Autoimmune Encephalitis

Autoimmune disease that can affect any part of the nervous system. Autoimmune diseases, including autoimmune encephalitis in children, can occur following an infection. They also can be related to a child having a systemic autoimmune disease or a dysfunctional immune system, or no clear health issues.

Norton Children’s Neuroscience Institute’s Michael L. Sweeney, M.D., completed his fellowship training in autoimmune neurology at the University of Utah and is a part of the Autoimmune Encephalitis Alliance network of physicians dedicated to the treatment of this disease.

Autoimmune Encephalitis

Encephalitis is a disease characterized by inflammation within the brain. Depending on the parts of the brain affected, symptoms can vary widely. These symptoms may include:

  • Seizures
  • Behavior or personality changes
  • Abnormal movements
  • Difficulty maintaining balance
  • Trouble thinking or speaking
  • Weakness
  • Numbness
  • Difficulty sleeping

Encephalitis has several specific names relating to the circumstances under which it arises. When encephalitis occurs without a clear reason, it is called autoimmune encephalitis. Post-infectious encephalitis occurs following an infection. If encephalitis occurs in the setting of a tumor or cancer, it is called paraneoplastic encephalitis.

Anti-NMDA-receptor encephalitis is the most common form of autoimmune encephalitis in children. It is caused by the presence of antibodies in a specific part of the nerve cells in the brain, called NMDA-receptors. In children, this form of encephalitis is rarely associated with a tumor called an ovarian teratoma.

Autoimmune Encephalitis in Children Diagnosis

Diagnosing autoimmune encephalitis can be challenging. Our team’s first step is to do a thorough medical workup of your child. We will document his or her complete medical history and perform tests, including a neurologic examination, imaging of the brain (MRI), electroencephalography (EEG), and spinal fluid analysis.

Our team typically will do blood testing to evaluate your child for signs of inflammation and autoimmune disorders. We also may test for specific antibodies in the blood and spinal fluid, which can be helpful in making a diagnosis. The presence of a specific antibody is not necessary to make a diagnosis. We also may screen for an underlying tumor, which typically involves whole-body imaging.

Treatment of Autoimmune Encephalitis in Children

Treatment in the hospital is aimed at reducing the amount of inflammation within your child’s brain. Our team may use high doses of steroids, intravenous immunoglobulin and plasmapheresis. If needed, your child also may receive additional immunotherapies, such as rituximab or cyclophosphamide.

Our team may use medications to treat symptoms caused by encephalitis. If your child has seizures, we may prescribe antiepileptic medications. For mood changes, we may prescribe antipsychotic and antidepressant medications.

Long-term follow-up with our team is important to address possible consequences of encephalitis such as seizures, mood and personality changes, and learning problems.

Neuroscience

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