Neuromyelitis Optica Spectrum Disorder with Severe Myelitis or Insufficient Response to Glucocorticoids

For some patients with neuromyelitis optica spectrum disorder, glucocorticoids alone do not adequately control an acute attack. In these situations, a distinct first-line therapeutic strategy is warranted.

Who this protocol applies to

This approach is indicated when a patient shows an insufficient response to glucocorticoids during a previous attack, has previously had a sufficient response to apheresis therapy, or is presenting with severe myelitis.

Treatment approach

In this clinical setting, apheresis-based therapy is recommended as the first-line intervention. For the most severe presentations, a combination strategy may also be considered. The specific modality, number of treatment cycles, and any concomitant options are detailed in the full protocol.

The complete regimen — including all therapeutic options and their sequencing — is available via the structured protocol below.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1007/s00415-024-12288-2

Apheresis therapy may be the first-line treatment option for patients with: insufficient response to glucocorticoids during previous attacks, sufficient response to apheresis therapy during previous attacks, severe myelitis.
Most studies performed an average of 5 cycles daily or every other day, but up to 10 cycles may be applied.
Either plasma exchange or immunoadsorption may be used as apheresis therapy.
For patients with severe attacks, concomitant treatment with high-dose glucocorticoids and apheresis may be used.

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