Behavior and personality characteristics of children and young adults with Prader-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 to 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 interviewed and completed questionnaires about their children's behavior and personality. Results: Individuals with PWS had more behavior problems than those in the comparison group. Some behaviors were specific to PWS. Younger PWS cases had fewer behavior problems than older PWS cases. Treated individuals had approximately the same degree of behavior problems as those untreated, even though a few symptoms occurred at lower rates. Conclusions: PWS is associated with behavior correlates that are not related to weight or IQ. In the first few years of life, children with PWS do not demonstrate the characteristic profile of preoccupation with food, ritualism, irritability, temper tantrums, and skin-picking which is typical of older individuals with PWS. Current therapies (including treatment with growth hormone) do not seem to radically affect the behavioral expression of the disorder, even though some problems tended to abate with treatment.