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- Negative waveform ERG
- Mizuo–Nakamura phenomenon
- cone-rod dystrophy
A 79-year-old Pakistanian man presented to the Jules Stein Eye Institute with progressive decline of vision in both eyes over four decades associated with nyctalopia. Past ocular history was significant for retinal detachment status post scleral buckle surgery (1982) and cataract extraction (2006) in the right eye. Past medical history was positive for coronary artery disease, atrial fibrillation, hypertension and hypercholesterolaemia. Family history was remarkable only for myopia, and he denied consanguinity.
Ocular exam revealed Snellen visual acuity of 20/200 OD and 20/400 OS. There was no relative afferent pupillary defect. Anterior segment exam was unremarkable. Dilated fundoscopic exam revealed an ovoid area of macular atrophy with pigment mottling in both eyes. There was mild retinal vascular attenuation but no evidence of bone spicule migration in either eye. Interestingly, the mid-peripheral fundus of both eyes exhibited a golden, tapetal-like sheen (figure 1A) which was extinguished after 30–45 min of dark adaptation (figure 1B).