This study evaluated the efficacy and safety of limited-dose eculizumab as rescue therapy for acute neuromyelitis optica spectrum disorder (NMOSD) attacks refractory to first-line treatment. Eight patients (six aquaporin-4 antibody seropositive and two seronegative) with incomplete responses to intravenous methylprednisolone with or without plasma exchange received one to four weekly doses of eculizumab (900 mg). Six patients with severe attacks (median nadir Expanded Disability Status Scale/Visual Outcome Scale: 9.0/11.5) showed only minimal improvement after conventional therapy. The remaining two, although not meeting severe criteria, also responded poorly. Add-on limited-dose eculizumab produced substantial neurological recovery: in myelitis, median Expanded Disability Status Scale improved from 8.5 to 3.5 at three months; in optic neuritis, median Visual Outcome Scale improved from 8.5 to 5.0. The proportion achieving marked-to-moderate improvement rose from 14.3% at one month to 85.7% at three months. Both seronegative patients responded favorably. Treatment was well tolerated, with no serious adverse events. These findings indicate that limited-dose eculizumab is an effective and safe option for accelerating recovery in refractory acute NMOSD attacks and may serve as a practical bridging strategy to long-term immunosuppression.

Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune condition driven by aquaporin-4 immunoglobulin G (AQP4-IgG). The current treatment paradigm focuses on mitigating clinical relapses and disability accumulation, leaving the underlying serological activity unaddressed. This study evaluated the capacity of satralizumab to achieve a comprehensive remission, as defined by the Serological, Relapse, and Accumulated-disability Remission (SERA-3) target. In this multicenter, real-world cohort study, patients with NMOSD from three tertiary centers in China initiating satralizumab were enrolled. AQP4-IgG levels were measured at baseline, 6, and 12 months. Clinical efficacy [annualized relapse rate (ARR), Expanded Disability Status Scale (EDSS)] and safety were evaluated. Heterogeneity in antibody response was analyzed, and baseline characteristics potentially associated with titer reduction were explored. Of the 19 patients who were AQP4-IgG seropositive at baseline, 7 (36.84

Background and aimRepulsive guidance molecule-A (RGMa) is well-known for its roles in T-cell-mediated neuroinflammation and CNS repair. Innate-adaptive immune interactions, particularly between neutrophils and Th17 cells, are emerging as critical drivers of neuroinflammatory pathology in multiple sclerosis (MS). Whether RGMa regulates these neutrophil-associated Th17 responses remains largely unknown. Elucidating this mechanism may reveal novel therapeutic targets for MS.MethodsSerum levels of RGMa, BMP4, neutrophil elastase (NE), IL-17A, and inflammatory markers were measured in 10 relapsing-remitting MS patients during acute relapse and remission phases, and in 10 healthy controls. In vitro transwell co-cultures using primary human neutrophils and CD3+ T cells were employed to evaluate the effects of RGMa monoclonal antibody (RGMa-mAb) treatment on neutrophil activation, Th17-associated responses, and T-cell migration. In parallel, experimental autoimmune encephalomyelitis (EAE) mice were treated with RGMa-mAb to assess neuroinflammation and BMP4-SMAD-related signaling changes in vivo.ResultsSerum RGMa and NE were elevated in acute-phase MS patients versus both remission and healthy controls (p < 0.05), whereas IL-17A was elevated versus healthy controls (p < 0.05). Correlation analyses showed that RGMa levels were positively associated with the neutrophil activation marker NE (r = 0.71), while serum Ly6G6D levels were correlated with IL-17A levels (r = 0.66), suggesting a potential link between neutrophil-associated inflammatory responses and Th17 activation during active disease. In vitro, RGMa-mAb treatment attenuated PMA-induced neutrophil activation, reduced Th17-associated inflammatory responses, and suppressed T-cell migration in the co-culture system. In EAE mice, RGMa-mAb ameliorated neurological deficits, decreased neutrophil- and Th17-associated inflammatory markers, and modulated BMP4-SMAD signaling.ConclusionRGMa contributes to neutrophil-associated Th17 inflammatory responses in CNS autoimmunity, potentially in association with BMP4-SMAD signaling. Targeting RGMa may therefore represent a potential therapeutic strategy for MS-associated neuroinflammation that warrants further investigation.

