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001005758 1001_ $$0P:(DE-HGF)0$$aMadlener, Marie$$b0$$eCorresponding author
001005758 245__ $$aGlutamic acid decarboxylase antibody-associated neurological syndromes: Clinical and antibody characteristics and therapy response
001005758 260__ $$aAmsterdam [u.a.]$$bElsevier Science$$c2023
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001005758 520__ $$aBackground: Antibodies against glutamic acid decarboxylase (GAD-abs) at high serum levels are associated with diverse autoimmune neurological syndromes (AINS), including cerebellar ataxia, epilepsy, limbic encephalitis and stiff-person syndrome. The impact of low serum GAD-ab levels in patients with suspected AINS remains controversial. Specific intrathecal GAD-ab synthesis may serve as a marker for GAD-ab-associated nervous system autoimmunity. We present characteristics of a multicentric patient cohort with suspected AINS associated with GAD antibodies (SAINS-GAD+) and explore the relevance of serum GAD-ab levels and intrathecal GAD-ab synthesis. Methods: All patients with SAINS-GAD+ included in the registry of the German Network for Research on Autoimmune Encephalitis (GENERATE) from 2011 to 2019 were analyzed. High serum GAD-ab levels were defined as RIA>2000 U/mL, ELISA>1000 U/mL, or as a positive staining pattern on cell-based assays. Results: One-hundred-one patients were analyzed. In descending order they presented with epilepsy/limbic encephalitis (39%), cerebellar ataxia (28%), stiff person syndrome (22%), and overlap syndrome (12%). Immunotherapy was administered in 89% of cases with improvements in 46%. 35% of SAINS-GAD+ patients had low GAD-ab serum levels. Notably, unmatched oligoclonal bands in CSF but not in serum were more frequent in patients with low GAD-ab serum levels. GAD-ab-levels (high/low) and intrathecal GAD-ab synthesis (present or not) did not impact clinical characteristics and outcome. Conclusions: Overall, immunotherapy in SAINS-GAD+ was moderately effective. Serum GAD-ab levels and the absence or presence of intrathecal GAD-ab synthesis did not predict clinical characteristics or outcomes in SAINS-GAD+. The detection of unmatched oligoclonal bands might outweigh low GAD-ab serum levels.
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001005758 7001_ $$0P:(DE-HGF)0$$aStrippel, Christine$$b1
001005758 7001_ $$0P:(DE-HGF)0$$aThaler, Franziska S.$$b2
001005758 7001_ $$0P:(DE-HGF)0$$aDoppler, Kathrin$$b3
001005758 7001_ $$0P:(DE-HGF)0$$aWandinger, Klaus P.$$b4
001005758 7001_ $$0P:(DE-HGF)0$$aLewerenz, Jan$$b5
001005758 7001_ $$0P:(DE-HGF)0$$aRingelstein, Marius$$b6
001005758 7001_ $$0P:(DE-HGF)0$$aRoessling, Rosa$$b7
001005758 7001_ $$aMenge, Til$$b8
001005758 7001_ $$aWickel, Jonathan$$b9
001005758 7001_ $$aKellingshaus, Christoph$$b10
001005758 7001_ $$aMues, Sigrid$$b11
001005758 7001_ $$aKraft, Andrea$$b12
001005758 7001_ $$aLinsa, Andreas$$b13
001005758 7001_ $$aTauber, Simone C.$$b14
001005758 7001_ $$0P:(DE-Juel1)164343$$aBerg, Florian Then$$b15
001005758 7001_ $$aGerner, Stefan T.$$b16
001005758 7001_ $$aPaliantonis, Asterios$$b17
001005758 7001_ $$aFinke, Alexander$$b18
001005758 7001_ $$aPriller, Josef$$b19
001005758 7001_ $$aSchirotzek, Ingo$$b20
001005758 7001_ $$aSüße, Marie$$b21
001005758 7001_ $$aSühs, Kurt W.$$b22
001005758 7001_ $$aUrbanek, Christian$$b23
001005758 7001_ $$aSenel, Makbule$$b24
001005758 7001_ $$aSommer, Claudia$$b25
001005758 7001_ $$aKuempfel, Tania$$b26
001005758 7001_ $$aPruess, Harald$$b27
001005758 7001_ $$0P:(DE-Juel1)131720$$aFink, Gereon R.$$b28
001005758 7001_ $$0P:(DE-HGF)0$$aLeypoldt, Frank$$b29
001005758 7001_ $$0P:(DE-HGF)0$$aMelzer, Nico$$b30
001005758 7001_ $$0P:(DE-HGF)0$$aMalter, Michael P.$$b31
001005758 773__ $$0PERI:(DE-600)1500645-1$$a10.1016/j.jns.2022.120540$$gVol. 445, p. 120540 -$$p120540 -$$tJournal of the neurological sciences$$v445$$x0022-510x$$y2023
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