Abstract
Abstract: :
Purpose: To measure mean rates of change of ocular function in patients with pericentral retinitis pigmentosa. Methods: We followed the change in Snellen visual acuity, Goldmann visual field area (V4e test light), and full-field ERG amplitude (0.5 Hz and 30 Hz flashes) for an average of 8.6 years in 8 patients (ages 33 - 77 years, 4 males and 4 females) with pericentral retinitis pigmentosa. The diagnosis was based on a visual acuity of 20/80 or better, a scotoma between the 5 degree and 20 degree isopters, and bone-spicule pigmentation in areas corresponding to the field loss. Patients presented with a geometric mean visual acuity of 20/28, a geometric mean visual field area of 9226 square degrees (108 degree equivalent diameter; norm equals 120 degrees or greater), and geometric mean ERG amplitudes of 176 µV to 0.5 Hz flashes (norm equals 350 µV or greater) and 36 µV to 30 Hz flashes (norm equals 50 µV or greater). Results: The mean exponential rates of decline were 8.0%/year for visual acuity, 3.0%/year for visual field area, and 4.1%/year and 5.0%/year for ERG amplitude to 0.5 Hz and 30 Hz flashes, respectively. Conclusion: The rate of visual acuity loss was within and the rates of visual field and ERG loss were below those previously reported for groups of patients with the common forms of retinitis pigmentosa.
Keywords: 562 retinal degenerations: hereditary • 352 clinical (human) or epidemiologic studies: natural history