Neuromyelitis optica spectrum disorder (NMOSD) patients with refractory attacks, despite standard therapy with high-dose intravenous methylprednisolone (IVMP) and plasma exchange (PLEX), often experience significant disability. While complement C5 inhibition has established efficacy in relapse prevention, its role in acute-phase management remains unclear. In this single-center, retrospective case series, eight patients (6 AQP4-IgG seropositive, 2 seronegative) with acute NMOSD and suboptimal responses to IVMP ± PLEX were treated with 1–4 weekly doses of ECU (900 mg per dose). The primary outcome was the change in functional scores (EDSS for myelitis and logMAR visual acuity (VA) of the worse eye for optic neuritis) from pre-ECU baseline to 1 and 3 months post-ECU. The secondary outcomes included the proportion of patients achieving good improvement, and safety. Six patients presented with severe, refractory attacks (median nadir EDSS/VA: 9.0/2.5) showing minimal response to conventional therapy (median post-IVMP/PLEX EDSS/VA: 8.75/2.5). The remaining two patients, while not meeting the criteria for a severe attack, also had an unsatisfactory response to first-line treatment. Add-on limited-dose ECU enhanced neurological function. In myelitis patients, the median EDSS score improved from 8.5 at baseline to 3.5 at 3 months. In optic neuritis patients, the median VA score improved from 1.9 to 0.5. The proportion of patients achieving good response increased from 42.9

Objectives: To explore the potential of RGMA gene polymorphisms as novel biomarkers for predicting disease activity in NMOSD. Methods: We enrolled 117 NMOSD patients and 100 healthy controls. Single nucleotide polymorphism genotyping for the RGMA gene was performed using Sanger sequencing. Associations between RGMA gene polymorphisms and clinical and imaging characteristics, and immune cell activation were analyzed. Results: Carriers of the rs725458-CC and rs4778099-AA genotypes experienced significantly earlier first relapses, but subsequently showed a lower relapse rate. The rs4778099-GG genotype was associated with a higher rate of AQP4-IgG seronegativity, while the rs4778099-AA genotype correlated with a higher likelihood of presenting circumventricular organ syndrome at onset. Carriers of the rs725458-TT genotype were more prone to longer spinal lesion spans. Elevated levels of CD3+T lymphocytes were observed in carriers of the rs725458-TT and rs4778099-GG genotypes during acute phases. Survival analysis revealed that the CC/AA genotypes were linked to earlier relapse, supported by Cox multivariate analysis which identified these genotypes, along with age of onset and onset symptoms, as key predictors of early relapse. Conclusion: RGMA gene polymorphisms, specifically rs725458-CC and rs4778099-AA, are key predictors of early NMOSD relapse. Integrating these markers with clinical data improves relapse risk prediction, enabling more targeted treatment strategies.

Background Severe disabling attacks neuromyelitis optica spectrum disorders (NMOSD) severely affect patients' quality of daily life and life safety. Methods This retrospective study enrolled consecutive Chinese patients suffering from NMOSD who visited the China-Japan Friendship Hospital (Beijing, China) between October, 2010 and February, 2023. Correlation analysis was used to perform feature selection. The prediction model was constructed using the support vector machine (SVM) and extreme gradient boosting (XGBoost) algorithm. Results A total of 356 patients (mean [SD] age, 34.45[15.22] years) and 1291 NMOSD attacks were eligible for this study. ON and age were positively and linearly correlated, and TM and age were negatively and linearly correlated. Throbbing headache and neuralgia showed a significant linear relationship with disabling episodes, and circumventricular organ- area postrema syndrome (CVO-APS) showed a significant linear relationship with disabling episodes only in a few cases. We select top three high correlation variable (HCV) for predicting ON and TM models. Then, we constructed prediction models based on the XGBoost using age as a feature, and HCV and warning symptoms (WS) as features respectively. The ML models showed reasonable predictive performance for ON (Age + WS: AUC, 0.809; Age + HCV: AUC, 0.787) and TM (Age + WS : AUC, 0.817; Age + HCV: AUC, 0.854) We also constructed the first prediction model about severe NMOSD attacks using XGBoost and SVM. Among them, the fundamental model incorporated the 16 features with AUC of Xgboost: 0.830 and SVM: 0.775. On the basis of the fundamental model, we also incorporated the results of nadir and remission the expanded disability status scale (EDSS) or visual outcome scale (VOS) from the last time attack as features to construct 12 optimized models. As a whole, the optimized models showed higher predictive performance for severe attack. The AUC for the model adding the nadir EDSS scores as features were XGBoost: 0.862, SVM: 0.741, adding the nadir VOS scores as features were XGBoost: 0.870, SVM: 0.723, adding the remission EDSS scores as features were XGBoost: 0884, SVM: 0.806, adding the remission VOS scores as features were XGBoost: 0.9998, SVM: 0.700, adding both nadir and remission EDSS scores as features were XGBoost: 0.899, SVM: 0.794, adding both nadir and remission VOS scores as features were XGBoost: 0.905 and SVM: 0.705. Conclusion This study innovatively identified associations between disabling attacks of NMOSD and age, warning symptoms and developed an easy-to-use, less costly and less invasive machine learning model for predicting NMOSD disabling attack symptoms and severe attacks.

