Moyamoya disease is the narrowing and ultimately closure of the internal carotid arteries, the two largest arteries feeding the brain. Moyamoya disease is progressive, meaning the vessel walls will continue to narrow over time.
The cause of moyamoya disease is unknown, although genetics are a likely factor. It affects mostly children and adolescents.
In response to the narrowing of the blood vessels, the body will begin to grow many small blood vessels to bypass the blockage.
The word moyamoya means “puff of smoke” in Japanese, and refers to the appearance of these networks of new blood vessels. The condition first was identified in Japan, where its incidence is higher than elsewhere.
The findings of narrow blood vessels at the base of the brain can also be associated with other diseases, and is referred to as moyamoya syndrome. This syndrome can be found in people with:
UPMC offers a variety of surgical techniques to treat Moyamoya disease, depending upon the exact location of the 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:
Adults with Moyamoya disease also often experience hemorrhagic stroke (a burst blood vessel inside the brain).
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.
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.
Revascularization procedures performed at UPMC include:
In rare circumstances, our endovascular team will evaluate the person for minimally invasive alternatives such as:
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.
How can we help you?
Schedule anappointment >
Ask a question >
Request our expertopinion >
1-877-986-9862(within the U.S.)
Affiliated with the University of Pittsburgh Schools of the Health Sciences
Supplemental content provided by Healthwise, Incorporated. To learn more, visit Healthwise.org
For help in finding a doctor or health service that suits your needs, call the UPMC Referral Service at 412-647-UPMC (8762) or 1-800-533-UPMC (8762). Select option 1.
UPMC is an equal opportunity employer. UPMC policy prohibits discrimination or harassment on the basis of race, color, religion, ancestry, national origin, age, sex, genetics, sexual orientation, gender identity, marital status, familial status, disability, veteran status, or any other legally protected group status. Further, UPMC will continue to support and promote equal employment opportunity, human dignity, and racial, ethnic, and cultural diversity. This policy applies to admissions, employment, and access to and treatment in UPMC programs and activities. This commitment is made by UPMC in accordance with federal, state, and/or local laws and regulations.
Medical information made available on UPMC.com is not intended to be used as a substitute for professional medical advice, diagnosis, or treatment. You should not rely entirely on this information for your health care needs. Ask your own doctor or health care provider any specific medical questions that you have. Further, UPMC.com is not a tool to be used in the case of an emergency. If an emergency arises, you should seek appropriate emergency medical services.
For UPMC Mercy Patients: As a Catholic hospital, UPMC Mercy abides by the Ethical and Religious Directives for Catholic Health Care Services, as determined by the United States Conference of Catholic Bishops. As such, UPMC Mercy neither endorses nor provides medical practices and/or procedures that contradict the moral teachings of the Roman Catholic Church.
Pittsburgh, PA, USA UPMC.com