Moyamoya Vasculopathy: Cause, Clinical Manifestations, Neuroradiologic Features, and Surgical Management

In moyamoya disease, the progressive occlusion of the distal portion of the internal carotid artery and its major branches is typically responsible for the formation of an extensive network of collateral vessels at the base of the brain. When moyamoya collateral network develops in association with various systemic or acquired diseases, the term moyamoya syndrome is used to denote this phenomenon. Sudden changes in the supraclinoid internal carotid artery and middle cerebral artery can be recognized with noninvasive neurovascular imaging techniques, which also allow a differential diagnosis with similar diseases such as degenerative steno-occlusive disease, cerebral vasculitis, and twig-like middle cerebral artery. Once the diagnosis is established, the definitive treatment for moyamoya disease is surgical revascularization, with the goal of increasing cerebral blood flow and preventing recurrent stroke. We provide a comprehensive review of the clinical and radiologic features in moyamoya vasculopathy along with its surgical management.