| Summary: | Praveen Kesav,1 Divya Manesh Raj,2 Seby John3 1Department of Neurology, Neurological Institute, Cleveland Clinic Abu Dhabi, Abu Dhabi, United Arab Emirates; 2Research Institute, Cleveland Clinic Abu Dhabi, Abu Dhabi, United Arab Emirates; 3Department of Neurology and Neuro Interventional Surgery, Neurological Institute, Cleveland Clinic Abu Dhabi, Abu Dhabi, United Arab EmiratesCorrespondence: Seby John, Staff Physician, Vascular Neurology and Neuro interventional Surgery, Neurological Institute, (C-226), Cleveland Clinic Abu Dhabi, Abu Dhabi, United Arab Emirates, Tel +97125019000 Ext 65932, Email Johns5@clevelandclinicabudhabi.aeAbstract: Fibromuscular dysplasia (FMD) is a rare idiopathic, segmental, noninflammatory and nonatherosclerotic arteriopathy of medium-sized arteries. It is classically considered to be a disease of young and middle adulthood, with females more commonly affected than males. FMD is a systemic disease. Although historically considered to be rare, cerebrovascular FMD (C-FMD) has now been recognized to be as common as the renovascular counterpart. Extracranial carotid and vertebral arteries are the most commonly involved vascular territories in C-FMD with the clinical presentation determined by vessels affected. Common symptoms include headaches and pulsatile tinnitus, with transient ischemic attacks, ischemic stroke and subarachnoid or intracerebral hemorrhage constituting the more severe clinical manifestations. Cervical artery dissection involving carotids more often than vertebral arteries and intracranial aneurysms account for the cerebrovascular pathologies detected in C-FMD. Our understanding regarding C-FMD has been augmented in the recent past on account of dedicated C-FMD data from North American, European and other international FMD cohorts. In this review article, we provide an updated and comprehensive overview on epidemiology, clinical presentation, etiology, diagnosis and management of C-FMD.Keywords: fibromuscular dysplasia, stroke in young, dissection, tinnitus
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