Neuromyelitis optica spectrum disorder in a patient with systemic lupus erythematosus and anti-phospholipid antibody syndrome

• Published in: Multiple sclerosis 2008-04, Vol.14 (3), p.425-427
• Main Authors: Mehta, Lahar R, Samuelsson, Melissa K, Kleiner, Anatole K, Goodman, Andrew D, Anolik, Jennifer H, Looney, R John, Schwid, Steven R
• Format: Article
• Language: English
• Subjects: Antiphospholipid Syndrome - complications; Antiphospholipid Syndrome - immunology; Antiphospholipid Syndrome - pathology; Aquaporin 4 - immunology; Biological and medical sciences; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; Female; Humans; Immunoglobulin G - blood; Lupus Vasculitis, Central Nervous System - complications; Lupus Vasculitis, Central Nervous System - immunology; Lupus Vasculitis, Central Nervous System - pathology; Magnetic Resonance Imaging; Medical sciences; Middle Aged; Multiple sclerosis and variants. Guillain barré syndrome and other inflammatory polyneuropathies. Leukoencephalitis; Myelitis, Transverse - complications; Myelitis, Transverse - immunology; Myelitis, Transverse - pathology; Neurology; Neuromyelitis Optica - complications; Neuromyelitis Optica - immunology; Neuromyelitis Optica - pathology; Prognosis
• ISSN: 1352-4585; 1477-0970
• DOI: 10.1177/1352458507084107

Neuromyelitis optica (NMO) is a demyelinating disease of the central nervous system characterized by severe episodes of optic nerve and spinal cord inflammation. NMO-IgG (anti-aquaporin-4) has been recently described as a sensitive and specific marker for NMO. As there have been prior published reports of an association between NMO and systemic autoimmune diseases, the prognostic value of the antibody test in these cases is uncertain. We describe a 47-year old woman with recurrent transverse myelitis and a long-standing history of systemic lupus erythematosus (SLE) and antiphospholipid antibody syndrome (APLS). While she did not have a history of optic neuritis, serological testing for the NMO-IgG was positive when she was admitted for her second episode of transverse myelitis. Testing for the NMO-IgG in cases of isolated or recurrent transverse myelitis attributed to current SLE and APLS may help clarify the diagnosis of a distinct disease process likely to cause recurrent and severe disability, warranting more aggressive immunotherapy. Multiple Sclerosis 2008; 14: 425—427. http://msj.sagepub.com