NINDS Vasculitis Syndromes of the Central and Peripheral Nervous Systems Information Page

Synonym(s):   Temporal Arteritis, Cranial Arteritis, Giant Cell Arteritis
Condensed from Vasculitis Syndromes of the Central and Peripheral Nervous Systems Fact Sheet

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What is Vasculitis Syndromes of the Central and Peripheral Nervous Systems ?

Vasculitis is an inflammation of the vascular system, which includes the veins, arteries, and capillaries.  Researchers think that inflammation is due to a faulty immune system response. Vasculitis can cause problems in any organ system, including the central (CNS) and peripheral (PNS) nervous systems.  Vasculitis disorders, or syndromes, of the CNS and PNS are characterized by the presence of inflammatory cells in and around blood vessels, and secondary narrowing or blockage of the blood vessels that nourish the brain, spinal cord, or peripheral nerves.    

A vasculitis syndrome may begin suddenly or develop over time.  Symptoms include:
headaches, especially a headache that doesn’t go away; fever; feeling out-of-sorts; rapid weight loss; confusion or forgetfulness leading to dementia; aches and pains in the joints and muscles; pain while chewing or swallowing; paralysis or numbness, usually in the arms or legs; and visual disturbances, such as double vision, blurred vision, or blindness

Although these disorders are rare, there are many of them.  Some of the better understood syndromes are:  temporal arteritis (also called giant cell arteritis or cranial arteritis), Primary angiitis of the CNS (granulomatous angiitis), Takayasu’s disease, Periarteritis nodosa, Kawasaki disease, Churg-Strauss syndrome, Wegener’s granulomatosis, systemic lupus erythematosis, scleroderma, rheumatoid arthritis, Sjogren’s syndrome, and Behcet’s disease.

Is there any treatment?

What is the prognosis?

What research is being done?

NIH Patient Recruitment for Vasculitis Syndromes of the Central and Peripheral Nervous Systems Clinical Trials

• At NIH Clinical Center
• Throughout the U.S. and Worldwide

Organizations

American Autoimmune Related Diseases Association 22100 Gratiot Avenue Eastpointe, MI   48201-2227 aarda@aarda.org http://www.aarda.org Tel: 586-776-3900 800-598-4668 Fax: 586-776-3903	National Organization for Rare Disorders (NORD) P.O. Box 1968 (55 Kenosia Avenue) Danbury, CT   06813-1968 orphan@rarediseases.org http://www.rarediseases.org Tel: 203-744-0100 Voice Mail 800-999-NORD (6673) Fax: 203-798-2291
National Eye Institute (NEI) National Institutes of Health, DHHS 31 Center Drive, Rm. 6A32 MSC 2510 Bethesda, MD   20892-2510 2020@nei.nih.gov http://www.nei.nih.gov Tel: 301-496-5248	National Institute of Allergy and Infectious Diseases (NIAID) National Institutes of Health, DHHS 6610 Rockledge Drive, MSC 6612 Bethesda, MD   20892-6612 http://www.niaid.nih.gov Tel: 301-496-5717

Prepared by:
Office of Communications and Public Liaison
National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, MD 20892

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Last updated February 20, 2008