Neuromyelitis optica spectrum disorder (NMOSD) is a disabling autoimmune disease. Neutrophil activation plays a crucial role in the neuroinflammatory damage observed during disease exacerbations. This study aims to elucidate the potential role of the repulsive guidance molecule A-bone morphogenetic protein 4 (RGMa-BMP4) signaling pathway in neutrophil activation during NMOSD attacks. We employed transcriptomic sequencing, quantitative PCR, flow cytometry, and Western blot analysis on peripheral blood samples from NMOSD patients in acute and remission phases. Additionally, an NMO rat model was established to investigate in vivo molecular mechanisms, focusing on key signaling molecules, including RGMa, BMP4, and SMADs. Transcriptomic analysis identified five genes associated with NMOSD pathogenesis or neutrophil activation, with RGMA, EGFR, and HLA-DOB showing the most significant differences. RT-qPCR confirmed elevated levels of RGMA, BMP4, and SMADs in the acute phase. Flow cytometry and Western blotting demonstrated an increased nuclear-to-cytoplasmic ratio of SMAD4 protein in neutrophils from acute-phase NMOSD patients. In the NMO rat model, we observed significant upregulation of RGMA, BMP4, and SMAD4 mRNA in brain and spinal cord tissues, along with enhanced nuclear translocation of SMAD4 protein. Furthermore, there was a marked increase in myeloperoxidase (MPO) mRNA expression, a marker of neutrophil activation, in both brain and spinal cord tissues in the model group. Our findings indicate that the RGMa-BMP4 signaling pathway likely plays a key role in neutrophil-mediated neuroinflammation during NMOSD attacks. These results suggest potential therapeutic targets within this pathway, warranting further investigation into their clinical implications.

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Objectives To investigate the impact of plasma exchange (PLEX) on NETosis-related regulators and their correlation with neurological improvement in NMOSD patients. Methods Twelve aquaporin-4 antibodies seropositive NMOSD patients were enrolled. NETosis-related regulators (myeloperoxidase [MPO], citrullinated histone H3 [CIT-H3], peptidyl arginine deiminase 4 [PAD4], neutrophil elastase [NE], CD64), pro-inflammatory cytokines (IL-1, IL-6, IL-12, TNF-α) and anti-inflammatory cytokines (IL-10, TGF-β1) were quantitatively assessed before and after PLEX treatment. Clinical assessments included expanded disability status scale (EDSS) and visual outcome scale (VOS) scores. Results Following PLEX, all patients showed symptom improvement, with 66.7 % achieving marked-to-moderate improvement (MMI) at 3 months. Key regulators, such as MPO, CIT-H3, PAD4, NE, and pro-inflammatory cytokines such as IL-1, IL-6, IL-12, and TNF-α, exhibited a statistically significant decrease immediately after the initial PLEX session (P < 0.05). Furthermore, CD64 levels demonstrated a substantial decline after the second PLEX session (P < 0.05). Conversely, the levels of anti-inflammatory cytokines, including IL-10 and TGF-β1, displayed an ascending trend post-PLEX. In clinical relevance analysis, among patients who reached MMI, the reductions in MPO, IL-1, and IL-6 exhibited statistically significant differences when compared to patients in the mild-to-no improvement group (P < 0.05). Pearson correlation analysis revealed that the percentage reduction in IL-6 levels after PLEX was positively correlated with the percentage reduction in patient EDSS/VOS scores (r = 0.638, P < 0.05). Conclusions This study highlights that reduced levels of NETosis-related regulators after PLEX contribute to clinical improvement, suggesting the potential involvement of NETosis in the acute neurological impairment observed in NMOSD.

