15 Questions - Cognitive issues and headaches associated with lupus

(July 2012) As many as half of all people with lupus describe feelings of confusion, fatigue, memory loss, and difficulty expressing their thoughts. This collection of symptoms is termed cognitive dysfunction, although many people call it "lupus fog." In addition, people with lupus may be twice as likely to experience migraine-like lupus headaches, commonly known as lupus headaches. For this month, Dr. Meggan Mackay answered your questions about coping with cognitive issues and lupus headaches.


1. How does the doctor determine if memory or cognitive issues are related to my lupus? Glendora, CA

This is an excellent question that gets right to the heart of our difficulties with the diagnosis of brain involvement in lupus. We know that memory and cognitive function are affected by many things including medications, anxiety, depression and infection. For lupus patients this is particularly difficult since steroids and many of the other medications used to treat the illness are known to affect brain function. Depression and anxiety are also common in lupus patients but the same question applies; are the depression, anxiety or memory and cognitive difficulties due to lupus, medications or a reaction to having a chronic illness? Currently, there is no way for your doctor to know for sure. Based on the severity of the problem, your doctor may ask for certain studies to be done such as MRI of the brain and possibly a lumbar puncture (a procedure where a small amount of spinal fluid is removed for testing) but results from these tests may not give the answer. Unfortunately at this point we are unable to distinguish the effects of lupus on cognitive function and memory from the effects of medications and reactive depression or anxiety. On the bright side, ongoing research in this area is identifying ways in which lupus can affect the brain that will lead to specific imaging studies or other non-invasive ways to determine causes of cognitive impairment in lupus.

2. Can you have lupus fog but have nothing show up on neurological psych tests? Fishers, IN

Yes, this is possible. Neuropsychological tests are tasks designed to assess different kinds of cognitive functions and they include assessments of intelligence, different kinds of memory, reasoning skills, language skills and attention. Individual results of these tests are compared to “normative” scores that are drawn from a large population. One problem with neuropsychological testing is that they can only be given once a year and therefore are better suited to detect changes that are permanent and/or progressive. Changes associated with lupus fog may be transient and therefore not detected on the neuropsychological tests. Additionally, the neuropsychological tests are often not sensitive enough to detect changes within individual lupus patients over time.

3. I have both the Lupus Fog and lupus migraines. Is there anything I can do to improve cognitive function? What are some good activities to do to keep the mind sharp despite lupus fog? Vestal, NY & Elizabeth, NJ

Improving cognitive function has been the subject of many heated debates and much research. The issue is whether brain cells can be encouraged to communicate more efficiently resulting in improved memory, concentration and cognition. Although many diets, vitamins, supplements, games designed to enhance mental agility (such as puzzles) and drugs aimed at stimulating neurotransmitter function (neurotransmitters are chemicals released by brain cells that affects communication between brain cells) have been widely promoted, it has been difficult to prove that any are truly effective. Keeping a diary of when the Lupus Fog occurs is an excellent way to begin attacking the problem. You may notice a pattern that relates to certain activities in your daily life that are related to the Lupus Fog and the migraines. Staying physically fit and keeping a healthy diet are critically important to good cognitive health. This means avoidance of nicotine, excessive caffeine, alcohol and illicit drug use but, equally importantly, it means regular exercise. Improved organization can also help overcome the difficulties and frustrations associated with worsening memory and concentration. Health professionals such as cognitive therapists can also help you to determine strategies that may help you.

4. Can people with lupus still have memory and attention problems even if they are being successfully treated for their systemic lupus? Tuscaloosa, AL

