Transient embolic aorto-arteritis. Presentation of a patient.

The clinical and postmortem data from a 14-year-old girl who had transient embolic aorto-arteritis are presented. This disease was first described in 1974 by Peiris and Wickremasinghe in Hindu patients and is characterized by repetitive embolic occlusion of intra- and extracranial vessels of the brain, originating from focal thrombotic lesions in the aorta and its primary branches. The main histopathological lesion is located in the elastic tissue of the media in these arteries with alterations that vary from mild arteritis of the vasa vasorum to degeneration and destruction of the media which predisposes to aneurysm formation. This new entity apparently affects only adolescents and young adults and has no relation to atherosclerosis or other non-specific arteritides previously described. To our knowledge, this is the first such patient reported in America.