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Neurotherapeutics. 2016 Jan;13(1):70-83. doi: 10.1007/s13311-015-0400-8.

Present and Future Therapies in Neuromyelitis Optica Spectrum Disorders.

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Department of Neurology, St. Josef-Hospital, Ruhr-University, Bochum, Germany.
Department of Neurology, St. Josef-Hospital, Ruhr-University, Bochum, Germany.


Neuromyelitis optica spectrum disorders (NMOSD) are important evolving entities, which have reached much attention in the recent years. NMOSD are characterized by inflammatory lesions in the optic nerves, spinal cord, and central parts of the brain, as well as an autoimmune process directed against aquaporin-4. As disability in NMOSD accumulates by inflammatory damage from attacks, both the treatment and prevention of attacks are decisive for the long-term outcome. NMOSD attacks are treated with high-dose intravenous corticosteroids and apheresis therapies, in particular therapeutic plasma exchange. In cases of incomplete remission, escalation of attack treatment is recommended. Preventive therapy is immunosuppressive and should by commenced as early as possible. Apart from classical immunosuppressants such as azathioprine and mycophenolate mofetil, repurposed biologicals are increasingly used. B-cell depletion with rituximab and other agents, inhibition of the interleukin-6 receptor with tocilizumab, and blockade of complement-mediated damage by eculizumab all are promising therapeutic strategies evaluated in randomized controlled trials. In this review, we will discuss present and future immunotherapies for NMOSD and also consider combination of treatments, plasma, cellular and other therapies. Current advances in immunopathological knowledge are translated into innovative concepts and begin a new era of NMOSD therapy.


Azathioprine; Devic disease; Eculizumab; Plasmapheresis; Rituximab; Tocilizumab

[Indexed for MEDLINE]
Free PMC Article

Conflict of interest statement

IK has received honoraria for consultancy or speaking and travel reimbursement from Bayer Health Care, Biogen Idec, Chugai, and Novartis; RG has received speaker’s and board honoraria from Baxter, Bayer Schering, Biogen Idec, CLB Behring, Genzyme, Merck Serono, Novartis, Talecris, TEVA, and Wyeth. The department of IK and RG has received grant support from Bayer Health Care, Biogen Idec, Genzyme, Merck Serono, Novartis, and TEVA.

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