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What is Lupus?

What is CNS Lupus?

Lupus is an autoimmune disease that can affect almost any part of your body, including the nervous system and brain. There are several terms doctors use to describe this: neuropsychiatric lupus (NPSLE), neurocognitive dysfunction, or central nervous system lupus (CNS lupus). The nervous system has three parts, any of which may be affected by lupus. ·  The central nervous system (CNS)—The brain and spinal cord. ·  The peripheral nervous system (PNS)—The network of nerves that connects the brain and spinal cord to the rest of the body, and gives skin and muscles the signals needed for sensation and movement. ·  The autonomic nervous system (ANS)—Allows communication between spinal and peripheral nerves and the brain and internal organs, and controls functions like breathing, blood flow, and heart rate. People with lupus can experience a number of complications when their nervous system is affected. The symptoms may come on suddenly or may come and go, but they will vary depending upon the location and extent of the tissue injury. These symptoms also can be present in other diseases, so diagnosing lupus-related nervous system disorders is often difficult. Neurologists are physicians who specialize in the nervous system. They may rely on a number of diagnostic tools to determine whether lupus is involved in cognitive problems: ·      Brain scans (magnetic resonance imaging (MRI) and computed tomography (CT) ·     Electroencephalograms (to capture the electrical pattern of brain activity) ·      Spinal tap (to examine fluid in the spinal column) Behavioral and cognitive tests may also be done to find out if your memory or other mental abilities have been affected. Depending on the symptoms, a variety of medications are available to treat lupus-related nervous system disorders, including non-steroidal anti-inflammatory drugs, antimalarials, and steroids. Your response to treatment may be rapid or gradual over several months. For many people with lupus, nervous system involvement is completely reversible. Central Nervous System (CNS): 
When lupus affects your central nervous system, many symptoms may occur, including: ·      Headaches ·      Confusion ·      Fatigue ·      Depression ·      Seizures ·      Strokes ·      Vision problems ·      Mood swings ·      Difficulty concentrating
· Lupus Fog or Cognitive Dysfunction
 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 with lupus call it "lupus fog." Cognitive dysfunction most often affects people with mild to moderately active lupus. The causes of these symptoms, and the reasons the symptoms tend to come and go, are unknown. Living with cognitive dysfunction can be very frustrating. However, you can learn to improve your concentration and lessen confusion and memory loss with a variety of coping skills, including puzzles, games, biofeedback, using a daily appointment calendar, and balancing daily activities to reduce stress. Lupus Headache Compared with the general population, people with lupus may be twice as likely to experience migraine-like lupus headaches, commonly known as lupus headaches. The features of lupus headaches are similar to migraines and may be seen more often in people who also have Raynaud’s phenomenon. However, headaches can also be caused by vasculitis, a symptom of active lupus due to inflammation of the blood vessels. 
Medication Side Effects Medications used to treat lupus can cause side effects that are similar to the symptoms of CNS lupus. If you have symptoms of CNS lupus you should consult a neurologist who can determine which symptoms are side effects of medication and which are due to lupus. The drugs most known for causing symptoms like those of CNS lupus are: ·      Non-steroidal anti-inflammatory drugs (NSAIDs) – May cause headache, dizziness, confusion, and in rare instances, meningitis-like symptoms ·      Antimalarials – Very high doses (not usually given for lupus) may cause manic behavior, seizures, psychosis ·      Corticosteroids – May cause agitation, confusion, mood swings, psychosis, depression ·      Anti-hypertensive medications – May cause depression or loss of sex drive A serious form of lupus called CNS vasculitis may occur when there is inflammation of the blood vessels of the brain. Characterized by high fevers, seizures, psychosis, and meningitis-like stiffness of the neck, CNS vasculitis is the most dangerous form of lupus involving the nervous system and usually requires hospitalization and high doses of corticosteroids to suppress the inflammation.

Introduction to Lupus

According to the Lupus Foundation of America, 1.5 million Americans are living with some form of lupus today. But a diagnosis of lupus can have several different meanings: Some people experience only mild disease activity that becomes apparent when they exhibit the classic lupus rash; others can have a life-threatening type of lupus that affects almost every part of their body..

