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Article
September 1993

Visual Evoked Potential Characteristics and Early Diagnosis of Pelizaeus-Merzbacher Disease

Author Affiliations

From the Department of Visual Systems Analysis, the Netherlands Ophthalmic Research Institute (Dr Apkarian), and the Department of Child Neurology, Academic Medical Center (Drs Koetsveld-Baart and Barth), Amsterdam, the Netherlands.

Arch Neurol. 1993;50(9):981-985. doi:10.1001/archneur.1993.00540090078014
Abstract

• Objective.  —Early diagnosis of Pelizaeus-Merzbacher disease; assessment of disease progression.

Design.  —Pediatric neuro-ophthalmology evaluation of visual function with pattern and luminance visual evoked potentials; behavioral state assessment; electrophysiological diagnostic test; baseline estimates at the age of 11 weeks; 1-year follow-up.

Setting.  —University hospital electrodiagnostic vision research laboratory.

Patients.  —Case study: X-linked Pelizaeus-Merzbacher disease; leukodystrophy; patient tested at the age of 11 weeks and at the age of 58 weeks for follow-up; five age-matched normal controls.

Interventions.  —Physical therapy, medication, evaluation.

Main Outcome.  —Early diagnosis; diagnostic confirmation; objective monitor of disease progression.

Results.  —Abnormal spatial and temporal vision and abnormal visual pathway maturation; visual evoked potentials of proband with Pelizaeus-Merzbacher disease were grossly abnormal, reflecting myelination disorder.

Conclusions.  —Visual evoked potential pediatric electrodiagnosis yields reliable measures of visual function and visual system maturation in leukodystrophy.

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