{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,22]],"date-time":"2026-06-22T23:49:57Z","timestamp":1782172197062,"version":"3.54.5"},"reference-count":51,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2021,3,30]],"date-time":"2021-03-30T00:00:00Z","timestamp":1617062400000},"content-version":"vor","delay-in-days":57,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100006108","name":"National Center for Advancing Translational Sciences","doi-asserted-by":"publisher","award":["UL1TR002384"],"award-info":[{"award-number":["UL1TR002384"]}],"id":[{"id":"10.13039\/100006108","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000030","name":"Centers for Disease Control and Prevention","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000030","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Epileptic Disorders"],"published-print":{"date-parts":[[2021,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p><jats:italic>Objective<\/jats:italic>: Corticosteroids are commonly used to treat refractory epilepsy in children, but the heterogeneity of the population and lack of standardized outcome measures have limited understanding of their effectiveness. We conducted a single\u2010center study of corticosteroids for epileptic encephalopathy to (a) identify domains for measurement and estimate potential effect sizes, (b) characterize heterogeneity, and (c) identify outcomes that may need better tools for measurement.<\/jats:p><jats:p><jats:italic>Methods<\/jats:italic>: In this retrospective single\u2010center cohort study, children with epileptic encephalopathy (excluding infantile spasms) were treated with a standardized course of oral dexamethasone or IV methylprednisolone. Long\u2010term video electroencephalography (EEG) was assessed via novel ordinal scales for five features: seizure semiology\/burden, epileptiform activity, slowing, organization, and sleep architecture. We abstracted parental assessment of functional domains (<jats:italic>i.e<\/jats:italic>., cognition) from the medical records. Pre\u2010treatment and post\u2010treatment EEG features, functional domains, and treatment regimens were compared.<\/jats:p><jats:p><jats:italic>Results<\/jats:italic>: Thirty\u2010five children with refractory epilepsy were included. Overall, 16\/35 (46%) of individuals had a &gt;50% reduction in seizure frequency from the pre\u2010treatment EEG to the initial post\u2010treatment EEG. In particular, tonic seizures (in a subset of 23 children) were reduced (24\u2010hour tonic seizure count pre\u2010treatment was 8 [4\u201013] and 3 [1\u20105] post\u2010treatment EEG#1, <jats:italic>p<\/jats:italic>=0.04). For follow\u2010up post\u2010treatment EEGs, there was: (1) better formation of sleep spindles (37% normal pre\u2010treatment to 63% normal post\u2010treatment; <jats:italic>p<\/jats:italic>=0.04); and (2) improvement in parental reported cognition (in 43%). Improved cognition was the only outcome that differed between the dexamethasone and methylprednisolone treated groups (58% for dexamethasone [<jats:italic>n<\/jats:italic>=11\/19] vs. 25% for methylprednisolone [<jats:italic>n<\/jats:italic>=4\/16]; <jats:italic>p<\/jats:italic>=0.03).<\/jats:p><jats:p><jats:italic>Significance<\/jats:italic>: Large studies should be powered to detect reductions in seizures (particularly tonic as we identified a 2.6\u2010fold reduction), improved EEG organization, and improved sleep architecture (21 percentage points). Cognitive improvements following steroid treatment, reported by parents, should be quantified and fully characterized in future work.<\/jats:p>","DOI":"10.1684\/epd.2021.1247","type":"journal-article","created":{"date-parts":[[2021,3,15]],"date-time":"2021-03-15T13:11:02Z","timestamp":1615813862000},"page":"111-122","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["Measurable outcomes for pediatric epileptic encephalopathy: a single\u2010center experience with corticosteroid therapy"],"prefix":"10.1002","volume":"23","author":[{"given":"Jacqueline S.","family":"Gofshteyn","sequence":"first","affiliation":[{"name":"Division of Child Neurology, Department of Pediatrics Weill Cornell Medicine  New York NY USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kevin","family":"Gurcharran","sequence":"additional","affiliation":[{"name":"Division of Child Neurology, Department of Pediatrics Weill Cornell Medicine  New York NY USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Belinda O.","family":"Marquis","sequence":"additional","affiliation":[{"name":"Division of Child Neurology, Department of Pediatrics Weill Cornell Medicine  New York NY USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jacqueline","family":"Lamothe","sequence":"additional","affiliation":[{"name":"Department of Pediatrics New York Presbyterian Hospital  New York NY USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dena","family":"Gourley","sequence":"additional","affiliation":[{"name":"Department of Pediatrics New York Presbyterian Hospital  New York NY USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zachary","family":"Grinspan","sequence":"additional","affiliation":[{"name":"Division of Child Neurology, Department of Pediatrics Weill Cornell Medicine  New York NY USA"},{"name":"Department of Healthcare Policy &amp; Research Weill Cornell Medicine  New York NY USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Srishti","family":"Nangia","sequence":"additional","affiliation":[{"name":"Division of Child Neurology, Department of Pediatrics Weill Cornell Medicine  New York NY USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2021,3,30]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.yebeh.2012.06.016"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.15585\/mmwr.mm6631a1"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/epi.14569"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.3389\/fneur.2017.00505"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pediatrneurol.2017.07.008"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(04)17400-X"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(05)70199-X"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.5698\/1535-7597.17.4.214"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/epi.13128"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0887-8994(02)00513-1"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.seizure.2005.07.002"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejpn.2015.04.006"},{"issue":"6","key":"e_1_2_8_14_1","article-title":"Corticosteroids including ACTH for childhood epilepsy other than epileptic spasms","author":"Mehta V","year":"2015","journal-title":"Cochrane database Syst Rev"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/epi.13306"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.seizure.2018.10.016"},{"issue":"3","key":"e_1_2_8_17_1","first-page":"173","article-title":"Intravenous methylprednisolone pulse therapy for children with epileptic encephalopathy","volume":"30","author":"Pera M.C.","year":"2015","journal-title":"Funct Neurol"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1111\/epi.13750"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamaneurol.2017.3949"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1227\/NEU.0000000000001165"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(08)70092-9"},{"key":"e_1_2_8_22_1","article-title":"Ketogenic diet and other dietary treatments for epilepsy","volume":"2","author":"Martin K.","year":"2016","journal-title":"Cochrane database Syst Rev"},{"key":"e_1_2_8_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejpn.2007.09.003"},{"key":"e_1_2_8_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pediatrneurol.2009.07.011"},{"key":"e_1_2_8_25_1","unstructured":"NordliDR.Clinical protocols as developed by Douglas R Nordli. 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