Clinical associations of headaches among patients with fibromuscular dysplasia: A Report from the US Registry for Fibromuscular Dysplasia

Abstract

Fibromuscular dysplasia (FMD) is a noninflammatory, nonatherosclerotic arterial disease that can affect any artery but most commonly affects the renal, carotid, and vertebral arteries, predisposing to stenosis, aneurysm, dissection, and/or tortuosity. 1-3 While headache is a common symptom reported by patients with FMD, little is known about this relationship. 4,5 The etiology of FMD is not known, nor is it understood why such a high percentage of patients with FMD experience headaches. 6 In addition to FMD, patients with associated vascular abnormalities, such as intracranial aneurysm and cervical carotid and vertebral artery dissection, also commonly experience headache. 5,7,8 The aim of this study was to report the frequency and characteristics of headache in a cohort of patients enrolled in the United States Registry for FMD and to identify clinical factors associated with the presence of this symptom. From 2009 through 2016, 1433 adult patients 18 years of age and older were enrolled in the Registry. Eligibility for enrollment was determined by site investigators based on identifying the characteristic appearance of multifocal or focal FMD from vascular imaging in at least one vascular bed. 2,5 Eligible subjects provided written informed consent. Demographic and clinical data, family history, headache-related variables, presenting signs and symptoms at the time of FMD diagnosis, and medications taken at the time of enrollment were recorded. An assessment of headache at initial Registry enrollment included query for presence of headache, type of headache (migraine or other), frequency of headache, whether headache was associated with menses, and whether headache(s) required suppressive medication. The Institutional Review Board (IRB) at the University of Michigan, the Data Coordinating Center for the US Registry of FMD, approved this study (HUM00022250). In addition, all participating sites in the Registry obtained individual institutional IRB approval. Patients were categorized into two groups: those with and those without reported headaches at the time of Registry enrollment. Univariate analysis was conducted by comparing demographics, past medical history, symptoms and signs at time of FMD diagnosis, arterial bed involvement, and family history between the two groups using Student's

The Relationship Among Pain Location, Complications, and Quality of Life in Individuals With Fibromuscular Dysplasia

“…We found that the majority of respondents were much more likely to have migraine headaches (n = 100; 61.7%) compared with women in the general population (25%) Gornik, et al, 2019 ). This is consistent with data from the United States Registry for FMD which reported symptoms of headache among 67.5% of patients with FMD, including more than one-quarter of headache sufferers reporting daily headache ( Wells et al, 2020 ). Our sample also reported a much higher likelihood of having a history of stroke (11.7%) compared with the general population (3.1%) ( CDC, 2016 ).…”

“…Although FMD is generally not considered a terminal disease, the morbid complications may be life-altering. Symptoms of FMD can include neck pain, claudication, migraine headache, pulsatile tinnitus (swooshing sound in the ears), and postprandial abdominal pain ( Gornik et al, 2019 ;Wells et al, 2020 ). Disease complications such as stroke have a known relationship to perceived health in other populations ( Mavaddat et al, 2016 ) and was reported in 10.1% of individuals with FMD in the United States Registry for FMD ( Gornik et al, 2019 ).…”

Current progress in clinical, molecular, and genetic aspects of adult fibromuscular dysplasia

“…New centres have been incrementally added across the United States to increase geographical, racial/ethnic, and clinical diversity of the Registry population. Key reports of the Registry published since 2012 include: description of the most common clinical manifestations of FMD, including hypertension, headache 6 , and pulsatile tinnitus 5 ; the finding of cerebrovascular (carotid, vertebral) involvement of FMD being as common as renal involvement 5 ; a report of a high prevalence of aneurysm or dissection among patients with FMD 7,8 ; a description of a higher prevalence of aneurysms and dissections in men with FMD 9 , and of a more benign vascular phenotype in patients diagnosed ≥65 years old 10 .…”

“…The most frequent presenting symptoms of neurological FMD are headaches and pulsatile tinnitus 5,98,99 . Headaches are reported by 70% of all FMD patients with headaches types being mostly migraine type and tension type 5,6,98 . The underlying pathophysiology of headaches in FMD patients remains unclear and possible mechanisms include alterations in cerebrovascular flow (e.g.…”

Fibromuscular dysplasia: its various phenotypes in everyday practice in 2021

“…Headache (especially migraine), pulsatile tinnitus, dizziness or light-headedness are nonspecific symptoms that may be the consequence of cerebrovascular FMD. However, patients can be asymptomatic or may present with focal neurological deficits, such as transient ischemic attack or stroke [3,4,9,[52][53][54][55][56]. In case of cervical artery dissection (ceAD), these focal neurological symptoms may be accompanied by neck, face or head pain [6].…”