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Moyamoya disease and syndrome: a review

Moyamoya disease is a chronic occlusive cerebrovascular disease that is non-inflammatory and non-atherosclerotic. It is characterized by endothelial hyperplasia and fibrosis of the intracranial portion of the carotid artery and its proximal branches, leading to progressive stenosis and occlusion, of...

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Autores principales: Demartini Jr., Zeferino, Teixeira, Bernardo CA., Koppe, Gelson Luis, Gatto, Luana A. Maranha, Roman, Alex, Munhoz, Renato Puppi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Publicação do Colégio Brasileiro de Radiologia e Diagnóstico por Imagem 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8864689/
https://www.ncbi.nlm.nih.gov/pubmed/35210662
http://dx.doi.org/10.1590/0100-3984.2021.0010
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author Demartini Jr., Zeferino
Teixeira, Bernardo CA.
Koppe, Gelson Luis
Gatto, Luana A. Maranha
Roman, Alex
Munhoz, Renato Puppi
author_facet Demartini Jr., Zeferino
Teixeira, Bernardo CA.
Koppe, Gelson Luis
Gatto, Luana A. Maranha
Roman, Alex
Munhoz, Renato Puppi
author_sort Demartini Jr., Zeferino
collection PubMed
description Moyamoya disease is a chronic occlusive cerebrovascular disease that is non-inflammatory and non-atherosclerotic. It is characterized by endothelial hyperplasia and fibrosis of the intracranial portion of the carotid artery and its proximal branches, leading to progressive stenosis and occlusion, often clinically manifesting as ischemic or hemorrhagic stroke with high rates of morbidity and mortality. On cerebral angiography, the formation of collateral vessels has the appearance of a puff of smoke (moyamoya in Japanese), which became more conspicuous with the refinement of modern imaging techniques. When there is associated disease, it is known as moyamoya syndrome. Treatments are currently limited, although surgical revascularization may prevent ischemic events and preserve quality of life. In this review, we summarize recent advances in moyamoya disease, covering aspects of epidemiology, etiology, presentation, imaging, and treatment strategies.
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spelling pubmed-88646892022-02-23 Moyamoya disease and syndrome: a review Demartini Jr., Zeferino Teixeira, Bernardo CA. Koppe, Gelson Luis Gatto, Luana A. Maranha Roman, Alex Munhoz, Renato Puppi Radiol Bras Review Article Moyamoya disease is a chronic occlusive cerebrovascular disease that is non-inflammatory and non-atherosclerotic. It is characterized by endothelial hyperplasia and fibrosis of the intracranial portion of the carotid artery and its proximal branches, leading to progressive stenosis and occlusion, often clinically manifesting as ischemic or hemorrhagic stroke with high rates of morbidity and mortality. On cerebral angiography, the formation of collateral vessels has the appearance of a puff of smoke (moyamoya in Japanese), which became more conspicuous with the refinement of modern imaging techniques. When there is associated disease, it is known as moyamoya syndrome. Treatments are currently limited, although surgical revascularization may prevent ischemic events and preserve quality of life. In this review, we summarize recent advances in moyamoya disease, covering aspects of epidemiology, etiology, presentation, imaging, and treatment strategies. Publicação do Colégio Brasileiro de Radiologia e Diagnóstico por Imagem 2022 /pmc/articles/PMC8864689/ /pubmed/35210662 http://dx.doi.org/10.1590/0100-3984.2021.0010 Text en https://creativecommons.org/licenses/by/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Review Article
Demartini Jr., Zeferino
Teixeira, Bernardo CA.
Koppe, Gelson Luis
Gatto, Luana A. Maranha
Roman, Alex
Munhoz, Renato Puppi
Moyamoya disease and syndrome: a review
title Moyamoya disease and syndrome: a review
title_full Moyamoya disease and syndrome: a review
title_fullStr Moyamoya disease and syndrome: a review
title_full_unstemmed Moyamoya disease and syndrome: a review
title_short Moyamoya disease and syndrome: a review
title_sort moyamoya disease and syndrome: a review
topic Review Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8864689/
https://www.ncbi.nlm.nih.gov/pubmed/35210662
http://dx.doi.org/10.1590/0100-3984.2021.0010
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