title: Severe Relapse After Cessation of Immunosuppressive Therapy in a Patient With Neuromyelitis Optica Spectrum Disorder.
abstract: BACKGROUND
  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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  This case suggests that clinicians should exercise caution before discontinuing effective IT in patients with NMOSD.
authors: Department of Neurology, Research Institute and Hospital of National Cancer Center, Goyang, Korea.
authors: Jae-Won
authors: JW
authors: Hyun
authors: Gayoung
authors: G
authors: Kim
authors: Yeseul
authors: Y
authors: Kim
authors: In Hye
authors: IH
authors: Jeong
authors: Su-Hyun
authors: SH
authors: Kim
authors: Ho Jin
authors: HJ
authors: Kim
mesh_terms: D000328:Adult; D051401:Aquaporin 4 / Q000276:immunology; D001323:Autoantibodies / Q000276:immunology; D001379:Azathioprine / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D055118:Medication Adherence; D009471:Neuromyelitis Optica / Q000188:drug therapy* / Q000276:immunology; D012008:Recurrence; D012720:Severity of Illness Index
chemical_list: C496030:AQP4 protein, human; D051401:Aquaporin 4; D001323:Autoantibodies; D007166:Immunosuppressive Agents; D001379:Azathioprine