Macular Sensitivity Measured With Microperimetry in Stargardt Disease in the Progression of Atrophy Secondary to Stargardt Disease (ProgStar) Study: Report No. 7

Overview

Journal JAMA Ophthalmol

Publisher American Medical Association

Specialty Ophthalmology

Date 2017 May 26

PMID 28542693

Citations 32

Authors

Etienne M Schonbach

Yulia Wolfson

Rupert W Strauss

Mohamed A Ibrahim

Xiangrong Kong

Beatriz Munoz

David G Birch

Artur V Cideciyan

Gesa-Astrid Hahn

Muneeswar Nittala

Janet S Sunness

Srinivas R Sadda

Sheila K West

Hendrik P N Scholl

Affiliations

Soon will be listed here.

Abstract

Importance: New outcome measures for treatment trials for Stargardt disease type 1 (STGD1) and other macular diseases are needed. Microperimetry allows mapping of light sensitivity of the macula and provides topographic information on visual function beyond visual acuity.

Objective: To measure and analyze retinal light sensitivity of the macula in STGD1 using fundus-controlled perimetry (microperimetry).

Design, Setting, And Participants: This was a multicenter prospective cohort study. A total of 199 patients and 326 eyes with molecularly confirmed (ABCA4) STGD1 underwent testing with the Nidek MP-1 microperimeter as part of the multicenter, prospective Natural History of the Progression of Atrophy Secondary to Stargardt Disease (ProgStar) study. Sensitivity of 68 retinal loci was tested, and the mean sensitivity (MS) was determined; each point was categorized as "normal," "relative," or "deep" scotoma.

Main Outcomes And Measures: Mean sensitivity and the number of points with normal sensitivity, relative, or deep scotomas.

Results: Mean (SD) patient age was 34.2 (14.7) years, mean (SD) best-corrected visual acuity of all eyes was 47.8 (16.9) Early Treatment Diabetic Retinopathy Study letter score (approximately 20/100 Snellen equivalent), and mean MS of all eyes of all 68 points was 11.0 (5.0) dB. The median number of normal points per eye was 49 (mean [SD], 41.3 [20.8]; range, 0-68); abnormal sensitivity and deep scotomas were more prevalent in the central macula. Mean sensitivity was lower in the fovea (mean [SD], 2.7 [4.4] dB) than in the inner (mean [SD], 6.8 [5.8] dB) and outer ring (mean [SD], 12.7 [5.3] dB). Overall MS per eye was 0.086 dB lower per year of additional age (95% CI, -0.13 to -0.041; P < .001) and 0.21 dB lower per additional year of duration of STGD1 (95% CI, -0.28 to -0.14; P < .001). Longer duration of STGD1 was associated with worse MS (β = -0.18; P < .001), with a lower number of normal test points (β = -0.71; P < .001), and with a higher number of deep scotoma points (β = -0.70; P < .001). We found 11 eyes with low MS (<6 dB) but very good best-corrected visual acuity of at least 72 Early Treatment Diabetic Retinopathy Study letter score (20/40 Snellen equivalent).

Conclusions And Relevance: We provide an extensive analysis of macular sensitivity parameters in STGD1 and demonstrate their association with demographic characteristics and vision. These data suggest microperimetry testing provides a more comprehensive assessment of retinal function and will be an important outcome measure in future clinical trials.

Citing Articles

Parafoveal Microperimetric Retinal Sensitivity as a Key Parameter Associated with Vision Loss in Retinitis Pigmentosa.

Hsiao Y, Huang H, Chen T, Lo J, Chen Y, Kuo H Diagnostics (Basel). 2024; 14(23).

PMID: 39682599 PMC: 11639803. DOI: 10.3390/diagnostics14232691.

Censoring the Floor Effect in Long-Term Stargardt Disease Microperimetry Data Produces a Faster Rate of Decline.

Charng J, Thompson J, Heath Jeffery R, Kalantary A, Lamey T, McLaren T Ophthalmol Sci. 2024; 4(6):100581.

PMID: 39280350 PMC: 11401193. DOI: 10.1016/j.xops.2024.100581.

Using Ensemble OCT-Derived Features beyond Intensity Features for Enhanced Stargardt Atrophy Prediction with Deep Learning.

Mishra Z, Wang Z, Sadda S, Hu Z Appl Sci (Basel). 2024; 13(14).

PMID: 39086558 PMC: 11288976. DOI: 10.3390/app13148555.

Recurrent and Concurrent Prediction of Longitudinal Progression of Stargardt Atrophy and Geographic Atrophy.

Mishra Z, Wang Z, Xu E, Xu S, Majid I, Sadda S medRxiv. 2024; .

PMID: 38405807 PMC: 10888984. DOI: 10.1101/2024.02.11.24302670.

Inter and intradevice assessment of microperimetry testing in aging eyes.

Coulibaly L, Mohamed H, Fuchs P, Schmidt-Erfurth U, Reiter G Sci Rep. 2024; 14(1):1049.

PMID: 38200267 PMC: 10781777. DOI: 10.1038/s41598-024-51539-0.

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