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Early Signs of Visual Perception and Evoked Potentials in Radiologically Asymptomatic Boys With X-linked Adrenoleukodystrophy

Department of Pediatrics and Developmental Biology, Graduate School of Medicine, Tokyo Medical and Dental University, Tokyo, Japan, Department of Developmental Disorders, National Institute of Mental Health, National Center of Neurology and Psychiatry, Tokyo, Japan

Masumi Inagaki, MD, PhD

Department of Developmental Disorders, National Institute of Mental Health, National Center of Neurology and Psychiatry, Tokyo, Japan, inagaki{at}ncnp.go.jp

Atsuko Gunji, PhD

Department of Developmental Disorders, National Institute of Mental Health, National Center of Neurology and Psychiatry, Tokyo, Japan

Yuki Inoue, MD

Department of Developmental Disorders, National Institute of Mental Health, National Center of Neurology and Psychiatry, Tokyo, Japan

Makiko Kaga, MD, PhD

Department of Developmental Disorders, National Institute of Mental Health, National Center of Neurology and Psychiatry, Tokyo, Japan

Shuki Mizutani, MD, PhD

Department of Pediatrics and Developmental Biology, Graduate School of Medicine, Tokyo Medical and Dental University, Tokyo, Japan

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.

Key Words: adrenoleukodystrophy • visual evoked potentials • event-related potentials

This version was published on August 1, 2009

Journal of Child Neurology, Vol. 24, No. 8, 927-935 (2009)
DOI: 10.1177/0883073808331354


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