title: Response monitoring in children with phenylketonuria.
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.
authors.affiliation: Department of Psychology, Washington University, St.Louis, MO 63130, USA.
authors.forename: Gabriel C
authors.initials: GC
authors.lastname: Araujo
authors.forename: Shawn E
authors.initials: SE
authors.lastname: Christ
authors.forename: Robert D
authors.initials: RD
authors.lastname: Steiner
authors.forename: Dorothy K
authors.initials: DK
authors.lastname: Grange
authors.forename: Binyam
authors.initials: B
authors.lastname: Nardos
authors.forename: Robert C
authors.initials: RC
authors.lastname: McKinstry
authors.forename: Desirée A
authors.initials: DA
authors.lastname: White
mesh_terms: D000293:Adolescent; D000367:Age Factors; D001288:Attention / Q000502:physiology; D002648:Child; D003072:Cognition Disorders / Q000209:etiology*; D005260:Female; D006801:Humans; D007266:Inhibition, Psychological; D008297:Male; D009483:Neuropsychological Tests; D010649:Phenylalanine / Q000652:urine; D010661:Phenylketonurias / Q000150:complications* / Q000523:psychology*; D011597:Psychomotor Performance / Q000502:physiology; D011930:Reaction Time / Q000502:physiology
chemical_list: D010649:Phenylalanine