Scotopic Microperimetric Assessment of Rod Function in Stargardt Disease (SMART) Study: Design and Baseline Characteristics (Report No. 1)

Ophthalmic Res. 2019;61(1):36-43. doi: 10.1159/000488711. Epub 2018 Jun 25.

Abstract

Purpose: To describe the study design and characteristics at first visit of participants in the longitudinal Scotopic Microperimetric Assessment of Rod Function in Stargardt Disease (SMART) study.

Methods: Scotopic microperimetry (sMP) was performed in one designated study eye in a subset of participants with molecularly proven ABCA4-associated Stargardt disease (STGD1) enrolled in a multicenter natural history study (ProgStar). Study visits were every 6 months over a period ranging from 6 to 24 months, and also included fundus autofluorescence (FAF).

Results: SMART enrolled 118 participants (118 eyes). At the first visit of SMART, the mean sensitivity in mesopic microperimetry was 11.48 (±5.05; range 0.00-19.88) dB and in sMP 11.25 (±5.26; 0-19.25) dB. For FAF, all eyes had a lesion of decreased autofluorescence (mean lesion size 3.62 [±3.48; 0.10-21.46] mm2), and a total of 76 eyes (65.5%) had a lesion of definitely decreased autofluorescence with a mean lesion size of 3.46 (±3.60; 0.21-21.46) mm2.

Conclusions: Rod function is impaired in STGD1 and can be assessed by sMP. Testing rod function may serve as a potential outcome measure for future clinical treatment trials. This is evaluated in the SMART study.

Keywords: Clinical trials; Endpoints; Mesopic microperimetry; Scotopic microperimetry; Stargardt disease.

Publication types

  • Multicenter Study

MeSH terms

  • Adult
  • Aged
  • Female
  • Humans
  • Longitudinal Studies
  • Macular Degeneration / congenital*
  • Macular Degeneration / physiopathology
  • Male
  • Middle Aged
  • Night Vision / physiology*
  • Research Design
  • Retinal Rod Photoreceptor Cells / physiology*
  • Stargardt Disease
  • Visual Acuity / physiology
  • Visual Field Tests
  • Visual Fields / physiology*
  • Young Adult