title: VGKC-complex/LGI1-antibody encephalitis: clinical manifestations and response to immunotherapy.
abstract: Leucine-rich glioma inactivated 1 (LGI1) was recently identified as a target protein in autoimmune synaptic encephalitis, a rare condition associated with autoantibodies against structures in the neuronal synapse. Studies dealing with LGI1 are small in number and the various outcomes of different therapeutic regimens are not well studied. Here, we analyzed clinical characteristics of 14 patients with LGI1 antibodies, and outcomes according to therapeutic strategies. Most patients exhibited abnormal brain positron emission tomography and that patients treated with steroids alone were more likely to relapse and had less favorable outcomes than those treated with steroids and intravenous immunoglobulins.
authors.affiliation: Department of Neurology, Laboratory for Neurotherapeutics, Comprehensive Epilepsy Center, Biomedical Research Institute, Seoul National University Hospital, Seoul, South Korea; Program in Neuroscience, Seoul National University College of Medicine, Seoul, South Korea.
authors.forename: Yong-Won
authors.initials: YW
authors.lastname: Shin
authors.forename: Soon-Tae
authors.initials: ST
authors.lastname: Lee
authors.forename: Jung-Won
authors.initials: JW
authors.lastname: Shin
authors.forename: Jangsup
authors.initials: J
authors.lastname: Moon
authors.forename: Jung-Ah
authors.initials: JA
authors.lastname: Lim
authors.forename: Jung-Ick
authors.initials: JI
authors.lastname: Byun
authors.forename: Tae-Joon
authors.initials: TJ
authors.lastname: Kim
authors.forename: Keon-Joo
authors.initials: KJ
authors.lastname: Lee
authors.forename: Young-Su
authors.initials: YS
authors.lastname: Kim
authors.forename: Kyung-Il
authors.initials: KI
authors.lastname: Park
authors.forename: Keun-Hwa
authors.initials: KH
authors.lastname: Jung
authors.forename: Sang Kun
authors.initials: SK
authors.lastname: Lee
authors.forename: Kon
authors.initials: K
authors.lastname: Chu
mesh_terms: D000328:Adult; D000368:Aged; D000906:Antibodies / Q000097:blood*; D001323:Autoantibodies / Q000097:blood; D001921:Brain / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology; D001927:Brain Diseases / Q000097:blood* / Q000000981:diagnostic imaging / Q000276:immunology / Q000628:therapy*; D004569:Electroencephalography; D004660:Encephalitis; D005260:Female; D019788:Fluorodeoxyglucose F18; D050031:Hashimoto Disease / Q000097:blood* / Q000000981:diagnostic imaging / Q000276:immunology / Q000628:therapy*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007167:Immunotherapy / Q000379:methods*; D047908:Intracellular Signaling Peptides and Proteins; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D049268:Positron-Emission Tomography; D011506:Proteins / Q000276:immunology*; D016896:Treatment Outcome
chemical_list: D000906:Antibodies; D001323:Autoantibodies; D016756:Immunoglobulins, Intravenous; D047908:Intracellular Signaling Peptides and Proteins; C116499:LGI1 protein, human; D011506:Proteins; D019788:Fluorodeoxyglucose F18
keywords: Encephalitis; Immunotherapy; LGI1