Rare Diseases Symptoms Automatic Extraction

Executive function in treated phenylketonuria as measured by the one-back and two-back versions of the continuous performance test.

[classical phenylketonuria]

The executive dysfunction hypothesis in treated phenylketonuria was investigated by means of the one-back and two-back versions of the continuous performance test. Eleven non-retarded, primary school-age children with classical phenylketonuria who had been treated early and continuously were indistinguishable from healthy, matched controls on the measures and test performance was not predicted by historical or concurrent plasma phenylalanine concentrations. Mean lifetime phenylalanine concentrations for the phenylketonuric subjects were within ranges currently recommended as dietary optima in the United Kingdom. The negative neuropsychological test findings add weight to the rationale for this policy.