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Autoimmune Encephalitis

Autoimmune Encephalitis (Encephalopathy) is a rare treatable group of CNS disorders 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 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 or ion channels.  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 cells 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.

PREVALENCE:

A unique feature of the autoimmune encephalitis 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 been reported to be higher among African Americans than Caucasians. Autoimmune Encephalitis prevalence~1.2 per 100,000 VS. Infectious Encephalitis ~1.0 per 100,000 ~14 per 100,000  had autoimmune encephalitis in their lifetime ~12 per 100,000 who had infectious encephalitis. This study allows us to estimate that approximately 1 million people worldwide had autoimmune encephalitis in their lifetime. It has also been estimated that, currently, about 90,000 people around the world develop autoimmune encephalitis each year.

GENETIC PREDISPOSITION: 

Most autoimmune encephalitides occur in patients with no apparent immunologic triggers, leading some investigators to postulate a genetic predisposition to these disorders.  One study suggested a genetic predisposition to anti-NMDAr  encephalitis in Maori and Pacific Island populations.  Four studies showed an association of anti-LGI1 encephalitis with HLA class II genes, including HLA-DRB1*07 (DR7) and HLA-DRB4 in a 2017 Dutch population study  and DRB1*07:01–DQB1*02:02 in a 2017 Korean population study.  A 2018 German study also showed a link with anti-LGI1 and HLA class II genes encompassing DRB1*07:01, DQA1*02:01 and DQB1*02:02 as well as a genetic component in anti-NMDAr encephalitis with HLA-I allele B*07:02.  Most recently in May 2018 study by the Oxford group showed a non-heritable but consistent genetic predisposition to LGI1 and CASPR2. Research just published out of China shows genetic link to anti-NMDAR encephalitis. This study for the first time, demonstrates an association between specific HLA class II alleles, DRB1*16:02 in anti-NMDAR encephalitis, providing novel insights into the mechanism by which the disease occurs. This adds to LGI1 and CASPR2 HLA-DRB associations.

Dr. Maarten Titulaer Webinar 1 hour 

Autoimmune Encephalitis – anti-NMDAr encephalitis and Beyond 2017 

Dr. Josep Dalmau

2016 AAN Autoimmune Encephalitis 

Gregory Day, MD, MSc, FRCPC 

Antibody Mediated Encephalitis in the Modern Era:

From Witchcraft to Neurologic Wizardry

Eric Lancaster, MD, PhD

Autoimmune Synaptic Encephalitis

Dr. Sarosh Irani
What is being done to improve Autoimmune encephalitis for the future?

Andrew McKeon, M.B., B.CH.,M.D

Co-Director of the Neuroimmunology

Our website is not a substitute for independent professional medical advice. Nothing contained on our website is intended to be used as medical advice. No content is intended to be used to diagnose, treat, cure or prevent any disease, nor should it be used for therapeutic purposes or as a substitute for your own health professional's advice. Although THE INTERNATIONAL AUTOIMMUNE ENCEPHALITIS SOCIETY  provides a great deal of information about AUTOIMMUNE ENCEPHALITIS, all content is provided for informational purposes only. The International Autoimmune Encephalitis Society  cannot provide medical advice.


International Autoimmune Encephalitis Society is a charitable non-profit 501(c)(3) organization. Tax ID# 81-3752344 Donations raised directly supports patients, families and caregivers impacted by autoimmune encephalitis and to educating healthcare communities around the world. Financial statement will be made available upon request.

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352-527-2470

IAES@AUTOIMMUNE-ENCEPHALITIS.ORG

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