A Case of Gyrate Atrophy of the Choroid and Retina with Hypogenitalism.

GYRATE atrophy of the choroid and retina is a very rare condition whichl was first described by Cutler in 1894. It is said to be one of the abiotrophies and to be related to retinitis pigmentosa. The evidence upon w-hich this is based is that the symptoms of night blindness, contracting visual fields and progression to blindness are common to both conditions. Moreover in gyrate atrophy there is some disturbance of the retinal pigment, and abnormalities are often found in the papilla and retinal vessels similar to those observed in pigmentary retinal degeneration. In gyrate atrophy myopia is said to be constant, in retinitis pigmentosa it is not uncommon, and cataract is frequently found in both conditions. A pedigree has been described by Bohm (1919) in which both abnormalities have been found, and Zorn (1920) and Beckershaus (1926) have seen retinitis pigmentosa and conditions somewhat similar to gyrate atrophy in other families. In its typical form gyrate atrophy may be recognised by an atrophic area around the disc, surrounded by a zone of normal choroid in which further atrophic patches are again seen towards the periphery. The atrophic portions are lobulated to form bays projecting into the normal choroid. Pigmentation is frequently seen at the edges of the bays as well as in discrete deposits in front of the retinal vessels. There has been some variability in the cases recorded and Sorsby (1939) has classified them into three groups whose names sufficiently illustrate their characteristics. (a) The garland type. (b) The multiple colobomatous type. (c) The disseminated pigmentary type. A case of the first type under Mr. Arnold Sorsby's care has no hypogenitalism and the glucose tolerance curve is normal. The cases published were reviewed by Usher (1935).