Moyamoya disease – overview

Moyamoya disease
Moyamoya disease

There is a rare condition in which the blood supply from major arteries to the brain is reduced or stopped because the arteries narrow down on the base of the skull. This condition is caused due to moyamoya disease in which the body tries to form certain small blood vessels as compensation to the blockage which entangles the vessels but still is not able to supply blood to the whole brain.

Moyamoya disease is more common in Japan and is a Japanese term that means ‘puff of smoke’. These arteries are the major arteries that take blood from the heart to the brain but due to the disease, they narrow down with time which causes many serious problems.

Problems caused by the moyamoya disease:

There are two major conditions that occur. They are:

  1. There is a significant decrease in brain functioning and cognitive decline because of the reduction of blood supply to the brain.
  2. It also causes severe repetitive strokes and even hemorrhages that occur because of these abnormal thin blood vessels and in the long term could prove to be very fatal.

The brain starts taking blood supply from the vessels of the scalp and skin which results in the formation of very thin vessels on the surface and base of the brain and are termed as moyamoya vessels. When a person goes through an X-Ray or an angiogram, this condition appears as a fine puff of smoke on the base of the brain and hence the name moyamoya disease. With time, it causes various severe attacks resulting in some problems of health and body. Mini-strokes are the primary symptom of the moyamoya disease. However, it could also cause headaches, fatigue, dizziness, sometimes even seizures, etc. which all are the various problems caused by neuro problems.


This disease is basically found in two age groups which are pediatric, i.e., children, and sometimes in adults, and is not common in middle normal age groups. The disease in severe health conditions can cause brain hemorrhage and as it is very progressive so eventually it is a huge risk to life. In most cases, the blockage occurs on both sides of the brain in the arteries. The areas in the brain that are deprived of blood go through various neurological disorders which results in the compromising condition of the side of the brain which doesn’t have this problem. 

Treatment options for the disease:

Moyamoya disease is chiefly a surgical disease which means that its essential treatment lies in the surgery. Therefore, there are certain important surgical techniques that are done to increase the blood supply to the person’s brain.

There are two ways of surgeries. They are:

  1. Direct Vascularization Technique: This is also known as the STA MCA Bypass in which the arteries of the scalp are directly put to the brain surface connecting it to the arteries of the brain that initially supplies the blood to the skin of the scalp. It is generally done in specialized centers.
  2. Indirect Vascularization Technique: This is commonly done in children as in the case of a child, direct vascularization of arteries is not possible. So in this technique, special tissues or arteries are put on the surface of the brain rather than combining the arteries to arteries of the brain, and with time, the brain starts to accept the blood supply from this tissue that might include putting an artery, muscle, or sometimes the underlying part of the scalp on the brain.

Results of the surgery:

The overall outcome of the disease in the people who went through these surgeries is good. The progression of this disease is reduced and eventually stopped in these people and they were able to go back to their normal lifestyle depending on the number of disabilities they already have because of the progressive outcome of the disease.

So the question, in general, is asked: Is the moyamoya disease curable? The answer to this is that it is suddenly not 100 percent curable but with time and successful surgeries in people with successful surgical results, the progression of the disease could be reduced or stopped in a span of time with successful surgical techniques. Therefore, these people will have exceptional long-term results when it comes to the prevention of strokes and minimizing the fatality rate.