Fibromuscular dysplasia of cervical and intracranial arteries. 2010

Emmanuel Touzé, and Catherine Oppenheim, and Denis Trystram, and Ghislain Nokam, and Marta Pasquini, and Sonia Alamowitch, and Dominique Hervé, and Pierre Garnier, and Elie Mousseaux, and Pierre-François Plouin
Department of Neurology, Université Paris Descartes, Pôle neuroscience, services de Neurologie et de Neuroradiologie, Hôpital Sainte-Anne, Paris, France. e.touze@ch-sainte-anne.fr

Fibromuscular dysplasia is an uncommon, segmental, nonatherosclerotic arterial disease of unknown aetiology. The disease primarily affects women and involves intermediate-sized arteries in many areas of the body, including cervical and intracranial arteries. Although often asymptomatic, fibromuscular dysplasia can also be associated with spontaneous dissection, severe stenosis that compromises the distal circulation, or intracranial aneurysm, and is therefore responsible for cerebral ischaemia or subarachnoid haemorrhage. Fibromuscular dysplasia affects middle and distal portions of the internal carotid and vertebral arteries, and occasionally, intracranial arteries. Several pathological and angiographic patterns exist. The most frequent pathological type is medial fibromuscular dysplasia, which is associated with the 'string of beads' angiographic pattern. Unifocal lesions are less common and can be associated with several pathological subtypes. The pathophysiology of the disease is widely unknown. Fibromuscular dysplasia may in fact result from various causes and reflect a non-specific response to different insults. The poor knowledge of the natural history and the lack of randomised trials that compared the different treatment options do not allow any satisfactory judgement to be made regarding the need for or the efficacy of any treatment.

UI MeSH Term Description Entries
D008279 Magnetic Resonance Imaging Non-invasive method of demonstrating internal anatomy based on the principle that atomic nuclei in a strong magnetic field absorb pulses of radiofrequency energy and emit them as radiowaves which can be reconstructed into computerized images. The concept includes proton spin tomographic techniques. Chemical Shift Imaging,MR Tomography,MRI Scans,MRI, Functional,Magnetic Resonance Image,Magnetic Resonance Imaging, Functional,Magnetization Transfer Contrast Imaging,NMR Imaging,NMR Tomography,Tomography, NMR,Tomography, Proton Spin,fMRI,Functional Magnetic Resonance Imaging,Imaging, Chemical Shift,Proton Spin Tomography,Spin Echo Imaging,Steady-State Free Precession MRI,Tomography, MR,Zeugmatography,Chemical Shift Imagings,Echo Imaging, Spin,Echo Imagings, Spin,Functional MRI,Functional MRIs,Image, Magnetic Resonance,Imaging, Magnetic Resonance,Imaging, NMR,Imaging, Spin Echo,Imagings, Chemical Shift,Imagings, Spin Echo,MRI Scan,MRIs, Functional,Magnetic Resonance Images,Resonance Image, Magnetic,Scan, MRI,Scans, MRI,Shift Imaging, Chemical,Shift Imagings, Chemical,Spin Echo Imagings,Steady State Free Precession MRI
D008297 Male Males
D002533 Cerebral Angiography Radiography of the vascular system of the brain after injection of a contrast medium. Angiography, Cerebral,Angiographies, Cerebral,Cerebral Angiographies
D002536 Cerebral Arteries The arterial blood vessels supplying the CEREBRUM. Arteries, Cerebral,Artery, Cerebral,Cerebral Artery
D002545 Brain Ischemia Localized reduction of blood flow to brain tissue due to arterial obstruction or systemic hypoperfusion. This frequently occurs in conjunction with brain hypoxia (HYPOXIA, BRAIN). Prolonged ischemia is associated with BRAIN INFARCTION. Cerebral Ischemia,Ischemic Encephalopathy,Encephalopathy, Ischemic,Ischemia, Cerebral,Brain Ischemias,Cerebral Ischemias,Ischemia, Brain,Ischemias, Cerebral,Ischemic Encephalopathies
D002561 Cerebrovascular Disorders A spectrum of pathological conditions of impaired blood flow in the brain. They can involve vessels (ARTERIES or VEINS) in the CEREBRUM, the CEREBELLUM, and the BRAIN STEM. Major categories include INTRACRANIAL ARTERIOVENOUS MALFORMATIONS; BRAIN ISCHEMIA; CEREBRAL HEMORRHAGE; and others. Brain Vascular Disorders,Intracranial Vascular Disorders,Vascular Diseases, Intracranial,Cerebrovascular Diseases,Cerebrovascular Insufficiency,Cerebrovascular Occlusion,Brain Vascular Disorder,Cerebrovascular Disease,Cerebrovascular Disorder,Cerebrovascular Insufficiencies,Cerebrovascular Occlusions,Disease, Cerebrovascular,Diseases, Cerebrovascular,Insufficiencies, Cerebrovascular,Insufficiency, Cerebrovascular,Intracranial Vascular Disease,Intracranial Vascular Diseases,Intracranial Vascular Disorder,Occlusion, Cerebrovascular,Occlusions, Cerebrovascular,Vascular Disease, Intracranial,Vascular Disorder, Brain,Vascular Disorder, Intracranial,Vascular Disorders, Brain,Vascular Disorders, Intracranial
D005260 Female Females
D005352 Fibromuscular Dysplasia An idiopathic, segmental, nonatheromatous disease of the musculature of arterial walls, leading to STENOSIS of small and medium-sized arteries. There is true proliferation of SMOOTH MUSCLE CELLS and fibrous tissue. Fibromuscular dysplasia lesions are smooth stenosis and occur most often in the renal and carotid arteries. They may also occur in other peripheral arteries of the extremity. Fibromuscular Dysplasia of Arteries,Arteries Fibromuscular Dysplasia,Arteries Fibromuscular Dysplasias,Dysplasia, Fibromuscular,Fibromuscular Dysplasias
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D014711 Vertebral Artery The first branch of the SUBCLAVIAN ARTERY with distribution to muscles of the NECK; VERTEBRAE; SPINAL CORD; CEREBELLUM; and interior of the CEREBRUM. Vertebral Arteries,Arteries, Vertebral,Artery, Vertebral

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