Description

Autoimmune encephalitis (en-sef-uh-LIE-tis) is a group of conditions that cause inflammation in the brain. This happens because the immune system mistakenly attacks the cells of the brain. Autoimmune encephalitis symptoms can vary but may include loss of memory, changes in thinking, behavior changes and seizures.

Autoimmune encephalitis is different from the encephalitis caused by viral or bacterial infections, known as encephalitis infectious. Infectious encephalitis is not caused by an immune reaction, and is treated with different medicines. The research has found that the number of people with autoimmune encephalitis is comparable with the number of people with encephalitis infectious.

Experts do not know what causes autoimmune encephalitis, also known as AE. For some people, the AE is triggered by certain types of cancer or infections. Autoimmune encephalitis can also be caused by medications. People with an autoimmune disease or a family history of autoimmune disease may be more likely to get AE. Health professionals use various tests to diagnose the autoimmune encephalitis.

Without treatment, the autoimmune encephalitis can cause serious complications, including death. But treatment can lead to recovery. Many people with AE to make a full recovery, but some may have lasting symptoms.

Symptoms

Autoimmune encephalitis symptoms can vary from person to person. But symptoms may occur in a predictable pattern depending on the type of autoimmune encephalitis. Many people have a headache, fever and other symptoms of an infection followed by:

  • The psychiatric symptoms that may include anxiety, panic attacks, changes in behavior, agitation, hallucinations, delusions, and difficulty organizing thoughts.
  • Problems with the memory.
  • Problems with speech, such as speaking less, or the repetition of words or phrases.
  • Movements that are not voluntary.
  • Seizures.
  • The changes in consciousness.
  • Less hours of sleep at the beginning of the disease, followed by the excess of sleep during the recovery.

Sometimes AE causes severe seizures that may need emergency care, known as status epilepticus. These seizures last more than five minutes or occur one after the other while the person is not conscious.

Autoimmune encephalitis symptoms may get worse over time. This course of the disease is known as progressive. The symptoms can also toggle between improving and getting worse. This course is known as relapsing-remitting. These are similar to the illness of the courses of experiences of people with multiple sclerosis.

When to see a doctor

Get emergency medical care if you or someone you are with severe symptoms of AE. This includes having a seizure that lasts more than five minutes or happen one after another, with a loss of consciousness. Also seek emergency medical attention by high fever and difficulty breathing.

Consult with your healthcare provider right away if you have other symptoms of autoimmune encephalitis. AE can become serious quickly if not treated.

Causes

Autoimmune encephalitis causes are not known. Autoimmune encephalitis, also known as AE, occurs when the immune system mistakenly attacks heathy cells of the brain.

Antibodies are part of the immune system. Help to protect the body against viruses, bacteria and other substances that can cause disease. But in autoimmune encephalitis, the antibodies target and attach themselves to certain receptors in the brain. This leads to inflammation in the brain, also known as the inflammation and other symptoms.

AE can be caused by:

  • Certain types of cancer. When this happens, it is known as paraneoplastic AE.
  • Infections, such as herpes simplex virus.
  • Certain drugs, such as monoclonal antibodies and the use of medications to suppress the immune system after a transplant.

Autoimmune encephalitis is more likely to occur in people who have an autoimmune disease or have a strong family history of autoimmune disease.

Types of

There are several different types of AE. Each type of autoimmune encephalitis is caused by the immune system attacks different receptors in the brain. Some of the types and the causes include:

  • Anti-NMDA-receptor autoimmune encephalitis. This is the most common type of AE. In this type, the antibodies attack the NMDA receptor in the brain. It often affects young women and children and can cause seizures, facial movements, psychosis, and other symptoms.
  • Anti-GABA-B receptor encephalitis. This type of EC is associated with a tumor, often small-cell lung cancer. This type of AE can cause seizures, confusion and loss of memory. The average age of diagnosis of this type, it is 60.
  • Anti-GABA-a receptor encephalitis. People with this type of AE are usually diagnosed around the age of 40, but children may also have this type of AE. This type of AE can cause seizures, movement disorders, and changes in thinking and behavior.
  • Anti-AMPA receptor encephalitis. This type of AE usually affects women and may cause confusion and memory loss. The average age of diagnosis is 62 years of age.
  • Anti-LGI1 encephalitis and limbic. This type of autoimmune encephalitis tends to affect men. Can cause memory loss, confusion and seizures. The average age of diagnosis is 60 years of age.
  • Anti-CASPR2 associated encephalitis. People with this type of AE often have symptoms that include confusion, loss of memory, problems with sleep, nerve pain, and other symptoms. This type can also cause a rare condition known as syndrome of Morvan. Morvan syndrome can cause hallucinations, loss of memory, changes in blood pressure and painful cramps. Men are more likely to have this type of AE. The average age of diagnosis is 60 years.
  • Anti-IgLON5 disease. People who have this type of AE has a dream-like symptoms include behaviors and movements after falling asleep. The average age of diagnosis is 64.

