Skip to main content
Log in

Effect of l-dopa on visual evoked potentials and neuropsychological tests in adult phenylketonuria patients

  • Published:
European Journal of Pediatrics Aims and scope Submit manuscript

Abstract

Eight adult, untreated patients with classical phenylketonuria received L-dopa and a decarboxylase inhibitor for 2 weeks. No effect of l-dopa therapy on choice reaction time tasks, sustained attention, frontal lobal function as well as latencies of visual evoked potentials was found. The results raise the question if adult patients with phenylketonuria really suffer from functional dopamine deficiency.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

Abbreviations

CWIT :

Colour Word Interference Task (Stroop)

Phe :

phenylalanine

SVAT/DPE :

Sonneville Visual Attention Task/subtest: Dot Pattern Exercise

VEP :

visual evoked potential

References

  1. Anderson GM (1985) Liquid chromatographic analysis of monoamines and their metabolites. In: Boulton AA, Baker GB, Baker JM (eds) Neuromefhods 2, amines and their metabolites. Human Press, New Jersey, pp 128–196

    Google Scholar 

  2. Bäumler G (1985) Farbe-Wort-Interferenztest (FWIT) nach J. R. Stroop. Verlag für Psychologie, Dr. CJ Hogrefe, Göttingen

    Google Scholar 

  3. Bench CJ, Frith CD, Grasby PM, Fristen KJ, Paulesu E, Frackowiak RSJ, Dolan RJ (1993) Investigations of the functional anatomy of attention using the Stroop test. Neuropsychologia 31: 907–923

    Article  PubMed  CAS  Google Scholar 

  4. Bodis-Wollner I, Yahr MD, Mylin L, Thornton J (1982) Dopaminergic deficiency and delayed visual evoked potentials in humans. Ann Neurol 11: 478–483

    Article  PubMed  CAS  Google Scholar 

  5. Diamond A, Ciaramitaro V, Donner E, Djali S, Robinson MB (1994) An animal model of early-treated PKU. J Neurosci 14:3072–3082

    PubMed  CAS  Google Scholar 

  6. Dyer RS, Howell WE, McPhail RL (1981) Dopamine depletion slows retinal transmission. Exp Neurol 71: 326–340

    Article  PubMed  CAS  Google Scholar 

  7. Hommes FA (1993) The effect of hyperphenylalaninemia on the muscarinic acetylcholine receptors in the HPH-5 mouse brain. J Inherited Metab Dis 16: 962–974

    Article  PubMed  CAS  Google Scholar 

  8. Joyce JN, Marshall JF, Bankuwicz KS, Kopin IJ, Jacobowitz DM (1985) Hemiparkinsonism in a monkey after unilateral internal carotide artery infusion of l-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) is associated with regional ipsilateral changes in striatal dopamine D-2 receptor density. Brain Res 382:360–364

    Article  Google Scholar 

  9. Lange KW, Robbins TW, Marsden CD, James M, Owen AM, Paul GM (1992) l-Dopa withdrawal in Parkinson’s disease selectively impairs cognitive performance in tests sensitive to frontal lobe functions. Psychopharma-cology 107:394–404

    Article  CAS  Google Scholar 

  10. Leenders KL, Poewe WH, Palmer AJ, Brenton DP, Frackowiack RSJ (1986) Inhibition of l-[18F]fluorodopa uptake on the human brain by amino acids demonstrated by positron emission tomography. Ann Neurol 20:258–262

    Article  PubMed  CAS  Google Scholar 

  11. Lou HC, Lykkelund C, Gerdes A-M, Udesen H, Bruhn P (1987) Increased vigilance and dopamine synthesis by large doses of tyrosine or phenylalanine restriction in phenylketonuria. Acta Paediatr Scand 76:560–565

    Article  PubMed  CAS  Google Scholar 

  12. Ludolph AC, Ullrich K, Nedjat S, Masur H, Bick U (1991) Neurological outcome in 22 treated adolescent patients with hyperphenylalaninemia. A clinical and electrophysiological study. Acta Neurol Scand 83:161–185

    Article  PubMed  CAS  Google Scholar 

  13. Mc Kean CM (1972) The effect of high phenylalanine concentrations on serotonin and catecholamine metabolism in human brain. Brain Res 47: 469–476

    Article  CAS  Google Scholar 

  14. Milner JD, Wurtmann RJ (1986) Effects of phenylalanine on the release of endogenous dopamine from rat striatal slices. J Neurochem 47:1444–1448

    Article  PubMed  CAS  Google Scholar 

  15. Nutt JG, Woodward WR, Hammerstad JP, Carter JH, Anderson JL (1984) The „on-off’ phenomenon in Parkinson’s disease. Relation to levodopa adsorption and transport. N Engl J Med 310: 483–488

    PubMed  CAS  Google Scholar 

  16. Pardo JV, Pardo PJ, Janer KW, Raichle ME (1990) The anterior cingulate cortex mediates processing selection in the Stroop attenentional conflict paradigm. Proc Natl Acad Sci USA 87:256–259

    Article  PubMed  CAS  Google Scholar 

  17. Phillis JW, Tebecis AK, York DH (1967) The inhibitory action of monoamines on lateral geniculate neurons. J Physiol 190:563–581

    PubMed  CAS  Google Scholar 

  18. Pietz J, Landwehr R, Kutsch A, Schmidt H, de Sonneville LMJ, Trefz F. Effect of high-dose tyrosine supplementation on brain function in adults with phenylketonuria. J Pediatr (in press)

  19. Pinder RM, Brodge RN, Sawyer PR, Speight TM, Avery GS (1976) Larodopa and decarboxylase inhibitors: a review of their clinical pharmacology and use in treatment of parkinsonism. Drugs 11:329–377

    Article  PubMed  CAS  Google Scholar 

  20. Schmidt E, Rupp A, Burgard P, Pietz J, Weglage J, Sonneville LMJ de (1995) Sustained attention in adult phenylketonuria: The influence of the concurrent phenylalanine blood-levels. J Clin Exp Neuropsychol (in press)

  21. Sonneville LMJ de (1988) Information procession and neonatal neurological optimality. Free University Press, Amsterdam

    Google Scholar 

  22. Stam GJ, Visser SL, Ob de Coul AAW, Sonneville LMJ de, Schellens RLLA, Brunia CHM, Smet JS de, Gielen G (1993) Disturbed frontal regulation of attention in Parkinson’s disease. Brain 116:1139–1158

    Article  PubMed  Google Scholar 

  23. Ullrich K, Möller H, Weglage J, Schuierer G, Bick U, Ludolph A, Hahn-Ullrich H, Fünders B (1995) White matter abnormalities in phenylketonuria: Results of magnetic resonance imaging. Acta Paediatr 73 [Suppl] 407:78–82

    Google Scholar 

  24. Weglage J, Pietsch M, Fünders B, Koch H-G, Ullrich K (1995) Deficits in selective attention processes in early treated patients with phenylketonuria — a correlate of frontal lobe dysfunction? Eur J Pediatr (in press)

  25. Welsh MC, Pennington BF, Ozonoff S, Rouse B, McCabe ERB (1990) Neuropsychology of early-treated phenylketonuria: specific executive function deficits. Child Dev 61:1697–1713

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Ullrich, K., Weglage, J., Pietsch, M. et al. Effect of l-dopa on visual evoked potentials and neuropsychological tests in adult phenylketonuria patients. Eur J Pediatr 155 (Suppl 1), S74–S77 (1996). https://doi.org/10.1007/PL00014256

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/PL00014256

Key words

Navigation