Because the most disabling manifestation of the rare disease complex under consideration is ocular, it is not surprising that the syndrome has been discussed chiefly in the ophthalmologic literature. Still, the existence of the syndrome, and certain aspects of it which have not always been widely recognized, demonstrate the relevance of systemic and ocular disease. The repeated occurrence of the disease pattern should interest the nosologist, who still must hesitate to designate it as a disease entity, when most or all of the same clinical picture may follow severe emotional trauma, expansion of an ependymoma of the third ventricle and, frequently, transient encephalitis. Certainly, the subject is of interest to the internist, the dermatologist and the neurologist. In this paper the historical background of the syndrome is briefly reviewed. A general description of the disease picture is presented, including the fact that the prodrome may be essentially either an encephalitis