Cognition and Cerebrovascular Imaging in Moyamoya Vasculopathy

Moyamoya vasculopathy (MMV) is a unique cerebrovascular disease of unknown etiology. It is a progressive intracranial arteriopathy characterized by bilateral stenosis of the distal portion of the internal carotid artery and proximal anterior and middle cerebral artery, resulting in transient ischemic attacks, or ischemic or hemorrhagic strokes. Furthermore, patients may suffer from cognitive impairment. Although this rare disorder is mostly seen in Eastern Asia, moyamoya is increasingly recognized in the Western world, especially in children and young adults. There are strong indications that neurosurgical intervention through direct, indirect or combined revascularization surgery improves cerebral perfusion and reduces the risk of ischemic stroke. Since its first description in 1969, increasing information on clinical characteristics, outcome, radiological parameters and the effect of revascularization has become available, however, the cognitive profile is yet to be characterized. The current thesis describes several studies in patients with MMV treated in our own institute in order to establish 1) clinical and radiological characteristics 2) the neurocognitive profile, determinants for cognitive impairment and the correlation with cerebral hemodynamics 3) new MRI features and its clinical use.