Absolutely. Cognitive impairment occurs fairly slowly over time in lupus patients with little to no association with disease activity outside of the brain. The reason for this has to do with the blood brain barrier and how lupus causes cognitive problems. Some patients with lupus may have cognitive problems related to antiphospholipid antibodies; these are the antibodies associated with an increased risk for blood clots and many tiny blood clots in the small blood vessels that feed the brain can result in memory and cognitive impairment over time. However, many lupus patients do not have these antibodies but they still experience memory and cognitive problems. We think other autoantibodies affect the brain in a different way. The brain is surrounded and protected by a special membrane called the blood brain barrier. The blood brain barrier is a very well-designed safety feature that separates the brain from the bloodstream so that everything in the blood cannot get into the brain. Important nutrients and oxygen can cross the blood brain barrier but it is difficult for inflammatory cells and proteins to get into the brain unless the barrier is disrupted. Therefore inflammation from lupus that occurs outside the brain such as arthritis, skin rashes, kidney disease etc. generally does not affect brain function. On the other hand, we now know that some of the lupus autoantibodies do get past the blood brain barrier and cause problems with the brain cells. In animal models of lupus, both anti-NMDAR antibodies and anti-ribosomal P antibodies have been shown to bind to brain cells and either kill the cells or change their function in certain areas of the brain that leads to cognitive problems. We suspect that these autoantibodies (and possibly others) do the same thing in lupus patients which may explain why there is usually no association between disease activity outside the brain and cognitive problems.

5. Does experiencing lupus fog increase the risk of developing Alzheimer’s or other types of dementia later in life? Lexington, KY

No, Alzheimer’s disease and other forms of dementia occur for other reasons and are not related to lupus fog. For example, if you look at the brain of someone who died with Alzheimer’s disease, you will see lots of plaques made from amyloid proteins that built up in the brain and interfered with brain cell communication. As far as we know, there are no characteristic findings in brains from lupus patients that had been experiencing cognitive problems and very few lupus patients experience that degree of severe dementia.

6. My doctor has told me that my cognitive and nervous system issues are from cerebritis. I cannot find much information on this. Is this different than lupus that affects the nervous system?

Another excellent question. “Cerebritis” is a confusing old term that has been used in the past to describe all kinds of brain involvement in lupus. In 1999 a panel of experts in the American College of Rheumatology defined 19 ways in which the nervous system can be affected by lupus and one of these is cognitive dysfunction. Other problems that can happen from lupus in the brain include strokes, acute confusion, psychosis, depression and anxiety, seizures, headache and others. In the past, many people would lump all of these different problems into one diagnosis of “cerebritis”. We know now that it is important to classify the nervous system problems separately so that we can group patients with similar problems and learn more about how their disease is causing the problem. For example, if we put lupus patients with cognitive difficulties in the same group as those with psychosis, we will probably not learn much about what is causing either problem. The term “cerebritis” is outdated and more specific diagnoses should be used. Additionally, some people have used the term “cerebritis” to mean vasculitis which is an inflammation inside the wall of blood vessels. Vasculitis occurs extremely rarely in the brain and has not been associated with cognitive problems; it has been associated with acute confusional problems, psychosis and seizures.

7. My wife has been diagnosed with SLE and cardiolipin antibody antiphospholipid, what are temporary and/or permanent damage that could be to produce in her brain and how could be prevent it? Oaxaca, Mexico

Antiphospholipid antibodies are the autoantibodies that increase the risk of forming blood clots in the arteries or veins. Approximately 30% of lupus patients have these autoantibodies but it is important to know that having the antiphospholipid antibodies does not mean that you will form blood clots. In order to have the “Antiphospholipid Syndrome” someone must have the autoantibodies and also have had at least one blood clot or pregnancy loss. With respect to the brain, these autoantibodies increase the risk of forming a clot in a large blood vessel in the brain and causing a stroke. Clinical problems will depend on where the stroke is located in the brain; some patients may experience loss of function in an arm, leg or both or speech problems. Antiphospholipid antibodies have also been associated with a risk of developing cognitive problems as a result of recurrent tiny blood clots in the small blood vessels that feed the brain. You would not notice each blood clot because they are so small. The cognitive problems would occur slowly over time as a result of many of these tiny clots. If someone has the Antiphospholipid Syndrome, they are usually treated with blood thinners to prevent clot. However, someone with these antiphospholipid antibodies and NO history of blood clots or pregnancy loss would not be treated with anticoagulation to prevent clots in the brain or anywhere else. The risks of using blood thinners outweigh any possible benefits of using blood thinners to prevent future events in these patients. Low dose aspirin is commonly recommended although few studies have demonstrated this to be effective.

