Xiangrong Kong1, Mohamed Ibrahim-Ahmed2, Millena G Bittencourt3, Rupert W Strauss4, David G Birch5, Artur V Cideciyan6, Ann-Margaret Ervin7, Alexander Ho8, Janet S Sunness9, Isabelle S Audo10, Michel Michaelides11, Eberhart Zrenner12, SriniVas Sadda8, Michael S Ip8, Sheila West3, Hendrik P N Scholl13. 1. Wilmer Eye Institute at the Johns Hopkins University (X.K., M.G.B., A.-M.E., S.W., H.P.N.S.), Baltimore, Maryland, USA; Department of Biostatistics (X.K.), Bloomberg School of Public Health, Johns Hopkins University, Baltimore, Maryland, USA; Department of Epidemiology (X.K., A.-M.E.), Bloomberg School of Public Health, Johns Hopkins University, Baltimore, Maryland, USA; Department of Health Behavior and Society (X.K.), Bloomberg School of Public Health, Johns Hopkins University, Baltimore, Maryland, USA. Electronic address: xkong4@jhu.edu. 2. Ocular Imaging Research and Reading Center (M.I.-A.), Sunnyvale, California, USA. 3. Wilmer Eye Institute at the Johns Hopkins University (X.K., M.G.B., A.-M.E., S.W., H.P.N.S.), Baltimore, Maryland, USA. 4. Moorfields Eye Hospital and UCL Institute of Ophthalmology (R.W.S., M.M.), London, UK; Department of Ophthalmology (R.W.S.), Kepler University Clinic, Linz, Linz, Austria; Department of Ophthalmology (R.W.S.), Medical University of Graz, Graz, Austria. 5. Retina Foundation of the Southwest (D.G.B.), Dallas, Texas, USA. 6. Scheie Eye Institute (A.V.C.), University of Pennsylvania, Philadelphia, Pennsylvania, USA. 7. Wilmer Eye Institute at the Johns Hopkins University (X.K., M.G.B., A.-M.E., S.W., H.P.N.S.), Baltimore, Maryland, USA; Department of Epidemiology (X.K., A.-M.E.), Bloomberg School of Public Health, Johns Hopkins University, Baltimore, Maryland, USA. 8. Doheny Image Reading Center (A.H., S.V.S., M.S.I.), Los Angeles, California, USA. 9. Greater Baltimore Medical Center (J.S.S.), Baltimore, Maryland, USA. 10. CHNO des Quinze-Vingts (I.S.A.), DHU Sight Restore, INSERM-DHOS CIC 1423, Sorbonne Universités, UPMC Univ Paris 06, INSERM, CNRS, Institut de la Vision, Paris, France. 11. Moorfields Eye Hospital and UCL Institute of Ophthalmology (R.W.S., M.M.), London, UK. 12. Universitäts-Augenklinik (E.Z.), University of Tübingen, Tübingen, Germany. 13. Wilmer Eye Institute at the Johns Hopkins University (X.K., M.G.B., A.-M.E., S.W., H.P.N.S.), Baltimore, Maryland, USA; Institute of Molecular and Clinical Ophthalmology Basel (IOB) (H.P.N.S.), Basel, Switzerland; Department of Ophthalmology (H.P.N.S.), University of Basel, Basel, Switzerland.
Abstract
PURPOSE: To estimate and compare cross-sectional scotopic versus mesopic macular sensitivity losses measured by microperimetry, and to report and compare the longitudinal rates of scotopic and mesopic macular sensitivity losses in ABCA4 gene-associated Stargardt disease (STGD1). DESIGN: This was a multicenter prospective cohort study. METHODS: Participants comprised 127 molecularly confirmed STGD1 patients enrolled from 6 centers in the United States and Europe and followed up every 6 months for up to 2 years. The Nidek MP-1S device was used to measure macular sensitivities of the central 20° under mesopic and scotopic conditions. The mean deviations (MD) from normal for mesopic macular sensitivity for the fovea (within 2° eccentricity) and extrafovea (4°-10° eccentricity), and the MD for scotopic sensitivity for the extrafovea, were calculated. Linear mixed effects models were used to estimate mesopic and scotopic changes. Main outcome measures were baseline mesopic mean deviation (mMD) and scotopic MD (sMD) and rates of longitudinal changes in the mMDs and sMD. RESULTS: At baseline, all eyes had larger sMD, and the difference between extrafoveal sMD and mMD was 10.7 dB (P < .001). Longitudinally, all eyes showed a statistically significant worsening trend: the rates of foveal mMD and extrafoveal mMD and sMD changes were 0.72 (95% CI = 0.37-1.07), 0.86 (95% CI = 0.58-1.14), and 1.12 (95% CI = 0.66-1.57) dB per year, respectively. CONCLUSIONS: In STGD1, in extrafovea, loss of scotopic macular function preceded and was faster than the loss of mesopic macular function. Scotopic and mesopic macular sensitivities using microperimetry provide alternative visual function outcomes for STGD1 treatment trials.
PURPOSE: To estimate and compare cross-sectional scotopic versus mesopic macular sensitivity losses measured by microperimetry, and to report and compare the longitudinal rates of scotopic and mesopic macular sensitivity losses in ABCA4 gene-associated Stargardt disease (STGD1). DESIGN: This was a multicenter prospective cohort study. METHODS: Participants comprised 127 molecularly confirmed STGD1 patients enrolled from 6 centers in the United States and Europe and followed up every 6 months for up to 2 years. The Nidek MP-1S device was used to measure macular sensitivities of the central 20° under mesopic and scotopic conditions. The mean deviations (MD) from normal for mesopic macular sensitivity for the fovea (within 2° eccentricity) and extrafovea (4°-10° eccentricity), and the MD for scotopic sensitivity for the extrafovea, were calculated. Linear mixed effects models were used to estimate mesopic and scotopic changes. Main outcome measures were baseline mesopic mean deviation (mMD) and scotopic MD (sMD) and rates of longitudinal changes in the mMDs and sMD. RESULTS: At baseline, all eyes had larger sMD, and the difference between extrafoveal sMD and mMD was 10.7 dB (P < .001). Longitudinally, all eyes showed a statistically significant worsening trend: the rates of foveal mMD and extrafoveal mMD and sMD changes were 0.72 (95% CI = 0.37-1.07), 0.86 (95% CI = 0.58-1.14), and 1.12 (95% CI = 0.66-1.57) dB per year, respectively. CONCLUSIONS: In STGD1, in extrafovea, loss of scotopic macular function preceded and was faster than the loss of mesopic macular function. Scotopic and mesopic macular sensitivities using microperimetry provide alternative visual function outcomes for STGD1 treatment trials.
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