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A national survey of Rett syndrome : behavioural characteristics
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Cianfaglione, Rina, Clarke, Angus, Kerr, Michael (Michael P.), Hastings, Richard P., Oliver, Chris, Moss, Joanna, Heald, Mary and Felce, David (2015) A national survey of Rett syndrome : behavioural characteristics. Journal of Neurodevelopmental Disorders, Volume 7 (Number 1). Article number 11. doi:10.1186/s11689-015-9104-y ISSN 1866-1947.
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Official URL: http://dx.doi.org/10.1186/s11689-015-9104-y
Abstract
Background: The aim was to gain a UK national sample of people with Rett syndrome (RTT) across the age range and compare their characteristics using a variety of relevant behavioural measures with a well-chosen contrast group.
Methods: The achieved sample was 91 girls and women, aged from 4 to 47 years, of whom 71 were known to be MECP2 positive. The contrast group (n = 66), matched for age, gender, language and self-help skills, comprised individuals with six other syndromes associated with intellectual disability. Parental questionnaire measures of RTT specific characteristics, impulsivity, overactivity, mood, interest and pleasure, repetitive behaviour and self-injury were administered.
Results: Hand stereotypies, breathing irregularities, night-time unrest and anxiety or inappropriate fear were commonly reported among the RTT sample. Problems of low mood were also reported as common. However,
mood and interest and pleasure were no lower than found in the contrast group. In addition, self-injury was lower than in the contrast group and was associated with factors found to predict self-injury in other groups of people with severe intellectual disabilities.
Conclusions: There is variability in the manifestation of problem behaviours potentially associated with the
syndrome across individuals, with some more severely affected in most areas than others. Some of this variability appears to be underpinned by genetic mutation.
| Item Type: | Journal Article | ||||||||
|---|---|---|---|---|---|---|---|---|---|
| Subjects: | R Medicine > RC Internal medicine | ||||||||
| Divisions: | Faculty of Social Sciences > Centre for Educational Development, Appraisal and Research (CEDAR) | ||||||||
| Library of Congress Subject Headings (LCSH): | Rett syndrome | ||||||||
| Journal or Publication Title: | Journal of Neurodevelopmental Disorders | ||||||||
| Publisher: | BioMed Central Ltd. | ||||||||
| ISSN: | 1866-1947 | ||||||||
| Official Date: | 4 March 2015 | ||||||||
| Dates: |
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| Volume: | Volume 7 | ||||||||
| Number: | Number 1 | ||||||||
| Article Number: | Article number 11 | ||||||||
| DOI: | 10.1186/s11689-015-9104-y | ||||||||
| Status: | Peer Reviewed | ||||||||
| Publication Status: | Published | ||||||||
| Access rights to Published version: | Open Access (Creative Commons) | ||||||||
| Date of first compliant deposit: | 28 July 2016 | ||||||||
| Date of first compliant Open Access: | 28 July 2016 | ||||||||
| Funder: | National Institute for Social Care and Health Research (Wales) (NISCHR) |
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