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Static Perimetry in the Rate of Progression in USH2A-related Retinal Degeneration (RUSH2A) Study: Assessment through Two Years

Duncan, Jacque L; Cheng, Peiyao; Maguire, Maureen G; Ayala, Allison A; Birch, David G; Cheetham, Janet K; Durham, Todd A; ... Foundation Fighting Blindness Consortium Investigator Group; + view all (2023) Static Perimetry in the Rate of Progression in USH2A-related Retinal Degeneration (RUSH2A) Study: Assessment through Two Years. American Journal of Ophthalmology , 250 pp. 103-110. 10.1016/j.ajo.2023.02.002. Green open access

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Abstract

PURPOSE: To evaluate disease progression using static perimetry (SP) in patients with USH2A-related retinal degeneration, including Usher syndrome type 2 (USH2) and nonsyndromic autosomal recessive retinitis pigmentosa (ARRP). DESIGN: Prospective, observational cohort study. METHODS: Setting:16 clinical sites in Europe and North America. STUDY POPULATION: Study participants with biallelic disease-causing sequence variants in USH2A with baseline best-corrected visual acuity (BCVA) letter score ≥54 (N=102). OBSERVATION PROCEDURES: SP, BCVA, full-field stimulus thresholds (FST), spectral domain optical coherence tomography macular scans, and fundus-guided mesopic microperimetry (MP) also were performed at baseline and annually. MAIN OUTCOME MEASURES: Total hill of vision (VTOT), hill of vision in the central 30° (V30), VTOT-V30 (VPERIPH), and mean sensitivity. RESULTS: The average decline (95% CI) was 2.05 (1.40, 2.70) decibel-steradian (dB-sr)/year for VTOT, 0.48 (0.32, 0.65) dB-sr/year for V30, 1.53 (0.97, 2.08) dB-sr/year for VPERIPH and 0.55 (0.40, 0.71) dB/year for mean sensitivity. Average percentage decline was 8.3 (5.5, 11.1) %/year for VTOT, 5.2 (3.0, 7.4) %/year for V30, 16.0 (9.5, 22.0) %/year for VPERIPH, and 5.1 (3.5, 6.7) %/year for mean sensitivity. Changes from baseline to Year 2 in all SP measures were highly correlated [rs ranging from 0.52 (V30 vs VPERIPH) to 0.98 (VTOT vs Vperiph)]. CONCLUSIONS: Quantitative measures of SP declined significantly over 2 years in USH2A-related retinal degeneration. The annual percent rate of change was greatest for VTOT and VPERIPH, while V30 and mean sensitivity changed least, reflecting earlier and more severe peripheral degeneration compared to central loss.

Type: Article
Title: Static Perimetry in the Rate of Progression in USH2A-related Retinal Degeneration (RUSH2A) Study: Assessment through Two Years
Location: United States
Open access status: An open access version is available from UCL Discovery
DOI: 10.1016/j.ajo.2023.02.002
Publisher version: https://doi.org/10.1016/j.ajo.2023.02.002
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
Keywords: Foundation Fighting Blindness Consortium Investigator Group
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > Institute of Ophthalmology
URI: https://discovery.ucl.ac.uk/id/eprint/10166720
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