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.
An 80-year-old man was admitted for subacute gait disturbance. His bilateral lower limb impairment had developed over 2 weeks. A general physical examination was normal. Neurological examination revealed muscle weakness of grade 4/5 affecting both lower limbs. Deep tendon reflexes were absent in both limbs without pathological reflexes. There was loss of deep sensation below T10. He had bladder and rectal disturbance. His EDSS score was 8.5. Findings from routine serum and CSF studies were normal. Spinal MRI on T2WI revealed high signals extending from Th9 to Th12 in the central part of the cord (Fig. ). The lesions showed no contrast enhancement. Brain MRI showed no abnormality. As autoimmune myelitis was suspected, he received high-dose methylprednisolone (1,000 mg/day for 3 days, 2 courses). His serum was positive for anti-AQP4 antibody on the 15th day of his hospitalization. He was diagnosed with NMOSD and treated with immunoadsorption plasmapheresis and followed up with daily prednisolone (30 mg). The muscle strength of the lower limb slightly improved; however, he was bedridden, and the paraplegia persisted. Repeat MRI showed marked regression of thoracic lesions on the 43rd day of his hospitalization. He was discharged to another hospital for rehabilitation 50 days after his hospitalization with daily prednisolone (10 mg). His EDSS score was 7.5.