{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,2]],"date-time":"2026-09-02T22:25:58Z","timestamp":1788387958618,"version":"build-2803163510"},"reference-count":33,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2022,1,29]],"date-time":"2022-01-29T00:00:00Z","timestamp":1643414400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Develop Med Child Neuro"],"published-print":{"date-parts":[[2022,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Aim<\/jats:title><jats:p>To estimate the prevalence, and evaluate presentation, treatment response, treatment side effects, and long\u2010term seizure outcomes in all known cases of children with Down syndrome and infantile spasms on the island of Ireland.<\/jats:p><\/jats:sec><jats:sec><jats:title>Method<\/jats:title><jats:p>This was a 10\u2010year retrospective multicentre review of clinical records and investigations, focusing on treatment response, side effects, and long\u2010term outcomes.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The prevalence of infantile spasms in Down syndrome was 3.0% during the study period. Fifty\u2010four infants were identified with median age of spasm onset at 201\u00a0days (interquartile range [IQR] 156\u2013242). Spasm cessation was achieved in 88% (<jats:italic>n<\/jats:italic>=46) at a median of 110 days (IQR 5\u201366). The most common first\u2010line medications were prednisolone (<jats:italic>n<\/jats:italic>=20, 37%), vigabatrin (<jats:italic>n<\/jats:italic>=18, 33.3%), and sodium valproate (<jats:italic>n<\/jats:italic>=9, 16.7%). At follow\u2010up (median age 23.7mo; IQR 13.4\u201340.6), 25% had ongoing seizures and 85% had developmental concerns. Treatment within 60 days did not correlate with spasm cessation. Seventeen children (31%) experienced medication side effects, with vigabatrin accounting for 52%.<\/jats:p><\/jats:sec><jats:sec><jats:title>Interpretation<\/jats:title><jats:p>Prednisolone is an effective and well\u2010tolerated medication for treating infantile spasms in Down syndrome. Despite the high percentage of spasm cessation, developmental concerns and ongoing seizures were common.<\/jats:p><\/jats:sec>","DOI":"10.1111\/dmcn.15153","type":"journal-article","created":{"date-parts":[[2022,1,29]],"date-time":"2022-01-29T19:14:35Z","timestamp":1643483675000},"page":"780-788","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["Response to treatment and outcomes of infantile spasms in Down\u00a0syndrome"],"prefix":"10.1111","volume":"64","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4663-0780","authenticated-orcid":false,"given":"Susan","family":"Harvey","sequence":"first","affiliation":[{"name":"Department of Neurology and Clinical Neurophysiology Children\u2019s Health Ireland at Temple Street  Dublin Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8678-7507","authenticated-orcid":false,"given":"Nicholas M.","family":"Allen","sequence":"additional","affiliation":[{"name":"School of Medicine National University of Ireland Galway  Galway Ireland"},{"name":"Department of Paediatrics Galway University Hospital  Galway Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mary D.","family":"King","sequence":"additional","affiliation":[{"name":"Department of Neurology and Clinical Neurophysiology Children\u2019s Health Ireland at Temple Street  Dublin Ireland"},{"name":"School of Medicine and Medical Science University College Dublin  Dublin Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bryan","family":"Lynch","sequence":"additional","affiliation":[{"name":"Department of Neurology and Clinical Neurophysiology Children\u2019s Health Ireland at Temple Street  Dublin Ireland"},{"name":"School of Medicine and Medical Science University College Dublin  Dublin Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3540-1333","authenticated-orcid":false,"given":"Sally A.","family":"Lynch","sequence":"additional","affiliation":[{"name":"School of Medicine and Medical Science University College Dublin  Dublin Ireland"},{"name":"National Rare Disease Office Mater Hospital Dublin  Dublin Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mary","family":"O\u2019Regan","sequence":"additional","affiliation":[{"name":"Department of Neurology and Clinical Neurophysiology Children\u2019s Health Ireland at Crumlin  Dublin Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Declan","family":"O\u2019Rourke","sequence":"additional","affiliation":[{"name":"Department of Neurology and Clinical Neurophysiology Children\u2019s Health Ireland at Temple Street  Dublin Ireland"},{"name":"School of Medicine and Medical Science University College Dublin  Dublin Ireland"},{"name":"School of Medicine Trinity College Dublin  Dublin Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0205-9220","authenticated-orcid":false,"given":"Amre","family":"Shahwan","sequence":"additional","affiliation":[{"name":"Department of Neurology and Clinical Neurophysiology Children\u2019s Health Ireland at Temple Street  Dublin Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0762-8277","authenticated-orcid":false,"given":"David","family":"Webb","sequence":"additional","affiliation":[{"name":"Department of Neurology and Clinical Neurophysiology Children\u2019s Health Ireland at Crumlin  Dublin Ireland"},{"name":"School of Medicine Trinity College Dublin  Dublin Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5488-1507","authenticated-orcid":false,"given":"Kathleen M.","family":"Gorman","sequence":"additional","affiliation":[{"name":"Department of Neurology and Clinical Neurophysiology Children\u2019s Health Ireland at Temple Street  Dublin Ireland"},{"name":"School of Medicine and Medical Science University College Dublin  Dublin Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"Irish Paediatric Neurology Group","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2022,1,29]]},"reference":[{"key":"e_1_2_10_2_1","unstructured":"Registration. 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