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Moyamoya Disease

Our specialty-trained neurosurgeons are among few in the region skilled at diagnosing and treating rare moyamoya disease. The condition name means “puff of smoke” in Japanese. The name comes from the appearance of the cluster of tiny blood vessels that signify the disease. Our staff have treated hundreds of patients for moyamoya disease.

At The University of Kansas Health System in Kansas City, we offer unparalleled experience recognizing and correcting this unusual and dangerous condition.

About moyamoya disease

Moyamoya disease is a rare blood vessel disorder. It is a condition that occurs when the walls of the carotid artery in the skull thicken – reducing blood flow to the brain – and tiny blood vessels open at the base of the brain to try to compensate for the blocked artery. Though they try, the tiny vessels cannot fully supply the blood the brain needs. This puts the patient at risk for transient ischemic attacks (TIA), stroke or aneurysm.

Moyamoya disease can lead to temporary or permanent brain damage and can be fatal if unrecognized and untreated.

Symptoms and risks

The symptoms of moyamoya disease are those that occur when reduced blood flow to the brain decreases the brain's oxygen supply. These may include:

  • Headache
  • Weakness, numbness or paralysis in the face, arm or leg, often on just 1 side of the body
  • Visual disturbances
  • Seizures
  • Speech difficulties, or difficulty understanding others (aphasia)
  • Developmental delays in children
  • Cognitive decline

Moyamoya disease most often affects children ages 5-10, but can affect anyone. When it affects adults, it is usually those 30-50 years old. It is most commonly found in East Asian countries – Korea, Japan, China – and in people of East Asian descent. It occurs in females slightly more frequently than in males.

Several of the signs of moyamoya disease are similar to those of stroke. If you think you or someone you know may be experiencing moyamoya disease or stroke, act FAST to help.

BE FAST graphic

BE FAST: Know the signs of stroke

  • Balance: Is balance or coordination changed?
  • Eyes: Is vision in 1 or both eyes difficult?
  • Face: Does 1 side of the face droop or is it numb?
  • Arms: Is 1 arm weak or numb?
  • Speech: Is speech slurred? Is the person hard to understand?
  • Time: If the person shows any of these symptoms call 911.

Diagnosis and treatment

Your doctors will begin with a full physical exam and discussion of your medical history and your family history.

Doctors may perform a variety of imaging studies – such as MRI, CT scan or PET scan – to create detailed pictures of your brain. One study that is helpful in diagnosis of moyamoya disease is the cerebral angiogram. This is a test in which dye is injected into your brain to help doctors visualize the blood flowing to the brain. The images will show any blockage and also will allow the doctors to see the cluster of tiny blood vessels at the base of the brain that is the telltale sign of moyamoya disease.

Medical therapy – such as blood thinners or anti-seizure medications – may be part of your treatment plan, but most people with moyamoya disease require surgery for lasting results. The neurosurgeon may perform direct or indirect surgical correction.

  • Direct revascularization is essentially a bypass procedure designed to restore blood flow to the brain immediately. Usually an artery from the scalp is connected to the major artery in the brain to bypass the cluster of tiny compensating blood vessels.
  • Indirect revascularization is designed to increase blood flow to the brain over a period of time. Usually a portion of the scalp artery is attached to the surface of the brain. This promotes new blood vessel growth.

Doctors will choose the direct method in adults when an immediate improvement in blood flow to the brain is necessary to avoid further and potentially permanent brain damage.

Moyamoya scan

Neurosurgical expertise beats moyamoya disease

In moyamoya disease, tiny blood vessels try to compensate for blocked blood flow to the brain. When this happened to OR technologist Jason Edwards, he sought lifesaving treatment from Paul Camarata, MD, a neurosurgeon he'd often scrubbed in for in the operating room.

Read Jason's story

Our experts

Why choose us

  • Our neurosciences program is the region's largest, and U.S. News & World Report has consistently ranked our neurology and neurosurgery services among the nation's top 50. Survival rates, patient safety and specialized staff all contribute to this success.
  • Our chief of neurosurgery is the only fellowship-trained cerebrovascular neurosurgeon in all of Kansas and most of Missouri. He has built unparalleled experience diagnosing and treating moyamoya disease throughout his career.