2025 American Academy of Neurology Abstract Website

Objective:

To describe two patients with seropositive neuromyelitis optica spectrum disorder (NMOSD) who were treated with solumedrol, plasma exchange (PLEX), and eculizumab.

Background:

NMOSD is a rare and potentially severe immune-mediated disorder marked by neuronal injury primarily affecting the optic nerves, spinal cord, and brainstem. NMOSD pathophysiology is complex, but it is mediated by auto-antibodies to the aquaporin-4 (AQP4) water channel, resulting in complement cascade activation, leading to astrocyte and neuronal cell death. NMOSD disability is driven primarily by acute exacerbations that are classically associated with poor recovery. Several highly effective preventative treatments are now FDA approved for seropositive NMOSD, whereas high dose corticosteroids and plasma exchange remain the mainstay of NMOSD acute attack treatment.

Design/Methods:

Case Series.

Results:

Case 1: 16-year-old female presented with quadriplegia, and was found to have a longitudinally extensive transverse myelitis (LETM) and brainstem lesion due to NMOSD. AQP4 antibody titer was > 1:100,000. She was intubated upon admission. High-dose corticosteroid and PLEX were initiated one day following presentation, and eculizumab were initiated eight days following presentation. She exhibited rapid improvement and within weeks, she recovered upper limb function and regained independence in daily activities.

Case 2: 53-year-old woman presented with quadriparesis, hemifacial spasm, and gait dysfunction, diagnosed with LETM due to NMOSD. AQP4 titer was > 1:10,000. High-dose corticosteroids and PLEX were initiated two days following presentation and eculizumab was initiated nine days following presentation. At discharge four weeks later, she exhibited 4/5 extremity strength, hemifacial spasm resolution, and mild residual gait issues.

Subsequent clinical data for both cases will be presented.

Conclusions:

Early and aggressive treatment of NMOSD with corticosteroids, PLEX, and eculizumab resulted in substantial improvement in both cases. Prompt intervention has the potential to enhance recovery and prevent long term disability, but further research is necessary to optimize treatment protocols in the hospital setting.