Table 1 of
Rosenberg, Mol Vis 2007;
13:1962-1969.
Table 1. Clinical classification of phenotypes
The table presents the clinical delineations used, distinguishing among five types and two subtypes. RPE represents retinal pigment epithelium, ERG represents electroretinography.
Phenotype Clinical characteristics
-------------------------------- -----------------------------------------------
1. Stargardt maculopathy Oval-shaped RPE irregularity and thinning,
"beaten bronze" reflexes, and perifoveal
yellow-grey flecks
2. Stargardt-flavimaculatus and Abundant fish-tail-like yellowish flecks
Fundus flavimaculatus outside the foveal area with foveal
atrophy of RPE
3a. Central and peripheral RPE- Ill-defined macular RPE-atrophy with
choriocapillaris atrophy coarse hyperpigmentation and peripheral
atrophic RPE speckles and/or webs
3b. Atypical RP (choroidoretinal Diffuse choroidoretinal dystrophy with
photoreceptor dystrophy) narrow arterioles, constricted visual fields,
and severely reduced full-field ERG rod
and cone responses
4. Cone-rod dystrophy Foveal RPE atrophy with early predominant
cone affection of the full-field ERG
5a. Bull's eye maculopathy Foveal bull's eye lesion without flecks,
varied involvement of peripheral RPE atrophy,
and normal or subnormal full-field ERG
5b. Unspecified maculopathy Foveal RPE atrophy without flecks and
without significant ERG changes
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