Learn About Moyamoya Disease

What is the definition of Moyamoya Disease?

Moyamoya disease is a disorder of blood vessels in the brain, specifically the internal carotid arteries and the arteries that branch from them. These vessels, which provide oxygen-rich blood to the brain, narrow over time. Narrowing of these vessels reduces blood flow in the brain. In an attempt to compensate, new networks of small, fragile blood vessels form. These networks, visualized by a particular test called an angiogram, resemble puffs of smoke, which is how the condition got its name: "moyamoya" is an expression meaning "something hazy like a puff of smoke" in Japanese.

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What are the causes of Moyamoya Disease?

The genetics of moyamoya disease are not well understood. Research suggests that the condition can be passed through families, and changes in one gene, RNF213, have been associated with the condition. Other genes that have not been identified may be involved in moyamoya disease. It is also likely that other factors (such as infection or inflammation) in combination with genetic factors play a role in the condition's development.

How prevalent is Moyamoya Disease?

Moyamoya disease was first identified in Japan, where it is most prevalent, affecting about 5 in 100,000 individuals. The condition is also relatively common in other Asian populations. It is ten times less common in Europe. In the United States, Asian Americans are four times more commonly affected than whites. For unknown reasons, moyamoya disease occurs twice as often in females as in males.

Is Moyamoya Disease an inherited disorder?

Up to 15 percent of Japanese people with moyamoya disease have one or more family members with the condition, indicating that the condition can be passed through generations in families; however, the inheritance pattern is unknown. Research suggests that the condition follows an autosomal dominant pattern, which means one copy of the altered gene in each cell is sufficient to cause the disorder. However, some people who have a copy of the altered gene never develop the condition, which is a situation known as reduced penetrance.

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What are the latest Moyamoya Disease Clinical Trials?
Imaging Investigation on the Evolution and Prognosis of Moyamoya Disease

Summary: To investigate the evolution of imaging appearances and cognitive function of Moyamoya disease (MMD) and to establish a prognosis evaluation system based on imaging biomarkers in MMD. The study may be helpful to optimize and improve the diagnosis and pretreatment assessment of MMD, and provide an important theoretical supplement to the existing guidelines for the management of MMD.

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Creating a Florida Cerebrovascular Disease Biorepository and Genomics Center

Summary: The purpose of this study is to create a state-wide biorepository and resource center for cerebrovascular diseases in Florida, which will include collecting medical history information and blood from families affected by cerebrovascular disease. The information and blood samples collected may be used in future research for the study of cerebrovascular disease and to learn about, prevent or treat o...

Who are the sources who wrote this article ?

Published Date: October 01, 2017Published By: National Institutes of Health

What are the Latest Advances for Moyamoya Disease?
Moyamoya Disease in Infants and Toddlers.
Effect of Age, Stage, and Type of Surgical Revascularization on Clinical and Angiographic Outcome in Moyamoya Disease - Experience from a Case Series of 175 Revascularization Procedures.
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Direct Bypass Surgery for Moyamoya and Steno-occlusive Vasculopathy: Clinical Outcomes, Intraoperative Blood Flow Analysis, Long-term Follow-up, and Long-term Bypass Patency in a Single Surgeon Case Series of 162 Procedures.