17 year old female presents with sharp, severe, and sudden onset of spontaneous neck pain followed by hypophonic voice, and hand and feet numbness within 15 minutes. Within one hour she was quadriplegic, hypotensive, and developed severe respiratory failure requiring intubation. She undergoes repetitive self induced cervical adjustment, but otherwise without any medical history.
Imaging showed restricted diffusion in the anterior medulla with extensive T2 hyperintensities from C2 to C6 isolated to the anterior segment.
Inflammatory myelopathy was suspected and was treated with IVIG, methypredisolone, rituximab, plasmapheresis, and cyclophosphamide without improvement. She was weaker on her right and was initially areflexic followed by hyperreflexic months later when evaluated at another institution. Negative studies include basic CSF studies, oligoclonal bands, autoimmune, vasculitis workup, and infectious evaluation.