Glossary

An antibody also known as an immunoglobulin, is a large Y-shaped protein produced by B-cells and plasma cells that is used by the immune system to identify bacteria, viruses, and damaged cells in our body and direct our immune system to remove them.

Autoimmune diseases arise from an inappropriate immune response of the body against and the body’s own healthy tissues (autoimmunity).

CellCept is an oral immunosuppressant, originally prescribed to patients undergoing organ transplant surgery, but now is used in many autoimmune conditions and recommended as a possible “second line” defense in AE. CellCept interferes with the formation of DNA in certain immune system cells that become overactive in cases of autoimmune disorders.

Most common side effects include nausea, vomiting, diarrhea, headache, dizziness or rash. Less common but more serious side effects include anemia, and blood clots. Click here for a complete list of side effects.

Cytoxan is a chemotherapy drug that comes in tablet or injectable form. It works by slowing or halting the growth of immune system cells. Common side effects include nausea, vomiting, and hair loss. Less common but more serious side effects include damage to the bladder, fertility problems, and bone marrow suppression.  Click here for a complete list of side effects.

EEG is an Electroencephalograph used to record the brain’s spontaneous electrical activity and can detect if a person is having seizures or abnormal brain activity. A routine EEG is usually over a short period of time usually 20–40 minutes and recorded from multiple electrodes placed on the scalp. For AE it is recommended patients have a prolonged EEG, ideally overnight.  A clinical use of EEG is in the diagnosis of encephalopathy.

Encephalitis is acute inflammation of the brain. This can be from infection, metabolic disease, or autoimmune disease.

Intravenous immunoglobulin (IVIG) is a blood product administered intravenously consisting of blood plasma donations from human donors. IVIG contains an antibody called IgG that helps to ward off infection, modulates immune system, and reduces inflammation, though the full extent of how IVIG works remains unknown. It is known that IVIG can work in many different ways, including increasing the removal of antibodies, inhibit binding of the harmful antibodies, and decrease the inflammatory response to antibodies.

Side effects include headaches, aseptic meningitis, dermatitis (peeling of skin on palms and soles), pulmonary edema, and allergic reactions. Click here for side effects.

Immunologist specializes in the diagnosis and treatment of problems with the immune system, including immunodeficiency, when the immune system is compromised or impaired, and autoimmunity, when the immune system attacks its own body.

MRI is a Magnetic Resonance Imaging scan. It can show the brain inflammation characteristic of encephalitis. Please note that not all autoimmune encephalitis patients have abnormal MRI scans.

Neurologist specializes in the diagnosis and treatment of diseases involving the nervous system, which includes the brain, spinal cord, and body’s network of nerves.

Plasmapheresis is a blood-cleansing intravenous procedure where blood is removed from a patient, treated, and then returned to the patient. The procedure works by removing the patient’s blood plasma, which contains the disease-causing autoantibodies.

Plasma Exchange and plasmapheresis are often used interchangeably, but they are different procedures. Plasma exchange (PLEX) is a procedure where plasma is separated from the blood, discarded in total, and replaced with a substitution fluid such as albumin or with donated plasma.

Potential risks  to both of the above,  including infection and bleeding, may emerge from the catheter insertion. Procedure side effects include blood clots and hypotension.

Prednisone, the most common form of corticosteroids prescribed to patients with autoimmune encephalitis, acts as an immunosuppressant, which suppresses the immune system from making harmful autoantibodies and decreases the inflammatory response to antibodies. Steroids also work as anti-inflammatory agents by reducing swelling in the brain.  Side effects include high blood glucose levels (important to those suffering from diabetes), weight gain, facial swelling, blurred vision, increased levels of infections, and osteoporosis. Psychiatric side effects also can occur, including insomnia, mood fluctuations, mania, depression, and anxiety. Click for a complete list of side-effects and more information.

Psychiatrist specializes in the diagnosis and treatment of mental disorders.

Receptor is a protein on the surface of a brain cell that receives chemical messages and is a key building block in the brain’s chemistry. In autoimmune encephalitis, the body’s immune system produces an antibody that binds to a receptor in the brain and causes cells to respond inappropriately. Treatment of AE involves eliminating or reducing these antibodies.

Rheumatology is a sub-specialty in internal medicine and pediatrics, devoted to diagnosis and therapy of rheumatic diseases.

Rituximab is cell-targeted antibody infusion that labels B cells to be removed and destroyed by the immune system, preventing them from becoming antibody producing cells and stimulating other immune cells.

Common side effects during infusion include serious infections fever, shaking, fatigue, or nausea. Other adverse effects include cardiac arrest, cytokine release syndrome, and types of immune toxicity. For a complete list of side effects and more information.

Steroids are medications used to treat inflammation. One of the most common prescribed oral steroid drugs is called Prednisone and IV steroid is methylprednisolone. .

Teratoma is a tumor comprised of tissue and/or organ components. Teratomas are normally benign, but it is believed that the teratomas (which sometimes contain brain tissue) act as the “trigger” in an AE patient’s autoimmune response. Surgical removal of the teratomas in autoimmune encephalitis patients with one is the first step in the recovery process.

 

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