Rapid Administration of High-Dose Intravenous Methylprednisolone Improves Visual Outcomes After Optic Neuritis in Patients With AQP4-IgG-Positive NMOSD.

Tetsuya Akaishi,Ryo Ogawa,Tadashi Ishii,Kazuo Fujihara,Michiaki Abe,Masashi Aoki,Toshiyuki Takahashi,Kimihiko Kaneko,Tatsuro Misu,Takayuki Takeshita,Noriko Himori,Juichi Fujimori,Toru Nakazawa,Ichiro Nakashima

doi:10.3389/fneur.2020.00932

Abstract

Objective: 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).Methods: 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.Results: 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, p = 0.0018). The lesion length on orbital MRI was also correlated with the eventual visual prognosis (rho = 0.68, p < 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 (p = 0.0004) and a younger age at onset (p = 0.0071) were significantly associated with better visual outcomes.Conclusions: 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.

Highlights

Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune-related neurological disorder that primarily causes astrocytic damage throughout the central nervous system and is characterized by the presence of serum anti-aquaporin-4 immunoglobulin G (AQP4-IgG) [1, 2]
Patients with NMOSD typically present with repeated attacks of optic neuritis (ON) and/or myelitis [3, 4], and are likely to relapse without proper relapse prevention treatments, acquiring neurological disabilities accumulated in a stepwise manner [5,6,7]
Among the 36 ON-involved eyes from 32 patients, 32 ONinvolved eyes from 29 patients were treated in the acute phase with high-dose intravenous methylprednisolone (IVMP) pulse therapy (1,000 mg/day) for 3 days, followed by low-dose oral prednisolone therapy for relapse prevention, whereas the remaining four ON episodes in three patients were not treated for unknown reasons

Summary

Introduction

Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune-related neurological disorder that primarily causes astrocytic damage throughout the central nervous system and is characterized by the presence of serum anti-aquaporin-4 immunoglobulin G (AQP4-IgG) [1, 2]. In the acute phase of attacks in NMOSD, immune suppression with high-dose intravenous methylprednisolone (IVMP) pulse therapy with or without oral tapering is the gold standard treatment at present [8,9,10,11,12]. Not a standard strategy, long-term oral low-dose corticosteroids are used as relapse prevention therapy in some facilities [17, 18]. Other monoclonal antibodies, such as eculizumab, tocilizumab, satralizumab, and inebilizumab, are known to effectively suppress autoimmunity and relapses in NMOSD, not all these listed drugs have been approved yet [19,20,21,22]

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