Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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November 13, 2014
Moyamoya Disease
- The term moyamoya ("hazy" in Japanese translation) describes any slowly progressive brain arteriopathy involving distal carotid or middle cerebral artery, uni- or bilaterally, and developing collateral vessels in a lenticulostriate distribution with the typical "puff of smoke" pattern on angiography.
- This condition does not refer to a specific disease or syndrome but rather to that peculiar angiographic pattern.
- In most cases, moyamoya is associated with phakomatoses, sickle cell disease, systemic arteritis, radiation-induced angiopathy, and collagenopathies. An idiopathic/primary form (the classical "moyamoya disease") is also described, which occurs more commonly in Asian populations.
- Key Diagnostic Features:
- Stenosis of ICA or MCA with multiple parenchymal (mainly in the basal ganglia) and/or leptomeninegeal collateral vessels
- "Puff of smoke" appearance on angiogram
- Ischemic foci in a water-shed distribution
- Ipsilateral FLAIR sulcal nonsuppression and postgadolinium leptomeningeal enhancement suggestive of engorged collateral circulation, termed the "ivy sign"
- DDx:
- Dissection
- Thrombosis
- AVM
- Rx: Bypass or stent placement
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