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The disease is associated with the development of dilated, fragile collateral vessels at the base of the brain, which are termed moyamoya vessels. The incidence of moyamoya disease is high in east Asia, and familial forms account for about 15% of patients with this disease. Moyamoya disease has several unique clinical features, which include two peaks of age distribution at 5 years and at about 40 years. Most paediatric patients have ischaemic attacks, whereas adult patients can have ischaemic attacks, intracranial bleeding, or both. Extracranial-intracranial arterial bypass, including anastomosis of the superficial temporal artery to the middle cerebral artery and indirect bypass, can help prevent further ischaemic attacks, although the beneficial effect on haemorrhagic stroke is still not clear. In this Review, we summarise the epidemiology, aetiology, clinical features, diagnosis, surgical treatment, and outcomes of moyamoya disease. Recent updates and future perspectives for moyamoya disease will also be discussed."}]}],"creator":[{"@id":"https://cir.nii.ac.jp/crid/1380866731651158528","@type":"Researcher","foaf:name":[{"@value":"Satoshi 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Houkin"}]}],"publication":{"publicationIdentifier":[{"@type":"PISSN","@value":"14744422"}],"prism:publicationName":[{"@value":"The Lancet Neurology"}],"dc:publisher":[{"@value":"Elsevier BV"}],"prism:publicationDate":"2008-11","prism:volume":"7","prism:number":"11","prism:startingPage":"1056","prism:endingPage":"1066"},"reviewed":"false","dc:rights":["https://www.elsevier.com/tdm/userlicense/1.0/"],"url":[{"@id":"https://api.elsevier.com/content/article/PII:S1474442208702400?httpAccept=text/xml"},{"@id":"https://api.elsevier.com/content/article/PII:S1474442208702400?httpAccept=text/plain"}],"createdAt":"2008-10-15","modifiedAt":"2023-05-20","foaf:topic":[{"@id":"https://cir.nii.ac.jp/all?q=Adult","dc:title":"Adult"},{"@id":"https://cir.nii.ac.jp/all?q=Diagnostic%20Imaging","dc:title":"Diagnostic Imaging"},{"@id":"https://cir.nii.ac.jp/all?q=Cerebral%20Revascularization","dc:title":"Cerebral Revascularization"},{"@id":"https://cir.nii.ac.jp/all?q=Brain","dc:title":"Brain"},{"@id":"https://cir.nii.ac.jp/all?q=Cerebral%20Arteries","dc:title":"Cerebral Arteries"},{"@id":"https://cir.nii.ac.jp/all?q=Brain%20Ischemia","dc:title":"Brain Ischemia"},{"@id":"https://cir.nii.ac.jp/all?q=Radiography","dc:title":"Radiography"},{"@id":"https://cir.nii.ac.jp/all?q=Ethnicity","dc:title":"Ethnicity"},{"@id":"https://cir.nii.ac.jp/all?q=Humans","dc:title":"Humans"},{"@id":"https://cir.nii.ac.jp/all?q=Age%20of%20Onset","dc:title":"Age of Onset"},{"@id":"https://cir.nii.ac.jp/all?q=Moyamoya%20Disease","dc:title":"Moyamoya Disease"},{"@id":"https://cir.nii.ac.jp/all?q=Child","dc:title":"Child"},{"@id":"https://cir.nii.ac.jp/all?q=Cerebral%20Hemorrhage","dc:title":"Cerebral Hemorrhage"}],"relatedProduct":[{"@id":"https://cir.nii.ac.jp/crid/1050001202066014336","@type":"Article","resourceType":"学術雑誌論文(journal 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Disease"}]},{"@id":"https://cir.nii.ac.jp/crid/1360002220576355200","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"Letter to the Editor: Posterior circulation in moyamoya"}]},{"@id":"https://cir.nii.ac.jp/crid/1360002220576828032","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"Pathophysiological consideration of medullary streaks on FLAIR imaging in pediatric moyamoya disease"}]},{"@id":"https://cir.nii.ac.jp/crid/1360004230877373056","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"Physiological Role of Mysterin/RNF213 in Zebrafish"}]},{"@id":"https://cir.nii.ac.jp/crid/1360004231270585216","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"Outer-diameter narrowing of the internal carotid and middle cerebral arteries in moyamoya disease detected on 3D constructive interference in steady-state MR image: is arterial constrictive remodeling a major pathogenesis?"}]},{"@id":"https://cir.nii.ac.jp/crid/1360004232328335744","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"Genetic Analysis of RNF213 c.14576G>A Variant in Nonatherosclerotic Quasi-Moyamoya Disease"}]},{"@id":"https://cir.nii.ac.jp/crid/1360004232555056000","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"Rare and Low-Frequency Variants in RNF213 Confer Susceptibility to Moyamoya Syndrome Associated with Hyperthyroidism"}]},{"@id":"https://cir.nii.ac.jp/crid/1360004233526493824","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"Moyamoya disease-associated protein 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with Endothelial Cell Transcriptome"}]},{"@id":"https://cir.nii.ac.jp/crid/1360017282466280832","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"Differentiation of Fibroblasts Into Myofibroblasts in the Arachnoid Membrane of Moyamoya Disease"}]},{"@id":"https://cir.nii.ac.jp/crid/1360021391871208704","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"“Transcallosal” periventricular anastomosis in moyamoya disease: the fourth periventricular anastomosis and a potential predictor of hemorrhage"}]},{"@id":"https://cir.nii.ac.jp/crid/1360022304972078592","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"Outcome prediction of pediatric moyamoya disease using midterm cerebral blood flow measured between staged 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