title: Autoimmune encephalopathies.
abstract: Antibody-mediated diseases of the central nervous system are a relatively new and challenging field in autoimmune neurologic disease and of major clinical importance in children and adults. The antibodies bind to cell-surface epitopes on neuronal or glial proteins, and the patients demonstrate either focal or more generalized clinical signs depending on the extent of brain regions targeted by the antibodies. The presence of seizures, movement disorders, autonomic dysfunction and sleep disorders, alongside neuroimaging and electrophysiological features may indicate a specific antibody-mediated disorder. However, phenotypic variation may be observed in children with the same antibody. Regardless, many patients benefit from immunotherapy with substantial improvement.
authors.affiliation: Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, University of Oxford, Level 6, West Wing, Oxford 3 9DU, UK; Children's Neurosciences, Evelina Children's Hospital @ Guy's and St Thomas' NHS Foundation Trust, King's Health Partners Academic Health Science Centre, Lambeth Palace Road, London SE1 7EH, UK. Electronic address: ming.lim@gstt.nhs.uk.
authors.forename: Ming
authors.initials: M
authors.lastname: Lim
authors.affiliation: Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, University of Oxford, Level 6, West Wing, Oxford 3 9DU, UK.
authors.forename: Yael
authors.initials: Y
authors.lastname: Hacohen
authors.affiliation: Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, University of Oxford, Level 6, West Wing, Oxford 3 9DU, UK. Electronic address: angela.vincent@ndcn.ox.ac.uk.
authors.forename: Angela
authors.initials: A
authors.lastname: Vincent
mesh_terms: D000293:Adolescent; D020274:Autoimmune Diseases of the Nervous System / Q000175:diagnosis* / Q000235:genetics / Q000276:immunology / Q000628:therapy*; D002648:Child; D003937:Diagnosis, Differential; D006801:Humans; D007167:Immunotherapy; D059906:Neuroimaging; D010641:Phenotype
keywords: AQP4; Antibody; Autoimmunity; Encephalitis; Immune-mediated; Immunotherapy; Limbic; MOG; N-methyl d-aspartate receptor; VGKC-complex