Immune-mediated Disorders of the Spinal Cord: Diagnosis, Treatment Strategies, and Outcomes in the 21st Century

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Background

During the last 15 years, the identification of specific biomarkers associated with spinal cord inflammation, and important refinements in the technique and interpretation of spine MRI, have dramatically improved our ability to differentiate between myelopathies. A definite etiology can now be identified in a number of spinal cord disorders previously labeled as idiopathic (i.e., idiopathic transverse myelitis), with important treatment and prognostic implications, and the imaging characteristics of spinal cord involvement often provide essential diagnostic clues for diseases affecting multiple locations of the central nervous system. Historical clinical concepts and definitions (i.e., complete vs partial transverse myelitis) can now be integrated with MRI parameters (i.e., sagittal extension and axial location of spinal cord T2-abnormalities) that allow a better stratification and classification of spinal cord disorders. Moreover, the increasing identification of patterns of gadolinium enhancement associated with different myelopathy etiologies further improves diagnostic precision.

Inflammatory immune-mediated disorders are common and often treatable causes of myelopathy that variably include multiple sclerosis, demyelinating myelopathies associated with aquaporin-4 (AQP4)-IgG and myelin oligodendrocyte glycoprotein (MOG)-IgG autoantibodies, paraneoplastic/autoimmune myelopathies, spinal cord sarcoidosis, the novel myelitis following SARS-CoV-2 infection or vaccination, and other myelitis associated with systemic disorders (e.g., Behçet, Sjogren). Differentiating between different immune-mediated myelopathies and other non-inflammatory myelopathy etiologies is crucial since treatment is often different.

The aim of this Research Topic is to summarize recent advances and novel findings on the diagnosis and management of immune-mediated inflammatory myelopathies that can affect children and adults.

We are interested in research articles or review articles addressing important or novel aspects of the diagnosis, differential diagnosis, and treatment/outcome of immune-mediated spinal cord disorders. Review articles should focus on specific etiologic categories of inflammatory myelopathies, or non inflammatory etiologies that can mimic spinal cord inflammation. Case reports presenting unusual clinical-MRI manifestations, diagnostic findings, or treatment response related to a specific myelitis etiology are also welcomed. Lastly, we encourage submission of commentaries or overviews on the historical evolution of classification, diagnostic criteria, and general clinical conceptions of spinal cord disorders.

Dr Flanagan has served on advisory boards for Alexion, Genentech and Horizon Therapeutics. He has received speaker honoraria from Pharmacy Times. He received royalties from UpToDate. Dr Flanagan was a site primary investigator in a randomized clinical trial on Inebilizumab in neuromyelitis optica spectrum disorder run by Medimmune/Viela-Bio/Horizon Therapeutics. Dr Flanagan has received funding from the NIH (R01NS113828). Dr Flanagan is a member of the medical advisory board of the MOG project. Dr Flanagan is an editorial board member of the Journal of the Neurological Sciences and Neuroimmunology Reports. A patent has been submitted on DACH1-IgG as a biomarker of paraneoplastic autoimmunity.

Keywords: Transverse myelitis, Multiple Sclerosis, Neuromyelitis optica, Aquaporin-4 antibodies, Myelin oligodendrocyte glycoprotein antibodies, NMOSD, MOGAD, AQP4-IgG, MOG-IgG

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