December 2002
Volume 43, Issue 13
Free
ARVO Annual Meeting Abstract  |   December 2002
Impairment of the Pupillary Light Reflex in Rpe65-/- Mice and Patients with Leber Congenital Amaurosis
Author Affiliations & Notes
  • TS Aleman
    Dept of Ophthalmology Scheie Eye Institute Philadelphia PA
  • ML Scott
    Dept of Ophthalmology Scheie Eye Institute Philadelphia PA
  • RJ Chen
    Dept of Ophthalmology Scheie Eye Institute Philadelphia PA
  • JD Chico
    Dept of Ophthalmology Scheie Eye Institute Philadelphia PA
  • AY Cheung
    Dept of Ophthalmology Scheie Eye Institute Philadelphia PA
  • JP Van Hooser
    Dept of Ophthalmology University of Washington Seattle WA
  • TM Redmond
    Laboratory of Retinal Cell and Molecular Biology NEI NIH Bethesda MD
  • K Palczewski
    Dept of Ophthalmology University of Washington Seattle WA
  • SG Jacobson
    Dept of Ophthalmology Scheie Eye Institute Philadelphia PA
  • AV Cideciyan
    Dept of Ophthalmology Scheie Eye Institute Philadelphia PA
  • Footnotes
    Commercial Relationships   T.S. Aleman, None; M.L. Scott, None; R.J. Chen, None; J.D. Chico, None; A.Y. Cheung, None; J.P. Van Hooser, None; T.M. Redmond, None; K. Palczewski, None; S.G. Jacobson, None; A.V. Cideciyan, None. Grant Identification: NIH EY 13385,EY 13203,FFB,Research to Prevent Blindness, Macula Vision Research Foundation
Investigative Ophthalmology & Visual Science December 2002, Vol.43, 3490. doi:
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    • Get Citation

      TS Aleman, ML Scott, RJ Chen, JD Chico, AY Cheung, JP Van Hooser, TM Redmond, K Palczewski, SG Jacobson, AV Cideciyan; Impairment of the Pupillary Light Reflex in Rpe65-/- Mice and Patients with Leber Congenital Amaurosis . Invest. Ophthalmol. Vis. Sci. 2002;43(13):3490.

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Abstract

Abstract: : Purpose: To characterize the pupillary light reflex (PLR) in a murine model of Leber congenital amaurosis (LCA) and patients with the disease. Methods: Direct PLR was recorded using a custom-built pupillometer in anesthetized, dark-adapted Rpe65-/- and control mice, 8-12 weeks of age. PLRs were elicited with increasing intensities (∼8 log u range) of 100 ms duration full field light stimuli. Oral 9-cis-retinal was administered to a subset of Rpe65-/- mice. PLR was recorded before and 1 week after cis-retinoid treatment. A group of patients with uncomplicated LCA underwent dark-adapted pupillometry using a comparable system Results: Baseline pupillary diameters in Rpe65-/- and control mice were similar. PLR thresholds of Rpe65-/- mice were elevated by 3-4 log u compared to those of controls. The waveforms of the PLR in Rpe65-/- mice could be simulated with ∼4 log dimmer stimuli presented to control mice. Supplementation with 9-cis-retinal lowered the PLR threshold by ∼2 log. LCA patients had baseline pupillary diameters similar to normal, but the PLR was abnormal with thresholds elevated by 3-5 log u. Pupillary constriction to brighter stimuli had smaller amplitude and slower kinetics than normal. Conclusions: Pupillometry was used to quantify the visual impairment and to probe transmission of retinal signals to higher nervous centers in a murine model of LCA and in LCA patients. The objective and non-invasive nature of the PLR measurement and the observed post-treatment change toward normal in the animal model suggest that this may be a useful additional outcome measure in future therapeutic trials of LCA.

Keywords: 562 retinal degenerations: hereditary • 535 pupillary reflex • 359 clinical research methodology 
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