Faster Sensitivity Loss around Dense Scotomas than for Overall Macular Sensitivity in Stargardt Disease: ProgStar Report No. 14 - 13/08/20

Doi : 10.1016/j.ajo.2020.03.020

Etienne M. Schönbach ^a,^b, Rupert W. Strauss ^a,^c,^d,^e, Mohamed A. Ibrahim ^a,^f, Jessica L. Janes ^g, David G. Birch ^h, Artur V. Cideciyan ⁱ, Janet S. Sunness ^j, Beatriz Muñoz ^a, Michael S. Ip ^k, SriniVas R. Sadda ^k, Hendrik P.N. Scholl ^a,^l,^m,^⁎
on behalf of the
ProgStar Study Group
Hendrik P.N. Scholl, Rupert W. Strauss, Yulia Wolfson, Millena Bittencourt, Syed Mahmood Shah, Mohamed Ahmed, Etienne Schönbach, Kaoru Fujinami, Elias Traboulsi, Justis Ehlers, Meghan Marino, Susan Crowe, Rachael Briggs, Angela Borer, Anne Pinter, Tami Fecko, Nikki Burgnoni, Janet S. Sunness, Carol Applegate, Leslie Russell, Michel Michaelides, Simona Degli Esposti, Anthony Moore, Andrew Webster, Sophie Connor, Jade Barnfield, Zaid Salchi, Clara Alfageme, Victoria McCudden, Maria Pefkianaki, Jonathan Aboshiha, Gerald Liew, Graham Holder, Anthony Robson, Alexa King, Daniela Ivanova Cajas Narvaez, Katy Barnard, Catherine Grigg, Hannah Dunbar, Yetunde Obadeyi, Karine Girard-Claudon, Hilary Swann, Avani Rughani, Charles Amoah, Dominic Carrington, Kanom Bibi, Emerson Ting, Mohamed Nafaz Illiyas, Hamida Begum, Andrew Carter, Anne Georgiou, Selma Lewism, Saddaf Shaheen, Harpreet Shinmar, Linda Burton, Paul Bernstein, Kimberley Wegner, Briana Lauren Sawyer, Bonnie Carlstrom, Kellian Farnsworth, Cyrie Fry, Melissa Chandler, Glen Jenkins, Donnel Creel, David Birch, Yi-Zhong Wang, Luis Rodriguez, Kirsten Locke, Martin Klein, Paulina Mejia, Artur V. Cideciyan, Samuel G. Jacobson, Sharon B. Schwartz, Rodrigo Matsui, Michaela Gruzensky, Jason Charng, Alejandro J. Roman, Eberhart Zrenner, Fadi Nasser, Gesa Astrid Hahn, Barbara Wilhelm, Tobias Peters, Benjamin Beier, Tilman Koenig, Susanne Kramer, José-Alain Sahel, Saddek Mohand-Said, Isabelle Audo, Caroline Laurent-Coriat, Ieva Sliesoraityte, Christina Zeitz, Fiona Boyard, Minh Ha Tran, Mathias Chapon, Céline Chaumette, Juliette Amaudruz, Victoria Ganem, Serge Sancho, Aurore Girmens, Hendrik P.N. Scholl, Rupert W. Strauss, Yulia Wolfson, Syed Mahmood Shah, Mohamed Ahmed, Etienne Schönbach, Robert Wojciechowski, Shazia Khan, David G. Emmert, Dennis Cain, Mark Herring, Jennifer Bassinger, Lisa Liberto, Sheila West, Ann-Margret Ervin, Beatriz Munoz, Xiangrong Kong, Kurt Dreger, Jennifer Jones, Srinivas Sadda, Michael S. Ip, Anamika Jha, Alex Ho, Brendan Kramer, Ngoc Lam, Rita Tawdros, Yong Dong Zhou, Johana Carmona, Akihito Uji, Amirhossein Hariri, Amy Lock, Anthony Elshafei, Anushika Ganegoda, Christine Petrossian, Dennis Jenkins, Edward Strnad, Elmira Baghdasaryan, Eric Ito, Feliz Samson, Gloria Blanquel, Handan Akil, Jhanisus Melendez, Jianqin Lei, Jianyan Huang, Jonathan Chau, Khalil G. Falavarjani, Kristina Espino, Manfred Li, Maria Mendoza, Muneeswar Gupta Nittala, Netali Roded, Nizar Saleh, Ping Huang, Sean Pitetta, Siva Balasubramanian, Sophie Leahy, Sowmya J. Srinivas, Swetha B. Velaga, Teresa Margaryan, Tudor Tepelus, Tyler Brown, Wenying Fan, Yamileth Murillo, Yue Shi, Katherine Aguilar, Cynthia Chan, Lisa Santos, Brian Seo, Christopher Sison, Silvia Perez, Stephanie Chao, Kelly Miyasato, Julia Higgins, Zoila Luna, Anita Menchaca, Norma Gonzalez, Vicky Robledo, Karen Carig, Kirstie Baker, David Ellenbogen, Daniel Bluemel, Theo Sanford, Daisy Linares, Mei Tran, Lorane Nava, Michelle Oberoi, Mark Romero, Vivian Chiguil, Grantley Bynum-Bain, Monica Kim, Carolina Mendiguren, Xiwen Huang, Monika Smith, Teresa Margaryan, Natalie Sarreal

