An 82-year-old, right-handed Caucasian man was admitted to our neurology service due to rapidly progressive, painless vision loss and sensory loss of the bilateral lower extremities. Prior to the onset of symptoms he was healthy and independent in his activities of daily living. Initially, his vision began to deteriorate in the left eye 2 weeks prior to presentation, and 1 week later he began to have painless vision loss in the right eye. Subsequently, he began to experience abdominal pain in a band around his umbilicus along with numbness that began in the mid-trunk and gradually extended to include his lower extremities, which ultimately led to his presentation to our emergency department.\nHis initial examination showed preserved light perception in the left eye with impaired perception of motion and no light perception in the right eye. He had patchy sensory loss to all modalities below T10. He was hyperreflexic in the left lower extremity with crossed adductors, crossed patellar reflex, and an extensor plantar response on the left. He had no discernable weakness or cranial nerve abnormalities. MRI brain/orbits, C-spine, and T-spine with/without contrast revealed bilateral pre-chiasmatic optic nerve and optic chiasm enhancement, along with patchy but longitudinally extensive hyperintense STIR signal and post-contrast enhancement within the thoracic spinal cord from T3 to T7.\nInitial laboratory testing was unremarkable, including CBC, comprehensive metabolic panel, B12, copper, rapid plasma regain, antinuclear antibodies, erythrocyte sedimentation rate, and C-reactive peptide. His CSF revealed 10 WBCs, 9 RBCs, glucose 52 (roughly 2/3 serum glucose), and protein 59. CSF oligoclonal bands were negative. Serum angiotensin-converting enzyme was low at 3.\nBased on his imaging and CSF findings his working diagnosis was NMO, but neurosarcoidosis was also on our differential given his abnormal age for presentation of NMO. He initially refused steroids, opting for PLEX alone due to the severe reaction he had to oral prednisone 2 months prior (angioedema with airway compromise). He completed 5 rounds of PLEX (every 48 h for 10 days). However, by hospital day 4 his symptoms worsened. His sensory level now extended from T4 and he began to have weakness of the bilateral lower extremities. Ultimately, his serum NMO-IgG returned positive with a titer >1: 100,000, confirming our diagnosis. He reconsidered his position on attempting intravenous steroids given the worsening strength of his upper extremities. Based on the suspicion of lowest probability of cross-reactivity, we initiated dexamethasone, opting for 25 mg every 6 h followed by a taper for 2 days. We moved the patient to an intensive care bed for close monitoring in the event of a severe reaction requiring intubation. His symptoms persisted but did not worsen. However, he suffered from psychosis and hallucinations likely as a consequence of steroid administration on his final 2 days of high-dose dexamethasone. He was ultimately discharged to a rehab facility prior to returning home and arrangements were made to prepare for outpatient rituximab infusions for NMO maintenance therapy.
An 88-year-old woman experienced numbness in her legs and moderate paraparesis with impaired gait since 2 days. Spinal MRI demonstrated a myelon lesion from vertebra T6-9 (Fig. b-c), which was initially attributed to compression myelopathy because of concomitant vertebral disc protrusions. Without specific treatment, she recovered partially and was able to walk with a crutch for >100 m (EDSS 6.0). Her previous medical history was negative for prior potential attacks, but included a transient ischemic attack with dysarthria for <24 h 1.5 years before; cerebral MRI had not shown inflammatory lesions.\nEight months later, she was re-admitted with an anew gait impairment and sensorimotor paraparesis (MRC grade 2–3, EDSS 8.5). MRI demonstrated a new T2 hyperintense myelon lesion from vertebra T10–T12 with central gadolinium enhancement (Fig. d–f). Visual evoked potentials had low amplitudes bilaterally and normal latencies. CSF showed mild lymphomonocytic pleocytosis and positive OCB. Serum AQP4-Ab were positive (1:3200, cell-based immunofluorescence assay), as were antibodies against dsDNA and cardiolipin. Diagnosis of AQP4-Ab positive NMOSD was established, and the first myelon lesion was retrospectively attributed also to NMOSD. Treatment included methylprednisolone 5 × 1 g, a second cycle of 5 × 2 g, and then plasma exchange. She recovered partially (EDSS 7.0). Azathioprine was given up to 150 mg/d (2.2 mg/kg); thiopurine S-methyltransferase activity was normal. After 5 months of therapy, regular blood testing revealed pancytopenia. Azathioprine was stopped, but thrombocytopenia persisted and she died of intestinal bleeding. In addition to azathioprine as a likely cause for bone marrow suppression and thrombocytopenia, she had also developed anti-platelet antibodies.