Electroretinograms and pattern visually evoked cortical potentials in central areolar choroidal dystrophy

We describe three patients with central areolar choroidal dystrophy whose electroretinograms (ERGs) and pattern visually evoked cortical potentials (VECPs) confirmed their macular dysfunction. Visual fields measured by Goldmann perimetry showed central relative scotomata corresponding to a dystrophic lesion. Dark adaptation curves were slightly abnormal either in the first curve or in the second curve, depending on the visual acuity. Color vision was disturbed irregularly. Fluorescein angiography revealed a loss of the choriocapillaries, and a hyperfluorescent border outlined the dystrophic lesion. Single-flash dark-adapted ERGs and scotopic ERGs were almost normal, while photopic and flicker ERGs showed slightly attenuated amplitudes. Conversely, VECPs to pattern reversal stimulation were greatly affected for both transient and steady-state stimuli. We conclude that, in central areolar choroidal dystrophy, pattern VECPs can provide more information on macular dysfunction than does the ERG.