Interhemispheric Transfer in Children with Early-Treated Phenylketonuria

Monica L. Gourovitch, Suzanne Craft*, S. Bruce Dowton, Peter Ambrose, Steven Sparta

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

31 Citations (Scopus)


Phenylketonuria (PKU) is a genetic disorder of amino acid metabolism that is associated with brain catecholamine depletion and deficient myelination. Although neuropsychological deficits have been documented in children with early-treated PKU (ETPKU), no study to date has examined possible effects of impaired myelination in this population. In the present study, interhemispheric transfer time was assessed for 14 children with ETPKU, 22 children with attention deficit-hyperactivity disorder, and 48 normal children, using a manual reaction time paradigm previously validated with callosal agenesis patients (Milner, 1982). Children with ETPKU demonstrated slowed interhemispheric transfer from the left to the right hemisphere as compared with the two other groups. The magnitude of slowing was correlated with age and phenylalanine levels at birth. Results support the hypothesis that abnormal myelination disrupts the development of interhemispheric connections in ETPKU, and suggest that left hemisphere projections may be particularly susceptible to such disruption.

Original languageEnglish
Pages (from-to)393-404
Number of pages12
JournalJournal of Clinical and Experimental Neuropsychology
Issue number3
Publication statusPublished - 1 Jun 1994
Externally publishedYes


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