Moyamoya disease is an uncommon cerebrovascular disease that is characterised by progressive stenosis of the terminal portion of the internal carotid artery and its main branches. 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.
Associated Clinical Trials
Surgical treatment of moyamoya disease in pediatric patients--comparison between the results of indirect and direct revascularization procedures
Long-term follow-up study after extracranial-intracranial bypass surgery for anterior circulation ischemia in childhood moyamoya disease
Possible roles of basic fibroblast growth factor in the pathogenesis of moyamoya disease: an immunohistochemical study
Mental outcome following encephaloduroarteriosynangiosis in children with moyamoya disease with the onset earlier than 5 years of age
Study of the posterior circulation in moyamoya disease. Part 2: Visual disturbances and surgical treatment
Clinical and cerebral angiographic evolutions of idiopathic progressive occlusive disease of the circle of Willis ("moyamoya" disease) in children
Temporary neurological deterioration caused by hyperperfusion after extracranial-intracranial bypass--case report and study of cerebral hemodynamics
Cerebral hemodynamics and "re-build-up" phenomenon on electroencephalogram in children with moyamoya disease
Cerebral blood flow in children with spontaneous occlusion of the circle of Willis (moyamoya disease): comparison with healthy children and evaluation of annual changes
Moyamoya disease: comparison of assessment with MR angiography and MR imaging versus conventional angiography
Cerebral circulation and oxygen metabolism in childhood moyamoya disease: a perioperative positron emission tomography study
The cerebrospinal fluid in patients with moyamoya disease (spontaneous occlusion of the circle of Willis) contains high level of basic fibroblast growth factor
Preoperative and postoperative evaluation of cerebral perfusion and vasodilatory capacity with 99mTc-HMPAO SPECT and acetazolamide in childhood Moyamoya disease
Regional cerebral hemodynamics during re-build-up phenomenon in childhood moyamoya disease. An analysis using 99mTc-HMPAO SPECT
Surgical therapy for adult moyamoya disease. Can surgical revascularization prevent the recurrence of intracerebral hemorrhage?
The relevance of hemodynamic factors to perioperative ischemic complications in childhood moyamoya disease
Cerebral hypoxia after hyperventilation causes "re-build-up" phenomenon and TIA in childhood moyamoya disease. A near-infrared spectroscopy study
A source of haemorrhage in adult patients with moyamoya disease: the significance of tributaries from the choroidal artery
Natural history of moyamoya disease: comparison of activity of daily living in surgery and non surgery groups
Superficial temporal artery to anterior cerebral artery direct anastomosis in patients with moyamoya disease
Posterior cerebral artery involvement in moyamoya disease: initial infarction and angle between PCA and basilar artery
A rare Asian founder polymorphism of Raptor may explain the high prevalence of Moyamoya disease among East Asians and its low prevalence among Caucasians.
P.R4810K, a polymorphism of RNF213, the susceptibility gene for moyamoya disease, is associated with blood pressure.
Reliability of near-infrared angiography and micro-Doppler sonography for evaluating microvascular anastomoses
Identification of RNF213 as a susceptibility gene for moyamoya disease and its possible role in vascular development
The contribution of imaging in diagnosis, preoperative assessment, and follow-up of moyamoya disease: a review
Prevention of secondary ischemic events by superficial temporal artery-middle cerebral artery bypass surgery after tuberculosis-induced vasculopathy in a 5-year-old child
Assessment of the difference in posterior circulation involvement between pediatric and adult patients with moyamoya disease
Prolonged and regionally progressive symptomatic cerebral hyperperfusion syndrome after superficial temporal artery-middle cerebral artery anastomosis in a patient with moyamoya disease
A case of unilateral moyamoya disease suffered from intracerebral hemorrhage due to the rupture of cerebral aneurysm, which appeared seven years later
Hemorrhage and risk of further hemorrhagic strokes following cerebral revascularization in Moyamoya disease: A review of the literature.
Selective neuropsychological impairments and related clinical factors in children with moyamoya disease of the transient ischemic attack type
Indocyanine green videoangiography for assessment of postoperative hyperperfusion in moyamoya disease
Haemodynamic analysis of vessel remodelling in STA-MCA bypass for Moyamoya disease and its impact on bypass patency
Involuntary movement in pediatric moyamoya disease patients: consideration of pathogenetic mechanism using neuroimaging studies
Headache attack followed by rapid disease progression in pediatric moyamoya disease--how should we manage it?
The roles of methylenetetrahydrofolate reductase 677C>T and 1298A>C polymorphisms in moyamoya disease patients
Intraoperative assessment of cortical perfusion by indocyanine green videoangiography in surgical revascularization for moyamoya disease
Outcome in adult patients with hemorrhagic moyamoya disease after combined extracranial-intracranial bypass
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