Abstract

To estimate mean rates of change of ocular function in patients with pigmented paravenous retinochoroidal atrophy (PPRCA). Retrospective observational case series. Fifteen patients aged 8 to 67 years with PPRCA were followed for 3 to 35 years (average follow-up time, 13 years) with measures of visual acuity, visual field area, and full-field electroretinogram amplitude. Mean annual exponential rates of change were quantified by repeated measures longitudinal regression. Estimated mean annual rates of change were -2.0% for visual acuity, +0.3% for visual field area, and -3.4% and -6.7% for 0.5 Hz and 30 Hz electroretinogram amplitudes, respectively. Patients with PPRCA have a slowly progressive disease with respect to the loss of peripheral vision.

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