Abstract
Steno-occlusive disease of the internal carotid arteries and/or the circle of Willis with development of collateral perforator vessels attempting to supply under-perfused parenchyma are the basis for moya-moya phenomenon with the classic "puff of smoke" appearance on cerebral angiogram. We describe two cases of moya-moya with unilateral macroangiopathy of the internal carotid artery and ipsilateral middle cerebral artery in two 11-year-old girls: a Down's syndrome patient, and a second idiopathic patient. The arteriopathy in our cases differs from typical or classically described moya-moya disease in that it was exclusively unilateral rather than symmetric and bilateral. The association of predominant deep grey matter (basal ganglia) strokes in children with coexisting ipsilateral parainsular infarcts, as in our cases, is potentially a red flag for ipsilateral macroangiopathy.
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