Progressive stenoocclusive arteriopathy, spontaneous occlusion of circle of Willis
Definitions
Progressive narrowing of distal internal carotid artery (ICA) & proximal circle of Willis (COW) vessels → characteristic adjacent clusters of collateral flow appearing as puff of smoke on real-time angiography
Moyamoya disease: Primary (idiopathic) moyamoya
More common in Japan, Korea
Moyamoya arteriopathy (a.k.a. moyamoya syndrome or secondary moyamoya) occurs in association with other disorders or after radiation treatment
IMAGING
General Features
CT Findings
MR Findings
Ultrasonographic Findings
Angiographic Findings
Nuclear Medicine Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Reporting Tips
Selected References
Kaseka ML et al: Distinct clinical and radiographic phenotypes in pediatric patients with moyamoya. Pediatr Neurol. 120:18-26, 2021
Wang LX et al: Ivy sign in moyamoya disease: a comparative study of the FLAIR vascular hyperintensity sign against contrast-enhanced MRI. AJNR Am J Neuroradiol. 42(4):694-700, 2021
Fan AP et al: Identifying hypoperfusion in moyamoya disease with arterial spin labeling and an [15O]-water positron emission tomography/magnetic resonance imaging normative database. Stroke. 50(2):373-80, 2019
Lee S et al: Monitoring cerebral perfusion changes after revascularization in patients with moyamoya disease by using arterial spin-labeling MR imaging. Radiology. 288(2):565-72, 2018
Kim DY et al: Infarct pattern and collateral status in adult moyamoya disease: a multimodal magnetic resonance imaging study. Stroke. 48(1):111-6, 2017
Qiao PG et al: Clinical assessment of cerebral hemodynamics in moyamoya disease via multiple inversion time arterial spin labeling and dynamic susceptibility contrast-magnetic resonance imaging: A comparative study. J Neuroradiol. 44(4):273-80, 2017
Blauwblomme T et al: Cerebral blood flow improvement after indirect revascularization for pediatric moyamoya disease: a statistical analysis of arterial spin-labeling MRI. AJNR Am J Neuroradiol. 37(4):706-12, 2016
Kim JS: Moyamoya disease: epidemiology, clinical features, and diagnosis. J Stroke. 18(1):2-11, 2016
Takagi Y et al: Histopathological characteristics of distal middle cerebral artery in adult and pediatric patients with moyamoya disease. Neurol Med Chir (Tokyo). 15;56(6):345-9, 2016
Titsworth WL et al: National analysis of 2454 pediatric moyamoya admissions and the effect of hospital volume on outcomes. Stroke. 47(5):1303-11, 2016
Griessenauer CJ et al: Encephaloduroarteriosynangiosis and encephalomyoarteriosynangiosis for treatment of moyamoya syndrome in pediatric patients with sickle cell disease. J Neurosurg Pediatr. 16(1):64-73, 2015
Derdeyn CP: Direct bypass reduces the risk of recurrent hemorrhage in moyamoya syndrome, but effect on functional outcome is less certain. Stroke. 45(5):1245-6, 2014
Ryoo S et al: High-resolution magnetic resonance wall imaging findings of moyamoya disease. Stroke. 45(8):2457-60, 2014
Hishikawa T et al: Assessment of the difference in posterior circulation involvement between pediatric and adult patients with moyamoya disease. J Neurosurg. 119(4):961-5, 2013
Noguchi T et al: Arterial spin-labeling MR imaging in moyamoya disease compared with clinical assessments and other MR imaging findings. Eur J Radiol. 82(12):e840-7, 2013
Currie S et al: Childhood moyamoya disease and moyamoya syndrome: a pictorial review. Pediatr Neurol. 44(6):401-13, 2011
Mugikura S et al: Posterior circulation and high prevalence of ischemic stroke among young pediatric patients with Moyamoya disease: evidence of angiography-based differences by age at diagnosis. AJNR Am J Neuroradiol. 32(1):192-8, 2011
Ibrahimi DM et al: Moyamoya disease in children. Childs Nerv Syst. 26(10):1297-308, 2010
Kim SK et al: Pediatric moyamoya disease: an analysis of 410 consecutive cases. Ann Neurol. 68(1):92-101, 2010
Mori N et al: The leptomeningeal "ivy sign" on fluid-attenuated inversion recovery MR imaging in Moyamoya disease: a sign of decreased cerebral vascular reserve? AJNR Am J Neuroradiol. 30(5):930-5, 2009
Park TS: Moyamoya disease in children. Neurosurg Focus. 24(2):E16a; discussion E16a, 2008
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References
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KEY FACTS
