[Relapsing transverse myelitis with anti-aquaporin 4 seropositivity: possible beneficial effects of ciclosporin].
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.
Arai
Motomi
M
Department of Neurology, Seirei Mikatahara General Hospital.
D051401:Aquaporin 4 / Q000276:immunology*; D001323:Autoantibodies / Q000097:blood*; D016572:Cyclosporine / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008875:Middle Aged; D009188:Myelitis, Transverse / Q000188:drug therapy* / Q000276:immunology*
D051401:Aquaporin 4; D001323:Autoantibodies; D007166:Immunosuppressive Agents; D016572:Cyclosporine