Fluctuating vision in Best disease.

Abstract

Best disease is a vitelliform macular dystrophy characterized by autosomal dominant inheritance, an abnormal electrooculogram, a normal electroretinogram, and a variety of fundus lesions. 1-4 We describe 2 patients from 2 well-characterized families with Best disease 1,3 who experienced transient visual loss with little or no change in the ophthalmoscopic appearance of their central vitelliform lesions. Report of Cases. Case 1. A 28-year-old man lost the central vision in his left eye 13 years ago from subfoveal choroidal neovascularization. He returned complaining of rapid loss of vision in his right eye during a 10-day period. He had no medical problems and was not receiving medication. Visual acuity measured 20/100 OD and 20/200 OS. In the right macula, the vitelliform lesion had slightly less yellowish material and more pigmentary changes than in the photographs taken 13 years earlier, but neither clinical examination nor fluorescein angiography showed any evidence of