Abstract：
Encephalopathy related to Status Epilepticus during slow Sleep (ESES) is a childhood epilepsy syndrome characterized by the appearance of cognitive, behavioral, and motor disturbances in conjunction with a striking activation of EEG epileptic abnormalities during non-REM sleep. After more than 50 years since the first description, the pathophysiological mechanisms underlying the appearance of encephalopathy in association with a sleep-related enhancement of epileptic discharges are incompletely elucidated. Recent experimental data support the hypothesis that the development of the ESES encephalopathic picture depends on a spike-induced impairment of the synaptic homeostasis processes occurring during normal sleep and that is particularly pronounced during the developmental age. During sleep, synaptic homeostasis is promoted by synaptic weakening/elimination after the increment of synaptic strength that occurs during wakefulness. The EEG can display modifications in synaptic strength by changes in sleep slow wave activity (SWA). Recent studies during active ESES have failed to show changes in sleep SWA, while these changes occurred again after recovery from ESES, thus supporting a spike-related interference on the normal homeostatic processes of sleep. This impairment, during the developmental period, can lead to disruption of cortical wiring and brain plastic remodeling, which lead to the, often irreversible, neuropsychological compromise typical of ESES. From the nosographic point of view, these pathophysiological data lend support to the maintenance of the term ESES, i.e., \"encephalopathy related to status epilepticus during sleep\". Indeed, this term conveys the concept that the extreme activation of epileptic discharges during sleep is directly responsible for the encephalopathy, hence the importance of defining this condition as an encephalopathy related to the exaggerated activation of epileptic activity during sleep. In this respect, ESES represents a genuine example of a \"pure\" epileptic encephalopathy in which sleep-related epileptic activity \"per se\" has a crucial role in determining the encephalopathic picture. This paper was presented at the 8th London-Innsbruck Colloquium on Status Epilepticus and Acute Seizures held in September 2022.
摘要：
与慢睡期癫痫持续状态相关的脑病（ESES）是一种儿童癫痫综合征，其特征是出现认知，行为，和运动障碍以及非REM睡眠期间EEG癫痫异常的惊人激活。在第一次描述50多年后，与睡眠相关的癫痫放电增强相关的脑病的病理生理机制尚未完全阐明.最近的实验数据支持以下假设：ESES脑病图的发展取决于正常睡眠期间发生的突触稳态过程的尖峰诱导损伤，并且在发育年龄期间尤为明显。在睡眠中,在觉醒过程中发生的突触强度增加后，突触减弱/消除可促进突触稳态。EEG可以通过睡眠慢波活动（SWA）的变化来显示突触强度的变化。在活跃的ESES期间，最近的研究未能显示睡眠SWA的变化，虽然这些变化在从ESES恢复后再次发生，从而支持对正常稳态睡眠过程的尖峰相关干扰。这种损害，在发育时期，会导致大脑皮层布线和大脑可塑性重塑的破坏，这导致了，往往是不可逆转的,ESES典型的神经心理学妥协。从nosography的角度来看，这些病理生理数据为维持ESES一词提供了支持，即，“与睡眠中癫痫持续状态相关的脑病”。的确,这个术语传达了这样一个概念，即睡眠期间癫痫放电的极端激活是脑病的直接原因，因此，将这种情况定义为与睡眠期间癫痫活动过度激活有关的脑病的重要性。在这方面,ESES代表了“纯”癫痫性脑病的真实例子，其中与睡眠相关的癫痫活动“本身”在确定脑病图上具有至关重要的作用。本文在2022年9月举行的第八届伦敦-因斯布鲁克癫痫发作和急性癫痫发作座谈会上发表。
