Autoimmune encephalitis: clinical spectrum and management

• Published in: Practical Neurology Vol. 21; no. 5; pp. 412 - 423
• Main Authors: Uy, Christopher E, Binks, Sophie, Irani, Sarosh R
• Format: Journal Article Book Review
• Language: English
• Published: England BMJ Publishing Group Ltd 01.10.2021; BMJ Publishing Group LTD; BMJ Publishing Group
• Subjects: Antibodies; Antigens; Autoantibodies; Autoimmune diseases; clinical neurology; Convulsions & seizures; Disease; Encephalitis; Encephalitis - diagnosis; Encephalitis - therapy; epilepsy; Hashimoto Disease - diagnosis; Hashimoto Disease - therapy; Humans; Immunoglobulins; immunology; Immunotherapy; limbic system; Mortality; Movement Disorders; neuroimmunology; Proteins; Review; Sleep; Tumors; epilepsy; clinical neurology; limbic system; neuroimmunology; immunology
• ISSN: 1474-7758; 1474-7766
• DOI: 10.1136/practneurol-2020-002567

Autoimmune encephalitis defines brain inflammation caused by a misdirected immune response against self-antigens expressed in the central nervous system. It comprises a heterogeneous group of disorders that are at least as common as infectious causes of encephalitis. The rapid and ongoing expansion of this field has been driven by the identification of several pathogenic autoantibodies that cause polysymptomatic neurological and neuropsychiatric diseases. These conditions often show highly distinctive cognitive, seizure and movement disorder phenotypes, making them clinically recognisable. Their early identification and treatment improve patient outcomes, and may aid rapid diagnosis of an underlying associated tumour. Here we summarise the well-known autoantibody-mediated encephalitis syndromes with neuronal cell-surface antigens. We focus on practical aspects of their diagnosis and treatment, offer our clinical experiences of managing such cases and highlight more basic neuroimmunological advances that will inform their future diagnosis and treatments.