Evidence map›Paper›PMID 32123047›Full record

ArticleNeurology(R) neuroimmunology & neuroinflammation2020

Neurologic syndromes related to anti-GAD65: Clinical and serologic response to treatment.

Amaia Muñoz-Lopetegi, Marienke A A M de Bruijn, Sanae Boukhrissi, Anna E M Bastiaansen, Mariska M P Nagtzaam, Esther S P Hulsenboom, Agnita J W Boon, Rinze F Neuteboom, Juna M de Vries, Peter A E Sillevis Smitt and 2 more

Erratum issuedOpen access · goldAbstract read
In one paragraph

Article in Neurology(R) neuroimmunology & neuroinflammation, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 109 papers, 2 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
109citing papers in PubMed, 2 pooled it
9.2field-weighted citation impact, top 2% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

109 citing papers in PubMed, 2 syntheses or guidelines pooled it, 190 citations in OpenAlex.

  1. Guideline
  2. Pooled it
  3. Suspected encephalitis in adults.Practical neurology · 2026
    Review
  4. Choroid Plexus Abnormalities in Autoimmune Encephalitis.CNS neuroscience & therapeutics · 2026
    Article
  5. Observational
  6. Review
  7. Autoimmune Encephalitis in Acute Care-Pathology, Diagnosis, and Management.Advanced science (Weinheim, Baden-Wurttemberg, Germany) · 2026
    Review
  8. Review
  9. Long-Term Outcomes in Stiff Person Spectrum Disorder.European journal of neurology · 2026
    Article
  10. Article
  11. Immune cell profiling reveals expanded stem cell-like memory T cells in anti-GAD65-associated neurological syndromes.Proceedings of the National Academy of Sciences of the United States of America · 2026
    Article
  12. Movement Disorders in Antibody-Associated Neurologic Diseases: A Nationwide Study.Neurology(R) neuroimmunology & neuroinflammation · 2026
    Observational
  13. Review
  14. Article
  15. Article
  16. Review
  17. Article
  18. Article
  19. Article
  20. Article

49 more citing papers are in PubMed but not listed here.

4 · The record

Corrections and comments

5 · Who and what money

Authors and funding

12 authors at 1 institution in 3 countries.

