Kortikosteroide und adrenocorticotropes Hormon bei Epilepsien der frühen Kindheit jenseits des Säuglingsalters

This article presents a short report on a systematic review (SR) collating data on the efficacy and tolerability of adrenocorticotropic hormone (ACTH) and corticosteroids (CST) in children with epilepsies other than infantile epileptic spasm syndrome (IESS) which showed no response to antiseizure medication (ASM). The SR included retrospective and prospective studies that reported on more than five patients, with clear case definitions and descriptions of treatment and outcomes. A total of 38 (2 controlled and 5 uncontrolled prospective, 31 retrospective) studies involving 1152 patients were included. The meta-analysis of aggregated data on seizure reduction > 50% and reduction of electroencephalography (EEG) spikes at the end of initial treatment resulted in pooled proportions (PP) of 0.60 (95% confidence interval, CI 0.52–0.67) and 0.56 (95% CI 0.43–0.68), respectively. The relapse rate was high (PP 0.33, 95% CI 0.27–0.40). Subgroup analyses and meta-regression showed no significant differences between the drugs used, a slightly better effect in developmental and/or epileptic encephalopathy with spike and wave activation in sleep (DEE-SWAS) and a weaker effect in patients with cognitive impairment and symptomatic etiology. The cumulative dosage of the initial treatment phase did not affect outcomes. Obesity and Cushing’s syndrome were the most frequent adverse effects occurring in trials addressing continuous ACTH (PP 0.73, 95% CI 0.48–0.89) or CST (PP 0.72, 95% CI 0.54–0.85) administration but rarely with intermittent intravenous or oral CST administration (PP 0.05, 95% CI 0.02–0.10). The validity of these results is limited by the high risk of bias in most included studies and a large heterogeneity among study data. The original SR was published under https://doi.org/10.1111/epi.17918.