BackgroundRituximab (RTX), an anti-CD20 monoclonal antibody, has shown promise in managing neuromyelitis optica spectrum disorders (NMOSD) by depleting B cells and reducing relapses. However, there is no consensus on the optimal RTX dosing regimen, and genetic factors, such as FCGR3A-V158F polymorphism, may influence treatment outcomes. This study investigates how FCGR3A-V158F genotypes influence RTX efficacy in Chinese NMOSD patients under varying dosing regimens and aims to optimize treatment protocols.MethodsWe conducted a retrospective analysis of 25 Chinese NMOSD patients treated with RTX, grouped into standardized and low-dosage regimens. FCGR3A-V158F genotypes were determined, and treatment responses were evaluated, including relapse rates, time to first relapse (TFR), B-cell depletion, dose adjustments, and treatment retention.ResultsAmong all patients, 15 received standardized dosages, while 10 received varied induction doses (500 mg to 1200 mg) in low-dose regimens. For FCGR3A-V158F genotypes, 15 had the FF genotype, and 10 were V carriers (3 VV genotype, 7 VF genotype). Regardless of dosing, FF genotype patients had a higher relapse rate post-RTX treatment compared to V carriers (P < 0.05). None of the 3 VV genotype patients in either dose group experienced relapses post-RTX. In both dose groups, FF genotype patients had significantly shorter TFR and required more RTX dose adjustments post-RTX treatment compared to V carriers in the standardized dosage group (P < 0.05). FF genotype patients in the low dosage group were more likely to experience insufficient B-cell depletion, had lower treatment retention rates, and more discontinuations than V carriers in the standardized dosage group (P < 0.05). Insufficient B-cell depletion significantly predicted clinical relapses after RTX treatment (P < 0.05). In survival analysis, FF genotype patients, regardless of dosing, experienced earlier relapses post-RTX treatment (P < 0.05).ConclusionsThis study highlights the importance of RTX dosage selection in NMOSD treatment, particularly for FCGR3A-FF genotype patients. Standard-dose RTX therapy with vigilant monitoring of peripheral blood B-cell levels is recommended for these individuals to optimize treatment efficacy.

Background: Neuromyelitis optica spectrum disorder (NMOSD) is a devastating autoimmune disorder with cycles of escalating relapse. Rates of diagnosis in the elderly are increasing. Therapeutic decision-making is more challenging in elderly patients due to multiple comorbidities and high risk of drug-induced side effects. Objective: This retrospective study assessed the efficacy and safety of standard plasma exchange (PLEX) treatment in an elderly population with NMOSD. Design: Seventy-six patients with NMOSD who received PLEX were apportioned to two groups as either elderly (⩾60 years, n = 26) or young (<60 years) at the time of the first procedure. Methods: Therapeutic response was judged according to functional recovery at 6 months, as reflected by Expanded Disability Status Scale (EDSS) and visual outcome scale (VOS) scores. Results: The mean age of the 26 elderly patients was 67.7 ± 7.9 years (range 60–87 years); the population was predominantly female (88.5%). PLEX sessions were generally well tolerated among the elderly. Compared with the young patients, the elderly had significantly more comorbidities and concomitant medications. Twenty-four (96.0%) elderly patients showed functional improvement at 6 months after PLEX, of which 15 (60.0%) experienced moderate-to-marked improvement. Six months after the initial PLEX treatment, the patients overall experienced a significant improvement in EDSS and VOS scores. Logistic regression showed that severe optic neuritis attack was a significant independent prognostic factor associated with poor PLEX response. The groups were comparable regarding overall or serious adverse events. The rate of transient hypotension was significantly higher in the elderly compared with the young. Conclusion: PLEX is an effective and safe therapy for elderly patients with NMOSD and should be considered a treatment option during NMOSD attacks. In the elderly, preventive measures against hypotension are recommended before PLEX.