Risk factors

The risk factors of autoimmune encephalitis, also known as AE, which include:

  • After having had the AE in the past, especially if it was not treated.
  • After you have had herpes simplex virus encephalitis.
  • Taking monoclonal antibodies or use of medications to suppress the immune system after a transplant.
  • Have a tumor, especially small-cell lung cancer.

Researchers are studying whether certain genes may be associated with autoimmune encephalitis.

Complications

Serious autoimmune encephalitis, also known as AE, it can lead to complications such as:

  • The attacks that you need emergency care, known as status epilepticus. The attack of the immune system in the brain during the EC can lead to seizures, and a condition called epilepsy, autoimmune. Sometimes seizures can last for more than five minutes or occur one after the other. The person is not aware between the seizures. These severe attacks which is known as status epilepticus.
  • There is not enough air that enters the lungs, known as respiratory failure. Persons with respiratory failure may require treatment with a machine that helps with breathing, known as mechanical ventilation.
  • Problems with the heart rhythm and blood pressure. AE can affect heart rate, blood pressure, digestion, and urination. These are known as autonomous functions.
  • Fevers. People with AE may have a high fever.

Another possible complication is that the condition may recur after recovery. This is known as a relapse. A relapse is more likely in people who had anti-LGI1 limbic encephalitis anti-CASPR2 associated encephalitis.

Prevention

Autoimmune encephalitis, also known as an AE, you can't always prevent. But getting cancer screenings can help your health care professional detect tumors and treated early. This could prevent the autoimmune encephalitis triggered by the cancer. Talk with your healthcare provider about your risk of cancer and if the cancer screening is recommended.

Diagnosis

Autoimmune encephalitis diagnosis involves a review of your symptoms, a physical examination and various tests. It is important to obtain an accurate diagnosis because autoimmune encephalitis, also known as an AE, you may be confused with other diseases.

The experts have created autoimmune encephalitis criteria to help health professionals diagnose individuals with AE. The health care professionals to search for patterns of symptoms that signal of AE. Also the proof of the signs that the antibodies are attacking the receptors in the brain to cause of the AE.

The tests can also help rule out other possible causes of your symptoms, such as infections or other autoimmune diseases.

Sometimes people are incorrectly diagnosed with autoimmune encephalitis. It is important for health professionals to consider all the possible conditions when making a diagnosis.

Laboratory tests

The evidence for autoimmune encephalitis includes the search for antibodies. AE is caused by antibodies in the brain that attack the proteins and receptors in the brain and cause symptoms.

Some tests can be done in the blood. Other tests are performed in the fluid that surrounds the brain and spinal cord, known as the cerebrospinal fluid. The cerebrospinal fluid is removed using a procedure known as a lumbar puncture. During the procedure, a healthcare professional will numb the lower part of the back and uses a hollow needle to remove cerebrospinal fluid for testing.

Images of the brain

Your healthcare provider may also recommend an mri of your brain. Mris can look for signs of autoimmune encephalitis or rule out other causes of your symptoms.

Other imaging tests may look for signs of cancer that may have motivated the AE.

EEG

An electroencephalogram, also known as an EEG, a test of the electrical activity in the brain. It can be shown that seizure activity and may help your healthcare provider diagnose AE. An EEG can also help rule out other conditions.

Treatment

Autoimmune encephalitis treatment focuses on the immune system, which mistakenly attacks the cells of the brain. If a tumor is causing autoimmune encephalitis, the first step is to diagnose and treat the tumor.

