Autoimmune Encephalitis and Autoantibodies: A Review of Clinical Implications

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.

Keywords: autoantibodies; autoimmunity; encephalitis; evaluation; paraneoplastic; seizures.