目的:探讨血浆置换治疗超晚发型视神经脊髓炎谱系疾病(VLONMOSD)的有效性和安全性.方法:回顾分析中日友好医院2013年11月～2019年11月收治的3例确诊为视神经脊髓炎谱系疾病(NMOSD)的超晚发型患者(发病年龄≥75岁)应用血浆置换(PE)的疗效与安全性.结果:3例患者中2例女性,1例男性,首次发病年龄分别为78岁、77岁、76岁,PE年龄分别为82岁、77岁、76岁,PE平均年龄78.3岁.共计4次发作应用PE治疗,其中3次为横贯性脊髓炎(TM)发作,1次为视神经炎(ON),例2的ON发作和例3的1次TM发作首选静脉滴注甲泼尼龙冲击无效后给予PE挽救治疗.3次TM发作经治疗后症状改善,例1的第二次TM复发症状显著改善,第一次TM复发症状轻度改善;例3的TM发作治疗后症状轻度改善,例2的1次ON发作经治疗后视力无明显改善.PE的不良反应依次为:血压下降、肝素相关性血小板计数减少,均为短暂且可逆性,未发生导致治疗终止的严重不良反应.结论:PE可作为VLONMOSD患者的首选或补救治疗措施,安全性基本良好.

Background:The area postrema syndrome (APS) is a unique diagnostic criterion for neuromyelitis optica spectrum disorders (NMOSD). However, APS has rarely been reported in cases of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). Case presentation:A 36-year-old woman presented with APS and clinical features of diffuse central nervous system involvement during the early stage of the disease. Owing to the absence of serum aquaporin 4 antibodies, she was initially misdiagnosed as a case of seronegative NMOSD. However, the distinct neuroimaging characteristics [symmetrical small punctuate gadolinium enhancing lesions (pepper-like)], typical clinical/radiological relapse, and intense steroid-dependence in this case, prompted us to correct the diagnosis as probable CLIPPERS. To prevent relapse, long-term oral steroids and an immunosuppressive agent were administered. Conclusions:CLIPPERS may present as APS, and should be considered in the differential diagnosis of NMOSD.

To clarify the existence of monophasic neuromyelitis optica spectrum disorders (NMOSD) and to identify predictive factors of long-term relapse-free form. We retrospectively analyzed 289 Chinese patients with NMOSD. Selected subjects were divided into three groups based on the time interval between disease onset and the first relapse, if any. Clinical and imaging data were acquired from each patient’s medical record and evaluated as predictive factors for NMOSD. In total, none of the participating patients exhibited a monophasic form of NMOSD. Rather, 241 patients were selected for relapse tendency analysis; 143 (59.3%) patients relapsed within the first year, 66 (27.4%) during 1–5 years, and 32 (13.3%) beyond 5 years. Such onset symptoms as optic neuritis (ON) and non-longitudinally extensive transverse myelitis (LETM) were independent prognostic factors for a prolonged remission interval (P < 0.05). The relapse rate was bi-modal for ON patients in the first year (47.9%) and beyond 5 years (24.0%) after disease onset, respectively. However, most TM and area postrema syndrome (APS) patients experienced an attack within the first year (61.3% for TM and 76.9% for APS). A survival analysis showed that attacks with APS (P < 0.0001) and TM (P < 0.05) have a significantly higher risk of early relapse than with ON and that seropositive aquaporin-4 antibody may shorten the relapse interval for all onset symptoms (P < 0.0001). Our study indicated that the monophasic form of NMOSD may not exist when a sufficient follow-up period is considered. Onset phenotypes with ON, non-APS, or non-LETM attacks had a lower risk of early relapse.

Recently, neuromyelitis optica spectrum disorders (NMOSD) appear to be a multi-organ disorder, however, the involvement of myocardium in NMOSD is extremely rare. In the present article, we present a young girl who manifested bilateral optic neuritis, area postrema syndrome, brainstem syndrome and transverse myelitis, as well as tachycardia, abnormal electrocardiograph, moderate elevation of myocardial biomarker and regional wall movement abnormalities, which confirmed the diagnosis of Takotsubo cardiomyopathy associated with NMOSD. The neurological deficits along with myocardial injury were recovered soon after the administration of intravenous methylprednisolone and intravenous immunogloblin. This is a rare case that should be paid attention and by which can not only broaden the symptom spectrum of NMOSD, but can also provide novel visions for further investigating the mechanism of organs damage.

