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

Abstract
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.
AuthorsMotomi Arai
JournalRinsho shinkeigaku = Clinical neurology (Rinsho Shinkeigaku) Vol. 49 Issue 1 Pg. 48-51 (Jan 2009) ISSN: 0009-918X [Print] Japan
PMID19227897 (Publication Type: English Abstract, Journal Article)
Chemical References
  • Aquaporin 4
  • Autoantibodies
  • Immunosuppressive Agents
  • Cyclosporine
Topics
  • Aquaporin 4 (immunology)
  • Autoantibodies (blood)
  • Cyclosporine (therapeutic use)
  • Female
  • Humans
  • Immunosuppressive Agents (therapeutic use)
  • Middle Aged
  • Myelitis, Transverse (drug therapy, immunology)

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