Sleep disturbances and behavioural problems in adults with Prader–Willi syndrome

Authors

  • A. P. H. M. Maas,

    1. GROW School for Oncology and Developmental Biology, Maastricht University, Maastricht, The Netherlands
    2. Governor Kremers Centre, Maastricht University, Maastricht, the Netherlands
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    • *

       Contributed equally to the study.

  • M. Sinnema,

    1. Governor Kremers Centre, Maastricht University, Maastricht, the Netherlands
    2. CAPHRI School for Public Health and Primary Care, Maastricht University, Maastricht, the Netherlands
    3. Department of Clinical Genetics, Academic Hospital Maastricht, Maastricht, the Netherlands
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    • *

       Contributed equally to the study.

  • R. Didden,

    1. Behavioural Science Institute, Radboud University, Nijmegen, and Trajectum, Zutphen, The Netherlands
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  • M. A. Maaskant,

    1. Governor Kremers Centre, Maastricht University, Maastricht, the Netherlands
    2. Department of Health Care and Nursing Science, Maastricht University, Maastricht, and Pergamijn, Echt, The Netherlands
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  • M. G. Smits,

    1. Department of Sleep-Wake Disorders and Chronobiology, Hospital Gelderse Vallei, Ede, The Netherlands
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  • C. T. R. M. Schrander-Stumpel,

    1. GROW School for Oncology and Developmental Biology, Maastricht University, Maastricht, The Netherlands
    2. Department of Clinical Genetics, Academic Hospital Maastricht, Maastricht, the Netherlands
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  • L. M. G. Curfs

    Corresponding author
    1. GROW School for Oncology and Developmental Biology, Maastricht University, Maastricht, The Netherlands
    2. Governor Kremers Centre, Maastricht University, Maastricht, the Netherlands
    3. CAPHRI School for Public Health and Primary Care, Maastricht University, Maastricht, the Netherlands
    4. Department of Clinical Genetics, Academic Hospital Maastricht, Maastricht, the Netherlands
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Mr Leopold M.G. Curfs, Academic Hospital Maastricht, Department of Clinical Genetics/Governor Kremers Centre, PO Box 5800, 6202 AZ Maastricht, The Netherlands (e-mail: leopold.curfs@gen.unimaas.nl).

Abstract

Background  Individuals with Prader–Willi syndrome (PWS) are at risk of sleep disturbances, such as excessive daytime sleepiness (EDS) and sleep apnoea, and behavioural problems. Sleep disturbances and their relationship with other variables had not been researched extensively in adults with PWS.

Method  Sleep disturbances and behavioural problems were investigated in adults with genetically confirmed PWS using standardised questionnaires. Results of adults with paternal deletion (n = 45) were compared with those of adults with maternal uniparental disomy (n = 33).

Results  Eleven adults with PWS (i.e. 15%) had a current sleep problem, mostly night waking problems. Twenty-six adults with PWS (i.e. 33%) suffered from severe EDS. No differences in prevalence of sleep disturbances between genetic subtypes were found. Seventeen adults with deletion (i.e. 38%) and 17 adults with maternal uniparental disomy (i.e. 52%) had behavioural problems. No significant relationships were found between sleep disturbances and behavioural problems.

Conclusions  In adults with PWS, EDS is the most common type of sleep disturbance. Men and individuals with relative high body mass index are at increased risk for EDS. More research, aimed at developing a suitable screening instrument for sleep apnoea in adults with PWS, is necessary. Clinical implications of the findings are discussed.

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