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Summary

Epilepsy with electrical status epilepticus in sleep (ESES) is a childhood epilepsy syndrome characterized by near-continuous epileptic activity in sleep and learning or behavioral problems. Children with ESES may have occasional visible epileptic seizures, but the epileptic activity in sleep is generally invisible. The aim of my PhD program was to 1) improve early recognition of children at risk of developing ESES, 2) to increase our knowledge of the causes of ESES and reveal mechanisms that lead to learning and behavioral problems and 3) to provide evidence on optimal treatment. We found that nearly all children with perinatal injury of the thalamus, an important regulatory brain structure located centrally in the brain, develop ESES during follow up and the severity of thalamic injury predicts their long-term development. We also found that excessive inflammation plays a role in children with epilepsy and especially in children with ESES. By measuring brain activity in sleep, we found that the epileptic activity in sleep locally disrupts the normal recovery function of sleep and this disruption is most severe in children with behavioral problems. In a meta-analysis of literature and a study within our own center, we found that normal anti-epileptic drugs are often not effective in treating ESES, whilst benzodiazepines (such as clobazam) and anti-inflammatory treatment (corticosteroids) are more effective. A randomized study comparing clobazam and corticosteroids in children with ESES (acronym: RESCUE ESES) is still ongoing.