Moyamoya disease is characterized by the closure of one or usually both internal carotid arteries in the intracranial region, resulting in the formation of a fine network of blood vessel growth at the base of the brain.
The term "moyamoya" was introduced to describe the hazy, puff-of-smoke appearance of the neovascularization that develops. This condition occurs more often in Japan but is found globally, and is most common in children younger than 10 and among adults in their 30s.
Conditions associated with moyamoya disease include neurofibromatosis, tuberous sclerosis, sickle cell anemia, meningitis, retinitis pigmentosa, fibromuscular dysplasia, atherosclerosis, Down's syndrome and Fanconi's anemia following radiation therapy to the skull base in children.
UCLA neurosurgeon Anthony Wang, MD, reviews moyamoya disease and moyamoya syndrome, discusses what we know about them and compares treatment options.
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