Division of Clinical Neurophysiology, University Children's Hospital Zurich, Zurich, Switzerland.
Clin Neurophysiol. 2011 Sep;122(9):1779-87. doi: 10.1016/j.clinph.2011.01.053. Epub 2011 Mar 26.
"Encephalopathy related to electrical status epilepticus during slow wave sleep" (ESES) is characterised by the electroencephalographic pattern of continuous spike waves during slow wave sleep (CSWS) and variable neuropsychological impairments. The synaptic homeostasis hypothesis predicts that the strength of synapses is decreased during sleep. The slope of slow waves during NREM sleep best reflects this "downscaling".
In a retrospective case control study, we analysed the time course of the slope of EEG slow waves of nine patients with ESES. The patients showed continuous spike waves (>85%) associated with regression or stagnation of cognitive functions. The data of the patient group were compared to nine healthy age and gender matched controls.
In control subjects we found the expected decrease of the slope of slow waves from the first to the last hour of sleep (17.2% decrease, p<0.001). In contrast, patients showed no significant change in slope across the night.
This finding may reflect a disruption of the downscaling process during sleep, which may contribute to the developmental regression in these children.
Thus, our findings contribute to the understanding of the pathomechanisms leading to the regression observed in children with ESES and support the view that the goal of the treatment in children with ESES should not only be to reduce seizures, but also to resolve the continuous spike wave activity.
“与睡眠慢波期癫痫持续状态相关的脑病”（ESES）的特征是脑电图呈现睡眠慢波期连续棘波（CSWS）和可变的神经心理障碍。突触稳态假说预测突触强度在睡眠期间降低。非快速眼动（NREM）睡眠期间慢波的斜率最好反映了这种“缩减”。
在一项回顾性病例对照研究中，我们分析了 9 例 ESES 患者的 EEG 慢波斜率的时间进程。这些患者表现出连续棘波（>85%），伴有认知功能的退化或停滞。将患者组的数据与 9 名年龄和性别匹配的健康对照进行比较。
在对照组中，我们发现慢波斜率从第一小时到最后一小时呈预期下降（下降 17.2%，p<0.001）。相比之下，患者在整个夜间的斜率没有明显变化。
这一发现可能反映了睡眠期间“缩减”过程的中断，这可能导致这些儿童的发育退化。
因此，我们的发现有助于理解导致 ESES 儿童出现退化的病理机制，并支持这样一种观点，即 ESES 儿童的治疗目标不仅应是减少癫痫发作，还应解决持续棘波活动。
