title: Pattern reversal visual evoked potentials in phenylketonuria.
abstract: 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.
authors.affiliation: Clinica Pediatrica V, Istituto di Scienze Biomediche, Milano.
authors.forename: M
authors.initials: M
authors.lastname: Giovannini
authors.forename: R
authors.initials: R
authors.lastname: Valsasina
authors.forename: R
authors.initials: R
authors.lastname: Villani
authors.forename: A
authors.initials: A
authors.lastname: Ducati
authors.forename: E
authors.initials: E
authors.lastname: Riva
authors.forename: A
authors.initials: A
authors.lastname: Landi
authors.forename: R
authors.initials: R
authors.lastname: Longhi
mesh_terms: D000293:Adolescent; D001921:Brain / Q000503:physiopathology*; D002648:Child; D002675:Child, Preschool; D004569:Electroencephalography; D005074:Evoked Potentials, Visual; D005260:Female; D006801:Humans; D008297:Male; D010661:Phenylketonurias / Q000503:physiopathology*