Autoimmune Encephalitis (AIE) in Children: A Q&A with Drs. Catherine Otten and Stephen Wong
Catherine Otten, MD, and Stephen Chee-Yung Wong, MD, lead the Inflammatory Brain Disorders Clinic at Seattle Children’s. With increasing evidence that children with severe problems related to thinking, learning and mental health have inflammatory brain disorders, Seattle Children’s created the clinic in 2021 to bring the latest advances in this fast-growing field to children in our region. The most common condition Drs. Otten and Wong see in patients is autoimmune encephalitis (AIE).
Q: Can you give us a quick reminder of what AIE is?
Autoimmune encephalitis is an inflammatory brain disease associated with antibodies that bind to cells in the brain. It is a type of noninfectious encephalitis. Patients can experience a wide range of neurologic and psychiatric symptoms, including seizures, abnormal movements, behavior changes, psychosis, autonomic dysfunction, cognitive symptoms, or altered level of consciousness. Different types of AIE can affect children and adults at different rates and with different symptoms. Often, patients may present with acutely to subacutely altered mental status, behavioral changes and seizures. Clinicians may be considering different types of encephalitis, including infectious and autoimmune encephalitis, in their differential diagnosis. Symptoms can progress, and a good portion will need critical care, and nearly all will be hospitalized acutely for treatment and management. Long-term, children typically continue to need treatment to manage relapses, refractory disease or sequelae from their AIE.
Q: How common is autoimmune encephalitis?
It is likely more common than we recognize! For instance, the California Encephalitis Project tracked the causes of encephalitis, and autoimmune causes of encephalitis were more common than any infectious agent. The most common type is anti-N-methyl-D-aspartate receptor encephalitis, or NMDARE, which affects women and children in higher proportion. AIE is not expected in very young infants under 6 months of age, but affects all ages otherwise. Read full post »