Vasculitis syndromes of the central and peripheral nervous systems

Vasculitis is inflammation of blood vessels (veins, arteries, and capillaries) that carry blood through the body. Vasculitis can affect blood vessels of any type, size, or location, including those in the central and peripheral nervous systems, where it affects the blood vessels that nourish the brain, spinal cord, and peripheral nerves. A vasculitis syndrome may begin suddenly or develop over time.

Inflammation from vasculitis can cause the walls of blood vessels to weaken, stretch, thicken, and swell or develop scarring, which can narrow the vessel and slow or completely stop the normal flow of blood. In the brain and elsewhere, the weakened vessel can burst, causing bleeding into surrounding tissues.

Vasculitis can cause strokes, blood clots, swelling, and other conditions that affect the brain and nervous system and other organs.

How vasculitis in the brain leads to stroke:

• Cerebral aneurysms (weak spots on a blood vessel in the brain that balloon out) can burst and spill blood into surrounding tissue (hemorrhagic stroke)
• Blood in the inflamed blood vessel can clot (thrombosis), blocking blood flow and causing ischemic stroke

Nervous system symptoms may include:

• Transient ischemic attack (TIA, sometimes also called a “mini-stroke”)
• Headaches, especially a headache that doesn't go away
• Swelling of the brain
• Confusion or forgetfulness leading to dementia
• Paralysis or numbness, usually in the arms or legs
• Seizures or convulsions
• Abnormal sensations or loss of sensation
• Trouble speaking or understanding
• Visual disturbances, such as double vision, blurred vision, or blindness

General symptoms may include:

• Fever
• Malaise (feeling restless and sad)
• Rapid weight loss
• Aches and pains in the joints and muscles
• Pain while chewing or swallowing
• Unusual rashes or skin discoloration
• Problems with the kidneys or other organs

Many forms of vasculitis can affect the brain, spinal cord, and nerves, including:

Giant cell arteritis (also known as temporal arteritis or cranial arteritis)

Giant cell arteritis affects the aorta, which brings blood to every branch of the body, including the arms, legs, and head. The temporal artery (found on both sides of the head and running across the temple) and the ophthalmic artery that supplies the eyes are often affected. Giant cell arteritis typically occurs in people age 50 years and older. Untreated temporal arteritis can cause strokes and even death. Symptoms include:

• New, severe headache
• Visual problems, including blurred or double vision, or sudden vision loss
• Pain in the jaw or tongue when chewing or swallowing
• Tenderness in the temporal arteries or the scalp

Primary angiitis of the central nervous system (also known as granulomatous angiitis). This rare disorder, which is confined to the brain and spinal cord, typically develops slowly and includes headache, dementia, behavioral changes, pain, sensory abnormalities, and tremor. Stroke, transient ischemic attack, multiple mini-strokes, and seizures can occur. The disorder can affect anyone of any age but peaks about age 50 and is most often seen in males. It is fatal if left untreated.

Takayasu's arteritis. This disease affects large arteries such as the aorta. It may cause strokes, headaches, dizziness, a feeling of cold or numbness in the limbs, problems with memory and thinking, and visual disturbances. It also may cause heart attacks and damage to the intestines. The disorder can cause partial to complete disability and can be fatal if left untreated. Most individuals with the disorder are female, with disease onset between the ages 15 and 40.

Polyarteritis nodosa. The onset of this rare disease can occur at any age but most often appears between the ages of 40 and 60. Men are affected more often than women. Damage to the peripheral nerves with neuropathy is more common than damage to the brain and spinal cord tissue. As the disease progresses the kidneys may fail and high blood pressure may develop rapidly. In some instances, the disease can recur after a few years. If untreated, the disorder is often fatal, ending in failure of vital organs.

Deficiency of adenosine deaminase 2 ( also known as DADA2). DADA2 is a rare, genetic form of vasculitis that is caused by a mutation in the CECR1 gene and can occur in more than one family member. Most individuals with DADA2 experience strokes in infancy or early childhood. DADA2 was discovered by researchers at the National Institutes of Health (NIH).

Kawasaki disease is a rare form of vasculitis that can cause stroke or brain damage in children. It primarily affects children age 5 or younger. Inflammation of the walls of blood vessels in the coronary arteries may cause aneurysms.

How is vasculitis in the nervous system diagnosed and treated?

Diagnosing vasculitis. Diagnosing vasculitis that affects the nervous system can be difficult, as some diseases have similar symptoms. Diagnosis depends on the number of blood vessels involved, their size, and their location as well as the types of other organs involved.

