{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,20]],"date-time":"2025-10-20T18:24:40Z","timestamp":1760984680445},"reference-count":17,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2013,2,7]],"date-time":"2013-02-07T00:00:00Z","timestamp":1360195200000},"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":[[2013,4]]},"abstract":"<jats:sec><jats:title>Aim<\/jats:title><jats:p>To determine the epidemiology of chronic ataxia in children in <jats:styled-content style=\"fixed-case\">M<\/jats:styled-content>anitoba, <jats:styled-content style=\"fixed-case\">C<\/jats:styled-content>anada.<\/jats:p><\/jats:sec><jats:sec><jats:title>Method<\/jats:title><jats:p>A retrospective study using multiple sources and disease codes identified children (age 0\u201316y) with chronic ataxia (&gt;2mo duration or recurrent episodes of ataxia) seen at <jats:styled-content style=\"fixed-case\">W<\/jats:styled-content>innipeg <jats:styled-content style=\"fixed-case\">C<\/jats:styled-content>hildren's <jats:styled-content style=\"fixed-case\">H<\/jats:styled-content>ospital from 1991 to 2008. Patients with isolated peripheral nerve diseases, vestibular disorders, or brain tumors were excluded.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>We identified 184 patients (males=females; mean age 15y, <jats:styled-content style=\"fixed-case\">SD<\/jats:styled-content> 7y 8mo) with chronic ataxia. Median age at the presenting symptom onset was 1\u00a0year 3\u00a0months and at ataxia onset 3\u00a0years 1\u00a0month. Median duration of follow\u2010up was 6\u00a0years 5\u00a0months. During the study period, the crude incidence rate was 5.77 in 10\u00a0000; the crude prevalence rate was 6.59 in 10\u00a0000; and the crude mortality rate 0.446 in 10\u00a0000. The most common presenting symptoms were developmental delay, ataxia, or seizures. The most common diagnoses (known in 129) were <jats:styled-content style=\"fixed-case\">A<\/jats:styled-content>ngelman syndrome (<jats:italic>n<\/jats:italic>=16), ataxia telangiectasia (<jats:italic>n<\/jats:italic>=13), mitochondrial disease (<jats:italic>n<\/jats:italic>=9), <jats:styled-content style=\"fixed-case\">F<\/jats:styled-content>riedreich ataxia (<jats:italic>n<\/jats:italic>=7), stroke (<jats:italic>n<\/jats:italic>=7), and familial\/genetic episodic ataxia (<jats:italic>n<\/jats:italic>=7).<\/jats:p><\/jats:sec><jats:sec><jats:title>Interpretation<\/jats:title><jats:p>Chronic ataxia is a relatively common early\u2010presenting symptom in childhood. A specific diagnosis is possible in 70% of patients after extensive investigations. The mortality rate is relatively low and the disease burden is high with significant comorbidities including developmental delay and epilepsy.<\/jats:p><\/jats:sec>","DOI":"10.1111\/dmcn.12081","type":"journal-article","created":{"date-parts":[[2013,2,7]],"date-time":"2013-02-07T19:25:21Z","timestamp":1360265121000},"page":"341-347","update-policy":"http:\/\/dx.doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":20,"title":["The epidemiology of intermittent and chronic ataxia in children in <scp>M<\/scp>anitoba, <scp>C<\/scp>anada"],"prefix":"10.1111","volume":"55","author":[{"given":"Michael S","family":"Salman","sequence":"first","affiliation":[{"name":"Section of Pediatric Neurology Department of Pediatrics and Child Health Children's Hospital University of Manitoba Winnipeg MB"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Esther J","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Child Health University of Manitoba Winnipeg MB"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anindita","family":"Tjahjadi","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Child Health University of Manitoba Winnipeg MB"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bernard N","family":"Chodirker","sequence":"additional","affiliation":[{"name":"Departments of Biochemistry and Medical Genetics Faculty of Medicine University of Manitoba Winnipeg MB Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2013,2,7]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.20662"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.21798"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/B978-0-444-51892-7.00014-0"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10048-009-0196-y"},{"key":"e_1_2_8_6_1","first-page":"15","article-title":"Epidemiology of the inherited ataxias","volume":"21","author":"Schoenberg BS","year":"1978","journal-title":"Adv Neurol"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1651-2227.2010.01819.x"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0895-4356(97)00202-3"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1469-8749.1996.tb12095.x"},{"key":"e_1_2_8_10_1","unstructured":"World Health Organization World Health Organization Geneva"},{"key":"e_1_2_8_11_1","unstructured":"Statistics Canada 1991 Statistics Canada Ottawa Ontario"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000339482.84392.bd"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1097\/00019052-200204000-00002"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1080\/09638280500190631"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.31423"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1172-1-47"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awp056"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1017\/S0317167100007794"}],"container-title":["Developmental Medicine &amp; Child Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fdmcn.12081","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/dmcn.12081","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,18]],"date-time":"2023-10-18T00:25:34Z","timestamp":1697588734000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/dmcn.12081"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2013,2,7]]},"references-count":17,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2013,4]]}},"alternative-id":["10.1111\/dmcn.12081"],"URL":"https:\/\/doi.org\/10.1111\/dmcn.12081","archive":["Portico"],"relation":{},"ISSN":["0012-1622","1469-8749"],"issn-type":[{"value":"0012-1622","type":"print"},{"value":"1469-8749","type":"electronic"}],"subject":[],"published":{"date-parts":[[2013,2,7]]},"assertion":[{"value":"2012-11-02","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2013-02-07","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}