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Madlener, Marie [Author]; Strippel, Christine [Author]; Kellingshaus, Christoph [Author]; Mues, Sigrid [Author]; Kraft, Andrea [Author]; Linsa, Andreas [Author]; Tauber, Simone C. [Author]; Berg, Florian Then [Author]; Gerner, Stefan T. [Author]; Paliantonis, Asterios [Author]; Finke, Alexander [Author]; Priller, Josef [Author]; Thaler, Franziska S. [Author]; Schirotzek, Ingo [Author]; Süße, Marie [Author]; Sühs, Kurt W. [Author]; Urbanek, Christian [Author]; Senel, Makbule [Author]; Sommer, Claudia [Author]; Kuempfel, Tania [Author]; Pruess, Harald [Author]; Fink, Gereon R. [Author]; Leypoldt, Frank [Author]; Doppler, Kathrin [Author]; [...]

Glutamic acid decarboxylase antibody-associated neurological syndromes: Clinical and antibody characteristics and therapy response

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  • Media type: E-Article
  • Title: Glutamic acid decarboxylase antibody-associated neurological syndromes: Clinical and antibody characteristics and therapy response
  • Contributor: Madlener, Marie [Author]; Strippel, Christine [Author]; Kellingshaus, Christoph [Author]; Mues, Sigrid [Author]; Kraft, Andrea [Author]; Linsa, Andreas [Author]; Tauber, Simone C. [Author]; Berg, Florian Then [Author]; Gerner, Stefan T. [Author]; Paliantonis, Asterios [Author]; Finke, Alexander [Author]; Priller, Josef [Author]; Thaler, Franziska S. [Author]; Schirotzek, Ingo [Author]; Süße, Marie [Author]; Sühs, Kurt W. [Author]; Urbanek, Christian [Author]; Senel, Makbule [Author]; Sommer, Claudia [Author]; Kuempfel, Tania [Author]; Pruess, Harald [Author]; Fink, Gereon R. [Author]; Leypoldt, Frank [Author]; Doppler, Kathrin [Author]; [...]
  • Published: Elsevier Science, 2023
  • Published in: Journal of the neurological sciences 445, 120540 - (2023). doi:10.1016/j.jns.2022.120540
  • Language: English
  • DOI: https://doi.org/10.1016/j.jns.2022.120540
  • ISSN: 0022-510X; 1878-5883
  • Origination:
  • Footnote: Diese Datenquelle enthält auch Bestandsnachweise, die nicht zu einem Volltext führen.
  • Description: 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.
  • Access State: Open Access