^a Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
^b Department of Ophthalmology, Case Western Reserve University, Cleveland, Ohio, USA
^c Moorfields Eye Hospital and University College London Institute of Ophthalmology, London, United Kingdom
^d Department of Ophthalmology, Kepler University Clinic, Linz, Linz, Austria
^e Department of Ophthalmology, Medical University of Graz, Graz, Austria
^f Byers Eye Institute, Stanford University, Palo Alto, California, USA
^g University Hospitals Clinical Research Center, Cleveland Medical Center, Cleveland, Ohio, USA
^h Retina Foundation of the Southwest, Dallas, TX, USA
ⁱ Scheie Eye Institute, University of Pennsylvania, Philadelphia, Pennsylvania, USA
^j Greater Baltimore Medical Center, Baltimore, Maryland, USA
^k Doheny Eye Institute, University of California Los Angeles, David Geffen School of Medicine, Los Angeles, California, USA
^l Institute of Molecular and Clinical Ophthalmology Basel, Basel, Switzerland
^m Department of Ophthalmology, University of Basel, Basel, Switzerland

^∗Inquiries to: Hendrik P.N. Scholl, Institute of Molecular and Clinical Ophthalmology Basel, Mittlere Strasse 91, CH-4031 Basel, SwitzerlandInstitute of Molecular and Clinical Ophthalmology BaselMittlere Strasse 91BaselCH-4031Switzerland

Abstract

Purpose

Mean sensitivity (MS) derived from a standard test grid using microperimetry is a sensitive outcome measure in clinical trials investigating new treatments for degenerative retinal diseases. This study hypothesizes that the functional decline is faster at the edge of the dense scotoma (eMS) than by using the overall MS.

Design

Multicenter, international, prospective cohort study: ProgStar Study.

Methods

Stargardt disease type 1 patients (carrying at least 1 mutation in the ABCA4 gene) were followed over 12 months using microperimetry with a Humphrey 10-2 test grid. Customized software was developed to automatically define and selectively follow the test points directly adjacent to the dense scotoma points and to calculate their mean sensitivity (eMS).

Results

Among 361 eyes (185 patients), the mean age was 32.9 ± 15.1 years old. At baseline, MS was 10.4 ± 5.2 dB (n = 361), and the eMS was 9.3 ± 3.3 dB (n = 335). The yearly progression rate of MS (1.5 ± 2.1 dB/year) was significantly lower (β = −1.33; P < .001) than that for eMS (2.9 ± 2.9 dB/year). There were no differences between progression rates using automated grading and those using manual grading (β = .09; P = .461).

Conclusions

In Stargardt disease type 1, macular sensitivity declines significantly faster at the edge of the dense scotoma than in the overall test grid. An automated, time-efficient approach for extracting and grading eMS is possible and appears valid. Thus, eMS offers a valuable tool and sensitive outcome parameter with which to follow Stargardt patients in clinical trials, allowing clinical trial designs with shorter duration and/or smaller cohorts.

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Highlights:

•	This study reports a novel automated approach to quantify progression of visual dysfunction.
•	This automated approach was validated relative to a manual grading.
•	The progression rate at the disease front in Stargardt disease is reported.
•	The new method may allow shorter clinical trials or smaller cohorts or both.

Le texte complet de cet article est disponible en PDF.

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Vol 216

P. 219-225 - août 2020 Retour au numéro

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Faster Sensitivity Loss around Dense Scotomas than for Overall Macular Sensitivity in Stargardt Disease: ProgStar Report No. 14 - 13/08/20

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Results

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