May 2008
Volume 49, Issue 13
Free
ARVO Annual Meeting Abstract  |   May 2008
Visual Function in Parallel Retinocortical Pathways in Affected Individuals From 8 Families With Autosomal Dominant Optic Atrophy
Author Affiliations & Notes
  • A. A. Reis
    IBILI, Faculty of Medicine, Coimbra, Portugal
    Ophthalmology, University Hospital, Coimbra, Portugal
  • T. Viegas
    IBILI, Faculty of Medicine, Coimbra, Portugal
  • C. Mateus
    IBILI, Faculty of Medicine, Coimbra, Portugal
  • E. D. Silva
    IBILI, Faculty of Medicine, Coimbra, Portugal
    Ophthalmology, University Hospital, Coimbra, Portugal
  • M. Castelo-Branco
    IBILI, Faculty of Medicine, Coimbra, Portugal
  • Footnotes
    Commercial Relationships  A.A. Reis, None; T. Viegas, None; C. Mateus, None; E.D. Silva, None; M. Castelo-Branco, None.
  • Footnotes
    Support  None.
Investigative Ophthalmology & Visual Science May 2008, Vol.49, 3827. doi:https://doi.org/
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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)

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Abstract

Purpose: : To characterize different phenotypes of Kjer optic atrophy along different visual pathways and to correlate them with structural features.

Methods: : 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.

Results: : 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.

Conclusions: : 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.

Keywords: color vision • contrast sensitivity • electroretinography: clinical 
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