Susac's syndrome: beneficial effects of corticosteroid therapy in a Japanese case.

Susac's syndrome is a rare disorder characterized by the triad of microangiopathy of the brain and retina with hearing loss. More than 50 affected individuals have been reported worldwide, all Caucasians. We herein identify the first Japanese patient with Susac's syndrome. A 36-year-old man developed recurrent subacute encephalopathy, bilateral sensorineural hearing loss, and retinal arteriolar occlusions, caused by microangiopathy from a year previously. T2-weighted MRI showed multiple high-signal lesions predominantly in the periventricular white matter. During the exacerbated phase both high-dose intravenous methyl-prednisolone and oral prednisone therapy produced beneficial effects. He showed definite remission within 2 years from the disease onset.