Oguchi's disease, first described by Chuta Oguchi in 1907. is a rare autosomal recessive trait characterized by congenital stationary night blindness and an unique morphological and functional abnormality of the retina. Patients have nonprogressive night blindness since young childhood with normal day vision, but they often claim improvement of light sensitivities when they remain long in the dark environment; dark-adaptation study demonstrates that highly elevated rod thresholds decrease several hours later and eventually result in a recovery to the normal or nearly normal level. The eyegrounds have an appearance of diffuse or patchy, silver-gray or golden-yellow metallic sheen and the retinal vessels stand out in relief against the radiant background. A prolonged dark adaptation of three hours ormore, leads to disappearance of the unusual discoloration the normal reddish appearance, called Mizuo-Nakamura phenomenon. Oguchi's disease is also unique in the electroretinographic responses in the light- and dark-adapted condition. Recent identification of the arrestin gene mutation in patients with Oguchi's disease may account for the characteristic fundus and functional abnormality.
The fundul photographs illustrate unique fundus appearance, taken in the ordinary light and in the prolonged dark adaptation condition, in two Japanese patients with Oguchi's disease, one patient in Tokyo and the other in Kagoshima.

OGUCHI, Chuta (1875-1945)

Japanese ophthalmologist, Professor Emeritus of Nagoya University. He graduated from a private medical school in Tokyo (Saisei Gakusha) and passed the National Examination for Medical Practice in 1891. He learned Ophthalmology under Dr. SUDA Tetsuzo, and then at the Postgraduate Course of Tokyo University under Prof. KOMOTO Jujiro. He became an Army Surgeon and worked at many hospitals in Japan and in Taiwan. In 1907 at the Army Hospital in Tokyo, he examined a soldier who complained of night blindness, first suspected of malingering. Dr. Oguchi found a peculiar fundus color with golden and mottled appearance and dark retinal vessels. He also found that the parents of this patient were cousins (J. Jpn. Ophthalmol. Soc. 11: 123, 1907). He further added similar cases in 1910, and concluded that this is a distinct clinical entity with congenital hemeralopia with autosomal recessive inheritance (Ueber die eigenartige Hemeralopie mit diffuse weissgraulicher Verfaerbung des Augenhintergrundes, v Graefe Arch Ophthalmol. 81: 109,1912). Prof. J. KOMOTO named it as Oguchi's Disease. Dr.Oguchi continued to study ophthalmology at University of Heidelberg and at the University of Munich in 1912-1914. After homecoming, he received the degree Doctor of Medical Sciences from Tokyo University in 1916. He was appointed the Professor and Chairman of the Department of Ophthalmology of Nagoya University in 1922 and worked until retirement in 1939. He delivered a special lecture at the 40th Congress of the Japanese Ophthalmological Society in 1936, and served as the 41st Congress President of the Society in 1937. He also served as a Member of the International Council of Ophthalmology 1929-1937. He was granted the Japan Academy of Science Award in 1933 for his work on Oguchi's disease. In recognition of his service, the Government conferred on him the Second Order of the Sacred Treasures.(International Biography and Bibliography of Ophthalmologists and Vision Scientists,IBBO. Jean-Paul Wayenborgh, Saiichi Mishima and C. Richard Keeler. J.-P. Wayenborgh, Belgium, 2002)