Abstract
Background A 38-year-old woman with systemic lupus erythematosus presented with headaches and bilateral hearing loss. Brain MRI was initially suggestive of small-vessel disease developing in the context of neuropsychiatric systemic lupus erythematosus. Several months later, the patient developed optic neuritis, followed by recurrent attacks of myelitis.
Investigations MRI of the spine revealed multifocal regions of myelitis affecting the cervical spine. Serological evaluation revealed the presence of neuromyelitis optica-IgG antibodies. MRI of the brain was nondiagnostic for multiple sclerosis.
Diagnosis Recurrent myelitis and optic neuritis, occurring in the context of neuromyelitis optica (also known as Devic's syndrome).
Management The patient had recurrent attacks of myelitis despite treatment with pulse cyclophosphamide. After initiation of rituximab, the patient experienced symptomatic improvement and had no further attacks of opticospinal disease.
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References
Dyck PJ et al. (2005) History of standard scoring, notation, and summation of neuromuscular signs. A current survey and recommendation. J Peripher Nerv Syst 10: 158–173
Frohman EM (2003) Multiple sclerosis. Med Clin North Am 87: 867–897
Balcer LJ (2006) Clinical practice. Optic neuritis. N Engl J Med 354: 1273–1280
Beck RW et al. (2003) High- and low-risk profiles for the development of multiple sclerosis within 10 years after optic neuritis: experience of the optic neuritis treatment trial. Arch Ophthalmol 121: 944–999
Charil A et al. (2006) MRI and the diagnosis of multiple sclerosis: expanding the concept of “no better explanation”. Lancet Neurol 5: 841–852
Barkhof F et al. (1997) Comparison of MRI criteria at first presentation to predict conversion to clinically definite multiple sclerosis. Brain 120: 2059–2069
Link H and Huang YM (2006) Oligoclonal bands in multiple sclerosis cerebrospinal fluid: an update on methodology and clinical usefulness. J Neuroimmunol 180: 17–28
Salvarani C et al. (2007) Primary central nervous system vasculitis: analysis of 101 patients. Ann Neurol 62: 442–451
Johnson RT and Richardson EP (1968) The neurological manifestations of systemic lupus erythematosus. Medicine (Baltimore) 47: 337–369
Birnbaum J and Kerr D (2007) Devic's syndrome in a woman with systemic lupus erythematosus: diagnostic and therapeutic implications of testing for the neuromyelitis optica IgG autoantibody. Arthritis Rheum 57: 347–351
Mathews MK (2005) Nonarteritic anterior ischemic optic neuropathy. Curr Opin Ophthalmol 16: 341–345
Wingerchuk DM et al. (2006) Revised diagnostic criteria for neuromyelitis optica. Neurology 66: 1485–1489
Wingerchuk DM et al. (1999) The clinical course of neuromyelitis optica (Devic's syndrome). Neurology 53: 1107–1114
Lennon VA et al. (2005) IgG marker of optic-spinal multiple sclerosis binds to the aquaporin-4 water channel. J Exp Med 202: 473–477
Pittock SJ et al. (2008) Neuromyelitis optica and non organ-specific autoimmunity. Arch Neurol 65: 78–83
Ferreira S et al. (2005) Multiple sclerosis, neuropsychiatric lupus and antiphospholipid syndrome: where do we stand. Rheumatology (Oxford) 44: 434–442
Wingerchuk DM et al. (2007) The spectrum of neuromyelitis optica. Lancet Neurol 6: 805–815
Wingerchuk DM and Weinshenker BG (2005) Neuromyelitis optica. Curr Treat Options Neurol 7: 173–182
Lucchinetti CF et al. (2002) A role for humoral mechanisms in the pathogenesis of Devic's neuromyelitis optica. Brain 125: 1450–1461
Cree BA et al. (2005) An open label study of the effects of rituximab in neuromyelitis optica. Neurology 64: 1270–1272
Acknowledgements
Désirée Lie, University of California, Irvine, CA, is the author of and is solely responsible for the content of the learning objectives, questions and answers of the Medscapeaccredited continuing medical education activity associated with this article.
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Birnbaum, J., Kerr, D. Optic neuritis and recurrent myelitis in a woman with systemic lupus erythematosus. Nat Rev Rheumatol 4, 381–386 (2008). https://doi.org/10.1038/ncprheum0818
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DOI: https://doi.org/10.1038/ncprheum0818
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