Autoimmune Encephalitis: Clinical Features, Pathophysiology, and Management

Abstract Autoimmune encephalitis is a neuropsychiatric syndrome of significant clinical importance which has emerged beyond the realms of its status as a rare disorder, and can be distinct from a purely paraneoplastic condition. Whilst the number of antibodies causing AE has expanded in recent years, the best-characterized clinicopathological syndromes relate to anti-NMDA receptor antibodies and anti-VGKC complex (LGI1 and Caspr2) receptor antibodies. There is an increasing body of experience regarding the diagnosis and treatment of these conditions, which this review explores, as well as summarizing the known antibodies causing autoimmune encephalitis to date. This review also highlights emerging topics such as antibody negative autoimmune encephalitis, and HSV-induced autoimmune encephalitis. The early recognition of this condition is important for clinicians in a variety of specialities ranging from neurologists and psychiatrists to intensivists, clinical immunologists, infectious diseases specialists, pediatricians, and general physicians.