Autoimmune Encephalitis and Autoantibodies: A Review of Clinical Implications

Abstract

Abstract Background Autoimmune encephalitis (AE) is a common cause of encephalitis. We review the most recent evidence on this neuroimmune condition and autoantibody testing currently available. Content Clinical criteria, neuroimaging and electroencephalography can facilitate the diagnosis of AE prior to obtaining autoantibody testing results, and lead to a diagnosis of AE even in the absence of a recognized antibody. Early treatment of AE has been found to correlate with improved long-term functional and cognitive outcomes. We suggest a clinical approach to diagnosis based on the predominant area of nervous system involvement and the results of ancillary testing that are widely available. We also propose a 2-tiered approach to the acute management of probable or definite AE. We, finally, provide guidance on the long-term management of AE—a challenging and understudied area. Summary Much work remains to be done to improve the care of patients with AE. As understanding of the pathophysiology and predisposing factors underlying this condition steadily increases, a more evidence-based, targeted approach to the treatment of AE is still desired. Nonetheless, looking at the progress made over the past 2 decades, since the discovery of the first autoantibodies associated with AE, one cannot help but feel optimistic about the road ahead.