Progression from Classical Oguchi Disease to Retinitis Pigmentosa after 50 Years

Abstract

Fundus photograph of a 77-year-old Japanese man with retinitis pigmentosa (RP) and homozygous c.636delT in S-antigen (SAG) (Fig A). The patient’s condition had been reported as Oguchi disease (Fig B) with Mizuo-Nakamura phenomenon at the 20th International Congress of Ophthalmology held at Munich in 1966. Fundus photograph of the same patient taken around that time (age ∼23) showed metallic sheen covering the posterior pole with no sign of retinal degeneration. These photographs mark the first example of progression of classical juvenile Oguchi disease to RP.