Moyamoya Disease

We don’t know exactly what causes moyamoya disease. You can be born with it (congenital) or it can develop over time (acquired). You may also have moyamoya disease with other health conditions. When this happens, we call it moyamoya syndrome or phenomenon. Some of these conditions could include:

• Down syndrome.
• Graves’ disease.
• Neurofibromatosis type 1.
• Sickle cell disease.
• Atherosclerosis.
• Radiation vasculopathy.

When you come for your first appointment, the first thing we’ll do is give you a physical exam to rule out other conditions that could be causing your symptoms. If your providers think you might have moyamoya disease, they’ll do special tests to learn more about what’s going on in your brain.

MRI

An MRI scan can’t diagnose moyamoya by itself, but it can give us a noninvasive way to take a detailed look inside your brain. These images offer valuable information that can help us make an accurate diagnosis. Our high field MRI imaging capabilities allow us to get the highest clarity and resolution in understanding the impact of moyamoya on your brain. 

Magnetic resonance angiography (MRA)

An MRA is a type of MRI that focuses on your blood vessels. Your providers may use an MRA to check how well blood is flowing to your brain and to see if anything looks unusual. Our advanced MRA imaging techniques allow us to visualize the underlying process happening in your brain blood vessels.

Cerebral angiography

During cerebral angiography, a radiologist will insert a small tube (catheter) into an artery in your arm or leg so they can inject a special contrast dye (iodine) into your bloodstream. They then use X-rays to create a “map” of how your blood flows through your blood vessels and show any narrowing. This minimally invasive test can also show us moyamoya disease’s characteristic “backup” blood vessels, which look like a “puff of smoke.” In fact, moyamoya means “puff of smoke” in Japanese.

Meet Our Moyamoya Disease Team

At Cleveland Clinic, you’ll have a care team of experienced healthcare providers from different specialties on your side. They’re experts at diagnosing and treating even the rarest brain blood vessel conditions, like moyamoya disease. Your care team could include:

• Vascular neurologists.
• Cerebrovascular neurosurgeons.
• Neuroradiologists.
• Physical therapists.
• Occupational therapists.
• Speech-language pathologists.
• Physician assistants.
• Nurse practitioners.
• Social workers.
• Patient care coordinators or nurses.

Moyamoya disease is a lifelong condition that may get worse over time. But with treatment, you can often remain stable for years without symptoms. If your providers confirm that you have moyamoya disease, they’ll start creating a treatment plan to match your specific needs and that takes into account things like:

• Your age and lifestyle.
• Your test results.
• Your risk of stroke or other complications.
• Your symptoms, like headaches, seizures or transient ischemic attacks (TIAs).

Medications

We can often treat milder forms of moyamoya disease with aspirin and other medications — combined with regular appointments and testing to make sure it doesn’t get worse. If your providers think medications are a good choice for you, they could prescribe:

• Aspirin: This can lower your risk of getting blood clots in the narrowed, “backup” blood vessels that form with moyamoya disease.
• Antiseizure medications: If you have frequent seizures, these medications (anticonvulsants) can prevent you from having them or reduce how many you have.
• Blood thinners: These medications (anticoagulants) thin your blood to prevent dangerous clots. They do have some risks, so your providers will only prescribe them if needed.
• Calcium channel blockers: These medications can manage headaches and symptoms of TIAs, like dizziness or numbness. They can also lower your blood pressure, which can sometimes decrease your risk of a stroke. Your provider will decide if you need a calcium channel blocker.

Surgery

If you’ve had frequent TIAs and/or other severe symptoms, you’re at a higher risk for blockages to get worse. And that means you could have a major stroke or bleeding in your brain. If that’s the case your provider may decide to do cerebral bypass surgery. This can help restore blood flow to your brain. Successful surgery can lower your risk of a stroke, brain bleed or other complications. It also can reduce or even eliminate your symptoms.

We try to use minimally invasive methods whenever we can to reduce scarring and complications. Minimally invasive options can also mean a faster recovery. Our surgeons make small incisions in your head and use tiny, special surgical tools and a camera to bypass (skip over) your blocked arteries and redirect blood flow to your brain using a healthy blood vessel from your scalp. This is called direct cerebral revascularization.

We might also use indirect revascularization and surround your brain with healthy tissue rich in blood vessels.

Follow-up Care at Cleveland Clinic

Because moyamoya disease is a condition that’s treatable but ongoing, you’ll continue to meet periodically with your care team for the rest of your life. We’ll keep a close eye on you and what’s happening with the blood vessels in your brain. If you’re taking medications, you’ll stay on them for as long as your provider feels is necessary — possibly for life. And you’ll learn ways to maintain your vascular health and how to keep tabs on your symptoms so you can recognize if they’re getting worse — or if you’re having a stroke.

Taking the Next Step

Moyamoya disease is a serious diagnosis, but it doesn’t have to slow you down. With the right care, you can reduce your risk of strokes and other serious symptoms that keep you from doing the things you love. At Cleveland Clinic, our providers are experts at diagnosing and treating this rare chronic condition. They’ll make sure you get the skilled, compassionate and personalized care you need to move forward with your life.