PMC6314191_01
FAF of the right (A) and left (B) eyes, showing an area of mottled RPE and a subtle ring of hyper-autofluorescence in the perifoveal area. SD-OCT demonstrating outer retina atrophy outside of the fovea in the right (C) and left (D) eyes.
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F1
Fundus autofluorescence (FAF) highlighted the area of RPE scalloping and a ring of hyper-autofluorescence in the perifoveal area (Figure 1). Spectral domain optical coherence tomography (SD-OCT) showed vitreomacular traction in the right eye and loss of the ellipsoid band, along with outer retinal atrophy, outside of the fovea in both eyes (Figure 1).
Full field ERG showing extinguished rod response and severely attenuated cone response. Tracing of normal subject is shown.
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F2
Rod responses were extinguished and cone responses were severely attenuated bilaterally (Figure 2).
Progressive loss of ERG within 3 months. Photopic 30 Hz flicker ERG using Ganzfeld stimulation, at presentation (A) and at three months (B).
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F3
Photopic 30 Hz-flicker ERG was performed using Ganzfeld stimulation and showed further reduction in the amplitude in the left eye (Figure 3).