Moyamoya disease. Its clinical picture, diagnosis and new therapies.

Abstract

Moyamoya disease is a chronic cerebral vasculopathy that leads to a slow progressive narrowing and occlusion of the principal arteries responsible for the anterior circulation of the brain. It causes a cerebrovascular insufficiency with the development of multiple anastomoses between internal and external carotid arteries. The diagnosis is based on the peculiar appearance in angiography. Although rare, it is not possible to omit it in the differential diagnosis of ischemic vascular accidents in children. The authors describe a case of a eight year old female caucasian whose diagnosis was suspected by means of the non invasive technique of angiographic magnetic resonance imaging and confirmed by angiography. Based on this case, the main aspects of the disease are described, with particular emphasis on clinical features, diagnosis and treatment.