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Arch Dis Child 1998;78:116-121 ( February )

Randomised controlled trial of tyrosine supplementation on neuropsychological performance in phenylketonuria

Mary Lou Smith,a William B Hanley,b Joe T R Clarke,b Paula Klim,a Wanda Schoonheyt,b Valerie Austin,b Denis C Lehotayc

a University of Toronto and Hospital for Sick Children, Toronto, Canada: Department of Psychology, b Department of Pediatrics and Division of Clinical Genetics, c Clinical Biochemistry

Correspondence to: Dr Mary Lou Smith, Department of Psychology, Erindale College, University of Toronto, Mississauga, Ontario L5L 1C6, Canada. e-mail: smithml{at}psych.utoronto.ca


Accepted 4 September 1997

OBJECTIVE---To test the efficacy of tyrosine supplementation, as an adjunct to dietary treatment, on neuropsychological test performance in individuals with phenylketonuria.
DESIGN---A randomised controlled trial of tyrosine supplementation using a double blind crossover procedure with three four week phases.
SETTING---The Hospital for Sick Children, Toronto.
PARTICIPANTS---21 individuals with phenylketonuria (ages 6 to 28 years, mean 11.3).
INTERVENTION---Participants were given 100 mg/kg body weight/d of L-tyrosine or L-alanine (placebo).
RESULTS---At baseline, performance on several of the neuropsychological test measures was correlated with tyrosine levels. Dietary supplements of tyrosine increased plasma tyrosine concentrations; however, no change in test performance was found across the tyrosine and placebo phases of the study.
CONCLUSIONS---Tyrosine supplementation of this type does not appear to alter neuropsychological performance in individuals with phenylketonuria.

Keywords: phenylketonuria; tyrosine; phenylalanine; frontal lobe function

© 1998 by Archives of Disease in Childhood


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