Some children have continuous epileptic activity in their brains at night, while they seldom if ever have epileptic seizures during the day. This relatively invisible form of epilepsy is called ESES syndrome. As a result, children often develop learning and behavioral problems. Not much is yet known about this syndrome. Medical researcher Bart van den Munckhof obtained his PhD in mid-October for research into causes, early recognition and better treatment.

ESES stands for Electrical Status Epilepticus Sleep. It is a kind of short circuit in the brain, which hinders recovery in sleep. Bart explains, “During the day new brain connections are constantly being made through everything you experience and learn. During sleep those connections are usually classified and tidied up. That which must be preserved is given a good place and that which is superfluous is broken down. If this goes well, the brain will have room again the next day to make new connections. With ESES, the short-circuits disturb this classification greatly, freeing up less room for new connections. The head is, as it were, still too full. In our research we measured the brain activity of children with ESES in sleep. We have found that the epileptic activity does in fact disrupt the normal recovery function of sleep. This disruption was the clearest in children who have not only learning problems but also behavioral problems.”

Damage control

It is not known how often ESES occur, because parents do not always notice that their child suffers from it: it just seems to sleep well. Of the children who sometimes or often have epileptic seizures during the day, a small percentage – less than two percent – also have ESES. In puberty children almost always grow over it, but until then it may have caused considerable damage. If the diagnosis is made in good time, treatment can be started to limit the damage.

In his research Bart found that ninety percent of children who suffer damage to the thalamus (an essential control center deep inside the brain) around birth will eventually get ESES. “The severity of that thalamic damage predicts how these children will develop in the long run. In addition, we found that increased inflammatory activity plays a role in the development of epilepsy and, in particular, ESES. As such we advocate that all children who have thalamic damage or inflammation of the brain should be monitored by a neonatologist and pediatric neurologist to recognize ESES at an early stage.”

Which treatment

By means of a meta-analysis of the literature and a study at the UMC Utrecht’s epilepsy center, Bart corroborated the theory that normal epilepsy drugs (antiepileptics) are often ineffective in the treatment of ESES. “Whereas tranquilizers (benzodiazepines) or anti-inflammatory drugs (corticosteroids) prove to be more effective. A European randomized trial, RESCUE ESES, is currently underway, which compares treatment with a tranquilizer (clobazam) with treatment with anti-inflammatory drugs (corticosteroids). In addition, we are looking whether, by measuring inflammatory activity, we can predict which children will and will not respond to treatment. The results of this study are likely to be useful in a few years’ time.”

The studies for which Bart obtained his PhD make an important contribution to the knowledge about ESES and can help in the early recognition and treatment of this severe epilepsy syndrome. Bart says, “In this way we hope to improve the development of children with ESES over time.”