8. How common is psychosis related to lupus? What are the symptoms and treatments? Eastvale, CA

Psychosis is a condition characterized by severe disturbances in thought processing and patients will usually exhibit bizarre and irrational behavior accompanied by hallucinations (seeing, hearing or feeling things that are not real) and delusional thoughts (false beliefs). Psychosis is a condition that is associated with many different illnesses that include mental illnesses such as schizophrenia and bipolar disorder, infections in the brain or the body, hormonal imbalances and abnormal levels of different chemical or proteins in the blood (this includes illicit drugs). Fortunately psychosis that is caused by lupus is a fairly rare occurrence; several studies suggest that it occurs only in 5-10% of patients. When a lupus patient has symptoms of psychosis the most important thing is to determine if the psychosis is caused by lupus or another problem; it is important to note that some lupus patients react very badly to corticosteroids and develop psychosis from their corticosteroid medication (prednisone). Because there is no specific test that will prove the psychosis is from active lupus, patients will undergo many tests of the blood and spinal fluid to make sure that there is no evidence of infection or other problems. Usually lupus patients with psychosis will also have other symptoms of disease flare such as rash, hair loss, arthritis or inflammation in another place like the lungs, heart or kidneys. If the psychosis is felt to be from active lupus, the treatment is usually high doses of corticosteroids and immunosuppression (drugs such as Azathioprine, cyclophosphamide, Mycophenolate mofetil) along with psychiatric medications typically used to control psychosis. The decision to treat psychosis aggressively is often difficult and the doctor must carefully consider all of the possibilities to avoid adding more corticosteroids and immunosuppression to a patient whose psychosis is from corticosteroids or from an infection or drug use.

9. My rheumatologist has told me time and time again that headaches are not a symptom of lupus. I've always argued with him on this point. Can you clear this question up for me? West Melbourne, FL

This is a very difficult question. It is clear that some patients experience more severe and frequent headaches during disease flares, leading many to think that headaches are a symptom of lupus. However, as discussed in question # 11, some studies have failed to show any difference in the frequency of headaches between lupus patients and healthy controls and there is no characteristic pattern for a lupus headache. Very importantly, if headaches are be caused by lupus we do not understand the mechanism very well. While we usually treat other disease flares such as rash, arthritis and kidney disease with fairly strong anti-inflammatory medications, we rarely use these to treat headache. Most headaches associated with lupus are treated with headache or migraine medications. Whether headaches are from lupus is a question that can only be answered on an individual basis.

10. How might vasculitis contribute to my severe headaches and past history of seizures?

Vasculitis is an inflammation in the wall of blood vessels. This inflammation disrupts the normally smooth wall of the vessel and allows cells in the blood steam to stick to the wall of the blood vessel and eventually form a clot. The clot blocks blood flow to the tissue beyond it and in the brain this is a big problem since brain cells rely on the blood vessels to deliver oxygen and nutrients. Brian tissue that is fed by the blood vessel with the clot then dies. Depending on the size of the blood vessel with the clot, a small or large part of the brain will be affected and patients will experience symptoms of a stroke (loss of function in certain areas such as an arm, leg or speech) and/or seizures. Seizures are generally caused by multiple brain cells firing large electrical signals that overload the brain. Remember however, that vasculitis in the brain is rare; strokes leading to seizures in lupus are usually associated with antiphospholipid antibodies or other common causes of stroke such as atherosclerotic disease and hypertension. Headaches in lupus are not usually associated with vasculitis. In fact, much controversy exists as to whether lupus patients actually experience more headaches than people of the same age and gender without lupus.

