TY - JOUR
T1 - Macular sensitivity measured with microperimetry in stargardt disease in the progression of atrophy secondary to stargardt disease (ProgStar) study report No. 7
AU - for the ProgStar Study Group
AU - Schönbach, Etienne M.
AU - Wolfson, Yulia
AU - Strauss, Rupert W.
AU - Ibrahim, Mohamed A.
AU - Kong, Xiangrong
AU - Muñoz, Beatriz
AU - Birch, David G.
AU - Cideciyan, Artur V.
AU - Hahn, Gesa Astrid
AU - Nittala, Muneeswar
AU - Sunness, Janet S.
AU - Sadda, Srini Vas R.
AU - West, Sheila K.
AU - Scholl, Hendrik P.N.
AU - Bittencourt, Millena
AU - Shah, Syed Mahmood
AU - Ahmed, Mohamed Ibrahim
AU - Fujinami, Kaoru
AU - Traboulsi, Elias
AU - Ehlers, Justis
AU - Marino, Meghan
AU - Crowe, Susan
AU - Briggs, Rachael
AU - Borer, Angela
AU - Pinter, Anne
AU - Fecko, Tami
AU - Brugnoni, Nikki
AU - Applegate, Carol
AU - Russell, Leslie
AU - Michaelides, Michel
AU - Esposti, Simona Degli
AU - Moore, Anthony
AU - Webster, Andrew
AU - Connor, Sophie
AU - Barnfield, Jade
AU - Salchi, Zaid
AU - Alfageme, Clara
AU - McCudden, Victoria
AU - Pefkianaki, Maria
AU - Aboshiha, Jonathan
AU - Liew, Gerald
AU - Holder, Graham
AU - Robson, Anthony
AU - King, Alexa
AU - Narvaez, Daniela Ivanova Cajas
AU - Barnard, Katy
AU - Grigg, Catherine
AU - Dunbar, Hannah
AU - Wojciechowski, Robert
AU - Ervin, Ann Margret
N1 - Funding Information:
The ProgStar studies are supported by the Foundation Fighting Blindness Clinical Research Institute and a grant to Foundation Fighting Blindness Clinical Research Institute by the US Department of Defense USAMRMC TATRC, Fort Meade, Maryland (grant numbers W81-XWH-07-1-0720 and W81XWH-09-2- 0189); Dr Etienne Schonbach is supported by grant LPDS 2015-14 from the Leopoldina Fellowship Program, German National Academy of Sciences. Dr RupertW. Strauss is supported by grant number J 3383-B23 from the Austrian Science Fund.
Publisher Copyright:
© 2017 American Medical Association. All rights reserved.
PY - 2017/7
Y1 - 2017/7
N2 - 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: Thiswas 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 lowMS (<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.
AB - 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: Thiswas 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 lowMS (<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.
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U2 - 10.1001/jamaophthalmol.2017.1162
DO - 10.1001/jamaophthalmol.2017.1162
M3 - Article
C2 - 28542693
AN - SCOPUS:85024385568
VL - 135
SP - 696
EP - 703
JO - JAMA Ophthalmology
JF - JAMA Ophthalmology
SN - 2168-6165
IS - 7
ER -