{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,11]],"date-time":"2026-05-11T13:14:46Z","timestamp":1778505286609,"version":"3.51.4"},"reference-count":21,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2015,9,16]],"date-time":"2015-09-16T00:00:00Z","timestamp":1442361600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"NISCHR Social Care Studentship"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J intellect Disabil Res"],"published-print":{"date-parts":[[2016,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>The aim was to gain a UK national sample of people with Rett syndrome across the age range and (1) conduct a cross\u2010sectional comparison of age groups and (2) undertake a longitudinal follow\u2010up.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>From 308 potential participants approached to take part, a sample of 91 girls and women was achieved (29.5%). Their ages ranged from 4 to 47\u2009years, and 71 were known to have a mutation in the methyl\u2010CpG binding protein\u20102 (<jats:italic>MECP2<\/jats:italic>) gene. Seventy\u2010two of the initial sample were followed up 16\u2009months later, and 50 returned completed assessments (69.4%). Their ages ranged from 7 to 48\u2009years, and 42 were <jats:italic>MECP2<\/jats:italic> positive. Parental questionnaire measures of Rett syndrome specific characteristics, impulsivity, overactivity, mood, interest and pleasure, repetitive behaviour and self\u2010injury were administered.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Adaptive behaviour and behavioural characteristics of Rett syndrome were similar across age groups and, where assessed, stable over time, as were repetitive behaviours generally and self\u2010injury. There was some suggestion of deterioration in health arising with ageing, principally contributed to by deteriorations in dental and gastro\u2010intestinal problems both with moderate effect sizes. Indicators of mood, interest and pleasure differed significantly across age groups. The total scale score significantly deteriorated over time, with a moderate effect size.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>This study provides further evidence for the post\u2010regression stability that characterises Rett syndrome. Emergent low mood in Rett syndrome requires further research.<\/jats:p><\/jats:sec>","DOI":"10.1111\/jir.12228","type":"journal-article","created":{"date-parts":[[2015,9,16]],"date-time":"2015-09-16T05:41:27Z","timestamp":1442382087000},"page":"182-190","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":27,"title":["Ageing in Rett syndrome"],"prefix":"10.1111","volume":"60","author":[{"given":"R.","family":"Cianfaglione","sequence":"first","affiliation":[{"name":"Welsh Centre for Learning Disabilities, Institute of Psychological Medicine and Clinical Neurosciences Cardiff University  Cardiff UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A.","family":"Clarke","sequence":"additional","affiliation":[{"name":"Institute of Cancer and Genetics Cardiff University  Cardiff UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Kerr","sequence":"additional","affiliation":[{"name":"Welsh Centre for Learning Disabilities, Institute of Psychological Medicine and Clinical Neurosciences Cardiff University  Cardiff UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0495-8270","authenticated-orcid":false,"given":"R.\u2009P.","family":"Hastings","sequence":"additional","affiliation":[{"name":"Centre for Educational Development Appraisal and Research University of Warwick  Coventry UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C.","family":"Oliver","sequence":"additional","affiliation":[{"name":"Cerebra Centre for Neurodevelopmental Disorders, School of Psychology University of Birmingham  Birmingham UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"D.","family":"Felce","sequence":"additional","affiliation":[{"name":"Welsh Centre for Learning Disabilities, Institute of Psychological Medicine and Clinical Neurosciences Cardiff University  Cardiff UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2015,9,16]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/13810"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1741-1130.2007.00109.x"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2788.2010.01338.x"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.37027"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1186\/s11689-015-9104-y"},{"key":"e_1_2_8_7_1","volume-title":"Discovering Statistics Using SPSS","author":"Field A.","year":"2009"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/mrdd.10020"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.1320250506"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.32361"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/cge.12063"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2788.2008.01047.x"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1352\/0895-8017(2002)107<0146:SIBSRA>2.0.CO;2"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.eurpsy.2007.11.008"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10803-008-0655-6"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPG.0b013e31824b6159"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1111\/1469-7610.00236"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.22124"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ridd.2010.04.011"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1348\/014466503762842039"},{"key":"e_1_2_8_21_1","volume-title":"The mood interest and pleasure questionnaire: manual for administration and scoring","author":"Ross E.","year":"2008"},{"key":"e_1_2_8_22_1","volume-title":"Vineland Adaptive Behavior Scales (Survey Ed.)","author":"Sparrow S. 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