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What Is Moyamoya Disease?

Moyamoya Disease is a rare, progressive cerebrovascular disorder. It is caused by the narrowing or blockage of the major arteries at the base of the brain, most commonly the internal carotid arteries. As these major vessels narrow, blood flow to the brain is dangerously reduced, increasing the risk of stroke. To compensate, the brain spontaneously grows a tangled network of tiny, fragile blood vessels nearby. The Japanese term Moyamoya means "puff of smoke," which is what this network of fragile collateral vessels looks like on an X-ray.

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What Is Moyamoya Disease?

Moyamoya Disease is a rare, progressive cerebrovascular disorder. It is caused by the narrowing or blockage of the major arteries at the base of the brain, most commonly the internal carotid arteries. As these major vessels narrow, blood flow to the brain is dangerously reduced, increasing the risk of stroke. To compensate, the brain spontaneously grows a tangled network of tiny, fragile blood vessels nearby. The Japanese term Moyamoya means "puff of smoke," which is what this network of fragile collateral vessels looks like on an X-ray.

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Symptoms and Risk of Stroke

The condition can cause two main types of neurological events:

  1. Transient Ischemic Attacks (TIAs) or Strokes: The most frequent symptoms, especially in adults, happen due to low blood flow, causing sudden weakness, trouble speaking, or changes in vision.
  2. Hemorrhagic Strokes: In children, the new, fragile Moyamoya vessels can rupture, causing bleeding in the brain. Symptoms are often triggered by crying, activity, or eating, which alters blood flow dynamics.

Diagnosis and Treatment

Diagnosis requires a brain imaging scan (like an MRI or cerebral angiography) to visualize the narrowed arteries and the "puff of smoke" vessel network. Because of the high risk of stroke, treatment is almost always surgical, often involving a revascularization procedure. The goal is to bypass the blocked major arteries by connecting a healthy artery from the scalp to the brain surface, dramatically increasing blood flow and reducing the risk of a future stroke.

Long-Term Prognosis

Without surgical intervention, the risk of a stroke or brain hemorrhage is extremely high. With revascularization surgery, the long-term prognosis is significantly improved, especially for children. Long-term care involves regular neurological follow-ups and monitoring, but the surgery often stabilizes the condition and allows for a substantial reduction in stroke risk.

Call Your Doctor Now

If you or your child experience recurrent, unexplained mini-strokes (TIAs), chronic headaches, or dizziness, see a neurologist immediately. Moyamoya is a progressive vascular condition that requires prompt diagnosis via angiography. Early revascularization surgery is often critical to prevent catastrophic stroke.

Frequently Asked Questions About Moyamoya Disease

Is Moyamoya Disease curable?

While the disease cannot be cured, surgical revascularization procedures can prevent future strokes and are highly effective in managing the disease and preventing its progression.

Is Moyamoya Disease contagious or genetic?

It is not contagious. While most cases occur spontaneously, there is a clear genetic link, and it is more common in East Asian populations, though it affects all races.

What is the difference between Moyamoya Disease and Moyamoya Syndrome?

Moyamoya Disease is when the cause is unknown. Moyamoya Syndrome is when the condition is caused by a known underlying disorder, such as sickle cell disease, Down syndrome, or certain vascular disorders.