Case Report : Moyamoya Disease in a Caucasian Infant with Early and Severe Clinical Presentation

Moyamoya disease is a chronic progressive cerebrovascular disease of the arteries around the circle of Willis with prominent arterial collateral circulation. We describe a 15 month old Caucasian female infant, who was admitted to our hospital due to left hemiparesis. Magnetic resonance imaging demonstrated occlusion at the terminal portion of the internal carotid artery, at the proximal portion of the anterior and middle cerebral arteries and stenosis of posterior cerebral arteries bilaterally. Magnetic resonance angiography and conventional cerebral angiography revealed the characteristic abnormal vascular network known as “puff of cigarette smoke”. An extensive laboratory workup was performed to rule out underlying diseases such as coagulopathy, autoimmune or infectious diseases. The infant underwent a surgical procedure of bilateral neurosurgical revascularization, encephalomyosynangiosis, initially on the right and then on the left cerebral hemisphere. Clinical improvement was gradually noticed after the surgery. Our patient is a rare case of MMD as she is non-Asian, with early and severe clinical presentation, and extended radiological lesions at the time of diagnosis.