By David B. Hellman, M.D.
In spring Sheila had been like any other 18 year old – happy, healthy, and eager to start college. But during the summer she developed joint pains, low grade fevers and a facial rash. A doctor found that she had anemia. In the fall she entered the hospital after she developed grand mal seizures that caused unpredictable loss of consciousness associated with rhythmic jerking of her arms and legs. From the parents I also learned Sheila’s personality had changed; The young woman with a dynamic personality and excellent grades had become withdrawn and prone to mutter things that did not make sense to anyone else. When I met her I could see that she had a redness of her cheeks in the “butterfly” pattern. Sheila was also hallucinating, mumbling about people she thought she saw under her bed. When it came to answering questions, Sheila, who had won a math contest in high school, could not accurately solve simple arithmetic problems. Her laboratory test confirmed my suspicions: Sheila had lupus affecting her brain. Her experience raises several questions.
Why is the brain important in lupus?
Lupus involvement of the brain or central nervous system (CNS) is important because it occurs so frequently and shows up in many different ways. Also, other conditions and diseases can mimic central nervous systems lupus, so precise diagnosis is both critical and difficult. The unique challenges of lupus and the brain test the patience of the patient, the support of family and friends, and the skill of the doctor. Happily, most patients with CNS lupus respond to the different treatments that are available.
How commonly does lupus affect the brain?
Many studies have shown that about half of patients with lupus develop brain involvement. For example, the late Mary Betty Stevens found that 37 percent of the patients studied during 1970-1975 and 52 percent of the patients studied during 1970-1975 and 52 percent of patients evaluated from 1980-1984 experienced nervous system disease caused by lupus. Although CNS disease can develop at anytime, the most frequent time of onset is in the first year of systemic lupus erythematosus (SLE). Patients who do not have CNS disease in the first year or two are less likely to ever develop brain disease.
What type of problems are caused?
One of the most striking features of SLE is the many different ways it can affect the brain. In most studies, depression, organic brain syndrome, and other psychiatric problems are the most common manifestations of CNS lupus. The cause of depression can be difficult to discern. In some people depression may be the temporary consequence of demoralizing circumstances. In other people depression appears to result from a direct effect of lupus on the brain. Organic brain syndrome is the label used to describe patients who have important impairments in their ability to think or to analyze problems. For example, Sheila’s inability to solve simple math problems indicated that she had developed an organic brain syndrome. Hallucinations, such as Sheila also experienced are another common psychiatric manifestation of lupus and the brain. Other neurological consequences of SLE can be seizures, strokes, balance problems, visual abnormalities, movement disorders, and spinal cord inflammation. Almost no patient with neurological disease develops all of these different manifestations. But most patients do present with more than one neurological problem. On this point Sheila was typical in that she suffered not only from seizures but also from hallucinations and an organic brain syndrome.
How does lupus cause these neurological symptoms?
Several mechanisms appear to be important. Some neurological problems are caused by inflammation around or in the blood vessels (vasculitis). An abnormal tendency in lupus to form blood clots accounts for some of the strokes which occur. Antibodies that attack the brain itself may be responsible for some of the other neurological manifestations.
How does the doctor determine that a neurological problem is caused by lupus?
The diagnosis of CNS lupus can be difficult and often requires the doctor to use many different types of information and tests. First, the doctor looks for other evidence of SLE, since most patients with CNS disease have active disease elsewhere in the body. For example, Sheila had active skin and joint disease from SLE. And her blood and urine tests showed she also had a low platelet count and protein in the urine. The presence of active SLE in other organs makes it more likely that any new neurological symptom is caused by lupus. Second, the doctor considers the type of brain problems the patient has. Some problems, such as the seizures, hallucinations, and organic brain syndrome are very common in SLE. Third, the doctor will use laboratory tests to make sure the neurological problems do not have another cause. Infections, reactions to medications, kidney failure, and severe hypertension are among the illnesses that can affect the brain and mimic CNS lupus. The diagnostic process may require the patient to undergo blood tests, CAT scans or MRIs of the brain, and spinal fluid analysis. All of this information helps the doctor make an accurate diagnosis.
How is CNS disease treated?
The treatment depends in part on what problems the patient has. Patients suffering seizures will respond, in part, to anticonvulsants. Other patients who develop strokes because of an abnormal tendency to form blood clots will need anticoagulants. Most patients will also need medications like prednisone or Cyclophosphamide (Cytoxan) to diminish the immune system’s attack on the brain and the rest of the body.
What is the prognosis for patients with involvement of the brain?
Overall, the prognosis is quite good. Dr. Steven’s study showed that patients with CNS disease as a group survived as well as patients without neurological disease. Recovering from CNS disease can be slow, requiring weeks or months. Such was the case for Sheila who gradually improved and eventually completed college.