Behavior and personality characteristics of children and young adults withPrader-Willi syndrome: A controlled study

Objective: To analyze (I) which behavior and personality characteristics in Prader-Willi syndrome (PWS) are primarily linked to the syndrome and not t o mental retardation or being overweight, (2) how early in life such traits appear. and (3) whether current therapies affect behavior. Method: Parents of a group of 44 individuals with PWS and of a comparison group were inter viewed and completed questionnaires about their children's behavior and per sonality. Results: Individuals with PWS had more behavior problems than tho se in the comparison group. Some behaviors were specific to PWS. Younger PW S cases had fewer behavior problems than older PWS cases. Treated individua ls had approximately the same degree of behavior problems as those untreate d, even though a few symptoms occurred at lower rates. Conclusions: PWS is associated with behavior correlates that are not related to weight or IQ. I n the first few years of life, children with PWS do not demonstrate the cha racteristic profile of preoccupation with food, ritualism, irritability, te mper tantrums, and skin-picking which is typical of older individuals with PWS. Current therapies (including treatment with growth hormone) do not see m to radically affect the behavioral expression of the disorder, even thoug h some problems tended to abate with treatment.