{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,1]],"date-time":"2026-11-01T18:51:45Z","timestamp":1793559105071,"version":"4.5.0"},"reference-count":44,"publisher":"FapUNIFESP (SciELO)","issue":"1","license":[{"start":{"date-parts":[[2022,2,1]],"date-time":"2022-02-01T00:00:00Z","timestamp":1643673600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2022,2,1]],"date-time":"2022-02-01T00:00:00Z","timestamp":1643673600000},"content-version":"am","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2022,2,1]],"date-time":"2022-02-01T00:00:00Z","timestamp":1643673600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Radiol Bras"],"abstract":"<jats:p>Abstract 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.<\/jats:p>","DOI":"10.1590\/0100-3984.2021.0010","type":"journal-article","created":{"date-parts":[[2022,2,9]],"date-time":"2022-02-09T07:07:23Z","timestamp":1644390443000},"page":"31-37","source":"Crossref","is-referenced-by-count":52,"title":["Moyamoya disease and syndrome: a review"],"prefix":"10.1590","volume":"55","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0683-5418","authenticated-orcid":false,"given":"Zeferino","family":"Demartini Jr.","sequence":"first","affiliation":[{"name":"Universidade Federal do Paran\u00e1 (UFPR),  Brazil; Complexo Hospital Pequeno Pr\u00edncipe,  Brazil; Pontif\u00edcia Universidade Cat\u00f3lica do Paran\u00e1 (PUCPR),  Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4769-6562","authenticated-orcid":false,"given":"Bernardo CA.","family":"Teixeira","sequence":"additional","affiliation":[{"name":"Universidade Federal do Paran\u00e1 (UFPR),  Brazil; Complexo Hospital Pequeno Pr\u00edncipe,  Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3127-6881","authenticated-orcid":false,"given":"Gelson Luis","family":"Koppe","sequence":"additional","affiliation":[{"name":"Complexo Hospital Pequeno Pr\u00edncipe,  Brazil; Pontif\u00edcia Universidade Cat\u00f3lica do Paran\u00e1 (PUCPR),  Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1940-2689","authenticated-orcid":false,"given":"Luana A. Maranha","family":"Gatto","sequence":"additional","affiliation":[{"name":"Pontif\u00edcia Universidade Cat\u00f3lica do Paran\u00e1 (PUCPR),  Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0890-3688","authenticated-orcid":false,"given":"Alex","family":"Roman","sequence":"additional","affiliation":[{"name":"Cleveland Clinic,  United Arab Emirates"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4783-4067","authenticated-orcid":false,"given":"Renato Puppi","family":"Munhoz","sequence":"additional","affiliation":[{"name":"University of Toronto,  Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"530","published-online":{"date-parts":[[2022,2]]},"reference":[{"key":"ref1","first-page":"37","article-title":"Hypoplasia of the bilateral internal carotid arteries","volume":"9","author":"Takeuchi K","year":"1957","journal-title":"Brain Nerve"},{"key":"ref2","doi-asserted-by":"crossref","first-page":"288","DOI":"10.1001\/archneur.1969.00480090076012","article-title":"Cerebrovascular \u201cmoyamoya\u201d disease. Disease showing abnormal net-like vessels in base of brain","volume":"20","author":"Suzuki J","year":"1969","journal-title":"Arch Neurol"},{"key":"ref3","doi-asserted-by":"crossref","first-page":"245","DOI":"10.2176\/nmc.52.245","article-title":"Health Labour Sciences Research Grant for Research on Measures for Intractable Diseases. Guidelines for diagnosis and treatment of moyamoya disease (spontaneous occlusion of the circle of Willis)","volume":"52","year":"2012","journal-title":"Neurol Med Chir (Tokyo)"},{"key":"ref4","doi-asserted-by":"crossref","first-page":"1124","DOI":"10.3174\/ajnr.A3819","article-title":"Clinical and angiographic features and stroke types in adult moyamoya disease","volume":"35","author":"Jang DK","year":"2014","journal-title":"AJNR Am J Neuroradiol"},{"key":"ref5","doi-asserted-by":"crossref","first-page":"371","DOI":"10.1007\/s10143-018-0994-5","article-title":"Progress in moyamoya disease","volume":"43","author":"Shang S","year":"2020","journal-title":"Neurosurg Rev"},{"key":"ref6","doi-asserted-by":"crossref","first-page":"22","DOI":"10.1007\/s11940-018-0511-8","article-title":"Surgical treatment of adult moyamoya disease","volume":"20","author":"Lee SU","year":"2018","journal-title":"Curr Treat Options 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S","year":"2018","journal-title":"Radiology"},{"key":"ref33","doi-asserted-by":"crossref","first-page":"1782","DOI":"10.1161\/STROKEAHA.116.013320","article-title":"Added value of vessel wall magnetic resonance imaging in the differentiation of moyamoya vasculopathies in a non-Asian cohort","volume":"47","author":"Mossa-Basha M","year":"2016","journal-title":"Stroke"},{"key":"ref34","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1080\/01616412.1991.11739976","article-title":"EC-IC bypass for adult patients with moyamoya disease","volume":"13","author":"Kobayashi H","year":"1991","journal-title":"Neurol Res"},{"key":"ref35","doi-asserted-by":"crossref","first-page":"S134","DOI":"10.1016\/S0303-8467(97)00074-7","article-title":"Superficial temporal artery to anterior cerebral artery direct anastomosis in patients with moyamoya disease","volume":"99","author":"Iwama T","year":"1997","journal-title":"Clin Neurol 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