Macular sensitivity measured with microperimetry in stargardt disease in the progression of atrophy secondary to stargardt disease (ProgStar) study report No. 7

for the ProgStar Study Group, Syed Mahmood Shah

Research output: Contribution to journalArticle

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: 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.

Original languageEnglish (US)
Pages (from-to)696-703
Number of pages8
JournalJAMA Ophthalmology
Volume135
Issue number7
DOIs
StatePublished - Jul 1 2017

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Visual Field Tests
Atrophy
Disease Progression
Scotoma
Visual Acuity
Photophobia
Diabetic Retinopathy
Outcome Assessment (Health Care)
Stargardt disease 1
Natural History
Cohort Studies
Therapeutics
Demography
Clinical Trials
Prospective Studies

ASJC Scopus subject areas

  • Ophthalmology

Cite this

Macular sensitivity measured with microperimetry in stargardt disease in the progression of atrophy secondary to stargardt disease (ProgStar) study report No. 7. / for the ProgStar Study Group; Shah, Syed Mahmood.

In: JAMA Ophthalmology, Vol. 135, No. 7, 01.07.2017, p. 696-703.

Research output: Contribution to journalArticle

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title = "Macular sensitivity measured with microperimetry in stargardt disease in the progression of atrophy secondary to stargardt disease (ProgStar) study report No. 7",
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: 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.",
author = "{for the ProgStar Study Group} and Sch{\"o}nbach, {Etienne M.} and Yulia Wolfson and Strauss, {Rupert W.} and Ibrahim, {Mohamed A.} and Xiangrong Kong and Beatriz Mu{\~n}oz and Birch, {David G.} and Cideciyan, {Artur V.} and Hahn, {Gesa Astrid} and Muneeswar Nittala and Sunness, {Janet S.} and Sadda, {Srini Vas R.} and West, {Sheila K.} and Scholl, {Hendrik P.N.} and Shah, {Syed Mahmood} and Shah, {Syed Mahmood} and Ahmed, {Mohamed Ibrahim} and Kaoru Fujinami and Elias Traboulsi and Justis Ehlers and Meghan Marino and Susan Crowe and Rachael Briggs and Angela Borer and Anne Pinter and Tami Fecko and Nikki Brugnoni and Carol Applegate and Leslie Russell and Michel Michaelides and Esposti, {Simona Degli} and Anthony Moore and Andrew Webster and Sophie Connor and Jade Barnfield and Zaid Salchi and Clara Alfageme and Victoria McCudden and Maria Pefkianaki and Jonathan Aboshiha and Gerald Liew and Graham Holder and Anthony Robson and Alexa King and Narvaez, {Daniela Ivanova Cajas} and Katy Barnard and Catherine Grigg and Hannah Dunbar and Robert Wojciechowski and Ervin, {Ann Margret}",
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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 - Shah, Syed Mahmood

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

PY - 2017/7/1

Y1 - 2017/7/1

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

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JF - JAMA Ophthalmology

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