What are the behavioral signs and symptoms of Prader-Willi syndrome (PWS)?

Updated: Oct 14, 2020
  • Author: Ann Scheimann, MD, MBA; Chief Editor: Luis O Rohena, MD, MS, FAAP, FACMG  more...
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Patients with Prader-Willi syndrome often demonstrate behavioral problems. [46]  Young children exhibit temper tantrums, stubbornness, and obsessive-compulsive behaviors.

Behavioral issues often compromise the level of academic performance. Obsessive-compulsive behaviors and perseveration are challenging for children with Prader-Willi syndrome in the classroom setting. [44]  Features of psychosis are present in 5-10% of young adults with Prader-Willi syndrome. [47, 48]

Food-seeking behaviors may include eating garbage, eating frozen food, and stealing resources to obtain food. High thresholds for vomiting and pain tolerance can complicate binging on spoiled foods and delay treatment for GI disease. Death due to choking episodes has been reported. [35] After episodes of binge eating (eg, at holidays), both thin and obese individuals with Prader-Willi syndrome have developed abdominal discomfort, with acute gastric dilation observed using radiography. Some patients have developed gastric necrosis. [14]

A study by Gito et al indicated that among persons with Prader-Willi syndrome, food-related behaviors are more severe in males whose condition is deletion related than in females with gene deletion or in males with maternal uniparental disomy. However, these behaviors were found to be milder in males with maternal uniparental disomy than in females with such disomy. [49]

In a small percentage of people with autism spectrum disorder (ASD), changes are found in 15q11.2-q3. Thus, it has been estimated that 25-41% of individuals with Prader-Willi syndrome have ASD, although Dykens et al suggest that these rates are questionable, having been derived from autism screeners given to parents. [50]

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