Pediatric Neuromyelitis Optica Spectrum Disorder Presenting as Intractable Vomiting

Abstract

Pediatric neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune demyelinating disease that affects the central nervous system. Common presentations include weakness and sensory changes secondary to transverse myelitis, or vision loss due to optic neuritis. Area postrema syndrome, characterized by intractable nausea, vomiting, or hiccups secondary to a demyelinating lesion in the chemoreceptor trigger zone, is part of the core diagnostic criteria for NMOSD. However, it has infrequently been described as an initial presentation of pediatric NMOSD. Here, we present the case of a 13-year-old female who presented with intractable vomiting, which was initially presumed to be secondary to gastrointestinal pathology. It was not until she developed transverse myelitis that the etiology of her intractable vomiting was identified as area postrema syndrome secondary to NMOSD. She was successfully treated with rituximab, and she remains relapse-free after the initiation of immunosuppressive therapy. This case highlights this rare presentation of NMOSD and emphasizes the need for increased awareness amongst pediatric providers in recognizing this diagnosis and preventing delays in treatment. Int J Clin Pediatr. 2022;11(2):35-38 doi: https://doi.org/10.14740/ijcp478