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Moyamoya Disease Treatment

Compassionate Care at RWJBarnabas

The Department of Neurosurgery at Rutgers Health and RWJBarnabas Health treats rare neurological conditions, including moyamoya disease, a progressive disorder affecting cerebrovascular blood vessels. These patients often present with a stroke after years of smaller blood vessels attempting to compensate for a loss of function in the larger vessels that are supposed to supply oxygenated blood to the brain. Moyamoya disease and strokes are both treatable in the stroke center.

Important note: If you suspect you or a loved one is having a stroke, seek immediate medical attention. Do not drive your or have someone else drive you to the hospital: Call 9-1-1 immediately, as paramedics may be able to administer life-saving treatments en route to the hospital.

What Is Moyamoya Disease?

Moyamoya is a progressive disease affecting the blood vessels of the brain. It is characterized by a narrowed and/or closed carotid artery that delivers blood to the brain. Because it is blocked, smaller blood vessels (“moyamoya” vessels) attempt to compensate by opening up to supply blood to the brain. Because this is still insufficient, it leads to a serious reduction in oxygen delivery, and this deprivation causes the Moyamoya symptoms, such as stroke.

Moyamoya means “puff of smoke” in Japanese and is most prevalent in the Japanese population, where it affects mostly females under the age of 20. Outside of Japan, approximately 1 per 300,000 people is diagnosed with the disease, although these are usually isolated cases. In the United States, the disease is very rare, occurring less than once in every 100,000 people, according to the National Organization for Rare Disorders. It is characterized by blocked arteries in the brain.

Moyamoya Disease Symptoms

If Moyamoya causes stroke, as it is likely to do because of the inadequate oxygenated blood supply to the brain, the patient may suffer from permanent side effects as a result. For example, many patients have facial paralysis, paralysis of the limbs, and loss of speech. Some temporary neurological function loss may also occur if the patient experiences a transient ischemic attack (also called “TIA” or a mini stroke), or a full-blown ischemic or hemorrhagic stroke, due to the blockage of oxygenated blood carried to the brain. It may also happen when the tiny blood vessels rupture and leak blood into the brain.

Moyamoya disease symptoms include:

  • Headaches

  • Seizures

  • Weakness of paralysis in the face, arm, or leg on one side of the body

  • Vision problems

  • Aphasia (difficulty speaking or understanding speech)

  • Cognitive impairments

  • Learning disabilities

If you suspect your loved one is having a stroke, look for these symptoms, and act “FAST”:

  • Face: If one side of the face droops, especially if the person is trying to smile, this can indicate facial paralysis.

  • Arms: Ask the person to raise both arms. It one drifts downwards, it could indicate a problem.

  • Speech: Ask the person to repeat a simple phrase, such as “let’s take the dog for a walk this afternoon.” If they cannot repeat it or their speech is slurred or strange, this could also indicate a stroke.

  • Time: If you notice any of these signs, you must call 9-1-1 immediately.

What Causes Moyamoya Disease?

Moyamoya disease has no known cause, although family history (genetics) may play a role, especially in individuals of Japanese origin. It can occur at any age, although it is most common in children between the ages of 5 and 10, and in adults between the ages of 30 and 50. Moyamoya disease is also more common in individuals with patients who have neurofibromatosis type 2.

Moyamoya Disease Treatment

Diagnosing moyamoya disease means reviewing your symptoms and discussing any medical history of strokes or other disorders. Your doctor will perform a complete physical exam and perhaps order tests before diagnosing moyamoya disease and/or an underlying condition.

Tests to diagnose moyamoya disease include:

  • Imaging tests: Magnetic resonance imaging (also called an MRI) scan, is useful in creating detailed images of the inner structures of the head and brain. Computerized tomography (CT) scans are slightly less detailed, as they mainly identify harder structures, such as bone, rather than the soft structure of the brain.

  • Cerebral angiography: A catheter (long, thin tube) is inserted into a blood vessel of the groin and guided into the brain using X-ray images. A special medical dye injected into the catheter will make the blood vessels more visible through X-ray imaging.

  • Transcranial Doppler ultrasound: Like an ultrasound for a pregnant woman, this utilizes sound waves to capture images of the brain and obtain information about the brain’s blood vessels.

  • Other scans: Your doctor may also recommend positron emission tomography (PET) scans or a single-photon emission computerized tomography (SPECT) scan to provide further visual images of brain activity, as well as measuring blood flow to various regions of the brain.

  • Other tests:If your doctor suspects another condition mimicking moyamoya disease, other tests may be ordered to rule out moyamoya.

Treatment for Moyamoya Disease

By examining your condition, your doctor can help determine the best treatment for your moyamoya disease. The goal is to reduce your symptoms and improve blood flow, while lowering your risk of having an ischemic stroke or bleeding on the brain, which can ultimately be fatal without treatment. Patients who forego treatment are more likely to experience cognitive decline and/or multiple strokes because of the progressive artery narrowing. If left untreated, moyamoya disease can be fatal if there is significant bleeding on the brain.

Medication

The primary treatment for moyamoya disease is medication to reduce the risk of having a stroke or to aid seizure control. These medications may include blood thinners, calcium channel blockers, and anti-seizure medications.

Revascularization Surgery

If symptoms worsen over time, or if tests indicate very low blood flow, your doctor may recommend revascularization surgery. This helps bypass the blocked arteries of the brain to restore blood flow and ultimately reduce the risk of having a stroke.

Request an appointment online now or call 833-656-3876.