PMC3808186_01
Brain MRI. (A, B) diffusion weighted sequence and apparent diffusion coefficient map, respectively, demonstrate a focal area of restriction of diffusion involving the right rostrum, genu and body of the Corpus Callosum with mild enhancement on post-gadolinium T1W sequence (C) and moderate edema and local mass effect on T2W sequence (D). A second lesion involving the right basal ganglia and genu of the right internal capsule demonstrates incomplete peripheral enhancement with central necrosis, without mass effect or restriction of diffusion.
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A subsequent brain MRI showed multiple supratentorial lesions predominately involving the rostrum of the corpus callosum, genu of the right internal capsule and basal ganglia and subcortical cingulate white matter, with variable degrees of enhancement associated with mass effect and restricted diffusion (Figure 1).
Cerebral angiography. Biplanar cerebral angiography of the left internal carotid artery ((A, B) lateral and AP respectively) demonstrated complete occlusion of the left supraclinoid internal carotid artery with a patent left anterior choroidal artery supplying lenticulostriatal vessels giving the angiographic appearance of a "puff of smoke," suggestive of Moyamoya angiographic pattern. Collateral flow from the right posterior choroidal artery supplying the pericollosal, frontopolar and callosmarginal arteries demonstrated on lateral view on selective angiogram of the left vertebral artery (C). Intracranial view of the right internal carotid artery in the AP projection (D) demonstrates an irregular M1 segment and complete occlusion of the mid A1 segment of anterior cerebral artery. Cortical branches also appeared irregularity consistent with a proliferative intracranial vasculopathy.
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Cerebral angiography (Figure 2) demonstrated bilateral intracranial arteriopathy with occlusion of the supraclinoid segment of the left intracranial internal carotid artery distal to the takeoff of the left anterior choroidal artery, occlusion of the right anterior cerebral artery, and severe disease in the proximal right middle cerebral artery.