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Moyamoya Disease in Children: What to Know

little girl laying on couch sick

Moyamoya disease is a rare condition, occurring in approximately one out of every one million individuals in the United States. This disorder predominantly impacts children. Moyamoya is a rare progressive condition in which the blood vessels, known as carotid arteries in the neck that bring blood to the brain, become narrowed or constricted. This can put your child at risk for stroke.

Puff of Smoke

Moyamoya in Japanese means puff of smoke because that’s what the new blood vessels look like on an X-ray.

When the arteries become blocked, new blood vessels develop to try and make up for the lost blood to the brain. These new blood vessels are known as collaterals. These new blood vessels can temporarily help with blood supply to the brain, but they do not last long and eventually stop working.

Symptoms of Moyamoya Disease

Parents will notice symptoms similar to stroke, or a mini-stroke including:

  • weakness on one side of the body
  • slurred speech
  • headaches
  • seizures

These symptoms may begin gradually and sometimes get better with time, or they can begin suddenly and continue.

Very rarely a child may have a brain hemorrhage, although this is more common in adults with moyamoya disease. See Pediatric Neurosurgery Group immediately if you notice any of the following symptoms.

  • nausea and vomiting
  • severe headaches
  • fatigue and lethargy
  • numbness in some part of the body

Known Causes of Moyamoya Disease

Some children with this disease also have other conditions like sickle cell anemia, Down Syndrome, congenital heart disease, and neurofibromatosis.

This disease is not clearly understood, but genetic defects or a traumatic injury can cause it. 7% of children with moyamoya disease are thought to have an inherited gene defect.

It is slightly more common in girls than in boys, and a bit more common in children of Asian descent. The disease peaks in two age groups: children approximately 5 years of age and adults in their mid-forties.

How Is Moyamoya Disease Diagnosed?

Since moyamoya disease is life threatening, it is critical to find an experienced professional like Pediatric Neurosurgery Group in Detroit for a proper diagnosis.

  • A CT scan uses X-rays to produce multiple 3D images of the brain.
  • An MRI with MRA (magnetic resonance angiography) is a test that uses radiofrequency to create 2 and 3D images of the brain and the arteries in the neck and head.
  • A cerebral angiogram is a minimally invasive procedure performed by an interventional radiologist. Using a contrast dye, it is the best imaging test to diagnose moyamoya disease.

Treatments for Moyamoya Disease

Prescription medications like aspirin can reduce the risk of stroke by lowering the possibility of a clot forming. Several kinds of surgery restore blood flow to the brain by opening narrowed blood vessels or by bypassing the blocked arteries.

Without surgery, most patients will suffer mental decline and multiple strokes. This means the dangerous narrowing of the brain’s blood vessels will get worse with time. This can take years or months, but surgery is the only way to reduce the risk of a potentially life-threatening stroke.

Most children who have surgery for moyamoya disease have an excellent recovery rate and live a productive life.

Contact Pediatric Neurosurgery Group at (313) 833-4490 if you suspect your child may have moyamoya disease, or if you need help managing the disease.

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