Glutamic acid decarboxylase antibody-associated neurological syndromes: Clinical and antibody characteristics and therapy response

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 A...

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Veröffentlicht in:Journal of the neurological sciences Jg. 445; S. 120540
Hauptverfasser: Madlener, Marie, 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.
Format: Journal Article
Sprache:Englisch
Veröffentlicht: Netherlands Elsevier B.V 15.02.2023
Schlagworte:
Autoantibodies
Autoimmune encephalitis
Cerebellar Ataxia - drug therapy
GAD
GENERATE
Glutamate Decarboxylase
Glutamic acid decarboxylase
Humans
Limbic Encephalitis - therapy
Neurology
Oligoclonal Bands
Stiff-Person Syndrome - therapy
Autoantibodies
E
MRI
EEG
Glutamic acid decarboxylase
IVIG
GAD
RIA
mRS
abs
AINS
PERM
CAT
SPS
CSF
LE
GENERATE
Autoimmune encephalitis
ELISA
Progressive encephalomyelitis with rigidity and myoclonus
Intravenous immunoglobulins
Epilepsy
Antibodies
Electroencephalography
Stiff-person
Cerebrospinal fluid
Radio-immuno-assay
German Network for Research on Autoimmune Encephalitis
Modified Rankin Scale
Autoimmune neurological syndromes
Enzyme-linked-immunosorbent assay
Cerebellar ataxia
Limbic encephalitis
Glutamic acid decarboxylase 65
Magnetic resonance imaging
ISSN:0022-510X, 1878-5883, 1878-5883
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Zusammenfassung: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. 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. 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. 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. •Immunotherapy is moderately effective neurological syndromes with GAD-antibodies.•Serum GAD-antibody levels does not predict clinical characteristics or outcomes.•The detection of oligoclonal bands might outweigh low GAD- antibody serum levels.
Bibliographie:ObjectType-Article-1
SourceType-Scholarly Journals-1
ObjectType-Feature-2
content type line 23
ISSN:0022-510X
1878-5883
1878-5883
DOI:10.1016/j.jns.2022.120540