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@ARTICLE{Madlener:1005758,
      author       = {Madlener, Marie and Strippel, Christine and Thaler,
                      Franziska S. and Doppler, Kathrin and Wandinger, Klaus P.
                      and Lewerenz, Jan and Ringelstein, Marius and Roessling,
                      Rosa and Menge, Til and Wickel, Jonathan and Kellingshaus,
                      Christoph and Mues, Sigrid and Kraft, Andrea and Linsa,
                      Andreas and Tauber, Simone C. and Berg, Florian Then and
                      Gerner, Stefan T. and Paliantonis, Asterios and Finke,
                      Alexander and Priller, Josef and Schirotzek, Ingo and
                      Süße, Marie and Sühs, Kurt W. and Urbanek, Christian and
                      Senel, Makbule and Sommer, Claudia and Kuempfel, Tania and
                      Pruess, Harald and Fink, Gereon R. and Leypoldt, Frank and
                      Melzer, Nico and Malter, Michael P.},
      title        = {{G}lutamic acid decarboxylase antibody-associated
                      neurological syndromes: {C}linical and antibody
                      characteristics and therapy response},
      journal      = {Journal of the neurological sciences},
      volume       = {445},
      issn         = {0022-510x},
      address      = {Amsterdam [u.a.]},
      publisher    = {Elsevier Science},
      reportid     = {FZJ-2023-01612},
      pages        = {120540 -},
      year         = {2023},
      abstract     = {Background: Antibodies against glutamic acid decarboxylase
                      (GAD-abs) at high serum levels are associated with diverse
                      autoimmune neurological syndromes (AINS), including
                      cerebellar ataxia, epilepsy, limbic encephalitis and
                      stiff-person syndrome. The impact of low serum GAD-ab levels
                      in patients with suspected AINS remains controversial.
                      Specific intrathecal GAD-ab synthesis may serve as a marker
                      for GAD-ab-associated nervous system autoimmunity. We
                      present characteristics of a multicentric patient cohort
                      with suspected AINS associated with GAD antibodies
                      (SAINS-GAD+) and explore the relevance of serum GAD-ab
                      levels and intrathecal GAD-ab synthesis. Methods: All
                      patients with SAINS-GAD+ included in the registry of the
                      German Network for Research on Autoimmune Encephalitis
                      (GENERATE) from 2011 to 2019 were analyzed. High serum
                      GAD-ab levels were defined as RIA>2000 U/mL, ELISA>1000
                      U/mL, or as a positive staining pattern on cell-based
                      assays. Results: One-hundred-one patients were analyzed. In
                      descending order they presented with epilepsy/limbic
                      encephalitis $(39\%),$ cerebellar ataxia $(28\%),$ stiff
                      person syndrome $(22\%),$ and overlap syndrome $(12\%).$
                      Immunotherapy was administered in $89\%$ of cases with
                      improvements in $46\%.$ $35\%$ of SAINS-GAD+ patients had
                      low GAD-ab serum levels. Notably, unmatched oligoclonal
                      bands in CSF but not in serum were more frequent in patients
                      with low GAD-ab serum levels. GAD-ab-levels (high/low) and
                      intrathecal GAD-ab synthesis (present or not) did not impact
                      clinical characteristics and outcome. Conclusions: Overall,
                      immunotherapy in SAINS-GAD+ was moderately effective. Serum
                      GAD-ab levels and the absence or presence of intrathecal
                      GAD-ab synthesis did not predict clinical characteristics or
                      outcomes in SAINS-GAD+. The detection of unmatched
                      oligoclonal bands might outweigh low GAD-ab serum levels.},
      cin          = {INM-3},
      ddc          = {610},
      cid          = {I:(DE-Juel1)INM-3-20090406},
      pnm          = {5251 - Multilevel Brain Organization and Variability
                      (POF4-525)},
      pid          = {G:(DE-HGF)POF4-5251},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {36608627},
      UT           = {WOS:000923071300001},
      doi          = {10.1016/j.jns.2022.120540},
      url          = {https://juser.fz-juelich.de/record/1005758},
}