Behavioral Problems in PWS Children Assessed Differently by Parents and Teachers, Study Finds

Behavioral Problems in PWS Children Assessed Differently by Parents and Teachers, Study Finds
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Parents and teachers attribute different severity to behavioral problems of children with Prader–Willi syndrome (PWS), according to a recent study.

Although parents see food-related issues, such as repetitive speech and compulsive behavior as being more severe, teachers report that the impact of such problems on daily activities is higher.

The study, “Food and Non-Food-Related Behavior across Settings in Children with Prader–Willi Syndrome,” was published in the journal Genes.

Although the most notable clinical feature of PWS is an insatiable appetite, behavioral problems are common. These may include temper outbursts, obsessions, compulsive behavior such as skin picking, repetitive speech, low self-esteem, social withdrawal, and sadness.

To understand how behaviors can manifest differently in home and school settings, and how they can vary with patients’ characteristics (such as their genetic profiles), 86 parents and 63 teachers evaluated 100 children with PWS (age range 3–18, average age 9.9).

Genetic profile was known in 83 children; 55 had genetic deletions, 27 had maternal uniparental disomy (UPD), and one had an imprinting error. While maternal UPD refers to a child receiving two copies of chromosome 15 from the mother and none from the father, resulting in complete loss of paternal genes, genomic imprinting is the process by which one gene copy is inactivated by a DNA modification called methylation.

Behavior was assessed using the Global Assessment of Individual’s Behavior (GAIB), a test designed to identify behavioral problems associated with PWS. Higher scores in this test mean greater severity.

GAIB is comprised of seven sub-scales that assess social competence, general behavior problems, food- and non-food-related behavior problems, obsessive speech and compulsive behavior, as well as the interference of obsessive speech and compulsive behavior on daily function.

The team at RTI International found that parents scored obsessive speech and compulsive behaviors related to food higher than teachers.

“These findings suggest that the food-seeking behaviors of children with PWS may be less problematic in the school setting than at home,” the scientists wrote.

Food-related behavior issues (including crying, frustration, anger, and talking too much) had higher scores in parents’ vs teachers’ reports when the mother did not have a college degree.

“It could be that mothers with lower education levels are at a disadvantage with respect to resources for managing the home environment for their child with PWS,” the investigators wrote. They added that more studies on the effect of different education and socio-economic levels of mothers on their ability to manage the child’s condition may help find efficient ways to provide support.

In contrast, scores of general and non-food-related behavior problems, as well as those of non-food-related obsessive speech and compulsive behavior, did not differ significantly between parents’ and teachers’ reports.

Although they scored food-related obsessive speech and compulsive behaviors lower than parents, teachers said that, when these problems happened, they had a greater impact on daily activities.

“This is not surprising given expectations for maintaining order and adhering to schedules in the classroom, which may lead to more opportunities for obsessive speech and compulsive behaviors to be disruptive,” the researchers wrote.

Regarding genetic subtypes, the only difference was in the higher scores of overall behavior problems given by teachers to children with UPD compared to those with a genetic deletion.

“This study is the first that we know of that specifically examines food-related and non-food-related behaviors across home and school settings in children with PWS. Understanding how these behaviors manifest across different settings can help inform strategies to reduce behavior problems and improve outcomes,” the team added.

Alberto Molano was born in Bogotá, Colombia. He studied medicine at Universidad del Rosario and obtained a Ph.D. in Immunology from Weill Cornell Graduate School of Medical Sciences in New York. He conducted research and authored or co-authored twenty publications on molecular and cellular immunology, autoimmunity, immunology of aging and parasite immunology.
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José holds a PhD in Neuroscience from Universidade of Porto, in Portugal. He has also studied Biochemistry at Universidade do Porto and was a postdoctoral associate at Weill Cornell Medicine, in New York, and at The University of Western Ontario in London, Ontario, Canada. His work has ranged from the association of central cardiovascular and pain control to the neurobiological basis of hypertension, and the molecular pathways driving Alzheimer’s disease.

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Alberto Molano was born in Bogotá, Colombia. He studied medicine at Universidad del Rosario and obtained a Ph.D. in Immunology from Weill Cornell Graduate School of Medical Sciences in New York. He conducted research and authored or co-authored twenty publications on molecular and cellular immunology, autoimmunity, immunology of aging and parasite immunology.

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