against Epilepsy (ILAE) definition and classification guidelines for the first time introduced the category of immune-mediated focal epilepsy in addition to structural, genetic, infectious, and metabolic aetiologies. Moreover, the ILAE Autoimmunity and Inflammation Taskforce recently provided a conceptual framework for the distinction between acute "provoked" seizures in the acute phase of autoimmune encephalitis from chronic "unprovoked" seizures due to autoimmune-associated epilepsy. The first category predominately applies to those autoimmune encephalitis patients with autoantibodies against cell surface neural antigens, in whom autoantibodies are assumed to exert a direct ictogenic effect without overt structural damage. These patients do not exhibit enduring predisposition to seizures after the "acute phase" encephalitis, and thus do not fulfil the definition of epilepsy. The second category applies to those autoimmune encephalitis patients with autoantibodies against intracellular neural antigens and Rasmussen's encephalitis, in whom T cells are assumed to cause epileptogenic effects through immune-inflammation and overt structural damage. These patients do exhibit enduring predisposition to seizures after the "acute phase" of encephalitis and thus fulfil the definition of epilepsy. AAE may result from both, ongoing brain autoimmunity and associated structural brain damage according to the current ILAE definition and classification guideline. We here discuss the difficulties of this concept and suggest an unbiased translationally validated and data-driven approach to predict in an individual encephalitis patient the propensity to develop (or not) AAE and the cognitive and behavioural outcome.
当前的国际抗癫痫联盟（ILAE）定义和分类指南首次引入了免疫介导性局灶性癫痫这一类别，除了结构性、遗传性、感染性和代谢性病因之外。此外，ILAE自身免疫与炎症特别工作组最近提供了一个概念框架，用于区分自身免疫性脑炎急性期的急性“诱发性”发作与自身免疫相关性癫痫所致的慢性“非诱发性”发作。第一类主要适用于那些针对细胞表面神经抗原产生自身抗体的自身免疫性脑炎患者，在这些患者中，自身抗体被认为在没有明显结构损伤的情况下发挥直接的致痫作用。这些患者在“急性期”脑炎后不会表现出持久的癫痫易感性，因此不符合癫痫的定义。第二类适用于那些针对细胞内神经抗原产生自身抗体的自身免疫性脑炎患者以及患有拉斯穆森脑炎的患者，在这些患者中，T细胞被认为通过免疫炎症和明显的结构损伤产生致痫作用。这些患者在脑炎“急性期”后确实表现出持久的癫痫易感性，因此符合癫痫的定义。根据当前的ILAE定义和分类指南，自身免疫性急性脑炎（AAE）可能由持续的脑部自身免疫和相关的脑部结构损伤共同导致。我们在此讨论这一概念的难点，并提出一种无偏倚的、经过翻译验证且以数据为驱动的方法，以预测个体脑炎患者发生（或不发生）AAE的倾向以及认知和行为结果。
