Fibromuscular dysplasia and multiple dissecting aneurysms of intracranial arteries a further cause of moyamoya syndrome

Abstract

A 16-year-old boy, who had sudden left-sided hemiplegia, died two weeks following onset of symptoms. A right carotid angiogram showed stenosis at the termination of the internal carotid artery. The middle cerebral artery had a beaded appearance and some of its branches were occluded. A basal “moyamoya” syndrome and transdural anastomoses were present. At autopsy, multiple intracranial dissecting aneurysms were found. Arteries of the body displayed fibromuscular dysplasia (FMD). The relevance of dysplasti c changes of intracranial arteries and the relationship to moyamoya syndrome are discussed. © 1976 American Heart Association, Inc.