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.
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.