Have you ever had a sudden, severe headache or a feeling of numbness on one side of your body? Sometimes there can be reasons behind these things that you can't even imagine. Today we're going to talk about a rare but very important medical condition. It's called Moyamoya Disease . The name sounds a bit strange, doesn't it? Let's see what it is.
What is Moyamoya Disease?
Simply put, Moyamoya Disease is a condition that affects the blood vessels in your brain. To be precise, it is a cerebrovascular disease . The main blood vessels that supply blood to your brain are the carotid arteries , which run from the sides of your neck to your brain. In this disease, one or both of these arteries gradually become narrowed or completely blocked. It can also affect the cerebral arteries , which supply blood to the front and middle parts of your brain.
Think about it, about two-thirds of the front of our brain is supplied with blood and oxygen by these major blood vessels. So what happens when these narrow? The amount of blood and oxygen needed by the brain decreases. It's like when a water pipe gets clogged, the water flow decreases.
Now our bodies are amazing. When a major blood vessel is blocked like this, the brain tries to make up for the shortfall by forming new, smaller blood vessels. But these new blood vessels are not as strong as the main blood vessels. They are often weak and small . It's like a major road being blocked and vehicles are being diverted through small side roads. These small blood vessels may not be able to supply the brain with enough blood. Also, these weak blood vessels can sometimes burst. This can cause brain bleeds and strokes .
What does the name "Moyamoya" mean?
"Moyamoya" is a Japanese word. It means "a plume of smoke" or "like a puff of smoke." Japanese doctors were the first to discover this disease. When they did a special scan of the blood vessels, called an angiogram , they saw that the newly formed small blood vessels were curved like plumes of smoke, or puffs of smoke. That's why this disease was named "Moyamoya disease." Isn't that a wonderful name?
How rare is Moyamoya Disease?
This is actually a very rare disease. It is most commonly seen in Japan. It is said that there is an average of five people per hundred thousand who develop this disease. Even in a country like America, there are fewer than 5,000 people with this disease. So it can be assumed that this is very rare in Sri Lanka as well. But from time to time, such patients are reported.
What are the symptoms of Moyamoya Disease?
The symptoms of this disease are caused by a decrease in blood flow to the brain. Often, the first time you notice this disease is when you have a stroke , or when you have a series of transient ischemic attacks (TIAs) . These TIAs are sometimes called "mini-strokes" by doctors. This means that the blood flow to the brain is temporarily interrupted and then restored. But this is a warning sign.
Other symptoms may include:
- Brain hemorrhage: This can be caused by the bursting of the weak small blood vessels we mentioned earlier.
- Headache: Frequent, sometimes severe headaches may occur.
- Developmental delays: Especially if young children develop this disease, their development may be slower than other children.
- Aneurysm: A balloon-like bulge in a part of a blood vessel. These can burst.
- Involuntary movements: Uncontrollable shaking of things like limbs.
- Problems with cognitive abilities: For example, difficulty learning, remembering, and concentrating.
- Sensory problems: Changes in vision, hearing, smell, touch, and taste may occur.
- Seizures: Seizure-like conditions.
You may have one or more of these symptoms. Not everyone will experience these symptoms in the same way.
What causes Moyamoya Disease?
In fact, researchers have not yet been able to find out exactly what causes this disease. However, it has been found that it has a genetic influence, and sometimes it can be an acquired condition due to other reasons. Research is still being done on the genes that can pass this disease from parents to children. It is also suspected that certain things such as inflammation or infection in the body can cause it.
Sometimes, these Moyamoya symptoms can occur with other medical conditions. This is called Moyamoya syndrome or Moyamoya phenomenon. For example:
- Down syndrome
- Graves' disease ( a disease related to the thyroid gland)
- Neurofibromatosis type 1 (genetic condition)
- Sickle cell disease ( a blood disease)
- Atherosclerosis (fatty deposits in blood vessels)
People with other diseases like these are more likely to develop symptoms of Moyamoya.
Is Moyamoya Disease genetic?
Yes, researchers have found that mutations , or changes, in a gene called RNF213 may be responsible for some cases of moyamoya. A protein produced by this gene called RNF213 is thought to be involved in blood vessel growth.
About 15% of Japanese people with Moyamoya disease have a family member who has the disease, suggesting a genetic link. However, the exact inheritance pattern is still unclear.
Who is more likely to develop Moyamoya Disease?
As we mentioned earlier, this is a rare disease. Although moyamoya disease, which is caused by genetic factors, is more common among Japanese people, it is now being reported among other ethnic groups as well.
Although scientists still don't understand the exact cause, women are about twice as likely to develop the disease as men.
Moyamoya disease can develop at any age. However, children between the ages of 5 and 10 and adults between the ages of 30 and 50 are most likely to develop the disease.
What are the possible complications of this disease?
If moyamoya disease is not treated properly, various complications can occur. These include:
- Paralysis
- Vision problems
- Speech problems
- Movement disorders
- Permanent brain damage
This is why it is important to recognize and treat the disease quickly.
How do you know if you have Moyamoya Disease?
If you have symptoms of moyamoya disease, a doctor will examine you and ask about your symptoms. Then, if moyamoya disease is suspected, they may order tests such as:
- Cerebral arteriography: In this test, a small tube, called a catheter, is inserted into an artery in your arm or leg. A special liquid containing iodine (contrast dye) is then injected into the bloodstream through the catheter. An X-ray is then taken to see how the liquid moves through the blood vessels. This allows you to see how narrow the blood vessels are and how the blood is flowing.
