Cognition and quality of life

Abstract

Moyamoya disease is an uncommon cerebrovascular disorder that is characterized by progressive stenosis of the supraclinoid internal carotid arteries (ICA) and its main branches within the circle of Willis. This occlusion results in the formation of a fine vascular network (the moyamoya vessels) at the base of the brain. Moyamoya vessels are dilated perforating arteries and function as collateral pathways. It is well known that pediatric patients with moyamoya disease often develop transient ischemic attacks (TIA) or cerebral infarction, while adult patients also suffer intracranial bleeding. Surgical revascularization such as superficial temporal artery to middle cerebral artery (STA-MCA) anastomosis and indirect bypass improves their cerebral hemodynamics and prevents further ischemic attacks [1]. With regard to stroke recurrence and activity of daily living (ADL), postoperative long-term outcome is favorable in both pediatric and adult patients [2-5]. However, it is also known that intellectual development is impaired in a certain subgroup of pediatric patients [6, 7]. Even after surgical revas-cularization, intellectual impairment has been reported to disturb an independent social life in more than 20% of the patients [3, 5, 8, 9]. In this chapter, the author reviews neuropsychological aspects in patients with moyamoya disease and discusses the strategies to improve their intellectual outcome. © 2010 Springer-Verlag Tokyo.