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May 1970

Butterfly—Shaped Pigment Dystrophy of the Fovea

Author Affiliations

Rotterdam, the Netherlands
From the Eye Hospital, Rotterdam Medical Faculty, Rotterdam, the Netherlands.

Arch Ophthalmol. 1970;83(5):558-569. doi:10.1001/archopht.1970.00990030558006

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.