Cerebrovascular Fibromuscular Dysplasia – A Practical Review

Praveen Kesav,1 Divya Manesh Raj,2 Seby John3 1Department of Neurology, Neurological Institute, Cleveland Clinic Abu Dhabi, Abu Dhabi, United Arab Emirates; 2Research Institute, Cleveland Clinic Abu Dhabi, Abu Dhabi, United Arab Emirates; 3Department of Neurology and Neuro Interventional Surgery, Ne...

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Main Authors: Kesav P, Manesh Raj D, John S
Format: Article
Language:English
Published: Dove Medical Press 2023-08-01
Series:Vascular Health and Risk Management
Subjects:
Online Access:https://www.dovepress.com/cerebrovascular-fibromuscular-dysplasia--a-practical-review-peer-reviewed-fulltext-article-VHRM
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author Kesav P
Manesh Raj D
John S
author_facet Kesav P
Manesh Raj D
John S
author_sort Kesav P
collection DOAJ
description Praveen Kesav,1 Divya Manesh Raj,2 Seby John3 1Department of Neurology, Neurological Institute, Cleveland Clinic Abu Dhabi, Abu Dhabi, United Arab Emirates; 2Research Institute, Cleveland Clinic Abu Dhabi, Abu Dhabi, United Arab Emirates; 3Department of Neurology and Neuro Interventional Surgery, Neurological Institute, Cleveland Clinic Abu Dhabi, Abu Dhabi, United Arab EmiratesCorrespondence: Seby John, Staff Physician, Vascular Neurology and Neuro interventional Surgery, Neurological Institute, (C-226), Cleveland Clinic Abu Dhabi, Abu Dhabi, United Arab Emirates, Tel +97125019000 Ext 65932, Email Johns5@clevelandclinicabudhabi.aeAbstract: Fibromuscular dysplasia (FMD) is a rare idiopathic, segmental, noninflammatory and nonatherosclerotic arteriopathy of medium-sized arteries. It is classically considered to be a disease of young and middle adulthood, with females more commonly affected than males. FMD is a systemic disease. Although historically considered to be rare, cerebrovascular FMD (C-FMD) has now been recognized to be as common as the renovascular counterpart. Extracranial carotid and vertebral arteries are the most commonly involved vascular territories in C-FMD with the clinical presentation determined by vessels affected. Common symptoms include headaches and pulsatile tinnitus, with transient ischemic attacks, ischemic stroke and subarachnoid or intracerebral hemorrhage constituting the more severe clinical manifestations. Cervical artery dissection involving carotids more often than vertebral arteries and intracranial aneurysms account for the cerebrovascular pathologies detected in C-FMD. Our understanding regarding C-FMD has been augmented in the recent past on account of dedicated C-FMD data from North American, European and other international FMD cohorts. In this review article, we provide an updated and comprehensive overview on epidemiology, clinical presentation, etiology, diagnosis and management of C-FMD.Keywords: fibromuscular dysplasia, stroke in young, dissection, tinnitus
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spelling doaj.art-5e84497de0924aaeb493b42d236306412023-08-29T19:31:00ZengDove Medical PressVascular Health and Risk Management1178-20482023-08-01Volume 1954355686237Cerebrovascular Fibromuscular Dysplasia – A Practical ReviewKesav PManesh Raj DJohn SPraveen Kesav,1 Divya Manesh Raj,2 Seby John3 1Department of Neurology, Neurological Institute, Cleveland Clinic Abu Dhabi, Abu Dhabi, United Arab Emirates; 2Research Institute, Cleveland Clinic Abu Dhabi, Abu Dhabi, United Arab Emirates; 3Department of Neurology and Neuro Interventional Surgery, Neurological Institute, Cleveland Clinic Abu Dhabi, Abu Dhabi, United Arab EmiratesCorrespondence: Seby John, Staff Physician, Vascular Neurology and Neuro interventional Surgery, Neurological Institute, (C-226), Cleveland Clinic Abu Dhabi, Abu Dhabi, United Arab Emirates, Tel +97125019000 Ext 65932, Email Johns5@clevelandclinicabudhabi.aeAbstract: Fibromuscular dysplasia (FMD) is a rare idiopathic, segmental, noninflammatory and nonatherosclerotic arteriopathy of medium-sized arteries. It is classically considered to be a disease of young and middle adulthood, with females more commonly affected than males. FMD is a systemic disease. Although historically considered to be rare, cerebrovascular FMD (C-FMD) has now been recognized to be as common as the renovascular counterpart. Extracranial carotid and vertebral arteries are the most commonly involved vascular territories in C-FMD with the clinical presentation determined by vessels affected. Common symptoms include headaches and pulsatile tinnitus, with transient ischemic attacks, ischemic stroke and subarachnoid or intracerebral hemorrhage constituting the more severe clinical manifestations. Cervical artery dissection involving carotids more often than vertebral arteries and intracranial aneurysms account for the cerebrovascular pathologies detected in C-FMD. Our understanding regarding C-FMD has been augmented in the recent past on account of dedicated C-FMD data from North American, European and other international FMD cohorts. In this review article, we provide an updated and comprehensive overview on epidemiology, clinical presentation, etiology, diagnosis and management of C-FMD.Keywords: fibromuscular dysplasia, stroke in young, dissection, tinnitushttps://www.dovepress.com/cerebrovascular-fibromuscular-dysplasia--a-practical-review-peer-reviewed-fulltext-article-VHRMfibromuscular dysplasiastroke in youngdissectiontinnitus
spellingShingle Kesav P
Manesh Raj D
John S
Cerebrovascular Fibromuscular Dysplasia – A Practical Review
Vascular Health and Risk Management
fibromuscular dysplasia
stroke in young
dissection
tinnitus
title Cerebrovascular Fibromuscular Dysplasia – A Practical Review
title_full Cerebrovascular Fibromuscular Dysplasia – A Practical Review
title_fullStr Cerebrovascular Fibromuscular Dysplasia – A Practical Review
title_full_unstemmed Cerebrovascular Fibromuscular Dysplasia – A Practical Review
title_short Cerebrovascular Fibromuscular Dysplasia – A Practical Review
title_sort cerebrovascular fibromuscular dysplasia ndash a practical review
topic fibromuscular dysplasia
stroke in young
dissection
tinnitus
url https://www.dovepress.com/cerebrovascular-fibromuscular-dysplasia--a-practical-review-peer-reviewed-fulltext-article-VHRM
work_keys_str_mv AT kesavp cerebrovascularfibromusculardysplasiandashapracticalreview
AT maneshrajd cerebrovascularfibromusculardysplasiandashapracticalreview
AT johns cerebrovascularfibromusculardysplasiandashapracticalreview