Children with PWS show distinct profiles of cognitive, emotional difficulties
Study: Understanding differences may help provide more personalized support
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Children with Prader-Willi syndrome (PWS) have different combinations of cognitive and emotional difficulties, and while families experience similar caregiving burden regardless of their child’s profile, understanding these differences may help provide more personalized support for their behavior and daily functioning, according to a study.
“This study has provided a better understanding of how cognitive, emotional and behavioral regulation abilities are intertwined in children with PWS,” the researchers wrote.
The study, “Behavior Regulation Abilities in Children with Prader-Willi Syndrome and Their Impact on Daily Life: At the Crossroads of Cognitive and Emotional Development,” was published in the Journal of Autism and Developmental Disorders.
Behavioral regulation problems recurrent in PWS
PWS is a rare, complex genetic condition that is marked by a wide range of symptoms, including language, cognitive, and emotional delay, as well as excessive hunger (hyperphagia), irritability, temper outbursts, and sleep disturbances.
“Behavioral regulation problems – mostly hyperphagia and temper tantrums – are recurrent in PWS, with a considerable variability in their manifestations that are underpinned by cognitive deficits and emotional difficulties,” researchers wrote.
In this study, a team of researchers in France set out to explore the links between emotional and cognitive performances in children with PWS, as well as potential associations with behavioral disorders and family life.
The study included 25 children diagnosed with PWS, ages 9 to 15 years, and 25 children of the same age who had typical development. The children completed tests measuring intelligence, attention, decision-making, ability to recognize emotions, irritability, self-regulation, and depression. Parents completed questionnaires about their child’s emotions, behavior, and the impact of caring for their child on family life.
Children with PWS had lower intelligence scores, slower cognitive speed
Overall, the children with PWS performed significantly worse than the children with typical development. They had lower intelligence scores, more difficulty with attention, slower cognitive speed, weaker executive functions (which help plan, solve problems, and adapt to new situations) and more problems recognizing emotions.
Notably, average intelligence scores in the PWS group fell in the mild intellectual disability range, although abilities varied greatly from one child to another.
Parents reported that children with PWS experienced significantly more mood swings and irritability than children without PWS. However, children with PWS did not rate their irritability as highly as their parents did for them, suggesting they may not fully recognize or report their own difficulties.
The researchers also examined how different abilities were related. In children with typical development, only a few measures were linked, and these were mostly within the same area, such as attention or emotions. In contrast, children with PWS showed many strong links between cognitive skills, emotional regulation, and behavior.
For children with PWS, higher levels of irritability were linked with more mood swings, poorer self-regulation, more symptoms of depression, stronger hyperphagia-related behaviors, and more frequent temper tantrums. Lower cognitive abilities were also linked to poorer attention, executive function, and emotion recognition. Some behavioral problems were also linked to poorer emotion recognition and attention.
In addition, greater behavior problems, hyperphagia-related behaviors, and temper tantrums were linked to a heavier burden on families, which was associated with a poorer lower quality of life.
“Parental burden was significantly related to the quality of life,” the researchers wrote.
Families reported similar levels of caregiving burden
Because children with PWS differed greatly from one another in emotional, cognitive, and behavioral domains, the researchers grouped them into three profiles.
The first group had the lowest intelligence scores, weaker attention, poorer executive function, and more difficulty recognizing emotions. Some children could not complete all of the tests because they had difficulties understanding the instructions.
The second group had the most emotional and behavioral problems. These children showed the highest levels of irritability, mood swings, aggression, withdrawal from others, and temper tantrums.
The third group had relatively mild cognitive, executive, and emotional difficulties, and performed closest to children with typical development. However, many still showed higher levels of anxiety, meaning excessive worry or fear, even when other emotional problems were less severe.
[The findings suggest that] the variability of behavioral problems observed in children with PWS seems to be underpinned by cognitive and emotional regulatory mechanisms.
Further analyses showed that despite the clear differences among the three groups, families reported similar levels of caregiving burden (light to moderate) and quality of life (moderate).
Parents of children in the third group reported a burden that was closer to light, while for parents of children in the first and second groups, there was a wide variation in burden, between light and severe thresholds.
This suggests that “parental burden is felt more when there is a pronounced disorder, whatever its nature,” the researchers wrote.
The findings suggest that “the variability of behavioral problems observed in children with PWS seems to be underpinned by cognitive and emotional regulatory mechanisms,” the team wrote, adding that the group-separation approach “allows us to better understand developmental trajectories and contributes to improving the routine care, school support and hence the daily lives of people with PWS and their families.”
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