Anti-NMDA Receptor Encephalitis

What are the signs and symptoms of Anti-NMDA Receptor Encephalitis?

Symptoms can depend on factors such as a child’s age and which parts of the nervous system are affected. Your child may experience:

• Flu-like symptoms
• Seizures
• Changes in thinking (cognition), speech and memory, including confusion and short-term memory loss
• Behavior changes, including irritability, aggression and sleep disorders
• Movement disorders, including rhythmic movements of arms and legs and unusual motions of the mouth and face
• Psychiatric symptoms, including hallucinations and psychosis

At first, a child may experience milder symptoms, including a fever and subtle changes to mood or thinking. Later, they may develop more severe symptoms such as seizures and lethargy and need to be hospitalized.

How is Anti-NMDA Receptor Encephalitis diagnosed?

The diagnosis for this encephalitis is confirmed by a blood or spinal-fluid test, or both. These tests can detect the antibodies that attack the NMDA receptors. It can take several weeks for doctors to receive your child’s test results.

Starting treatment early makes a big difference in how a child recovers from this disease. While we wait for the test results, we will try to rule out other conditions, such as infections, metabolic diseases, psychiatric disorders and other inflammation in the brain. We will also use other tools to try and diagnose your child so we can start treating them as soon as possible. Our diagnostic approaches could include:

• Giving your child an MRI (magnetic resonance imaging) to look for changes in their brain
• Having a neurologist examine them
• Using an EEG (electroencephalogram) to monitor the electrical activity in your child’s brain

What are the causes of Anti-NMDA Receptor Encephalitis?

This type of encephalitis was identified fairly recently, and doctors still aren’t completely sure what causes it. There likely are a variety of genetic and environmental triggers. Doctors and researchers are working hard to understand what they might be. In some cases, an infection or a tumor can set off an immune response that mistakenly attacks the NMDA receptors.

How is Anti-NMDA Receptor Encephalitis treated?

We see children with this type of encephalitis every year, giving us the experience to recognize and treat this condition. We will use different therapies to suppress the immune system and limit the damage that encephalitis can cause.

In the beginning, therapies usually include:

• High-dose steroids, which reduce inflammation
• Plasma exchange/plasmapheresis (PLEX), which is the most effective treatment in a hospital setting. Like dialysis, this process can clean your child’s blood of the antibodies it carries. Children’s Health℠ has the specialized equipment to treat your child with PLEX, and we often administer it more quickly than other hospitals. This may give your child the opportunity to get better faster.
• Intravenous immunoglobulin (IVIG), which consists of antibodies from the purified blood plasma of healthy donors. IVIG can reduce inflammation and affect the creation and destruction of the disease-causing antibodies.
• Surgery to remove the tumor, in children whose encephalitis was triggered by a tumor.

For children who do not respond to the initial treatment or need longer-term treatment, therapies include:

• Rituximab, which targets the antibody-producing cells and is often used in other autoimmune diseases.
• Cyclophosphamide, which works by slowing or stopping the immune cell response and is often used in cancer therapy.