Terminology
Imaging
Pathology
Clinical Issues
TERMINOLOGY
Synonyms
Progressive stenoocclusive arteriopathy, spontaneous occlusion of circle of Willis
Definitions
Progressive narrowing of distal internal carotid artery (ICA) & proximal circle of Willis (COW) vessels → characteristic adjacent clusters of collateral flow appearing as puff of smoke on real-time angiography
Moyamoya disease: Primary (idiopathic) moyamoya
More common in Japan, Korea
Moyamoya arteriopathy (a.k.a. moyamoya syndrome or secondary moyamoya) occurs in association with other disorders or after radiation treatment
IMAGING
General Features
CT Findings
MR Findings
Ultrasonographic Findings
Angiographic Findings
Nuclear Medicine Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Reporting Tips
Selected References
Kaseka ML et al: Distinct clinical and radiographic phenotypes in pediatric patients with moyamoya. Pediatr Neurol. 120:18-26, 2021
Wang LX et al: Ivy sign in moyamoya disease: a comparative study of the FLAIR vascular hyperintensity sign against contrast-enhanced MRI. AJNR Am J Neuroradiol. 42(4):694-700, 2021
Fan AP et al: Identifying hypoperfusion in moyamoya disease with arterial spin labeling and an [15O]-water positron emission tomography/magnetic resonance imaging normative database. Stroke. 50(2):373-80, 2019
Lee S et al: Monitoring cerebral perfusion changes after revascularization in patients with moyamoya disease by using arterial spin-labeling MR imaging. Radiology. 288(2):565-72, 2018
Kim DY et al: Infarct pattern and collateral status in adult moyamoya disease: a multimodal magnetic resonance imaging study. Stroke. 48(1):111-6, 2017
Qiao PG et al: Clinical assessment of cerebral hemodynamics in moyamoya disease via multiple inversion time arterial spin labeling and dynamic susceptibility contrast-magnetic resonance imaging: A comparative study. J Neuroradiol. 44(4):273-80, 2017
Blauwblomme T et al: Cerebral blood flow improvement after indirect revascularization for pediatric moyamoya disease: a statistical analysis of arterial spin-labeling MRI. AJNR Am J Neuroradiol. 37(4):706-12, 2016
Kim JS: Moyamoya disease: epidemiology, clinical features, and diagnosis. J Stroke. 18(1):2-11, 2016
Takagi Y et al: Histopathological characteristics of distal middle cerebral artery in adult and pediatric patients with moyamoya disease. Neurol Med Chir (Tokyo). 15;56(6):345-9, 2016
Titsworth WL et al: National analysis of 2454 pediatric moyamoya admissions and the effect of hospital volume on outcomes. Stroke. 47(5):1303-11, 2016
Griessenauer CJ et al: Encephaloduroarteriosynangiosis and encephalomyoarteriosynangiosis for treatment of moyamoya syndrome in pediatric patients with sickle cell disease. J Neurosurg Pediatr. 16(1):64-73, 2015
Derdeyn CP: Direct bypass reduces the risk of recurrent hemorrhage in moyamoya syndrome, but effect on functional outcome is less certain. Stroke. 45(5):1245-6, 2014
Ryoo S et al: High-resolution magnetic resonance wall imaging findings of moyamoya disease. Stroke. 45(8):2457-60, 2014
Hishikawa T et al: Assessment of the difference in posterior circulation involvement between pediatric and adult patients with moyamoya disease. J Neurosurg. 119(4):961-5, 2013
Noguchi T et al: Arterial spin-labeling MR imaging in moyamoya disease compared with clinical assessments and other MR imaging findings. Eur J Radiol. 82(12):e840-7, 2013
Currie S et al: Childhood moyamoya disease and moyamoya syndrome: a pictorial review. Pediatr Neurol. 44(6):401-13, 2011
Mugikura S et al: Posterior circulation and high prevalence of ischemic stroke among young pediatric patients with Moyamoya disease: evidence of angiography-based differences by age at diagnosis. AJNR Am J Neuroradiol. 32(1):192-8, 2011
Ibrahimi DM et al: Moyamoya disease in children. Childs Nerv Syst. 26(10):1297-308, 2010
Kim SK et al: Pediatric moyamoya disease: an analysis of 410 consecutive cases. Ann Neurol. 68(1):92-101, 2010
Mori N et al: The leptomeningeal "ivy sign" on fluid-attenuated inversion recovery MR imaging in Moyamoya disease: a sign of decreased cerebral vascular reserve? AJNR Am J Neuroradiol. 30(5):930-5, 2009
Park TS: Moyamoya disease in children. Neurosurg Focus. 24(2):E16a; discussion E16a, 2008
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