A doctor will review the person's medical history, perform a physical exam to confirm signs and symptoms, and order diagnostic tests and procedures, including:

• Blood and urine tests to look for signs of inflammation (such as abnormal levels of certain proteins, antibodies, and blood cells)
• Analysis of the fluid that surrounds the brain and spinal cord (cerebrospinal fluid) to check for infection and signs of inflammation
• Biopsy of brain or nerve tissue (involving removal of a small piece of tissue that is studied under a microscope)
• Diagnostic imaging such as computed tomography (CT) and magnetic resonance imaging (MRI) to provide images of the brain, nerves and other organs, and tissues, and detect weakened or blocked vessels.
• Angiogram (x-ray imaging using a special dye that is released into the bloodstream) to detect the degree of narrowing of blood vessels in the brain, head, or neck
• Ultrasound to produce high-resolution images of the blood vessel walls and organs to look for inflammation

Treating vasculitis. Treatment for vasculitis is aimed at reducing inflammation, controlling the anti-inflammatory response, and suppressing the abnormal immune system activity.

Medications to treat vasculitis include:

• Corticosteroid drugs such as prednisone to reduce blood vessel inflammation
• Immunosuppressive drugs such as cyclosporine to suppress or weaken the immune system
• Monoclonal antibodies that attach to certain abnormally functioning immune cells (B cells) and kill them
• Blood thinners and anti-clotting medications
• Immunoglobulin therapy, which involves intravenous injections of antibodies (protein produced by a blood cell to make a specific immune response) that are collected from a pool of healthy volunteers to lessen the infectious attack on the immune system

Other procedures to treat vasculitis include:

• Plasma exchange, or plasmapheresis, which involves taking blood out of the body and removing components in the blood's plasma that are thought to be harmful and then transfusing the blood plus replacement plasma back into the body
• Surgery to bypass blocked vessels or treat aneurysms (although this is rare)

What are the latest updates on vasculitis in the nervous system?

The National Institute of Neurological Disorders and Stroke (NINDS) is the main funder of research on the brain and nervous system and a component of the National Institutes of Health (NIH). Other NIH institutes that support research on vasculitis include the National Heart, Lung, and Blood Institute (NHLBI) and the National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS).

Several NINDS-funded investigators are studying blood vessel damage and cerebral blood flow as it relates to stroke. A better understanding of the mechanisms involved in injury and repair of blood vessels in the brain may lead to improvements in the treatment and prevention of other vascular disorders.

The Vasculitis Translational Research Program at NIAMS is dedicated to performing clinical and translational research in all types of vasculitis. Objectives of the program are to follow people with the disorder over time to see how it affects them and to discover new aspects of vasculitis that will help doctors learn how to better care for individuals with the disease.

NIH supports the Vasculitis Clinical Research Consortium, 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, including giant cell arteritis, polyarteritis nodosa, Takayasu's arteritis, and polyarteritis nodosa. The Consortium's website provides information about clinical research and clinical trial opportunities and helps individuals connect with expert doctors and patient support groups.

More information about NIH-funded research on vasculitis and other disorders can be found using NIH RePORTER, a searchable database of current and past research projects supported by NIH and other federal agencies. RePORTER also includes links to publications and resources from these projects.

How can I or my loved one help improve care for people with vasculitis in the nervous system?

Consider participating in a clinical trial so clinicians and scientists can learn more about vasculitis and related disorders. Clinical research uses human volunteers to help researchers learn more about a disorder and perhaps find better ways to safely detect, treat, or prevent disease.

All types of volunteers are needed—those who are healthy or may have an illness or disease—of all different ages, sexes, races, and ethnicities to ensure that study results apply to as many people as possible, and that treatments will be safe and effective for everyone who will use them.

For information about participating in clinical research visit NIH Clinical Research Trials and You. Learn about clinical trials currently looking for people with vasculitis at Clinicaltrials.gov.

Where can I find more information about vasculitis in the nervous system?

Information may be available from the following organizations and resources:

American Autoimmune Related Diseases Association
Phone: 586-776-3900 or 800-598-4668

National Heart, Lung and Blood Institute (NHLBI)
Phone: 301-592-8573 or 877-645-2448

National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS)
Phone: 301-495-4484 or 877-226-4267

National Organization for Rare Disorders (NORD)
Phone: 203-744-0100 or 800-999-6673

Content source: https://www.ninds.nih.gov/health-information/disorders/vasculitis-nervous-system Accessed July 17, 2023.

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