June 2017
Volume 58, Issue 8
Open Access
ARVO Annual Meeting Abstract  |   June 2017
Natural progression of scotopic and photopic macular function loss in patients with Stargardt disease: The SMART Study.
Author Affiliations & Notes
  • Millena Bittencourt
    Ophthalmology, Johns Hopkins University, Edmond, Oklahoma, United States
  • Mohamed A Ibrahim
    Ophthalmology, Johns Hopkins University, Edmond, Oklahoma, United States
  • Xiangrong Kong
    Ophthalmology, Johns Hopkins University, Edmond, Oklahoma, United States
  • Rupert Wolfgang Strauss
    Ophthalmology, Moorfields Eye Hospital and UCL Institute of Ophthalmology, London, United Kingdom
    Ophthalmology, Johannes Kepler University Linz, Linz, Austria
  • Ann-Margret Ervin
    Epidemiology, Johns Hopkins University, Baltimore, Maryland, United States
  • Alexander Ho
    Doheny Eye Institute, Los Angeles, California, United States
  • Muneeswar Gupta Nittala
    Doheny Eye Institute, Los Angeles, California, United States
  • Isabelle S Audo
    ophthalmology, CHNO des Quinze-Vingts, DHU Sight Restore, INSERM-DHOS CIC 1423, Paris, France
    Ophthalmology, Sorbonne Universités, UPMC Univ Paris 06, INSERM, CNRS, Paris, France
  • David G Birch
    Retina Foundation of the Southwest, Dallas, Texas, United States
  • Hendrik P Scholl
    ophthalmology, University of Basel, Basel, Switzerland
  • Footnotes
    Commercial Relationships   Millena Bittencourt, None; Mohamed Ibrahim, None; Xiangrong Kong, None; Rupert Strauss, None; Ann-Margret Ervin, None; Alexander Ho, None; Muneeswar Gupta Nittala, None; Isabelle Audo, None; David Birch, None; Hendrik Scholl, Acucela Inc. (F), Boehringer Ingelheim Pharma GmbH & Co. KG (C), Daiichi Sankyo, Inc.: (C), Genentech Inc./F. Hoffmann-La Roche Ltd.: (R), Gensight Biologics (C), Genzyme Corp./Sanofi (R), Gerson Lehrman Group (C), Guidepoint (C), Intellia Therapeutics, Inc (C), NightstaRx Ltd.: (F), QLT, Inc. (F), ReNeuron Group Plc/Ora Inc. (R), Shire (C), Vision Medicines, Inc. (C)
  • Footnotes
    Support  grant numbers W81-XWH-07-1-0720 and W81XWH-09-2-0189
Investigative Ophthalmology & Visual Science June 2017, Vol.58, 3281. doi:
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    • Get Citation

      Millena Bittencourt, Mohamed A Ibrahim, Xiangrong Kong, Rupert Wolfgang Strauss, Ann-Margret Ervin, Alexander Ho, Muneeswar Gupta Nittala, Isabelle S Audo, David G Birch, Hendrik P Scholl; Natural progression of scotopic and photopic macular function loss in patients with Stargardt disease: The SMART Study.
      . Invest. Ophthalmol. Vis. Sci. 2017;58(8):3281.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Purpose : To assess changes in scotopic and photopic macular function in patients with Stargardt disease type 1 (STGD1) using microperimetry (MP).

Methods : Patients molecularly (ABCA4 gene mutations) confirmed, a sub-cohort of the ProgStar Study, were enrolled and followed every 6 months. The best eye from every patient was tested using a custom pattern on the MP1S system (Nidek Technologies). Patients underwent mesopic MP first using a white stimulus; they were then dark-adapted, and scotopic MP performed using a combination of blue stimuli and 1ND filter. Tests were graded at the Doheny Image Reading Center. Mean sensitivity (MS) in the ring 4-10 degrees from the fovea was calculated for both the mesopic (mMS) and scotopic (sMS) tests. The distance of the center of gravity of fixation points (preferred retinal locus or PRL) from the anatomical fovea was measured. Here we report changes from baseline to the 6-month visit.

Results : MP1 data from 59 patients from 6 centers were analyzed. 61% were female, 90% were white. The mean age was 34.2 (Standard deviation [SD] 13.4) years, the mean age of symptom onset was 25.3 (SD 13.4) years, and the mean disease duration was 9 years (SD 7.1). At baseline, the mean best corrected visual acuity was 53.2 letters (SD±19.1). The mean mMS was 12 (SD±5.0) dB, and the mean sMS was 11.3 (SD±5.4) dB. The mean mPRL was 4.9 degrees(SD 4.2) and the mean sPRL was 5.0 degrees (SD 3.8). Over 6 months, there was no significant change in mMS (change=-0.22, 95%CI (-4.5, 4.8) dB) or sMS (change=0.49, 95%CI (-4.9, 7.1)dB) . The changes in mMS and of sMS showed a Pearson correlation coefficient of 0.53 (p<0.0001). There was also no significant change in mPRL (change=0, 95%CI -9,6 degree), or in sPRL (0.03, 95%CI -7, 9 degrees). The changes in mPRL and of sPRL showed a Pearson correlation coefficient of 0.53 (p<0.0001).

Conclusions : Neither scotopic nor mesopic macular sensitivity showed statistically significant reduction at month 6. Similarly, there were no observed changes in PRL distance under scotopic or mesopic testing conditions at month 6. A longer follow-up period may likely be needed to estimate the rate of change in macular sensitivity and the dynamics of the PRL

This is an abstract that was submitted for the 2017 ARVO Annual Meeting, held in Baltimore, MD, May 7-11, 2017.

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