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K. Kuniyoshi, N. Uno, M. Irifune, Y. Shimomura; Rod (-Cone) Dystrophy With Bull’s-Eye Maculopathy . Invest. Ophthalmol. Vis. Sci. 2003;44(13):5091.
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© ARVO (1962-2015); The Authors (2016-present)
Purpose: To report clinical features of cases with rod(-cone) dystrophy who have bull’s-eye maculopathy. Methods: Subjects were 8 Japanese cases of rod(-cone) dystrophy (3 male cases and 5 female cases; mean age: 60) who had bull’s-eye maculopathy and retinal degeneration surrounding the vascular arcade in both eyes. Electroretinography (ERG), Goldmann perimetry, and dark adaptmetry were performed in all cases. Results: All cases had no visual symptoms until twenties. Six cases had family history of visual disturbance, and they might have autosomal-recessive traits. Goldmann perimetry showed absolute scotoma corresponding the retinal degenerations. Advanced cases showed big central scotoma. Dark adaptmetry showed an elevation of 2-4 log units as a final threshold. ERG revealed non-recordable rod responses and normal or slightly reduced cone responses. The b/a ratio in flash ERG showed less than 1.0 in 6 cases. ERG recorded with 100-msec. photo-stimulation showed significantly larger d-wave (off-response) than b-wave (on-response). These electrophysiological findings resembled those in Oguchi disease. Retinitis pigmentosa, Stargardt’s disease / fundus flavimaculatus, congenital retinoschisis, Goldmann-Favre disease, and enhanced s-cone syndrome should be considered as different diagnoses. Conclusions: These cases may be a new entity of retinal degeneration.
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