The Library
Ageing in Rett syndrome
Tools
Cianfaglione, Rina, Clarke, A., Kerr, M., Hastings, Richard P., Oliver, C. and Felce, D. (2016) Ageing in Rett syndrome. Journal of Intellectual Disability Research, 60 (2). pp. 182-190. doi:10.1111/jir.12228 ISSN 0964-2633.
Research output not available from this repository.
Request-a-Copy directly from author or use local Library Get it For Me service.
Official URL: http://dx.doi.org/10.1111/jir.12228
Abstract
Background
The aim was to gain a UK national sample of people with Rett syndrome across the age range and (1) conduct a cross-sectional comparison of age groups and (2) undertake a longitudinal follow-up.
Methods
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 years, and 71 were known to have a mutation in the methyl-CpG binding protein-2 (MECP2) gene. Seventy-two of the initial sample were followed up 16 months later, and 50 returned completed assessments (69.4%). Their ages ranged from 7 to 48 years, and 42 were MECP2 positive. Parental questionnaire measures of Rett syndrome specific characteristics, impulsivity, overactivity, mood, interest and pleasure, repetitive behaviour and self-injury were administered.
Results
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-injury. There was some suggestion of deterioration in health arising with ageing, principally contributed to by deteriorations in dental and gastro-intestinal 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.
Conclusions
This study provides further evidence for the post-regression stability that characterises Rett syndrome. Emergent low mood in Rett syndrome requires further research.
Item Type: | Journal Article | ||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|
Divisions: | Faculty of Social Sciences > Centre for Educational Development, Appraisal and Research (CEDAR) | ||||||||||
Journal or Publication Title: | Journal of Intellectual Disability Research | ||||||||||
Publisher: | Wiley-Blackwell Publishing Ltd. | ||||||||||
ISSN: | 0964-2633 | ||||||||||
Official Date: | February 2016 | ||||||||||
Dates: |
|
||||||||||
Volume: | 60 | ||||||||||
Number: | 2 | ||||||||||
Page Range: | pp. 182-190 | ||||||||||
DOI: | 10.1111/jir.12228 | ||||||||||
Status: | Peer Reviewed | ||||||||||
Publication Status: | Published | ||||||||||
Access rights to Published version: | Restricted or Subscription Access |
Request changes or add full text files to a record
Repository staff actions (login required)
View Item |