Autoimmune encephalitis : epidemiology, pathophysiology and clinical spectrum (Part 2)

Abstract

Autoimmune encephalitis (AE) represents a growing number of severe autoimmune-inflammatory diseases affecting both the white and grey matter of the brain. In part 1 of this series, we focused on the epidemiology, pathophysiology and clinical presentation of this condition, with two illustrative cases. In this part, we introduce the clinical criteria for AE, particularly for the diagnosis of anti-N-methyl-D-aspartate (NMDA) receptor encephalitis, which were developed to facilitate immune treatment in suspected cases before antibody results are available. We subsequently discuss the work-up, differential diagnosis and treatment options for patients with this disease.