Journal of Inherited Metabolic Disease

, Volume 11, Issue 4, pp 416–421 | Cite as

Pattern reversal visual evoked potentials in phenylketonuria

  • M. Giovannini
  • R. Valsasina
  • R. Villani
  • A. Ducati
  • E. Riva
  • A. Landi
  • R. Longhi
Article

Summary

The pathogenesis of brain dysfunction in phenylketonuria (PKU) is still under investigation. Hyperphenylalaninaemia results in increased turnover of myelin. In order to demonstrate the derangement of myelinization in PKU we studied the visual evoked potentials (VEP) in 14 PKU patients and in 20 normal subjects. VEP findings were correlated with the metabolic control of the disease and with the electroencephalographic findings. VEP were more sensitive than the EEG in detecting a neurological dysfunction. VEP are influenced by dietary control and are normal only in children with good metabolic control.

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Copyright information

© SSIEM and MTP Press Limited 1988

Authors and Affiliations

  • M. Giovannini
    • 1
  • R. Valsasina
    • 1
  • R. Villani
    • 2
  • A. Ducati
    • 2
  • E. Riva
    • 1
  • A. Landi
    • 2
  • R. Longhi
    • 1
  1. 1.Clinica Pediatrica V, Istituto di Scienze BiomedicheUniversità di MilanoMilano
  2. 2.Istituto di NeurochirurgiaUniversità di MilanoMilano

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