Autoimmune encephalitis: recent clinical and biological advances

• Published in: Journal of neurology Vol. 270; no. 8; pp. 4118 - 4131
• Main Authors: Varley, James A., Strippel, Christine, Handel, Adam, Irani, Sarosh R.
• Format: Journal Article
• Language: English
• Published: Berlin/Heidelberg Springer Berlin Heidelberg 01-08-2023; Springer Nature B.V
• Subjects: Acids; Antibodies; Ataxia; Autoantibodies; Autoimmune diseases; Autoimmune Diseases of the Nervous System - diagnosis; Autoimmune Diseases of the Nervous System - therapy; Cognitive ability; Convulsions & seizures; Disease; Encephalitis; Encephalitis - diagnosis; Encephalitis - therapy; Hashimoto Disease - diagnosis; Hashimoto Disease - therapy; Humans; Immunology; Immunotherapy; Kinases; Medicine; Medicine & Public Health; Nervous system; Neurological diseases; Neurological Update; Neurology; Neuroradiology; Neurosciences; Patients; Phenotypes; Proteins; LGI1; Encephalitis; NMDAR; Aautoimmune; Limbic; Immunotherapy
• ISSN: 0340-5354; 1432-1459
• DOI: 10.1007/s00415-023-11685-3

In 2015, we wrote a review in The Journal of Neurology summarizing the field of autoantibody-associated neurological diseases. Now, in 2023, we present an update of the subject which reflects the rapid expansion and refinement of associated clinical phenotypes, further autoantibody discoveries, and a more detailed understanding of immunological and neurobiological pathophysiological pathways which mediate these diseases. Increasing awareness around distinctive aspects of their clinical phenotypes has been a key driver in providing clinicians with a better understanding as to how these diseases are best recognized. In clinical practice, this recognition supports the administration of often effective immunotherapies, making these diseases ‘not to miss’ conditions. In parallel, there is a need to accurately assess patient responses to these drugs, another area of growing interest. Feeding into clinical care are the basic biological underpinnings of the diseases, which offer clear pathways to improved therapies toward enhanced patient outcomes. In this update, we aim to integrate the clinical diagnostic pathway with advances in patient management and biology to provide a cohesive view on how to care for these patients in 2023, and the future.