Moyamoya disease is a neurological disorder caused by narrowing of the two main arteries that supply the brain with blood. This narrowing slows down the blood flow to the brain and causes various symptoms. This disease is usually seen in children. In many people who do not receive treatment, problems such as mental decline and paralysis can occur as a result of narrowing of the arteries. Without treatment, fatal outcomes such as cerebral hemorrhage can occur.
It can be a stroke, mini-stroke or bleeding in the brain. It can also affect how the brain works. Moyamoya disease mostly affects children, but adults can also get it. In addition to strokes and brain hemorrhages, Moyamoya disease can also impair brain function. It can lead to disability and cognitive and developmental delays. Although collateral blood vessels can help in the short term, they eventually stop working. Left untreated, moyamoya disease can be fatal, but there are ways to treat it.
Where does moyamoya disease come from?
Moyamoya disease was first discovered in Japan. Although it can occur in anyone, it is more common in Asian countries than in North America or Europe. This may be due to genetic characteristics in Asian populations. However, only about 10% of cases in Asian people are due to a genetic link.
In Japanese, the term "moyamoya" means "puff of smoke". It refers to how the collateral veins look when they form.
What Causes Moyamoya Disease?
The cause of this disease is not known exactly. According to the researches, traumatic blows to the head are caused by various factors. Along with these factors, the following conditions may increase the risk of developing moyamoya disease;
Genetic: This condition may be genetic. According to research, genetic mutations linked to the disease have been identified.
Current health: People with other conditions such as Down syndrome, Graves' disease, atherosclerosis and radiation vasculopathy may be more likely to develop it.
Gender: Women are more likely to develop moyamoya disease.
Age: Moyamoya disease can occur at any age. It is most common in children aged 5-15 years. Adults can also get it, but this is less common. People with moyamoya disease are usually diagnosed between the ages of 30 and 50.
What are the Symptoms of Moyamoya Disease?
Symptoms may vary depending on the age of the person. Symptoms may start gradually or suddenly and may develop and persist over time. Doctors usually diagnose moyamoya disease in children after they have had a stroke or mini-stroke, while adults often have a hemorrhagic stroke due to bleeding into the brain. In addition to strokes, symptoms of moyamoya disease may include
- Vision problems
- Headache
- Disruptions of consciousness
- Seizures
- Involuntary movements
- Cognitive and/or sensory impairment
- Weakness, numbness or paralysis - usually on one side of your body
- Difficulty understanding speech or verbal communication
- Aneurysm
In some cases, children with moyamoya disease have a brain hemorrhage. Symptoms of brain hemorrhage in children may include
- Vision changes
- Severe headache
- Vomiting
- Nausea
- Fatigue and lethargy
- Numbness in one part of the body
How is Moyamoya Disease Diagnosed?
A neurologist can diagnose moyamoya disease. They can use any of the following tests to do this;
Electroencephalogram (EEG): The healthcare professional attaches electrodes to the scalp with a special adhesive substance, then the machine looks at electrical activity in the brain.
Transcranial Doppler ultrasound: This is a painless scan. Transcranial Doppler allows the blood circulation in the brain to be assessed.
Computed tomography (CT): An imaging technique that uses special X-rays to produce detailed pictures or scans of parts of the body.
Magnetic resonance imaging (MRI): Magnets and radio waves create an image of the brain and look at how much blood is moving through your blood vessels. This allows them to see how much blood your brain is receiving, which is important for assessing the severity of moyamoya disease.
Cerebral angiogram: Also described as a brain angiogram. It places a thin tube into a blood vessel in the groin area and then guides it all the way to your brain. They then inject dye so that they can use X-rays to see the blood vessels.
How is Moyama Disease Treated?
Moyamoya disease usually gets worse over time. But there are treatments that can help. Moyamoya disease treatment may include using medication to reduce the risk of stroke. Blood thinners, anti-seizure medicines or calcium channel blockers may be used to reduce the risk of stroke or manage seizures. These medicines should be used under specialist supervision.
Revascularization surgery is also an option for some people. Surgeons can use various techniques to restore blood flow. Children may respond better to this than adults. In some cases, surgery can stop strokes from happening.
Therapy treatment Therapy is administered to patients to treat the physical losses that usually result from a stroke. The aim is to help the body regain its lost function. The person can then resume his or her professional life. They can meet their daily needs. Psychological therapy is also recommended. A solution is also provided for problems such as depression and anxiety that may occur due to the symptoms of the disease.