Rinsho Shinkeigaku (Clinical Neurology)

Brief Clinical Note

Relapsing transverse myelitis with anti-aquaporin 4 seropositivity: possible beneficial effects of ciclosporin

Motomi Arai, M.D., Ph.D.

Department of Neurology, Seirei Mikatahara General Hospital

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/μl 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 1,000 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 glucocorticoid. 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.
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(CLINICA NEUROL, 49: 48|51, 2009)
key words: transverse myelitis, neuromyelitis optica, anti-aquaporin 4 antibody, corticosteroid, ciclosporin

(Received: 18-Aug-08)