Sleep slow wave homeostasis and cognitive functioning in children with electrical status epilepticus in sleep.

STUDY OBJECTIVES: Encephalopathy with electrical status epilepticus in sleep (ESES) is characterised 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 behavioural problems. METHODS: 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 analysed. Differences in slow wave slope (SWS) and overnight slope course between the epileptic focus and non-focus electrodes and relations to neurodevelopment and behaviour were analysed. RESULTS: 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 behavioural 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). CONCLUSION: Slow wave homeostasis - a marker of synaptic homeostasis - is disturbed by epileptiform activity in ESES. Behavioural problems, but not neurodevelopmental test results, were related to severity of this disturbance.