Autosomal dominantly inherited retinitis pigmentosa. Visual acuity loss by subtype.
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.
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D012174:Retinitis Pigmentosa / Q000145:classification / Q000235:genetics / Q000503:physiopathology*; D014792:Visual Acuity