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Retinitis pigmentosa in a male aged 48 years (Fam1-365). Rapid progression over a 20-year period from relatively preserved photoreceptors and RPE to a complete loss of rod and greatly reduced cone function. Lower limits of normal (P < 0.05) are 85 μV for rod ERG, 200 μV for standard combined ERG, 50 μV for 30-Hz flicker ERG, and 75 μV for single flash cone ERG.
