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Lupus and the Nervous System

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Updated March 13, 2008

The three main components of the nervous system are the central nervous system (brain and spinal cord), the peripheral nervous system (limbs and organs), and the autonomic nervous system (a control system, maintaining homeostasis in the body). The role of lupus in the autonomic nervous system remains unclear.

In this overview, we will focus mainly on the central nervous system and some of the ways lupus affects the system.

The Central Nervous System:

The effects of lupus on the central nervous system are wide ranging. Lupus can cause a number of central nervous system complications, including, but not limited to, cognitive dysfunction, seizures, strokes, and headaches. These problems may be related to vasculopathy (disease affecting the blood vessels), autoantibodies, accelerated cardiovascular disease and inflammatory molecules. Lupus patients may also develop central nervous system vasculitis.

Central nervous system vasculitis (CNS vasculitis) is specific to inflammation of the brain and spinal cord’s blood vessels, and is possibly the most serious complication associated with systemic lupus erythematosus (SLE). Its occurrence as a primary disease is less frequent than its occurrence as a secondary disease, as with SLE.

Signs and symptoms of central nervous system vasculitis include high fevers, seizures (one-time or persistent), psychosis, stiffness of the neck, and severe headaches that are non-responsive to conventional treatment.

Seizures and strokes may occur independent of vasculitis and may be related to autoantibodies that increase the risk of stroke.

CNS vasculitis is difficult to diagnose, and is sometimes considered a diagnosis that comes via a team effort. Most tests, such as CT (computed tomography) scans, MRIs (magnetic resonance imaging), and spinal fluid samples provide clues more than determine the diagnosis.

This is a very generalized description, but once diagnosed, physicians may treat true CNS vasculitis via a combination of high doses of corticosteroids and cyclophosphamide, given in a hospital setting.

Nearly 10% of all lupus patients could encounter this form of vasculitis and it is the only form of central nervous system disease included in the American College of Rheumatology criteria for defining SLE.

Cognitive Dysfunction:

Lupus patients may, at some points in their life, feel confused, have difficulty expressing themselves, and discover some memory impairment, all associated with their disease. Collectively, these signs and symptoms are labeled as cognitive dysfunction. The reason they are associated with lupus is unknown.

Symptoms associated with cognitive dysfunction are often managed with anti-malarials or corticosteroids. Some behavioral therapy might also prove useful.

Headache

Headaches associated lupus are similar to migraines in their intensity and are usually treated similarly. Corticosteriods can also be helpful.

Fibromyalgia :

It is estimated that about 20% of those suffering with SLE also have fibromyalgia, a disorder causing muscle pain and fatigue in specific areas of the body, such as the neck, shoulders, back, hips, arms, and legs. They’re called “tender points” because they are tender to the touch. This is often treated with pain medications and physical therapy, while the emotional effects of the disorder are treated with antidepressants and counseling.

What Your Doctor Wants to Know:

It is important to let your doctor know if you are suffering from any signs or symptoms that could be associated with the nervous system. Your doctor will want to determine the exact cause.

He may conduct a number of tests, including a physical exam and a laboratory evaluation, which could include blood work and urinalysis. Exams specific to determining and detecting nervous system involvement in lupus include:

  • Sedimentation rate, a nonspecific screening test that indirectly measures how much inflammation is in the body.
  • ANA (antinuclear antibody) test, which identifies autoantibodies that attack the body's own tissues and cells.
  • Anti-DNA test, which confirms whether there are antibodies being produced to the genetic material in the cell.
  • Anti-ribosomal P antibodies test.
  • Complement, a blood test that measures the activity of certain proteins in the liquid portion of your blood.

Your doctor might also conduct a series of neurological tests such as CT, SPECT (single photon emission computed tomography) or MRI scans, electroencephalogram, spinal tap (to check for cells, protein components and antineuronal antibodies), or PET (positron emission tomography) scan.

Treatment:

Specific treatments are noted under the categories above, but it should be noted that response to individual treatment runs the gamut from dramatic to gradual. But for most, nervous system involvement is completely reversible.

Sources:

Nervous System. Lupus Foundation of America. January 2008.

What is Vasculitis?. National Heart, Lung and Blood Institute. August 2006.

What You Need to Know About Central Nervous System Vasculitis. Cleveland Clinic. Department of Rheumatic and Immunologic Diseases. October 2006.

Fast Facts about Fibromyalgia. National Institute of Arthritis and Musculoskeletal and Skin Diseases.

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