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Age-related working memory impairments in children with prefrontal dysfunction associated with phenylketonuria
Desirée A White1, Marsha J Nortz, Tammy Mandernach
1Department of Psychology, Washington University, St Louis, Missouri 63130, USA. dawhite@artsci.wustl.edu
Insights
Children with phenylketonuria (PKU) show working memory deficits that emerge with age, indicating a developmental deficit rather than a delay. This impacts cognitive development in individuals with PKU.
Area of Science:
- Neuroscience
- Developmental Psychology
- Metabolic Disorders
Background:
- The prefrontal cortex is vital for working memory.
- Age-related prefrontal changes influence childhood working memory development.
- Phenylketonuria (PKU) is a metabolic disorder linked to prefrontal dysfunction.
Purpose of the Study:
- To investigate the working memory development in children with early-treated PKU.
- To compare the working memory trajectory in children with PKU to typically developing children.
Main Methods:
- Evaluated working memory using a recognition procedure.
- Assessed memory for letters, abstract objects, and spatial locations.
- Included 20 children with PKU and 20 controls (ages 6-17).
Main Results:
- Children with PKU demonstrated poorer performance across all tested materials.
- Working memory deficits in PKU were more pronounced in older children.
- A significant difference in developmental trajectory was observed compared to controls.
Conclusions:
- Findings suggest a developmental deficit, not a delay, in working memory for children with PKU.
- Prefrontal dysfunction in PKU impacts working memory development.
- Early intervention in PKU may need to address cognitive trajectories.
Abstract:
The prefrontal cortex of the brain has been shown to play a crucial role in working memory, and age-related changes in prefrontal function may contribute to the improvements in working memory that are observed during childhood. We examined the developmental trajectory of working memory in school-age children with early-treated phenylketonuria (PKU), a metabolic disorder that results in prefrontal dysfunction. Using a recognition procedure, we evaluated working memory for letters, abstract objects, and spatial locations in 20 children with PKU and 20 typically developing control children. Children in both groups ranged from 6 to 17 years of age. Our findings revealed poorer performance across all three types of materials for children with PKU. In addition, there was a significant difference in the developmental trajectory of working memory for children with PKU as compared with controls. Specifically, deficits were not apparent in younger children with PKU. Instead, deficits were observed only in older children, suggesting the presence of a developmental deficit rather than a developmental delay in the working memory of children with PKU.