A syndrome of arterial-occlusive retinopathy and encephalopathy

An analysis of two new cases and four previously reported cases produced evidence for a syndrome of arterial-occlusive retinopathy and encephalopathy. All six patients were women; they ranged in age from 21 to 40 years. The clinical features of this condition include multiple branch retinal arterial occlusions and encephalopathy in which behavioral and memory disturbances predominate early. Hearing loss is frequent. Except for cerebrospinal fluid pleocytosis and an increased cerebrospinal fluid protein level, there are few laboratory or radiographic abnormalities. The disease may be responsive to corticosteroid therapy. There are some similarities between this syndrome and systemic lupus erythematosus but it appears to be a distinct disease entity. A comparison of the retinal findings with those described in experimental allergic encephalitis suggests that this may be a virally induced immune-mediated disease. Although only four clearly documented examples of this syndrome have been reported, we suspect that cases may have been overlooked because of failure to recognize arterial branch occlusions in the peripheral retina.