This rare hereditary retinal dystrophy has not been reported previously in this country, although several reports have appeared in the British and European literature.1-7 The name "central senile areolar choroidal atrophy" was appended to the disorder in 1884 by Nettleship1 who first described this entity; and other authors,4,5,8 on the basis of the clinical picture, have used the term "central areolar choroidal sclerosis." However, the genetic nature of the disorder and the absence of a circumscribed sclerosis of the choroidal vessels make the term "central areolar choroidal dystrophy" preferable.
Report of Cases
Case 1 (Fig 1 and 2).
—This 57-year-old white female, the mother of patients 2 and 3, has had a bilateral decrease in visual acuity since age 14. This was of very rapid onset, and there was no progression of the symptoms after age 20. She prefers a darkened environment for good visual acuity.
CARR RE. Central Areolar Choroidal Dystrophy. Arch Ophthalmol. 1965;73(1):32-35. doi:10.1001/archopht.1965.00970030034008