CLINICAL AND HISTOPATHOLOGIC FINDINGS IN CLUMPED PIGMENTARY RETINAL DEGENERATION

Objective: To describe the clinical and histopathologic features of cl umped pigmentary retinal degeneration (CPRD). Design: Retrospective ca se series. Setting: Tertiary referral center. Patients: Twenty-four-pa tients, aged 7 to 83 years, were identified from the medical record fi les of the Berman-Gund Laboratory, Boston, Mass, as having the clinica l features of CPRD. The autopsy eye from a SG-year-old man with CPRD w as studied with light and electron microscopy. Main Outcome Measures: Visual acuities, visual fields, dark-adaptation thresholds, and result s of electroretinograms; histopathologic study of an autopsy eye. Resu lts: The functional deficit of patients with CPRD seems to be similar to that of patients with typical retinitis pigmentosa. Different degre es of severity were observed among patients of similar age. The histop athologic data showed that the clinically distinct areas of clumped pi gment are due to excessive accumulation of melanin granules in retinal pigment epithelial cells. Conclusion: Based on the distinct clinical and histopathologic appearance, CPRD should be considered as a separat e form of retinal degeneration.