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    Validating the Rett Syndrome Gross Motor Scale

    238671_238671.PDF (203.8Kb)
    Access Status
    Open access
    Authors
    Downs, Jennepher
    Stahlhut, M.
    Wong, K.
    Syhler, B.
    Bisgaard, A.
    Jacoby, P.
    Leonard, H.
    Date
    2016
    Type
    Journal Article
    
    Metadata
    Show full item record
    Citation
    Downs, J. and Stahlhut, M. and Wong, K. and Syhler, B. and Bisgaard, A. and Jacoby, P. and Leonard, H. 2016. Validating the Rett Syndrome Gross Motor Scale. PLoS ONE. 11 (1): e0147555.
    Source Title
    PLoS ONE
    DOI
    10.1371/journal.pone.0147555
    School
    School of Physiotherapy and Exercise Science
    Remarks

    This open access article is distributed under the Creative Commons license http://creativecommons.org/licenses/by/4.0/

    URI
    http://hdl.handle.net/20.500.11937/32198
    Collection
    • Curtin Research Publications
    Abstract

    © 2016 Downs et al. Rett syndrome is a pervasive neurodevelopmental disorder associated with a pathogenic mutation on the MECP2 gene. Impaired movement is a fundamental component and the Rett Syndrome Gross Motor Scale was developed to measure gross motor abilities in this population. The current study investigated the validity and reliability of the Rett Syndrome Gross Motor Scale. Video data showing gross motor abilities supplemented with parent report data was collected for 255 girls and women registered with the Australian Rett Syndrome Database, and the factor structure and relationships between motor scores, age and genotype were investigated. Clinical assessment scores for 38 girls and women with Rett syndrome who attended the Danish Center for Rett Syndrome were used to assess consistency of measurement. Principal components analysis enabled the calculation of three factor scores: Sitting, Standing and Walking, and Challenge. Motor scores were poorer with increasing age and those with the p.Arg133Cys, p.Arg294* or p.Arg306Cys mutation achieved higher scores than those with a large deletion. The repeatability of clinical assessment was excellent (intraclass correlation coefficient for total score 0.99, 95% CI 0.93–0.98). The standard error of measurement for the total score was 2 points and we would be 95% confident that a change 4 points in the 45-point scale would be greater than within-subject measurement error. The Rett Syndrome Gross Motor Scale could be an appropriate measure of gross motor skills in clinical practice and clinical trials.

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