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Williams Syndrome

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Sleep in Children with Neurodevelopmental Disabilities

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Abstract

Williams syndrome (Williams-Beuren syndrome, or WBS) is a congenital disorder caused by deletions of multiple contiguous genes on the long arm of one chromosome 7 (del 7q11.23). There is a distinctive pattern of developmental delays, personality and behavior, physical phenotype, and multiorgan involvement. Sleep problems in children with Williams syndrome are supported by many studies, including research using questionnaires, actigraphy, overnight polysomnography, electroencephalographic (EEG) spectral analysis, and endocrine markers. Frequently reported sleep disturbances in Williams syndrome include sleep anxiety, prolonged sleep latency, and frequent night wakings. Polysomnography studies have shown decreased sleep efficiency, increased wake after sleep onset, and the intriguing feature of increased slow-wave (N3) sleep in subjects with Williams syndrome compared to typically developing controls. EEG studies support spectral power changes that are altered characteristically in Williams syndrome, and other work indicates abnormalities in melatonin and cortisol could contribute to disrupted sleep. In the future, it will be critical to determine what sleep patterns may indeed be unique to Williams syndrome versus non-specific changes seen more broadly in other populations of children with neurodevelopmental disabilities.

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Author information

Authors and Affiliations

  1. Genetics Division, Department of Pediatrics, Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Paige Kaplan

  2. Departments of Neurology and Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA

    Thornton B. A. Mason II

  3. Division of Neurology, Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Thornton B. A. Mason II

Authors
  1. Paige Kaplan
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  2. Thornton B. A. Mason II
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Correspondence to Thornton B. A. Mason II .

Editor information

Editors and Affiliations

  1. Departments of Pediatrics and Neurology, Virginia Commonwealth University School of Medicine, Richmond, VA, USA

    Jennifer A. Accardo

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Kaplan, P., Mason, T.B.A. (2019). Williams Syndrome. In: Accardo, J. (eds) Sleep in Children with Neurodevelopmental Disabilities. Springer, Cham. https://doi.org/10.1007/978-3-319-98414-8_18

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  • DOI: https://doi.org/10.1007/978-3-319-98414-8_18

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-98412-4

  • Online ISBN: 978-3-319-98414-8

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