Behavior and emotional disturbance in Prader-Willi syndrome

Stewart L Einfeld, Arabella Smith, Seeta Durvasula, Tony Florio, Bruce J Tonge

Research output: Contribution to journalArticleResearchpeer-review

Abstract

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 was compared with that of control individuals derived from a community sample (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 overall, and especially in antisocial behavior. In addition, some individual behaviors were more common in PWS subjects than controls. When these behaviors are considered together with findings from other studies using acceptably rigorous methods, a consensus behavior phenotype for PWS can be formulated. This will provide a valid foundation for studies of the mechanism of genetic pathogenesis of behavior in PWS.
Original languageEnglish
Pages (from-to)123-127
Number of pages5
JournalAmerican Journal of Medical Genetics
Volume82
Issue number2
DOIs
Publication statusPublished - 1999

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