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| Hauptseite > Publikationsdatenbank > Glutamic acid decarboxylase antibody-associated neurological syndromes: Clinical and antibody characteristics and therapy response > print |
| Hauptseite > Publikationsdatenbank > Glutamic acid decarboxylase antibody-associated neurological syndromes: Clinical and antibody characteristics and therapy response > print |
| 001 | 1005758 | ||
| 005 | 20230929112520.0 | ||
| 024 | 7 | _ | |a 10.1016/j.jns.2022.120540 |2 doi |
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| 100 | 1 | _ | |a Madlener, Marie |0 P:(DE-HGF)0 |b 0 |e Corresponding author |
| 245 | _ | _ | |a Glutamic acid decarboxylase antibody-associated neurological syndromes: Clinical and antibody characteristics and therapy response |
| 260 | _ | _ | |a Amsterdam [u.a.] |c 2023 |b Elsevier Science |
| 336 | 7 | _ | |a article |2 DRIVER |
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| 520 | _ | _ | |a 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. |
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| 773 | _ | _ | |a 10.1016/j.jns.2022.120540 |g Vol. 445, p. 120540 - |0 PERI:(DE-600)1500645-1 |p 120540 - |t Journal of the neurological sciences |v 445 |y 2023 |x 0022-510x |
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