Pathophysiology of Acute Disseminated Encephalomyelitis

Update on Acute Disseminated Encephalomyelitis in Children and Adolescents

“…The expression of the MOG protein in mature oligodendrocytes suggests its possible role in the maturation of the oligodendrocytes themselves, as well as playing a key role in maintaining the integrity of myelin, in adhesion and interaction between cells [ 11 ]. The consequent activation of an inflammatory cascade determines an increase in vascular permeability, edema, and blood–brain barrier (BBB) breakdown by the production of cytokines and chemokines, contributes to neuronal damage and causes the infiltration of immune cells in the CNS, thus sustaining the pathogenetic process [ 9 ]. This means that ADEM could be a spectrum of MOG-associated disorders.…”

“…As described for MS, there is also evidence of genetic susceptibility in ADEM; indeed, patients show an increased frequency of haplotype HLA-DRB1, which seems to determine immunoreactivity to epitopes of myelin proteins. In addition, the dysregulation of the immune system can sustain this process by the breakdown of tolerance versus self-antigens [9]. mune and inflammatory process of the CNS triggered by an environmental event, such as infection or vaccination occurring in genetically susceptible individuals (Figure 1).…”

A Case of Autoimmune Acute Disseminated Encephalomyelitis in an Adult Female with Systemic Lupus Erythematosus

“…ADEM is thought to be an autoimmune disease in which it is theorized that autoimmune constituents attack myelin components such as myelin basic protein, proteolipid protein, and myelin oligodendrocyte protein leading to demyelination and diffuse white matter changes [4]. Demyelination is theorized to be precipitated by T cell activation through a cascade of inflammation involving Inflammatory cytokines including tumor necrosis factor α (TNFα), interleukin 2 (IL2) and interferon γ (INFγ) [5]. Due to the active role of inflammatory cytokines in the pathogenesis of ADEM, any disease contributing to systemic formation of inflammatory cytokines can potentially be an etiologic factor for the initiation of ADEM.…”

Radiologic-Pathologic Correlation of COVID-19-Associated Acute Disseminated Encephalomyelitis

“…The pathophysiology of ADEM in the setting of COVID-19 infection is not fully understood, however, the mechanism of other settings of ADEM including molecular mimicry, direct central nervous system infection with an inflammatory cascade, and bystander activation could be explored [ 15 ]. High-dose steroids, intravenous immunoglobulins, and plasma exchanges have been a frequent treatment for suspected ADEM in the setting of COVID-19, as they have been shown to promote better long-term outcomes in non-COVID-19-related cases of ADEM [ 9 , 13 , 16 ].…”