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|Classification and external resources|
Oguchi disease, also called congenital stationary night blindness, Oguchi type 1 or Oguchi disease 1, is an autosomal recessive form of congenital stationary night blindness associated with fundus discoloration and abnormally slow dark adaptation.
Oguchi disease present with nonprogressive night blindness since young childhood or birth with normal day vision, but they frequently claim improvement of light sensitivities when they remain for some time in a darkened environment.
On examination patients have normal visual fields but the fundi have a diffuse or patchy, silver-gray or golden-yellow metallic sheen and the retinal vessels stand out in relief against the background.
A prolonged dark adaptation of three hours or more, leads to disappearance of this unusual discoloration and the appearance of a normal reddish appearance. This is known as the Mizuo-Nakamura phenomena and and is thought to be caused by the overstimulation of rod cells.
Other conditions with similar appearing fundi include
These conditions do not show the Mizuo-Nakamura phenomenon.
Oguchi's disease is unique in its electroretinographic responses in the light- and dark-adapted conditions. The A- and b-waves on single flash electroretinograms (ERG) are decreased or absent under lighted conditions but increase after prolonged dark adaptation. There are nearly undetectable rod b waves in the scotopic 0.01 ERG and nearly negative scotopic 3.0 ERGs.
Dark-adaptation studies have shown that highly elevated rod thresholds decrease several hours later and eventually result in a recovery to the normal or nearly normal level.
The S, M and L cone systems are normal.
Cause and Genetics
It was described by Chuta Oguchi (1875-1945), a Japanese ophthalmologist, in 1907. The characteristic fundal appearances were described by Mizuo in 1913.
Eye disease - pathology of the eye (H00-H59, 360-379)
lacrimal system: Dacryoadenitis - Epiphora - Dacryocystitis
orbit: Exophthalmos - Enophthalmos
|Optic nerve and visual pathways||
Optic neuritis - Papilledema - Optic atrophy - Leber's hereditary optic neuropathy - Dominant optic atrophy - Optic disc drusen - Glaucoma - Toxic and nutritional optic neuropathy - Anterior ischemic optic neuropathy
accommodation and refraction
Paralytic strabismus: Ophthalmoparesis - Progressive external ophthalmoplegia - Palsy (III, IV, VI) - Kearns-Sayre syndrome
Other strabismus: Esotropia/Exotropia - Hypertropia - Heterophoria (Esophoria, Exophoria) - Brown's syndrome - Duane syndrome
|Visual disturbances and blindness||
Amblyopia - Leber's congenital amaurosis - Subjective (Asthenopia, Hemeralopia, Photophobia, Scintillating scotoma) - Diplopia - Scotoma - Anopsia (Binasal hemianopsia, Bitemporal hemianopsia, Homonymous hemianopsia, Quadrantanopia) - Color blindness (Achromatopsia, Dichromacy, Monochromacy) - Nyctalopia (Oguchi disease) - Blindness/Low vision
Trachoma - Onchocerciasis
See also congenital
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