This study aimed to investigate the underlying pathological muscle damage in neuromyelitis optica spectrum disorder (NMOSD) patients without muscular symptoms. We prospectively enrolled 15 patients with aquaporin 4 (AQP4) antibody seropositive NMOSD and 16 patients with non-NMOSD diseases as a control group. Biceps biopsy samples from 18 patients were examined. Six NMOSD patients exhibited inflammatory lesions/edema in lower muscles on muscle MRI. On histopathological examination, NMOSD samples showed significantly decreased IgG-targeting AQP4 expression on sarcolemma compared with non-NMOSD samples in terms of the area of positive staining and integrated optical density. Muscle biopsy can support the differential diagnosis of NMOSD.

OBJECTIVE:To explore the clinical, electrophysiological and imaging features of a patient with Krabbe disease caused by GALC mutation.METHODS:A comprehensive analysis including clinical investigation and genetic testing was carried out.RESULTS:The patient presented with peripheral neuropathy with electrophysiological anomaly suggestive of asymmetric demyelinating neuropathy. Brain imaging revealed leukoencephalopathy. Genetic analysis has identified compound heterozygous mutations in exons 5 and 11 of the GALC gene, namely c.461C>A and c.1244G>A.CONCLUSION:Krabbe disease is a group of disorders featuring substantial phenotypic heterogeneity. Genetic and enzyme testing has become indispensable for accurate diagnosis for this disease.

Neuromyelitis optica (NMO) spectrum disorder (NMOSD) is a devastating autoimmune inflammatory disorder of the central nervous system, which can result in blindness or paralysis. Currently, there is a dire need for new treatment options in the clinic. Several case series have shown that mycophenolate mofetil (MMF) may be an effective treatment for NMOSD patients. The dosing of MMF in the treatment of NMOSD has been poorly studied. Therefore, we evaluated the efficacy, tolerability, influential factors and optimal dosage of MMF in Chinese patients with NMOSD.

Purpose: The purposes of this article were to evaluate the short-term outcome of plasma exchange (PLEX) for neuromyelitis optica spectrum disorders (NMOSDs) in Chinese patients and to identify the factors predictive of a favorable response to therapy. Methods: We retrospectively analyzed data from 29 Chinese patients with NMOSD. All patients received 2 to 7 sessions of PLEX every other day. Expanded Disability Status Scale (EDSS) scores were estimated at baseline, at relapse, and before and at follow-up after PLEX. Patients were assigned to 1 of 2 groups according to treatment responses of marked to moderate improvement and mild to no improvement. Findings: Twenty-four of 29 patients (82.8%) showed functional improvement at 1 month after PLEX, 9 of whom experienced moderate to marked improvement. Early PLEX initiation and a lower baseline EDSS score were independent prognostic factors (both, P < 0.05). In addition, relapse symptoms of nonoptic neuritis and acute transverse myelitis plus circumventricular organs, seronegativity' for aquaporin-4 antibodies, shorter initial therapy PLEX interval, and no prior optic neuritis attacks were predictive factors significantly associated with a favorable response to treatment (all, P < 0.05). The delay time pre-PLEX was inversely correlated with reduction in EDSS score. The percentage reductions in EDSS score in groups receiving PLEX on days <= 15 and days 16 to 30 were significantly greater than those in the groups treated on days 31 to 60 and days 61 to 90 (all, P < 0.05). Most PLEX sessions were generally well tolerated. (C) 2018 Elsevier HS Journals, Inc. All rights reserved.

To facilitate the diagnosis of anti-NMDAR encephalitis presenting with brain lesions in unconventional locations (BLUL) on MRI, we retrospectively analyzed forty-five Chinese patients. Eighteen (40.0%) of their MRI initially exhibited one or more BLUL. These locations predominantly included cerebral gray matter (cortex, basal ganglia and thalamus), as well as white matter and brainstem. Due to these BLUL, thirteen (72.2%) patients were originally misdiagnosed with other diseases and developed poor clinical and imaging outcomes. Therefore, anti-NMDAR encephalitis has unpredictable MRI findings that easily obscure its diagnosis and cause serious sequelae. Anti-NMDAR antibody tests are highly recommended in patients with BLUL.