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Autoimmune Encephalitis | Encephalitis International

Autoimmune encephalitis

Some types of autoimmune encephalitis which is where the immune system is attacking the brain, such as acute disseminated encephalomyelitis (ADEM), are caused by infection in which case the term ‘post-infectious encephalitis’ is used.

Other forms of autoimmune encephalitis are associated with finding specific antibodies in blood such as VGKC complex (anti-LGI1 and Caspr2), NMDA receptor, GAD, AMPAR and GABA antibodies.

Antibodies, also called immunoglobulins, are large Y-shaped proteins which identify and help remove foreign antigens such as viruses and bacteria. The reason why these antibodies are produced by the immune system in people with autoimmune encephalitis is not known in most cases. Sometimes a tumour (benign or cancerous) may generate the antibody.

Signs and symptoms of autoimmune encephalitis

The signs and symptoms of autoimmune encephalitis can vary widely depending on the individual and the underlying cause of the condition. Common symptoms include:

  • Psychiatric symptoms: These can range from psychosis and depression to anxiety, significantly impacting the patient’s mental health.
  • Seizures: These can be focal or generalised, adding to the complexity of the condition.
  • Abnormal movements: Patients may experience tremors, rigidity, and chorea, which can interfere with daily activities.
  • Cognitive impairment: Memory loss and difficulty with concentration are common, affecting the patient’s ability to function normally.
  • Autonomic dysfunction: Changes in heart rate and blood pressure can occur, complicating the clinical picture.
  • Sleep disturbances: Insomnia and hypersomnia are frequent, further affecting the patient’s quality of life.

In some cases, autoimmune encephalitis can present with a distinct clinical syndrome, such as limbic encephalitis. This condition is characterised by inflammation of the limbic system, leading to significant memory impairment and psychiatric symptoms.

Diagnosis of autoimmune encephalitis

Diagnosing autoimmune encephalitis can be challenging, as the symptoms can be non-specific and may resemble those of other conditions, such as infectious encephalitis or psychiatric disorders.

A definitive diagnosis typically requires a combination of clinical evaluation, imaging studies, and laboratory tests, including:

  • Cerebrospinal fluid (CSF) analysis: This test helps detect the presence of autoantibodies, which are crucial for diagnosing autoimmune encephalitis.
  • Magnetic resonance imaging (MRI) or computed tomography (CT) scans: These imaging studies are used to rule out other causes of brain inflammation and to identify characteristic changes associated with autoimmune encephalitis.
  • Electroencephalogram (EEG): This test evaluates for seizures or abnormal brain activity, which are common in autoimmune encephalitis.
  • Blood tests: These tests help evaluate for underlying infections or autoimmune disorders that could be contributing to the condition.

Treatment of autoimmune encephalitis

Treatment for autoimmune encephalitis typically involves a combination of immunosuppressive medications and supportive care. The goal of treatment is to reduce inflammation and prevent further damage to the brain. Common treatment approaches include:

  • Corticosteroids: Medications like prednisone are used to reduce inflammation.
  • Intravenous immunoglobulin (IVIG): This therapy helps modulate the immune response.
  • Plasmapheresis: This procedure removes autoantibodies from the blood, reducing their harmful effects.
  • Rituximab: This monoclonal antibody targets CD20-positive B cells, reducing autoantibody production.
  • Anticonvulsants: These medications are used to control seizures.
  • Psychiatric medications: These are prescribed to manage psychiatric symptoms, improving the patient’s quality of life.

Long-term Outlook and Care

The long-term outlook for autoimmune encephalitis varies depending on the underlying cause and the effectiveness of treatment. In general, patients who receive prompt and aggressive treatment tend to have better outcomes. However, some patients may experience persistent cognitive impairment, psychiatric symptoms, or abnormal movements, which can impact their quality of life.

Long-term care for autoimmune encephalitis typically involves a multidisciplinary approach, including:

  • Regular follow-up: Ongoing monitoring with a neurologist or immunologist is essential to detect relapses or complications early.
  • Cognitive rehabilitation: This therapy helps address persistent cognitive impairment, aiding in the patient’s recovery.
  • Psychiatric care: Managing ongoing psychiatric symptoms is crucial for improving the patient’s mental health.
  • Physical therapy: This helps address abnormal movements or motor impairment, enhancing the patient’s functional abilities.
  • Supportive care: Addressing sleep disturbances, autonomic dysfunction, and other symptoms is vital for improving the patient’s overall well-being.

Early recognition and treatment of autoimmune encephalitis are critical to improving outcomes and reducing morbidity and mortality.

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Page Created: 15 November 2023
Last Modified: 18 October 2024
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