Have you ever thought about how amazing and complex our brain is? This tiny organ controls our every thought, every movement, and every memory. So, the brain needs a continuous, uninterrupted flow of blood that provides oxygen and nutrients. But imagine what happens if the main arteries that carry blood to the brain become blocked? Today we are going to talk about one such rare, but very important disease to be aware of. That is Moyamoya Disease.
Simply put, what is Moyamoya?
Stroke is a disease that affects the blood vessels in our brain. Medically speaking, it is a cerebrovascular disease. There are two main blood vessels in our neck that carry blood to the brain, called the carotid arteries. In stroke, one or sometimes both of these main arteries gradually narrow and become blocked. It can also affect another important artery in the brain, the cerebral arteries. These are the main blood vessels that supply blood to the front parts of our brain.
Imagine that the main water pipe that brings water to your house is blocked. What happens then? There is no water. So, it tries to get water from the small pipes inside the house. Our brain is the same. When the main blood vessel is blocked, the brain creates many new small, thin blood vessels to make up for the lack. These are like side roads. But the problem is, these new blood vessels are very weak and thin. Therefore, they cannot be supplied with blood as well as a main vein. These weak veins can sometimes burst. In such cases, Moyamoya Disease can also cause bleeding inside the brain (Brain hemorrhage). Also, serious conditions like stroke can occur because the brain does not get enough blood.
Why is this called "Moyamoya"?
"Moyamoya" is a Japanese word. It means "like a puff of smoke." When the Japanese doctors who first discovered this disease performed an angiogram of the blood vessels in the brain, they saw that the network of newly formed small blood vessels was tangled like a puff of smoke. That's why this disease was named "Moyamoya."
What are the symptoms of moyamoya disease?
The main symptoms of this disease are caused by a decrease in blood supply to the brain. Sometimes the disease is first recognized after a stroke or several transient ischemic attacks (TIAs). These small strokes are sometimes called "mini-strokes." This means that the symptoms of a stroke come and go for a while.
Let's look at some of the common symptoms that can be seen in this disease.
| Symptom | A simple explanation |
|---|---|
| Stroke or mini-strokes (TIAs) | Symptoms such as numbness on one side of the body, difficulty speaking, and drooping of one side of the face. These symptoms usually resolve after a short time in TIAs. |
| Brain hemorrhage | Bleeding in the brain occurs when a weak, small blood vessel bursts. This can cause a severe headache. |
| Headache | Frequent, sometimes severe headaches. |
| Seizures | The occurrence of fit-like conditions. |
| Uncontrolled movements | Body parts, such as limbs, move uncontrollably. |
| Developmental delays | If this disease affects young children, in particular, they may experience delays in their development and learning. |
| Sensory problems | Problems with vision, hearing, smell, touch, or taste. |
What causes Moyamoya disease?
In fact, the exact cause of this disease has not yet been found . But researchers believe that it has a genetic link. That is, it can be passed down through generations. Since this disease is especially common among Japanese people, it has been found that there is some connection in their genes. It has now been identified that changes in a gene called `RNF213` may contribute to this.
Sometimes, this Moyamoya phenomenon can occur with other medical conditions. Then we call it Moyamoya syndrome. For example:
- Down syndrome
- Graves' disease
- Neurofibromatosis type 1
- Sickle cell disease
- Atherosclerosis
A person with diseases such as are at risk of developing a condition called Moyamoya.
Who is most likely to get this disease?
Moyamoya is a very rare disease . It is rarely reported in countries like Sri Lanka. But it is important to be aware of this disease.
- This disease can develop at any age, but it is most common in children between the ages of 5 and 10 and adults between the ages of 30 and 50 .
- Although the exact cause is unknown, women are about twice as likely to develop this disease as men.
- This disease is commonly seen among people in Japan and other East Asian countries.
How do you accurately diagnose this disease?
