Department of Neurosciences, Central Clinical School, Faculty of Medicine, Nursing, and Health Sciences, Monash University, Melbourne, Victoria, Australia.
Department of Neurology, Alfred Health, Melbourne, Victoria, Australia.
Epilepsia. 2020 Jun;61(6):1033-1044. doi: 10.1111/epi.16515. Epub 2020 May 13.
Epilepsy is a common neurological disorder that increases the risk of morbidity and mortality. Autoimmune epilepsy is a subset of epilepsy that occurs in the setting of autoimmunity, such as in autoimmune encephalitis (AIE). AIE is an autoimmune disorder characterized by immune-mediated neuroinflammation resulting in a variety of neurological symptoms, including psychiatric disturbance, cognitive dysfunction, and seizures. Seizures in AIE are thought to be a result of antibodies directed against neuronal cell-surface proteins involved in synaptic transmission. The role of blood-brain barrier dysfunction, myeloid cell infiltration, and the initiation of proinflammatory cascades in epileptogenesis has been shown to be important in animal models and human patients with epilepsy. Epileptogenesis in AIE is likely to arise from the synergistic effect of both innately driven neuroinflammation and antibody-induced hyperexcitability. Together, these processes produce persistent drug-resistant seizures that contribute to the morbidity seen in AIE. Understanding the proinflammatory pathways involved in this process may improve diagnostics and provide alternative treatment targets in AIE.
癫痫是一种常见的神经系统疾病，会增加发病率和死亡率。自身免疫性癫痫是癫痫的一个子集，发生在自身免疫的情况下，如自身免疫性脑炎 (AIE)。AIE 是一种自身免疫性疾病，其特征是免疫介导的神经炎症导致各种神经症状，包括精神障碍、认知功能障碍和癫痫发作。AIE 中的癫痫发作被认为是针对参与突触传递的神经元细胞表面蛋白的抗体的结果。在动物模型和癫痫患者中，已经表明血脑屏障功能障碍、髓样细胞浸润以及促炎级联的启动在癫痫发生中起重要作用。AIE 中的癫痫发生可能源于先天驱动的神经炎症和抗体诱导的过度兴奋的协同作用。这些过程共同产生持续的耐药性癫痫发作，导致 AIE 中出现的发病率。了解该过程中涉及的促炎途径可能会改善诊断并为 AIE 提供替代治疗靶点。
