TY  - JOUR
AU  - Madlener, Marie
AU  - Strippel, Christine
AU  - Thaler, Franziska S.
AU  - Doppler, Kathrin
AU  - Wandinger, Klaus P.
AU  - Lewerenz, Jan
AU  - Ringelstein, Marius
AU  - Roessling, Rosa
AU  - Menge, Til
AU  - Wickel, Jonathan
AU  - Kellingshaus, Christoph
AU  - Mues, Sigrid
AU  - Kraft, Andrea
AU  - Linsa, Andreas
AU  - Tauber, Simone C.
AU  - Berg, Florian Then
AU  - Gerner, Stefan T.
AU  - Paliantonis, Asterios
AU  - Finke, Alexander
AU  - Priller, Josef
AU  - Schirotzek, Ingo
AU  - Süße, Marie
AU  - Sühs, Kurt W.
AU  - Urbanek, Christian
AU  - Senel, Makbule
AU  - Sommer, Claudia
AU  - Kuempfel, Tania
AU  - Pruess, Harald
AU  - Fink, Gereon R.
AU  - Leypoldt, Frank
AU  - Melzer, Nico
AU  - Malter, Michael P.
TI  - Glutamic acid decarboxylase antibody-associated neurological syndromes: Clinical and antibody characteristics and therapy response
JO  - Journal of the neurological sciences
VL  - 445
SN  - 0022-510x
CY  - Amsterdam [u.a.]
PB  - Elsevier Science
M1  - FZJ-2023-01612
SP  - 120540 -
PY  - 2023
AB  - 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.
LB  - PUB:(DE-HGF)16
C6  - 36608627
UR  - <Go to ISI:>//WOS:000923071300001
DO  - DOI:10.1016/j.jns.2022.120540
UR  - https://juser.fz-juelich.de/record/1005758
ER  -