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Updated: Jul 3, 2026

Psychophysiological Assessment of the Effectiveness of Emotion Regulation Strategies in Childhood
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Psychophysiological Assessment of the Effectiveness of Emotion Regulation Strategies in Childhood

Published on: February 11, 2017

Behavior Regulation Abilities in Children With Prader-Willi Syndrome and Their Impact on Daily Life: At the

Nawelle Famelart1, A M Camblats2, J Cortadellas3

  • 1Centre de Référence Maladies Rares PRADORT, Hôpital des Enfants, CHU de Toulouse, 330, Avenue de Grande Bretagne, TSA 70034, 31059, Toulouse Cedex 9, France. famelart.n@chu-toulouse.fr.

Journal of Autism and Developmental Disorders
|July 1, 2026
PubMed

Insights

Children with Prader-Willi Syndrome (PWS) show distinct cognitive and emotional regulation deficits. These underlying mechanisms contribute to varied behavioral issues in PWS, impacting daily life differently across individuals.

Area of Science:

  • Neurodevelopmental Disorders
  • Pediatric Neurology
  • Genetics

Background:

  • Prader-Willi Syndrome (PWS) is a rare genetic disorder characterized by hypothalamic dysfunction, global developmental delay, and specific developmental trajectories.
  • Behavioral and psychiatric disorders are common in children with PWS, manifesting with significant variability.

Purpose of the Study:

  • To investigate the interplay between emotion, cognitive, and behavioral regulation in children with PWS.
  • To understand how these regulatory abilities impact the daily lives of children with PWS.

Main Methods:

  • A cohort of 25 children with PWS (ages 9-15) and 25 age-matched controls participated.
  • Assessments evaluated cognitive/executive functions, affective/behavioral issues, and family impact for both groups.

Main Results:

  • Children with PWS exhibited significant deficits in cognitive, executive, and affective domains compared to controls.
  • Inter-individual variability was high, with cluster analysis identifying three subgroups: cognitive/executive deficits, high affective problems, or relatively preserved skills.
  • No significant differences in family impact were found across the three PWS subgroups.

Conclusions:

  • Cognitive and emotional regulatory mechanisms appear to underlie the diverse behavioral problems observed in children with PWS.
  • This study provides novel insights into the heterogeneity of PWS manifestations and their regulatory underpinnings.
Abstract

Keywords:
Behavioral regulationChildrenCognitionEmotionImpact of familyPrader-Willi syndrome

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