Moyamoya Disease

• The first description of the moyamoya syndrome was done in Japanese and therefore it has been named after the Japanese word for "puff of smoke".

• A brain scan shows a cloud of blood vessels tangle. Initially, there is a narrowing of blood vessels, causing branches and more branches of blood vessels-tangle grow around the brains to provide  oxygen and nutrition.

On the left-hand side: "puff of smoke" tangle of blood vessels

On the right-hand side: Process of vasoconstriction and vascular tangle formation during moyamoya.

• If the brains get delivered too little oxygen through these blood vessels, they exhibit TIAs (Trans Ischaemic Accident) see also the page about cerebral hemorrhage.

• This can cause a stroke if the blood capillaries tangle don’t manage to cope with the blood supply that is required.

• In English the disease is also called arterial occlusive disease which means it is a disease of occluded arteries.

• Although the disease is most commonly seen among children of 0-10 years and usually among children of Asian descent and for women in their thirties it occurs for all ages.

• In part of the cases, the syndrome is caused by an error in the genetic material. However, several factors are required to actually develop moyamoya. By this, when one child of a family has developed it, it is difficult to indicate whether other children of the family can get the disease as well. For this reason sometimes a genetic consultation is done.

Read more about Moyamoya on the website of NIH (the National Institute of Neurological Disorders an Stroke (in the U.S)).

A video with an explanation about the disease: