Moyamoya Disease in Adult: A Clinical Case

Abstract

Moyamoya disease is an entity characterized by progressive and prolonged occlusion of the terminal intracranial portion of the internal carotid artery and of the Willis polygon. We present the case of a 33-year-old female patient observed in the Emergency Department of Internal Medicine for sudden onset right hemiparesis. Anamnesis revealed no family history for increased risk for thrombosis, an unmedicated dyslipidemia (cLDL 165 mg/dL), overweight, and 9 pack year smoking load. During hospitalization there was a progressive improvement of the condition, but imaging exams revealed a neovascularization phenomenon typical of Moyamoya’s disease and the surgical treatment was proposed. Moyamoya disease is a rare entity whose etiology remains unknown. The clinical presentation is characterized by ischemia or intracranial hemorrhage. Surgical intervention is the best treatment. The description of this case aims to draw attention to the importance of the knowledge of this entity.