Oser Nadine, Hubacher Martina, Nageleisen-Weiss Annette, van Mierlo Pieter, Huber Reto, Weber Peter, Bölsterli Bigna K, Datta Alexandre N
Department of Pediatric Neurology and Developmental Medicine, University of Basel Children's Hospital, Basel, Switzerland.
Epilog NV, Ghent, Belgium.
Epilepsy Behav Rep. 2021 Sep 30;16:100488. doi: 10.1016/j.ebr.2021.100488. eCollection 2021.
The epilepsy-aphasia spectrum consists of epilepsies with a strong activation of epileptic discharges during non-rapid-eye-movement (NREM) sleep, variable seizure burden and language problems. The homeostatic decrease of slow waves (SW) during NREM sleep (i.e. their amplitude/slope and power) has been related to brain recovery and cognitive function. Epileptic discharges during NREM-sleep were related to an impairment of the decrease of the slope of SW and to cognitive deficits. In this longitudinal case study, we aim to relate this electrophysiological marker, i.e. overnight change of slope of SW, to imaging and behavior. We report a young girl with a fluctuating course in the epilepsy-aphasia spectrum, ranging from the benign end with self-limited childhood epilepsy with centrotemporal spikes (SLECTS) to the severe end with epileptic encephalopathy with continuous spike waves during sleep (CSWS) with two phases of cognitive regression. She was documented over a period of six years including 12 PSGs, six language fMRIs and seven neuropsychological assessments. We longitudinally studied focal and total spike wave index (SWI), detected SW during NREM sleep, calculated their slopes (first and last hour of NREM sleep and overnight change). Deterioration of overnight decrease of the slope of SW was paralleled by the occurrence of the EEG picture of bilateral synchronous electrical status epilepticus during sleep (ESES) and neuropsychological deficits, and this impairment was reversible with resolution of ESES and was accompanied by cognitive improvement. A laterality switch from left to right sided language dominance occurred during recovery from the second regression phase. This might reflect a compensating process. Later, the laterality switched back to the left, possibly facilitated by a low SWI on the left hemisphere. The qualitative analysis of this case supports the view that the longitudinal course of the overnight change of the slope of SW, as an objective, quantitative EEG measure, is related to the course of cognitive function and functional language MR analysis.
癫痫-失语谱系包括在非快速眼动（NREM）睡眠期间癫痫放电强烈激活、癫痫发作负担可变以及存在语言问题的癫痫。NREM睡眠期间慢波（SW）的稳态减少（即其振幅/斜率和功率）与大脑恢复和认知功能有关。NREM睡眠期间的癫痫放电与SW斜率下降受损以及认知缺陷有关。在这项纵向病例研究中，我们旨在将这种电生理标志物，即SW斜率的夜间变化，与影像学和行为联系起来。我们报告了一名年轻女孩，其在癫痫-失语谱系中病程波动，从良性的伴有中央颞区棘波的自限性儿童癫痫（SLECTS）到严重的伴有睡眠期持续棘慢波的癫痫性脑病（CSWS），伴有两个认知衰退阶段。我们对她进行了为期六年的记录，包括12次多导睡眠图（PSG）、6次语言功能磁共振成像（fMRI）和7次神经心理学评估。我们纵向研究了局灶性和总棘慢波指数（SWI），在NREM睡眠期间检测SW，计算其斜率（NREM睡眠的第一个小时和最后一个小时以及夜间变化）。SW斜率夜间下降的恶化与睡眠期双侧同步癫痫性电持续状态（ESES）的脑电图表现和神经心理学缺陷的出现平行，并且随着ESES的缓解这种损害是可逆的，并伴有认知改善。在从第二个衰退阶段恢复期间，语言优势半球从左侧转变为右侧。这可能反映了一个代偿过程。后来，优势半球又转回左侧，可能是由于左半球较低的SWI所促进。该病例的定性分析支持这样一种观点，即SW斜率夜间变化的纵向过程，作为一种客观、定量的脑电图测量方法，与认知功能过程和功能性语言磁共振分析有关。