- Magnetic Resonance Angiography (MRA): This is a painless test that uses a magnetic field, radio waves, and a computer to look at your blood vessels.
- Magnetic Resonance Imaging (MRI): This is a similar technique to MRA. It takes pictures of the inside of your body and can assess the effects of the disease on your brain.
These tests are what allow doctors to tell exactly whether you have Moyamoya disease or not.
What are the stages of Moyamoya Disease?
Moyamoya disease is a progressive disease. This means that if left untreated, the disease will gradually get worse. Doctors use a classification called "Suzuki stages" to describe the severity of the disease. They use the information from the MRA scan to determine which stage of the disease you are in. These Suzuki stages, in simple terms, are as follows:
- Stage 1: There is a narrowing or blockage only in the inner part of the carotid artery.
- Stage 2: All the apical branches of the carotid artery have begun to thin out. Those "smoky" blood vessels (the small, smoky-looking ones) are barely visible on the angiogram.
- Stage 3: The blood vessels are clearly visible on the angiogram. You can clearly see that "smoke plume" appearance.
- Stage 4: The blood vessels in the dura mater gradually begin to diminish. In their place, alternative blood vessels (transdural collateral vessels) begin to form.
- Stage 5: The number of blood vessels decreases further, and more alternative blood vessels develop.
- Stage 6: The blood vessels in the brain have almost completely disappeared. The carotid artery is completely narrowed or blocked.
Understanding these stages accurately helps doctors plan treatment.
What are the treatments for Moyamoya Disease?
When treating moyamoya disease, the main goal is to control the symptoms and try to prevent the condition from getting worse. For this, your doctor may prescribe you some medications:
- Aspirin: Aspirin helps prevent or reduce blood clots in those newly formed small, weak blood vessels.
- Antiseizure medications: These are given to prevent epileptic seizures (convulsions) caused by epilepsy.
- Anticoagulants: These are blood thinners. They help prevent blood from clotting. However, these medications have a risk, which is that if bleeding occurs, it can be difficult to stop. Therefore, doctors give these ވަރަށް cautiously, only in special cases.
- Calcium channel blockers: These can help reduce headaches caused by migraines. However, these medications can also lower blood pressure, which can increase the risk of stroke. Therefore, they are only used in special cases.
But remember, these medications cannot stop the narrowing of the blood vessels. Therefore, the disease may gradually worsen. If this happens, your doctor may suggest you have bypass surgery . This surgery:
- Creating a new blood route using healthy arteries in the scalp, bypassing the blocked artery.
- Redirecting blood flow to the affected parts of the brain.
- Opening up narrowed blood vessels.
Your doctor will explain to you in detail which surgical procedure is best suited for your situation, its side effects, and possible complications.
Can Moyamoya Disease be prevented?
There is no proven way to prevent Moyamoya syndrome, which is caused by genetic factors. However, the risk of developing Moyamoya syndrome can be reduced by controlling risk factors related to blood vessels and reducing the risk of atherosclerosis .
Also, if you have Moyamoya disease, taking the medication prescribed by your doctor exactly and as prescribed can help control your symptoms and prevent complications.
What can someone with this disease expect?
For many people, Moyamoya disease gradually worsens over time. As the blood vessels become narrower, they can even have a stroke . If left untreated, Moyamoya disease can lead to severe neurological damage, stroke, and even death.
However, some people can remain stable for years without any symptoms. When symptoms begin to appear, the doctor may suggest surgery.
What is the life expectancy of someone with Moyamoya Disease?
If the disease is diagnosed early and treatment begins quickly, people with Moyamoya disease can live a normal life.
However, if left untreated, this disease can be fatal. That's why early diagnosis and treatment are so important.
When should someone with Moyamoya Disease seek emergency medical attention?
If you experience any signs of a stroke , you should seek emergency medical attention immediately . Time is of the essence here.
The American Stroke Association uses an acronym called FAST to make it easier for people to remember. Let's look at what it means in Sinhala:
- F - Face droop: Does one side of your face feel numb? If you try to smile, does one side of your face droop?
- A - Arm weakness: Can you raise both arms? Does one arm drop down?
- S - Speech difficulty: Is your speech slurred? Or is it slow? Can you repeat a simple sentence?
- T - Time to call emergency services: If you see any of these signs of a stroke, call 911 immediately and get an ambulance, or go to the hospital right away. Time is of the essence!
Other symptoms of a stroke may include sudden onset of:
- Confusion
- Numbness on one side of the body
- Vision problems
- Difficulty walking
- Very severe headache
Moyamoya Disease is a rare condition that can lead to strokes and brain hemorrhages. Although researchers have not yet identified the exact cause, it is more common in Japanese people. If you have any of the symptoms of Moyamoya, see a doctor immediately. Early diagnosis and treatment of Moyamoya Disease can be the difference between life and death. It is also important to know the symptoms of a stroke and take immediate action if one occurs.
Summary (Take-Home Message)
So, I hope you now have some understanding of the Moyamoya Disease we talked about today. Here are some important things to remember:
- This is a rare disease that causes blood vessels in the brain to narrow.
- Symptoms such as stroke , headache , and seizures may occur.
- It is very important to recognize and treat the disease quickly.
- Treatment includes medications and, if necessary , bypass surgery .
- Being aware of the symptoms of a stroke (FAST) can be lifesaving.
If you or someone you know has these symptoms, please seek medical advice. Don't worry, there are solutions for everything. The most important thing is to act quickly.
` Moyamoya, brain diseases, stroke, blood vessels, neurological diseases, genetic diseases, headaches
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