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Moyamoya Disease: What You Should Know About This Stroke Risk

POSTED BY: Florida Hospital

Facial drop. Loss of motor function. Slurred or delayed speech. Disturbed vision. You might recognize these symptoms as signs of a stroke. But what if they were also symptoms of another serious vascular disease?

Sometimes, an unlikely culprit for stroke — even in younger populations — is a rare condition called Moyamoya disease.

According to Ravi Gandhi, MD, neurosurgeon at Florida Hospital, soon to be AdventHealth, Moyamoya disease is often diagnosed after a stroke or hemorrhage (bleed) in the brain occurs.

Moyamoya Disease Explained

Dr. Gandhi shares, “Moyamoya causes the carotid arteries (the main arteries carrying blood to the brain) to become tight, restricting blood flow to the brain. This then leads to smaller blood vessels enlarging to take over the role of the carotid arteries. The disease is called Moyamoya because when the smaller blood vessels are imaged, they appear as a “puff of smoke,” which is the meaning of “moya” in Japanese.”

While fairly uncommon, affecting about one to two people per million in the U.S., Dr. Gandhi explains that Moyamoya disease, among many other highly specialized neurovascular diseases, is something he and his team commonly treats.

“Japanese women have the highest rate of Moyamoya disease or those with a family history. There is some genetic component to this disease, which is not well understood at this time,” says Dr. Gandhi. 

“Moyamoya syndrome is a second type, which is associated with other diseases or syndromes like hyperthyroidism, neurofibromatosis and down syndrome,” he adds.

Moyamoya disease can present in childhood up to age 5 to 15, who are most often diagnosed after they present with ischemia or a stroke. The next age group this disease tends to affect is age 30 to 50, who can present with a hemorrhage (bleed) in the brain, stroke or temporary ischemic attack (TIA or mini stroke).

“The reason this happens is because the little blood vessels that have become enlarged to compensate for the carotid artery can’t keep up with the demand of blood that the brain requires. Over time, this can lead to the vessels rupturing or being overwhelmed so that enough blood does reach the brain."

Treatment for Moyamoya Disease

“While Moyamoya is not reversible, there are highly successful treatments available at larger, qualified medical centers, like Florida Hospital,” says Dr. Gandhi.

Treatment is focused on supplying more blood to the brain through surgical procedures.

Dr. Gandhi describes, “Through a bypass procedure we can take a blood vessel that usually supplies blood to the scalp and reroute it to a blood vessel to the brain. Or, another procedure called encephalo-duro-arterio-synangiosis (EDAS) places a more superficial artery, the covering of the brain, or sometimes muscle in contact with the brain surface. A hole is cut in the skull directly beneath the artery and it is sutured to the surface of the brain. In time, little blood vessels will sprout and take over blood supply to the brain.”

Symptoms of Moyamoya Disease

While most people learn of their Moyamoya disease after a more serious symptom such as a stroke or hemorrhage, it is sometimes diagnosed by being vigilant about more subtle symptoms of a vascular disease. This is especially true among younger populations who are less likely to suspect that their symptoms are related to a stroke.

Dr. Gandhi explains that some less obvious symptoms can include:

  • Vision changes
  • Cognitive delay or problems with memory
  • Unusual headaches
  • Stroke symptoms that come and go (common with TIAs)

“Symptoms of a TIA can be exactly like a stroke, but they might get better within 24 hours. It’s very important that if anyone has symptoms of a TIA or stroke, they get help from a major medical center as soon as possible, even if the symptoms go away. This increases the chances of survival and reduces the risk of permeant neurological deficits.  A TIA is often a warning of a larger, permanent stroke to come. Patients who have a TIA have a 20 to 40 percent risk of developing a stroke. Through highly specialized procedures, we can open up blood vessels to get blood flow to the brain – few hospitals have this capability, but we do at Florida Hospital Orlando,” advises Dr. Gandhi.

One way to easily recognize these symptoms is to know the acronym Act F.A.S.T.:
• Facial drooping: A section of the face, usually only on one side, that is drooping and hard to move. This may look like a crooked smile.
• Arm weakness: The inability to raise one's arm fully
• Speech difficulties: An inability or difficulty to understand or produce speech
• Time: With any of these symptoms, time is of the essence. Call 911 or go to the nearest hospital.

“Another risk factor for stroke is stress because of increased stress hormones in the body, and the brain’s increased demand for blood,” notes Dr. Gandhi. This can include emotional stress or physical stress from an unrelated medical condition or procedure.

Positive Outcomes and Hope for Those with Moyamoya

“Once a patient has a successful bypass procedure, the chance of a future stroke is low,” says Dr. Gandhi.

Long-term, patients with Moyamoya are often advised to take the same medications used to treat stroke, as well as adopt a strict diet and make other lifestyle changes that promote vascular health.

“We also closely monitor the bypass to see if it is growing and maturing to revascularize the brain. Six months after the procedure we obtain images of the brain’s blood vessels through an angiogram. Then, we monitor patients annually and keep a careful eye on any symptoms,” explains Dr. Gandhi. 

He concludes, “The good news is that with the right treatment from experts and skilled neurosurgeons with expertise in treating neurovascular conditions, patients with Moyamoya disease can lead very healthy lives. It’s wonderful to help patients by offering these procedures with confidence at Florida Hospital.”

Learn more about Dr. Gandhi and Florida Hospital’s neurosurgery team.