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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 blood vessels, which includes the veins, arteries, and capillaries.  Inflammation occurs with infection or is thought to be due to a faulty immune system response. It also can be caused by other immune system disease, an allergic reaction to medicines or toxins, and by certain blood cancers.  Vasculitic disorders 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 vasculitic syndrome may begin suddenly or develop over time.  Symptoms include headaches, especially a headache that doesn’t go away; fever, rapid weight loss; confusion or forgetfulness leading to dementia; swelling of the brain,  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

Some of the better understood vasculitis syndromes are  temporal arteritis (also called giant cell arteritis or cranial arteritis--a chronic inflammatory disorder of large blood vessels) and Takayasu’s disease, which affects larger aortas and may cause stoke. 

Is there any treatment?

Treatment for a vasculitis syndrome depends upon the specific diagnosis, which can be difficult, as some diseases have similar symptoms of vasculitis.  Most of the syndromes respond well to steroid drugs, such as prednisolone.  Some may also require treatment with an immunosuppressive drug, such as cyclophosphamide.  Aneurysms involved with vasculitis can be treated surgfically.

What is the prognosis?

The prognosis is dependent upon the specific syndrome, however, some of the syndromes are fatal if left untreated.

What research is being done?

The mission of the National Institute of Neurological Disorders and Stroke (NINDS) is to seek fundamental knowledge of the brain and nervous system and to use that knowledge to reduce the burden of neurological disease.  Several NINDS-funded investigators are studying blood vessel damage and cerebral blood flow as it relates to stroke.  The NINDS also funds research on vascular cognitive impairment, which is an important contributor to aging-related cognitive decline and is the result of impaired performance of the brain's small blood vessels.  Additionally, the NINDS and other institutes of the National Institutes of Health (NIH) conduct research relating to vasculitis syndromes in laboratories at the NIH and also support vasculitis research through grants to major medical institutions across the country.  The NINDS supports The Vasculitis Clinical Research Consortium (VCRC), a network of academic medical centers, patient support organizations, and clinical research resources dedicated to conducting clinical research and improving the care of individuals with various vasculitis disorders.

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


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

NINDS health-related material is provided for information purposes only and does not necessarily represent endorsement by or an official position of the National Institute of Neurological Disorders and Stroke or any other Federal agency. Advice on the treatment or care of an individual patient should be obtained through consultation with a physician who has examined that patient or is familiar with that patient's medical history.

All NINDS-prepared information is in the public domain and may be freely copied. Credit to the NINDS or the NIH is appreciated.

Last Modified May 12, 2016