Autoimmune Encephalitis (Encephalopathy) is a rare disease that can be progressive or relapse-remitting. It is caused when the immune system makes auto-antibodies that are not supposed to be there.  These auto-antibodies (aka antibodies) begin to attack healthy brain cells wrongly identifying those healthy brain cells as foreign.   An autoimmune response is now occurring as the immune system attacks and destroys the brain’s healthy cells.  Your own body’s immune system is attacking your brain, causing inflammation of the brain. 

​

 

The antibody attacks by targeting special receptors in the brain. The antibodies can target the attack to receptors on the cell surface of healthy nerve cells in the brain or target the attack to synaptic receptors that are exposed to the attacking antibody. These antibodies bind to the healthy brain cell on the outside of the cell.  These antibodies are called extracellular because they bind or attach themselves to the healthy brain cell’s outside surface.  The healthy brain cells are now destroyed or no longer function properly.   Severe brain inflammation occurs. The brain now malfunctions.   All brain functions can be compromised: emotions, psychosis, memory, cognition, problem solving, speech, movement, seizures, balance, visual processing planning, sensory, hunger, thirst, behavior and personality traits, often followed by suppressed levels of consciousness and coma may occur.

​

These diseases are not always cancer-related which makes them different from the classical paraneoplastic neurological diseases, that are associated with, but not caused by, the intracellular antibodies. (See: Paraneoplastic Autoimmune Encephalitis)  

​

Most importantly Autoimmune Encephalitis IS TREATABLE and almost invariably responds to immunotherapies with considerable potential to reverse the impairments caused by these attacking antibodies.  Immunotherapies can slow down the progression of the disease, stop the antibodies from attacking and with some treatments kill the attacking antibody which may sometimes leads to complete recovery.

​

 A unique feature of the autoimmune encephalitides is that they can affect patients of all ages including infants and the elderly, some types more often occurring in children and young adults.  Autoimmune Encephalitis occurs more frequently in women than in men and they can develop with or without an underlying tumor.  

 

The incidence of autoimmune encephalitis has increased over time and is attributed to increased recognition of auto antibody-positive cases. The incidence rate is  1.2/100,000 person-years (2006-2015) and prevalence higher among African-Americans than Caucasians.

​

The first antibody was identified in 2005.  Since that time, many antibodies have been identified and still some are yet to be discovered.