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, coma, encephalopathy, stroke, seizures, and headaches. These problems may be related to vasculopathy (a disease affecting the blood vessels), autoantibodies, accelerated cardiovascular disease, and inflammatory molecules.
Lupus patients may also develop central nervous system vasculitis (CNS vasculitis). This condition is specific to inflammation of the brain and spinal cord’s blood vessels and is possibly one of the most serious complications 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.
Lupus can cause blood vessel abnormality through inflammation as well as other mechanisms. Signs and symptoms of central nervous system vascular involvement include high fevers, seizures (one-time or persistent), psychosis, neck stiffness, severe headaches, depression, encephalopathy, and coma.
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 determining the diagnosis.
This is a very generalized description, but once diagnosed, healthcare providers 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.
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.
The cause of these cognitive issues probably varies—current research suggests certain medications or an autoimmune response may be responsible. Treatment may vary depending on what is thought to be the most likely cause. Your healthcare provider may also prescribe steroids, or reduce them if you’re already taking them. Aspirin is another option: one study has shown aspirin may help prevent a decline in cognitive function. Treating any associated depression is also important, and some behavioral therapy might prove useful.
The prevalence of headaches in lupus patients is similar to control populations. Migraine and tension headaches are the most common. However, the sudden development of headaches, especially with associated neurological symptoms, should prompt evaluation for a more unusual cause of the headache (such as vasculitis).
It is estimated that about 20% of those suffering from 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 Healthcare Provider Wants to Know
It is important to let your healthcare provider know if you are suffering from any signs or symptoms that could be associated with the nervous system. Your practitioner 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. Tests and 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 antibody test.
- Complement, a blood test that measures the activity of certain proteins in the liquid portion of your blood.
- Antiphospholipid antibody test.
- Antiganglioside antibody test.
- Testing for antibodies to microtubule-associated protein 2.
Your healthcare provider might also conduct a series of neurological tests such as CT, SPECT (single-photon emission computed tomography) or MRI scans, electroencephalogram, a spinal tap (to check for cells, protein components, and antineuronal antibodies), or PET (positron emission tomography) scan.
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.