PMC4327332_01
Fundus photos of both eyes showing optic nerves with a normal appearance, and pigment mottling in the macula in the right (a) and the left eye (b). Goldmann visual fields documented bilateral central scotoma to the I4e test isopter, spanning a 10-degree radius from the center of vision both in the left (c) and the right eye (d).
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F1
A dilated fundoscopy (fig. 1a, b) revealed a normal appearance of his optic nerves. Goldmann perimetry revealed bilateral central scotoma to the I4e test object, spanning a 10-degree radius from the center of vision (fig. 1c, d).
ERG recordings. A Ganzfeld stimulator, with bright flash intensity calibrated to 2.12 cd s/m2, was used. The flash duration was 10 ms. The band pass of the amplifiers was set at 0.3-300 Hz. A 24-dB neutral density filter over a strobe lamp was presented as a dim white flash stimulus. The background of the Ganzfeld dome was then illuminated at 39 cd/m2. Using the same background illumination, the bright flash was presented at 30 Hz and then sweeps were averaged. Dim scotopic (a, e) and bright scotopic (b, f) were normal, while bright photopic was unrecordable (c, g) and 30-Hz flicker showed reduced and delayed tracings (d, h), both upon first presentation as well as 4 years later (a-h).
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F2
Analysis of these findings suggested a cone-specific degeneration (fig. 2a-d). Repeat ERG 4 years after initial testing showed a bilateral cone dysfunction (fig. 2e-h).