Moyamoya Disease Care
Expert center for Moyamoya disease in adults
Specialty Care for Adults with Moyamoya
Beth Israel Deaconess Medical Center (BIDMC) neurologists offer specialized care for those with Moyamoya disease. If you or a loved one has this complex condition, our experienced team can help.
Dedicated Adult Moyamoya Center
The Adult Moyamoya Center brings together nationally renowned experts from throughout the BIDMC health system. We combine experts from stroke and vascular neurology, neurosurgery and radiology. Our team provides state-of-the art evaluation and treatment to adults with known or suspected Moyamoya disease.
Ours is one of the most experienced groups in the nation to treat this disease in adults. In addition to providing comprehensive care, our team conducts research. Our Moyamoya disease research aims to accomplish these important steps:
- Better understand the cause of this disease
- Improve existing treatments
- Develop new therapies
What is Moyamoya Syndrome?
Moyamoya disease is a rare, long-term blood vessel (vascular) disorder. If you have the disease, the carotid artery in the skull becomes progressively narrowed or blocked. This reduces blood flow to the brain over time.
Risk Factors for Moyamoya Disease
Most people with Moyamoya disease are diagnosed during childhood. However, more and more adults (often in their 30s or 40s) are receiving the diagnosis.
Females are more likely to develop the disease than males. As Moyamoya disease has become more recognized beyond childhood, it is now considered a cause of hemorrhage and stroke in adults.
Complications from Moyamoya Disease
In response to a reduced blood flow, your body develops a fragile network of blood vessels. On imaging studies, this resembles a puff of smoke (called “moyamoya” in Japanese).
The reduced blood flow to the brain can eventually cause these issues:
- Hemorrhagic stroke (caused by a ruptured blood vessel)
- Ischemic stroke (caused by a blood clot)
- Seizures
More About Moyamoya Disease Care
The cause of Moyamoya disease is not known. However, a family’s genes appear to be a factor in about 10–15% of those with the disease.
Researchers originally believed that Moyamoya disease primarily affected people of Asian descent. However, more recent research has shown that it affects people of all ethnicities.
The symptoms depend on age. Children are likely to have transient ischemic attacks (TIAs) or mini-strokes. Adults with Moyamoya disease tend to suffer hemorrhagic strokes. These can recur, cause lasting damage and be fatal.
Learn more about stroke care in our Stroke Center.
These are common symptoms of a brain hemorrhage:
- Lethargy
- Nausea or vomiting
- Numbness or tingling of the body
- Severe headaches
- Visual changes, such as loss of vision in one eye or blurry vision
Adults also may have cognitive issues such as memory loss, confusion or poor motor skills.
Our team of experts will provide a comprehensive evaluation. You will undergo imaging studies, such as:
- CT angiography
- MRI
- Magnetic resonance angiography (MRA)
Then, you will undergo evaluation by our team’s neurologist and one of our neurosurgeons. After this, our team meets to review the information. We will work together to determine the best course of treatment that will offer you the best possible outcomes.
We will create your customized care plan based on the results of your imaging tests, your symptoms and other factors. For many people, the first course of treatment will be medications (antiplatelets) and careful monitoring.
Revascularization Surgery
However, because the disease will invariably worsen, many people will require revascularization surgery. The goal of this surgery is to restore blood flow to the affected area of the brain. This results in reduced incidence of hemorrhage and stroke.
At BIDMC, our highly experienced neurosurgeons offer two types of revascularization procedures:
- Direct revascularization surgery, or extracranial to intracranial bypass procedure (EC-IC bypass). The surgeon uses a scalp artery to create a detour. This immediately increases blood flow to the affected area of the brain.
- Indirect bypass procedure, or encephaloduroarteriosynangiosis (EDAS). This results in the growth of new blood vessels to the affected area of the brain over the course of months.
The effectiveness of both procedures is equivalent. However, there is some evidence that EDAS may be safer.
The MoyaMoya Foundation is a nonprofit organization founded to support patients and their families by:
- Promoting awareness of moyamoya disease by working with healthcare professionals and patient advocates
- Supporting and encouraging research of moyamoya disease
- Providing assistance to families/patients undergoing moyamoya treatment for expenses not covered by insurance
As part of the MoyaMoya Foundation's Medical Advisory Board of Directors, Dr. Christopher S. Ogilvy volunteers his time and shares his expertise to review content, provide guidance, and give directional advice regarding moyamoya research and medical advancements.
Advancing Care, Research & Education
Department of Neurology
The Department of Neurology offers exceptional patient care, education opportunities and research programs.
Neurology Research
Our top-tier research programs and laboratories work to advance the knowledge and treatment of neurological disorders.
Neurology Education
Our team offers comprehensive education and training programs for healthcare professionals, including fellowships and residencies.
