As the blood supply to the brain lessens, the chances of blood clot formations start to rise.
Small blood vessels begin to work around the arteries to create "side roads," which attempt to transport oxygen to the oxygen-low areas of the brain. These networks of blood vessels are fragile, and can eventually break, causing bleeding and hemorrhaging.
The condition puts victims at a high risk for strokes and transient ischemic attacks. With only a few hundred cases reported annually, it is extremely uncommon.
The syndrome is seen most often in two groups: children and adults in their 20's and 30's. For over 20 years, hospital centers such as Children's Hospital Boston have developed effective surgical procedures for treating children and adults with Moyamoya. (SOURCE: Mayo Clinic, childrenshospital.org)
SYMPTOMS: General symptoms of Moyamoya Syndrome are nausea, vomiting, strong headache and fatigue. More serious symptoms are gradually seen, such as seizures, loss of consciousness, twitching and visual problems. Patients are usually brought into the emergency room when actual stroke symptoms are exhibited, such as numbness on one full side of the body, slurred or delayed speech and impaired vision. Brain hemorrhages can occur in this stage, and are the most common cause of death. (SOURCE Mayo Clinic, childrenshospital.org)
SURGICAL TREATMENT: There are two kinds of procedures that are designed to bypass the narrowed carotid artery and forge a new supply of blood to the affected areas of the brain. The first are direct revascularization procedures, in which the scalp artery is sutured directly onto an artery on the brain's surface.
Also called superficial temporal artery to middle cerebral artery (STA-MCA) surgeries, these procedures directly bypass the narrowed artery. The second are indirect revascularization procedures, including pial synangiosis and Encephaloduroarteriosynangiosis (EDAS), during which surgeons relocate a healthy blood vessel from the scalp so that it fits beneath the skull and can be stitched to the surface of the brain.
This transplanted vessel can grow a new network of healthy arteries and bring a steady flow of oxygen-rich blood to the damaged areas.
Encephalopmyosynangiosis (EMS) is a procedure during which a muscle in the temple is dissected, fed through a hole in the skull and placed onto the brain's surface. Other holes are then drilled to allow for new blood vessels to prosper.
Indirect procedures allow for a much more immediate flow of blood to the brain, and are usually most effective in children patients.
The majority of Moyamoya Syndrome victims who undergo these surgical procedures do not experience more strokes or complication later in life. (SOURCE: Mayo Clinic, childrenshospital.org)
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