11. I experience chronic migraines as part of my SLE. I am seeing a neurologist for treatment of them. Is that the best specialist, in your opinion? When should I seek medical attention due to the headaches? Raritan, NJ & Staten Island, NY

Severe headaches, including migraine headaches, have been associated with lupus for a long time. However, many studies have failed to show that lupus patients have more frequent headaches than a population of people of the same age and gender without lupus. There is no characteristic type of headache in lupus patients and the headaches do not appear to be the result of an inflammatory process in the brain. Spinal fluid (the fluid that surrounds the brain) does not show inflammatory cells or proteins in lupus patients experiencing headache and generally the headaches do not get better with medications such as corticosteroids and immunosuppressive drugs. Therefore it is always appropriate to seek the help of a neurologist for the treatment of migraine headaches. While it is important to continue with your rheumatologist to make sure the rest of your lupus is under control, a neurologist is very experienced with the treatment for migraine headaches. If you have been seeing a neurologist and your headaches follow a predictable pattern, there is no need to seek help frequently. However, you should always seek immediate help if the headache is particularly severe, you have a fever with it or you are experiencing problems moving or thinking.

12. I've had Lupus for 13 years I'm 32. The past few years I've developed what my rheumatologist called "the fog". I've always had migraines but never heard them referred to a lupus headache? What’s the difference and is there an association with the longer more frequent intense headaches and the fog I've experienced. I can be bed ridden for days to weeks at a time with debilitating pain. Springfield, OR

Lupus headaches are difficult to characterize and there is some discussion within the rheumatology community as to whether there is a specific “Lupus Headache”. Several different kinds of headaches including migraines, tension headaches, cluster headaches (headaches that occur on one side of the head and are associated with intense pain; they occur regularly for a few weeks and then do not recur for a prolonged period of time) and refractory headaches have all been associated with lupus. Headaches that developed years before the onset of lupus may not be related to lupus at all. Multiple studies have failed to demonstrate any consistent associations between headaches and other lupus disease activity or between the presence of antiphospholipid antibodies and Raynaud’s phenomenon and lupus headaches. Imaging studies of the brain also do not show any characteristic findings in patients that experience migraines or other forms of lupus headache. Lupus fog refers to cognitive problems associated with lupus that include difficulties with memory, concentration, speech and logical reasoning. Lupus fog is separate from lupus headaches and studies of have not found cognitive impairment in lupus patients with chronic headaches including migraine headaches.

13. What are some of the triggers to lupus headaches? Can sun exposure or excessive fatigue contribute to my headaches? Sulphur, LA

Several studies have failed to identify special triggers for headaches in lupus patients compared to age and gender matched subjects without lupus. Additionally, other studies have failed to show a consistent association between lupus headaches and other disease activity. However, as with most headaches in any patient population, if you notice that certain things trigger your headaches, avoidance of these triggers would be the best therapy! This is why most neurologists will encourage patients to keep a headache diary.

14. I started having migraine headaches shortly after being diagnosed with systemic lupus. A wonderful neurologist got them under control but now I'm having similar but not as intense headaches on a fairly regular basis. What is the preferred treatment for lupus headaches? Is it okay to take over the counter migraine medications for these headaches? Margate, FL

If you have a neurologist who was able to help with the migraine headaches, she/he should be consulted about these other headaches. Different kinds of headaches will respond to different types of treatment. As explained in question #12, “lupus headaches” include multiple headache types. There is no preferred treatment for “lupus headaches”; treatment will depend on the kind of headache you are experiencing.

15. Can my lupus headache cause vertigo or does this involve the lupus affecting other parts of the nervous system? What is the best way to treat vertigo? Warwick, NY

Vertigo is a condition where you experience the sensation of the room spinning around you. It can occur randomly and episodically and can be quite problematic. Severe attacks can result in incapacitating imbalance, nausea and vomiting. There are a few causes of vertigo; one is inflammation in the inner ear that is usually the result of an inner ear infection that clears up on its own. In some patients, vertigo is part of an aura that is associated with migraine headaches. More serious causes include things that directly affect the brain such as strokes or multiple sclerosis. Another more unusual cause of vertigo is Meniere’s disease; this is an inner ear problem that also causes tinnitus (constant ringing sensation in the ear). Some studies have shown an association between Meniere’s disease and autoimmune diseases such as lupus. There have been isolated reports of active brain disease in lupus causing vertigo however this is very rare.