Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2009 Jan;23(1):130-4.
doi: 10.1037/a0013488.

Response monitoring in children with phenylketonuria

Gabriel C Araujo  1 , Shawn E Christ, Robert D Steiner, Dorothy K Grange, Binyam Nardos, Robert C McKinstry, Desirée A White
Affiliations

Response monitoring in children with phenylketonuria

Gabriel C Araujo et al. Neuropsychology. 2009 Jan.

Abstract

Phenylketonuria (PKU) is characterized by a disruption in the metabolism of phenylalanine and is associated with dopamine deficiency (Diamond, Prevor, Callender, & Druin, 1997) and cerebral white matter abnormalities (e.g., Anderson et al., 2007). From a neuropsychological perspective, prefrontal dysfunction is thought to underlie the deficits in executive abilities observed in individuals with PKU (Christ, Steiner, Grange, Abrams, & White, 2006; Diamond et al., 1997; White, Nortz, Mandernach, Huntington, & Steiner, 2001, 2002). The purpose of our study was to examine a specific aspect of executive ability, response monitoring, as measured by posterror slowing. The authors examined posterror reaction time (RT) in 24 children with well-controlled, early treated PKU and 25 typically developing control children using a go/no-go task. Results showed that RTs of both controls and children with PKU slowed significantly following the commission of errors. The magnitude of posterror slowing, however, was significantly less for children with PKU. These findings indicate deficient response monitoring in children with PKU.

PubMed Disclaimer

Figures

Figure 1
Figure 1
Mean number of errors for PKU and control groups.
Figure 2
Figure 2
Mean RT for PKU and control groups.
See this image and copyright information in PMC

References

    1. Anderson PJ, Wood SJ, Francis DE, Coleman L, Anderson V, Boneh Are neuropsychological impairments in children with early-treated phenylketonuria (PKU) related to white matter abnormalities or elevated phenylalanine levels? Developmental Neuropsychology. 2007;32:645–668. - PubMed
    1. Antshel KM, Waisbren SE. Developmental timing of exposure to elevated levels of phenylalanine is associated with ADHD symptom expression. Journal of Abnormal Child Psychology. 2003;31:565–574. - PubMed
    1. Arnold GL, Vladutiu CJ, Orlowski CC, Blakely EM, DeLuca J. Prevalence of stimulant use for attentional dysfunction in children with phenylketonuria. Journal of Inherited Metabolic Disease. 2004;27:137–143. - PubMed
    1. Carter CS, Braver TS, Barch DM, Botvinick MM, Noll D, Cohen JD. Anterior cingulate cortex, error detection, and the online monitoring of performance. Science. 1998;280:747–749. - PubMed
    1. Christ SE, Steiner RD, Grange DK, Abrams RA, White DA. Inhibitory control in children with phenylketonuria. Developmental Neuropsychology. 2006;30:845–864. - PubMed

Publication types