Tags

Type your tag names separated by a space and hit enter

Cerebrospinal fluid findings in patients with myelin oligodendrocyte glycoprotein (MOG) antibodies. Part 1: Results from 163 lumbar punctures in 100 adult patients.
J Neuroinflammation. 2020 Sep 03; 17(1):261.JN

Abstract

BACKGROUND

New-generation cell-based assays have demonstrated a robust association of serum autoantibodies to full-length human myelin oligodendrocyte glycoprotein (MOG-IgG) with (mostly recurrent) optic neuritis, myelitis, and brainstem encephalitis, as well as with neuromyelitis optica (NMO)-like or acute-disseminated encephalomyelitis (ADEM)-like presentations. However, only limited data are yet available on cerebrospinal fluid (CSF) findings in MOG-IgG-associated encephalomyelitis (MOG-EM; also termed MOG antibody-associated disease, MOGAD).

OBJECTIVE

To describe systematically the CSF profile in MOG-EM.

MATERIAL AND METHODS

Cytological and biochemical findings (including white cell counts and differentiation; frequency and patterns of oligoclonal bands; IgG/IgM/IgA and albumin concentrations and CSF/serum ratios; intrathecal IgG/IgA/IgM fractions; locally produced IgG/IgM/IgA concentrations; immunoglobulin class patterns; IgG/IgA/IgM reibergrams; Link index; measles/rubella/zoster (MRZ) reaction; other anti-viral and anti-bacterial antibody indices; CSF total protein; CSF L-lactate) from 163 lumbar punctures in 100 adult patients of mainly Caucasian descent with MOG-EM were analyzed retrospectively.

RESULTS

Most strikingly, CSF-restricted oligoclonal IgG bands, a hallmark of multiple sclerosis (MS), were absent in almost 90% of samples (N = 151), and the MRZ reaction, the most specific laboratory marker of MS known so far, in 100% (N = 62). If present, intrathecal IgG (and, more rarely, IgM) synthesis was low, often transient and mostly restricted to acute attacks. CSF WCC was elevated in > 50% of samples (median 31 cells/μl; mostly lymphocytes and monocytes; > 100/μl in 12%). Neutrophils were present in > 40% of samples; activated lymphocytes were found less frequently and eosinophils and/or plasma cells only very rarely (< 4%). Blood-CSF barrier dysfunction (as indicated by an elevated albumin CSF/serum ratio) was present in 48% of all samples and at least once in 55% of all patients (N = 88) tested. The frequency and degree of CSF alterations were significantly higher in patients with acute myelitis than in patients with acute ON and varied strongly depending on attack severity. CSF L-lactate levels correlated significantly with the spinal cord lesion load in patients with acute myelitis (p < 0.0001). Like pleocytosis, blood-CSF barrier dysfunction was present also during remission in a substantial number of patients.

CONCLUSION

MOG-IgG-positive EM is characterized by CSF features that are distinct from those in MS. Our findings are important for the differential diagnosis of MS and MOG-EM and add to the understanding of the immunopathogenesis of this newly described autoimmune disease.

