Cerebrovascular fibromuscular dysplasia: a report of 5 cases and literature review

Abstract

Objective To analyze the basal conditions of 5 patients with cerebrovascular fibromuscular dysplasia (FMD), and explore the clinical presentation, imaging features, therapies and outcomes of cerebrovascular FMD. Methods Five patients with cerebrovascular FMD, admitted to our hospital from January 2012 to April 2013, were chosen in our study; their medical history and imaging features were collected, and their clinical presentation, imaging features, therapies and outcomes were retrospectively analyzed when combining with literature review. Results Five patients all presented with stroke, including four having hemiplegia as the initial symptom, and one having headache with nausea and vomiting as the main performance. One was diagnosed as having subarachnoid hemorrhage by CT scan, and four had different infarction lesions in lobes and basal ganglia on MRI. Artery dissection was discovered in all patients by cerebral digital substraction angiography, including two with right internal carotid artery dissection, one with right vertebral artery dissection combined with aneurysm in anterior communicating artery associated withstring-of-beads appearance on C1 segment of right internal carotid artery and left vertebral artery, one with bilateral vertebral artery dissection, and the last one with bilateral internal carotid artery dissection and moyamoya disease-like vessels. No abnormality was discovered in two patients by renal artery angiography. The patient with anterior communicating artery aneurysm was treated with endovascular aneurysm embolization, having poor prognosis. In the remaining 4 patients with cerebral infarction, two were treated with carotid artery stenting, and the other 2 were only given antiplatelet therapy. No patient suffered cerebrovascular accidents during the 12-month follow-up. Conclusions Cerebrovascular FMD is a rare cause of young stroke, and patients with cerebrovascular FMD often manifest headache, neck bruit, carotid artery dissection and stroke. It is particularly important to make definitive diagnosis by performing cerebrovascular imaging examinations and give treatment accordingly. The long term outcome of FMD is not clear now. Key words: Fibromuscular dysplasia; Stroke; Artery dissection; Antiplatelet treatment