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.
A 79-year-old man was admitted to a municipal hospital with a bilateral sensory level below T3 and gait ataxia since the last month (EDSS 6.5). Magnetic resonance imaging (MRI) revealed spinal cord lesions at vertebra C1-4, at T3/4, and brain microangiopathy. Cerebrospinal fluid (CSF) analysis showed mildly increased albumin, but normal immunoglobulin G (IgG), negative oligoclonal bands (OCB), and a normal cell count. Autoimmune myelitis was suspected, and symptoms improved after intravenous glucocorticoids (EDSS 5.0).\nFour months later, a second attack occurred with severe left-sided optic neuritis and worsening of sensorimotor symptoms (EDSS 9.0). MRI revealed an enlarged myelon lesion (medulla oblongata to vertebra C5, partially gadolinium-enhancing). CSF analysis showed pleocytosis (32/µl). Symptoms improved after intravenous steroids (EDSS 8.0).\nAfter another month, a third attack occurred with dysphagia, a high-cervical transverse spinal cord syndrome with marked tetraparesis, and another severe optic neuritis (blindness in left eye; EDSS 9.0). He was admitted to a university hospital. MRI revealed a lesion extending over the entire myelon and brainstem involvement (Fig. a). CSF analysis showed 43 cells/µl including granulocytes. AQP4-Ab were positive in serum and CSF (ΔMFI 816 and 181, respectively, FACS-based assay), and diagnosis of AQP4-Ab positive NMO [] as part of NMOSD [] was established. Intravenous steroids were given, followed by plasma exchange, without improvement. After further deterioration with respiratory insufficiency because of high cervical myelon involvement (EDSS 9.5) and pneumonia, he required intensive care and died shortly thereafter.