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Combination of cyclosporine A with corticosteroids is effective for the treatment of neuromyelitis optica

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Abstract

Neuromyelitis optica (NMO) and associated NMO spectrum disorders (NMOSDs) are neuroinflammatory diseases that frequently result in severe neurological disabilities. The aim of this study was to explore additional treatment options for NMO/NMOSD patients who are seropositive for anti-aquaporin 4 (AQP4) antibodies. We retrospectively evaluated the efficacy of immunosuppressants for NMO/NMOSDs by reviewing the clinical records of 52 patients confirmed as seropositive for anti-AQP4 antibodies. Of the 52 patients, 26 (23 women, three men) had received at least one kind of immunosuppressant other than corticosteroids. After eliminating ineligible cases, we evaluated the following 24 treatments in 22 patients (20 women, two men) that used azathioprine (AZA) (n = 9), cyclophosphamide (n = 1), cyclosporine A (CyA) (n = 9), tacrolimus (n = 2), methotrexate (n = 1), and mizoribine (n = 2). Both AZA and CyA treatments allowed us to decrease the median dose of the coadministered prednisone without affecting the expanded disability severity scale scores. In patients with relapsing-remitting courses, the annual relapse rate decreased from 1.7 (1.2–2.7) to 0.47 (0.36–0.59) after AZA treatments (n = 6, P = 0.028), and also showed a significant decrease from 2.7 (1.8–4.3) to 0.38 (0–0.97) after CyA treatment (n = 8, P = 0.012). These results indicate that CyA as well as AZA may help stabilize the disease activity in NMO/NMOSD patients seropositive for anti-AQP4 antibodies. This is the first case series study demonstrating the efficacy of CyA for the treatment of NMO/NMOSDs.

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Acknowledgments

We would like to thank Dr Toshiyuki Takahashi, Department of Neurology, Tohoku University Graduate School of Medicine, Japan and Dr Takuya Matsushita, Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, Japan for their support for measuring anti-AQP4 antibodies. This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

Conflicts of interest

The authors declare that they have no conflict of interest.

Ethical standard

All human studies must state that they have been approved by the appropriate ethics committee and have therefore been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki.

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Authors and Affiliations

  1. Department of Neurology, Tenri Hospital, 200, Mishima-cho, Tenri, Nara, 632-8552, Japan

    Takashi Kageyama & Toshihiko Suenaga

  2. Department of Neurology, Graduate School of Medicine, Kyoto University, Kyoto, Japan

    Mika Komori & Ryosuke Takahashi

  3. Department of Neurology, Kinki University School of Medicine, Osaka, Japan

    Katsuichi Miyamoto & Susumu Kusunoki

  4. Department of Neurology, Kitano Hospital, Osaka, Japan

    Akihiko Ozaki, Sadayuki Matsumoto & Takayuki Kondo

  5. Center of Neuroimmunology, Takeda Hospital, Kyoto, Japan

    Takayuki Kondo

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  1. Takashi Kageyama
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  2. Mika Komori
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Correspondence to Takashi Kageyama.

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Kageyama, T., Komori, M., Miyamoto, K. et al. Combination of cyclosporine A with corticosteroids is effective for the treatment of neuromyelitis optica. J Neurol 260, 627–634 (2013). https://doi.org/10.1007/s00415-012-6692-2

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  • DOI: https://doi.org/10.1007/s00415-012-6692-2

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