KEARNS-SAYRES SYNDROME

The paper describes Kearns-Sayre's syndrome, a rare hereditary neuromu scular disease, in a patient aged 17 years. The clinical picture of th e disease had a classical triade: external ophthalmoplegia, pigmentary retinopathy, and cardiac conduction disturbances. This triade was sup plemented with other polymorphous symptoms characteristic of the syndr ome, such as moderate myopathic syndrome, hemeralopia, physical infant ilism, hypogonadism, pyramidal syndrome. Bifascular block in the His-P urkinje system was accompanied by mitral prolapse. The problems of ear ly diagnosis of the syndrome and choice of adequate therapeutical meth ods are discussed.