If you have the symptoms we discussed earlier, your doctor will first examine you and ask you about your symptoms. Then, if there is a suspicion of moyamoya disease, they will recommend some special tests to confirm it.
| Test | What do you do with this? |
|---|---|
| Cerebral arteriography | This is the best test to diagnose this disease. In this test, a small tube (catheter) is inserted into an artery in the arm or leg, and a special dye is injected into the blood vessels through it. X-ray images are then taken to see how thin the blood vessels are and how well the blood is flowing. |
| MRA Scan (Magnetic Resonance Angiography) | This is a painless test that uses a magnetic field and radio waves to take detailed pictures of the blood vessels in the brain. |
| MRI scan (Magnetic Resonance Imaging) | A technique similar to MRA is used to examine the condition of brain tissue. This can help determine if there is any damage to the brain due to reduced blood flow. |
Is there a treatment for moyamoya disease?
Yes, there are treatments to manage this disease. There are two main goals of treatment. One is to control symptoms. The other is to improve blood flow to the brain and reduce the risk of serious conditions like stroke.
1. Treatment with medication
The doctor may prescribe various medications depending on the condition of the patient.
- Aspirin: This medication helps prevent blood clots and facilitate blood flow through those small blood vessels.
- Antiseizure medications: If the disease causes epileptic seizures, these medications are given to control them.
- Anticoagulants: Although these help prevent blood clots, doctors only recommend these in very special cases because of the risk of bleeding.
- Calcium channel blockers: These can help reduce symptoms like headaches. However, they can also lower blood pressure, which may increase the risk of stroke, so they should be used with caution.
The most important thing is that medications cannot stop the thinning of blood vessels , so the disease can gradually worsen.
2. Surgical treatment (Bypass Surgery)
If the disease cannot be controlled with medications, and if it gradually worsens, the doctor may recommend surgery. This is called bypass surgery.
Simply put, this involves creating a new route for blood to flow to the brain, bypassing a blocked blood vessel. This involves taking a healthy artery that usually supplies blood to the scalp and connecting it to a blood vessel in the brain. It's like building a new flyover to bypass a busy road. This surgery can increase the amount of blood that reaches the brain, greatly reducing the risk of stroke.
Your doctor will explain to you in detail which surgical procedure is best for you, and what its advantages and disadvantages are.
When to seek emergency medical help? Stroke symptoms (FAST)
It is extremely important for someone with Moyamoya disease to be aware of the symptoms of a stroke . The sooner treatment is received, the less damage to the brain can be prevented. The acronym FAST is used to help you remember the symptoms of a stroke.
| Stroke Symptoms (FAST) - Act Immediately! | |
|---|---|
| F - Face | Does one side of your face feel numb? Does one side of your face twitch when you smile? |
| A - Arms | Can you raise both arms? Does one arm fall down? |
| S - Speech | Does the conversation get confusing? Do you stutter? Is it difficult to say a simple sentence? |
| T - Time | If you have any of these symptoms, don't delay. Go to a hospital's Emergency Department (ETU) immediately or call an ambulance. Time is of the essence! |
In addition to these, symptoms such as sudden confusion, numbness on one side of the body, vision problems, difficulty walking, or a severe, unbearable headache can also be symptoms of a stroke.
When living with this disease, if the disease is diagnosed early and treated properly, most people can live a normal life. If left untreated, the disease can become severe and even fatal. Therefore, it is very important to be aware of the symptoms and follow the doctor's instructions exactly.
Take-Home Message
- Moyamoya disease is a rare condition in which the main artery supplying blood to the brain gradually narrows and becomes blocked.
- This can lead to a decrease in blood supply to the brain, leading to serious conditions such as stroke or brain hemorrhage.
- The main symptoms include stroke-like symptoms, headaches, and seizures.
- The disease can be successfully managed by early detection and treatment with medication or surgery.
- If you or someone you know is experiencing symptoms of a stroke (FAST), you should go to a hospital's Emergency Department (ETU) immediately. Wasting time can increase brain damage.
- If you have any doubts or questions about this, talk to your doctor for advice.
💬 අදහස් (0)
තවමත් කිසිදු අදහසක් පළ කර නොමැත. ඔබේ අදහස පළමු වරට මෙහි එක් කරන්න.
ඔබේ අදහස එක් කරන්න