25/39 7/6/23, 12:05 PM Moyamoya disease and moyamoya syndrome: Etiology, clinical features, and diagnosis - UpToDate 168. Horie N, Morikawa M, Nozaki A, et al. "Brush Sign" on susceptibility-weighted MR imaging indicates the severity of moyamoya disease. AJNR Am J Neuroradiol 2011; 32:1697. 169. Yu X, Yuan L, Jackson A, et al. Prominence of Medullary Veins on Susceptibility-Weighted Images Provides Prognostic Information in Patients with Subacute Stroke. AJNR Am J Neuroradiol 2016; 37:423. 170. Muraoka S, Araki Y, Taoka T, et al. Prediction of Intracranial Arterial Stenosis Progression in Patients with Moyamoya Vasculopathy: Contrast-Enhanced High-Resolution Magnetic Resonance Vessel Wall Imaging. World Neurosurg 2018; 116:e1114. 171. Ryoo S, Cha J, Kim SJ, et al. High-resolution magnetic resonance wall imaging findings of Moyamoya disease. Stroke 2014; 45:2457. 172. Kodama N, Aoki Y, Hiraga H, et al. Electroencephalographic findings in children with moyamoya disease. 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Hasuo K, Mihara F, Matsushima T. MRI and MR angiography in moyamoya disease. J Magn Reson Imaging 1998; 8:762. 181. Yamada I, Nakagawa T, Matsushima Y, Shibuya H. High-resolution turbo magnetic resonance angiography for diagnosis of Moyamoya disease. Stroke 2001; 32:1825. 182. Suzuki J, Kodama N. Moyamoya disease a review. Stroke 1983; 14:104. 183. Suzuki J, Takaku A. Cerebrovascular "moyamoya" disease. Disease showing abnormal net- like vessels in base of brain. Arch Neurol 1969; 20:288. Topic 1131 Version 41.0 https://www.uptodate.com/contents/moyamoya-disease-and-moyamoya-syndrome-etiology-clinical-features-and-diagnosis/print 26/39 7/6/23, 12:05 PM Moyamoya disease and moyamoya syndrome: Etiology, clinical features, and diagnosis - UpToDate GRAPHICS 40-year-old with moyamoya disease Digital subtraction angiogram shows stenosis of right supraclinoid carotid, proximal middle cerebral artery a artery (oval), and lenticulostriate moyamoya collateral vessels (arrows). Courtesy of Glenn A Tung, MD, FACR. Graphic 129102 Version 2.0 https://www.uptodate.com/contents/moyamoya-disease-and-moyamoya-syndrome-etiology-clinical-features-and-diagnosis/print 27/39 7/6/23, 12:05 PM Moyamoya disease and moyamoya syndrome: Etiology, clinical features, and diagnosis - UpToDate Morning glory disc anomaly The disc is large and there is a central, white tuft of glial tissue. The retinal vessels proceed radially from the disc. Courtesy of Karl C Golnik, MD. Graphic 55325 Version 2.0 https://www.uptodate.com/contents/moyamoya-disease-and-moyamoya-syndrome-etiology-clinical-features-and-diagnosis/print 28/39 7/6/23, 12:05 PM Moyamoya disease and moyamoya syndrome: Etiology, clinical features, and diagnosis - UpToDate 9-year-old with unilateral moyamoya disease Digital subtraction angiogram shows stenosis of right supraclinoid and proximal middle cerebral arteries (cir lenticulostriate moyamoya collateral vessels (arrows). Courtesy of Glenn A Tung, MD, FACR. Graphic 129103 Version 2.0 https://www.uptodate.com/contents/moyamoya-disease-and-moyamoya-syndrome-etiology-clinical-features-and-diagnosis/print 29/39 7/6/23, 12:05 PM Moyamoya disease and moyamoya syndrome: Etiology, clinical features, and diagnosis - UpToDate Aneurysm in moyamoya disease Cerebral angiogram showing aneurysm (arrow) in patient with moyamoya disease. Courtesy of Nijasri Suwanwela, MD. Graphic 64850 Version 4.0 https://www.uptodate.com/contents/moyamoya-disease-and-moyamoya-syndrome-etiology-clinical-features-and-diagnosis/print 30/39 7/6/23, 12:05 PM Moyamoya disease and moyamoya syndrome: Etiology, clinical features, and diagnosis - UpToDate 9-year-old male with moyamoya disease who presented with repeated episodes of transient left hemiparesis (A) Noncontrast head CT scan shows low-density area of infarction in the right basal ganglia (arrow). (B) MRI FLAIR image depicting a high-signal-intensity area in the right basal ganglia and multiple small hyperintense areas in both basal ganglia consistent with infarction. (C, D) Selective intra-arterial DSA (anteroposterior projection) shows severe stenoses of the distal right and left internal carotid arteries. Abnormal network of blood vessels (puff of smoke or moyamoya vessels) in the vicinity of the stenotic areas were noted (arrows). CT: computed tomography; MRI: magnetic resonance imaging; FLAIR: fluid-attenuated inversion recovery; DSA: digital subtraction angiogram. Courtesy of Nijasri Suwanwela, MD. Graphic 74421 Version 8.0 https://www.uptodate.com/contents/moyamoya-disease-and-moyamoya-syndrome-etiology-clinical-features-and-diagnosis/print 31/39 7/6/23, 12:05 PM Moyamoya disease and moyamoya syndrome: Etiology, clinical features, and diagnosis - UpToDate 54-year-old with moyamoya disease T2-weighted sequence on magnetic resonance imaging shows lenticulostriate moyamoya collateral vessels