Previous studies were either based on relatively small patient numbers, included mainly pediatric patients, and/or did not take into account Caucasian patients. So far, only limited data are available on the cerebrospinal fluid (CSF) profile in MOG-EM. Based on evidence from (a) immunological studies suggesting a direct pathogenic impact of MOG-IgG, (b) neuropathological studies demonstrating discrete histopathological features, (c) serological studies reporting a lack of aquaporin-4 (AQP4)-IgG in almost all MOG-IgG-positive patients, and (d) cohort studies suggesting differences in clinical and paraclinical presentation, treatment response and prognosis, MOG-IgG is now considered to denote a disease entity in its own right, distinct from classic MS and from AQP4-IgG-positive NMO spectrum disorders (NMOSD), which is now often referred to as MOG-IgG-associated encephalomyelitis (MOG-EM) or MOG-IgG-associated autoimmune disease. Over the past few years, several studies using new-generation cell-based assays (CBA) have demonstrated a robust association of immunoglobulin G (IgG) autoantibodies targeting full-length, conformationally intact human myelin oligodendrocyte glycoprotein (MOG) with (mostly recurrent) optic neuritis (ON), myelitis, and brainstem encephalitis, as well as with neuromyelitis optica (NMO)-like and acute-disseminated encephalomyelitis (ADEM)-like presentations, rather than with classic multiple sclerosis (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. MOG-IgG-positive EM is characterized by CSF features that are distinct from those in MS. Like pleocytosis, blood–CSF barrier dysfunction was present also during remission in a substantial number of patients. CSF l-lactate levels correlated significantly with the spinal cord lesion load in patients with acute myelitis ( p < 0.0001). 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.
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. Neutrophils were present in > 40% of samples activated lymphocytes were found less frequently and eosinophils and/or plasma cells only very rarely (< 4%). CSF WCC was elevated in > 50% of samples (median 31 cells/μl mostly lymphocytes and monocytes > 100/μl in 12%). If present, intrathecal IgG (and, more rarely, IgM) synthesis was low, often transient and mostly restricted to acute attacks. 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). Material and methodsĬytological 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.
To describe systematically the CSF profile in MOG-EM. 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). 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. Journal of Neuroinflammation volume 17, Article number: 261 ( 2020)
Cerebrospinal fluid findings in patients with myelin oligodendrocyte glycoprotein (MOG) antibodies.
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