Skip to main content
Advertisement
  • Main menu
  • User menu
  • Search
  • English ▼
    • English
    • Afrikaans
    • Albanian
    • Amharic
    • Arabic
    • Armenian
    • Azerbaijani
    • Basque
    • Belarusian
    • Bengali
    • Bosnian
    • Bulgarian
    • Catalan
    • Cebuano
    • Chichewa
    • Chinese (Simplified)
    • Chinese (Traditional)
    • Corsican
    • Croatian
    • Czech
    • Danish
    • Dutch
    • Esperanto
    • Estonian
    • Filipino
    • Finnish
    • French
    • Frisian
    • Galician
    • Georgian
    • German
    • Greek
    • Gujarati
    • Haitian Creole
    • Hausa
    • Hawaiian
    • Hebrew
    • Hindi
    • Hmong
    • Hungarian
    • Icelandic
    • Igbo
    • Indonesian
    • Irish
    • Italian
    • Japanese
    • Javanese
    • Kannada
    • Kazakh
    • Khmer
    • Korean
    • Kurdish (Kurmanji)
    • Kyrgyz
    • Lao
    • Latin
    • Latvian
    • Lithuanian
    • Luxembourgish
    • Macedonian
    • Malagasy
    • Malay
    • Malayalam
    • Maltese
    • Maori
    • Marathi
    • Mongolian
    • Myanmar (Burmese)
    • Nepali
    • Norwegian
    • Pashto
    • Persian
    • Polish
    • Portuguese
    • Punjabi
    • Romanian
    • Russian
    • Samoan
    • Scottish Gaelic
    • Serbian
    • Sesotho
    • Shona
    • Sindhi
    • Sinhala
    • Slovak
    • Slovenian
    • Somali
    • Spanish
    • Sudanese
    • Swahili
    • Swedish
    • Tajik
    • Tamil
    • Telugu
    • Thai
    • Turkish
    • Ukrainian
    • Urdu
    • Uzbek
    • Vietnamese
    • Welsh
    • Xhosa
    • Yiddish
    • Yoruba
    • Zulu

Main menu

  • Home
  • Content
    • Current Issue
    • Accepted Manuscripts
    • Article Preview
    • Past Issue Archive
    • Video Articles
    • AJNR Case Collection
    • Case of the Week Archive
    • Case of the Month Archive
    • Classic Case Archive
  • Special Collections
    • AJNR Awards
    • Low-Field MRI
    • Alzheimer Disease
    • ASNR Foundation Special Collection
    • Photon-Counting CT
    • View All
  • Multimedia
    • AJNR Podcasts
    • AJNR SCANtastic
    • Trainee Corner
    • MRI Safety Corner
    • Imaging Protocols
  • For Authors
    • Submit a Manuscript
    • Submit a Video Article
    • Submit an eLetter to the Editor/Response
    • Manuscript Submission Guidelines
    • Statistical Tips
    • Fast Publishing of Accepted Manuscripts
    • Graphical Abstract Preparation
    • Imaging Protocol Submission
    • Author Policies
  • About Us
    • About AJNR
    • Editorial Board
    • Editorial Board Alumni
  • More
    • Become a Reviewer/Academy of Reviewers
    • Subscribers
    • Permissions
    • Alerts
    • Feedback
    • Advertisers
    • ASNR Home

User menu

  • Alerts
  • Log in

Search

  • Advanced search
American Journal of Neuroradiology
American Journal of Neuroradiology

American Journal of Neuroradiology

ASHNR American Society of Functional Neuroradiology ASHNR American Society of Pediatric Neuroradiology ASSR
  • Alerts
  • Log in

