Intracranial fibromuscular dysplasia. Report of two cases and review of literature.

Intracranial fibromuscular dysplasia (IFMD) is a rare angiopathy of unknown origin primarily affecting children and young women. We report here on two cases of IFMD; the patients, two females, aging 29 and 25 years, suffered from acute, complete stroke. In one of them emergency extra-intracranial by-pass was attempted without evidence of positive influence on the patient's outcome. In both patients angiography showed the pathognomonic sign of "string of bead" at the level of intracerebral arteries. Cases of IFMD previously reported in the literature are then reviewed and etiology, clinical presentation, pathology, diagnosis and possible treatment of this rare disease are discussed.