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In moyamoya disease, arteries to your brain become narrow and may even close, leading to reduced delivery of oxygen-rich blood to your brain. This lack of blood flow to the brain can cause stroke and other symptoms.
Moyamoya disease is a rare, progressive blood vessel (vascular) disorder in which the carotid artery in the skull becomes blocked or narrowed, reducing blood flow to your brain. Tiny blood vessels then open up at the base of the brain in an attempt to supply the brain with blood. The word “moyamoya” means “puff of smoke” in Japanese, a term describing the appearance of this cluster of tiny blood vessels.
These tiny clusters of blood vessels cannot supply the necessary blood and oxygen to the brain, resulting in temporary or permanent brain injury.
The condition may cause a ministroke (transient ischemic attack, or TIA), stroke, bulge or ballooning in a blood vessel (aneurysm) or bleeding in the brain. It can also affect how well your brain functions and cause cognitive and developmental delays or disability.
Moyamoya disease most commonly affects children, but adults may have the condition. Moyamoya disease is found all over the world, but it’s more common in East Asian countries, especially Korea, Japan and China and in people of East Asian descent.
Moyamoya disease may occur at any age, though symptoms most commonly occur between 5 and 10 years of age in children and between 30 and 50 years of age in adults.
The first symptom of moyamoya disease is usually stroke or recurrent transient ischemic attacks (TIAs), especially in children. Adults may also experience these symptoms but more often experience bleeding in the brain (hemorrhagic stroke) from abnormal brain vessels.
Accompanying signs and symptoms of moyamoya disease related to reduced blood flow to the brain include:
These symptoms can be triggered by exercise, crying, coughing, straining or fever.
Seek immediate medical attention if you notice any signs or symptoms of a stroke or TIA, even if they seem to fluctuate or disappear.
Think “FAST” and do the following:
Call 911 or your local emergency number right away. Don’t wait to see if symptoms go away. Every minute counts. The longer a stroke goes untreated, the greater the potential for brain damage and disability.
If you’re with someone you suspect is having a stroke, watch the person carefully while waiting for emergency assistance.
The exact cause of moyamoya disease is unknown. Moyamoya disease is more common in Japan, Korea and China, but it also occurs in other parts of the world.
Researchers believe the higher concentration of moyamoya disease in these Asian countries strongly suggests the disease may have genetic causes.
Moyamoya is also associated with certain conditions, such as Down syndrome, sickle cell anemia, neurofibromatosis type 1 and hyperthyroidism.
Though the cause of moyamoya disease is unknown, certain factors may increase your risk of having the condition, including:
Most complications from moyamoya disease are associated with the effects of stroke, such as:
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