Decreased vigilance and neurotransmitter synthesis after discontinuation of dietary treatment for phenylketonuria in adolescents

Four adolescent or young adult patients with phenylketonuria were examined before and after discontinuation of dietary treatment. Plasma and CSF phenylalanine concentrations increased about two-fold in three patients. In these patients the CSF concentration of the dopamine and serotonin metabolites...

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Bibliographic Details
Published in:European journal of pediatrics 1985-05, Vol.144 (1), p.17-20
Main Authors: LOU, H. C, GUTTLER, F, LYKKELUND, C, BRUHN, P, NIEDERWIESER, A
Format: Article
Language:English
Subjects:
Adolescent
Adult
Amino Acids - metabolism
Aminoacid disorders
Arousal
Biological and medical sciences
Dopamine - biosynthesis
Errors of metabolism
Female
Homovanillic Acid - metabolism
Humans
Hydroxyindoleacetic Acid - metabolism
Male
Medical sciences
Metabolic diseases
Phenylketonurias - diet therapy
Phenylketonurias - metabolism
Serotonin - biosynthesis
Time Factors
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Summary:Four adolescent or young adult patients with phenylketonuria were examined before and after discontinuation of dietary treatment. Plasma and CSF phenylalanine concentrations increased about two-fold in three patients. In these patients the CSF concentration of the dopamine and serotonin metabolites homovanillic acid (HVA) and 5-hydroxyindoleacetic acid (5-HIAA) decreased markedly; 5-HIAA to extremely low values. The reaction time variability increased in these patients. In the fourth case plasma phenylalanine levels, CSF HVA and 5-HIAA levels, and reaction time variability were essentially unchanged. The relationship between reaction time variability and the CSF 5-HIAA level for all four patients could be presented as a linear function. However, a causal relationship is still unproven. These preliminary findings demonstrate that there may be hazards in the discontinuation of dietary treatment, even in adolescents or young adults, for neurotransmitter metabolism and mental function.
ISSN:0340-6199
1432-1076
DOI:10.1007/BF00491918