title: Early signs of visual perception and evoked potentials in radiologically asymptomatic boys with X-linked adrenoleukodystrophy.
abstract: The aim was to identify the electrophysiological and psychological signs at a very early stage in asymptomatic boys with childhood cerebral X-linked adrenoleukodystrophy. Flash visual evoked potentials, pattern reversal, and visual event-related potentials were recorded in 6 radiologically asymptomatic boys with adrenoleukodystrophy and 22 control boys. The latency and amplitude of P100 of visual evoked potentials and P1 of event-related potentials were evaluated. Though all patients had normal intelligence quotient, performance intelligence quotient was significantly lower than verbal intelligence quotient in 2 patients. Both P100 and P1 amplitudes were significantly greater in adrenoleukodystrophy than in controls. The difference between performance intelligence quotient and verbal intelligence quotient exhibited significant correlation with P100 amplitude. Enlargement of visual evoked potentials might be a sign of cerebral involvement preceding the appearance of abnormalities on magnetic resonance imaging. Follow-up of asymptomatic boys with both electrophysiological and neuropsychological tests may serve as an aid for deciding the timing of therapeutic intervention.
authors.affiliation: Department of Developmental Disorders, National Institute of Mental Health, National Center of Neurology and Psychiatry, Tokyo, Japan.
authors.forename: Wakana
authors.initials: W
authors.lastname: Furushima
authors.forename: Masumi
authors.initials: M
authors.lastname: Inagaki
authors.forename: Atsuko
authors.initials: A
authors.lastname: Gunji
authors.forename: Yuki
authors.initials: Y
authors.lastname: Inoue
authors.forename: Makiko
authors.initials: M
authors.lastname: Kaga
authors.forename: Shuki
authors.initials: S
authors.lastname: Mizutani
mesh_terms: D000293:Adolescent; D000326:Adrenoleukodystrophy / Q000473:pathology / Q000503:physiopathology*; D000375:Aging; D002648:Child; D005074:Evoked Potentials, Visual; D006801:Humans; D007360:Intelligence; D007361:Intelligence Tests; D008279:Magnetic Resonance Imaging; D008297:Male; D009483:Neuropsychological Tests; D010775:Photic Stimulation; D011930:Reaction Time; D013997:Time Factors; D014796:Visual Perception / Q000502:physiology*