{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,9]],"date-time":"2026-10-09T16:15:02Z","timestamp":1791562502643,"version":"4.3.4"},"reference-count":15,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2019,8,16]],"date-time":"2019-08-16T00:00:00Z","timestamp":1565913600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Epilepsia"],"published-print":{"date-parts":[[2019,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objective<\/jats:title><jats:p>To determine the underlying etiologies in a contemporary cohort of infants with infantile spasms and to examine response to treatment.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Identification of the underlying etiology and response to treatment in 377 infants enrolled in a clinical trial of the treatment of infantile spasms between 2007 and 2014 using a systematic review of history, examination, and investigations. They were classified using the pediatric adaptation of International Classification of Diseases, Tenth Revision (<jats:styled-content style=\"fixed-case\">ICD<\/jats:styled-content>\u201010).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>A total of 219 of 377 (58%) had a proven etiology, of whom 128 (58%) responded, 58 of 108 (54%) were allocated hormonal treatment, and 70 of 111 (63%) had combination therapy. Fourteen of 17 (82%, 95% <jats:styled-content style=\"fixed-case\">confidence interval [CI]<\/jats:styled-content> 59% to 94%) infants with stroke and infarct responded (compared to 114 of 202 for the rest of the proven etiology group (56%, 95% <jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content> 48% to 62%, chi\u2010square 4.3, <jats:italic>P<\/jats:italic>\u00a0=\u00a0.037): the better response remains when treatment allocation and lead time are taken into account (odds ratio 5.1, 95% <jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content> 1.1 to 23.6, <jats:italic>P<\/jats:italic>\u00a0=\u00a0.037). Twenty of 37 (54%, 95% <jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content> 38% to 70%) infants with Down syndrome had cessation of spasms compared to 108 of 182 (59%, 95% <jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content> 52% to 66%, chi\u2010square 0.35, <jats:italic>P<\/jats:italic>\u00a0=\u00a0.55) for the rest of the proven etiology group. The lack of a significant difference remains after taking treatment modality and lead\u2010time into account (odds ratio 0.8, 95% <jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content> 0.4 to 1.7, <jats:italic>P<\/jats:italic>\u00a0=\u00a0.62). In Down syndrome infants, treatment modality did not appear to affect response: 11 of 20 (55%) allocated hormonal therapy responded, compared to 9 of 17 (53%) allocated combination therapy.<\/jats:p><\/jats:sec><jats:sec><jats:title>Significance<\/jats:title><jats:p>This classification allows easy comparison with other classifications and with our earlier reports. Stroke and infarct have a better outcome than other etiologies, whereas Down syndrome might not respond to the addition of vigabatrin to hormonal treatment.<\/jats:p><\/jats:sec>","DOI":"10.1111\/epi.16305","type":"journal-article","created":{"date-parts":[[2019,8,16]],"date-time":"2019-08-16T15:45:23Z","timestamp":1565970323000},"page":"1861-1869","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":91,"title":["The underlying etiology of infantile spasms (West syndrome): Information from the International Collaborative Infantile Spasms Study (<scp>ICISS<\/scp>)"],"prefix":"10.1111","volume":"60","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6602-0894","authenticated-orcid":false,"given":"John P.","family":"Osborne","sequence":"first","affiliation":[{"name":"Department for Health University of Bath Bath UK"},{"name":"Children's Department Royal United Hospitals Bath NHS Foundation Trust Bath UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stuart W.","family":"Edwards","sequence":"additional","affiliation":[{"name":"Department for Health University of Bath Bath UK"},{"name":"Children's Department Royal United Hospitals Bath NHS Foundation Trust Bath UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fabienne","family":"Dietrich Alber","sequence":"additional","affiliation":[{"name":"Division of Neurology\/Neuropsychology University Children's Hospital Zurich Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eleanor","family":"Hancock","sequence":"additional","affiliation":[{"name":"Children's Department Royal United Hospitals Bath NHS Foundation Trust Bath UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anthony L.","family":"Johnson","sequence":"additional","affiliation":[{"name":"Medical Research Council Clinical Trials Unit at UCL Institute of Clinical Trials and Methodology London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Colin R.","family":"Kennedy","sequence":"additional","affiliation":[{"name":"Clinical Neurosciences Faculty of Medicine University of Southampton Southampton General Hospital Southampton UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marcus","family":"Likeman","sequence":"additional","affiliation":[{"name":"Department of Paediatric Radiology Bristol Royal Hospital for Children Bristol UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew L.","family":"Lux","sequence":"additional","affiliation":[{"name":"Department of Paediatric Neurology Bristol Royal Hospital for Children Bristol UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mark","family":"Mackay","sequence":"additional","affiliation":[{"name":"Neurology Department The Royal Children's Hospital Melbourne Parkville Vic. 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