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January 2020; 7 (1) ArticleOpen Access

Neuromyelitis optica spectrum disorders with unevenly clustered attack occurrence

Tetsuya Akaishi, Ichiro Nakashima, Toshiyuki Takahashi, Michiaki Abe, Tadashi Ishii, Masashi Aoki
First published November 22, 2019, DOI: https://doi.org/10.1212/NXI.0000000000000640
Tetsuya Akaishi
From the Department of Neurology (T.A., T.T., M.A.), Tohoku University School of Medicine, Sendai, Japan; Department of Education and Support for Regional Medicine (T.A., M.A., T.I.), Tohoku University Hospital, Sendai, Japan; Department of Neurology (I.N.), Tohoku Medical and Pharmaceutical University, Sendai, Japan; and Department of Neurology (T.T.), National Hospital Organization Yonezawa National Hospital, Yonezawa, Japan.
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Ichiro Nakashima
From the Department of Neurology (T.A., T.T., M.A.), Tohoku University School of Medicine, Sendai, Japan; Department of Education and Support for Regional Medicine (T.A., M.A., T.I.), Tohoku University Hospital, Sendai, Japan; Department of Neurology (I.N.), Tohoku Medical and Pharmaceutical University, Sendai, Japan; and Department of Neurology (T.T.), National Hospital Organization Yonezawa National Hospital, Yonezawa, Japan.
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Toshiyuki Takahashi
From the Department of Neurology (T.A., T.T., M.A.), Tohoku University School of Medicine, Sendai, Japan; Department of Education and Support for Regional Medicine (T.A., M.A., T.I.), Tohoku University Hospital, Sendai, Japan; Department of Neurology (I.N.), Tohoku Medical and Pharmaceutical University, Sendai, Japan; and Department of Neurology (T.T.), National Hospital Organization Yonezawa National Hospital, Yonezawa, Japan.
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Michiaki Abe
From the Department of Neurology (T.A., T.T., M.A.), Tohoku University School of Medicine, Sendai, Japan; Department of Education and Support for Regional Medicine (T.A., M.A., T.I.), Tohoku University Hospital, Sendai, Japan; Department of Neurology (I.N.), Tohoku Medical and Pharmaceutical University, Sendai, Japan; and Department of Neurology (T.T.), National Hospital Organization Yonezawa National Hospital, Yonezawa, Japan.
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Tadashi Ishii
From the Department of Neurology (T.A., T.T., M.A.), Tohoku University School of Medicine, Sendai, Japan; Department of Education and Support for Regional Medicine (T.A., M.A., T.I.), Tohoku University Hospital, Sendai, Japan; Department of Neurology (I.N.), Tohoku Medical and Pharmaceutical University, Sendai, Japan; and Department of Neurology (T.T.), National Hospital Organization Yonezawa National Hospital, Yonezawa, Japan.
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Masashi Aoki
From the Department of Neurology (T.A., T.T., M.A.), Tohoku University School of Medicine, Sendai, Japan; Department of Education and Support for Regional Medicine (T.A., M.A., T.I.), Tohoku University Hospital, Sendai, Japan; Department of Neurology (I.N.), Tohoku Medical and Pharmaceutical University, Sendai, Japan; and Department of Neurology (T.T.), National Hospital Organization Yonezawa National Hospital, Yonezawa, Japan.
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Citation
Neuromyelitis optica spectrum disorders with unevenly clustered attack occurrence
Tetsuya Akaishi, Ichiro Nakashima, Toshiyuki Takahashi, Michiaki Abe, Tadashi Ishii, Masashi Aoki
Neurol Neuroimmunol Neuroinflamm Jan 2020, 7 (1) e640; DOI: 10.1212/NXI.0000000000000640

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    Figure 1 The number of patients with ≥2 attacks based on the rate of attacks with optic neuritis (ON)

    ON = optic neuritis

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    Figure 2 Clinical course and relapses in each of the patients with ≥3 attacks (n = 43)

    The presented clinical courses imply a clustered occurrence of attacks, irrespective of the duration from clinical onset.

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    Figure 3 Tendency of clustered attacks in NMOSD, irrespective of treatments

    (A) Histograms of the months between 2 tandem attacks (black bars) and the months of relapse-free period by June 2019 (white bars) in all 69 enrolled patients. The center of the gray-colored diamond is the average and its width is the standard error (SE). (B) Histogram of the months between 2 tandem attacks during the medication-free period. (C) Histogram of the months between 2 tandem attacks during the treatment period with oral relapse-preventive therapy. NMOSD = neuromyelitis optica spectrum disorder.

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    Figure 4 Kaplan-Meier survival analysis comparing relapse-free periods without medications between 0 and 24 months from onset and later

    The analysis compared the relapse-free period without relapse-preventive therapies between 0 and 24 months from the onset (36 patients with no relapse-preventive therapy) and 24–48 months from the onset (14 patients without relapses in the first 24 months). The latter group represents the “nonclustered” intermittent period status. The former group showed a shorter relapse-free period and a higher relapse rate than the latter group (p = 0.0206, generalized Wilcoxon test).

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