Akaishi Tetsuya, Takeshita Takayuki, Himori Noriko, Takahashi Toshiyuki, Misu Tatsuro, Ogawa Ryo, Kaneko Kimihiko, Fujimori Juichi, Abe Michiaki, Ishii Tadashi, Fujihara Kazuo, Aoki Masashi, Nakazawa Toru, Nakashima Ichiro
Department of Neurology, Tohoku University Graduate School of Medicine, Sendai, Japan.
Department of Education and Support for Regional Medicine, Tohoku University Hospital, Sendai, Japan.
Front Neurol. 2020 Sep 2;11:932. doi: 10.3389/fneur.2020.00932. eCollection 2020.
The purpose of this study was to elucidate the rapid impact of high-dose intravenous methylprednisolone pulse therapy (1,000 mg/day for 3 days) on the eventual visual prognosis in patients with serum anti-aquaporin-4 immunoglobulin G (AQP4-IgG)-positive neuromyelitis optica spectrum disorders (NMOSDs) who had an attack of optic neuritis (ON). Data from 32 consecutive NMOSD patients (1 male and 31 female) with at least one ON attack, involving a total of 36 ON-involved eyes, were evaluated. The following variables at ON onset were evaluated: sex, age at the first ON episode, visual acuity at nadir, visual acuity after 1 year, duration from ON onset to treatment for an acute ON attack, cycles of high-dose intravenous methylprednisolone pulse therapy for the ON attack, and cycles of plasmapheresis for the ON attack. Among the 36 ON-involved eyes, 27 eyes were studied using orbital MRI with a short-T1 inversion recovery sequence and gadolinium-enhanced fat-suppressed T1 imaging before starting treatment in the acute phase. In univariate analyses, a shorter duration from ON onset to the initiation of high-dose intravenous methylprednisolone pulse therapy favorably affected the eventual visual prognosis 1 year later (Spearman's rho = 0.50, = 0.0018). The lesion length on orbital MRI was also correlated with the eventual visual prognosis (rho = 0.68, < 0.0001). Meanwhile, the days to steroid pulse therapy and lesion length on orbital MRI did not show a significant correlation. These findings suggest that the rapidness of steroid pulse therapy administration affects the eventual visual prognosis independent of the severity of ON. In multivariate analysis, a shorter time from ON onset to the start of acute treatment ( = 0.0004) and a younger age at onset ( = 0.0071) were significantly associated with better visual outcomes. Rapid initiation of high-dose intravenous methylprednisolone pulse therapy is essential to preserve the eventual visual acuity in patients with serum AQP4-IgG-positive NMOSD. Once clinicians suspect acute ON with serum AQP4-IgG, swift administration of steroid pulse therapy before confirming the positivity of serum AQP4-IgG would be beneficial for preserving visual function.
本研究的目的是阐明大剂量静脉注射甲基强的松龙脉冲疗法（1000毫克/天，共3天）对血清抗水通道蛋白4免疫球蛋白G（AQP4-IgG）阳性的视神经脊髓炎谱系障碍（NMOSD）且患有视神经炎（ON）发作的患者最终视觉预后的快速影响。评估了32例连续的NMOSD患者（1例男性和31例女性）的数据，这些患者至少有一次ON发作，共涉及36只受累眼睛。评估了ON发作时的以下变量：性别、首次ON发作时的年龄、最低点视力、1年后的视力、从ON发作到急性ON发作治疗的持续时间、针对ON发作的大剂量静脉注射甲基强的松龙脉冲疗法的疗程，以及针对ON发作的血浆置换疗程。在36只受累眼睛中，27只眼睛在急性期开始治疗前使用短T1反转恢复序列和钆增强脂肪抑制T1成像进行眼眶MRI检查。在单因素分析中，从ON发作到开始大剂量静脉注射甲基强的松龙脉冲疗法的时间较短对1年后的最终视觉预后有有利影响（Spearman等级相关系数=0.50，P=0.0018）。眼眶MRI上的病变长度也与最终视觉预后相关（等级相关系数=0.68，P<0.0001）。同时，类固醇脉冲疗法的天数与眼眶MRI上的病变长度未显示出显著相关性。这些发现表明，类固醇脉冲疗法给药的快速性独立于ON的严重程度影响最终视觉预后。在多因素分析中，从ON发作到开始急性治疗的时间较短（P=0.0004）和发病年龄较小（P=0.0071）与更好的视觉结果显著相关。快速开始大剂量静脉注射甲基强的松龙脉冲疗法对于保留血清AQP4-IgG阳性NMOSD患者的最终视力至关重要。一旦临床医生怀疑血清AQP4-IgG阳性合并急性ON，在确认血清AQP4-IgG阳性之前迅速给予类固醇脉冲疗法将有利于保留视觉功能。
