Behavior and emotional disturbance in Prader-Willi syndrome

To determine if persons with the Prader-Willi syndrome (PWS) have increased psychopathology when compared with matched controls, and whether there is a specific behavior phenotype in PWS, the behavior of 46 persons with PWS w as compared with that of control individuals derived from a community sampl e (N = 454) of persons with mental retardation (MR), Behaviors were studied using the Developmental Behaviour Checklist, an instrument of established validity in the evaluation of behavioral disturbance in individuals with MR . PWS subjects were found to be more behaviorally disturbed than controls o verall, and especially in antisocial behavior. In addition, some individual behaviors were more common in PWS subjects than controls. When these behav iors are considered together with findings from other studies using accepta bly rigorous methods, a consensus behavior phenotype for PWS can be formula ted. This will provide a valid foundation for studies of the mechanism of g enetic pathogenesis of behavior in PWS, (C) 1999 Wiley-Liss, Inc.