• Jae-Won Hyun, Gayoung Kim, Yeseul Kim, In Hye Jeong, Su-Hyun Kim, and Ho Jin Kim.
• Department of Neurology, Research Institute and Hospital of National Cancer Center, Goyang, Korea.
• Neurologist. 2016 Nov 1; 21 (6): 97-98.

IntroductionThe 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 ReportA 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.ConclusionThis case suggests that clinicians should exercise caution before discontinuing effective IT in patients with NMOSD.

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