Title: 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. The patient was recruited through the Japanese Retinitis Pigmentosa Registry Project. Ethical approval for this study was granted by the Institutional Review Board of Tohoku University School of Medicine. The old fundus photograph showing classical Oguchi disease was recovered from the articles of Dr. Takehisa Oguchi (deceased). (Magnified version of Fig A-B is available online at www.aaojournal.org).