Two Cases of Moyamoya Disease Occurred in a Family

ABSTRACT

Moyamoya disease is a rare obstructive cerebrovascular disease characterized by a cerebral angiographic picture of bilateral stenosis or occlusion of main cerebral arteries with an abnormal vascular network at the base of the brain. Although its pathogenesis is not clear, there is extensive evidence that this disease has tendency to show inheritance and familial occurrence. We have experinced such two cases of moyamoya disease. One was 4-year-old boy who had a history of TIA and bilateral hemiparesis(CaseI) and another was 8-year-old boy. The older brother of the Case I had unilateral symptom. They were treated with EDAS and split duroence-phalosynangiosis and had good results in their neurologic and postoperative angiographic state.