 The first lupus diagnosis dates back to the Middle Ages. The term "lupus" comes from the Latin word for wolf — the 12th century physician Rogerius thought that the skin lesions seen in lupus looked like a wolf's bite. It was not until 1872 that physicians documented a more severe form of lupus. Today, experts know that lupus is actually an autoimmune disease — a condition that occurs when the immune system turns against itself. Normally, the immune system makes proteins called antibodies that protect the body from infections. In people with autoimmune disease, however, the immune system loses the ability to tell the difference between outside invaders and the body's normal cells. The inflammation caused by "auto-antibodies" is what causes the symptoms seen in lupus.

- Chris Iliades,MD Medically reviewed by Lindsey Marcellin, MD, MPH, and Farrokh Sohrabi, MD


What are the different types of Lupus?

There are several types of lupus that are recognized today. These include:  ·      Cutaneous lupus:
This was the first type of lupus to ever be diagnosed. This form of the condition affects only the skin, causing thick, red, scaly rashes on the face, neck, and scalp. After the rash goes away it can leave scarring and can cause hair loss in the scalp area. The most common type of cutaneous lupus is called discoid lupus. The rash of discoid lupus may last for a few days or, for some, go on for years. It can go away for a long time and then come back. Discoid lupus is much more common in women than men and usually occurs between the ages of 20 and 45. In about 10 percent of people with discoid lupus, the condition progresses to systemic lupus, a more serious form of the disease. It may also be that these people already had systemic lupus, and the rash was just the first symptom. ·      Systemic lupus:
Systemic lupus is the most common type of lupus to be diagnosed. It is usually referred to simply as "lupus." This kind of lupus can affect almost any part of the body. Ninety percent of people diagnosed with systemic lupus are women in their child-bearing years. In the United States, the highest incidence is found in African-Americans. Symptoms of systemic lupus can be mild or severe and tend to come and go over time. Common symptoms include: o  Painful joints o  Fevers o  Rashes caused by sun exposure o  Hair loss o  Loss of circulation in toes or fingers o  Swelling in the legs o  Ulcers inside the mouth o  Swollen glands o  Extreme tiredness ·      Drug-induced lupus. This type of lupus diagnosis is very rare and occurs as a result of taking certain types of medications for a long period of time. The most common medications linked to this form of lupus are hydralazine (Apresoline), used to treat high blood pressure, and procainamide (Pronestyl), used to treat heart disease. However, many of the drugs that can cause drug-induced lupus are being used less frequently today. Symptoms may be similar to those of systemic lupus and can include muscle and joint pain, fever, and rash. Men are more likely to have drug-induced lupus than women. Once the medication is stopped, symptoms ofdrug-induced lupus typically go away within six months, and it does not lead to systemic lupus. ·      Neonatal lupus. This type of lupus diagnosis is also very rare. It occurs when a mother with certain kinds of lupus antibodies transfers them to her child at the time of birth. The mother may have these antibodies without having lupus herself. In fact, only about 40 percent of mothers of babies with neonatal lupus have lupus. Symptoms in the child can include a rash, anemia, and, very infrequently, heart problems. Heart problems may require the use of a pacemaker, but most children with neonatal lupus go on to lead normal lives. In the vast majority of cases, neonatal lupus does not need to be treated and disappears within a few weeks. Most mothers with lupus do not have babies with neonatal lupus. ·      Childhood lupus
Systemic lupus can also occur in children. Although many of the symptoms are the same as in adult lupus, childhood lupus is more common in boys and is more likely to affect the kidneys. Childhood lupus may require more aggressive treatment than adult lupus. Cutaneous, drug-induced, and neonatal lupus are milder forms of lupus that can resolve on their own. Although there is no cure for systemic lupus, drug treatment is usually effective in relieving symptoms, and people with systemic lupus can lead active, productive lives.

By Chris Iliades, MD Medically reviewed by Lindsey Marcellin, MD, MPH, and Farrokh Sohrabi, MD