Literature DB >> 21121991

Working memory subsystems and task complexity in young boys with Fragile X syndrome.

S Baker1, S Hooper, M Skinner, D Hatton, J Schaaf, P Ornstein, D Bailey.   

Abstract

BACKGROUND: Working memory problems have been targeted as core deficits in individuals with Fragile X syndrome (FXS); however, there have been few studies that have examined working memory in young boys with FXS, and even fewer studies that have studied the working memory performance of young boys with FXS across different degrees of complexity. The purpose of this study was to investigate the phonological loop and visual-spatial working memory in young boys with FXS, in comparison to mental age-matched typical boys, and to examine the impact of complexity of the working memory tasks on performance.
METHODS: The performance of young boys (7 to 13-years-old) with FXS (n = 40) was compared with that of mental age and race matched typically developing boys (n = 40) on measures designed to test the phonological loop and the visuospatial sketchpad across low, moderate and high degrees of complexity. Multivariate analyses were used to examine group differences across the specific working memory systems and degrees of complexity.
RESULTS: Results suggested that boys with FXS showed deficits in phonological loop and visual-spatial working memory tasks when compared with typically developing mental age-matched boys. For the boys with FXS, the phonological loop was significantly lower than the visual-spatial sketchpad; however, there was no significant difference in performance across the low, moderate and high degrees of complexity in the working memory tasks. Reverse tasks from both the phonological loop and visual-spatial sketchpad appeared to be the most challenging for both groups, but particularly for the boys with FXS.
CONCLUSIONS: These findings implicate a generalised deficit in working memory in young boys with FXS, with a specific disproportionate impairment in the phonological loop. Given the lack of differentiation on the low versus high complexity tasks, simple span tasks may provide an adequate estimate of working memory until greater involvement of the central executive is achieved.
© 2010 The Authors. Journal of Intellectual Disability Research © 2010 Blackwell Publishing Ltd.

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Year:  2010        PMID: 21121991      PMCID: PMC4437210          DOI: 10.1111/j.1365-2788.2010.01343.x

Source DB:  PubMed          Journal:  J Intellect Disabil Res        ISSN: 0964-2633


  40 in total

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2.  Inference deficits in women with Fragile X Syndrome: A problem in working memory.

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5.  The neurocognitive phenotype of female carriers of fragile X: additional evidence for specificity.

Authors:  M M Mazzocco; B F Pennington; R J Hagerman
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6.  Cognitive profiles associated with the fra(X) syndrome in males and females.

Authors:  L S Freund; A L Reiss
Journal:  Am J Med Genet       Date:  1991-03-15

7.  Further delineation of the executive deficit in males with fragile-X syndrome.

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8.  Assessment of cognitive decline associated with aging: a comparison of individuals with Down syndrome and other etiologies.

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Authors:  P P Wang; U Bellugi
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10.  Lifespan changes in working memory in fragile X premutation males.

Authors:  Kim M Cornish; Cary S Kogan; Lexin Li; Jeremy Turk; Sebastien Jacquemont; Randi J Hagerman
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  25 in total

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3.  Cognitive aspects of Fragile X syndrome.

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Journal:  Wiley Interdiscip Rev Cogn Sci       Date:  2014-05-29

4.  Cognitive, environmental, and linguistic predictors of syntax in fragile X syndrome and Down syndrome.

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5.  Differential Relationships of Anxiety and Autism Symptoms on Social Skills in Young Boys With Fragile X Syndrome.

Authors:  Debra L Reisinger; Jane E Roberts
Journal:  Am J Intellect Dev Disabil       Date:  2017-09

Review 6.  Conceptualizing neurodevelopmental disorders through a mechanistic understanding of fragile X syndrome and Williams syndrome.

Authors:  Lawrence K Fung; Eve-Marie Quintin; Brian W Haas; Allan L Reiss
Journal:  Curr Opin Neurol       Date:  2012-04       Impact factor: 5.710

7.  Degraded speech sound processing in a rat model of fragile X syndrome.

Authors:  Crystal T Engineer; Tracy M Centanni; Kwok W Im; Kimiya C Rahebi; Elizabeth P Buell; Michael P Kilgard
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8.  Reading and phonological skills in boys with fragile X syndrome.

Authors:  Jessica Klusek; Anna W Hunt; Penny L Mirrett; Deborah D Hatton; Stephen R Hooper; Jane E Roberts; Donald B Bailey
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9.  Outcome measures for clinical trials in fragile X syndrome.

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10.  The Role of Executive Function in Independent Living Skills in Female Adolescents and Young Adults With Fragile X Syndrome.

Authors:  Arianna Martin; Eve-Marie Quintin; Scott S Hall; Allan L Reiss
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