Autoimmune encephalitis: Recent updates and emerging challenges |
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| Institution: | 1. Neuroimmunology Group, Institute for Neuroscience and Muscle Research, The Kids Research Institute at the Children’s Hospital at Westmead, University of Sydney, Locked Bag 4001, Westmead, Sydney, NSW 2145, Australia;2. Department of Neurology, Westmead Hospital, Westmead, Sydney, NSW, Australia;3. TY Nelson Department of Neurology and Neurosurgery, Children’s Hospital at Westmead, Westmead, Sydney, NSW, Australia;1. Epworth Healthcare, Melbourne, VIC, Australia;2. Brain and Spine Tumour Clinic, Melbourne, VIC, Australia;3. Monash University, Clayton, Melbourne, VIC, Australia;4. Department of Neurosurgery, Royal Melbourne Hospital, Grattan Street, Parkville, Melbourne, VIC, Australia;5. Faculty of Health, Deakin University, Burwood, Melbourne, VIC, Australia;1. Neuropediatric Unit, Department of Women''s and Children''s Health, Karolinska Institutet, Sweden;2. Department of Clinical Neurophysiology, Karolinska University Hospital, Sweden;3. Department of Immunology, Genetics and Pathology, Uppsala University, Sweden;1. Neuroimmunology Group, Institute for Neuroscience and Muscle Research, The Kids Research Institute at the Children’s Hospital at Westmead;2. The Walker Unit, Concord Centre for Mental Health, Concord West, Discipline of Psychiatry, Sydney Medical School, University of Sydney, Sydney, Australia.;1. Children''s Hospital Augsburg, Section of Neuropediatrics, Klinikum Augsburg, Germany;2. Epilepsy Centre Bethel, Krankenhaus Mara, Bielefeld, Germany;3. Laboratory Krone, Bad Salzuflen, Germany;1. Section of Child Neurology, Department of Pediatrics, Nationwide Children''s Hospital, Columbus, Ohio;2. College of Medicine, The Ohio State University, Columbus, Ohio;3. Department of Radiology, Nationwide Children''s Hospital, Columbus, Ohio;4. Section of Rheumatology, Department of Pediatrics, Nationwide Children''s Hospital, Columbus, Ohio |
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| Abstract: | The knowledge of immune dysregulation and autoimmunity in neurological disorders has expanded considerably in recent times. Recognition of clinical syndromes, reliable methods of diagnosis, and early targeted immunotherapy can lead to a favourable outcome in acute and subacute neurological disorders that may be associated with significant morbidity and mortality if left untreated. This review focuses on the rapidly expanding field of autoimmune encephalitis. We describe the differences between limbic encephalitis associated with antibodies targeting intracellular antigens, and neuronal surface antibody syndromes (NSAS) where the antigens are primarily receptors or synaptic proteins located on the neuronal cell surface. We chronologically highlight important developments in NSAS by focusing on voltage gated potassium channel complex-associated antibody mediated encephalitis, anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis, and anti-dopamine 2 receptor antibody-associated basal ganglia encephalitis. Contentious issues such as the complexities of using serum antibodies as biomarkers, the initiation of central nervous system autoimmunity, and possible pathogenic mechanisms of these antibodies will be reviewed. The therapeutic challenges that clinicians face such as the timing of therapy and the role of second-line therapy will be discussed, with crucial concepts highlighted in the form of clinical vignettes. Future directions will involve the identification of novel antigens and methods to establish their pathogenicity, as well as evaluation of the most efficacious therapeutic strategies in patients with established NSAS. |
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| Keywords: | Anti-NMDAR encephalitis Autoimmune encephalitis Autoimmune epilepsy Basal ganglia encephalitis Limbic encephalitis VGKC encephalitis |
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