{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,14]],"date-time":"2026-02-14T02:59:05Z","timestamp":1771037945941,"version":"3.50.1"},"reference-count":20,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2018,7,3]],"date-time":"2018-07-03T00:00:00Z","timestamp":1530576000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"P\u00e4ij\u00e4t-H\u00e4me Joint Municipal Authority"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Molec Gen &amp; Gen Med"],"published-print":{"date-parts":[[2018,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Intellectual disability (<jats:styled-content style=\"fixed-case\">ID<\/jats:styled-content>) and dementia reflect disturbed cortical function during and after developmental age, respectively. Due to the wide heterogeneity of<jats:styled-content style=\"fixed-case\">ID<\/jats:styled-content>population the decline in cognitive and adaptive skills may be different in distinct genetic subgroups.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Using the British Present Psychiatric State\u2013learning Disabilities assessment (<jats:styled-content style=\"fixed-case\">PPS<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">LD<\/jats:styled-content>) questionnaire the dementia signs were screened in 62, 22 and 44 individuals (&gt; 35\u00a0year of age) with Down (<jats:styled-content style=\"fixed-case\">DS<\/jats:styled-content>,<jats:styled-content style=\"fixed-case\">OMIM<\/jats:styled-content>number 190685), Williams (<jats:styled-content style=\"fixed-case\">WS<\/jats:styled-content>,<jats:styled-content style=\"fixed-case\">OMIM<\/jats:styled-content>number, 194050), and Fragile X syndrome (<jats:styled-content style=\"fixed-case\">FXS<\/jats:styled-content>,<jats:styled-content style=\"fixed-case\">OMIM<\/jats:styled-content>number 309550), respectively. The median age of those with<jats:styled-content style=\"fixed-case\">FXS<\/jats:styled-content>(59\u00a0years) was higher than of those with<jats:styled-content style=\"fixed-case\">DS<\/jats:styled-content>(50\u00a0years) and<jats:styled-content style=\"fixed-case\">WS<\/jats:styled-content>(53\u00a0years).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Most study participants with<jats:styled-content style=\"fixed-case\">DS<\/jats:styled-content>(80%) and<jats:styled-content style=\"fixed-case\">FXS<\/jats:styled-content>(89%) were or had been moderately or severely intellectually disabled while most participants with<jats:styled-content style=\"fixed-case\">WS<\/jats:styled-content>(73%) were or had been mildly or moderately disabled at adolescent age. The adolescent (premorbid) level of<jats:styled-content style=\"fixed-case\">ID<\/jats:styled-content>did not correlate with the dementia score. The median scores were 11\/27, 1\/27, and 0\/27 in<jats:styled-content style=\"fixed-case\">DS<\/jats:styled-content>,<jats:styled-content style=\"fixed-case\">WS<\/jats:styled-content>, and<jats:styled-content style=\"fixed-case\">FXS<\/jats:styled-content>subgroups, respectively. Dementia that was confirmed by brain imaging, manifested as Alzheimer disease and as moya\u2010moya disease associated vascular dementia in<jats:styled-content style=\"fixed-case\">DS<\/jats:styled-content>and as vascular dementia in<jats:styled-content style=\"fixed-case\">WS<\/jats:styled-content>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>This survey suggests that the risk of dementia varies depending on the cause of<jats:styled-content style=\"fixed-case\">ID<\/jats:styled-content>and that the severity of<jats:styled-content style=\"fixed-case\">ID<\/jats:styled-content>in adolescence does not predict the development of dementia at a later age. Consequently, the<jats:styled-content style=\"fixed-case\">ID<\/jats:styled-content>and dementia should be understood as separate clinical entities that need to be taken into account in the health management of intellectually disabled people. This is important for the arrangement of appropriate and timely interventions, which can be expected to delay the need for institutionalization.<\/jats:p><\/jats:sec>","DOI":"10.1002\/mgg3.430","type":"journal-article","created":{"date-parts":[[2018,7,4]],"date-time":"2018-07-04T04:10:16Z","timestamp":1530677416000},"page":"855-860","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":19,"title":["Signs indicating dementia in Down, Williams and Fragile X syndromes"],"prefix":"10.1002","volume":"6","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3041-5253","authenticated-orcid":false,"given":"Oili","family":"Sauna\u2010Aho","sequence":"first","affiliation":[{"name":"KTO\u2010Special Welfare District of Varsinais\u2010Suomi Paimio Finland"},{"name":"Departments of Child Neurology and Public Health University of Turku Turku Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6373-3792","authenticated-orcid":false,"given":"Nina","family":"Bjelogrlic\u2010Laakso","sequence":"additional","affiliation":[{"name":"Special Services for Developmentally Disabled Tampere University Hospital Tampere Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Auli","family":"Siren","sequence":"additional","affiliation":[{"name":"Special Services for Developmentally Disabled Tampere University Hospital Tampere Finland"},{"name":"Department of Pediatric Neurology Kanta\u2010H\u00e4me Central Hospital H\u00e4meenlinna Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maria","family":"Arvio","sequence":"additional","affiliation":[{"name":"KTO\u2010Special Welfare District of Varsinais\u2010Suomi Paimio Finland"},{"name":"Departments of Child Neurology and Public Health University of Turku Turku Finland"},{"name":"Department of Neurology P\u00e4ij\u00e4t\u2010H\u00e4me Joint Municipal Authority Lahti Finland"},{"name":"PEDEGO University of Oulu Oulu Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2018,7,3]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/cge.12639"},{"key":"e_1_2_7_3_1","first-page":"1108","article-title":"Middle\u2010aged people with Down syndrome receive good care in home towns (In Finnish)","volume":"15","author":"Arvio M.","year":"2013","journal-title":"Finnish Medical Journal"},{"key":"e_1_2_7_4_1","first-page":"3","article-title":"Down syndrome \u2010 Onset age of dementia","volume":"7","author":"Arvio M.","year":"2017","journal-title":"Journal of Alzheimer's Disease & Parkinsonism"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/cge.12787"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-0004.1997.tb02549.x"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/jar.12269"},{"issue":"6","key":"e_1_2_7_8_1","doi-asserted-by":"crossref","first-page":"e00431","DOI":"10.1002\/brb3.431","article-title":"Mortality in individuals with intellectual disabilities in Finland","volume":"24","author":"Arvio M.","year":"2016","journal-title":"Brain and Behavior"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2788.2003.00447.x"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2788.1997.tb00740.x"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1099-1166(199706)12:6<662::AID-GPS594>3.0.CO;2-T"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.39.8.554"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1990.00530030029011"},{"key":"e_1_2_7_14_1","doi-asserted-by":"crossref","first-page":"369","DOI":"10.1111\/j.1365-2788.1996.tb00642.x","article-title":"Further evaluation of the Dementia Questionnaire for Persons with Mental Retardation (DMR)","volume":"40","author":"Evenhuis H. 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