Moyamoya disease is a rare, progressive cerebrovascular disorder caused by blocked arteries at the base of the brain. The name “moyamoya” means “puff of smoke” in Japanese.
Moyamoya disease is a cerebrovascular disorder with rare but real risk for many kids. It is a serious syndrome in which the walls of the internal carotid arteries thicken and become narrow. These arteries supply blood to important areas of the brain.
Moyamoya disease causes the flow of oxygen-rich blood to slow down gradually and increases the likelihood of clot formation. This reduced blood and formation of blood clots are major risk factors for either a transient ischemic attack (TIA) also called a “mini-stroke,” or a full-fledged stroke.
Moyamoya is a Japanese word meaning “puff of smoke.” The name has been given to the disease because of its characteristics: wispy, tangled appearance of the new blood vessels that emerge in the brain (as the body attempts to compensate for the inadequate blood supply).
Moyamoya disease is a progressive condition, meaning that symptoms worsen over time and the child's chances of suffering a stroke increases. Because Moyamoya disease becomes progressively more severe over time, children with the condition will eventually need surgery to restore normal and healthy blood flow to their brains and reduce the likelihood of a stroke.
The only proven treatment for is surgery to create a healthy, adequate new supply of blood for the impacted areas of the brain. The doctor may also recommend medication to manage some of your child's symptoms.
But, it's important to emphasize that no medication can stop either the progression of narrowing in the brain's blood vessels or the development of the thin, fragile vessels that characterize Moyamoya disease. Surgery is the mainstay of treatment for Moyamoya.
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