Ferrell et al. (1983) reported a large family segregating autosomal dominant atypical vitelliform macular dystrophy. In this family, fluorescein angiography was more helpful than electrooculogram (EOG) in ascertaining affected persons. Early signs were minimal angiographic changes in the ... Ferrell et al. (1983) reported a large family segregating autosomal dominant atypical vitelliform macular dystrophy. In this family, fluorescein angiography was more helpful than electrooculogram (EOG) in ascertaining affected persons. Early signs were minimal angiographic changes in the macula and peripapillary region, and small yellow lesions in the macula and periphery. Moderate accumulations of the yellow material in the central and peripheral retina, and advanced depigmented lesions of the central and peripheral retina and peripapillary region were also documented in family members. These findings were similar to those in typical vitelliform macular dystrophy (153700), which involves the retinal pigment epithelium (RPE) and invariably has an abnormal EOG. Hittner et al. (1984) reported further on the 5-generation family studied by Ferrell et al. (1983). Forty-three of 101 at-risk members were affected (43%). Vision varied from 20/20 to 20/200. EOG studies were normal or reduced and did not correlate with visual acuity. Retinal findings consisted of macular or extramacular punctate yellow lesions or both in the RPE, which were hypofluorescent by angiography.