[Fibromuscular dysplasia and its neurological manifestations]

The authors present the current data on the classification, epidemiology, etiology, neurological manifestations, prognosis, diagnosis, and treatment of patients with fibromuscular dysplasia (FMD). The review is based on the selection of publications by searching PubMed for keywords from the first sources until March 2019. FMD is a segmental non-atherosclerotic and non-inflammatory disease of large- and medium-caliber arteries leading to their stenosis. The disease occurs mostly in women (90%), and manifests itself in the 5th decade of life. In the cerebrovascular form of FMD, the extracranial internal carotid artery and the vertebral artery are usually affected. Diagnosis is based on the identification of alternation of narrowing and dilation of arteries using angiography (the string of beads sign (multifocal form)). Neurological manifestations include headache, tinnitus, and ischemic stroke, usually due to the dissection or stenosis, rarely, intracerebral or subarachnoid hemorrhages. The prognosis in most cases is favorable, relapses of strokes are rare. Treatment includes antiplatelet agents, if they are ineffective to prevent recurrence of ischemic stroke, endovascular treatment is carried out. Approaches to the treatment of intracranial aneurysms do not differ from those in patients without FMD.