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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
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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 tangled vessels that form to compensate for the blockage. This condition usually affects children, but can affect adults. Affected people are at increased risk for blood clots, strokes, and transient ischemic attacks (TIAs) which are frequently accompanied by seizures and muscular weakness, or paralysis on one side of the body. Affected people may also have disturbed consciousness, speech deficits (usually aphasia), sensory and cognitive impairments, involuntary movements, and vision problems. Researchers believe that Moyamoya disease is an inherited condition because it tends to run in families.[1]

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

Last updated: 7/1/2014

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

In Depth Information

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  • MeSH® (Medical Subject Headings) is a terminology tool used by the National Library of Medicine. Click on the link to view information on this topic.
  • Online Mendelian Inheritance in Man (OMIM) is an catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs.  Access to this database is free of charge.
  • 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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Other Names for this Disease
  • Moyamoya disease 1
  • Moyamoya disease 2
  • Moyamoya disease 3
  • Moyamoya disease, primary
  • Moyamoya disease, secondary
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.