Results of phenylalanine tolerance tests and EEG examination in patients under treatment for phenylketonuria.
The EEG of 10 phenylketonuric children treated by a diet and of 4 children with hyperphenylalaninaemia on a normal diet was recorded during L-phenylalanine tolerance tests. The first recording was made before administering the L-phenylalanine load (100 mg/kg b.w.) and the second 60-120 min after. In 6 of the phenylketonuric children, nonspecific abnormality became more pronounced or increased, or previously absent epileptic graphoelements appeared. A pathological EEG response to a L-phenylalanine load was found in 1 child with hyperphenylalaninaemia. The authors do not recommend relaxation of the diet in children with a pathological EEG response to a L-phenylalanine load.
D000328:Adult; D002648:Child; D003937:Diagnosis, Differential; D004569:Electroencephalography; D006579:Heterozygote; D006801:Humans; D010649:Phenylalanine; D010661:Phenylketonurias / Q000175:diagnosis* / Q000235:genetics
D010649:Phenylalanine