Neuropsychiatric SLE: Lupus and the Brain

Kyriakos A. Kirou, MD, DSc, FACP
Kyriakos A. Kirou, MD, DSc, FACP

Director, Lupus Nephritis Program, Hospital for Special Surgery
Clinical Co-Director, Mary Kirkland Center for Lupus Care, Hospital for Special Surgery
Assistant Attending Physician, Hospital for Special Surgery
Assistant Scientist, Hospital for Special Surgery

Systemic lupus erythematosus (lupus) may affect any organ of the human body. When lupus affects the brain, spinal cord, or nerves, we call this neuropsychiatric SLE (NPSLE).

NPSLE is one of the most difficult problems for people with lupus as it is often serious and also not well understood. Lupus of the nervous system may involve symptoms. It is very hard to know whether NPSLE symptoms are indeed due to lupus, versus due to other common problems because some symptoms are also frequent in healthy people. For example, headaches can be due to lupus but can also be due to migraines, tension headaches, sinus problems etc. Similarly, mild depression, mild memory problems, and neuropathies can be due to lupus or other common conditions.

Common Symptoms

The most common lupus-related nervous system problems include:

  • seizures
  • severe depression
  • and stroke.

These problems usually occur at times of lupus flares in other body organs as well (such as arthritis, skin rash, or kidney disease). To be sure, doctors first exclude other causes for these problems such as atherosclerosis (hardening of the blood vessels) in a patient with a stroke, and brain injury or tumor in a patient with seizures.

Somewhat less common neuropsychiatric lupus problems include sudden confusion, psychosis, and neuropathy which can cause foot weakness or numbness. Mild memory problems are not unusual. However, severe dementia is very uncommon. Finally, other more rare problems include transverse myelitis, and non-infectious meningitis. Transverse myelitis is a serious condition due to inflammation affecting the spinal cord. If untreated, it may cause bladder incontinence and paralysis of the legs.

Causes of NPSLE

Doctors caring for a lupus patient with neurologic problems always try to exclude and treat infections first, as the immune system is weak from the disease and the drugs used to treat it.  Another common diagnostic problem is the “mood swings” that are due to high doses of steroids (such as prednisone). Tests that are often used to help diagnosis of neuropsychiatric lupus may include spinal tap to remove fluid for analysis, electroencephalogram (EEG) to diagnose seizures, nerve conduction studies in case of neuropathies, and MRI to image the brain or spinal cord.

How lupus causes these neuropsychiatric problems is not very clear. In many cases, it may be due to autoantibodies (antibodies that are against our own body). These antibodies, under certain conditions, are able to reach the brain and cause inflammation. In other cases, the problems are due to the release of proteins that cause inflammation within the brain. In general, these problems happen when the lupus is very active (flaring). Strokes can occur in patients that have antiphospholipid antibodies in their blood. These antibodies are known to increase risk for blood clots which are usually the cause of stroke. 

Treatment Options

The treatment of neuropsychiatric lupus depends on the particular problem and its cause. When the problem is due to very active lupus (flaring), the disease is treated with steroids and immunosuppressive drugs (such as cyclophosphamide, mycophenolate mofetil, azathioprine etc). In strokes that are due to blood clots, blood thinners are used, when there is no contraindication. Seizures due to lupus are also treated with anti-seizure medications.

In conclusion, neuropsychiatric SLE is a serious complication of lupus that requires prompt attention and treatment. Good communication of the patient and doctor is very important for early diagnosis of the problem and effective treatment. The rheumatologist usually works together with a neurologist for the best care for these patients. When patients are treated this way, the results are often very good and rewarding to both patients and doctors.


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