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Genetic and Rare Diseases Information Center (GARD)

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Moyamoya disease


Other Names for this Disease
  • Moyamoya disease 1
  • Moyamoya disease 2
  • Moyamoya disease 3
  • Moyamoya disease, primary
  • Moyamoya disease, secondary
More Names
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Overview


Moyamoya disease is a rare, progressive, blood vessel disease caused by blocked arteries at the base of the brain in an area called the basal ganglia. The name "moyamoya" means "puff of smoke" in Japanese and describes the look of the tangle of tiny vessels formed to compensate for the blockage. This condition primarily affects children, but can affect adults. In moyamoya disease the carotid arteries are thickened, slowing blood flow to the brain and increasing the risk for blood clots, strokes, and transient ischemic attacks (TIA), frequently accompanied by seizures and muscular weakness or paralysis on one side of the body. Individuals with this disorder may have disturbed consciousness, speech deficits (usually aphasia), sensory and cognitive impairments, involuntary movements, and vision problems.  Because it tends to run in families, researchers think that Moyamoya disease is the result of inherited genetic abnormalities.[1] 

Moyamoya syndrome is a related term that refers to cases of moyamoya disease that occurs in association with other conditions or risk factors (e.g., neurofibromatosis, tuberculosis meningitis, sickle cell disease, leptospirosis, brain tumors, Sturge-Weber syndrome, tuberous sclerosis).[2] 

Last updated: 9/19/2011

References

  1. NINDS Moyamoya Disease Information Page. National Institute of Neurological Disorders and Stroke (NINDS). March 2011; http://www.ninds.nih.gov/disorders/moyamoya/moyamoya.htm. Accessed 9/19/2011.
  2. Smith ER & Scott RM. Moyamoya: epidemiology, presentation, and diagnosis. Neurosurg Clin N Am. July 2010; http://www.ncbi.nlm.nih.gov/pubmed/20561502. Accessed 9/19/2011.
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Basic Information

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  • PubMed is a searchable database of medical literature and lists journal articles that discuss Moyamoya disease. Click on the link to view a sample search on this topic.

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