%0 Journal Article
%A Madlener, Marie
%A Strippel, Christine
%A Thaler, Franziska S.
%A Doppler, Kathrin
%A Wandinger, Klaus P.
%A Lewerenz, Jan
%A Ringelstein, Marius
%A Roessling, Rosa
%A Menge, Til
%A Wickel, Jonathan
%A Kellingshaus, Christoph
%A Mues, Sigrid
%A Kraft, Andrea
%A Linsa, Andreas
%A Tauber, Simone C.
%A Berg, Florian Then
%A Gerner, Stefan T.
%A Paliantonis, Asterios
%A Finke, Alexander
%A Priller, Josef
%A Schirotzek, Ingo
%A Süße, Marie
%A Sühs, Kurt W.
%A Urbanek, Christian
%A Senel, Makbule
%A Sommer, Claudia
%A Kuempfel, Tania
%A Pruess, Harald
%A Fink, Gereon R.
%A Leypoldt, Frank
%A Melzer, Nico
%A Malter, Michael P.
%T Glutamic acid decarboxylase antibody-associated neurological syndromes: Clinical and antibody characteristics and therapy response
%J Journal of the neurological sciences
%V 445
%@ 0022-510x
%C Amsterdam [u.a.]
%I Elsevier Science
%M FZJ-2023-01612
%P 120540 -
%D 2023
%X 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.
%F PUB:(DE-HGF)16
%9 Journal Article
%$ 36608627
%U <Go to ISI:>//WOS:000923071300001
%R 10.1016/j.jns.2022.120540
%U https://juser.fz-juelich.de/record/1005758