What is Moyamoya disease?

Moyamoya disease is a rare brain condition that affects the blood vessels. Inside your head, you have major tubes called carotid arteries. These arteries act like main pipes, bringing blood and oxygen to the front two-thirds of your brain.

In people with Moyamoya disease, these main blood vessels become very narrow or completely closed. Because the vessels are blocked, your brain cannot get enough blood and oxygen it needs to work properly.

Over time, your brain tries to fix this problem on its own. It grows a bunch of new, tiny backup blood vessels to get around the blockage. However, because these backup vessels are rushed into service as emergencies, they are small, weak, and fragile. They often fail to bring enough blood to the brain, and they can easily tear or bleed. This can lead to brain bleeding or strokes.

What does "Moyamoya" mean?

The word "Moyamoya" comes from Japan, and it means "puff of smoke." When doctors first used specialized X-ray scans to examine the brain vessels of patients with this condition, they noticed something unusual. The tiny, tangled backup blood vessels looked just like a blurry puff of smoke on the scan.

How rare is this disease?

Moyamoya is a very uncommon condition. It is found most often in East Asian countries, but doctors are now finding it in people of all backgrounds and ethnicities.

What are the symptoms of Moyamoya disease?

Most symptoms happen because the brain is starving for blood and oxygen. The very first sign is usually a stroke or a "mini-stroke" (which doctors call a TIA, or transient ischemic attack).

Other common symptoms include:

• Headaches: Severe or recurring pains in the head.
• Seizures: Impulsive, uncontrolled electrical disturbances in the brain.
• Involuntary movements: Parts of your body moving or twitching on their own without your control.
• Mental changes: Trouble learning, memorizing things, or paying attention.
• Developmental delays: Children lagging behind in normal growth or learning milestones.
• Sensation problems: Numbness, tingling, or strange feelings in the body.
• Aneurysms: Weak spots in the blood vessels that balloon out and might burst.

What causes Moyamoya disease?

Doctors and scientists do not yet know the exact cause of Moyamoya disease. However, they believe it can be passed down through family genes, or it can occur after an infection or severe inflammation.

Sometimes, Moyamoya happens alongside other health conditions. When it is linked to another existing medical problem, doctors call it Moyamoya syndrome. Some of these linked conditions include:

• Down syndrome
• Sickle cell disease
• Graves' disease (a thyroid problem)
• Neurofibromatosis type 1 (a genetic nerve disorder)
• Atherosclerosis (hardened and clogged arteries)

Is it a genetic condition?

Yes, it seems to be heavily linked to genetics. Scientists discovered changes in a specific gene called Ring Finger Protein 213, which helps build blood vessels. In about 15% of people with the disease, another blood relative has it too. While it runs in families, doctors still do not fully understand exactly how it passes from parents to children.

Who gets Moyamoya disease?

Anyone can get it, but certain groups are more likely to develop it:

• Gender: Females are twice as likely to get it as males, though we do not know why.
• Age: It can happen at any age, but it usually peaks in two groups: young children between 5 and 10, and adults between 30 and 50.

What are the complications?

If the brain goes without enough blood for too long, it can cause lasting damage. Complications include:

• Paralysis (not being able to move parts of your body)
• Trouble speaking or understanding words
• Vision problems
• Long-term brain injury
• Trouble walking or balancing

Diagnoses and Tests

How will a doctor know if you have Moyamoya disease?

A doctor will start by examining you and asking about your medical history. If they think your brain vessels are narrow, they will order special imaging tests:

• MRI (Magnetic Resonance Imaging): A painless scan that uses magnets and radio waves to take detailed pictures of your brain. It shows if your brain has suffered any damage from a lack of blood.
• MRA (Magnetic Resonance Angiography): This is similar to an MRI, but it uses a computer to focus specifically on the shape and health of your blood vessels.
• Cerebral Arteriography: For this test, a doctor slides a tiny, flexible tube into a blood vessel in your leg or arm and guides it up toward your neck. They release a safe dye into your bloodstream and take X-rays. This creates a detailed map of your blood flow and shows exactly where the narrowing is.

What are the stages of Moyamoya disease?

