The difficulties in treating children with rare epileptic encephalopathies

[...]the results of a meta-analysis suggested superior efficacy of corticosteroids over benzodiazepines in cognitive outcomes on the basis of retrospective data.6 No head-to-head comparison of these two drugs has been done, but this knowledge gap is now filled by the long-awaited publication, by Marleen van Arnhem and colleagues in The Lancet Neurology, of the results of the RESCUE-ESES trial.7 RESCUE-ESES was a randomised non-blinded comparison of treatment with corticosteroids (either continuous treatment with 1–2 mg/kg per day of prednisolone orally or pulse treatment with 20 mg/kg per day of methylprednisolone intravenously for 3 days every 4 weeks) or clobazam (0·5–1·2 mg/kg per day orally) for 6 months. The difference in IQ responder rate was no longer apparent at the 18-month reanalysis, but there were significant confounders (including attrition), and treatments administered by the treating physicians differed, which might have had an effect. Selective reduction of synaptic connections occurs overnight during slow-wave sleep (recuperative synaptic downscaling).8 Synaptic homoeostasis is closely correlated with slow-wave activity.9 However, the study by van Arnhem and colleagues7 did not show a significant correlation between cognitive scores and spike-wave index.