Highest potential mean-to-standard deviation ratio (MSDR) for each single outcome measure at different weightings with all possible weight combinations of the other metrics.

<p>MSDRs for best-corrected visual acuity (grey) decrease at increasing weight. MSDRs for transition zones of questionably decreased autofluorescence (blue) increase until 25% weight, but gradually decrease at higher weights. MSDRs for transition zones of definitely decreased autofluorescence decrease at weights higher than 5%. MSDRs for loss of the ellipsoid zone (green) are constant, but decrease substantially at weights higher than approximately 70%. MSDRs for loss of the external limiting membrane (black) decrease at weights higher than approximately 80%.</p

<i>ABCA4</i> variants in early-onset Stargardt patients from Radboud university medical center (Radboudumc) and Moorfields Eye Hospital (MEH).

<p><i>ABCA4</i> variants in early-onset Stargardt patients from Radboud university medical center (Radboudumc) and Moorfields Eye Hospital (MEH).</p

Characteristics of early-onset Stargardt cohorts from Radboud university medical center (Radboudumc) and Moorfields Eye Hospital (MEH).

<p>Characteristics of early-onset Stargardt cohorts from Radboud university medical center (Radboudumc) and Moorfields Eye Hospital (MEH).</p

Yearly progression rate of changes in retinal eccentricity (<i>ε</i>) by visual function, fundus autofluorescence and optical coherence tomography.

<p>Yearly progression rate of changes in retinal eccentricity (<i>ε</i>) by visual function, fundus autofluorescence and optical coherence tomography.</p

Schematic and representative images of measurements in fundus autofluorescence imaging and spectral-domain optical coherence tomography.

<p>Patient 9: area of questionably decreased autofluorescence (QDAF, blue), 1.37 mm²; area of definitely decreased autofluorescence (black), 0.33 mm²; transverse loss of external limiting membrane (ELM-loss, red), 1.75 mm, transverse loss of ellipsoid zone (EZ-loss, blue), 2.24 mm; best-corrected visual acuity, 20/100; <i>ABCA4</i> variants, c.1622T>C;3113C>T:p.[Leu541Pro;Ala1038Val] and c.6316C>T:p.(Arg2106Cys).</p

Visual acuity as a function of degrees of retinal eccentricity.

<p>Visual acuity as a function of degrees of retinal eccentricity.</p

Weighted composite score and predicted outcome.

<p>Matching colors represent the right and left eye of the same patient. (A) Results from six early-onset Stargardt patients. (B) The predicted outcome in the replication cohort showed comparable results.</p