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Age-related working memory impairments in children with prefrontal dysfunction associated with phenylketonuria

Published online by Cambridge University Press:  11 January 2002

DESIRÉE A. WHITE ,
MARSHA J. NORTZ ,
TAMMY MANDERNACH ,
KATHLEEN HUNTINGTON  and
ROBERT D. STEINER
DESIRÉE A. WHITE
Affiliation:
Department of Psychology, Washington University, St. Louis, Missouri
MARSHA J. NORTZ
Affiliation:
Department of Psychology, Washington University, St. Louis, Missouri
TAMMY MANDERNACH
Affiliation:
Department of Psychology, Washington University, St. Louis, Missouri
KATHLEEN HUNTINGTON
Affiliation:
Child Development and Rehabilitation Center, Doernbecher Children's Hospital, Portland, Oregon
ROBERT D. STEINER
Affiliation:
Child Development and Rehabilitation Center, Doernbecher Children's Hospital, Portland, Oregon Departments of Pediatrics and Molecular and Medical Genetics, Oregon Health Sciences University, Portland, Oregon
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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. (JINS, 2002, 8, 1–11.)

Keywords

Working memoryPrefrontal cortexDopaminePhenylketonuriaChildren
Type
Research Article
Information
Journal of the International Neuropsychological Society , Volume 8 , Issue 1 , January 2002 , pp. 1 - 11
Copyright
© 2002 The International Neuropsychological Society

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