Home > Publications Database > KCNA2 IgG autoimmunity in neuropsychiatric diseases. > print

001     268498
005     20240403131605.0
024 7 _ |a 10.1016/j.bbi.2024.01.220
|2 doi
024 7 _ |a pmid:38309639
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024 7 _ |a 0889-1591
|2 ISSN
024 7 _ |a 1090-2139
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024 7 _ |a altmetric:159407421
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037 _ _ |a DZNE-2024-00244
041 _ _ |a English
082 _ _ |a 150
100 1 _ |a Arlt, Friederike A
|0 P:(DE-2719)9002282
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|e First author
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245 _ _ |a KCNA2 IgG autoimmunity in neuropsychiatric diseases.
260 _ _ |a Orlando, Fla.
|c 2024
|b Academic Press
336 7 _ |a article
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336 7 _ |a Output Types/Journal article
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336 7 _ |a Journal Article
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336 7 _ |a ARTICLE
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336 7 _ |a JOURNAL_ARTICLE
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336 7 _ |a Journal Article
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520 _ _ |a Autoantibodies against the potassium voltage-gated channel subfamily A member 2 (KCNA2) have been described in a few cases of neuropsychiatric disorders, but their diagnostic and pathophysiological role is currently unknown, imposing challenges to medical practice.We retrospectively collected comprehensive clinical and paraclinical data of 35 patients with KCNA2 IgG autoantibodies detected in cell-based and tissue-based assays. Patients' sera and cerebrospinal fluid (CSF) were used for characterization of the antigen, clinical-serological correlations, and determination of IgG subclasses.KCNA2 autoantibody-positive patients (n = 35, median age at disease onset of 65 years, range of 16-83 years, 74 % male) mostly presented with cognitive impairment and/or epileptic seizures but also ataxia, gait disorder and personality changes. Serum autoantibodies belonged to IgG3 and IgG1 subclasses and titers ranged from 1:32 to 1:10,000. KCNA2 IgG was found in the CSF of 8/21 (38 %) patients and in the serum of 4/96 (4.2 %) healthy blood donors. KCNA2 autoantibodies bound to characteristic anatomical areas in the cerebellum and hippocampus of mammalian brain and juxtaparanodal regions of peripheral nerves but reacted exclusively with intracellular epitopes. A subset of four KCNA2 autoantibody-positive patients responded markedly to immunotherapy alongside with conversion to seronegativity, in particular those presenting an autoimmune encephalitis phenotype and receiving early immunotherapy. An available brain biopsy showed strong immune cell invasion. KCNA2 autoantibodies occurred in less than 10 % in association with an underlying tumor.Our data suggest that KCNA2 autoimmunity is clinically heterogeneous. Future studies should determine whether KCNA2 autoantibodies are directly pathogenic or develop secondarily. Early immunotherapy should be considered, in particular if autoantibodies occur in CSF or if clinical or diagnostic findings suggest ongoing inflammation. Suspicious clinical phenotypes include autoimmune encephalitis, atypical dementia, new-onset epilepsy and unexplained epileptic seizures.
