{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T13:52:02Z","timestamp":1790171522096,"version":"4.1.0"},"reference-count":58,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2024,2,27]],"date-time":"2024-02-27T00:00:00Z","timestamp":1708992000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Epilepsia"],"published-print":{"date-parts":[[2024,5]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>We conducted a systematic review investigating the efficacy and tolerability of adrenocorticotropic hormone (ACTH) and corticosteroids in children with epilepsies other than infantile epileptic spasm syndrome (IESS) that are resistant to anti\u2010seizure medication (ASM). We included retrospective and prospective studies reporting on more than five patients and with clear case definitions and descriptions of treatment and outcome measures. We searched multiple databases and registries, and we assessed the risk of bias in the selected studies using a questionnaire based on published templates. Results were summarized with meta\u2010analyses that pooled logit\u2010transformed proportions or rates. Subgroup analyses and univariable and multivariable meta\u2010regressions were performed to examine the influence of covariates. We included 38 studies (2 controlled and 5 uncontrolled prospective; 31 retrospective) involving 1152 patients. Meta\u2010analysis of aggregate data for the primary outcomes of seizure response and reduction of electroencephalography (EEG) spikes at the end of treatment yielded pooled proportions (PPs) of 0.60 (95% confidence interval [CI] 0.52\u20130.67) and 0.56 (95% CI 0.43\u20130.68). The relapse rate was high (PP 0.33, 95% CI 0.27\u20130.40). Group analyses and meta\u2010regression showed a small benefit of ACTH and no difference between all other corticosteroids, a slightly better effect in electric status epilepticus in slow sleep (ESES) and a weaker effect in patients with cognitive impairment and \u201csymptomatic\u201d etiology. Obesity and Cushing's syndrome were the most common adverse effects, occurring more frequently in trials addressing continuous ACTH (PP 0.73, 95% CI 0.48\u20130.89) or corticosteroids (PP 0.72, 95% CI 0.54\u20130.85) than intermittent intravenous or oral corticosteroid administration (PP 0.05, 95% CI 0.02\u20130.10). The validity of these results is limited by the high risk of bias in most included studies and large heterogeneity among study results. This report was registered under International Prospective Register of Systematic Reviews (PROSPERO) number CRD42022313846. We received no financial support.<\/jats:p>","DOI":"10.1111\/epi.17918","type":"journal-article","created":{"date-parts":[[2024,2,27]],"date-time":"2024-02-27T10:50:20Z","timestamp":1709031020000},"page":"1155-1175","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["Efficacy and safety of corticosteroids and ACTH in epileptic syndromes beyond Infantile Epileptic Spasms Syndrome (IESS): A systematic review and meta\u2010analysis"],"prefix":"10.1111","volume":"65","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4638-3460","authenticated-orcid":false,"given":"Rudolf","family":"Korinthenberg","sequence":"first","affiliation":[{"name":"Division of Neuropediatrics and Muscular Disorders, Faculty of Medicine University Medical Center (UMC), University of Freiburg  Freiburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Bast","sequence":"additional","affiliation":[{"name":"Epilepsy Center Kork  Kehl Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Edda","family":"Haberlandt","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Adolescent Medicine Dornbirn Hospital  Dornbirn Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ulrich","family":"Stephani","sequence":"additional","affiliation":[{"name":"Clinic for Children and Adolescents II (Neuropediatrics, Social Pediatrics) University Hospital Schleswig Holstein (UKSH)  Kiel Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6288-9915","authenticated-orcid":false,"given":"Adam","family":"Strzelczyk","sequence":"additional","affiliation":[{"name":"Epilepsy Center Frankfurt Rhine\u2010Main, Department of Neurology Goethe University and University Hospital Frankfurt  Frankfurt am Main Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gerta","family":"R\u00fccker","sequence":"additional","affiliation":[{"name":"Medical Faculty and Medical Center Institute for Medical Biometry and Statistics, University of Freiburg  Freiburg 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