Pathogenesis of autoimmune demyelination: from multiple sclerosis to neuromyelitis optica spectrum disorders and myelin oligodendrocyte glycoprotein antibody‐associated disease

Abstract

Autoimmunity plays a significant role in the pathogenesis of demyelination. Multiple sclerosis (MS), neuromyelitis optica spectrum disorders (NMOSD) and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) are now recognised as separate disease entities under the amalgam of human central nervous system demyelinating disorders. While these disorders share inherent similarities, investigations into their distinct clinical presentations and lesion pathologies have aided in differential diagnoses and understanding of disease pathogenesis. An interplay of various genetic and environmental factors contributes to each disease, many of which implicate an autoimmune response. The pivotal role of the adaptive immune system has been highlighted by the diagnostic autoantibodies in NMOSD and MOGAD, and the presence of autoreactive lymphocytes in MS lesions. While a number of autoantigens have been proposed in MS, recent emphasis on the contribution of B cells has shed new light on the well-established understanding of T cell involvement in pathogenesis. This review aims to synthesise the clinical characteristics and pathological findings, discuss existing and emerging hypotheses regarding the aetiology of demyelination and evaluate recent pathogenicity studies involving T cells, B cells, and autoantibodies and their implications in human demyelination.

Epidemiological investigation of multiple sclerosis and related medical utilisation in Taiwan

“…19 Although antibody assays for AQP4 are negative in children, recommendations for differential diagnosis have been provided. 23,24 Although these criteria can markedly increase diagnostic accuracy, further studies are needed to confirm this potential reason for the slight decrease in the standardised incidence. There was no significant time trend in the incidence in terms of the sex ratio.…”

Seizures and epilepsy in multiple sclerosis, aquaporin 4 antibody‐positive neuromyelitis optica spectrum disorder, and myelin oligodendrocyte glycoprotein antibody‐associated disease

“…Also, similar to that in AE, autoimmune‐associated epilepsy in MS/AQP4‐NMOSD/MOGAD has a poor response to immunotherapy and requires long‐term ASM therapy 16,19 . The difference between autoimmune‐associated epilepsy in MS/AQP4‐NMOSD/MOGAD and that in AE also lies in prevalence and the underlying pathogenesis 21,22,28 …”

“…However, in contrast to AE, seizures have a lesser prevalence in MS/AQP4‐NMOSD/MOGAD 5,15,19,20 . This can be attributed to a direct blow to neurons by antibodies in AE, versus glia‐mediated damage in inflammatory demyelinating disorders 21,22 …”

“…16,19 The difference between autoimmune-associated epilepsy in MS/AQP4-NMOSD/MOGAD and that in AE also lies in prevalence and the underlying pathogenesis. 21,22,28 Key points to distinguish acute symptomatic seizures from autoimmune-associated epilepsy in MS/AQP4-NMOSD/MOGAD include their radiological features, responsiveness to immune-targeted treatment, and outcomes (Table 1). In brief, acute symptomatic seizures are characterized by reversible findings on magnetic resonance imaging (MRI), immunotherapy-responsiveness, and a better prognosis.…”

General anesthesia, using remimazolam, for the patient with myelin oligodendrocyte glycoprotein antibody associated disease (MOGAD): A case report

“…Myelin oligodendrocyte glycoprotein antibody associated disease (MOGAD) is one of demyelinating diseases of nervous system. Although MOGAD is a different disease from multiple sclerosis (MS) but MOGAD has been occasionally misdiagnosed with MS. [1][2][3] MOGAD is one of auto-immune disease. Both MDGAD itself, and the drugs for the prevention and the treatment of symptoms and signs should be considered to establish anesthesia plan for the patient with MOGAD.…”