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Autosomal Dominantly Inherited Retinitis PigmentosaVisual Acuity Loss by Subtype
Marilyn D. Farber, DrPH;
Gerald A. Fishman, MD;
Robert A. Weiss, MD
Arch Ophthalmol. 1985;103(4):524-528.
Abstract
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Sixty-eight patients with autosomal dominant retinitis pigmentosa were divided into two distinct subtypes and studied for visual function using a life-table analysis. Type 1 patients (n = 23) had diffuse pigmentation, concentric visual field loss, and no recordable electroretinogram. Type 2 patients (n = 45) as a group had regionalized pigmentation, sectorial field loss, and some recordable electroretinogram. The cumulative probability of maintaining a visual acuity of 6/12 (20/40) or better over each decade of life decreased rapidly in eyes of type 1 patients; for type 2 eyes, this probability remained above 90% through the fifth decade of life. The presence of an atrophic-appearing foveal lesion was associated with a reduction in visual acuity to 6/15 (20/50) or worse in a majority of patients. Data from this study indicate that investigators should look for subtypes within the major genetic groupings of retinitis pigmentosa for more accurate assessment of a patient's potential for maintaining good central visual acuity.
Author Affiliations
From the Department of Ophthalmology, Eye and Ear Infirmary, University of Illinois at Chicago.
Footnotes
Accepted for publication Oct 18, 1984.
Reprint requests to Eye and Ear Infirmary, University of Illinois, 1855 W Taylor St, Chicago, IL 60612 (Dr Farber).
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