Cerebrovascular fibromuscular dysplasia

Background: Cerebrovascular fibromuscular dysplasia (FMD), that is, involving the cervical or intracranial arteries, is frequently overlooked despite its association with several neurological manifestations. Objectives: To describe cerebrovascular FMD. Design: Systematic review. Data sources and methods: We searched PubMed, Embase, and Medline up to December 2024. We identified additional studies by handsearching the bibliographies of relevant studies. We included articles describing at least one aspect of epidemiology, manifestations, prognosis, and management of cerebrovascular FMD and preferentially focused on studies including ⩾15 patients. The quality of evidence was rated according to the Oxford Centre for Evidence-Based Medicine levels of evidence. Results: We included 119 studies. Cerebrovascular FMD predominantly affects women around 50 years of age and most frequently involves the cervical arteries. Cerebrovascular FMD is frequently asymptomatic and incidentally diagnosed. The most common presenting symptoms are headache (8%–52%) and pulsatile tinnitus (17%–23%). Patients with FMD have a higher prevalence of cervical artery dissection (7%–17%) and intracranial saccular aneurysm (7%) compared to the general population. The main complications of cerebrovascular FMD are ischemic stroke (7%–10%), transient ischemic attack (5%–12%), subarachnoid hemorrhage (2%–3%), and intracerebral hemorrhage (0.01%). Webs are the predominant form of FMD in Black populations. Consensus-based recommendations have been summarized in the first international consensus statement on the diagnosis and management of FMD. Imaging of all arteries from brain-to-pelvis is recommended to detect asymptomatic lesions. Antiplatelet therapy is reasonable for patients with cerebrovascular FMD to prevent thromboembolic complications. Endovascular treatment can be considered in patients with risk factors for rupture of an unruptured intracranial aneurysm and in those with recurrent ischemic events despite medical management. Conclusion: Existing cross-sectional studies have improved the understanding of the manifestations of cerebrovascular FMD. However, knowledge gaps remain regarding the genetics, pathophysiology, management, and prognosis of cerebrovascular FMD, which should be addressed in future research.