Event-related potentials in adults with Phenylketonuria

Jonson Moyle, Allison Fox, Michael Bynevelt, M. Arthur, John Burnett

Research output: Chapter in Book/Conference paperConference paperpeer-review


Objective: Phenylketonuria (PKU) is an autosomal recessive disorder of phenylalanine metabolism that if untreated leads to severe cognitive difficulties and structural brain damage. Early dietary therapy prevents the global cognitive impairments, however, diet-treated patients still experience cognitive and neurophysiological abnormalities. Specifically, deficits in sustained attention and response inhibition are observed in adults with PKU. The event-related potential (ERP) permits the detection of changes in cortical activity related to sensory and cognitive processes, which is not possible through examining behavioural outcomes. This study used ERPs to examine attentional and inhibitory differences between early-treated PKU adults and matched controls. Participants and Methods : Electroencephalogram scalp recordings, used to derive ERPs, were recorded from eighteen participants (PKU n=9; control n=9) while they completed a visual Go-Nogo task. Frontocentral and parieto-occipital sites were examined. Results : No behavioural differences were found between the two groups. Despite the absence of behavioural differences, repeated measures ANOVA revealed the PKU group had significantly decreased P1 and N1 peak amplitudes at occipital and parieto-occipital sites in both Go and Nogo conditions and reduced fronto-central Nogo-N2 amplitude compared to the control group (all p
Original languageEnglish
Title of host publicationJournal of the International Neuropsychological Society
Number of pages1
Publication statusPublished - 2006
EventThirty-Fourth Annual International Neuropsychological Society Conference - Boston, United States
Duration: 1 Feb 20064 Feb 2006


ConferenceThirty-Fourth Annual International Neuropsychological Society Conference
Country/TerritoryUnited States


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