Fibromuscular Dysplasia: An Update for the Headache Clinician

Abstract

This review summarizes the most recent literature regarding FMD, including epidemiology, clinical manifestations, imaging practices, and treatment. Special attention will be paid to the association of headaches and FMD. Correct diagnosis, optimal medical management, and appropriate referral for vascular intervention are vital elements of the treatment of patients with FMD. There is a great need for more clinical research regarding the epidemiology, pathophysiology, and optimal treatment of headache in the FMD patient population.

It could be in the kidneys: fibromuscular dysplasia and the association with headaches and mood disorders

“…The prevalence of anxiety and depression is nearly double in patients with FMD when compared with that of the general population,3 and more than half of the patients with FMD experience headaches, usually migraine headaches 4. The possibility of FMD should be suspected in young patients who present for evaluation of chronic migraine headache, particularly in the setting of pulsatile tinnitus, early-onset HTN, or cervical or abdominal bruit 5…”

Hemicrania continua in carotid artery dissection – symptomatic cases or linked pathophysiology?

“…FMD used to be considered a rare disorder, but imaging studies suggest incidental findings of FMD close to 6% in the renal and up to 3.2% in brain-supplying arteries, with many subjects being asymptomatic (12). The majority of headaches in FMD are rather of the recurrent type (13); in the context of carotid artery dissection associated with FMD, it may be necessary to separate new onset acute headaches from patients with longer histories of recurrent headaches. Given the observation that most patients with FMD are female (91%) and middle-aged (mean 52 years) (12), it may be rational to screen middle-aged women with cervical artery dissection for other manifestations of FMD, in addition to imaging of brain supplying arteries.…”

Co-existence of Three Types of Fibromuscular Dysplasia associated with True Posterior Communicating Artery Aneurysm

“…However, few dysplasias are currently examined histologically, so an angiographic diagnostic classification has been suggested based on differentiation between unifocal and multifocal appearance. Angiographically, FMD has also been recognized as three main types: (1) multifocal ('string-ofbeads' appearance), (2) unifocal (solitary stenosis < 1 cm in length) and (3) tubular (stenosis at least 1 cm in length) [3,5]. Intimal fibroplasia is characterized by a focal, band-like, concentric stenosis (unifocal with solitary stenosis < 1 cm in length) (Figure 1d and Figure 1e), the lesion of medial fibroplasia resembles a "string of beads" appearance (multifocal) (Figure 1f and Figure 2c), adventitial fibroplasia appears as tubular stenosis (stenosis at least 1 cm in length) which is extremely rare type of all FMD lesions (Figure 1b and Figure 1c), seen in less than 1% of arterial stenosis [1].…”

“…Importantly, when stenosis occurs, occlusion is rarely complete. The diagnosis of FMD can be established by histopathology or angiography [5,6].…”