Scholarlink

Purpose <p>Prader-Willi Syndrome (PWS) is a rare, complex, genetic neurodevelopmental disorder that induces a hypothalamic dysfunction with a global developmental delay and specific trajectories. Behavioral and psychiatric disorders are recurrent in PWS, emerging during childhood and varying considerably in their manifestations. This study aimed to gain a better understanding of the relationship between emotion, cognitive and behavioral regulation abilities in children with PWS, and explore the impact on daily life.</p> Methods <p>Twenty-five children with PWS aged between 9 and 15 took part in the study, along with an equal number of age-matched control children. A series of assessments was proposed to children and their parents to measure cognitive and executive competencies, affective and behavioral problems, and family impact.</p> Results <p>The children with PWS were clearly distinguished from the control group, displaying marked deficits in cognitive skills, including executive ones, and affective problems. Inter-task correlation analyses showed that many variables were significantly interrelated in the PWS group, in contrast with the control group. Inter-individual variation was strong and cluster analyses revealed that three subgroups emerged; one marked by cognitive and executive deficits, a second by a high level of affective problems, while a third displayed relatively preserved cognitive, executive and emotional skills. The three clusters did not differ in the family impact.</p> Conclusion <p>The variability of behavioral problems observed in children with PWS seems to be underpinned by cognitive and emotional regulatory mechanisms. This original approach is discussed in light of the literature.</p>

错误：搜索内容不能为空，请输入英文关键词
错误：关键词超出字数限制，请精简
高级检索

Behavior Regulation Abilities in Children With Prader-Willi Syndrome and Their Impact on Daily Life: At the Crossroads of Cognitive and Emotional Development

  • 期刊论文

  • 发表日期：

    2026年7月2日

查看全文
  • Nawelle Famelart,
  • A. M. Camblats,
  • J. Cortadellas,
  • M. Valette,
  • C. Brochado,
  • S. Faye,
  • C. Molinas,
  • M. Glattard,
  • J. Maire,
  • S. Çabal,
  • G. Diene,
  • M. Tauber,
  • V. Postal

摘要

Purpose

Prader-Willi Syndrome (PWS) is a rare, complex, genetic neurodevelopmental disorder that induces a hypothalamic dysfunction with a global developmental delay and specific trajectories. Behavioral and psychiatric disorders are recurrent in PWS, emerging during childhood and varying considerably in their manifestations. This study aimed to gain a better understanding of the relationship between emotion, cognitive and behavioral regulation abilities in children with PWS, and explore the impact on daily life.

Methods

Twenty-five children with PWS aged between 9 and 15 took part in the study, along with an equal number of age-matched control children. A series of assessments was proposed to children and their parents to measure cognitive and executive competencies, affective and behavioral problems, and family impact.

Results

The children with PWS were clearly distinguished from the control group, displaying marked deficits in cognitive skills, including executive ones, and affective problems. Inter-task correlation analyses showed that many variables were significantly interrelated in the PWS group, in contrast with the control group. Inter-individual variation was strong and cluster analyses revealed that three subgroups emerged; one marked by cognitive and executive deficits, a second by a high level of affective problems, while a third displayed relatively preserved cognitive, executive and emotional skills. The three clusters did not differ in the family impact.

Conclusion

The variability of behavioral problems observed in children with PWS seems to be underpinned by cognitive and emotional regulatory mechanisms. This original approach is discussed in light of the literature.