About Moyamoya Disease
In moyamoya disease, the large arteries inside the skull (vessels that carry blood to the brain) narrow and often completely close off. Small branches of these arteries enlarge to bypass the blockage, probably as a response to this narrowing. The cause of the narrowing is unknown.
These small branches that grow larger (and maybe more numerous) give the disorder its name. “Moyamoya” is a Japanese term used to describe the hazy appearance of these small vessels on an angiogram.
This disorder is common in Asia, where it most frequently affects children. In North America, it is much less common and generally affects women in their 3rd, 4th and 5th decades of life, frequently causing stroke.
The treatment of patients with moyamoya is difficult because so little is known about the disease. Some people never have a stroke while others may have several. There are surgical procedures that may improve blood flow to the brain. These procedures may cause complications, however, and may not always achieve the desired result of improving brain blood flow. Sometimes blood flow to the brain is normal despite the blockages because of connections from normal arteries that bypass the blockages. People with normal blood flow may not have anything to gain from surgery.
The Washington University Moyamoya Center was established in 2006 with the receipt of a five-year grant from the National Institute of Neurological Disorders and Stroke to perform a long-term study of the outcome of people with moyamoya disease. This grant, and the center, were built on a foundation of excellence in Vascular Neurology, Vascular Neurosurgery, and Radiology.
Ongoing NIH funded grant to study the natural history of patients with moyamoya disease and their outcome after surgery. (NINDS R01 NS051631, The role of cerebral hemodynamics in moyamoya disease). This study involves measurement of brain blood flow and oxygen use with a PET scanner, and clinical follow up over years.
High-resolution MR-imaging study to image the wall of the narrowed artery. This is a pilot study supported by Mallinckrodt Institute of Radiology.
- Hallemeier CL, Rich KM, Grubb TL, Jr., Chicoine MR, Moran CJ, Cross DT III, Zipfel GJ, Dacey RG Jr, Derdeyn CP. Outcome in north american adults with Moyamoya phenomenon. Stroke 2006; 37:1490-1496.
- Zipfel GJ, Fox DJ Jr, Rivet DJ. Moyamoya disease in adults: the role of cerebral revascularization. Skull Base. 2005 Feb;15(1):27-41.
- Dunn GP, Refai D, Derdeyn CP, Zipfel GJ. Angiographic patterns of revascularization in North American Moyamoya. Accepted for presentation, Congress of Neurological Surgeons, October 2006.