Cognitive and speech-language performance in children with ataxia telangiectasia

Anja Vinck*, Mijke M. M. Verhagen, Marjo van Gerven, Imelda J. M. de Groot, Corry M. R. Weemaes, Ben A. M. Maassen, Michel A. A. P. Willemsen

*Bijbehorende auteur voor dit werk

    OnderzoeksoutputAcademicpeer review

    20 Citaten (Scopus)


    Objective: To describe cognitive and speech-language functioning of patients with ataxia-telangiectasia (A-T) in relation to their deteriorating (oculo)motor function.

    Design: Observational case series.

    Methods: Cognitive functioning, language, speech and oral-motor functioning were examined in eight individuals with A-T (six boys, two girls), taking into account the confounding effects of motor functioning on test performance.

    Results: All patients, except the youngest one, suffered from mild-to-moderate/severe intellectual impairment. Compared to developmental age, patients showed cognitive deficits in attention, (non) verbal memory and verbal fluency. Furthermore, dysarthria and weak oral-motor performance was found. Language was one of the patients' assets.

    Conclusion: In contrast to the severe deterioration of motor functioning in A-T, cognitive and language functioning appeared to level off with a typical profile of neuropsychological strengths and weaknesses. Based on our experiences with A-T, suggestions are made to determine a valid assessment of the cognitive and speech-language manifestations.

    Originele taal-2English
    Pagina's (van-tot)315-322
    Aantal pagina's8
    TijdschriftDevelopmental Neurorehabilitation
    Nummer van het tijdschrift5
    StatusPublished - 2011

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