​Moyamoya Disease (Moyamoya Syndrome)

Diagnosing Moyamoya Disease

Symptoms of Moyamoya disease

Because Moyamoya disease may be an inherited genetic abnormality, a doctor will also review the family history.

Signs and symptoms of Moyamoya disease may include:

• Aphasia (loss of ability to use or comprehend words)
• Blurred vision
• Cognitive (reasoning or remembering) impairment
• Convulsions
• Involuntary movements
• Numbness in arms and legs
• Seizures
• Severe headaches

Adults with Moyamoya disease also often experience hemorrhagic stroke (a burst blood vessel inside the brain).

Testing to diagnose Moyamoya disease

CT and MRI scans may provide initial indications of the disease. If the disease is suspected, cerebral angiography, a test that creates images of the blood flow through the brain, is conducted to establish the diagnosis.

UPMC is one of the few centers in the nation to have all major methods of measuring brain blood flow.

Moyamoya Disease Treatments

There is no evidence that medications slow the progression of Moyamoya disease. At UPMC, highly trained and experienced neurosurgeons use revascularization procedures to bypass narrowed or blocked arteries and restore blood supply to the brain.

Surgery and revascularization

Revascularization procedures performed at UPMC include:

• External carotid to internal carotid (EC-IC) bypass: The doctor attaches one of the arteries in the scalp (superficial temporal artery) to one of the major arteries in the brain (middle cerebral artery).
• EDAS (encephaloduroarteriosynangiosis): The doctor frees up, without severing, a scalp artery over a course of several inches and then makes a small temporary opening in the skull directly beneath the artery. The artery is then sutured to the surface of the brain and the bone replaced.
• EMS (encephalomyosynangiosis): A muscle in the temple region of the forehead is dissected and placed onto the surface of the brain through an opening in the skull. Multiple burr holes are drilled into the skull to allow new blood vessels to grow.
• STA-MCA (superficial temporal artery-middle cerebral artery) bypass: A scalp artery is directly sutured to a brain surface artery to bring blood directly to where the brain needs it most.

In rare circumstances, our endovascular team will evaluate the person for minimally invasive alternatives such as:

• Angioplasty
• Stenting to widen the narrowed artery

Without surgery, the majority of people with Moyamoya disease will experience mental decline and multiple strokes because of the progressive narrowing of the arteries. If untreated, Moyamoya disease can be fatal.