Department of Pediatric Neurology, Brain Center, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands.
Department of Pediatric Neuropsychology, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands.
Sleep. 2020 Nov 12;43(11). doi: 10.1093/sleep/zsaa088.
Encephalopathy with electrical status epilepticus in sleep (ESES) is characterized by non-rapid eye movement (non-REM)-sleep-induced epileptiform activity and acquired cognitive deficits. The synaptic homeostasis hypothesis describes the process of daytime synaptic potentiation balanced by synaptic downscaling in non-REM-sleep and is considered crucial to retain an efficient cortical network. We aimed to study the overnight decline of slow waves, an indirect marker of synaptic downscaling, in patients with ESES and explore whether altered downscaling relates to neurodevelopmental and behavioral problems.
Retrospective study of patients with ESES with at least one whole-night electroencephalogram (EEG) and neuropsychological assessment (NPA) within 4 months. Slow waves in the first and last hour of non-REM-sleep were analyzed. Differences in slow-wave slope (SWS) and overnight slope course between the epileptic focus and non-focus electrodes and relations to neurodevelopment and behavior were analyzed.
A total of 29 patients with 44 EEG ~ NPA combinations were included. Mean SWS decreased from 357 to 327 µV/s (-8%, p < 0.001) across the night and the overnight decrease was less pronounced in epileptic focus than in non-focus electrodes (-5.6% vs. -8.7%, p = 0.003). We found no relation between SWS and neurodevelopmental test results in cross-sectional and longitudinal analyses. Patients with behavioral problems showed less SWS decline than patients without and the difference was most striking in the epileptic focus (-0.9% vs. -8.8%, p = 0.006).
Slow-wave homeostasis-a marker of synaptic homeostasis-is disturbed by epileptiform activity in ESES. Behavioral problems, but not neurodevelopmental test results, were related to severity of this disturbance.
电癫痫持续状态相关的脑病(ESES)的特征是无快速眼动(非快速眼动)睡眠诱导的癫痫样活动和获得性认知缺陷。突触稳态假说描述了白天突触增强与非快速眼动睡眠中突触缩小平衡的过程,被认为对保持有效的皮质网络至关重要。我们旨在研究 ESES 患者非快速眼动睡眠中慢波(突触缩小的间接标志物)的夜间下降,并探讨改变的缩小是否与神经发育和行为问题有关。
对至少有一次完整夜间脑电图(EEG)和神经心理评估(NPA)的 ESES 患者进行回顾性研究,时间在 4 个月内。分析非快速眼动睡眠第一小时和最后一小时的慢波。分析癫痫灶和非癫痫灶电极之间慢波斜率(SWS)和夜间斜率变化的差异,以及与神经发育和行为的关系。
共纳入 29 例患者,共 44 例 EEG~NPA 组合。平均 SWS 从夜间 357µV/s 下降至 327µV/s(-8%,p<0.001),在癫痫灶中下降幅度小于非癫痫灶电极(-5.6% vs. -8.7%,p=0.003)。在横断面和纵向分析中,我们发现 SWS 与神经发育测试结果之间没有关系。有行为问题的患者 SWS 下降幅度小于无行为问题的患者,在癫痫灶中差异最为明显(-0.9% vs. -8.8%,p=0.006)。
慢波稳态——突触稳态的标志物——在 ESES 中受到癫痫样活动的干扰。行为问题,而不是神经发育测试结果,与这种干扰的严重程度有关。