A 56-year-old female with hyperlipidemia presented with a 1-week history of progressive headache and right eye vision loss. MRI revealed right optic pre-chiasmatic and orbital segment neuritis. She received a 3-day course of IV steroids with modest symptom improvement. Seven years later, she developed intermittent dizziness, gait instability, persistent hiccups, nausea, & vomiting. During hospitalization, she experienced right palm numbness with a sensory level at T5. MRI suggested a possible cerebellar vermis infarct, prompting stroke workup and dual antiplatelet therapy.  

Three weeks later, she developed diffuse facial paresthesia, and a repeat MRI showed enhancement of the dorsal brainstem, 4th ventricle, and internal auditory canal, findings atypical for subacute stroke. APQ4 and MOG antibodies were negative. CSF analysis showed 9 nucleated cells (98% lymphocytes), IgG index of 0.60, protein of 16 mg/dL, glucose of 68 mg/dL, and negative oligoclonal bands. She was treated with a 5-day course of pulse steroids, improving her hand and foot numbness.

A week later, she had a recurrence of bilateral lower extremity numbness, with significant improvement following plasmapheresis. Long-term treatment with rituximab was planned. Despite negative APQ4 antibodies, the patient met clinical criteria for NMOSD.