MOG encephalomyelitis: distinct clinical, MRI and CSF features in patients with longitudinal extensive transverse myelitis as first clinical presentation

Abstract

Background Based on clinical, immunological and histopathological evidence, MOG-IgG-associated encephalomyelitis (MOG-EM) has emerged as a distinct disease entity different from multiple sclerosis (MS) and aquaporin-4-antibody-positive neuromyelitis optica spectrum disorder (NMOSD). MOG-EM is associated with a broader clinical phenotype including optic neuritis, myelitis, brainstem lesions and acute disseminated encephalomyelitis with a substantial clinical and radiological overlap to other demyelinating CNS disorders. Objective To evaluate common clinical, MRI and CSF findings, as well as therapy responses in patients with longitudinal extensive transverse myelitis (LETM) as initial clinical presentation of MOG-EM. Methods After excluding patients with a known diagnosis of MS, we identified 153 patients with myelitis of which 7 fulfilled the inclusion criteria and were investigated for MRI, CSF and clinical parameters. Results Patients with LETM as first clinical presentation of MOG-EM display similar characteristics, namely a lack of gadolinium-enhancement in spinal cord MRI, marked pleocytosis, negative oligoclonal bands, a previous history of infections/vaccinations and response to antibody-depleting treatments for acute attacks and long-term treatment. Conclusions We identify common pathological findings in patients with LETM as first clinical presentation of MOG-EM which distinguishes it from other forms of LETM and should lead to testing for MOG-IgG in these cases. Keywords Myelin oligodendrocyte glycoprotein (MOG) antibodies • Diagnosis • Myelitis • Longitudinal extensive transverse myelitis (LETM) • Neuromyelitis optica spectrum disorders (NMOSD) Abbreviations MOG-EM MOG-IgG-associated encephalomyelitis MS Multiple sclerosis NMOSD Neuromyelitis optica spectrum disorder LETM Longitudinal extensive transverse myelitis ADEM Acute disseminated encephalomyelitits ON Optic neuritis MRI Magnetic resonance imaging FLAIR Fluid-attenuated inversion recovery CSF Cerebrospinal fluid EDSS Expanded disability status scale VEP Visual evoked potential

MOG-antibody-associated longitudinal extensive myelitis after ChAdOx1 nCoV-19 vaccination

“…MOG-positive myelitis has also been found after other vaccinations and infections. 15,16 MOG-associated encephalomyelitis is an autoimmune disease often presenting with optic neuritis, myelitis and brainstem lesions. 16 In general, MOG antibody screening is indicated in case of LETM, recurrent optic neuritis or brainstem encephalitis.…”

State-of-the-Art Review: Demyelinating Diseases in Indonesia

“…The role of immunoglobulin G serum antibody to myelin oligodendrocyte glycoprotein (MOG-IgG), a glycoprotein located on the myelin surface of the CNS, has been a focus [28,29].…”

“…It has substantial overlap with other demyelinating diseases in terms of clinical and radiological features; thus, the diagnosis should always be accompanied by MOG-IgG testing. The proposed diagnostic criteria for MOG-EM are based on a combination of [1] monophasic or relapsing acute optic neuritis, myelitis, brainstem encephalitis, or encephalitis, or any combination thereof; [2] MRI or electrophysiological (visual evoked potentials in patients with isolated optic neuritis) findings compatible with CNS demyelination; and [3] seropositivity for MOG-IgG as detected by means of a cell-based assay employing full-length human MOG as target antigen [ 28 , 29 ].…”

Regulatory B Cells and Its Role in Central Nervous System Inflammatory Demyelinating Diseases

“…Compared with NMOSD patients, patients with MOG antibody associated encephalomyelitis usually have a single course of the disease and show better recovery of neurological deficits after the attack. Based on clinical, immunological and histopathological evidence, encephalomyelitis associated with MOG antibodies has been regarded as a distinct disease entity different from MS and NMOSD ( 147 ).…”