19 patients showing the features of diffuse retinal pigment epitheliopathy, as described by Zweng and Little, were studied. Diffuse retinal pigment epitheliopathy resembles both pigment epithelial detachment and central serous choriopathy, but can be differentiated by the following characteristic features: widespread distribution of small pigment epithelial detachments, little or no leakage visible on the fluorescence angiogram, extensive pigmentary changes, chronic course with exacerbations and remissions, and fair visual prognosis.
Fluorescein angiography was performed in 71 persons at risk to develop Leber's Optic Neuropathy (L.O.N.), in 10 females carriers of this disease and in 5 patients during the acute phase. The examination was performed in three university eye clinics. The angiograms were randomly mixed with an equal number of 'controls' and reviewed in a blind fashion on two occasions by two observers. A scoring system was developed on the basis of the peripapillary microvascular changes known to occur in the acute phase of L.O.N. All 5 patients could be recognised in this manner. In the pictures of one of the clinics, which were of good quality, a significant difference in scoring between the at risk and control groups appeared to exist. The results suggest that carriers also may have the same fluorescein angiographic abnormalities. One man from the at risk group who obtained high abnormal scores-identical to patients in the acute phase-developed L.O.N. 6 months later.
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