Halász Péter
National Institute of Clinical Neuroscience, Lotz K. u. 18, Budapest 1026, Hungary.
Behav Neurol. 2015;2015:231676. doi: 10.1155/2015/231676. Epub 2015 Jun 14.
System epilepsy is an emerging concept interpreting major nonlesional epilepsies as epileptic dysfunctions of physiological systems. I extend here the concept of reflex epilepsy to epilepsies linked to input dependent physiological systems. Experimental and clinical reseach data were collected to create a coherent explanation of underlying pathomechanism in AE and NFLE. We propose that AE should be interpreted as epilepsy linked to the corticothalamic burst-firing mode of NREM sleep, released by evoked vigilance level oscillations characterized by reactive slow wave response. In the genetic variation of NFLE the ascending cholinergic arousal system plays an essential role being in strong relationship with a gain mutation of the nicotinic acethylcholin receptors, rendering the arousal system hyperexcitable. I try to provide a more unitary interpretation for the variable seizure manifestation integrating them as different degree of pathological arosuals and alarm reactions. As a supporting hypothesis the similarity between arousal parasomnias and FNLE is shown, underpinned by overlaping pathomechanism and shared familiarity, but without epileptic features. Lastly we propose that both AE and NFLE are system epilepsies of the sleep-wake system representing epileptic disorders of the antagonistic sleep/arousal network. This interpretation may throw new light on the pathomechanism of AE and NFLE.
系统性癫痫是一个新兴概念,将主要的非损伤性癫痫解释为生理系统的癫痫性功能障碍。在此,我将反射性癫痫的概念扩展到与输入依赖性生理系统相关的癫痫。收集了实验和临床研究数据,以对觉醒期癫痫(AE)和夜间额叶癫痫(NFLE)的潜在发病机制作出连贯解释。我们提出,AE应被解释为与非快速眼动睡眠的皮质丘脑爆发式放电模式相关的癫痫,由以反应性慢波反应为特征的诱发警觉水平振荡所引发。在NFLE的基因变异中,上行胆碱能觉醒系统起着至关重要的作用,它与烟碱型乙酰胆碱受体的增益突变密切相关,使觉醒系统过度兴奋。我试图为各种发作表现提供一个更统一的解释,将它们整合为不同程度的病理性觉醒和警觉反应。作为一个支持性假设,觉醒期异态睡眠和NFLE之间的相似性得以展现,其基础是重叠的发病机制和共同的特点,但不具备癫痫特征。最后,我们提出AE和NFLE都是睡眠 - 觉醒系统的系统性癫痫,代表了睡眠/觉醒拮抗网络的癫痫性疾病。这种解释可能为AE和NFLE的发病机制带来新的启示。