Advanced Search

English ▼
  • English
  • Afrikaans
  • Albanian
  • Amharic
  • Arabic
  • Armenian
  • Azerbaijani
  • Basque
  • Belarusian
  • Bengali
  • Bosnian
  • Bulgarian
  • Catalan
  • Cebuano
  • Chichewa
  • Chinese (Simplified)
  • Chinese (Traditional)
  • Corsican
  • Croatian
  • Czech
  • Danish
  • Dutch
  • Esperanto
  • Estonian
  • Filipino
  • Finnish
  • French
  • Frisian
  • Galician
  • Georgian
  • German
  • Greek
  • Gujarati
  • Haitian Creole
  • Hausa
  • Hawaiian
  • Hebrew
  • Hindi
  • Hmong
  • Hungarian
  • Icelandic
  • Igbo
  • Indonesian
  • Irish
  • Italian
  • Japanese
  • Javanese
  • Kannada
  • Kazakh
  • Khmer
  • Korean
  • Kurdish (Kurmanji)
  • Kyrgyz
  • Lao
  • Latin
  • Latvian
  • Lithuanian
  • Luxembourgish
  • Macedonian
  • Malagasy
  • Malay
  • Malayalam
  • Maltese
  • Maori
  • Marathi
  • Mongolian
  • Myanmar (Burmese)
  • Nepali
  • Norwegian
  • Pashto
  • Persian
  • Polish
  • Portuguese
  • Punjabi
  • Romanian
  • Russian
  • Samoan
  • Scottish Gaelic
  • Serbian
  • Sesotho
  • Shona
  • Sindhi
  • Sinhala
  • Slovak
  • Slovenian
  • Somali
  • Spanish
  • Sudanese
  • Swahili
  • Swedish
  • Tajik
  • Tamil
  • Telugu
  • Thai
  • Turkish
  • Ukrainian
  • Urdu
  • Uzbek
  • Vietnamese
  • Welsh
  • Xhosa
  • Yiddish
  • Yoruba
  • Zulu
  • Home
  • Content
    • Current Issue
    • Accepted Manuscripts
    • Article Preview
    • Past Issue Archive
    • Video Articles
    • AJNR Case Collection
    • Case of the Week Archive
    • Case of the Month Archive
    • Classic Case Archive
  • Special Collections
    • AJNR Awards
    • Low-Field MRI
    • Alzheimer Disease
    • ASNR Foundation Special Collection
    • Photon-Counting CT
    • View All
  • Multimedia
    • AJNR Podcasts
    • AJNR SCANtastic
    • Trainee Corner
    • MRI Safety Corner
    • Imaging Protocols
  • For Authors
    • Submit a Manuscript
    • Submit a Video Article
    • Submit an eLetter to the Editor/Response
    • Manuscript Submission Guidelines
    • Statistical Tips
    • Fast Publishing of Accepted Manuscripts
    • Graphical Abstract Preparation
    • Imaging Protocol Submission
    • Author Policies
  • About Us
    • About AJNR
    • Editorial Board
    • Editorial Board Alumni
  • More
    • Become a Reviewer/Academy of Reviewers
    • Subscribers
    • Permissions
    • Alerts
    • Feedback
    • Advertisers
    • ASNR Home
  • Follow AJNR on Twitter
  • Visit AJNR on Facebook
  • Follow AJNR on Instagram
  • Join AJNR on LinkedIn
  • RSS Feeds

AJNR Awards, New Junior Editors, and more. Read the latest AJNR updates

Case of the Month

Section Editor: Nicholas Stence, MD
Children's Hospital Colorado, Aurora, CO

Submit a Case Previous Cases

August 2022
  • Description
  • Week 1
  • Week 2
  • Week 3
  • Week 4
  • Discussion
Loading

Next Case of the Month Coming September 6...

