Monthly Archives: August 2024

Case #67: Evidence of ELM as a Biomarker for Stargardt Disease – Page 20 of 20

Authors Sarah Gleason, OD, FAAO, DIPL ABO Sarah Gleason is an Associate Clinical Professor at SUNY College of Optometry. She graduated from SUNY in 2018 and subsequently completed a residency in Family Practice/Ocular Disease at NYC Health and Hospitals/Gotham Health East New York before returning to the college as a fulltime faculty member. Currently,

Case #67: Evidence of ELM as a Biomarker for Stargardt Disease – Page 20 of 202024-08-20T14:03:27+00:00

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Authors Caroline Donato, OD Caroline Donato received her doctorate of optometry from State University of New York (SUNY) College of Optometry in 2023. Following her completion of her degree, she completed her residency in Ocular Disease at SUNY College of Optometry in 2024. She has a passion for ocular disease, particularly that of the retina and ocular surface disease. 

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Case #67: Evidence of ELM as a Biomarker for Stargardt Disease – Page 18 of 20

Authors Shelby Leach, OD, MPH, FAAOShelby Leach obtained her Doctor of Optometry degree from the University of California, Berkeley School of Optometry in 2017 as a member of the Beta Sigma Kappa honor society. Upon completion of a Pediatrics/Primary Care Residency also at UC Berkeley, she joined SUNY College of

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References Allikmets R,, Shroyer NF,, Singh N,, et al. Mutation of the Stargardt disease gene (ABCR) in age-related macular degeneration. Science. 1997; 277: 1805–1807.Y Rotenstreich, GA Fishman, Ri Anderson 2003 Visual Acuity Loss and Clinical Observations in a Large Series of Patients with Stargardt Disease. American Academy of Ophthalmology. 110(6): 1151-1158N. Lois,

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Case #67: Evidence of ELM as a Biomarker for Stargardt Disease – Page 15 of 20

Comments & Conclusions It is important to note that treatment for the two younger siblings at ages 4 and 5 was being considered but was ultimately rejected by the parents. The treatment offered was Gildeuretinal (ALK-001, Alkeus), which works by reducing the rate of vitamin A dimerization.9 It will remain

Case #67: Evidence of ELM as a Biomarker for Stargardt Disease – Page 15 of 202024-08-20T14:02:15+00:00

Case #67: Evidence of ELM as a Biomarker for Stargardt Disease – Page 13 of 20

Image 11: Macula OCT of Patient #3 at 4-years-old  Preserved inner and outer retinal layers with thickened ELM present in both eyes. Image 12: Macula OCT of Patient #3 at 15-years-old  Complete outer retinal atrophy across retina, with outer retinal hyperreflective flecks above the level of the RPE.

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Case #67: Evidence of ELM as a Biomarker for Stargardt Disease – Page 12 of 20

Image 10: Fundus photo/FAF of Patient #3 at 15-years-old, BCVA 20/400 OD, 20/300 OS Color photos: Mild macular pigmentary mottling of the maculae. Yellow fleck-like lesions present throughout both posterior poles. Fundus autofluorescence: Hypo autofluorescence of the maculae with a wide surrounding hyper autofluorescent ring. Diffuse hyper and hypo autofluorescent flecks

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Image 9: Fundus photo/FAF of Patient #3 at 4-years-old, BCVA 20/25+ OD and OS and completely asymptomatic Normal-appearing fundi with robust ILM sheen. Subtly hypo autofluorescent foveae with surrounding early hyper autofluorescent ring. Symmetry between the eyes suggests an inherited retinal degeneration.

Case #67: Evidence of ELM as a Biomarker for Stargardt Disease – Page 11 of 202024-08-20T14:01:18+00:00