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@ARTICLE{Upadhya:277509,
      author       = {Upadhya, Manoj and Stumpf, Alexander and O'Brien-Cairney,
                      Jack and Cordero Gomez, Cesar and Döring, Jan Michael and
                      Hoffmann, Julius and Mueller, Susanne and Fukata, Yuko and
                      van Hoof, Scott and Dhangar, Divya and Wilson, Max A and
                      Atwal, Arunvir and Rosch, Richard and Woodhall, Gavin and
                      Boehm-Sturm, Philipp and Fukata, Masaki and Kreye, Jakob and
                      Schmitz, Dietmar and Wright, Sukhvir K and Kornau,
                      Hans-Christian and Prüss, Harald},
      title        = {{P}atient-derived monoclonal {LGI}1 autoantibodies elicit
                      seizures, behavioral changes and brain {MRI} abnormalities
                      in rodent models.},
      journal      = {Brain, behavior and immunity},
      volume       = {126},
      issn         = {0889-1591},
      address      = {Orlando, Fla. [u.a.]},
      publisher    = {Elsevier},
      reportid     = {DZNE-2025-00420},
      pages        = {342 - 355},
      year         = {2025},
      abstract     = {Limbic encephalitis with leucine-rich glioma inactivated 1
                      (LGI1) protein autoantibodies is associated with cognitive
                      impairment, psychiatric symptoms, and seizures, including
                      faciobrachial dystonic seizures (FBDS). Patient-derived
                      LGI1-autoantibodies cause isolated symptoms of memory
                      deficits in mice and seizures in rats. Using a multimodal
                      experimental approach, we set out to improve the validity of
                      existing in vivo rodent models to further recapitulate the
                      full clinical syndrome of anti-LGI1 antibody mediated
                      disease.A monoclonal anti-LGI1 antibody (anti-LGI1 mAb)
                      derived from a patient's CSF antibody-secreting cell was
                      infused intracerebroventricularly (ICV) into rats and mice
                      for one or two weeks, respectively. Cellular excitability of
                      CA3 pyramidal neurons was determined in hippocampal slices.
                      Structural changes in mouse brains were explored using MRI.
                      Antibody effects on behavior and brain activity of rats were
                      studied using video-EEG.Anti-LGI1 mAbs augmented the
                      excitability of CA3 pyramidal neurons and elicited
                      convulsive and non-convulsive spontaneous epileptic seizures
                      in mice and rats. Mice displayed a hypoactive and anxious
                      phenotype during behavioral testing. MRI revealed acutely
                      increased hippocampal volume after ICV anti-LGI1 mAb
                      infusion. Video-EEG recordings of juvenile rats uncovered
                      two peaks of seizure frequency during the 7-day antibody
                      infusion period resembling the natural progression of
                      seizures in human anti-LGI1 encephalitis.Our data strongly
                      corroborate and extend our understanding of the direct
                      pathogenic and epileptogenic role of human LGI1
                      autoantibodies.},
      keywords     = {Animal model (Other) / Human monoclonal antibody (Other) /
                      LGI1 (Other) / Limbic encephalitis (Other) / Seizure
                      (Other)},
      cin          = {AG Prüß / AG Schmitz},
      ddc          = {150},
      cid          = {I:(DE-2719)1810003 / I:(DE-2719)1810004},
      pnm          = {353 - Clinical and Health Care Research (POF4-353) / 351 -
                      Brain Function (POF4-351)},
      pid          = {G:(DE-HGF)POF4-353 / G:(DE-HGF)POF4-351},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:39984135},
      doi          = {10.1016/j.bbi.2025.02.019},
      url          = {https://pub.dzne.de/record/277509},
}