Moyamoya is a progressive disease, meaning it will get worse over time if it is not treated. Doctors use six stages to describe how far the disease has advanced. They look at your scans to see which stage you are in:

• Stage 1: The main internal carotid artery just begins to narrow.
• Stage 2: The narrowing spreads to nearby branches, and the tiny "puff of smoke" backup vessels start to form.
• Stage 3: The narrowing gets worse, and the "puff of smoke" vessels become very thick and highly visible.
• Stage 4: The tiny backup vessels start to shrink and fade away. The brain starts trying to get blood from vessels outside the skull instead.
• Stage 5: The original backup vessels shrink further, and external blood vessels take over more of the job.
• Stage 6: The main internal carotid artery is completely blocked, and the original "puff of smoke" vessels vanish entirely. The brain now relies entirely on blood vessels from outside the skull.

What is the treatment for Moyamoya disease?

There is no cure that can reverse arterial narrowing, so treatment focuses on managing symptoms and protecting the brain.

A doctor may prescribe several types of medications:

• Aspirin: This helps prevent blood clots from forming in the narrow or fragile backup vessels.
• Antiseizure medicines: These help prevent and control seizures caused by poor blood flow to the brain.
• Blood thinners (Anticoagulants): These help keep blood flowing smoothly to prevent clots. Because they can cause dangerous bleeding if you fall or get hurt, doctors only use them in very specific situations.
• Calcium channel blockers: These can help ease severe headaches. However, they can lower your blood pressure, which might actually increase your stroke risk, so doctors use them with extreme caution.

When is surgery needed?

Since medicine cannot open the blocked arteries, the disease will usually continue to progress. If symptoms do not stop, a doctor will recommend brain surgery.

This surgery is called a bypass. During the procedure, a surgeon takes a healthy blood vessel from your scalp and connects it directly to the brain. This creates a new route for blood to flow, bypassing the blocked areas. Your doctor will talk with you about the best surgical option, the side effects, and the risks involved.

What should I expect if I have this condition?

For most people, the condition gets worse over time. Without proper treatment, the risk of a major stroke or permanent disability is very high. However, some people can stay stable for years without any severe symptoms. If you get diagnosed early and receive the right treatment or surgery, you can live a long, normal, and healthy life.

Can Moyamoya disease be prevented?

You cannot prevent the genetic type of Moyamoya disease since it is built into your DNA. However, you can protect your blood vessels from getting worse. You can do this by managing your overall health—eating a good diet, exercising, keeping your blood pressure under control, and taking your medications exactly as your doctor tells you to.

What are the emergency signs of this disease?

If you have Moyamoya disease, you must know the warning signs of a stroke so you can get help instantly.

Remember the word F.A.S.T. to help you spot a stroke right away:

• F – Face drooping: Look in the mirror or ask someone to smile. Does one side of the face droop or feel numb? Is the smile uneven?
• A – Arm weakness: Try to raise both arms straight up. Does one arm drift downward? Is one arm weak or numb?
• S – Speech difficulty: Try to speak. Is your speech slurred or hard to understand? Can you repeat a simple sentence?
• T – Time: If you see any of these signs, even if they go away, you need to call for an emergency ambulance or go to the nearest hospital immediately. Every single minute counts.

Other urgent warning signs that mean you need to go to the hospital right away include:

• Sudden confusion or trouble understanding people.
• Sudden numbness or weakness in your leg, face, or arm, especially on just one side of your body.
• Sudden trouble seeing out of one or both eyes.
• Sudden dizziness, loss of balance, or trouble walking.
• A sudden, incredibly severe headache that comes out of nowhere with no known cause.

Why choose Tender Palm Super Speciality Hospital for Moyamoya disease treatment in Lucknow, India?

Tender Palm Super-Speciality Hospital offers advanced Moyamoya Disease treatment in Lucknow, India, at an affordable cost. We have a team of experienced neurologists, neurosurgeons, neurovascular specialists, and rehabilitation experts who provide accurate diagnosis, personalized neurological care, and advanced treatment for Moyamoya Disease. Our neurosciences team has extensive experience in successfully treating Moyamoya Disease in Lucknow, India.

To seek an Expert Consultation for Moyamoya Disease Treatment in Lucknow, India:

Call us at +91-9076972161
Email at care@tenderpalm.com

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