Author Affiliations: Division of Epidemiology and Clinical Research (Dr Cukras), Ophthalmic Genetics and Visual Function Branch (Drs Cukras, Caruso, Zein, and Sieving), Office of the Scientific Director (Dr Wong), and Office of the Clinical Director (Ms Cunningham), National Eye Institute, National Institutes of Health, Bethesda, Maryland.
We agree with Smith that the subject of hyperautofluorescent changes in Stargardt disease is an interesting one that may be illuminating to understanding underlying disease pathophysiology. In our report, we had examined longitudinal changes in fundus autofluorescence over a series of time points in a number of affected eyes. Our observations describe a consistent pattern of temporal and spatial progression in the flecks associated with Stargardt disease. The general pattern was one in which brighter hyperautofluorescent flecks move centrifugally from the center of the macula. As these flecks move outward, they leave in their wake darker, hypoautofluorescent lesions. As a result, fundus autofluorescence at a particular point in the fleck's path may be described to be first increasing with time, becoming increasingly hyperautofluorescent relative to background, then decreasing back to background levels, and subsequently decreasing further to become hypoautofluorescent relative to background. We agree with Smith that within the time of our study (11-57 months), we did not observe further decreases in fundus autofluorescence in the tracks left behind by progressing flecks that may be described as frank atrophy (ie, the confluent absence of autofluorescence signal suggestive of retinal pigment epithelial cell loss).
Cukras CA, Wong WT, Caruso R, Cunningham D, Zein W, Sieving P. Fundus Autofluorescence Patterns in Stargardt Disease Over Time—Reply. Arch Ophthalmol. 2012;130(10):1354-1355. doi:10.1001/archophthalmol.2012.2008