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Encyclopedia of Clinical Neuropsychology pp 2760–2764Cite as

Prader-Willi Syndrome

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Synonyms

Prader-Willi-Labhart syndrome; PWS

Short Description or Definition

Prader-Willi syndrome (PWS) is a genetic disorder resulting from a sporadic hemizygous deletion or aberration on paternal chromosome 15, specifically the region 15q11.2–13. Characteristic facial features include a long face, narrow forehead, and almond-shaped eyes (Cassidy and Schwartz 1998). Common features include short stature, hypotonia, hypogonadism, hyperphagia, obesity, and cognitive delays (Driscoll et al. 2016). Additionally, thermoinstability, hypopigmentation, strabismus, and speech difficulties have been observed. A range of behavioral features have been described, including higher rates of both internalizing and externalizing behavior problems.

Categorization

The critical region 15q11–13, implicated in both PWS and Angelman syndrome, is involved in a process known as genetic imprinting, which occurs when the expression of genes is dependent upon the parent of origin. Both a maternal and a paternal...

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References and Readings

  • For More Information, See the Prader-Willi Association; www.Pwsausa.Org

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Author information

Authors and Affiliations

  1. Department of Psychology and the Neuroscience Institute, Georgia State University, Atlanta, GA, USA

    Kristen Smith

  2. Department of Psychology, University of Wisconsin-Milwaukee, Milwaukee, WI, USA

    Bonita P. “Bonnie” Klein-Tasman

Authors
  1. Kristen Smith
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  2. Bonita P. “Bonnie” Klein-Tasman
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Corresponding author

Correspondence to Kristen Smith .

Editor information

Editors and Affiliations

  1. Department of Physical Medicine and Rehabilitation, Virginia Commonwealth University, Richmond, VA, USA

    Jeffrey S. Kreutzer

  2. Kessler Foundation, Pleasant Valley Way, West Orange, NJ, USA

    John DeLuca

  3. Independent Practice, Wynnewood, PA, USA

    Bruce Caplan

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Smith, K., Klein-Tasman, B.P.“. (2018). Prader-Willi Syndrome. In: Kreutzer, J.S., DeLuca, J., Caplan, B. (eds) Encyclopedia of Clinical Neuropsychology. Springer, Cham. https://doi.org/10.1007/978-3-319-57111-9_1588

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