536 _ _ |a 353 - Clinical and Health Care Research (POF4-353)
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588 _ _ |a Dataset connected to CrossRef, PubMed, , Journals: pub.dzne.de
650 _ 7 |a Autoantibody
|2 Other
650 _ 7 |a Autoimmune dementia
|2 Other
650 _ 7 |a Autoimmune encephalitis
|2 Other
650 _ 7 |a Epilepsy
|2 Other
650 _ 7 |a Immunotherapy
|2 Other
650 _ 7 |a KCNA2
|2 Other
650 _ 7 |a Kv1.2
|2 Other
650 _ 7 |a Autoantibodies
|2 NLM Chemicals
650 _ 7 |a KCNA2 protein, human
|2 NLM Chemicals
650 _ 7 |a Kv1.2 Potassium Channel
|2 NLM Chemicals
650 _ 2 |a Animals
|2 MeSH
650 _ 2 |a Humans
|2 MeSH
650 _ 2 |a Male
|2 MeSH
650 _ 2 |a Adolescent
|2 MeSH
650 _ 2 |a Young Adult
|2 MeSH
650 _ 2 |a Adult
|2 MeSH
650 _ 2 |a Middle Aged
|2 MeSH
650 _ 2 |a Aged
|2 MeSH
650 _ 2 |a Aged, 80 and over
|2 MeSH
650 _ 2 |a Female
|2 MeSH
650 _ 2 |a Autoimmunity
|2 MeSH
650 _ 2 |a Retrospective Studies
|2 MeSH
650 _ 2 |a Autoantibodies
|2 MeSH
650 _ 2 |a Seizures
|2 MeSH
650 _ 2 |a Autoimmune Diseases of the Nervous System
|2 MeSH
650 _ 2 |a Mammals
|2 MeSH
650 _ 2 |a Kv1.2 Potassium Channel
|2 MeSH
650 _ 2 |a Encephalitis
|2 MeSH
650 _ 2 |a Hashimoto Disease
|2 MeSH
700 1 _ |a Miske, Ramona
|b 1
700 1 _ |a Machule, Marie-Luise
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700 1 _ |a Broegger Christensen, Peter
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700 1 _ |a Mindorf, Swantje
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700 1 _ |a Teegen, Bianca
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700 1 _ |a Borowski, Kathrin
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700 1 _ |a Buthut, Maria
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700 1 _ |a Rößling, Rosa
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700 1 _ |a Sánchez-Sendín, Elisa
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700 1 _ |a van Hoof, Scott
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700 1 _ |a Cordero-Gómez, César
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700 1 _ |a Bünger, Isabel
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700 1 _ |a Radbruch, Helena
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700 1 _ |a Kraft, Andrea
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700 1 _ |a Ayzenberg, Ilya
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700 1 _ |a Klausewitz, Jaqueline
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700 1 _ |a Hansen, Niels
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700 1 _ |a Timäus, Charles
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700 1 _ |a Körtvelyessy, Peter
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700 1 _ |a Postert, Thomas
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700 1 _ |a Baur-Seack, Kirsten
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700 1 _ |a Rost, Constanze
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700 1 _ |a Brunkhorst, Robert
|b 23
700 1 _ |a Doppler, Kathrin
|b 24
700 1 _ |a Haigis, Niklas
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700 1 _ |a Hamann, Gerhard
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700 1 _ |a Kunze, Albrecht
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700 1 _ |a Stützer, Alexandra
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700 1 _ |a Maschke, Matthias
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700 1 _ |a Melzer, Nico
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700 1 _ |a Rosenow, Felix
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700 1 _ |a Siebenbrodt, Kai
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700 1 _ |a Stenør, Christian
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700 1 _ |a Dichgans, Martin
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700 1 _ |a Georgakis, Marios K
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700 1 _ |a Fang, Rong
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700 1 _ |a Petzold, Gabor C
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700 1 _ |a Görtler, Michael
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700 1 _ |a Zerr, Inga
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700 1 _ |a Wunderlich, Silke
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700 1 _ |a Mihaljevic, Ivan
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700 1 _ |a Turko, Paul
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700 1 _ |a Schmidt Ettrup, Marianne
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700 1 _ |a Buchholz, Emilie
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700 1 _ |a Foverskov Rasmussen, Helle
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700 1 _ |a Nasouti, Mahoor
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700 1 _ |a Talucci, Ivan
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700 1 _ |a Maric, Hans M
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700 1 _ |a Heinemann, Stefan H
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700 1 _ |a Endres, Matthias
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700 1 _ |a group, DEMDAS study
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700 1 _ |a Komorowski, Lars
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700 1 _ |a Prüss, Harald
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773 _ _ |a 10.1016/j.bbi.2024.01.220
|g Vol. 117, p. 399 - 411
|0 PERI:(DE-600)1462491-6
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|t Brain, behavior and immunity
|v 117
|y 2024
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