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Assessing Residual Cone Function in Retinitis Pigmentosa Patients
Tasneem A Arsiwalla1,2, Elisa E Cornish1,3,2, Phuc Vuong Nguyen1
1Save Sight Institute, The Discipline of Clinical Ophthalmology and Eye Health, Faculty of Medicine and Health, The University of Sydney, Sydney, New South Wales, Australia.
Translational Vision Science & Technology
|December 28, 2020
Summary
Full-field electroretinogram (ffERG) and optical coherence tomography (OCT) measures objectively track cone function decline in retinitis pigmentosa (RP). These findings correlate with visual acuity, aiding disease progression monitoring.
Area of Science:
- Ophthalmology
- Neuroscience
- Genetics
Background:
- Retinitis pigmentosa (RP) is a group of inherited retinal diseases causing progressive vision loss.
- Cone photoreceptors are crucial for detailed, color vision, and are affected in RP, leading to central vision impairment.
Purpose of the Study:
- To investigate cone function deterioration in retinitis pigmentosa (RP) patients.
- To assess the correlation between cone function measures and visual acuity (VA).
- To evaluate the utility of electroretinography and OCT in monitoring RP progression.
Main Methods:
- Retrospective review of clinical records (2008-2018) of RP patients undergoing repeat electrophysiology.
- Collected data included full-field electroretinogram (ffERG) (30 Hz flicker and fused flicker amplitude [FFAmp]), pattern electroretinogram (pERG) (p50 and n95), and macular optical coherence tomography (OCT) (ellipsoid zone [EZ] and outer segment thickness).
Main Results:
- Global cone function declined by 18.7% annually, with significant correlation between FFAmp and VA (P < 0.00001).
- pERG p50 reduction correlated with VA and EZ layer changes (P < 0.05).
- Ellipsoid zone (EZ) layer showed annual shortening and thinning rates of 4.3% and 4.4%, respectively, correlating with VA (P < 0.05).
Conclusions:
- ffERG and OCT provide objective measures of residual cone function in RP patients.
- These objective parameters correlate with visual acuity, assisting in monitoring disease progression.
- Quantifying cone function decline aids in patient counseling regarding prognosis.

