Management of Neuromyelitis Optica Spectrum Disorder and Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease

Acute Attack Management

High-dose intravenous methylprednisolone is first-line for acute attacks in patients with Neuromyelitis Optica Spectrum Disorder and Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease 1
Plasma exchange or immunoadsorption should be initiated early if steroids fail, as MOG-IgG and AQP4-IgG positive patients show particular responsiveness to antibody-depleting treatments 1, 2
Slow steroid taper is mandatory in patients with Neuromyelitis Optica Spectrum Disorder and Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease, as both conditions carry high risk of flare-ups after rapid steroid cessation 1, 2, 3

The American Academy of Neurology recommends starting preventive therapy immediately after first attack in patients with Neuromyelitis Optica Spectrum Disorder, with FDA-approved options including eculizumab and satralizumab 3
In patients with Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease, preventive therapy is reserved for relapsing disease only, with observational benefit reported from rituximab, IVIG, and oral immunosuppressants 1, 2, 3

Beta-interferons, natalizumab, and fingolimod, approved for Multiple Sclerosis, can exacerbate Neuromyelitis Optica Spectrum Disorder and Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease due to differences in immunopathogenesis 1, 3

MOG-IgG titers are disease-activity dependent, and should be retested if initially negative but clinical suspicion is high, during acute attacks, treatment-free intervals, or 1-3 months after plasma exchange/IVIG 4
Close monitoring during steroid taper is necessary to detect early flare-ups in patients with Neuromyelitis Optica Spectrum Disorder and Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease 1, 2

Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease has a more favorable prognosis than Neuromyelitis Optica Spectrum Disorder, with lower EDSS scores at follow-up even with brainstem involvement 2
Brainstem involvement in first event does not indicate worse prognosis for Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease, unlike Multiple Sclerosis 3