MOYAMOYA DISEASE Meaning and
Definition
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Moyamoya disease is a rare and progressive cerebrovascular disorder characterized by the narrowing and blockage of the arteries at the base of the brain. The name "moyamoya" is derived from the Japanese term meaning "puff of smoke," which describes the appearance of the small, tangled blood vessels that develop as collateral circulation in response to the blocked arteries.
This condition primarily affects the internal carotid arteries, which are responsible for supplying oxygen-rich blood to the brain. The progressive narrowing of these arteries restricts blood flow, leading to insufficient oxygen and nutrient delivery to the brain tissue. As a result, the body responds by creating abnormal collateral blood vessels to compensate for the blocked arteries. Unfortunately, these collateral vessels are prone to rupture and can cause serious complications such as hemorrhagic strokes.
Moyamoya disease often presents with symptoms such as recurrent transient ischemic attacks (TIAs), strokes, and bleeding in the brain. These symptoms typically manifest as weakness or paralysis on one side of the body, difficulty speaking, severe headaches, and seizures. Additionally, children with moyamoya disease may experience cognitive and developmental delays.
While the exact cause of moyamoya disease remains unknown, certain genetic and environmental factors may contribute to its development. The condition is predominantly seen in individuals of Asian descent, and it can affect both children and adults. Treatment options for moyamoya disease include surgical interventions aimed at improving blood flow, such as direct bypass procedures and indirect revascularization techniques, as well as medications to manage associated symptoms and prevent complications. Regular monitoring and follow-up care are crucial in managing this chronic condition.
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Etymology of MOYAMOYA DISEASE
The term "Moyamoya Disease" has its origins in the Japanese language. "Moyamoya" (もやもや) translates to "hazy" or "puff of smoke" in English. It is a descriptive term used to depict the appearance of the tiny blood vessels formed to compensate for the blockage of larger vessels in the brain, which resemble a hazy or puff of smoke when observed on medical imaging. The term was first coined by Japanese doctors Takeuchi and Shimizu in 1969 to describe the unique angiographic appearance of the disease.
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