Authors+Show Affiliations

Molecular Neuroimmunology Group, Department of Neurology, University of Heidelberg, Heidelberg, Germany. sven.jarius@med.uni-heidelberg.de.Institute of Clinical Neuroimmunology, University Hospital and Biomedical Center, Ludwig-Maximilians University Munich, Munich, Germany.Department of Neurology, Charité - Universitätsmedizin Berlin, Berlin, Germany. Experimental and Clinical Research Center, Max Delbrueck Center for Molecular Medicine, and Charité Universitätsmedizin Berlin, Berlin, Germany.Molecular Neuroimmunology Group, Department of Neurology, University of Heidelberg, Heidelberg, Germany.Department of Neurology, Hannover Medical School, Hannover, Germany. Department of Clinical Neuroimmunology and Neurochemistry, Hannover Medical School, Hannover, Germany.Department of Neurology, Medical Faculty, Heinrich Heine University, Düsseldorf, Germany. Department of Neurology, Center for Neurology and Neuropsychiatry, LVR-Klinikum, Heinrich Heine University, Düsseldorf, Germany.Institute of Neurology, Medical University of Vienna, Vienna, Austria.Department of Neurology, St Josef Hospital, Ruhr-University Bochum, Bochum, Germany.Department of Neurology, Charité - Universitätsmedizin Berlin, Berlin, Germany.Department of Neurology with Institute of Translational Neurology, University Hospital Münster, Münster, Germany.Department of Regional Health Research, Odense, Denmark. Department of Molecular Medicine, University of Southern Denmark, Odense, Denmark.Institute of Neurology, Medical University of Vienna, Vienna, Austria.Institute of Neurology, Medical University of Vienna, Vienna, Austria.Department of Neuropathology, University of Göttingen, Göttingen, Germany.Caritas Hospital Bad Mergentheim, Bad Mergentheim, Germany.Euroimmun AG, Lübeck, Germany.Department of Neurology, Medical University Innsbruck, Innsbruck, Austria.Department of Neuropathology, University of Göttingen, Göttingen, Germany.Department of Neurology, Charité - Universitätsmedizin Berlin, Berlin, Germany. Experimental and Clinical Research Center, Max Delbrueck Center for Molecular Medicine, and Charité Universitätsmedizin Berlin, Berlin, Germany.Molecular Neuroimmunology Group, Department of Neurology, University of Heidelberg, Heidelberg, Germany.Division of Pediatric Neurology, Department of Pediatrics I, Medical University of Innsbruck, Innsbruck, Austria.Department of Neurology, St Josef Hospital, Ruhr-University Bochum, Bochum, Germany. Marianne-Strauβ-Klinik, Behandlungszentrum Kempfenhausen für Multiple Sklerose Kranke gGmbH, Berg, Germany.Department of Neurology, Medical Faculty, Heinrich Heine University, Düsseldorf, Germany.Department of Neurology, Hannover Medical School, Hannover, Germany.Department of Pediatric Neurology, Children's Hospital Datteln, University Witten/Herdecke, Witten, Germany.Department of Neurology, Medical University Innsbruck, Innsbruck, Austria.Institute of Clinical Neuroimmunology, University Hospital and Biomedical Center, Ludwig-Maximilians University Munich, Munich, Germany.Department of Neurology, Charité - Universitätsmedizin Berlin, Berlin, Germany. Experimental and Clinical Research Center, Max Delbrueck Center for Molecular Medicine, and Charité Universitätsmedizin Berlin, Berlin, Germany.Molecular Neuroimmunology Group, Department of Neurology, University of Heidelberg, Heidelberg, Germany.No affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

