Neuromyelitis optica spectrum disorder (NMOSD) is an inflammatory disease of the central nervous system characterized by relapses and autoimmunity caused by antibodies against the astrocyte water channel protein aquaporin-4. Over the past decade, there have been significant advances in the biologic knowledge of NMOSD, which resulted in the IDENTIFICATION of variable disease phenotypes, biomarkers, and complex inflammatory cascades involved in disease pathogenesis. Ongoing clinical trials are looking at new treatments targeting NMOSD relapses. This review aims to provide an update on recent studies regarding issues related to NMOSD, including the pathophysiology of the disease, the potential use of serum and cerebrospinal fluid cytokines as disease biomarkers, the clinical utilization of ocular coherence tomography, and the comparison of different animal models of NMOSD.

Background: We investigated the therapeutic effects and related mechanisms of algae oil (ALG) to protect retinal ganglion cells (RGCs) in a rat model of anterior ischemic optic neuropathy (rAION). Methods: Rats were daily gavaged with ALG after rAION induction for seven days. The therapeutic effects of ALG on rAION were evaluated using flash visual evoked potentials (FVEPs), retrograde labeling of RGCs, TUNEL assay of the retina, and ED1 staining of optic nerves (ONs). The levels of inducible nitric oxide synthase (iNOS), IL-1β, TNF-α, Cl-caspase-3, ciliary neurotrophic factor (CNTF), and p-ERK were analyzed by using western blots. Results: Protection of visual function in FVEPs amplitude was noted, with a better preservation of the P1–N2 amplitude in the ALG-treated group (p = 0.032) than in the rAION group. The density of RGCs was 2.4-fold higher in the ALG-treated group compared to that in the rAION group (p < 0.0001). The number of ED1-positive cells in ONs was significantly reduced 4.1-fold in the ALG-treated group compared to those in the rAION group (p = 0.029). The number of apoptotic RGCs was 3.2-fold lower in number in the ALG-treated group (p = 0.001) than that in the rAION group. The ALG treatment inhibited ERK activation to reduce the levels of iNOS, IL-1β, TNF-α, and Cl-caspase-3 and to increase the level of CNTF in the rAION model. Conclusion: The treatment with ALG after rAION induction inhibits ERK activation to provide both anti-inflammatory and antiapoptotic effects in rAION.

Background To investigate the one-year visual and anatomical outcomes of combination therapy with intravitreal aflibercept (IVA) and photodynamic therapy (PDT) for treating polypoidal choroidal vasculopathy (PCV). Methods This was a retrospective case-series study, including 30 eyes from 30 patients with treatment-naïve PCV treated by combination therapy with IVA and PDT. Best-corrected visual acuity (BCVA), central retinal thickness (CRT), complete polyp regression rate, and dry macula rate were recorded every 3 months during 12-month follow-up. Clinical factors associated with final visual outcome and retreatment were investigated. Results The mean LogMAR BCVA was significantly improved from 0.73 ± 0.65 at baseline to 0.51 ± 0.60 ( p = 0.01), and the mean CRT was also significantly improved from 339 ± 96 μm at baseline to 244 ± 43 μm at 12-month follow-up ( p <  0.001). Complete regression of polypoidal lesions was 76.7%, and dry macula rate was 100% at 12 months. Better final BCVA was associated with younger age and better baseline BCVA ( p = 0.02 and p <  0 001). The patients without complete polyp regression at 3-month follow-up were associated with retreatment ( p = 0.03). Conclusion In this study, combination therapy with IVA and PDT had significant visual and anatomical improvements to PCV patients during one-year follow-up. Better baseline BCVA and younger age were found to be associated with better visual outcome. Electronic supplementary material The online version of this article (10.1186/s40360-019-0310-1) contains supplementary material, which is available to authorized users.

