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Autoimmune Encephalitis

Also Known As: AE, Autoimmune brain inflammation, Anti-NMDA receptor encephalitis (specific subtype), Autoimmune limbic encephalitis
System: Immune System and Nervous System
Autoantibodies:
  • Anti-NMDA receptor

  • Anti-LGI1

  • Anti-CASPR2

  • Anti-GAD65

  • Anti-AMPAR, Anti-GABA, others

Primary Organ or Body Part Affected: Brain (especially the limbic system, cortex, and subcortical regions)
Acceptance as Autoimmune: Confirmed
Autoimmune Encephalitis

What is Autoimmune Encephalitis?

Autoimmune Encephalitis is a serious but treatable condition in which the body’s immune system mistakenly attacks the brain, causing inflammation. This can lead to confusion, memory loss, seizures, personality changes, and even psychosis. It can come on suddenly and often mimics psychiatric or viral illnesses at first.

AE can occur on its own or be triggered by infections, cancer (paraneoplastic), or unknown causes. The condition varies based on the autoantibody involved, with anti-NMDA receptor encephalitis being one of the most well-known forms.

What are the signs and symptoms of Autoimmune Encephalitis?

  • Sudden memory loss or confusion

  • Personality or behavior changes

  • Hallucinations or delusions

  • Anxiety or paranoia

  • Seizures

  • Movement disorders (twitches, jerks, abnormal posture)

  • Trouble speaking or understanding language

  • Sleep disturbances

  • Decreased consciousness or coma (in severe cases)

What are the causes of Autoimmune Encephalitis?

Autoimmune encephalitis is caused by antibodies that attack specific brain proteins, disrupting brain function. Common causes include:

  • Antibodies (e.g., anti-NMDA receptor, anti-GAD65, anti-AMPAR, anti-LGI1)

  • Infections that trigger autoimmunity (post-viral)

  • Cancer (especially ovarian teratomas, lung cancer, or breast cancer) triggering an immune response

  • Idiopathic (no known cause in some cases)

Diagnosis

  • MRI brain scan

  • Lumbar puncture (spinal tap) to look for inflammation

  • EEG to check for abnormal brain activity

  • Blood and CSF antibody testing

  • Cancer screening (CT, PET scan, ultrasound depending on age and gender)

Treatment

  • High-dose corticosteroids (to reduce inflammation)

  • IVIG (intravenous immune globulin) or plasmapheresis (removes harmful antibodies)

  • Immunosuppressive drugs (e.g., rituximab, cyclophosphamide)

  • Treating underlying cancer (if present)

  • Rehabilitation for speech, memory, and motor function recovery

Prognosis

  • Many patients recover fully or significantly, especially with early diagnosis and treatment

  • Recovery may take months to years, and some may have lingering cognitive or emotional effects

  • Relapses can occur and need long-term monitoring

Prevalence

  • Estimated at 5–10 cases per million people per year

  • Increasing recognition in both adults and children

  • Anti-NMDA receptor encephalitis is more common in young women, often associated with ovarian teratomas

Citations

  1. Dalmau J, et al. (2011). Clinical experience and laboratory investigations in patients with anti-NMDAR encephalitis. Lancet Neurol.

  2. Graus F, et al. (2016). A clinical approach to diagnosis of autoimmune encephalitis. Lancet Neurol.

  3. Mayo Clinic. Autoimmune Encephalitis Overview.
    https://www.mayoclinic.org

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