What is it?
Moyamoya disease is a cerebrovascular disorder in which the arteries of the base of the brain, in an area referred to as the basal ganglia, are blocked. The disease was first discovered in the 1960s in Japan, but cases have now been found in many places in the world including America. The term "moyamoya" means "puff of smoke" in Japanese, which refers to how the tiny vessels formed look as they try to balance the blockage. It is most commonly found in children, but cases have been found in adults also. Moyamoya disease has been found to run in families, so researchers think that it may be a result of abnormalites in genes.
Moyamoya disease is a cerebrovascular disorder in which the arteries of the base of the brain, in an area referred to as the basal ganglia, are blocked. The disease was first discovered in the 1960s in Japan, but cases have now been found in many places in the world including America. The term "moyamoya" means "puff of smoke" in Japanese, which refers to how the tiny vessels formed look as they try to balance the blockage. It is most commonly found in children, but cases have been found in adults also. Moyamoya disease has been found to run in families, so researchers think that it may be a result of abnormalites in genes.
What are the symptoms?
The beginning symptoms of Moyamoya disease are stroke, recurrent TIAs (mini-strokes) commonly accompanied by muscular weakness/paralysis affecting one side of the body, or seizures. Older victims of the disease often experience a hemorrhagic stroke from recurring blood clots in the brain vessels. Other symptoms include problems with consciousness and vision, speech deficits (aphasia), sensory and cognitive impairments, and involuntary movements.
What are some treatments?
Surgery involving revascularization (opening narrowed blood vessels or bypassing blocked vessels in the brain) to restore blood flow is the most common treatment. Surgery is usually more successful in children than adults, but after surgery the symptoms tend to go away. However, without surgery, the arteries continue to progressively narrow and the majority of individuals will experience mental decline and multiple strokes; some can experience results as fatal as an intracerebral hemorrhage.
References:
http://www.ninds.nih.gov/disorders/moyamoya/moyamoya.htm
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