{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,8]],"date-time":"2025-12-08T22:25:14Z","timestamp":1765232714014,"version":"3.40.5"},"reference-count":27,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2019,4,8]],"date-time":"2019-04-08T00:00:00Z","timestamp":1554681600000},"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":["Develop Med Child Neuro"],"published-print":{"date-parts":[[2020,3]]},"abstract":"<jats:sec><jats:title>Aim<\/jats:title><jats:p>To evaluate the utility of Ability Captured Through Interactive Video Evaluation (<jats:styled-content style=\"fixed-case\">ACTIVE<\/jats:styled-content>) scaled scores to quantify meaningful change in individuals with spinal muscular atrophy (<jats:styled-content style=\"fixed-case\">SMA<\/jats:styled-content>) types 2 or 3 due to disease progression or treatment.<\/jats:p><\/jats:sec><jats:sec><jats:title>Method<\/jats:title><jats:p><jats:styled-content style=\"fixed-case\">ACTIVE<\/jats:styled-content> is a custom\u2010designed video game that measures workspace volume (<jats:styled-content style=\"fixed-case\">WSV<\/jats:styled-content>). Participants included 62 individuals with <jats:styled-content style=\"fixed-case\">SMA<\/jats:styled-content> (mean age [<jats:styled-content style=\"fixed-case\">SD<\/jats:styled-content>] 10y 9mo [5y], range 2y 9mo\u201324y) and 362 frequency\u2010matched controls (mean age [<jats:styled-content style=\"fixed-case\">SD<\/jats:styled-content>] 10y 9mo [3y 6mo], range 3y 2mo\u201324y 9mo). Participants completed <jats:styled-content style=\"fixed-case\">ACTIVE<\/jats:styled-content>, other traditional assessments, and patient\u2010reported outcomes. Responsiveness to change was evaluated by comparing longitudinal data on untreated participants to those receiving Spinraza.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p><jats:styled-content style=\"fixed-case\">ACTIVE<\/jats:styled-content> was significantly correlated to the Hammersmith Functional Motor Scales Expanded and Revised Upper Limb Module (<jats:italic>\u03c1<\/jats:italic>=0.85 and <jats:italic>\u03c1<\/jats:italic>=0.92 respectively; <jats:italic>p<\/jats:italic>&lt;0.001). Relevance to patients and families was established by strong correlations to the Patient Reported Outcomes Measurement Information System self\u2010 and parent proxy\u2010measures of upper extremity ability (<jats:italic>\u03c1<\/jats:italic>=0.63 and <jats:italic>\u03c1<\/jats:italic>=0.70 respectively; <jats:italic>p<\/jats:italic>&lt;0.001). Responsiveness to change was demonstrated by significant change in scaled scores after treatment (median 15.9 points, Wilcoxon signed\u2010rank test <jats:italic>p<\/jats:italic>&lt;0.01). A preliminary minimum clinically important difference is presented.<\/jats:p><\/jats:sec><jats:sec><jats:title>Interpretation<\/jats:title><jats:p>These results suggest that <jats:styled-content style=\"fixed-case\">ACTIVE WSV<\/jats:styled-content> scores are a meaningful assessment with which to quantify change over time in individuals with <jats:styled-content style=\"fixed-case\">SMA<\/jats:styled-content> types 2 and 3.<\/jats:p><\/jats:sec><jats:sec><jats:title>What this paper adds<\/jats:title><jats:p>\n<jats:list list-type=\"bullet\">\n\n<jats:list-item><jats:p>Ability Captured Through Interactive Video Evaluation (ACTIVE) quantifies upper extremity function in spinal muscular atrophy.<\/jats:p><\/jats:list-item>\n\n<jats:list-item><jats:p>ACTIVE's scaled workspace volume strongly correlates to self\u2010 and parent\u2010report of function.<\/jats:p><\/jats:list-item>\n\n<jats:list-item><jats:p>ACTIVE quantifies meaningful change after treatment.<\/jats:p><\/jats:list-item>\n<\/jats:list>\n<\/jats:p><\/jats:sec>","DOI":"10.1111\/dmcn.14230","type":"journal-article","created":{"date-parts":[[2019,4,9]],"date-time":"2019-04-09T05:52:59Z","timestamp":1554789179000},"page":"303-309","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["ACTIVE (Ability Captured Through Interactive Video Evaluation) workspace volume video game to quantify meaningful change in spinal muscular atrophy"],"prefix":"10.1111","volume":"62","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2263-7569","authenticated-orcid":false,"given":"Lindsay N","family":"Alfano","sequence":"first","affiliation":[{"name":"Center for Gene Therapy Nationwide Children's Hospital Columbus OH USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0573-9504","authenticated-orcid":false,"given":"Natalie F","family":"Miller","sequence":"additional","affiliation":[{"name":"Center for Gene Therapy Nationwide Children's Hospital Columbus OH USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2800-3855","authenticated-orcid":false,"given":"Megan A","family":"Iammarino","sequence":"additional","affiliation":[{"name":"Center for Gene Therapy Nationwide Children's Hospital Columbus OH USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Melissa","family":"Moore Clingenpeel","sequence":"additional","affiliation":[{"name":"Biostatistics Core Research Institute at Nationwide Children's Hospital Columbus OH USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Suzanne L","family":"Lowes","sequence":"additional","affiliation":[{"name":"University of Dayton Dayton OH USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Margaret E","family":"Dugan","sequence":"additional","affiliation":[{"name":"Center for 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USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4206-0557","authenticated-orcid":false,"given":"Linda P","family":"Lowes","sequence":"additional","affiliation":[{"name":"Center for Gene Therapy Nationwide Children's Hospital Columbus OH USA"},{"name":"Department of Pediatrics The Ohio State University College of Medicine Columbus OH 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