Command Central

When lupus affects the nervous system, many problems arise.

Jenny Allan - adapted from Lupus Now, Lupus Foundation of America, Spring 2005

It was last year, nine years after being diagnosed with lupus, that Patti Renfro started to notice problems with her memory. As an assistant manager of a 280-unit apartment complex in Nashville, Renfro found it hard to remember simple things that once came easy. It wasn't long before these memory lapses began to take a toll on her work.
"My short term started to go, and I was forgetting what things were called. I would even forget how to tie my shoes."
During this time Renfro says she was also having terrible headaches, dizzy spells, and sometimes a loss of vision where "all of a sudden, for seconds at a time, I couldn't see."
Renfro is like many people with lupus who suffer bouts of memory loss, headaches, strokes, and cognitive dysfunction ( which generally means a difficulty concentrating or reasoning ) - all symptoms that can arise when lupus affects the body's nervous system.

Nervous System 101
The nervous system is made up of the brain, the spinal cord, and the nerves throughout the body. It has three distinct parts :

and controls functions such as rate of breathing, blood flow, and heart rate.
We know a lot about how our nervous system works and we know that it controls all of our thoughts, memories, behaviours, and movements. We also know that different part of the brain control these functions ( see "Your brain at Work" ).
However, what doctors don't know much about is why and how lupus attacks the nervous systems and what, if anything can be done to prevent it.

When Lupus invades the nervous system
In 1999, the American College of Rheumatology ( ACR ) created a comprehensive definition for nervous system involvement in Lupus, and the term "neuropsychiatric systemic lupus erythematosus" is now commonly used to refer the condition.
Major signs that lupus has affected the nervous system
Reprinted with permission from "A Guide for Patients and Their families", Daniel J. Wallace, OUP
  HeadacheCognitive dysfunction ( eg. not thinking clearly )
  MeningitisVisual changes
  Movement disorders   Autonomic neuropathy ( eg. flushing, mottled skin)
  ParalysisAltered mental alertness
  SeizureAltered behaviour
  StrokePeripheral neuropathy ( eg. numbness, tingling and burning of the hands and/or feet )

Other experts agree on the shorter list ( shown here ) of definite symptoms that appear when the nervous system has been affected in a person with lupus. However, according the National Institute of Neurological Disorders and Stroke, although these symptoms may be present, it still can be difficult to know which ones can be directly attributed to lupus. John G Hanly, Queen Elizabeth II Heath Sciences Center at Dalhousie University in Halifax, Nova Scotia, notes that although nervous systems involvement in lupus was recognised more that 100 years ago, it is probably the least understood feature of the disease. "Our knowledge of the basic mechanisms and the consequences of nervous system disorders is not as advanced as with other organ system involvement, like the kidneys or the skin" says Hanly.
C Michael Neuwlet, who teaches medicine at the University of California-San Francisco and Stanford University, agrees. "Central nervous system lupus is probably the most common manifestation of lupus, but exacting criteria are lacking" says Neuwelt, who also treats rheumatic diseases at East Bay Rheumatology Group in San Leandro, CA. "When the brain is involved, we really don't have well-defined markers."
Most researchers do agree, however, that cognitive function may offer the best clues to how lupus affects the nervous and how lupus itself may lead to problems in cognition and behaviour.

Cognitive Dysfunction - aka "Lupus Fog"
Robin Brey, a neurologist at the University of Texas Health Chapter, says that cognitive dysfunction definitely is the most common system of CNS involvement that she sees. "The way I relate to the term 'cognitive dysfunction' is that it's a problem of difficulty finding words and retrieving information," Brey explains. "My patiens say it feels like they have brain fog."
Indeed, the term "lupus fog" is almost universally known to people with the disease. According to Brey, the difficulty in retrieving information was evident in patients who participated in the National Institute of Health-funded study on cognitive function - named Brain Conections - that was recently completed. "Often in lupus, people have difficulty accessing information that is in their brain," she says.
Those in the Conections study were evaluated every four months with a wide variety of tests, including neurological and rheumatological exams. In one test, participants were given a list of words, and after about 10 minutes were asked to recall as many of the words as they could.
"They often don't do very well" says Brey. "But if we then show them a list of 20 words, they can pick out the ten we originally showed them. So, there is an interference with access to the information that exists in their brains."
Brey says patients are frustrated by their inability to retrieve information they know they've learned. "Our patients know the memory is there and stored, but the more they try, the more frustrated they get" she says. "Then it is even harder to remember."
Brey suggests that people first realise that being frustrated or anxious makes the problem worse. "Try to decrease that frustration by not worrying about it" she says. "If you have trouble remembering the word you want, use a different word. And also make lists that can prompt you to remember."
These type of strategies and memory aids are really the best tools to cope with "lupus fog" because there are not yet any medications that can treat the condition. "There is no medicine for this" Brey says. "That's partly because at this point we don't know why it happens. It could be an effect of antibiotic use, or it could be a brain chemical malfunction. We need more research to figure that out."

What about "Lupus Headache" ?
If cognitive dysfunction is the most common symptom seen in neuropsychiatric lupus, headache comes in second. However, the existence of a specific type of headache that is a symptom of neuropsychiatric lupus is still open to debate.
According to the book, Systemic Lupus Erythematosus, most experts agree that there is a higher prevalence of headaches seen in people with lupus than in any other group. Yet, scientists have been unable to make a correlation between lupus and the clinical or neuropsychiatric symptoms. This raises the question whether headache really represents active neuropsychiatric lupus. Also, the term lupus headache was deemed too vague for ACR's definitions of neuropsychiatric lupus, which instead describe specific types of headache - such as migraine and tension - and includes detailed criteria that classify each type.
Brey notes that often people with lupus are so concerned with other symptoms and issues related to their disease that they don't feel the need to talk with their doctor about headaches. But "if people with lupus have headaches that are not handled by Tylenol, it is really important to bring it up with the doctor to get treatment" she says.
She adds that her research indicates that fewer than half of lupus patients with headaches are offered treatment by their regular doctors. "The same strategies that work in people without lupus - biofeedback, relaxation techniques and medicinal treatment - will work for those with lupus" she says.

Making the diagnosis
According to the ACR, in order for your doctor to assess whether there has been some nervous system involvement due to lupus - and also to exclude other causes of the neurological symptoms - a variety of tests are needed :

Perhaps the main difficulty in diagnosing neuropsychiatric lupus, though, is that many of its symptoms occur in the absence of active lupus.
 

Disclaimer :
The Lupus Group of W.A. (Inc.) does not recommend or endorse any products, drugs, treatments, procedures, medical or health professional in this article. We suggest you discuss this information with your doctor or specialist.