GYRATE ATROPHY OF THE CHOROID AND RETINA

Gyrate atrophy of the choroid and retina is characterized by autosomal recessive inheritance, progressive chorioretinal atrophy beginning in late childhood, and hyperornithinemia with ornithinuria caused by def icient ornithine aminotransferase activity. In this paper, four patien ts with gyrate atrophy are described. All patients had visual impairme nt, mental retardation, hyperornithinemia, hypolysinemia, ornithinuria and lysinuria. The first case had hypermetropic astigmatism in contra st to other reported gyrate atrophies. These are the first reported ca ses from Turkey, but gyrate atrophy may not be rare in Turkey since th e frequency of some other metabolic disorders has also been reported t o he high. It is suggested that gyrate atrophy must he considered in a ll patients with chorioretinal atrophy.