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Cerebrospinal fluid findings in patients with myelin oligodendrocyte glycoprotein (MOG) antibodies. Part 2: Results from 108 lumbar punctures in 80 pediatric patients

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Title:Cerebrospinal fluid findings in patients with myelin oligodendrocyte glycoprotein (MOG) antibodies. Part 2: Results from 108 lumbar punctures in 80 pediatric patients
Creators Name:Jarius, S. and Lechner, C. and Wendel, E.M. and Baumann, M. and Breu, M. and Schimmel, M. and Karenfort, M. and Marina, A.D. and Merkenschlager, A. and Thiels, C. and Blaschek, A. and Salandin, M. and Leiz, S. and Leypoldt, F. and Pschibul, A. and Hackenberg, A. and Hahn, A. and Syrbe, S. and Strautmanis, J. and Häusler, M. and Krieg, P. and Eisenkölbl, A. and Stoffels, J. and Eckenweiler, M. and Ayzenberg, I. and Haas, J. and Höftberger, R. and Kleiter, I. and Korporal-Kuhnke, M. and Ringelstein, M. and Ruprecht, K. and Siebert, N. and Schanda, K. and Aktas, O. and Paul, F. and Reindl, M. and Wildemann, B. and Rostásy, K.
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 children with MOG-EM. MATERIAL AND METHODS: Cytological and biochemical findings (including white cell counts [WCC] and differentiation; frequency and patterns of oligoclonal bands; IgG/IgM/IgA and albumin concentrations and CSF/serum ratios; intrathecal IgG/IgM/IgA 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 108 lumbar punctures in 80 pediatric 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 89% of samples (N = 96), and the MRZ reaction, the most specific laboratory marker of MS known so far, in 100% (N = 29). If present at all, intrathecal IgG synthesis was low, often transient and mostly restricted to acute attacks. Intrathecal IgM synthesis was present in 21% and exclusively detectable during acute attacks. CSF WCC were elevated in 54% of samples (median 40 cells/μl; range 6-256; mostly lymphocytes and monocytes; > 100/μl in 11%). Neutrophils were present in 71% of samples; eosinophils, activated lymphocytes, and plasma cells were seen only rarely (all < 7%). Blood-CSF barrier dysfunction (as indicated by an elevated albumin CSF/serum ratio) was present in 46% of all samples (N = 79) and at least once in 48% of all patients (N = 67) tested. CSF alterations were significantly more frequent and/or more pronounced in patients with acute spinal cord or brain disease than in patients with acute ON and varied strongly depending on attack severity. CSF L-lactate levels correlated significantly with the spinal cord lesions load (measured in vertebral segments) in patients with acute myelitis (p = 0.0099). An analysis of pooled data from the pediatric and the adult cohort showed a significant relationship of QAlb (p < 0.0005), CST TP (p < 0.0001), and CSF L-lactate (p < 0.0003) during acute attacks with age. CONCLUSION: MOG-IgG-associated EM in children is characterized by CSF features that are distinct from those in MS. With regard to most parameters, no marked differences between the pediatric cohort and the adult cohort analyzed in Part 1 were noted. Our findings are important for the differential diagnosis of pediatric MS and MOG-EM and add to the understanding of the immunopathogenesis of this newly described autoimmune disease.
Keywords:MOG Antibody-Associated Disease (MOGAD), Myelin Oligodendrocyte Glycoprotein (MOG), Antibodies, Encephalomyelitis, Cerebrospinal Fluid, Lumbar Puncture, Optic Neuritis, Transverse Myelitis, Neuromyelitis Optica (Devic Syndrome), NMO Spectrum Disorders, Brainstem Encephalitis, Acute Disseminated Encephalomyelitis (ADEM), Children, Multiple Sclerosis (MS), Oligoclonal Bands
Source:Journal of Neuroinflammation
Publisher:BioMed Central
Page Range:262
Date:3 September 2020
Official Publication:https://doi.org/10.1186/s12974-020-01825-1
PubMed:View item in PubMed

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