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Treatment choice and categorization of hypsarrhythmia were determined clinically at each site. Response to therapy was defined as resolution of clinical spasms (and hypsarrhythmia if present) without relapse 3 months after initiation.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Eighty\u2010two percent of patients had hypsarrhythmia, but this was not associated with gender, mean age, preexisting developmental delay or epilepsy, etiology, or response to first\u2010line therapy. Infants with hypsarrhythmia were more likely to receive standard treatment (adrenocorticotropic hormone, prednisolone, or vigabatrin [odds ratio (<jats:styled-content style=\"fixed-case\">OR<\/jats:styled-content>) 2.6, 95% confidence interval (<jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content>) 1.4\u20134.7] and preexisting epilepsy reduced the likelihood of standard treatment (<jats:styled-content style=\"fixed-case\">OR<\/jats:styled-content>3.2, 95%<jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content>1.9\u20135.4). Hypsarrhythmia was not a determinant of response to treatment. A logistic regression model demonstrated that later age of onset (<jats:styled-content style=\"fixed-case\">OR<\/jats:styled-content>1.09 per month, 95%<jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content>1.03\u20131.15) and absence of preexisting epilepsy (<jats:styled-content style=\"fixed-case\">OR<\/jats:styled-content>1.7, 95%<jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content>1.06\u20132.81) had a small impact on the likelihood of responding to the first\u2010line treatment. However, receiving standard first\u2010line treatment increased the likelihood of responding dramatically: vigabatrin (<jats:styled-content style=\"fixed-case\">OR<\/jats:styled-content>5.2 ,95%<jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content>2\u201313.7), prednisolone (<jats:styled-content style=\"fixed-case\">OR<\/jats:styled-content>8, 95%<jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content>3.1\u201320.6), and adrenocorticotropic hormone (ACTH;<jats:styled-content style=\"fixed-case\">OR<\/jats:styled-content>10.2, 95%<jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content>4.1\u201325.8) .<\/jats:p><\/jats:sec><jats:sec><jats:title>Significance<\/jats:title><jats:p>First\u2010line treatment with standard therapy was by far the most important variable in determining likelihood of response to treatment of infantile spasms with or without hypsarrhythmia.<\/jats:p><\/jats:sec>","DOI":"10.1111\/epi.13937","type":"journal-article","created":{"date-parts":[[2017,11,3]],"date-time":"2017-11-03T14:41:20Z","timestamp":1509720080000},"page":"2098-2103","source":"Crossref","is-referenced-by-count":81,"title":["The impact of hypsarrhythmia on infantile spasms treatment response: Observational cohort study from the National Infantile Spasms Consortium"],"prefix":"10.1111","volume":"58","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4544-3724","authenticated-orcid":false,"given":"Scott T.","family":"Demarest","sequence":"first","affiliation":[{"name":"Departments of Pediatrics and Neurology School of Medicine University of Colorado Anschutz Medical Campus Aurora Colorado U.S.A."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3175-3908","authenticated-orcid":false,"given":"Ren\u00e9e A.","family":"Shellhaas","sequence":"additional","affiliation":[{"name":"Departments of Pediatrics &amp; Communicable Diseases (Division of Pediatric Neurology) University of Michigan Ann Arbor Michigan U.S.A."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"William D.","family":"Gaillard","sequence":"additional","affiliation":[{"name":"Center for Neuroscience Children's National Health System Washington District of Columbia U.S.A."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cynthia","family":"Keator","sequence":"additional","affiliation":[{"name":"Jane and John Justin Neurosciences Department Cook Children's Hospital Fort Worth Texas U.S.A."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katherine C.","family":"Nickels","sequence":"additional","affiliation":[{"name":"Departments of Neurology and Pediatrics Mayo Clinic Rochester Minnesota U.S.A."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shaun A.","family":"Hussain","sequence":"additional","affiliation":[{"name":"Department of Pediatric Neurology Mattel Children's Hospital at UCLA Los Angeles California U.S.A."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tobias","family":"Loddenkemper","sequence":"additional","affiliation":[{"name":"Division of Epilepsy and Clinical Neurophysiology Department of Neurology Boston Children's Hospital Boston Massachusetts U.S.A."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anup D.","family":"Patel","sequence":"additional","affiliation":[{"name":"Departments of Neurology and Pediatrics Nationwide Children's Hospital and the Ohio State University College of Medicine Columbus Ohio U.S.A."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Russell P.","family":"Saneto","sequence":"additional","affiliation":[{"name":"Department of Neurology\/Division of Pediatric Neurology Seattle Children's Hospital University of Washington Seattle Washington 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