European Journal of Pediatrics

, Volume 144, Issue 1, pp 17–20

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

  • H. C. Lou
  • F. Güttler
  • C. Lykkelund
  • P. Bruhn
  • A. Niederwieser
Original Investigations

DOI: 10.1007/BF00491918

Cite this article as:
Lou, H.C., Güttler, F., Lykkelund, C. et al. Eur J Pediatr (1985) 144: 17. doi:10.1007/BF00491918

Abstract

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.

Key words

Attention Serotonin Dopamine PKU 

Abbreviations

PKU

phenylketonuria

RT

reaction time

HVA

homovanillic acid

5-HIAA

5-hydroxyindoleacetic acid

Copyright information

© Springer-Verlag 1985

Authors and Affiliations

  • H. C. Lou
    • 1
  • F. Güttler
    • 1
  • C. Lykkelund
    • 1
  • P. Bruhn
    • 2
  • A. Niederwieser
    • 3
  1. 1.The John F. Kennedy InstituteGlostrupDenmark
  2. 2.Department of NeurologyRigshospitaletCopenhagen 0Denmark
  3. 3.Department of PediatricsUniversity of ZürichZürichSwitzerland

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