Natural course of retinitis pigmentosa over a three-year interval

Ninety-four patients, 6 to 49 years old, with progressive forms of retinitis pigmentosa were examined at baseline and annually for three consecutive years with respect to visual acuity, kinetic visual fields, dark-adaptation thresholds, computer-averaged electroretinograms, and fundus photographs. A subset was recalled within two months of a given visit to measure intervisit variability and to develop criteria for what constitutes significant (P less than .01) functional change. Over a three-year interval full-field electroretinograms declined significantly in 66 of 86 patients (77%) with detectable responses at baseline. Patients lost an average of 16% to 18.5% of remaining electroretinographic amplitude per year and 4.6% of remaining visual field area per year. Bone spicule pigmentation increased in 41 of 76 patients for whom we could make comparisons over a three-year interval (54%). Visual acuity and dark-adaptation thresholds remained relatively stable.