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    Longitudinal hand function in Rett syndrome

    Access Status
    Fulltext not available
    Authors
    Downs, Jennepher
    Bebbington, A.
    Kaufmann, W.
    Leonard, H.
    Date
    2011
    Type
    Journal Article
    
    Metadata
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    Citation
    Downs, J. and Bebbington, A. and Kaufmann, W. and Leonard, H. 2011. Longitudinal hand function in Rett syndrome. Journal of Child Neurology. 26 (3): pp. 334-340.
    Source Title
    Journal of Child Neurology
    DOI
    10.1177/0883073810381920
    ISSN
    0883-0738
    URI
    http://hdl.handle.net/20.500.11937/23479
    Collection
    • Curtin Research Publications
    Abstract

    Loss of hand function is a core feature of Rett syndrome. This article describes longitudinal hand function at 3 time points for 72 subjects participating in the Australian Rett Syndrome Database. Approximately 40% of subjects with some grasping abilities lost skill over the 3- to 4-year period between video assessments. In these subjects, a decrease in hand function was seen less frequently in girls 13 to 19 years old than in those younger than 8 years, in subjects with some mobility compared with those who were wheelchair bound, and in those who had previously been able to finger feed. Relationships with the magnitude of change reflected these findings. Change in hand function did not vary with clinical severity. The results for all subjects were similar to results obtained when analysis was restricted to those with a pathogenic mutation. Variability in the longitudinal course of hand function in Rett syndrome was observed.

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