Systemic Arterial Involvement in Moyamoya Disease

Moyamoya disease is a rare cerebrovascular occlusive disorder that is characterized by stenosis or occlusion of the distal internal carotid or proximal anterior or middle cerebral arteries, which causes the formation of multiple tiny collateral vascular networks (moyamoya vessels) at the base of 1the brain [1, 2, 3]. While this disorder commonly occurs alone (moyamoya disease), it occasionally occurs with well-recognized associated conditions including sickle cell disease, neurofibromatosis type I, cranial therapeutic irradiation, and Down syndrome (moyamoya syndrome) [4].