59-year-old female transferred after four weeks of ascending weakness to the level of upper extremities with bowel and bladder incontinence. Prior to transfer she had received three doses of intravenous methylprednisolone.
Upon presentation, patient was ventilator dependent, locked-in, but reported normal sensation through eye-blinking. Magnetic resonance imaging (MRI) brain and cervical-spine revealed diffusion restriction in right corona radiata, T2 prolongation in anterior medulla and upper cervical cord, and enhancement to C2- C3 levels, respectively, suggestive of NMOSD. Cerebrospinal fluid cytomorphology and flow cytometry were inconclusive for lymphoma/leukemia, but oligoclonal bands were present. NMOSD serology was negative. MR spectroscopy demonstrated NAA reduction, mild lipid lactate peak, and relative reduction of choline on the side of the lesion, favoring demyelination. Patient received 5-days of IV methylprednisolone, followed by 7 sessions of plasma exchange without clinical improvement.
A stereotactic needle biopsy demonstrated proliferation of perivascular large lymphoid cells with eosinophilic bodies and positivity for CD20, CD45 & BCL-6, MUM-1 indicative of a B-cell lymphoma.