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Title: Abnormal dark-adapted electroretinogram in Best's vitelliform macular degeneration. Author: Lachapelle P, Quigley MG, Polomeno RC, Little JM. Journal: Can J Ophthalmol; 1988 Oct; 23(6):279-84. PubMed ID: 3203242. Abstract: It is generally well accepted that in Best's vitelliform macular degeneration (BVMD) the electroretinogram (ERG) is normal whereas the electro-oculogram (EOG) is markedly abnormal. We describe a patient in whom BVMD was suspected on the basis of the clinical findings, EOG and family history (one of her daughters had the typical vitelliform lesion). However, her dark-adapted ERG was markedly abnormal. Similar anomalies were found in the dark-adapted ERG of the daughter. While the temporal features of the various ERG waves were well preserved, a substantial decrease in the amplitude of specific segments of the ERG signal was observed. A similar decrease in the amplitude of the oscillatory potentials was also found. We believe that this unusual combination of BVMD and abnormal dark-adapted ERG may be due to the reported reduced penetrance and variable expressivity of the BVMD gene(s).[Abstract] [Full Text] [Related] [New Search]