Abstract：
Seizures are a very common manifestation of autoimmune encephalitis (AE), ranging from 33% to 100% depending on the antigen, most often accompanied by other clinical features such as behavioral changes, movement disorders, memory deficits, autoimmune disturbances, and altered levels of consciousness. Unusual seizure frequency, resistance to antiepileptic treatment, and often, definitive response to immunotherapy emphasize the importance for neurologists to consider the probable etiology of immune disorders. Studies on pathogenic mechanisms of autoantibodies have improved the understanding of different pathophysiologies and clinical characteristics of different AE groups. In encephalitis with antibodies to neuronal extracellular antigens, autoantibodies play a direct role in disease pathogenesis. They have access to target antigens and can potentially alter the structure and function of antigens but induce relatively little neuronal death. Prompt immunotherapy is usually very effective, and long-term antiepileptic treatment may not be needed. In contrast, in encephalitis with antibodies against intracellular antigens, autoantibodies may not be directly pathogenic but serve as tumor markers. These autoantibodies cannot reach intracellular target antigens and are considered to result from a T-cell-mediated immune response against antigens released by apoptotic tumor cells, which contain nerve tissue or express neuronal proteins. Neuronal loss is frequently described and predominantly induced through cytotoxic T-cell mechanisms. They often exhibit an inadequate response to immunotherapy and require early tumor treatment. Long-term antiepileptic treatment is usually needed. In conclusion, each neural autoantibody can specifically precipitate seizures. Early proper management of these cases may help prevent neurological deterioration and manage the occurrence of seizures. Consequently, confirmation of the presence of neuronal autoantibodies is strongly recommended even in patients with confirmed AE, as they are not only essential in achieving a good outcome but also may provide evidence for underlying neoplasia.
摘要：
癫痫发作是自身免疫性脑炎（AE）的一种非常常见的表现，根据抗原的不同，从33%到100%不等，最常伴有其他临床特征，如行为变化，运动障碍,记忆缺陷,自身免疫性疾病，改变了意识水平。不寻常的癫痫发作频率,抵抗抗癫痫治疗，而且经常,明确的免疫疗法反应强调了神经科医师考虑免疫疾病可能病因的重要性.对自身抗体致病机制的研究提高了对不同AE组的不同病理生理和临床特征的理解。在具有神经元细胞外抗原抗体的脑炎中，自身抗体在疾病的发病机制中起直接作用。它们可以接触靶抗原，并可能改变抗原的结构和功能，但诱导相对较少的神经元死亡。迅速免疫疗法通常非常有效，可能不需要长期抗癫痫治疗。相比之下,在具有针对细胞内抗原的抗体的脑炎中，自身抗体可能不是直接致病的，但可作为肿瘤标志物。这些自身抗体不能到达细胞内靶抗原，被认为是由T细胞介导的针对凋亡肿瘤细胞释放的抗原的免疫应答引起的。含有神经组织或表达神经元蛋白。神经元丢失经常被描述并且主要通过细胞毒性T细胞机制诱导。它们通常表现出对免疫疗法的反应不足，需要早期肿瘤治疗。通常需要长期抗癫痫治疗。总之,每种神经自身抗体都能特异性诱发癫痫发作。对这些病例的早期适当管理可能有助于防止神经系统恶化和控制癫痫发作的发生。因此,即使在已确诊的AE患者中，也强烈建议确认存在神经元自身抗体，因为它们不仅对获得良好的结果至关重要，而且可能为潜在的肿瘤形成提供证据。
