September 2016
Volume 57, Issue 12
Open Access
ARVO Annual Meeting Abstract  |   September 2016
Two-color pupillometry in enhanced S-cone syndrome
Author Affiliations & Notes
  • Frederick T Collison
    The Pangere Center for Inherited Retinal Diseases, The Chicago Lighthouse, Chicago, Illinois, United States
  • Jason C Park
    Department of Ophthalmology and Visual Sciences, University of Illinois at Chicago, Chicago, Illinois, United States
  • Gerald A. Fishman
    The Pangere Center for Inherited Retinal Diseases, The Chicago Lighthouse, Chicago, Illinois, United States
    Department of Ophthalmology and Visual Sciences, University of Illinois at Chicago, Chicago, Illinois, United States
  • J Jason McAnany
    Department of Ophthalmology and Visual Sciences, University of Illinois at Chicago, Chicago, Illinois, United States
  • Footnotes
    Commercial Relationships   Frederick Collison, None; Jason Park, None; Gerald Fishman, None; J Jason McAnany, None
  • Footnotes
    Support  The Pangere Family Foundation, Gary, Indiana (GAF); National Institutes of Health Research grants EY019510 (JM) and EY001792 (UIC core grant) and an unrestricted departmental grant from Research to Prevent Blindness.
Investigative Ophthalmology & Visual Science September 2016, Vol.57, 619. doi:
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    • Get Citation

      Frederick T Collison, Jason C Park, Gerald A. Fishman, J Jason McAnany; Two-color pupillometry in enhanced S-cone syndrome. Invest. Ophthalmol. Vis. Sci. 2016;57(12):619.

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

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Abstract

Purpose : The purpose of this study was to evaluate pupillary light reflexes (PLRs) mediated by rod, cone, and intrinsically photosensitive retinal ganglion cell pathways as indices of outer- and inner-retinal function in enhanced S-cone syndrome (ESCS) patients.

Methods : Four patients with ESCS (ages 16-23 years) participated in the study. Subjects were tested with long- and short-wavelength single-flash ERG stimuli under light adapted conditions. They were also tested with an established pupillometry protocol involving 1-second duration, full-field, long- and short-wavelength stimuli. The PLR was measured as a function of stimulus luminance (-4 log cd/m2 to 2.6 log cd/m2 under dark-adapted conditions and -1 log cd/m2 to 2.6 log cd/m2 under light-adapted conditions). Transient relative pupillary responses were measured under all conditions, and a sustained pupillary response was measured under the highest luminance dark-adapted condition.

Results : Two-color light-adapted full-field ERGs demonstrated larger amplitude responses for short-wavelength stimuli relative to long-wavelength stimuli, with 3 of 4 ESCS patients having super-normal a-wave amplitudes to the short-wavelength stimulus. B/A wave ratios were reduced in all four cases. Transient PLRs elicited by low luminance stimuli under dark-adapted conditions (rod-mediated) were unrecordable, whereas the sustained PLRs elicited by high luminance stimuli (melanopsin-mediated) were normal. Cone-mediated PLRs were recordable for all four patients, but generally reduced in amplitude. However, the cone-mediated PLR was larger for the short-wavelength stimulus compared to the photopically matched long-wavelength stimulus at high luminances, a pattern that was not observed for control subjects. None of the PLR conditions demonstrated “super-normal” findings.

Conclusions : ESCS patients appear to have generally well-preserved cone- and melanopsin-mediated PLRs, indicating intact inner-retinal function. Two-color pupillometry demonstrates greater sensitivity to short-wavelength light under higher-luminance conditions and could complement the ERG as a tool for evaluating retinal function in ESCS.

This is an abstract that was submitted for the 2016 ARVO Annual Meeting, held in Seattle, Wash., May 1-5, 2016.

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