Moyamoya disease

Discussion:

The described features are those of Moyamoya disease with occluded distal ICAs as well as left MCA causing extensive acute left cerebral infarction.

The Ivy sign refers to the leptomeningeal hyper intense FLAIR signal or brighnt sulci due to slow-flowing engorged pial vessels as well as thickened arachnoid membranes.

The Moyamoya means a puff or spiral of smoke describing the cloud-like lenticulostriate and thalamostriate collaterals.

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