Moyamoya syndrome

Last revised by Rohit Sharma ◉ on 23 Sep 2024

Citation, DOI, disclosures and article data

Citation:

Gaillard F, Sharma R, Thibodeau R, et al. Moyamoya syndrome. Reference article, Radiopaedia.org (Accessed on 28 Mar 2025) https://doi.org/10.53347/rID-10725

DOI:

https://doi.org/10.53347/rID-10725

Permalink:

https://radiopaedia.org/articles/10725

rID:

10725

Article created:

13 Sep 2010, Frank Gaillard ◉ ◈

Disclosures:

At the time the article was created Frank Gaillard had no recorded disclosures.

View Frank Gaillard's current disclosures

Last revised:

23 Sep 2024, Rohit Sharma ◉

Disclosures:

At the time the article was last revised Rohit Sharma had no financial relationships to ineligible companies to disclose.

View Rohit Sharma's current disclosures

Revisions:

18 times, by 12 contributors - see full revision history and disclosures

Systems:

Vascular, Central Nervous System

Sections:

Syndromes

Tags:

syndrome

Synonyms:

Moyamoya phenomenon
Moyamoya pattern
Moyamoya syndrome
Moyamoya angiopathy

Moyamoya syndrome, also termed moyamoya pattern or phenomenon or angiopathy, is due to numerous conditions that can cause arterial occlusion of the circle of Willis, with resultant collaterals, and appearances reminiscent of moyamoya disease.

These conditions include ^1-4:

vessel wall abnormalities
phakomatoses
- neurofibromatosis type 1 (NF1)
- tuberous sclerosis complex
connective tissue disorders
- systemic lupus erythematosus (SLE)
- antiphospholipid syndrome
blood dyscrasias
infection
less common causes
- Graves disease ⁵
- Down syndrome
- ulcerative colitis
- Apert syndrome
- oral contraceptive use

Quiz questions

References

1. Hsu SW, Chaloupka JC, Fattal D. Rapidly progressive fatal bihemispheric infarction secondary to Moyamoya syndrome in association with Graves thyrotoxicosis. AJNR Am J Neuroradiol. 2006;27 (3): 643-7. AJNR Am J Neuroradiol (full text) - Pubmed citation
2. Dähnert W. Radiology Review Manual. Lippincott Williams & Wilkins. (2007) ISBN:0781766206. Read it at Google Books - Find it at Amazon
3. Reeder MM, Bradley WG. Reeder and Felson's gamuts in radiology, comprehensive lists of roentgen differential diagnosis. Springer. (2003) ISBN:0387955887. Read it at Google Books - Find it at Amazon
4. Aviv RI, Benseler SM, Silverman ED et-al. MR imaging and angiography of primary CNS vasculitis of childhood. AJNR Am J Neuroradiol. 2006;27 (1): 192-9. AJNR Am J Neuroradiol (full text) - Pubmed citation
5. Jiang T, Perry A, Dacey RG et-al. Intracranial atherosclerotic disease associated with moyamoya collateral formation: histopathological findings. J. Neurosurg. 2013;118 (5): 1030-4. doi:10.3171/2013.1.JNS12565 - Free text at pubmed - Pubmed citation
6. Lazzaro MA, Cochran EJ, Lopes DK et-al. Moyamoya syndrome in an adult with essential thrombocythemia. Neurol Int. 2011;3 (1): e3. doi:10.4081/ni.2011.e3 - Free text at pubmed - Pubmed citation
7. Hua le H, Dodd RL, Schwartz NE. Concurrent stenoocclusive disease of intracranial and extracranial arteries in a patient with polycythemia vera. Case Rep Med. 29;2012: 151767. doi:10.1155/2012/151767 - Free text at pubmed - Pubmed citation
8. Kornblihtt LI, Cocorullo S, Miranda C et-al. Moyamoya syndrome in an adolescent with essential thrombocythemia: successful intracranial carotid stent placement. Stroke. 2005;36 (8): E71-3. doi:10.1161/01.STR.0000174193.89864.55 - Pubmed citation