Moyamoya Disease

  • Background:
    • Rare idiopathic progressive vasculopathy characterized by stenosis and occlusion of cerebral arteries, mostly terminal portions of the bilateral internal carotid arteries and their proximal branches, which results in the formation of collateral vessels
    • Bimodal age peaks: 5–10 years and fourth decade
  • Clinical Presentation:
    • Children: Recurrent transient ischemic attacks; hemorrhage is rare
    • Adults: Transient ischemic attacks, infarcts, hemorrhage, migraines
  • Key Diagnostic Features:
    • T2-FLAIR: Linear hyperintensity along the cerebral sulci (“ivy sign") – caused by slow retrograde flow of engorged pial arteries via leptomeningeal anastomosis
    • CT/MRI postcontrast: Multiple lenticulostriate collaterals and netlike thin vessels in cisterns
    • Digital subtraction angiography: Characteristic “puff of smoke” appearance
    • Perfusion CT/MRI: Useful in evaluation of cerebral hemodynamic status
  • Differential Diagnoses:
    • Cranial radiotherapy
    • Atherosclerosis
    • Neurofibromatosis type 1
    • Cerebral vasculitis
  • Treatment
    • Antithrombotic drugs – to prevent the recurrence of stroke and are associated with reduced mortality
    • Surgical revascularization
      • Indirect bypass: Encephalo-duro-arterio-synangiosis; more effective in children
      • Direct bypass: Superficial temporal artery-middle cerebral artery; more common in adults

Suggested Reading

  1. Wang L-X, Wang H, Hao F-B, et al. Ivy sign in Moyamoya disease: a comparative study of the FLAIR vascular hyperintensity sign against contrast-enhanced MRI. AJNR Am J Neuroradiol 2021;42:694–700
  2. Strother MK, Anderson MD, Singer RJ, et al. Cerebrovascular collaterals correlate with disease severity in adult North American patients with Moyamoya disease. AJNR Am J Neuroradiol 2014;35:1318–24
  3. Michael Scott R, Smith ER. Moyamoya disease and Moyamoya syndrome. N Engl J Med 2009;360:1226–37

Current Issue

American Journal of Neuroradiology: 46 (7)
American Journal of Neuroradiology
Vol. 46, Issue 7
1 Jul 2025
  • Table of Contents
  • Index by author
  • Complete Issue (PDF)
Sign up for alerts
Advertisement

Case Collections

Clasic Case Archive
Case of the Week Archive
Case of the Month Archive
Advertisement

Indexed Content

  • Current Issue
  • Accepted Manuscripts
  • Article Preview
  • Past Issues
  • Editorials
  • Editor's Choice
  • Fellows' Journal Club
  • Letters to the Editor
  • Video Articles

Cases

  • Case Collection
  • Archive - Case of the Week
  • Archive - Case of the Month
  • Archive - Classic Case

More from AJNR

  • Trainee Corner
  • Imaging Protocols
  • MRI Safety Corner
  • Book Reviews

Multimedia

  • AJNR Podcasts
  • AJNR Scantastics

Resources

  • Turnaround Time
  • Submit a Manuscript
  • Submit a Video Article
  • Submit an eLetter to the Editor/Response
  • Manuscript Submission Guidelines
  • Statistical Tips
  • Fast Publishing of Accepted Manuscripts
  • Graphical Abstract Preparation
  • Imaging Protocol Submission
  • Evidence-Based Medicine Level Guide
  • Publishing Checklists
  • Author Policies
  • Become a Reviewer/Academy of Reviewers
  • News and Updates

About Us

  • About AJNR
  • Editorial Board
  • Editorial Board Alumni
  • Alerts
  • Permissions
  • Not an AJNR Subscriber? Join Now
  • Advertise with Us
  • Librarian Resources
  • Feedback
  • Terms and Conditions
  • AJNR Editorial Board Alumni

American Society of Neuroradiology

  • Not an ASNR Member? Join Now

© 2025 by the American Society of Neuroradiology All rights, including for text and data mining, AI training, and similar technologies, are reserved.
Print ISSN: 0195-6108 Online ISSN: 1936-959X

Powered by HighWire

We use cookies on this site to enhance your user experience. By clicking any link on this page you are giving your consent for us to set cookies.