Department of Neuroimmunology, Center for Brain Research, Vienna, Austria.
Brain Pathol. 2012 May;22(3):412-21. doi: 10.1111/j.1750-3639.2012.00580.x.
Seizures are a prominent clinical feature of encephalitis. Recent data suggest the adaptive as well as innate immune system to be involved directly in the pathomechanism of epileptogenesis. Cytotoxic T-cells and antibody-mediated complement activation are major components of the adaptive immune system, which can induce neurodegeneration, thereby probably contributing to epileptic encephalitis. The innate immune system operates via interleukin-1 and toll-like receptor-associated mechanisms and was shown to play a direct role in epileptogenesis. Here, we review neuropathology hallmarks of various encephalitis conditions such as Rasmussen encephalitis (RE) but also introduce the more recently discovered antibody-associated voltage-gated potassium channel complex (VGKC), N-methyl-D-aspartate receptor (NMDAR) or glutamic acid decarboxylase (GAD) 65 encephalitides. Neuropathological investigations are used to determine specific cellular components and molecular mechanisms used by the immune system to provoke neurodegeneration and to promote epileptogenesis. Based on recent findings, we propose concepts for the stratification of epileptic encephalitis. Knowledge of the role of the innate immunity has already translated into clinical treatment strategies and may help to discover novel drug targets for these epileptic disorders.
癫痫发作是脑炎的一个突出临床特征。最近的数据表明，适应性免疫和固有免疫系统直接参与了癫痫发生的病理机制。细胞毒性 T 细胞和抗体介导的补体激活是适应性免疫系统的主要组成部分，它们可诱导神经退行性变，从而可能导致癫痫性脑炎。固有免疫系统通过白细胞介素 1 和 Toll 样受体相关机制发挥作用，并被证明在癫痫发生中起直接作用。在这里，我们回顾了各种脑炎的神经病理学特征，如 Rasmussen 脑炎（RE），但也介绍了最近发现的抗体相关电压门控钾通道复合物（VGKC）、N-甲基-D-天冬氨酸受体（NMDAR）或谷氨酸脱羧酶（GAD）65 脑炎。神经病理学研究用于确定免疫系统用于引发神经退行性变和促进癫痫发生的特定细胞成分和分子机制。基于最近的发现，我们提出了癫痫性脑炎的分层概念。对固有免疫作用的认识已经转化为临床治疗策略，并可能有助于发现这些癫痫疾病的新药物靶点。
