An 82-year-old woman was admitted for acute gait disturbance and hypoesthesia over 3 days. A general physical examination was normal. Neurological examination revealed muscle weakness of grade 4/5 affecting the right lower limb and grade 1/5 affecting the left lower limb. Deep tendon reflexes were hyperactive in both limbs. Babinski and Chaddock signs were positive on both sides. There was a loss of position and vibration sense combined with hypesthesia below Th10. She had bladder and rectal disturbance. Her Expanded Disability Status Scale (EDSS) score was 8.0. Findings from routine serum studies were normal. Cerebrospinal fluid (CSF) analysis showed a cell count of 2/mL (100% mononuclear cells), normal glucose level, an increased protein concentration of 48 mg/dL, a myelin basic protein (MBP) level of 862 mg/dL (normal range <102 mg/dL), immunoglobulin G (IgG) and albumin ratios of 1.18 (normal range <0.7), and positive oligoclonal bands. Spinal magnetic resonance imaging (MRI) on a T2-weighted image (T2WI) demonstrated two high signals extending from Th1 to Th2 and Th7 to Th9 in the central part of the cord (Fig. , arrows). The lesions showed no contrast enhancement. Brain MRI showed no abnormality. As autoimmune myelitis was suspected, she received high-dose methylprednisolone (1,000 mg/day for 3 days, 3 courses). Her serum was positive for anti-AQP4 antibody (cell-based assay) 17 days after the onset. She was diagnosed with NMOSD and treated with immunoadsorption plasmapheresis and followed up with daily prednisolone (30 mg). Her condition improved and she was able to walk using a walking instrument. Repeat MRI showed marked regression of thoracic lesions 47 days after the onset. She was discharged to another hospital for rehabilitation 50 days after the onset with daily prednisolone (15 mg). Her EDSS score was 5.5.
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.