Abstract
Abstract: :
Purpose:To examine the retinal functions of patients with adult-onset vitelliform macular dystrophy (AOVMD) electrophysiologically. Methods:Full-field electroretinograms (ERGs), multifocal ERGs, (mfERGs), and electrooculgrams (EOGs) were recorded from six patients with AOVMD. Patientsí ages ranged from 40 to 54 years at the time of the initial diagnosis. Results:The visual acuity was 20/20 in 5 eyes, 20/25 in 3 eyes, 20/30 in 2 eyes, and 20/40 in 2 eyes. Bilateral macular lesions at different stages, eg, pre-vitelliform, vitelliform, and scramble egg stages, were observed ophthalmoscopically. Full-field cone and rod ERGs were normal in all eyes except in one eye where the visual acuity had decreased to 20/30. The mfERGs from the macular area were markedly reduced in all eyes. The L/D ratio determined by EOG was normal (≷1.85) in 7 eyes and subnormal in 5 eyes ranging from 1.31 to 1.71. Scanning laser ophthalmoscopy showed multiple lesions with an irregular reflex in the deep retina scattered throughout the posterior pole. Conclusion:These findings indicate that the macular functions in patients with AOVMD were markedly disturbed although the visual acuity was relatively preserved. The L/D ratio examined with the EOG was also decreased in some cases, however, the defect was far milder than Best's disease, indicating less severe damage to the RPE.
Keywords: 460 macula/fovea • 393 electrophysiology: clinical • 395 electroretinography: clinical