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Ophthalmology · Retina: Oguchi disease
Sample itemA boy has lifelong non-progressive night blindness. His fundus has a golden-yellow sheen that returns to a normal colour after several hours in the dark and reappears after light exposure. What is the diagnosis?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
B. Oguchi disease
Oguchi disease is a recessive form of congenital stationary night blindness in which the fundus colour normalises after prolonged dark adaptation, the Mizuo-Nakamura phenomenon. Vision and fields are otherwise preserved and the condition does not progress.
Option by option
A. Retinitis pigmentosa
Retinitis pigmentosa is progressive with bone-spicule pigment and field loss.
B. Oguchi disease (correct)
Correct. A golden sheen that normalises in the dark is the hallmark of Oguchi disease.
C. Vitamin A deficiency
Vitamin A deficiency causes night blindness with xerosis and Bitot spots, and is not lifelong from birth.
D. Fundus albipunctatus
Fundus albipunctatus shows numerous small white dots at the level of the RPE without a colour change that reverses.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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