Maladaptive behavior differences in Prader-Willi syndrome due to paternal deletion versus maternal uniparental disomy

Maladaptive behavior was compared across 23 people with Prader-Willi syndro me due to paternal deletion to 23 age- and gender-matched subjects with mat ernal uniparental disomy. Controlling for the higher IQs of the uniparental disomy group, deleted cases showed significantly higher maladaptive rating s on the Child Behavior Checklist's Internalizing, Externalizing, and Total domains as well as more symptom-related distress on the Yale-Brown Obsessi ve-Compulsive Scale. Across both measures, deleted cases were more apt to s kin-pick, bite their nails, hoard, overeat, sulk, and withdraw. A dampening of symptom severity is suggested in Prader-Willi syndrome cases due to mat ernal uniparental disomy. Findings are compared to Angelman syndrome, and p ossible genetic mechanisms are discussed, as are implications for Prader-Wi lli syndrome and obsessive-compulsive behaviors.