Severe Relapse After Cessation of Immunosuppressive Therapy in a Patient With Neuromyelitis Optica Spectrum Disorder

Introduction: The optimal duration of immunosuppressive therapy (IT) for neuromyelitis optica spectrum disorder (NMOSD) has not been established. Here, we report a case of severe relapse after early cessation of IT. Case Report: A 32-year-old woman presented with a 2-week history of intractable vomiting and hiccups followed by quadriplegia with respiratory insufficiency. Spinal cord MRI showed longitudinally extensive transverse myelitis (LETM) and aquaporin-4-immunoglobulin-G (AQP4-IgG) was positive. She was diagnosed with NMOSD and IT was initiated. She did not experience any clinical attacks during 42 months of IT and her AQP4-IgG status was negative. IT was discontinued on her own decision; 36 months later, the patient experienced a severe LETM relapse with paraplegia, and her AQP4-IgG status reverted to positive. Conclusion: This case suggests that clinicians should exercise caution before discontinuing effective IT in patients with NMOSD.