Aquaporin-4 (AQP4)-IgG-positive neuromyelitis optica spectrum disorder (NMOSD) is
an autoimmune astrocytopathic disease and severe attacks of NMOSD can quickly lead
to blindness and paralysis. The international diagnostic criteria of NMOSD (2015)
have facilitated early diagnosis of the disease which allows for early treatment.
Some disease modifying drugs for multiple sclerosis are inefficacious and immunosuppressive
drugs (corticosteroid, azathioprine, mycophenolate mofetil, etc) have been used to
prevent relapse in AQP4-IgG-positive NMOSD. A few years ago, the results of randomized
controlled trials of four monoclonal antibodies (anti-C5, anti-IL-6R, anti-CD19, and
anti-CD20) in AQP4-IgG-positive NMOSD cases were reported and they are expected to
change the therapeutic landscape. Efficacy, safety, tolerability, and practical considerations,
including patient's life style and potential cost, are important in deciding on the
therapeutic strategy. Other therapeutic approaches, such as stem cell transplantation
and tolerance induction, may also be promising in this disease. AQP4-IgG-seronegative
NMOSD is a diagnostic challenge and could be a heterogeneous group of diseases. Myelin
oligodendrocyte glycoprotein (MOG)-IgG-associated disease (MOGAD) is a newly recognized
entity with NMOSD and other clinical phenotypes. Some cases of MOGAD are relapsing.
In addition to immunosuppressants used in AQP4-IgG-positve NMOSD, intravenous immunoglobulins
appear to be efficacious in MOGAD. Overall, rituximab can reduce the relapse rate,
but unlike AQP4-IgG-positive NMOSD, relapse may occur in some MOGAD patients receiving
rituximab despite effective B cell depletion. In this presentation, the therapeutic
issues and evidence of AQP4-IgG-positive NMOSD, AQP4-IgG-negative NMOSD including
MOGAD will be reviewed and the challenges that lie ahead will be discussed.
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