Cervicocranial fibromuscular dysplasia in Taiwanese ischemic stroke patients

Clinical research of cervicocranial fibromuscular dysplasia (FMD) is rare in Asian populations. Our study reviewed Taiwanese ischemic stroke patients with cervicocranial FMD and compared them with previous reports. Methods: Between 2000 and 2011, we collected 19 consecutive cervicocranial FMD patients who received demographic registration, a blood test for excluding vasculitis, and comprehensive angiography. Cerebral ultrasound, vascular images and clinical outcomes (Barthel index, modified Rankin scale, recurrent stroke, or death) were monitored during follow-up. Results: Of the 19 patients, 16 (84%) had carotid FMD, while 7 (37%) had vertebral FMD. Only 2 investigated patients (13%) had renal FMD and 1 (5%) had cerebral aneurysm. 14 (74%) presented acute arterial dissection. All patients received medical treatment and had neither recurrent stroke nor dissection during follow-up. In the literature review of 225 FMD patients, 3.6% had recurrent stroke during follow-up, and some reported surgical procedure or angioplasty could give a good clinical outcome in progressing ischemia irrelevant to the cause of stenosis. Conclusion: In Taiwanese cervicocranial FMD patients, arterial dissection was one of the most common clinical presentations. Most of our patients had isolated involvement of the cervicocranial artery and carried a favorable outcome under medical treatment.