Aggressive and Refractory Attack of AQP4-IgG-Positive Neuromyelitis Optica Spectrum Disorder Treated With...

Area of Science:

• Neuroimmunology
• Autoimmune Neurology

Background:

• Neuromyelitis Optica Spectrum Disorder (NMOSD) is a severe autoimmune astrocytopathy targeting aquaporin-4 (AQP4) channels, leading to complement-mediated neural injury.
• Ravulizumab, a C5 inhibitor, is approved for NMOSD relapse prevention but its efficacy in acute attacks is unestablished.

Observation:

• A 58-year-old woman with relapsing AQP4-IgG-positive NMOSD experienced a fulminant attack with tetraparesis, progressing despite methylprednisolone and plasma exchange.
• Emergent administration of ravulizumab (2,700 mg) followed meningococcal prophylaxis, leading to cessation of neurological decline.

Findings:

• The patient demonstrated gradual motor recovery and improved functional capacity post-ravulizumab treatment, although residual disability remained.
• This case suggests ravulizumab may offer clinical stabilization in NMOSD attacks refractory to conventional therapies.

Implications:

• While preliminary, this suggests a potential role for ravulizumab in managing acute, treatment-resistant NMOSD exacerbations.
• Further controlled studies are needed to confirm the efficacy, safety, and optimal timing of complement inhibition in acute NMOSD relapses.