Continuous spikes during slow wave sleep (CSWS) syndrome is an age-related and self-limited severe epileptic encephalopathy characterized by electrical status epilepticus in sleep (ESES) on electroencephalogram, seizures, and developmental regression. The mechanisms that lead to the development of CSWS syndrome are complex and not clear. Surprisingly, steroids and benzodiazepines offer a good treatment outcome compared to conventional anti-epileptic drugs. Of concern, this condition has a long-term poor prognosis due to the persistence of neuropsychological impairment. Many questions are yet to be answered in this syndrome. Why epileptiform discharges are age-related and self-limiting? Why spike wave discharges occur during non-rapid eye movement sleep and not during the rapid eye movement sleep? Why steroids and benzodiazepines are more efficacious compared to conventional anti-epileptic drugs? In this article, we attempt to discuss these questions by studying the relationship between sleep and hormones, cytokines, and neurotransmitters. Low levels of growth hormone and melatonin, as well as high levels of IL-6 in cases with CSWS syndrome unpin the pathomechanisms. The transient expression of GluN2A subunit, decreased GABAergic inhibition during brain development, declination of non-rapid eye movement sleep in favor of rapid eye movement sleep due to decrease of growth hormone releasing hormone in aging, neuronal loss or decrease in synaptic strength could explain the disappearance of CSWS syndrome in adolescence. Steroids seem to work in CSWS syndrome via: enhancement of GABAA inhibition, suppression of IL-6, and by favoring rapid eye movement sleep which is seizure free.

Continuous spikes during slow wave sleep syndrome; Pathomechanisms; Hormones; Cytokines; Neurotransmitters; Steroids