Cases-Cunillera Silvia, Quatraccioni Anne, Rossini Laura, Ruffolo Gabriele, Ono Tomonori, Baulac Stéphanie, Auvin Stéphane, O'Brien Terence J, Henshall David C, Akman Özlem, Sankar Raman, Galanopoulou Aristea S
Université Paris Cité, Institute of Psychiatry and Neuroscience of Paris (IPNP), INSERM U1266, Neuronal Signaling in Epilepsy and Glioma, Paris, France.
Institute of Neuropathology, Section for Translational Epilepsy Research, Medical Faculty, University of Bonn, Bonn, Germany.
Epilepsia. 2024 Dec;65(12):3457-3468. doi: 10.1111/epi.18126. Epub 2024 Oct 14.
Epilepsy represents a common neurological disorder in patients with developmental brain lesions, particularly in association with malformations of cortical development and low-grade glioneuronal tumors. In these diseases, genetic and molecular alterations in neurons are increasingly discovered that can trigger abnormalities in the neuronal network, leading to higher neuronal excitability levels. However, the mechanisms underlying epilepsy cannot rely solely on assessing the neuronal component. Growing evidence has revealed the high degree of complexity underlying epileptogenic processes, in which glial cells emerge as potential modulators of neuronal activity. Understanding the role of glial cells in developmental brain lesions such as malformations of cortical development and low-grade glioneuronal tumors is crucial due to the high degree of pharmacoresistance characteristic of these lesions. This has prompted research to investigate the role of glial and immune cells in epileptiform activity to find new therapeutic targets that could be used as combinatorial drug therapy. In a special session of the XVI Workshop of the Neurobiology of Epilepsy (WONOEP, Talloires, France, July 2022) organized by the Neurobiology Commission of the International League Against Epilepsy, we discussed the evidence exploring the genetic and molecular mechanisms of glial cells and immune response and their implications in the pathogenesis of neurodevelopmental pathologies associated with early life epilepsies.
癫痫是发育性脑损伤患者中常见的神经系统疾病,特别是与皮质发育畸形和低级别神经胶质神经元肿瘤相关。在这些疾病中,越来越多地发现神经元中的基因和分子改变会引发神经网络异常,导致神经元兴奋性水平升高。然而,癫痫的发病机制不能仅仅依赖于对神经元成分的评估。越来越多的证据表明,致痫过程具有高度复杂性,其中神经胶质细胞成为神经元活动的潜在调节因子。由于这些病变具有高度药物抵抗性,了解神经胶质细胞在发育性脑损伤(如皮质发育畸形和低级别神经胶质神经元肿瘤)中的作用至关重要。这促使研究人员研究神经胶质细胞和免疫细胞在癫痫样活动中的作用,以寻找可作为联合药物治疗的新治疗靶点。在国际抗癫痫联盟神经生物学委员会组织的第十六届癫痫神经生物学研讨会(2022年7月,法国塔洛雷斯,WONOEP)的一次特别会议上,我们讨论了探索神经胶质细胞和免疫反应的遗传和分子机制及其在与早期癫痫相关的神经发育性疾病发病机制中的意义的证据。