Summary Purpose The aim of this study was to evaluate the long‐term cognitive outcome in children with continuous spikes and waves during slow wave sleep (CSWS syndrome). Methods We reviewed the neuropsychological tests of 25 children diagnosed with CSWS between 1987 and 2010 and with a mean follow‐up of 13.5 years. Key Findings Cognitive performances worsened during CSWS in virtually all patients. Seven patients (28%) with nonlesional epilepsy had a positive outcome; three patients (12%) showed persistence of motor deficit without involvement of cognitive functions; and seven patients (28%) who presented a long duration of CSWS (mean = 28.1 months) had a negative cognitive outcome. In 6 patients (24%) with structural or metabolic disorders before CSWS onset cognitive outcomes did not change; 2 patients (8%) had a negative outcome irrespective of the duration or presence of other neurologic disorders before CSWS onset. Forty‐four percent of children with CSWS demonstrated permanent cognitive impairment. Significance The long‐term outcome of CSWS syndrome is variable and seems to depend on treatment response, disease duration, and underlying etiology.

Epileptic encephalopathies are age-dependent conditions, typically drug resistant, characterized by severe cognitive impairment due to the epileptic activity itself. Corticosteroid therapy with adrenocorticotrophic hormone, hydrocortisone, prednisone or methylprednisolone is one of the therapeutic approaches tried in these children. Although the first use of steroids as antiepileptic drugs dates back to 1950, there is no wide agreement about the ideal treatment regimen in epileptic encephalopathies other than West syndrome; no evidence from class I and II studies are available at the moment. In this study, we present a literature review on steroid therapy in childhood epilepsy with special regard to effectiveness, tolerability and possible mechanisms of action.