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Karen Holopigian, Jane Green, James Whelan, Marie Burstedt, Zhenzhong Su, Jean-Yves Deslandes, Xiao Ni, Michael Wald, Cynthia L Grosskreutz, Kalliopi Stasi; Humphrey visual field and flicker electroretinogram changes over two-year follow-up in patients with RLBP1 retinitis pigmentosa enrolled in a prospective Natural History Study. Invest. Ophthalmol. Vis. Sci. 2017;58(8):3246. doi: https://doi.org/.
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Visual field defects and abnormal full-field electroretinograms (ERGs) occur early in retinitis pigmentosa due to RLBP1 mutations (RLBP1 RP) and can be used to follow disease progression. Visual fields and ERGs together with other functional and structural endpoints were monitored in a prospective Natural History Study in RLBP1 RP patients. Here, we report preliminary 2-year results on test-retest variability and progression of Humphrey visual fields and flicker ERGs (results from other endpoints are presented in additional abstracts).
This non-interventional, two-center (Sweden and Canada), prospective study included evaluations of 45 RLBP1 RP patients every 6 months. All participants had Humphrey visual fields (HVFs) using the SITA standard 30-2 program. A subset of patients (n=15) had full-field light-adapted 30 Hz flicker ERGs with Ganzfeld stimulation (after pupil dilation). Annual progression was estimated as the slope of a random effect linear growth model and test-retest variability was assessed using the intra-class correlation coefficient (ICC). Between-eye correlations were performed for each measurement and at each visit.
HVF mean deviation (MD) and ERG amplitude and implicit time preliminary results (mean values), as well as annual progression slope and test-retest variability (ICCs) for right (OD) and left (OS) eyes are displayed in the table. Statistically significant Pearson’s correlations between the two eyes at baseline and at each follow-up visit were noted for HVF MD ranging from 0.908 to 0.984 (p <0.001); for ERG amplitude ranging from 0.941 to 0.983 (p <0.001) and for ERG implicit time ranging from 0.923 to 0.964 (p <0.001).
RLBP1 RP patients showed HVF and flicker ERG deficits consistent with photoreceptor degeneration. Both eyes were similarly affected with respect to HVF and ERG measurements. There was no statistically significant progression over the 2-year follow-up.
This is an abstract that was submitted for the 2017 ARVO Annual Meeting, held in Baltimore, MD, May 7-11, 2017.
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