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Adaptive and maladaptive behavior in Prader-Willi syndrome.
E M Dykens1, R M Hodapp, K Walsh
1Yale University School of Medicine, New Haven, Connecticut.
Journal of the American Academy of Child and Adolescent Psychiatry
|November 1, 1992
Summary
Adaptive and maladaptive behaviors in Prader-Willi syndrome were studied. Daily living skills improved with age, while socialization remained a challenge, with persistent behavioral issues into adulthood.
Area of Science:
- Neurodevelopmental Disorders
- Behavioral Psychology
Background:
- Prader-Willi syndrome (PWS) is a complex genetic disorder affecting multiple body systems.
- Understanding behavioral profiles is crucial for PWS management and support.
Purpose of the Study:
- To examine adaptive and maladaptive behavior development in adolescents and adults with PWS.
- To identify age-related behavioral patterns in this population.
Main Methods:
- Cross-sectional study of 21 individuals with PWS.
- Utilized Vineland Adaptive Behavior Scales and Child Behavior Checklist (CBCL).
Main Results:
- Adaptive strengths noted in daily living skills, increasing with age.
- Socialization deficits were prominent, particularly coping skills.
- Externalizing behaviors (e.g., tantrums, defiance) persisted into adulthood.
- Aging in PWS may correlate with increased confusion, withdrawal, and fatigue.
Conclusions:
- Daily living skills show positive development in PWS, but socialization remains a significant challenge.
- Maladaptive behaviors, especially externalizing ones, are persistent and require ongoing management.
- Further research across a wider age range is needed to fully understand PWS behavioral trajectories.