Four of five white brothers and a son of one of them were found to have peculiar, bilateral, butterfly-shaped pigment dystrophy of the fovea. Their cases have been followed up since 1965. Three of them have normal visual acuity, and two have a slightly diminished visual acuity. Four of the five affected individuals have a pathological electro-oculogram, which reflects a diffuse abnormality of the retina. At funduscopy only two of the five affected subjects have peripheral alterations. Probably this condition is transmitted as an autosomal dominant.
Deutman AF, van Blommestein JDA, Henkes HE, Waardenburg PJ, Driest ES. Butterfly—Shaped Pigment Dystrophy of the Fovea. Arch Ophthalmol. 1970;83(5):558–569. doi:10.1001/archopht.1970.00990030558006
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