WHAT IS MOYAMOYA DISEASE ?
Moyamoya disease is a rare, cerebrovascular disorder caused by blocked arteries at the base of the brain in an area called basal ganglia. In Japanese the name "moyamoya" means "puff of smoke" and describes the look of the tangle of tiny vessels formed to compensate for the blockage. In the 1960's, this disease was first described in Japan and since has been found in individuals in the United States, Europe, Australia, and Africa. Moyamoya disease primarily affects children, but it can occur in adults also. Because it tends to run in families, researchers think that it is the result of inherited genetic abnormalities. Approximately 10% of the cases are genetic caused and are termed primary Moyamoya disease. Secondary Moyamoya disease refers to cases in which the disease is a result of another underlying disorder. In this case it is very important for the physician to determine the root underlying cause.
SIGNS AND SYMPTOMS
The signs most typically include paralysis of the feet, legs or upper extremities. Headaches, mental retardation, various vision problems, speech deficits, and psychiatric problems.
In children, the first symptom of Moyamoya disease is often a stroke, or recurrent transient ischemic attacks (TIA's, commonly referred to as "mini-strokes"), frequently followed by muscular weakness affecting one side of the body or seizures. Adults often experience a hemorrhagic stroke due to recurring blood clots in the affected brain vessels.
TREATMENT
There is no cure for moyamoya disease, but treatment may help manage the signs and symptoms. Treatment may include:
- Corticosteroid medications
- Blood-thinning medications to reduce the risk of blood clots
- Revascularization surgery to bypass blocked arteries or open narrowed blood vessels in order to restore blood to the brain
Without treatment, Moyamoya disease can be fatal as a result of intracerebral hemorrhage.
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