Fibromuscular dysplasia of cervical and intracranial arteries.
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.
Touzé
Emmanuel
E
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
Oppenheim
Catherine
C
Trystram
Denis
D
Nokam
Ghislain
G
Pasquini
Marta
M
Alamowitch
Sonia
S
Hervé
Dominique
D
Garnier
Pierre
P
Mousseaux
Elie
E
Plouin
Pierre-François
PF
D002545:Brain Ischemia / Q000453:epidemiology / Q000473:pathology; D002533:Cerebral Angiography; D002536:Cerebral Arteries / Q000473:pathology*; D002561:Cerebrovascular Disorders / Q000145:classification / Q000453:epidemiology / Q000473:pathology*; D005260:Female; D005352:Fibromuscular Dysplasia / Q000145:classification / Q000453:epidemiology / Q000473:pathology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D014711:Vertebral Artery / Q000473:pathology*