Medications

Immunotherapy works by reducing the activity of the immune system and improvement of the inflammation.

Two types of immunotherapy is administered INTRAVENOUSLY in a vein in the arm. The majority of the people of this therapy in a hospital. The medications include:

  • Methylprednisolone (Solu-Medrol). This high-dose steroid that is given daily for 3 to 7 days.
  • The Immunoglobulin (Ivig). This medication can be given daily for 2 to 5 days.

Other treatment options include:

  • Oral corticosteroids. With this medication, you start with a larger dose and then slowly lower your dose over weeks to months, known as tapering.
  • The Plasma exchange. This therapy gets rid of antibodies that are causing the immune system to attack the cells of the brain. During the exchange of plasma, the liquid part of the blood is extracted and separated from your blood cells. The blood cells are put back in your body and your body makes more of plasma.

If your symptoms respond to immunotherapy, the medication dose will be slowly lowered over time, known as tapering. You can take an oral corticosteroid at a lower dose over several weeks. Or you can get a monthly dose of methylprednisolone or immunoglobulin through an IV for several months.

If your symptoms do not improve, your healthcare provider may also recommend medicines rituximab (Rituxan, Truxima, others), cyclophosphamide, or tocilizumab (Actemra, Tofidence, Tyenne). These medications can improve symptoms and help to lower the chances of AE to return.

Most people recover with treatment. Before you receive treatment, the more quickly you can recover. Early treatment decreases the odds of having symptoms lasting due to AE or have another outbreak of autoimmune encephalitis.

Therapies

You may need treatment of the complications, such as epilepsy, the dream of the conditions and problems with movements. You may also need rehabilitation if AE affected his memory, thinking skills or speech. Occupational and speech therapists, along with mental health professionals and other specialists, you can assist in your recovery.

If the cancer is active autoimmune encephalitis, is treated for the cancer and monitored to check if the cancer comes back. Sometimes, people with other symptoms related to AE you need to get the regular care of specialists.

The long-term prognosis may vary from person to person. Complete recovery may take months or years. Many people continue to have symptoms related to the thinking and behavior of more than a year. But treatment continues to improve the symptoms of 18 months to two years.

Some people recover fully, while others may have symptoms that last, that are mild or more serious. Early treatment also helps to improve the long-term prognosis.

People who have recovered from certain types of autoimmune encephalitis, such as anti-NMDA receptor encephalitis, anti-LGI1 encephalitis, they run the risk that the symptoms return. Sometimes, the symptoms return after several years.

Preparing for your appointment

Autoimmune encephalitis can cause severe symptoms that need emergency medical care. Your care team includes specialists, including doctors who specialize in the brain and the nervous system, known as neurologists.

If their symptoms are not so severe, you could start by seeing your regular health care provider. Your health care professional may refer you to a neurologist or other specialist. Here's some information to help you prepare for your appointment.

What you can do

When you make the appointment, ask if there is something that you need to do in advance, such as fasting before a specific test. Make a list of:

  • Your symptoms, including any that seem unrelated to the reason for your appointment.
  • Key personal information, including major stresses, recent life changes and family medical history.
  • All medications, vitamins or supplements that you are taking, including the dosage.
  • Questions to ask your health care professional.

Have a friend or family member, if possible, to help you remember the information they give you.

To the autoimmune encephalitis, some basic questions to ask include:

  • What is likely causing my symptoms?
  • Other that the most likely cause, what are other possible causes of the symptoms?
  • What tests do I need?
  • Is my condition likely to be short-term or duration?
  • What is the best course of action?
  • What are the alternatives to the primary approach you're suggesting?
  • I have these other health conditions. How can I best manage them together?
  • There are restrictions that must be followed?
  • You should see a specialist?
  • Are there brochures or other printed material I can have? What sites do you recommend?

Do not hesitate to ask other questions.

What to expect from your doctor

Your healthcare provider is likely to ask several questions, such as:

  • When did your symptoms begin?
  • Do your symptoms occur all the time or intermittently?
  • How bad are the symptoms?
  • What, if anything, seems to improve your symptoms?
  • What, if anything, appears to worsen your symptoms?
Symptoms and treatment of Autoimmune encephalitis