Bauer Jan, Becker Albert J, Elyaman Wassim, Peltola Jukka, Rüegg Stephan, Titulaer Maarten J, Varley James A, Beghi Ettore
Department of Neuroimmunology, Center for Brain Research Medical University of Vienna, Vienna, Austria.
Section for Translational Epilepsy Research, Department of Neuropathology, University of Bonn - Medical Center, Bonn, Germany.
Epilepsia. 2017 Jul;58 Suppl 3(Suppl Suppl 3):57-68. doi: 10.1111/epi.13784.
Inflammatory mechanisms have been increasingly implicated in the origin of seizures and epilepsy. These mechanisms are involved in the genesis of encephalitides in which seizures are a common complaint. Experimental and clinical evidence suggests different inflammatory responses in the brains of patients with epilepsy depending on the etiology. In general, activation of both innate and adaptive immunity plays a role in refractory forms of epilepsy. Epilepsies in which seizures develop after infiltration of cells of the adaptive immune system in the central nervous system (CNS) include a broad range of epileptic disorders with different (known or unknown) etiologies. Infiltration of lymphocytes is observed in autoimmune epilepsies, especially the classical paraneoplastic encephalitides with antibodies against intracellular tumor antigens. The presence of lymphocytes in the CNS also has been found in focal cerebral dysplasia type 2 and in cortical tubers. Various autoantibodies have been shown to be associated with temporal lobe epilepsy (TLE) and hippocampal sclerosis of unknown etiology, which may be due to the presence of viral DNA. During the last decade, an increasing number of antineuronal autoantibodies directed against membranous epitopes have been discovered and are associated with various neurologic syndromes, including limbic encephalitis. A major challenge in epilepsy is to define biomarkers, which would allow the recognition of patient populations who might benefit from immune-modulatory therapies. Some peripheral inflammatory markers appear to be differentially expressed in patients with medically controlled and medically refractory and, as such, could be used for diagnostic, prognostic, or therapeutic purposes. Establishing an autoimmune basis in patients with drug-resistant epilepsy allows for efficacious and targeted immunotherapy. Although current immunotherapies can give great benefit to the correctly identified patient, there are limitations to their efficacy and they may have considerable side effects. Thus the identification of new immunomodulatory compounds remains of utmost importance.
炎症机制在癫痫发作和癫痫的起源中所起的作用越来越受到关注。这些机制参与了脑炎的发病过程,而癫痫发作是脑炎患者的常见症状。实验和临床证据表明,根据病因不同,癫痫患者大脑中的炎症反应也有所不同。一般来说,先天性免疫和适应性免疫的激活在难治性癫痫中都发挥着作用。在适应性免疫系统细胞浸润中枢神经系统(CNS)后发生癫痫发作的癫痫类型包括一系列病因不同(已知或未知)的癫痫疾病。在自身免疫性癫痫中,尤其是在具有针对细胞内肿瘤抗原抗体的经典副肿瘤性脑炎中,可观察到淋巴细胞浸润。在2型局灶性脑发育异常和皮质结节中也发现了中枢神经系统中存在淋巴细胞。各种自身抗体已被证明与颞叶癫痫(TLE)和病因不明的海马硬化有关,这可能是由于病毒DNA的存在。在过去十年中,越来越多针对膜表位的抗神经元自身抗体被发现,并与包括边缘性脑炎在内的各种神经综合征相关。癫痫研究中的一个主要挑战是确定生物标志物,以便识别可能从免疫调节治疗中获益的患者群体。一些外周炎症标志物在药物控制良好和药物难治的患者中似乎有差异表达,因此可用于诊断、预后或治疗目的。在耐药性癫痫患者中确定自身免疫基础有助于进行有效且有针对性的免疫治疗。尽管目前的免疫疗法能使正确识别的患者受益匪浅,但其疗效存在局限性,且可能有相当大的副作用。因此,鉴定新的免疫调节化合物仍然至关重要。