{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,6]],"date-time":"2026-09-06T06:17:54Z","timestamp":1788675474121,"version":"build-2803163510"},"reference-count":18,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2026,1,24]],"date-time":"2026-01-24T00:00:00Z","timestamp":1769212800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,1,24]],"date-time":"2026-01-24T00:00:00Z","timestamp":1769212800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Epileptic Disorders"],"published-print":{"date-parts":[[2026,4]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Developmental\/Epileptic encephalopathy with spike and wave activation in sleep (DEE\u2010SWAS) comprises a spectrum of childhood\u2010onset epilepsies, characterized by near\u2010continuous spike\u2010wave discharges during non\u2010REM sleep which can result in cognitive, behavioral, and motor regression. Negative myoclonic status in the context of DEE\u2010SWAS is extremely rare, and management is difficult as conventional anti\u2010seizure medications (ASM) and immunomodulatory therapies often show limited efficacy. Adrenocorticotropic hormone (ACTH), classically used for infantile spasms, has recently been reported as a potential therapy for refractory DEE\u2010SWAS.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Case Presentation<\/jats:title>\n                    <jats:p>We report a 7\u2010year\u2010old girl with prior developmental encephalopathy, who experienced recurrent unprovoked seizures from 5\u2009years of age, followed by progressive ataxia and language regression. Clinical examination revealed facial dysmorphism, truncal and gait ataxia, oral apraxia with atonic head drops. Video EEG revealed background slowing with bilateral centro\u2010parieto\u2010temporal spikes markedly activated in sleep, with clusters of atonic neck and truncal drop seizures suggestive of negative myoclonic status leading to \u2018pseudo\u2010ataxia\u2019. Despite treatment with multiple ASM, intravenous methylprednisolone and immunoglobulin, there was worsening encephalopathy and anterior opercular syndrome. Given therapeutic refractoriness, ACTH was initiated. The patient showed dramatic improvement: resolution of status, a marked reduction in EEG spike\u2010wave burden, recovery of gait and language to baseline, and restoration of social interaction.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>This case demonstrates for the first time the potential of ACTH in refractory DEE\u2010SWAS with negative myoclonic status epilepticus, wherein conventional immunomodulation fails. The sustained clinical and electrographic improvement highlights the broader neuro\u2010modulatory mechanisms of ACTH and supports its consideration in treatment algorithms. Larger prospective studies are warranted to compare the efficacy and safety profile of various corticosteroids in DEE.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/epd2.70177","type":"journal-article","created":{"date-parts":[[2026,1,24]],"date-time":"2026-01-24T13:15:58Z","timestamp":1769260558000},"page":"496-504","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["\u2018Pseudo\u2010ataxic\u2019 negative myoclonic status in developmental and epileptic encephalopathy with spike\u2010wave activation in sleep: Utility of\n                    <scp>ACTH<\/scp>\n                    therapy beyond west syndrome"],"prefix":"10.1002","volume":"28","author":[{"given":"Manisha","family":"K.Y.","sequence":"first","affiliation":[{"name":"Department of Neurology, R. Madhavan Nayar Centre for Comprehensive Epilepsy Care Sree Chitra Tirunal Institute for Medical Sciences and Technology  Thiruvananthapuram Kerala India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8083-4264","authenticated-orcid":false,"given":"Kiren George","family":"Koshy","sequence":"additional","affiliation":[{"name":"Department of Neurology, R. Madhavan Nayar Centre for Comprehensive Epilepsy Care Sree Chitra Tirunal Institute for Medical Sciences and Technology  Thiruvananthapuram Kerala India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jayakumari","family":"Nandana","sequence":"additional","affiliation":[{"name":"Department of Neurology, R. Madhavan Nayar Centre for Comprehensive Epilepsy Care Sree Chitra Tirunal Institute for Medical Sciences and Technology  Thiruvananthapuram Kerala India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ashalatha","family":"Radhakrishnan","sequence":"additional","affiliation":[{"name":"Department of Neurology, R. Madhavan Nayar Centre for Comprehensive Epilepsy Care Sree Chitra Tirunal Institute for Medical Sciences and Technology  Thiruvananthapuram Kerala India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0972-3307","authenticated-orcid":false,"given":"Ramshekhar N.","family":"Menon","sequence":"additional","affiliation":[{"name":"Department of Neurology, R. Madhavan Nayar Centre for Comprehensive Epilepsy Care Sree Chitra Tirunal Institute for Medical Sciences and Technology  Thiruvananthapuram Kerala India"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,1,24]]},"reference":[{"key":"e_1_2_12_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1388-2457(00)00408-9"},{"key":"e_1_2_12_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1528-1167.2009.02211.x"},{"key":"e_1_2_12_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/epi.17241"},{"key":"e_1_2_12_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.seizure.2012.12.003"},{"key":"e_1_2_12_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.yebeh.2025.110308"},{"key":"e_1_2_12_7_1","doi-asserted-by":"publisher","DOI":"10.1177\/2329048X17715965"},{"key":"e_1_2_12_8_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awh661"},{"key":"e_1_2_12_9_1","doi-asserted-by":"publisher","DOI":"10.3390\/children9050759"},{"key":"e_1_2_12_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.yebeh.2020.107469"},{"key":"e_1_2_12_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1528-1167.2009.02224.x"},{"key":"e_1_2_12_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.seizure.2020.08.025"},{"key":"e_1_2_12_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0074\u20107742(02)49013\u20107"},{"key":"e_1_2_12_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.66"},{"key":"e_1_2_12_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/14651858.CD005222.pub3"},{"key":"e_1_2_12_16_1","doi-asserted-by":"publisher","DOI":"10.1111\/epi.17918"},{"key":"e_1_2_12_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neubiorev.2016.02.007"},{"key":"e_1_2_12_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.eplepsyres.2011.03.004"},{"key":"e_1_2_12_19_1","doi-asserted-by":"publisher","DOI":"10.1002\/14651858.CD013132"}],"container-title":["Epileptic Disorders"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/epd2.70177","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/epd2.70177","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/epd2.70177","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,16]],"date-time":"2026-04-16T06:44:00Z","timestamp":1776321840000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/epd2.70177"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,1,24]]},"references-count":18,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2026,4]]}},"alternative-id":["10.1002\/epd2.70177"],"URL":"https:\/\/doi.org\/10.1002\/epd2.70177","archive":["Portico"],"relation":{},"ISSN":["1294-9361","1950-6945"],"issn-type":[{"value":"1294-9361","type":"print"},{"value":"1950-6945","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,1,24]]},"assertion":[{"value":"2025-10-12","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-01-05","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-01-24","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}