Amaia Muñoz-LopetegiFrom the Department of Neurology (A.M.-L., M.A.A.M.d.B., A.E.M.B., M.M.P.N., E.S.P.H., A.J.W.B., R.F.N., J.M.d.V., P.A.E.S.S., M.J.T.) and Department of Immunology (S.B., M.W.J.S.), Erasmus MC University Medical Center; Department of Neurology (A.M.-L.), IDIBAPS, Barcelona, Spain; and Health Care Provider of the European Reference Network on Immunodeficiency, Autoinflammatory and Autoimmune Diseases (ERN-RITA) (M.J.T.), Rotterdam, the Netherlands.
Marienke A A M de BruijnFrom the Department of Neurology (A.M.-L., M.A.A.M.d.B., A.E.M.B., M.M.P.N., E.S.P.H., A.J.W.B., R.F.N., J.M.d.V., P.A.E.S.S., M.J.T.) and Department of Immunology (S.B., M.W.J.S.), Erasmus MC University Medical Center; Department of Neurology (A.M.-L.), IDIBAPS, Barcelona, Spain; and Health Care Provider of the European Reference Network on Immunodeficiency, Autoinflammatory and Autoimmune Diseases (ERN-RITA) (M.J.T.), Rotterdam, the Netherlands.
Sanae BoukhrissiFrom the Department of Neurology (A.M.-L., M.A.A.M.d.B., A.E.M.B., M.M.P.N., E.S.P.H., A.J.W.B., R.F.N., J.M.d.V., P.A.E.S.S., M.J.T.) and Department of Immunology (S.B., M.W.J.S.), Erasmus MC University Medical Center; Department of Neurology (A.M.-L.), IDIBAPS, Barcelona, Spain; and Health Care Provider of the European Reference Network on Immunodeficiency, Autoinflammatory and Autoimmune Diseases (ERN-RITA) (M.J.T.), Rotterdam, the Netherlands.
Anna E M BastiaansenFrom the Department of Neurology (A.M.-L., M.A.A.M.d.B., A.E.M.B., M.M.P.N., E.S.P.H., A.J.W.B., R.F.N., J.M.d.V., P.A.E.S.S., M.J.T.) and Department of Immunology (S.B., M.W.J.S.), Erasmus MC University Medical Center; Department of Neurology (A.M.-L.), IDIBAPS, Barcelona, Spain; and Health Care Provider of the European Reference Network on Immunodeficiency, Autoinflammatory and Autoimmune Diseases (ERN-RITA) (M.J.T.), Rotterdam, the Netherlands.
Mariska M P NagtzaamFrom the Department of Neurology (A.M.-L., M.A.A.M.d.B., A.E.M.B., M.M.P.N., E.S.P.H., A.J.W.B., R.F.N., J.M.d.V., P.A.E.S.S., M.J.T.) and Department of Immunology (S.B., M.W.J.S.), Erasmus MC University Medical Center; Department of Neurology (A.M.-L.), IDIBAPS, Barcelona, Spain; and Health Care Provider of the European Reference Network on Immunodeficiency, Autoinflammatory and Autoimmune Diseases (ERN-RITA) (M.J.T.), Rotterdam, the Netherlands.
Esther S P HulsenboomFrom the Department of Neurology (A.M.-L., M.A.A.M.d.B., A.E.M.B., M.M.P.N., E.S.P.H., A.J.W.B., R.F.N., J.M.d.V., P.A.E.S.S., M.J.T.) and Department of Immunology (S.B., M.W.J.S.), Erasmus MC University Medical Center; Department of Neurology (A.M.-L.), IDIBAPS, Barcelona, Spain; and Health Care Provider of the European Reference Network on Immunodeficiency, Autoinflammatory and Autoimmune Diseases (ERN-RITA) (M.J.T.), Rotterdam, the Netherlands.
Agnita J W BoonFrom the Department of Neurology (A.M.-L., M.A.A.M.d.B., A.E.M.B., M.M.P.N., E.S.P.H., A.J.W.B., R.F.N., J.M.d.V., P.A.E.S.S., M.J.T.) and Department of Immunology (S.B., M.W.J.S.), Erasmus MC University Medical Center; Department of Neurology (A.M.-L.), IDIBAPS, Barcelona, Spain; and Health Care Provider of the European Reference Network on Immunodeficiency, Autoinflammatory and Autoimmune Diseases (ERN-RITA) (M.J.T.), Rotterdam, the Netherlands.
Rinze F NeuteboomFrom the Department of Neurology (A.M.-L., M.A.A.M.d.B., A.E.M.B., M.M.P.N., E.S.P.H., A.J.W.B., R.F.N., J.M.d.V., P.A.E.S.S., M.J.T.) and Department of Immunology (S.B., M.W.J.S.), Erasmus MC University Medical Center; Department of Neurology (A.M.-L.), IDIBAPS, Barcelona, Spain; and Health Care Provider of the European Reference Network on Immunodeficiency, Autoinflammatory and Autoimmune Diseases (ERN-RITA) (M.J.T.), Rotterdam, the Netherlands.ORCID 0000-0001-6136-4981
Juna M de VriesFrom the Department of Neurology (A.M.-L., M.A.A.M.d.B., A.E.M.B., M.M.P.N., E.S.P.H., A.J.W.B., R.F.N., J.M.d.V., P.A.E.S.S., M.J.T.) and Department of Immunology (S.B., M.W.J.S.), Erasmus MC University Medical Center; Department of Neurology (A.M.-L.), IDIBAPS, Barcelona, Spain; and Health Care Provider of the European Reference Network on Immunodeficiency, Autoinflammatory and Autoimmune Diseases (ERN-RITA) (M.J.T.), Rotterdam, the Netherlands.
Peter A E Sillevis SmittFrom the Department of Neurology (A.M.-L., M.A.A.M.d.B., A.E.M.B., M.M.P.N., E.S.P.H., A.J.W.B., R.F.N., J.M.d.V., P.A.E.S.S., M.J.T.) and Department of Immunology (S.B., M.W.J.S.), Erasmus MC University Medical Center; Department of Neurology (A.M.-L.), IDIBAPS, Barcelona, Spain; and Health Care Provider of the European Reference Network on Immunodeficiency, Autoinflammatory and Autoimmune Diseases (ERN-RITA) (M.J.T.), Rotterdam, the Netherlands.
Marco W J SchreursFrom the Department of Neurology (A.M.-L., M.A.A.M.d.B., A.E.M.B., M.M.P.N., E.S.P.H., A.J.W.B., R.F.N., J.M.d.V., P.A.E.S.S., M.J.T.) and Department of Immunology (S.B., M.W.J.S.), Erasmus MC University Medical Center; Department of Neurology (A.M.-L.), IDIBAPS, Barcelona, Spain; and Health Care Provider of the European Reference Network on Immunodeficiency, Autoinflammatory and Autoimmune Diseases (ERN-RITA) (M.J.T.), Rotterdam, the Netherlands.
Maarten J TitulaerFrom the Department of Neurology (A.M.-L., M.A.A.M.d.B., A.E.M.B., M.M.P.N., E.S.P.H., A.J.W.B., R.F.N., J.M.d.V., P.A.E.S.S., M.J.T.) and Department of Immunology (S.B., M.W.J.S.), Erasmus MC University Medical Center; Department of Neurology (A.M.-L.), IDIBAPS, Barcelona, Spain; and Health Care Provider of the European Reference Network on Immunodeficiency, Autoinflammatory and Autoimmune Diseases (ERN-RITA) (M.J.T.), Rotterdam, the Netherlands. m.titulaer@erasmusmc.nl.ORCID 0000-0002-1033-3840
MACOM (United States) · US

