Moyamoya disease is a progressive, occlusive disease of the cerebral vessels with particular involvement of the circle of Willis and the arteries that form it. Collateral vessels develops around the blocked artery to compensate for the blockage, but the collateral vessels are small, weak, and prone to hemorrhage, aneurysm and thrombosis. On conventional X-ray angiography, these collateral vessels have the appearance of a "puff of smoke" for which it is named as Moyamoya (Moyamoya is term for puff of smoke in japanese). It can present as migraine type headache, recurrent transient ischemic attacks (TIAs), stroke or hemorrhage in the brain. Therapy is primarily directed at the complications of the disease.
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| Rich collaterals around the blocked artery giving the appearance of puff of smoke. |
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