Demyelinating Neurological Syndromes: The Role of Autoimmunity

Demyelination of the central nervous system (CNS) is a multifaceted clinical presentation, and is often challenging for the practicing neurologist in both diagnostic and therapeutic terms. It may occur either in the setting of primary autoimmune diseases affecting the CNS (such as Multiple Sclerosis (MS), Neuromyelitis Optica or Myelin oligodendrocyte glycoprotein antibody disorders (MOGAD) demyelination), or in the context of systemic autoimmune diseases (SAD) (including Systemic Lupus Erythematosus or Sjogren’s syndrome). Given the distinct pathogenetic pathways between different demyelinating entities, accurate diagnosis and institution of appropriate treatment modalities is of paramount importance. For instance, while administration of interferon-β is beneficial in the setting of demyelination related to MS, it could be detrimental in the context of systemic lupus erythematosus, hallmarked by activation of type I Interferons. Alternatively, these patients could benefit from administration of anti-B cell therapies or other immunomodulatory therapies.

Taken together, patients with CNS demyelination in a background of systemic autoimmune activation, fulfilling or not the diagnosis of SAD, represent a challenging clinical entity regarding pathogenesis, diagnostic approach, and appropriate treatment options.

In view of these challenges, we welcome contributions of Original Research, Reviews, and Case Series aimed at elucidating the pathogenesis, clinical spectrum, diagnosis, and therapeutic interventions in demyelinating syndromes of various autoimmune etiology.