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Norrie's disease in an Asian family
D G Harendra de Silva1, D B de Silva
1Faculty of Medicine, University of Ruhuna, Galle, Sri Lanka.
The British Journal of Ophthalmology
|January 1, 1988
Abstract:
Norrie's disease, a congenital progressive oculo-acoustico-cerebral degenerative condition, is a sex linked recessive disorder. Previously described as atrophia oculi congeneti, it is associated with bilateral pseudotumour of the retina, lens, and corneal opacities, and phthisis bulbi. Some patients develop progressive deterioration of mental function and hearing. We report a Sri Lankan family with typical features of Norrie's disease.