This title appears in the Scientific Report :
2023
Please use the identifier:
http://dx.doi.org/10.1016/j.jns.2022.120540 in citations.
Please use the identifier: http://hdl.handle.net/2128/34462 in citations.
Glutamic acid decarboxylase antibody-associated neurological syndromes: Clinical and antibody characteristics and therapy response
Glutamic acid decarboxylase antibody-associated neurological syndromes: Clinical and antibody characteristics and therapy response
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...
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Personal Name(s): | Madlener, Marie (Corresponding author) |
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Strippel, Christine / Thaler, Franziska S. / Doppler, Kathrin / Wandinger, Klaus P. / Lewerenz, Jan / Ringelstein, Marius / Roessling, Rosa / Menge, Til / Wickel, Jonathan / Kellingshaus, Christoph / Mues, Sigrid / Kraft, Andrea / Linsa, Andreas / Tauber, Simone C. / Berg, Florian Then / Gerner, Stefan T. / Paliantonis, Asterios / Finke, Alexander / Priller, Josef / Schirotzek, Ingo / Süße, Marie / Sühs, Kurt W. / Urbanek, Christian / Senel, Makbule / Sommer, Claudia / Kuempfel, Tania / Pruess, Harald / Fink, Gereon R. / Leypoldt, Frank / Melzer, Nico / Malter, Michael P. | |
Contributing Institute: |
Kognitive Neurowissenschaften; INM-3 |
Published in: | Journal of the neurological sciences, 445 (2023) S. 120540 - |
Imprint: |
Amsterdam [u.a.]
Elsevier Science
2023
|
DOI: |
10.1016/j.jns.2022.120540 |
Document Type: |
Journal Article |
Research Program: |
Multilevel Brain Organization and Variability |
Link: |
Restricted OpenAccess |
Publikationsportal JuSER |
Please use the identifier: http://hdl.handle.net/2128/34462 in citations.
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. |