Cerebral Angiography in Moyamoya Syndrome Secondary to Vasculopathy in Down Syndrome

Moyamoya disease (MMD) is an idiopathic intracranial vasculopathy characterized by progressive bilateral stenosis or occlusion of the terminal internal carotid arteries (ICA) and their main branches with compensatory collateral angiogenesis. It is more prevalent among Asian populations and has a typical angiographic appearance. A similar angiographic pattern is associated with vasculopathy due to several medical conditions including Down syndrome and is termed Moyamoya syndrome (MMS). We present neuroimages for a case of an early stage of MMS secondary to vasculopathy in Down syndrome in a young woman who presented with ischemic stroke.