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Diseases

Genetic and Rare Diseases Information Center (GARD)

Primary angiitis of the central nervous system


Other Names for this Disease
  • PACNS
  • Primary central nervous system vasculitis
  • Primary CNS vasculitis
  • Granulomatous angiitis of the central nervous system
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Overview

Primary angiitis of the central nervous system is a rare form of vasculitis (inflammation of blood vessels) affecting the blood vessels that nourish the brain, spinal cord and peripheral nerves. This condition can lead to narrowing and blockage of the blood vessels of the central nervous system which can eventually cause aneurysms, ischemia and/or hemmorrhage. The cause of this condition is unknown. Signs and symptoms of this condition may begin suddenly or develop over time. Some of the symptoms may incude headaches that do not go away, fever, rapid weight loss, confusion or forgetfulness, and general malaise. Treatment for this condition involves a course of immunosuppresive steroids.[1][2]
Last updated: 10/31/2012

References

  1. Noufal M. Primary Angiitis of the CNS. Medscape Reference. April 2012; http://emedicine.medscape.com/article/1484146-treatment. Accessed 10/29/2012.
  2. NINDS. Vasculitis Syndromes of the Central and Peripheral Nervous Systems Fact Sheet. February 7, 2012; http://www.ninds.nih.gov/disorders/vasculitis/detail_vasculitis.htm. Accessed 10/31/2012.
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Basic Information

In Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • 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 Primary angiitis of the central nervous system. Click on the link to view a sample search on this topic.
Other Names for this Disease
  • PACNS
  • Primary central nervous system vasculitis
  • Primary CNS vasculitis
  • Granulomatous angiitis of the central nervous system
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.