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A. A. Reis, T. Viegas, C. Mateus, E. D. Silva, M. Castelo-Branco; Visual Function in Parallel Retinocortical Pathways in Affected Individuals From 8 Families With Autosomal Dominant Optic Atrophy. Invest. Ophthalmol. Vis. Sci. 2008;49(13):3827. doi: https://doi.org/.
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© ARVO (1962-2015); The Authors (2016-present)
To characterize different phenotypes of Kjer optic atrophy along different visual pathways and to correlate them with structural features.
Novel computerized psychophysical assessment methods (CCT - Cambridge Colour Test and CSF - Metropsis Contrast Sensitivity Function Test) were used to evaluate visual function in a population of 13 subjects (26 eyes) from 8 families with Autosomal Dominant Optic Atrophy (ADOA). This evaluation was completed with electrophysiological assessment (Pattern ERG, Pattern and Multifocal VEP) and Automated Static Perimetry (ASP). Statistical analysis was performed using ANOVA at a significance level of p< 0.05.
CCT shows evidence for severe damage of all cone populations, and of similar magnitude, implying concomitant damage of parvo and koniocellular pathways. Achromatic contrast sensitivity was severely impaired for all six spatial frequency channels studied. A decrease on PERG amplitude was found and is consistent with the observed Pattern VEP/mfVEP and ASP impairments.
Our results suggest that all types of fibers are damaged in ADOA. Multimodal psychophysical and electrophysiological methods are good quantitative markers to understand the pathophysiology of damage of central and peripheral pathways in this condition.
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