Evaluation of typical and atypical retinal pigmentary dystrophy by three dimensional analysis (XY plane and time) of averaged electroretinograms

: Retinal functional imaging of patients with typical and atypical retinal pigmentary dystrophies was investigated by three dimensional (XY plane and time) analysis of ERG topography by comparing visual field and fluorescein angiographic findings. The three dimensional analysis revealed that the area of maximal amplitude deviated to the skin area closest to the dominant location of the retinal pigmentary dystrophy (the so-called paradoxical localization). In patients with temporoinferior sectorial retinal pigmentary dystrophy, for example, the maximal amplitude of the a-, b-waves and retinal oscillatory potentials deviated toward the temporoinferior side on the surface topography. These characteristic phenomena of a- and b-waves were found in 60.8% of all patients. Flicker topography with a stimulus frequency of 30 Hz was especially successful in showing the existence and location of paramacular involvement of retinal dystrophy within the area surrounding temporal vascular arcades. The detectability of macular asymmetric involvement was 65.2%. No significant topographic changes were detected in cases in the early stage with no remarkable visual field defects, or in the end stage with remarkable concentric field defects and complicated glaucomatous visual field defects. A comparative study of topographic changes, visual field changes and fluorescein angiographic findings showed that topographic changes in the a-, b-waves, retinal oscillatory potentials and 30Hz flicker components coincided more closely with visual field changes than fluorescein angiographic findings. We proposed that retinal pigmentary dystrophy is not a homogeneous lesion in its progression and believe that the ERG topography method can, by the imaging of dominant locations, detect this disease as well as visual field testing.