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NINDS Disorders is an index of neurological conditions provided by the National Institute of Neurological Disorders and Stroke. This valuable tool offers detailed descriptions, facts on treatment and prognosis, and patient organization contact information for over 500 identified neurological disorders.

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Vasculitis Syndromes of the Central and Peripheral Nervous Systems

Vasculitis is an inflammation of blood vessels, which includes the veins, arteries, and capillaries.  Researchers think that inflammation occurs with infection or is thought to be due to a faulty immune system response. 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; 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 blindnessAlthough 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.

Treatment

Treatment for a vasculitis syndrome depends upon the specific diagnosis.  Most of the syndromes respond well to steroid drugs, such as prednisolone.  Some may also require treatment with an immunosuppressive drug, such as cyclophosphamide.

Prognosis

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

Research

The National Institute of Neurological Disorders and Stroke (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 vasculitis.

View a list of studies currently seeking patients.

View more studies on this condition.

Read additional information from Medline Plus.

Organizations

National Eye Institute (NEI)

National Institutes of Health, DHHS
31 Center Drive, Rm. 6A32 MSC 2510
Bethesda, MD 20892-2510
Tel: 301-496-5248

NIAID Office of Communications and Government Relations

National Institutes of Health, DHHS
5601 Fishers Lane, MSC 9806
Bethesda, MD 20892
Tel: 301-496-5717

American Autoimmune Related Diseases Association

National organization that works to alleviate suffering and the socioeconomic impact of autoimmunity. Dedicated to the eradication of autoimmune diseases through fostering and facilitating collaboration in the areas of education, research, and patient services.

22100 Gratiot Avenue
Eastpointe, MI 48021-2227
Tel: 586-776-3900 800-598-4668
Fax: 586-776-3903

National Organization for Rare Disorders (NORD)

Federation of voluntary health organizations dedicated to helping people with rare "orphan" diseases and assisting the organizations that serve them. Committed to the identification, treatment, and cure of rare disorders through programs of education, advocacy, research, and service.

55 Kenosia Avenue
Danbury, CT 06810
Tel: 203-744-0100 Voice Mail 800-999-NORD (6673)
Fax: 203-798-2291

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