Neurologic syndromes related to anti-GAD65: Clinical and serologic response to treatment
| Autor: | Maarten J. Titulaer, Marco W.J. Schreurs, Mariska M.P. Nagtzaam, Agnita J.W. Boon, Juna M. de Vries, Peter A. E. Sillevis Smitt, Esther Hulsenboom, Amaia Muñoz-Lopetegi, Sanae Boukhrissi, Rinze F. Neuteboom, Anna E M Bastiaansen, Marienke A.A.M. de Bruijn |
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| Přispěvatelé: | Neurology, Immunology |
| Jazyk: | angličtina |
| Rok vydání: | 2020 |
| Předmět: |
0301 basic medicine
Male medicine.medical_treatment Gastroenterology Serology 0302 clinical medicine Outcome Assessment Health Care Young adult Child Aged 80 and over biology Glutamate Decarboxylase Limbic encephalitis Middle Aged Neurology Child Preschool Immunohistochemistry Female Immunotherapy Antibody medicine.symptom Adult medicine.medical_specialty Adolescent Cerebellar Ataxia Stiff-Person Syndrome Article 03 medical and health sciences Young Adult Autoimmune Diseases of the Nervous System Internal medicine Limbic Encephalitis medicine Humans Aged Autoantibodies Retrospective Studies Epilepsy Cerebellar ataxia business.industry Retrospective cohort study medicine.disease 030104 developmental biology biology.protein Neurology (clinical) business 030217 neurology & neurosurgery |
| Zdroj: | Neurology(R) neuroimmunology & neuroinflammation, 7(3). Lippincott Williams & Wilkins Neurology® Neuroimmunology & Neuroinflammation |
| ISSN: | 2332-7812 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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