Book an Appointment

All You Need to Know About Moyamoya Disease

Moyamoya disease is a progressive condition that results in damage to the brain's blood circulatory system. Moyamoya disease is a progressive cerebrovascular condition that affects the brain's blood vessels. The carotid artery, one of the main blood vessels supplying the brain, narrows (stenosis) or becomes blocked (occlusion). Small blood vessels at the base of the brain develop to compensate for the reduced blood flow to affected areas.These microscopic vessels are called 'moyamoya' vessels, giving the disease its name. The symptoms of moyamoya happen due to diminished oxygen delivery to the brain as a result of an ineffective blood supply.

A common symptom is stroke, which causes transient loss of neurologic function of body parts or speech (transient ischaemic attacks, or "TIA"), or paralysis of the face, arms, or legs, loss of speech, etc. Seizures, migraines, vision abnormalities, and developmental delays are possible side effects. A genetic component accounts for about 10–30% of Moyamoya cases, particularly in East Asian countries like Japan, Korea, and China. Moyamoya disease is the term used to describe patients who have this arteriopathy (damaged blood arteries). The cause of this disease is either unknown or hereditary. Let’s know more about symptoms, causes, and treatment options of moyamoya disease.

What is Moyamoya Disease?

An uncommon blood vessel condition called Moyamoya disease causes the carotid artery in the head to constrict or become blocked. The carotid artery is the major blood vessel that supplies blood to the brain tissue. Blood flow to the brain is diminished when this artery gets blocked. Then, in an effort to provide blood to the brain, tiny blood veins near the base of the brain start developing.

The illness may lead to a stroke or transient ischaemic attack (TIA). Additionally, it may result in brain haemorrhage. Moyamoya illness can impair brain function and result in developmental and cognitive delays as well as disabilities.

Children are often affected by Moyamoya illness. However, the illness can also affect adults. Moyamoya disease can affect individuals all over the world. However, it's more prevalent in East Asian nations—particularly China, Japan, and Korea. In such populations, specific genetic variables are responsible for this disease.

What are the Symptoms of Moyamoya Disease?

Moyamoya disease may affect individuals at any age. However, symptoms are more prevalent in adults aged 30 to 50 and children aged 5 to 10. It's critical to identify symptoms early in order to avoid consequences like stroke.

Adults and children with Moyamoya illness experience distinct symptoms. Usually, a stroke or repeated transient ischaemic attack (TIA) is the first symptom in children. Such symptoms can also occur in adults. However, hemorrhagic strokes, which are characterised by bleeding in the brain tissue, may also be noticed in adults. This bleeding is primarily caused by the abnormal formation of small blood vessels in the brain.

Moyamoya disease symptoms are related to diminished blood flow to the brain tissue. Such symptoms may get triggered by exercise, crying, coughing, straining or a fever. These symptoms are:

Headache
Weakness, numbness or paralysis in your face, arm or leg (on one side of your body)
Seizures
Cognitive or developmental delays
Aphasia (trouble in speaking or understanding)
Vision problems
Involuntary movements

What are Moyamoya Disease Treatment Options?

In order to manage Moyamoya disease, you must use specific medications to control your symptoms. Your doctor may recommend or prescribe the following medications:

Aspirin to prevent or reduce blood clots in the smaller, backup blood vessels that develop.
Antiseizure medications to prevent seizures that moyamoya disease causes.
Anticoagulants may cause thinning of your blood to prevent blood clots. However, these medications carry risks, such as the potential for difficult-to-stop bleeding. Only in specific circumstances will your doctor prescribe them.
Calcium channel blockers can help to reduce moyamoya disease headaches. However, these medications can also decrease blood pressure, lowering the risk of stroke. Doctors advise them only in specific circumstances.

Since arteries may continue to narrow despite medication, resulting in disease progression, surgical intervention is often necessary.. Your doctor might then opt for surgery for moyamoya disease. This bypass surgery is planned with following objectives:

Uses healthy scalp arteries as donor arteries to get around obstructed arteries.
Modifies the direction of blood flow to the afflicted areas.
Opens up constricted blood vessels.

Can Moyamoya Disease be Prevented?

It is not possible to prevent hereditary types of Moyamoya disease, but managing risk factors can help reduce complications. By controlling vascular risk factors and lowering your risk of atherosclerosis, however, you can lessen your chance of getting moyamoya syndrome. If you follow your doctor's instructions, you might be able to reduce your symptoms and prevent complications associated with moyamoya disease.

What are the Consequences of Moyamoya Disease?

The majority of moyamoya disease patients experience worsening symptoms with time. As their blood vessels narrow more and more, patients can probably experience a stroke. Moyamoya disease can cause stroke and other neurological impairments if left untreated. However, some people don't have any symptoms for years. When symptoms appear, your doctor might recommend bypass surgery.
People with moyamoya disease can live normal lives if they are diagnosed early and treated promptly. Moyamoya illness can be lethal if left untreated.

Conclusion

Moyamoya disease is an uncommon ailment that can cause brain haemorrhages and strokes. Although its right cause is unknown, the illness seems to primarily affect those of Japanese ancestry. An appointment with your doctor is necessary if you exhibit any of the signs of moyamoya disease. With moyamoya disease, early detection and treatment are essential. Recognising the symptoms of a stroke is particularly crucial since they require prompt medical intervention.

Explore symptoms, causes, and treatment options of moyamoya disease. IBS Hospital offers expert guidance on moyamoya disease management. Book your consultation today for personalized care of your loved ones!

FAQs

Q1: Who is affected by Moyamoya disease?
A: It can occur in both children and adults, with two peaks of onset: one in childhood (ages 5-10) and another in adulthood (ages 30-50). It is more common in East Asian populations, though it can affect people of any ethnicity.

Q2: Is Moyamoya disease curable?
A: There is no cure, but treatment can manage symptoms and reduce the risk of strokes. Early diagnosis and intervention are crucial for better outcomes.

Q3: Is Moyamoya disease hereditary?
A: It can have a genetic component, especially in familial cases, but it is not always inherited. Family members may be at higher risk and should consider screening if a relative is diagnosed.

By -Dr Aaksha Shukla | January 28, 2025 | 9 Min Read