on right (arrow). Courtesy of Glenn A Tung, MD, FACR. Graphic 129104 Version 2.0 https://www.uptodate.com/contents/moyamoya-disease-and-moyamoya-syndrome-etiology-clinical-features-and-diagnosis/print 32/39 7/6/23, 12:05 PM Moyamoya disease and moyamoya syndrome: Etiology, clinical features, and diagnosis - UpToDate 40-year-old with moyamoya disease "ivy sign" Fluid-attenuated inversion recovery sequence magnetic resonance imaging shows linear and curvilinear hyperintensities in the cerebral sulci consistent with "ivy sign" (arrows). Courtesy of Glenn A Tung, MD, FACR. Graphic 129105 Version 2.0 https://www.uptodate.com/contents/moyamoya-disease-and-moyamoya-syndrome-etiology-clinical-features-and-diagnosis/print 33/39 7/6/23, 12:05 PM Moyamoya disease and moyamoya syndrome: Etiology, clinical features, and diagnosis - UpToDate Moyamoya disease "brush sign" The conspicuity of multiple deep medullary veins on susceptibility- weighted imaging sequence (oval) from reduced oxygen supply relative to tissue demand resulting in an increase in the concentration of deoxyhemoglobin in venous blood. Republished with permission of the American Society of Neuroradiology, from: Horie N, Morikawa M, Nozaki A, et al. "Brush sign" on susceptibility-weighted MR imaging indicates the severity of moyamoya disease. Am J Neurorad 2011; 32:1697; permission conveyed through Copyright Clearance Center, Inc. Copyright 2011. https://www.uptodate.com/contents/moyamoya-disease-and-moyamoya-syndrome-etiology-clinical-features-and-diagnosis/print 34/39 7/6/23, 12:05 PM Moyamoya disease and moyamoya syndrome: Etiology, clinical features, and diagnosis - UpToDate Graphic 129106 Version 2.0 https://www.uptodate.com/contents/moyamoya-disease-and-moyamoya-syndrome-etiology-clinical-features-and-diagnosis/print 35/39 7/6/23, 12:05 PM Moyamoya disease and moyamoya syndrome: Etiology, clinical features, and diagnosis - UpToDate Intracerebral hemorrhage with intraventricular hemorrhage due to moyamoya syndrome Noncontrast head CT (A) showing hematoma in the right corpus striatum and lateral ventricles. Digital subtraction angiogram (B) showing critical stenosis of proximal right middle cerebral artery (arrow), prominent collateralization of both lenticulostriate vessels (circle), and branches of the anterior cerebral artery (thick arrows). CT: computed tomography. Courtesy of Glenn A Tung, MD, FACR. Graphic 132277 Version 1.0 https://www.uptodate.com/contents/moyamoya-disease-and-moyamoya-syndrome-etiology-clinical-features-and-diagnosis/print 36/39 7/6/23, 12:05 PM Moyamoya disease and moyamoya syndrome: Etiology, clinical features, and diagnosis - UpToDate Magnetic resonance angiography (MRA) of a patient with moyamoya disease A) There is severe narrowing of the distal part of the both carotid arteries (arrows). In addition, there is markedly reduced flow in the left middle cerebral artery and absence of both anterior cerebral arteries. B) Lateral projection MRA demonstrates severe narrowing of the distal internal carotid artery (arrow). Courtesy of Nijasri Suwanwela, MD. Graphic 51947 Version 2.0 https://www.uptodate.com/contents/moyamoya-disease-and-moyamoya-syndrome-etiology-clinical-features-and-diagnosis/print 37/39 7/6/23, 12:05 PM Moyamoya disease and moyamoya syndrome: Etiology, clinical features, and diagnosis - UpToDate 12-year-old with moyamoya syndrome secondary to neurofibromatosis type 1 Magnetic resonance angiogram shows occlusion of left supraclinoid internal carotid artery (circles) and lentic collateral vessels (arrow). Courtesy of Glenn A Tung, MD, FACR. Graphic 129107 Version 3.0 https://www.uptodate.com/contents/moyamoya-disease-and-moyamoya-syndrome-etiology-clinical-features-and-diagnosis/print 38/39 7/6/23, 12:05 PM Moyamoya disease and moyamoya syndrome: Etiology, clinical features, and diagnosis - UpToDate Contributor Disclosures Nijasri Charnnarong Suwanwela, MD No relevant financial relationship(s) with ineligible companies to disclose. Jos Biller, MD, FACP, FAAN, FAHA No relevant financial relationship(s) with ineligible companies to disclose. Douglas R Nordli, Jr, MD No relevant financial relationship(s) with ineligible companies to disclose. Glenn A Tung, MD, FACR No relevant financial relationship(s) with ineligible companies to disclose. Richard P Goddeau, Jr, DO, FAHA No relevant financial relationship(s) with ineligible companies to disclose. Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found, these are addressed by vetting through a multi-level review process, and through requirements for references to be provided to support the content. Appropriately referenced content is required of all authors and must conform to UpToDate standards of evidence. Conflict of interest policy https://www.uptodate.com/contents/moyamoya-disease-and-moyamoya-syndrome-etiology-clinical-features-and-diagnosis/print 39/39