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

objectiveAntibodies against glutamic acid decarboxylase 65 (anti-GAD65) are associated with a number of neurologic syndromes. However, their pathogenic role is controversial. Our objective was to describe clinical and paraclinical characteristics of anti-GAD65 patients and analyze their response to immunotherapy.

methodsRetrospectively, we studied patients (n = 56) with positive anti-GAD65 and any neurologic symptom. We tested serum and CSF with ELISA, immunohistochemistry, and cell-based assay. Accordingly, we set a cutoff value of 10,000 IU/mL in serum by ELISA to group patients into high-concentration (n = 36) and low-concentration (n = 20) groups. We compared clinical and immunologic features and analyzed response to immunotherapy.

resultsClassical anti-GAD65-associated syndromes were seen in 34/36 patients with high concentration (94%): stiff-person syndrome (7), cerebellar ataxia (3), chronic epilepsy (9), limbic encephalitis (9), or an overlap of 2 or more of the former (6). Patients with low concentrations had a broad, heterogeneous symptom spectrum. Immunotherapy was effective in 19/27 treated patients (70%), although none of them completely recovered. Antibody concentration reduction occurred in 15/17 patients with available pre- and post-treatment samples (median reduction 69%; range 27%-99%), of which 14 improved clinically. The 2 patients with unchanged concentrations showed no clinical improvement. No differences in treatment responses were observed between specific syndromes.

conclusionMost patients with high anti-GAD65 concentrations (>10,000 IU/mL) showed some improvement after immunotherapy, unfortunately without complete recovery. Serum antibody concentrations' course might be useful to monitor response. In patients with low anti-GAD65 concentrations, especially in those without typical clinical phenotypes, diagnostic alternatives are more likely.

Indexed as

Autoimmune Diseases of the Nervous SystemCerebellar AtaxiaEpilepsyLimbic EncephalitisOutcome Assessment, Health CareStiff-Person SyndromeAdolescentAdultAgedAged, 80 and overAutoantibodiesChildChild, PreschoolFemaleGlutamate DecarboxylaseHumansAutoantibodiesGlutamate Decarboxylaseglutamate decarboxylase 2

Identifiers

PMID32123047
PMCPMC7136051
OpenAlexW3009841438

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.