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Prader-Willi Syndrome

  • By: Katherine Newton & William A. Rae
  • In: Encyclopedia of School Psychology
  • Edited by: Steven W. Lee
  • Subject:School/Educational Psychology (general), School Psychology, Educational Psychology

Prader-Willi syndrome (PWS) is a rare neurobehavioral genetic disorder that affects numerous organ systems. It occurs in approximately 1 in 10,000 to 30,000 births and affects all races and genders. PWS impacts the functioning of the hypothalamus, a regulation center located in the brain, resulting in decreased growth hormone, altered reproductive hormones (e.g., hypogonadism), disruption in appetite control, and altered regulation of the autonomic nervous system (the nervous system associated with involuntary reactions). In infancy, PWS is associated with a poor sucking reflex and weak muscle tone. In later childhood, it is associated with:

  • Excessive food seeking (with subsequent morbid obesity)
  • Delayed development
  • Mental retardation or learning disability (Intelligence test scores can range from 20 to 115; an average intelligence score for an individual with no disabilities ...
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