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Response monitoring in children with phenylketonuria

Gabriel C Araujo1, Shawn E Christ, Robert D Steiner

  • 1Department of Psychology, Washington University, St.Louis, MO 63130, USA.

Neuropsychology
|February 13, 2009
PubMed

Insights

Children with phenylketonuria (PKU) show impaired response monitoring. This study found that children with PKU exhibit less posterror slowing, indicating a deficit in their ability to monitor and adjust their performance after making mistakes.

Area of Science:

  • Neuropsychology
  • Developmental Psychology
  • Metabolic Disorders

Background:

  • Phenylketonuria (PKU) disrupts phenylalanine metabolism, leading to dopamine deficiency and white matter abnormalities.
  • Neuropsychological studies link prefrontal dysfunction to executive ability deficits in individuals with PKU.
  • Executive functions, crucial for cognitive control, are often impaired in PKU.

Purpose of the Study:

  • To investigate response monitoring, a key executive ability, in children with PKU.
  • To measure posterror slowing as an indicator of response monitoring efficiency.
  • To compare response monitoring between early-treated children with PKU and typically developing controls.

Main Methods:

  • A go/no-go task was administered to assess reaction times.
  • Posterror reaction times were analyzed in 24 children with well-controlled, early-treated PKU.
  • Reaction times of 25 typically developing children served as controls.

Main Results:

  • Both control children and children with PKU demonstrated significant posterror slowing.
  • The magnitude of posterror slowing was significantly reduced in children with PKU compared to controls.
  • This suggests a deficiency in response monitoring in children with PKU.

Conclusions:

  • Children with PKU exhibit deficient response monitoring.
  • Impaired response monitoring may contribute to executive function deficits observed in PKU.
  • Early and well-controlled treatment may not fully normalize response monitoring abilities.

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