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Pattern reversal visual evoked potentials in phenylketonuria
Author(s) -
Giovannini M.,
Valsasina R.,
Villani R.,
Ducati A.,
Riva E.,
Landi A.,
Longhi R.
Publication year - 1988
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1007/bf01800430
Subject(s) - visual evoked potentials , medicine , electroencephalography , evoked potential , myelin , endocrinology , neuroscience , audiology , psychology , central nervous system , psychiatry
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.