Disease Course of Patients With Pericentral Retinitis Pigmentosa

Purpose To estimate the mean rates of decline of ocular function in patients with an atypical form of retinitis pigmentosa, termed "pericentral retinitis pigmentosa." Design Retrospective observational study. Methods setting: Single-center. patient population: Eighteen patients (ages 32 to 65 years) with pericentral retinitis pigmentosa followed for 3 to 26 years. observational procedures: Snellen visual acuity, Goldmann visual field area (V-4e white test light), and full-field electroretinogram (ERG) amplitudes (0.5 Hz and 30 Hz white flashes). main outcome measures: Mean annual exponential rates of change quantified by repeated-measures longitudinal regression. Results Estimated mean annual rates of decline of remaining ocular function were 1.2% for visual acuity, 1.9% for visual field area, 2.5% for ERG amplitude to 0.5 Hz flashes, and 2.9% for ERG amplitude to 30 Hz flashes. Conclusions The mean rates of loss of remaining ocular function of patients with pericentral retinitis pigmentosa were generally slower than those previously reported for patients with typical forms of retinitis pigmentosa.