PMC5586868_01
Cerebral angiogram showing occlusion of the distal internal carotid artery (arrow) and characteristic 'puff of smoke' appearance (arrowhead).
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F1
Digital subtraction angiogram revealed narrowing of the distal internal carotid arteries, proximal anterior and middle cerebral arteries, worse on the right, with the 'puff of smoke' appearance diagnostic of the moyamoya syndrome (fig. 1).
Brain biopsy (hematoxylin and eosin stain) showing lymphocytic infiltration of the vessel wall (arrowheads) and perivascular tissue, suggestive of vasculitis.
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F2
T lymphocyte infiltrates (immune phenotyping CD3+ and CD20-) in the vessel wall and perivascular brain tissue (fig. 2). There was vascular endothelial cell swelling, and white matter tissue showed no evidence of demyelination or visible gliosis.
Follow-up CT angiography at 6 months showing stenosis and occlusion at the terminus of the internal carotid arteries (arrowhead) and extensive collateral formation (arrow).
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F3
Follow-up CT angiography (at 6 months) revealed stenosis and occlusion at the terminus of the internal carotid arteries and extensive collateral formation (fig. 3).