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@ARTICLE{Arlt:268498,
      author       = {Arlt, Friederike A and Miske, Ramona and Machule,
                      Marie-Luise and Broegger Christensen, Peter and Mindorf,
                      Swantje and Teegen, Bianca and Borowski, Kathrin and Buthut,
                      Maria and Rößling, Rosa and Sánchez-Sendín, Elisa and
                      van Hoof, Scott and Cordero-Gómez, César and Bünger,
                      Isabel and Radbruch, Helena and Kraft, Andrea and Ayzenberg,
                      Ilya and Klausewitz, Jaqueline and Hansen, Niels and
                      Timäus, Charles and Körtvelyessy, Peter and Postert,
                      Thomas and Baur-Seack, Kirsten and Rost, Constanze and
                      Brunkhorst, Robert and Doppler, Kathrin and Haigis, Niklas
                      and Hamann, Gerhard and Kunze, Albrecht and Stützer,
                      Alexandra and Maschke, Matthias and Melzer, Nico and
                      Rosenow, Felix and Siebenbrodt, Kai and Stenør, Christian
                      and Dichgans, Martin and Georgakis, Marios K and Fang, Rong
                      and Petzold, Gabor C and Görtler, Michael and Zerr, Inga
                      and Wunderlich, Silke and Mihaljevic, Ivan and Turko, Paul
                      and Schmidt Ettrup, Marianne and Buchholz, Emilie and
                      Foverskov Rasmussen, Helle and Nasouti, Mahoor and Talucci,
                      Ivan and Maric, Hans M and Heinemann, Stefan H and Endres,
                      Matthias and Komorowski, Lars and Prüss, Harald},
      collaboration = {group, DEMDAS study},
      title        = {{KCNA}2 {I}g{G} autoimmunity in neuropsychiatric diseases.},
      journal      = {Brain, behavior and immunity},
      volume       = {117},
      issn         = {0889-1591},
      address      = {Orlando, Fla.},
      publisher    = {Academic Press},
      reportid     = {DZNE-2024-00244},
      pages        = {399 - 411},
      year         = {2024},
      abstract     = {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.},
      keywords     = {Animals / Humans / Male / Adolescent / Young Adult / Adult
                      / Middle Aged / Aged / Aged, 80 and over / Female /
                      Autoimmunity / Retrospective Studies / Autoantibodies /
                      Seizures / Autoimmune Diseases of the Nervous System /
                      Mammals / Kv1.2 Potassium Channel / Encephalitis / Hashimoto
                      Disease / Autoantibody (Other) / Autoimmune dementia (Other)
                      / Autoimmune encephalitis (Other) / Epilepsy (Other) /
                      Immunotherapy (Other) / KCNA2 (Other) / Kv1.2 (Other) /
                      Autoantibodies (NLM Chemicals) / KCNA2 protein, human (NLM
                      Chemicals) / Kv1.2 Potassium Channel (NLM Chemicals)},
      cin          = {AG Prüß / AG Dichgans / AG Endres / AG Düzel / AG
                      Petzold ; AG Petzold / AG Zerr},
      ddc          = {150},
      cid          = {I:(DE-2719)1810003 / I:(DE-2719)5000022 /
                      I:(DE-2719)1811005 / I:(DE-2719)5000006 / I:(DE-2719)1013020
                      / I:(DE-2719)1440011-1},
      pnm          = {353 - Clinical and Health Care Research (POF4-353)},
      pid          = {G:(DE-HGF)POF4-353},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:38309639},
      doi          = {10.1016/j.bbi.2024.01.220},
      url          = {https://pub.dzne.de/record/268498},
}