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What is Moyamoya Disease?

Moyamoya disease is a rare and progressive disorder. According to the National Institute of Neurological Disorders and Stroke, it is caused by blocked arteries at the base of the brain in an area called the base ganglia. Moyamoya was first described in Japan in the 1960s and, in Japanese, its name means "puff of smoke", which describes how the tangle of blocked arteries appear in an x-ray. Since its original description in the 1960s, Moyamoya has also been found in the US, Europe, Australia, and Africa.

Primarily, Moyamoya affects children, with a stroke or recurrent transient ischemic attacks (TIA) appearing as the first symptom. These symptoms are often accompanied by paralysis affecting one side of the body, muscular weakness, or seizures. Moyamoya also affects adults, who - due to recurring blood clots in the affected brain vessels - experience a hemorrhagic stroke. Some other symptoms include disturbed consciousness, speech deficits, sensory/cognitive impairments, involuntary movements, and vision problems.

Currently, there are several types of revascularization surgery that can help the blood flow to the brain. Usually, children respond better to the surgery than do adults. Without treatment, Moyamoya disease can be fatal.

The following organizations can provide more information on Moyamoya and clinic trials, as well as provide support to people with Moyamoya and their families.