title: Intrathecal-specific glutamic acid decarboxylase antibodies at low titers in autoimmune neurological disorders.
abstract: Autoantibodies to glutamic acid decarboxylase (Gad-Abs) are implicated in various neurological syndromes. The present study aims to identify intrathecal-specific GAD-Abs and to determine clinical manifestations and treatment outcomes. Nineteen patients had GAD-Abs in cerebrospinal fluid but not in paired serum samples. Neurological syndromes included limbic encephalitis, temporal lobe epilepsy, cerebellar ataxia, autonomic dysfunction, and stiff-person syndrome. Immunotherapy had beneficial effects in 57.1% of patients, and the patients with limbic encephalitis responded especially well to immunotherapy. Intrathecal-specific antibodies to GAD at low titers may appear as nonspecific markers of immune activation within the central nervous system rather than pathogenic antibodies causing neuronal dysfunction.
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: Jun-Sang
authors.initials: JS
authors.lastname: Sunwoo
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. Electronic address: stemcell.snu@gmail.com.
authors.forename: Kon
authors.initials: K
authors.lastname: Chu
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: Jung-Ick
authors.initials: JI
authors.lastname: Byun
authors.affiliation: Department of Neurology, Ewha Womans University School of Medicine and Ewha Medical Research Institute, Seoul, South Korea.
authors.forename: Jangsup
authors.initials: J
authors.lastname: Moon
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: Jung-Ah
authors.initials: JA
authors.lastname: Lim
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: Tae-Joon
authors.initials: TJ
authors.lastname: Kim
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: Soon-Tae
authors.initials: ST
authors.lastname: Lee
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: Keun-Hwa
authors.initials: KH
authors.lastname: Jung
authors.affiliation: Department of Neurology, Seoul National University Hospital Healthcare System Gangnam Center, Seoul, South Korea.
authors.forename: Kyung-Il
authors.initials: KI
authors.lastname: Park
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: Daejong
authors.initials: D
authors.lastname: Jeon
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: Ki-Young
authors.initials: KY
authors.lastname: Jung
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; Protein Metabolism Medical Research Center, Seoul National University College of Medicine, Seoul, South Korea.
authors.forename: Manho
authors.initials: M
authors.lastname: Kim
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. Electronic address: sangkun2923@gmail.com.
authors.forename: Sang Kun
authors.initials: SK
authors.lastname: Lee
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000097:blood* / Q000134:cerebrospinal fluid*; D020274:Autoimmune Diseases of the Nervous System / Q000097:blood* / Q000134:cerebrospinal fluid* / Q000175:diagnosis; D015415:Biomarkers / Q000097:blood / Q000134:cerebrospinal fluid; D005260:Female; D005968:Glutamate Decarboxylase / Q000097:blood* / Q000134:cerebrospinal fluid*; D006801:Humans; D008297:Male; D008875:Middle Aged; D013116:Spinal Cord / Q000378:metabolism; D055815:Young Adult
chemical_list: D001323:Autoantibodies; D015415:Biomarkers; D005968:Glutamate Decarboxylase
keywords: Autoantibodies; Cerebrospinal fluid; Glutamic acid decarboxylase; Immunotherapy; Limbic encephalitis