The patient was a 76-year-old woman who had been suffering from Parkinson disease for 5 years. She had Stage III disease, as assessed using the Hoehn and Yahr (H-Y) Scale, and exhibited a stable condition under the control of l-dopa and dopamine agonists. Two weeks prior to admission, the patient became speechless gradually and spent most of the day in bed. Thus, she was admitted to our hospital for further evaluations. The patient exhibited worsening of bradykinesia and cogwheel rigidity. She required guardian's support for everyday life and was diagnosed with H-Y stage IV. She could walk with minimal assistance. Most strikingly, her consciousness level was impaired and estimated to be Glasgow Coma Scale (GCS) E3V3M5. Involvement of the cranial nerves or cerebellar dysfunction was not obvious. No clinical evidence obtained at that time indicated the involvement of the spinal cord. In addition to the neurological disturbances, a tumor was observed in the right breast, which was later pathologically proven to be invasive ductal carcinoma. Cranial fluid-attenuated inversion recovery (FLAIR) magnetic resonance imaging (MRI) examination revealed a high-signal-intensity lesion in the right peri- and intralateral ventricle (Fig. , b). Part of this lesion and the meninges exhibited gadolinium enhancement (Fig. , d). Cerebrospinal fluid (CSF) examination demonstrated the presence of pleocytosis (159 cells/mm3) and an increased protein content (171 mg/dL). A cytological analysis was negative and no significant viral antibody titers were detected. Moreover, no bacterial cultures were obtained. The levels of the myelin basic protein were normal (88 pg/mL; normal <102 pg/mL) and no oligoclonal bands were observed. Anti-AQP-4 antibodies were detected (28.5 U/mL; normal, <3 U/mL) in her serum, whereas various auto-antibodies that indicate paraneoplastic neurological syndromes, such as anti-Yo, anti-Hu, Ro, and others, were negative. Based on these findings, the patient was diagnosed as having late-onset anti-AQP-4 antibody-positive NMOSD with invasive ductal carcinoma and 5-year history of Parkinson disease. Optic neuritis was not observed at that time. The patient received pulsed steroid therapy as the initial treatment, which improved her consciousness level significantly, to GCS E4V4M6. The abnormal signal alterations on MRI also improved (Fig. , f). The results of CSF examination indicating the presence of 32 cells/mm3 and an elevated protein contents of 100 mg/dL were considered to represent partial improvement. After the initial pulsed steroid treatment, the patient received total mastectomy and further rehabilitation therapy for Parkinson disease. Two weeks after the mastectomy, she developed abrupt-onset bilateral leg weakness and walking difficulties; therefore, involvement of the spinal cord was suspected. MRI of the spinal cord revealed the presence of multiple spindle-shaped T2 high-signal-intensity alterations that were indicative of myelitis (Fig. , b). Thus, pulsed steroid therapy was administered again, after which the MRI signal alterations of the spinal cord were reduced (Fig. ) and the patient's leg muscle strength improved. The patient refused further treatment, such as plasma exchange or other immune-modifying medications. Subsequently, the patient was transferred to another hospital for further rehabilitation.
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.