Autoantibodies, Encephalopathies, and Epilepsy

In this chapter, we describe the clinical manifestations and known mechanisms
of encephalopathies with seizures as a prominent symptom which respond to
immunomodulatory therapies. In the last several years, new autoantigens involved in
aberrant immune response have been discovered, widening the spectrum of epilepsies
with an autoimmune etiology. Given this trend, it is possible that the number of
epilepsies associated with an autoimmune response will increase over time. Recent
clinical guidelines for the diagnosis of autoimmune encephalitis have been published.
These guidelines encourage clinicians to start early immunotherapy while
awaiting results for autoantibody testing in “possible” and “probable” autoimmune
encephalitis and provide criteria for the diagnosis of “autoantibody-negative
but probable autoimmune encephalitis,” which may benefit from immunomodulation.