A 59-year-old Japanese woman developed
numbness below the level of the lower chest (day 1). She showed mild
paraparesis and mild sensory disturbance below the level of Th8. Anti-HTLV-1, antinuclear, and anti-
SS-A antibodies were negative. The cerebrospinal fluid yielded 10 lymphocytes/microl and
IgG index 0.46. MRI demonstrated a centrally located hyperintense spinal cord lesion at the level of Th4-6, but there were no lesions in the brain. Weakness improved after two courses of
intravenous infusion of
methylprednisolone 1000 mg. On day 67, she developed
paraplegia,
urinary retention, a Th4 sensory level, and loss of position sense in the lower limbs. EDSS score was 8.0. MRI demonstrated a cord lesion extending from C6 through Th10 levels. Serum anti-
aquaporin 4 (AQP4) antibody was positive. After immunoadsorption
plasmapheresis, strength improved. Visual evoked potential was delayed in the right eye. This case represents a high-risk syndrome of
neuromyelitis optica. As the patient had diabetes,
ocular hypertension, and a high risk of
osteoporosis, she was started on
ciclosporin in addition to a
tapering dose of glucocorti-cold. The patient has remained relapse free for more than one year and serum anti-AQP4 antibody became negative. This case suggests possible beneficial effects of
cyclosporin in preventing attacks of
myelitis with anti-AQP4 seropositivity.