June 2020
Volume 61, Issue 7
Free
ARVO Annual Meeting Abstract  |   June 2020
Patterns of photoreceptor-specific temporal contrast sensitivity losses in patients with different phenotypic variants of retinitis pigmentosa
Author Affiliations & Notes
  • Cord R H Huchzermeyer
    Department of Ophthalmology, Friedrich-Alexander-University Erlangen-Nuremberg, Erlangen, Germany
    Department of Ophthalmology, University Hospital Erlangen, Germany
  • Julien Fars
    Department of Ophthalmology, Friedrich-Alexander-University Erlangen-Nuremberg, Erlangen, Germany
    Department of Ophthalmology, University Hospital Erlangen, Germany
  • Birgit Lorenz
    Ophthalmology, Justus-Liebig-University Giessen, Giessen, Germany
    Department of Ophthalmology, Universitaetsklinikum Giessen and Marburg GmbH, Giessen Campus, Giessen, Germany
  • Jan J Kremers
    Department of Ophthalmology, Friedrich-Alexander-University Erlangen-Nuremberg, Erlangen, Germany
    Department of Ophthalmology, University Hospital Erlangen, Germany
  • Footnotes
    Commercial Relationships   Cord Huchzermeyer, None; Julien Fars, None; Birgit Lorenz, None; Jan Kremers, None
  • Footnotes
    Support  DFG SPP2127 Grant HU 2340/1-1 | KR 1317/16-1 (German Research Foundation)
Investigative Ophthalmology & Visual Science June 2020, Vol.61, 2336. doi:
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    • Get Citation

      Cord R H Huchzermeyer, Julien Fars, Birgit Lorenz, Jan J Kremers; Patterns of photoreceptor-specific temporal contrast sensitivity losses in patients with different phenotypic variants of retinitis pigmentosa. Invest. Ophthalmol. Vis. Sci. 2020;61(7):2336.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Purpose : We previously measured photoreceptor-specific perifoveal temporal contrast sensitivities in patients with inherited retinal diseases (IRD) using the silent-substitution paradigm. However, the interpretation of the results in terms of pathophysiological mechanisms is not straightforward. We tested whether clinical phenotype and photoreceptor-specific sensitivity loss are correlated.

Methods : Fourteen patients with retinitis pigmentosa participated in this study. They were categorized into five different groups according to funduscopy, OCT, and fundus autofluoresence (hyperreflective ring on FAF and normal ellipsoid zone (EZ) in the center: n=5, abnormal central EZ: n=3, cystoid macular edema: n=3, female carriers of RPGR mutations with typical FAF: n=2, choroideremia: n=1).
Flicker detection thresholds of L-, M-, S-cone- and rod isolating stimuli (294 phot Td mean retinal illuminance) were determined with an LED stimulator. We calculated sensitivity losses in dB compared with age-corrected normal values and averaged sensitivity losses at 1, 2, and 4Hz . Permutation analysis based on Euclidean distances in 3D space (L-cone-, S-cone-, and rod-sensitivities) was performed to test whether phenotypes were clustered.

Results : L- and M-cone sensitivity losses were strongly correlated, so that the M-cone loss data were not included in the analysis. Our categorization according to phenotype resulted in significantly smaller distances within groups compared with random classification (p = 0.0325, 10000 permutations).

Conclusions : Our results show that photoreceptor-specific sensitivity losses mediated by the color and the rod system correlate with the clinical phenotype. Compared with existing measures of photoreceptor-specific retinal function, our technique has the advantage that temporal frequency and retinal illuminance can be varied and that state of retinal adaptation is identical for all photoreceptor types. This offers interesting opportunities to investigate how photoreceptor damage affects processing in the (normal) inner retina and which implication this has for functional tests.

This is a 2020 ARVO Annual Meeting abstract.

 

The L-cone- vs. rod-driven sensitivity losses of the 14 patients (small symbols) and the centers of gravity for each cluster (large symbols). The histogram shows the averaged Euclidian distances for 10000 random permutations and the vertical line shows the real value.

The L-cone- vs. rod-driven sensitivity losses of the 14 patients (small symbols) and the centers of gravity for each cluster (large symbols). The histogram shows the averaged Euclidian distances for 10000 random permutations and the vertical line shows the real value.

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