{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T16:32:24Z","timestamp":1791304344979,"version":"4.3.1"},"reference-count":25,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2016,10,21]],"date-time":"2016-10-21T00:00:00Z","timestamp":1477008000000},"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":[[2017,4]]},"abstract":"<jats:sec><jats:title>Aim<\/jats:title><jats:p>To determine whether ataxia rating scales are reliable disease biomarkers for early onset ataxia (<jats:styled-content style=\"fixed-case\">EOA<\/jats:styled-content>).<\/jats:p><\/jats:sec><jats:sec><jats:title>Method<\/jats:title><jats:p>In 40 patients clinically identified with <jats:styled-content style=\"fixed-case\">EOA<\/jats:styled-content> (28 males, 12 females; mean age 15y 3mo [range 5\u201334y]), we determined interobserver and intraobserver agreement (interclass correlation coefficient [<jats:styled-content style=\"fixed-case\">ICC<\/jats:styled-content>]) and discriminant validity of ataxia rating scales (International Cooperative Ataxia Rating Scale [<jats:styled-content style=\"fixed-case\">ICARS<\/jats:styled-content>], Scale for Assessment and Rating of Ataxia [<jats:styled-content style=\"fixed-case\">SARA<\/jats:styled-content>], and Brief Ataxia Rating Scale [<jats:styled-content style=\"fixed-case\">BARS<\/jats:styled-content>]). Three paediatric neurologists independently scored <jats:styled-content style=\"fixed-case\">ICARS<\/jats:styled-content>,<jats:styled-content style=\"fixed-case\"> SARA<\/jats:styled-content> and <jats:styled-content style=\"fixed-case\">BARS<\/jats:styled-content> performances recorded on video, and also phenotyped the primary and secondary movement disorder features. When ataxia was the primary movement disorder feature, we assigned patients to the subgroup \u2018<jats:styled-content style=\"fixed-case\">EOA<\/jats:styled-content> with core ataxia\u2019 (<jats:italic>n<\/jats:italic>=26). When ataxia concurred with other prevailing movement disorders (such as dystonia, myoclonus, and chorea), we assigned patients to the subgroup \u2018<jats:styled-content style=\"fixed-case\">EOA<\/jats:styled-content> with comorbid ataxia\u2019 (<jats:italic>n<\/jats:italic>=12).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p><jats:styled-content style=\"fixed-case\">ICC<\/jats:styled-content> values were similar in both <jats:styled-content style=\"fixed-case\">EOA<\/jats:styled-content> subgroups of \u2018core\u2019 and \u2018comorbid\u2019 ataxia (0.92\u20130.99; <jats:styled-content style=\"fixed-case\">ICARS<\/jats:styled-content>,<jats:styled-content style=\"fixed-case\"> SARA<\/jats:styled-content>, and <jats:styled-content style=\"fixed-case\">BARS<\/jats:styled-content>). Independent of the phenotype, the severity of the prevailing movement disorder predicted the ataxia rating scale scores (<jats:italic>\u03b2<\/jats:italic>=0.83\u20130.88; <jats:italic>p<\/jats:italic>&lt;0.05).<\/jats:p><\/jats:sec><jats:sec><jats:title>Interpretation<\/jats:title><jats:p>In patients with <jats:styled-content style=\"fixed-case\">EOA<\/jats:styled-content>, the reliability of ataxia rating scales is high. However, the discriminative validity for \u2018ataxia\u2019 is low. For adequate interpretation of ataxia rating scale scores, application in uniform movement disorder phenotypes is essential.<\/jats:p><\/jats:sec>","DOI":"10.1111\/dmcn.13291","type":"journal-article","created":{"date-parts":[[2016,10,21]],"date-time":"2016-10-21T10:02:43Z","timestamp":1477044163000},"page":"427-432","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":42,"title":["Reliability and discriminant validity of ataxia rating scales in early onset ataxia"],"prefix":"10.1111","volume":"59","author":[{"given":"Rick","family":"Brandsma","sequence":"first","affiliation":[{"name":"Department of Neurology Beatrix Children's Hospital University Medical Center Groningen University of Groningen Groningen the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tjitske F","family":"Lawerman","sequence":"additional","affiliation":[{"name":"Department of Neurology Beatrix Children's Hospital University Medical Center Groningen University of Groningen Groningen the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marieke J","family":"Kuiper","sequence":"additional","affiliation":[{"name":"Department of Neurology Beatrix Children's Hospital University Medical Center Groningen University of Groningen Groningen the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roelineke J","family":"Lunsing","sequence":"additional","affiliation":[{"name":"Department of Neurology Beatrix Children's Hospital University Medical Center Groningen University of Groningen Groningen the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Huibert","family":"Burger","sequence":"additional","affiliation":[{"name":"Department of General Practice University Medical Center Groningen University of Groningen Groningen the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Deborah A","family":"Sival","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Beatrix Children's Hospital University Medical Center Groningen University of Groningen Groningen the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2016,10,21]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/B978-0-7506-9852-8.00013-8"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-0004.1993.tb04450.x"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awp056"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1177\/0883073803018001S0301"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/dmcn.12804"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-510X(96)00231-6"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000219042.60538.92"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.22681"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.20781"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jns.2007.09.021"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.21544"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1111\/dmcn.12369"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1469-8749.2011.03931.x"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1469-8749.2009.03334.x"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.earlhumdev.2009.09.002"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(16)00131-9"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(15)00217-3"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/121.4.589"},{"key":"e_1_2_7_20_1","doi-asserted-by":"publisher","DOI":"10.20982\/tqmp.08.1.p023"},{"key":"e_1_2_7_21_1","doi-asserted-by":"publisher","DOI":"10.1037\/1040-3590.6.4.284"},{"key":"e_1_2_7_22_1","doi-asserted-by":"publisher","DOI":"10.2307\/2529310"},{"key":"e_1_2_7_23_1","volume-title":"Applied Regression Analysis and Other Multivariate Analysis Methods","author":"Kleinbaum DG","year":"1988"},{"key":"e_1_2_7_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(15)00201-X"},{"key":"e_1_2_7_25_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejpn.2007.11.006"},{"key":"e_1_2_7_26_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jns.2014.04.001"}],"container-title":["Developmental Medicine &amp; 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