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.