A Pilot Study Comparing Treatments for Severe Attacks of Neuromyelitis Optica Spectrum Disorders: Intravenous Methylprednisolone (IVMP) with Add-on Plasma Exchange (PLEX) Versus Simultaneous Ivmp and PLEX

Background: Our previous retrospective study demonstrated that NMOSD patients with an acute attack who did not respond to IVMP alone, however, showed further significant improvement after treatment with PLEX at 6 month-follow-up.

Objective: To compare the efficacy between treatments with intravenous methylprednisolone (IVMP) with subsequent add-on plasma exchange (PLEX) and a combination of simultaneous IVMP and PLEX in neuromyelitis optica spectrum disorders (NMOSD) patients with a severe acute attack.

Method: We conducted a prospective, randomized, controlled, pilot study of the treatments for a severe acute attack in NMOSD patients.

Results: There were eleven AQP4-positive NMOSD patients in the study. One received only IVMP, five received IVMP with subsequent add-on PLEX treatment, and the other five received simultaneous IVMP and PLEX treatment. The attacks comprised myelitis (57.1%) and optic neuritis (42.9%). Both treatments with IVMP followed by subsequent add-on PLEX when needed (not-respond to IVMP treatment) and a combination treatment of simultaneous IVMP+PLEX from the outset showed clinical improvement measured by EDSS at 6 months follow-up, compared to those at the attacks (p-value = 0.07 in IVMP add-on PLEX group and p-value = 0.05 in IVMP+PLEX group), respectively. Although, a trend of a better outcome stratified by EDSS toward early PLEX initiation with IVMP+PLEX treatment was observed at 6 months follow-up, however not significantly.

Conclusion: Early treatment with PLEX should be encouraged especially in NMOSD with a severe acute attack.