The authors performed a retrospective and comparative study to compare the efficacy of intravitreal aflibercept and bevacizumab for patients with myopic choroidal neovascularization (mCNV). The patients with treatment-naïve mCNV received 1 + PRN intravitreal bevacizumab from March 2008 to February 2013, while from March 2013 to July 2016 patients were treated by 1 + PRN intravitreal aflibercept, all with monthly follow-up for 12 months. Primary outcome measures included change in central foveal thickness (CFT) in 1 mm by spectral-domain optic coherence tomography, and best corrected visual acuity (BCVA) at month 12. Complications after injections were recorded. The intra-group changes in CFT and BCVA were compared with Wilcoxon signed rank test, the between-group difference compared with Wilcoxon rank sum test. Fisher’s exact test was used for categorical comparison between groups. Seventy-eight eyes of 78 patients were collected. There were 42 eyes in bevacizumab group, with mean age of 53.2 ± 5.4 years and 27 female patients of them. The mean BCVA significantly improved from baseline 0.56 ± 0.35 logMAR to 0.35 ± 0.35 logMAR at Month 12 after bevacizumab treatment (p < 0.001). The mean CFT significantly decreased from baseline 315.3 ± 25.6 μm to 253.7 ± 24.4 μm at Month 12 following intravitreal bevacizumab (p < 0.001). There were 36 eyes in aflibercept group, with mean age of 52.8 ± 6.8 years and 24 female patients of them. The mean BCVA significantly improved from baseline 0.61 ± 0.47 logMAR to 0.38 ± 0.41 logMAR at Month 12 after aflibercept treatment (p < 0.001). The mean CFT significantly decreased from baseline 328.2 ± 19.8 μm to 241.8 ± 27.2 μm at Month 12 following intravitreal aflibercept (p < 0.001). The baseline demographics, lens status, axial length, refractive errors, duration of symptoms, BCVA, and CFT did not differ significantly between groups (p > 0.05). There was no significant difference between bevacizumab and aflibercept groups in BCVA and CFT from Month 1 to Month 12 (p > 0.05). Injection number of aflibercept was 2.11 ± 0.41, less than that of bevacizumab (3.23 ± 0.38) during 12-month period (p = 0.01). There were no systemic thromboembolic event, elevated intraocular pressure, retinal detachment, or infectious endophthalmitis following injections in both groups. We concluded that both aflibercept and bevacizumab can effectively treat choroidal neovascularization in high myopes. Intravitreal aflibercept had similar efficacy but less treatment number than bevacizumab for mCNV during 12-month period.

Purpose: To compare the two-year visual and anatomical outcomes of combination therapy of photodynamic therapy (PDT) with intravitreal aflibercept (IVA) or intravitreal ranibizumab (IVR) for patients with polypoidal choroidal vasculopathy (PCV), and to investigate the clinical factors with final visual outcome and retreatment. Methods: A retrospective medical chart review was performed for 55 eyes from 55 patients with PCV treated by a combination therapy of prompt PDT with either IVA (n = 30) or IVR (n = 25). Baseline data and treatment outcomes during the 24-month follow-up were compared between the two groups. Primary outcomes were the changes in best-corrected visual acuity (BCVA) and the rate of complete polyp regression. Secondary outcomes were the changes in central retinal thickness (CRT) and the rate of dry macula. Retreatment was administered in cases with persistent or recurrent submacular or intramacular fluid. Results: The BCVA significantly improved in the IVA/PDT group at every 6-month visit compared to the baseline. In the IVR/PDT group, there was a significant improvement of BCVA only at 6-months and 12-months, but not at 18-months and 24-months compared to the baseline. There were no significant differences in the BCVA change or CRT change between the two groups at every 6-month visit. A complete polyp regression rate at 3-months was 53.3% in IVA/PDT, and 52.0% in IVR/PDT. Significantly higher dry macula rate in Month 6 and 18 in the IVA/PDT group than in IVR/PDT group. Retreatment was performed in 26.7% patients in IVA/PDT, and in 60.0% patients in the IVR/PDT group. There were significantly lower retreatment rates in the IVA/PDT group than those in the IVR/PDT group. Better final BCVA was associated with better baseline BCVA and a younger age. Retreatment was associated with complete polyp regression at 3-months. Conclusions: Significant visual improvement was demonstrated in the IVA/PDT group at every 6-month visit, but only at a 6-month and a 12-month follow-up in the IVR/PDT group. Although changes of the BCVA/CRT and complete polyp regression rate were comparable between two groups, the IVA/PDT group required less retreatment and attained more dry macula results. Better baseline BCVA and younger age were associated with a better visual outcome.

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Neuromyelitis optica (NMO) is an autoimmune demyelinating disease with pathogenic autoantibodies that act against the astrocyte water channel protein, i.e. aquaporin-4: the disease is associated with recurrent episodes of optic neuritis (ON) and transverse myelitis, often resulting in severe disability. The main goals in treatment of NMO include acute symptomatic therapy and long-term stabilization of symptoms by preventing relapse. In recent years, ongoing randomized controlled trials in NMO patients have studied evidence for treatment. Briefly, acute-stage management (with pulse therapy using corticosteroids and/or plasmapheresis) and maintenance therapy (including rituximab, mycophenolate mofetil, and azathioprine) have been recommended in some case series and retrospective studies. Because of the high prevalence of liver disease, all NMO patients in Taiwan should be screened for hepatitis B and C before treatment is initiated. Although immunosuppression and plasma exchange are the mainstays of therapy for NMO ON, several selective and potentially therapeutic strategies targeting specific steps in NMO pathogenesis including blockers of NMO-IgG binding and inhibitors of granulocyte function have been evaluated in recent years.