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September 2009

Nineteen Episodes of Recurrent Myelitis in a Woman With Neuromyelitis Optica and Systemic Lupus Erythematosus

Author Affiliations

Author Affiliations: Department of Neurology (Drs Nasir, Kerr, and Birnbaum), and Division of Rheumatology, Department of Medicine (Dr Birnbaum), The Johns Hopkins University School of Medicine, Baltimore, Maryland.

Arch Neurol. 2009;66(9):1160-1163. doi:10.1001/archneurol.2009.194

Objectives  To describe the case of a patient with systemic lupus erythematosus (SLE) and neuromyelitis optica (NMO) who experienced 19 recurrent attacks of myelitis.

Design  Case report.

Setting  An outpatient neurorheumatology clinic at the Johns Hopkins Hospital devoted to care of patients with neurological manifestation of rheumatic diseases.

Patient  A woman with NMO and SLE.

Intervention  Rituximab therapy.

Main Outcome Measures  Clinical and neuroimaging features of relapsing disease.

Results  Recurrent and increasingly severe myelitis attacks still occurred after treatment with rituximab.

Conclusions  It may be progressively more difficult to prevent relapses and commensurate disability in patients with later stages of relapsing NMO. Recognition of NMO as a distinct diagnostic entity in patients with SLE and other rheumatic diseases is crucial, in that institution of earlier targeted immunosuppressant treatment may be more effective than later targeted immunosuppression. The cellular arm of the immune system may be recruited by pathogenic B cells and may explain why relapses may occur after treatment with B cell–depleting therapy.