Moyamoya disease, a rare cerebrovascular disease that Asians need to be aware of

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Moyamoya is a Japanese word that means “a puff of smoke.” It is a disease first identified in Japan in 1969 (reference). The incidence rate in Japan is approximately 1 in 200,000 cases. This disease is more commonly found in Asian populations than in Western populations and occurs twice as often in females as in males.

 

This disease became widely discussed in Thailand when Nong C.D., a Thai child actor, was diagnosed with it in 2015. The condition was severe enough to require surgery. Today, we would like everyone to get to know this disease together.

 

What is Moyamoya Disease?

Moyamoya disease is a disorder of the carotid arteries, which are large arteries responsible for carrying oxygen-rich blood and nutrients to the brain. When these arteries become narrowed, blood flow to the brain is insufficient. The body tries to compensate by creating a network of tiny blood vessels to replace the blocked arteries, resulting in a web-like pattern of small vessels throughout the brain tissue. On X-ray images, this pattern resembles smoke, which is the origin of the disease’s name.

 

What symptoms indicate Moyamoya disease?

The main symptom of this disease is “weakness on one side of the body.” In the early stages, symptoms are usually temporary, lasting a few hours or sometimes days, and then gradually improve. However, in severe cases, it can lead to permanent hemiparesis (paralysis on one side) and may cause seizures.

 

Who is at risk of Moyamoya disease?

The answer is “both children and adults”. It is commonly found in children aged 6-9 years, while adults typically show symptoms between 40-50 years old. The difference is that children often experience ischemic symptoms (lack of blood flow to the brain), whereas adults are more likely to have brain hemorrhages.

 

The causes of the disease can be due to multiple factors, including congenital genetic abnormalities, certain infections, other brain diseases, and brain radiation therapy, all of which can trigger the condition.

 

Diagnosis of Moyamoya Disease

Diagnosis usually requires detailed imaging of the brain’s blood vessels, such as Magnetic Resonance Angiography (MRA) or CT angiography (CTA). These imaging techniques use magnetic waves to clearly visualize abnormal blood vessels.

 

How is Moyamoya Disease treated?

Treatment of Moyamoya disease is primarily based on the severity of symptoms.

  • If symptoms are not severe, treatment may involve the use of aspirin or blood-thinning medications to prevent blood clots in the brain’s blood vessels.
  • If symptoms are severe and affect daily life, surgery to create a bypass for the brain’s blood vessels, known as bypass surgery, may be considered.

 

Understanding Brain Blood Vessel Bypass Surgery

Bypass surgery is performed to create a new pathway for blood flow, bypassing the narrowed or blocked arteries. There are three main types:

 

  • Direct Bypass Surgery involves directly connecting an external artery to a brain artery, allowing immediate and effective blood flow to the brain.
  • Indirect Bypass Surgery stimulates the growth of new blood vessels by placing tissue with small blood vessels onto the surface of the brain area lacking blood flow, encouraging new vessel growth into the brain tissue.

 

Combination of both methods involves performing both direct artery connection and placing vascularized tissue to stimulate new vessel growth in the same patient. This approach increases the chances of restoring brain blood flow in both the short and long term.

 

Postoperative Care After Brain Blood Vessel Surgery

  • Most patients need to stay in the hospital for about 7-10 days after surgery.
  • Avoid heavy physical activities or strenuous exertion for at least 1-2 months.
  • Attend follow-up appointments for close monitoring of symptoms.
  • Undergo regular physical therapy as recommended by specialists.

 

The Brain and Nervous System Center at Phyathai 3 Hospital has a team of specialized doctors and surgeons who provide comprehensive diagnosis, treatment, surgery, and rehabilitation for brain and nervous system disorders. Equipped with modern technology and internationally recognized standards, we can help with all brain health issues… For brain matters, trust Phyathai 3.

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