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. 2015 Nov;17(11):48.
doi: 10.1007/s11940-015-0378-x.

Acute and Chronic Management of Neuromyelitis Optica Spectrum Disorder

Affiliations

Acute and Chronic Management of Neuromyelitis Optica Spectrum Disorder

Elena Sherman et al. Curr Treat Options Neurol. 2015 Nov.

Abstract

Neuromyelitis optica and neuromyelitis optica spectrum disorder (NMO/NMOSD) is a rare but clinically aggressive demyelinating disease of the central nervous system (CNS) caused by antibodies against water channel protein aquaporin 4 (AQP4) in the astrocytic foot processes. Patients typically present with optic neuritis (ON) or longitudinally extensive transverse myelitis (LETM). The majority of patients with NMOSD show good response to treatment with steroids and plasmapheresis in the acute setting; however, 90 % of patients will eventually have clinical relapses and accrue permanent disability. Currently, immune modulation is the mainstay of maintenance therapy with anti CD-20 (rituximab, Rituxan™) having collectively the strongest evidence to support its use and mycophenolate mofetil having comparable reductions in absolute relapse rate (ARR) and expanded disability status scale (EDSS) scores. Azathioprine, mitoxantrone, and methotrexate also have retrospective case series data that demonstrate reduction in ARR and stabilization of EDSS but with higher relapse rates and exposure to greater risk of treatment toxicities. Excitingly, multiple novel therapies are under clinical study for patients who are refractory to these first-line therapies including monoclonal antibodies targeting interleukin-6 (IL-6), CD19, CD20, complement, and neutrophil elastase inhibitors which may provide additional options for patients with severe clinical presentations. Importantly, no randomized clinical trials have been published to date comparing clinical outcomes of different maintenance therapies in NMOSD. Several trials are currently underway, and results will help guide future management decisions as current evidence is from many small, retrospective case series and cohort studies with many potential confounds.

Keywords: Neuromyelitis optica; Treatment.

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Figures

Fig. 1
Fig. 1
Treatment algorithm for acute and chronic management of NMOSD.

References

References and Recommended Reading

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance
    1. Pittock SJ, Weinshenker BG, Lucchinetti CF, Wingerchuk DM, Corboy JR, Lennon VA. Neuromyelitis optica brain lesions localized at sites of high aquaporin 4 expression. Arch Neurol. 2006;63(7):964–8. doi: 10.1001/archneur.63.7.964. - DOI - PubMed
    1. Pittock SJ, Lucchinetti CF. Neuromyelitis optica and the evolving spectrum of autoimmune aquaporin-4 channelopathies: a decade later. Ann N Y Acad Sci. 2015 - PMC - PubMed
    1. Bizzoco E, Lolli F, Repice AM, Hakiki B, Falcini M, Barilaro A, et al. Prevalence of neuromyelitis optica spectrum disorder and phenotype distribution. J Neurol. 2009;256(11):1891–8. doi: 10.1007/s00415-009-5171-x. - DOI - PubMed
    1. Wingerchuk DM, Banwell B, Bennett JL, Cabre P, Carroll W, Chitnis T, et al. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology. 2015;85(2):177–89. doi: 10.1212/WNL.0000000000001729. - DOI - PMC - PubMed
    1. Wingerchuk DM, Lennon VA, Pittock SJ, Lucchinetti CF, Weinshenker BG. Revised diagnostic criteria for neuromyelitis optica. Neurology. 2006;66(10):1485–9. doi: 10.1212/01.wnl.0000216139.44259.74. - DOI - PubMed

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