Moyamoya Disease

Excerpt

Moyamoya disease was first described in Japanese literature in 1957; however, Suzuki and Takaku first coined the term “moyamoya disease” in 1969. Moyamoya disease is an isolated chronic, usually bilateral, vasculopathy of undetermined etiology characterized by progressive narrowing of the terminal intracranial portion of the internal carotid artery (ICA) and circle of Willis. Moyamoya syndrome corresponds to the same moyamoya arteriopathy, but in the context of either neurological or extraneurological conditions, whether inherited or acquired. A fragile network of abundant collateral vessels develops in response to chronic brain ischemia, known as moyamoya vessels, which appear as a “puff of smoke” on angiography. Patients most often present with ischemic strokes or transient ischemic attacks (TIAs); however, intracranial hemorrhages are also common due to the fragility of the abnormal moyamoya vessels. Surgical revascularization is the mainstay of treatment for both ischemic and hemorrhagic presentations of moyamoya disease.