Moyamoya Disease

A rare blood vessel disorder in which a ring of blood vessels at the base of your brain and the uppermost (distal) segments of the arteries supplying the brain progressively narrow is called Moyamoya (in Japanese, “puff of smoke") disease. This disease can cause blood flow to your brain to become reduced.

This disease may cause a mini-stroke (transient ischemic attack). Mostly this disease occurs in Japan and other Asian countries but people in Europe, America and other countries may also have this.

The symptoms and signs of moyamoya disease usually are consistent with a stroke such as:

• blurry vision,
• weakness on one side of the body,
• headaches,
• slurred speech,
• seizures.

These symptoms can either subside over time or emerge gradually. Brain hemorrhage occurs usually in adults with moyamoya disease.

Signs of a brain hemorrhage may include:

• vomiting,
• nausea,
• change in vision,
• fatigue,
• numbness in part of the body,
• severe headache. 

There is no specific cause of moyamoya.

A variety of factors can trigger the process in the brain’s arteries.

Some of these factors include:

• gender, this disease is mostly common in girls than boys,
• race, mostly in Asian children,
• genetic, this is believed to be an inherited gene defect in about 7 percent of children with moyamoya disease,
• other conditions such as Down syndrome, sickle cell disease, congenital heart disease and neurofibromatosis.

Your doctor will likely do some tests to confirm the diagnosis of moyamoya disease.

These tests may include:

• magnetic resonance imaging (MRI) to take detailed pictures of bones, tissues and organs,
• magnetic resonance angiogram (MRA) to look or “map” the child’s blood vessels,
• computed tomography (CT) scan that uses X-ray to give three dimensional image of tissues, bones and blood vessels.

Your doctor may recommend medication to manage some of your child’s symptoms that includes:

• calcium channel blockers (verapamil) to stop the calcium form entering the cell s of the blood vessels and the heart to lower blood pressure so the severe headaches will be reduced,
• anti-clotting drugs (aspirin) to prevent the formation of blood clots,
• surgery, bypassing narrowed arteries and creating a new blood supply to decrease stroke,
• indirect surgeries  to develop new blood vessel growth like EDAS (encephaloduroarteriosynangiosis)that a small new arterial vessels begin will begin to develop, EMS (encephalomyosynangiosis) to form a supplementary source of blood that will flow to the brain, omental transposition/transfer that new vessels will eventually develop and grow in the brain, dural inversion,
• direct arterial bypass or superficial temporal to middle cerebral artery anastomosis (STA-MCA), a vessel in the brain and a blood vessel from the scalp will be joined together to improve blood flow.

There is no known prevention of moyamoya disease. Manage your symptoms to avoid major strokes.

There are no homeopathic remedies to ease the symptoms of moyamoya disease.

Lifestyle modifications are necessary in order to cope with moyamoya disease.

Patients with moyamoya disease will have minimal restrictions after surgery.

Medicines should be taken as daily aspirin for the rest of their lives because it thins the blood, which promotes flow through the grafts. 

Some of the risk factors associated with the progression of moyamoya may include:

• gender, mostly in women,
• race, mostly with Japanese heritage or other Asian race,
• if you have a family history of moyamoya,
• age, common in children rather than adults,
• if you have been diagnosed with Down syndrome,
• radiation exposure,
• various congenital syndrome, and sickle anemia and other hematologic disorders.