Department of Pediatric Neurology, Reference Center for Rare Epilepsies, Inserm U663, Necker Enfants Malades Hospital, Paris, France.
Epilepsia. 2012 Sep;53 Suppl 4:58-62. doi: 10.1111/j.1528-1167.2012.03614.x.
The role of immunity and inflammation in epilepsy have long been suggested by the anticonvulsant activity of steroids in some infancy and childhood epilepsies. The role of fever and infection in exacerbating seizures due to possible proinflammatory molecules, the increased frequency of seizures in systemic autoimmune diseases like systemic lupus erythematous, and, recently, the detection of autoantibodies in some unexplained epilepsies reinforced the causal place of immunity and inflammation in epilepsies with unknown etiology. In this article, we summarize epilepsies where clinical and biologic data strongly support the pathogenic role of autoantibodies (e.g., limbic encephalitides, N-methyl-d-aspartate [NMDA] encephalitis) and epilepsies where immune-mediated inflammation occurs, but the full pathogenic cascade is either not clear (e.g., Rasmussen's encephalitis) or only strongly hypothesized (idiopathic hemiconvulsion-hemiplegia syndrome [IHHS] and fever-induced refractory epilepsy in school-aged children [FIRES]). We emphasize the electroclinical features that would help to diagnose these conditions, allowing early immunomodulating therapy. Finally, we raise some questions that remain unclear regarding diagnosis, mechanisms, and future therapies.
免疫和炎症在癫痫中的作用长期以来一直被提示，因为一些婴儿和儿童癫痫中的类固醇具有抗惊厥作用。发热和感染可能通过促炎分子加重癫痫发作的作用，全身性自身免疫性疾病（如系统性红斑狼疮）中癫痫发作的频率增加，以及最近在一些原因不明的癫痫中检测到自身抗体，这些都加强了免疫和炎症在病因不明的癫痫中的因果关系。在本文中，我们总结了临床和生物学数据强烈支持自身抗体致病作用的癫痫（如边缘性脑炎、N-甲基-D-天冬氨酸[NMDA]脑炎）和发生免疫介导炎症的癫痫，但完整的致病级联反应尚不清楚（如 Rasmussen 脑炎）或仅被强烈假设（特发性偏瘫性惊厥-偏瘫综合征[IHHS]和学龄儿童发热性难治性癫痫[FIRES]）。我们强调了有助于诊断这些疾病的电临床特征，以便早期进行免疫调节治疗。最后，我们提出了一些关于诊断、机制和未来治疗的仍不清楚的问题。
