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Moyamoya Syndrome

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 Neurosurgery
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Moyamoya syndrome is a very rare disease in which the walls of the internal carotid arteries - which supply blood to important areas of the brain - become thickened. This gradually slows the flow of blood to the brain and increases the likelihood of blood clot formation, both of which can lead to strokes and transient ischemic attacks.

In this condition, small blood vessels also form a network of "side roads" trying to supply oxygen to the oxygen-starved areas of the brain once served by the narrowed arteries. These many tiny blood vessels show up clearly on an angiogram, explaining the name for the disease; in Japanese, "moyamoya" means puff of smoke.

Patient's Story
Image The Lyons' Story
"At night, when they're sleeping, you think -- are they ok in there?" A fleeting concern for many parents, but Robin Lyons and her husband, Paul, are wondering if a rare illness is quietly, unsuspectingly stealing life from their children, Michael, 3, and Paul, 8.
More fragile than normal blood vessels, arteries in this network can also break and bleed into the brain, causing hemorrhages (In the brain, hemorrhage can be even more dangerous than elsewhere in the body, as brain tissue becomes irritated and inflamed and the pressure inside the skull increases), in certain patients.

Rare condition
Because the condition is so rare (only a few hundred cases are diagnosed each year), just a handful of medical centers have a substantial track record in treating it. Neurosurgeons at Children's Hospital Boston developed one of the most commonly used surgical repair procedures more than 20 years ago and have had extraordinary success in treating young patients with moyamoya.

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Related topics:
Angiogram
Brain: Anatomy
Embolization
see entire list
Moyamoya blood vessels
Appearance of Moyamoya blood vessels on an angiogram
Image Philanthropic dynamo Eight-year-old David Youngerman leads a happy, healthy life even after he was diagnosed with Moyamoya - a rare cerebrovascular disease caused by blocked arteries at the base of the brain...
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