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July 1982

Best's Macular Dystrophy

Author Affiliations

San Francisco

Arch Ophthalmol. 1982;100(7):1067. doi:10.1001/archopht.1982.01030040045002

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This issue of the Archives contains two excellent articles on the histopathology of Best's macular dystrophy (BMD), each of which contributes to our understanding of the dystrophy. Weingeist et al (p 1108) report on one of those all too infrequent occurrences in which eyes were obtained from a young patient with well-documented BMD. In this study, the retinal pigment epithelium (RPE) was found by light microscopy to be diffusely abnormal, a finding that is consistent with the well-known electro-oculographic (EOG) abnormality in BMD. The RPE cytoplasm was filled with PAS-positive granular material believed to be lipofuscin. Abnormal mucopolysaccharide was not found. The lipofuscin was most prominent in the macula but was also present in the retinal periphery. Lipofuscin was also found between the sensory retina and the RPE and within macrophages in the subsensory retinal space. Electron microscopy of the entire RPE disclosed fewer melanosomes than normal. Electron-dense granular material

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