P. Griffiths et al., EXECUTIVE FUNCTION IN TREATED PHENYLKETONURIA AS MEASURED BY THE ONE-BACK AND 2-BACK VERSIONS OF THE CONTINUOUS PERFORMANCE-TEST, Journal of inherited metabolic disease, 21(2), 1998, pp. 125-135
The executive dysfunction hypothesis in treated phenylketonuria was in
vestigated by means of the one-back and two-back versions of the conti
nuous performance test. Eleven non-retarded, primary school-age childr
en with classical phenylketonuria who had been treated early and conti
nuously were indistinguishable from healthy, matched controls on the m
easures and test performance was not predicted by historical or concur
rent plasma phenylalanine concentrations. Mean lifetime phenylalanine
concentrations for the phenylketonuric subjects were within ranges cur
rently recommended as dietary optima in the United Kingdom. The negati
ve neuropsychological test findings add weight to the rationale for th
is policy.