32883348

Citation

Jarius, Sven, et al. "Cerebrospinal Fluid Findings in Patients With Myelin Oligodendrocyte Glycoprotein (MOG) Antibodies. Part 1: Results From 163 Lumbar Punctures in 100 Adult Patients." Journal of Neuroinflammation, vol. 17, no. 1, 2020, p. 261.
Jarius S, Pellkofer H, Siebert N, et al. Cerebrospinal fluid findings in patients with myelin oligodendrocyte glycoprotein (MOG) antibodies. Part 1: Results from 163 lumbar punctures in 100 adult patients. J Neuroinflammation. 2020;17(1):261.
Jarius, S., Pellkofer, H., Siebert, N., Korporal-Kuhnke, M., Hümmert, M. W., Ringelstein, M., Rommer, P. S., Ayzenberg, I., Ruprecht, K., Klotz, L., Asgari, N., Zrzavy, T., Höftberger, R., Tobia, R., Buttmann, M., Fechner, K., Schanda, K., Weber, M., Asseyer, S., ... Wildemann, B. (2020). Cerebrospinal fluid findings in patients with myelin oligodendrocyte glycoprotein (MOG) antibodies. Part 1: Results from 163 lumbar punctures in 100 adult patients. Journal of Neuroinflammation, 17(1), 261. https://doi.org/10.1186/s12974-020-01824-2
Jarius S, et al. Cerebrospinal Fluid Findings in Patients With Myelin Oligodendrocyte Glycoprotein (MOG) Antibodies. Part 1: Results From 163 Lumbar Punctures in 100 Adult Patients. J Neuroinflammation. 2020 Sep 3;17(1):261. PubMed PMID: 32883348.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Cerebrospinal fluid findings in patients with myelin oligodendrocyte glycoprotein (MOG) antibodies. Part 1: Results from 163 lumbar punctures in 100 adult patients. AU - Jarius,Sven, AU - Pellkofer,Hannah, AU - Siebert,Nadja, AU - Korporal-Kuhnke,Mirjam, AU - Hümmert,Martin W, AU - Ringelstein,Marius, AU - Rommer,Paulus S, AU - Ayzenberg,Ilya, AU - Ruprecht,Klemens, AU - Klotz,Luisa, AU - Asgari,Nasrin, AU - Zrzavy,Tobias, AU - Höftberger,Romana, AU - Tobia,Rafik, AU - Buttmann,Mathias, AU - Fechner,Kai, AU - Schanda,Kathrin, AU - Weber,Martin, AU - Asseyer,Susanna, AU - Haas,Jürgen, AU - Lechner,Christian, AU - Kleiter,Ingo, AU - Aktas,Orhan, AU - Trebst,Corinna, AU - Rostasy,Kevin, AU - Reindl,Markus, AU - Kümpfel,Tania, AU - Paul,Friedemann, AU - Wildemann,Brigitte, AU - ,, Y1 - 2020/09/03/ PY - 2019/11/08/received PY - 2020/04/23/accepted PY - 2020/9/5/entrez PY - 2020/9/5/pubmed PY - 2021/7/22/medline KW - Acute disseminated encephalomyelitis (ADEM) KW - Antibodies KW - Cerebrospinal fluid KW - Encephalomyelitis KW - Lumbar puncture KW - MOG antibody-associated disease (MOGAD) KW - Multiple sclerosis (MS) KW - Myelin oligodendrocyte glycoprotein (MOG) KW - Neuromyelitis optica (Devic syndrome) KW - Oligoclonal bands KW - Optic neuritis KW - Transverse myelitis SP - 261 EP - 261 JF - Journal of neuroinflammation JO - J Neuroinflammation VL - 17 IS - 1 N2 - BACKGROUND: New-generation cell-based assays have demonstrated a robust association of serum autoantibodies to full-length human myelin oligodendrocyte glycoprotein (MOG-IgG) with (mostly recurrent) optic neuritis, myelitis, and brainstem encephalitis, as well as with neuromyelitis optica (NMO)-like or acute-disseminated encephalomyelitis (ADEM)-like presentations. However, only limited data are yet available on cerebrospinal fluid (CSF) findings in MOG-IgG-associated encephalomyelitis (MOG-EM; also termed MOG antibody-associated disease, MOGAD). OBJECTIVE: To describe systematically the CSF profile in MOG-EM. MATERIAL AND METHODS: Cytological and biochemical findings (including white cell counts and differentiation; frequency and patterns of oligoclonal bands; IgG/IgM/IgA and albumin concentrations and CSF/serum ratios; intrathecal IgG/IgA/IgM fractions; locally produced IgG/IgM/IgA concentrations; immunoglobulin class patterns; IgG/IgA/IgM reibergrams; Link index; measles/rubella/zoster (MRZ) reaction; other anti-viral and anti-bacterial antibody indices; CSF total protein; CSF L-lactate) from 163 lumbar punctures in 100 adult patients of mainly Caucasian descent with MOG-EM were analyzed retrospectively. RESULTS: Most strikingly, CSF-restricted oligoclonal IgG bands, a hallmark of multiple sclerosis (MS), were absent in almost 90% of samples (N = 151), and the MRZ reaction, the most specific laboratory marker of MS known so far, in 100% (N = 62). If present, intrathecal IgG (and, more rarely, IgM) synthesis was low, often transient and mostly restricted to acute attacks. CSF WCC was elevated in > 50% of samples (median 31 cells/μl; mostly lymphocytes and monocytes; > 100/μl in 12%). Neutrophils were present in > 40% of samples; activated lymphocytes were found less frequently and eosinophils and/or plasma cells only very rarely (< 4%). Blood-CSF barrier dysfunction (as indicated by an elevated albumin CSF/serum ratio) was present in 48% of all samples and at least once in 55% of all patients (N = 88) tested. The frequency and degree of CSF alterations were significantly higher in patients with acute myelitis than in patients with acute ON and varied strongly depending on attack severity. CSF L-lactate levels correlated significantly with the spinal cord lesion load in patients with acute myelitis (p < 0.0001). Like pleocytosis, blood-CSF barrier dysfunction was present also during remission in a substantial number of patients. CONCLUSION: MOG-IgG-positive EM is characterized by CSF features that are distinct from those in MS. Our findings are important for the differential diagnosis of MS and MOG-EM and add to the understanding of the immunopathogenesis of this newly described autoimmune disease. SN - 1742-2094 UR - https://www.unboundmedicine.com/medline/citation/32883348/Cerebrospinal_fluid_findings_in_patients_with_myelin_oligodendrocyte_glycoprotein__MOG__antibodies__Part_1:_Results_from_163_lumbar_punctures_in_100_adult_patients_ L2 - https://jneuroinflammation.biomedcentral.com/articles/10.1186/s12974-020-01824-2 DB - PRIME DP - Unbound Medicine ER -