Moyamoya Disease and Moyamoya Syndrome

Moyamoya disease is a cerebrovascular condition predisposing affected patients to stroke in association with progressive stenosis of the intracranial internal carotid arteries and their proximal branches. Patients with characteristic moyamoya vasculopathy plus associated conditions are categorized as having moyamoya syndrome. This review describes the demographic characteristics, pathogenesis, evaluation, and treatment of moyamoya disease and syndrome. Moyamoya Moyamoya disease is a cerebrovascular condition predisposing affected patients to stroke in association with progressive stenosis of the intracranial internal carotid arteries and their proximal branches. This review describes the demographic characteristics, pathogenesis, evaluation, and treatment of moyamoya disease and syndrome. The moyamoya syndrome is a cerebrovascular condition that predisposes affected patients to stroke in association with progressive stenosis of the intracranial internal carotid arteries and their proximal branches. Reduced blood flow in the major vessels of the anterior circulation of the brain leads to compensatory development of collateral vasculature by small vessels near the apex of the carotid, on the cortical surface, leptomeninges, and branches of the external carotid artery supplying the dura and the base of the skull. In rare cases, this process also involves the posterior circulation, including the basilar and posterior cerebral arteries. First described in 1957 . . .