Pattern-reversal visual evoked potentials in phenylketonuric children

Pattern-reversal visual evoked potentials (PR-VEPs) and EEG were recorded in 14 phenylketonuric (PKU) children on a low-phenylalanine (phe) diet; the data obtained were correlated with metabolic parameters, namely, the actual phe plasma level, the mean phe plasma level in the last year, an the beginning of the diet. PR-VEPs seem to be more sensitive than EEG in detecting neurophysiological derangements in these subjects; in fact PR-VEPs were pathological in six patients while EEG detected three; no significant alterations were found in the neurophysiological tests among the children with good metabolic control, and only one child was abnormal among the six on an early dietetic regimen; in contrast, six of the nine subjects presenting with high mean phe plasma levels (greater than 10 mg/100 ml) and five of the eight whose diet started after the 2nd month of life showed pathological PR-VEPs.