Autoimmune Encephalitis
Nervous System Confirmed Autoimmune

Autoimmune Encephalitis

Also known as: AE, Autoimmune brain inflammation, Anti-NMDA receptor encephalitis (specific subtype), Autoimmune limbic encephalitis

Primary organ/tissue: Brain (especially the limbic system, cortex, and subcortical regions)

Overview

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. AE can occur on its own or be triggered by infections, cancer (paraneoplastic), or unknown causes.

Symptoms

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).

Causes

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, or idiopathic.

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.

Autoantibodies

AutoantibodyNotes
Anti-NMDA receptor
Anti-LGI1
Anti-CASPR2
Anti-GAD65
Anti-AMPAR
Anti-GABA

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.