Ageing in Rett syndrome

Research output: Contribution to journalArticlepeer-review

Standard

Ageing in Rett syndrome. / Cianfaglione, R.; Clarke, A.; Kerr, M.; Hastings, R. P.; Oliver, C.; Felce, D.

In: Journal of Intellectual Disability Research, Vol. 60, No. 2, 02.2016, p. 182-190.

Research output: Contribution to journalArticlepeer-review

Harvard

Cianfaglione, R, Clarke, A, Kerr, M, Hastings, RP, Oliver, C & Felce, D 2016, 'Ageing in Rett syndrome', Journal of Intellectual Disability Research, vol. 60, no. 2, pp. 182-190. https://doi.org/10.1111/jir.12228

APA

Cianfaglione, R., Clarke, A., Kerr, M., Hastings, R. P., Oliver, C., & Felce, D. (2016). Ageing in Rett syndrome. Journal of Intellectual Disability Research, 60(2), 182-190. https://doi.org/10.1111/jir.12228

Vancouver

Cianfaglione R, Clarke A, Kerr M, Hastings RP, Oliver C, Felce D. Ageing in Rett syndrome. Journal of Intellectual Disability Research. 2016 Feb;60(2):182-190. https://doi.org/10.1111/jir.12228

Author

Cianfaglione, R. ; Clarke, A. ; Kerr, M. ; Hastings, R. P. ; Oliver, C. ; Felce, D. / Ageing in Rett syndrome. In: Journal of Intellectual Disability Research. 2016 ; Vol. 60, No. 2. pp. 182-190.

Bibtex

@article{51d08cbd73574c85a0596d3a63e8aeda,
title = "Ageing in Rett syndrome",
abstract = "BackgroundThe 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.MethodsFrom 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 wereMECP2 positive. Parental questionnaire measures of Rett syndrome specific characteristics, impulsivity, overactivity, mood, interest and pleasure, repetitive behaviour and self-injury were administered.ResultsAdaptive 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.ConclusionsThis study provides further evidence for the post-regression stability that characterises Rett syndrome. Emergent low mood in Rett syndrome requires further research.",
keywords = "Ageing, Intellectual disabilities, Rett syndrome",
author = "R. Cianfaglione and A. Clarke and M. Kerr and Hastings, {R. P.} and C. Oliver and D. Felce",
year = "2016",
month = feb,
doi = "10.1111/jir.12228",
language = "English",
volume = "60",
pages = "182--190",
journal = "Journal of Intellectual Disability Research",
issn = "0964-2633",
publisher = "Wiley",
number = "2",

}

RIS

TY - JOUR

T1 - Ageing in Rett syndrome

AU - Cianfaglione, R.

AU - Clarke, A.

AU - Kerr, M.

AU - Hastings, R. P.

AU - Oliver, C.

AU - Felce, D.

PY - 2016/2

Y1 - 2016/2

N2 - BackgroundThe 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.MethodsFrom 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 wereMECP2 positive. Parental questionnaire measures of Rett syndrome specific characteristics, impulsivity, overactivity, mood, interest and pleasure, repetitive behaviour and self-injury were administered.ResultsAdaptive 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.ConclusionsThis study provides further evidence for the post-regression stability that characterises Rett syndrome. Emergent low mood in Rett syndrome requires further research.

AB - BackgroundThe 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.MethodsFrom 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 wereMECP2 positive. Parental questionnaire measures of Rett syndrome specific characteristics, impulsivity, overactivity, mood, interest and pleasure, repetitive behaviour and self-injury were administered.ResultsAdaptive 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.ConclusionsThis study provides further evidence for the post-regression stability that characterises Rett syndrome. Emergent low mood in Rett syndrome requires further research.

KW - Ageing

KW - Intellectual disabilities

KW - Rett syndrome

UR - http://www.scopus.com/inward/record.url?scp=84941671143&partnerID=8YFLogxK

U2 - 10.1111/jir.12228

DO - 10.1111/jir.12228

M3 - Article

VL - 60

SP - 182

EP - 190

JO - Journal of Intellectual Disability Research

JF - Journal of Intellectual Disability Research

SN - 0964-2633

IS - 2

ER -