Overview

Autoimmune encephalitis is a rare but serious condition where the immune system attacks the brain, causing inflammation. It can lead to rapid changes in behavior, memory loss, seizures, and even psychosis. In Korea, awareness and early diagnosis of autoimmune encephalitis are increasing, thanks to improved neuroimmunology services and access to advanced imaging and antibody testing.

What is Autoimmune Encephalitis?

Autoimmune encephalitis (AE) is a group of inflammatory brain diseases caused by the body’s immune response mistakenly targeting brain cells. This attack can be triggered by antibodies targeting receptors, proteins, or cells in the brain, leading to swelling and dysfunction. It is distinct from viral encephalitis and may be associated with underlying cancers (paraneoplastic) or occur without them (non-paraneoplastic).

Common subtypes include:

• Anti-NMDA receptor encephalitis
• Anti-LGI1 encephalitis
• Anti-GABA receptor encephalitis
• Paraneoplastic limbic encephalitis

Symptoms

Symptoms of autoimmune encephalitis may develop over days or weeks and can include:

• Confusion or disorientation
• Memory loss
• Seizures
• Personality changes or psychosis
• Abnormal movements (tremors, rigidity)
• Speech difficulties
• Hallucinations or delusions
• Sleep disturbances
• Loss of consciousness in severe cases

Causes

Autoimmune encephalitis is caused by the immune system producing antibodies that attack brain structures. This may happen:

• Spontaneously (idiopathic)
• After infection
• In association with cancers (e.g., ovarian teratoma, lung cancer)
• Due to genetic or environmental triggers

Risk Factors

• Female gender (especially in anti-NMDA receptor type)
• History of autoimmune disease
• Presence of tumors (especially ovarian teratomas in women)
• Viral infections triggering immune response
• Genetic susceptibility

Complications

If not diagnosed and treated early, autoimmune encephalitis can lead to:

• Permanent cognitive impairment
• Chronic seizures
• Behavioral or psychiatric disorders
• Coma or death in severe cases
• Need for long-term neurological rehabilitation

Prevention

There is no guaranteed prevention, but risk can be reduced by:

• Prompt treatment of associated cancers
• Early recognition and treatment of initial symptoms
• Regular health screenings for those with autoimmune history
• Avoiding immune triggers (e.g., certain infections, environmental toxins)

Treatment Options in Korea

South Korea has advanced capabilities for diagnosing and managing autoimmune encephalitis, with expert care available at institutions such as Severance Hospital, Seoul National University Hospital, and Samsung Medical Center.

1. Diagnosis

• MRI of the brain to detect inflammation
• EEG to monitor brain activity and seizures
• Lumbar puncture (CSF analysis)
• Blood and cerebrospinal fluid (CSF) antibody testing
• PET scans or CT to detect underlying tumors
• Neuropsychological testing

2. Medical Treatments

• High-dose corticosteroids to reduce inflammation
• IVIG (Intravenous Immunoglobulin)
• Plasmapheresis to remove harmful antibodies
• Immunosuppressants such as rituximab or cyclophosphamide
• Antiseizure medications for seizure control
• Antipsychotics to manage behavioral symptoms

3. Rehabilitation & Long-Term Support

• Neurological rehabilitation for motor and cognitive recovery
• Psychiatric support for emotional and behavioral issues
• Speech and occupational therapy
• Regular follow-up with neurology and immunology specialists

4. Integrated Approaches in Korea

• Traditional Korean Medicine (Hanbang) support:
  • Herbal tonics for immune modulation
  • Acupuncture to manage neurologic symptoms
• Diet and lifestyle management programs at tertiary care centers