Journal of Central Nervous System Disease最新文献

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Construction and Validation of a Risk Prediction Model Incorporating Temporal Muscle Thickness for Adverse Outcome in Acute Ischemic Stroke Patients. 考虑颞肌厚度的急性缺血性脑卒中不良后果风险预测模型的构建与验证。
IF 2.4
Journal of Central Nervous System Disease Pub Date : 2026-07-05 eCollection Date: 2026-01-01 DOI: 10.1177/11795735261466349
Huanpeng Wang, Yanchun Wu, Xiaojia Wu, Shuyan Su, Ziting Peng, Minping Lin, Xiaoqin Xu, Dongli Chen, Hong Zhang, Ruibin Huang
{"title":"Construction and Validation of a Risk Prediction Model Incorporating Temporal Muscle Thickness for Adverse Outcome in Acute Ischemic Stroke Patients.","authors":"Huanpeng Wang, Yanchun Wu, Xiaojia Wu, Shuyan Su, Ziting Peng, Minping Lin, Xiaoqin Xu, Dongli Chen, Hong Zhang, Ruibin Huang","doi":"10.1177/11795735261466349","DOIUrl":"10.1177/11795735261466349","url":null,"abstract":"<p><strong>Background: </strong>Sarcopenia significantly impacts stroke prognosis. Temporal muscle thickness (TMT) is an emerging metric for sarcopenia.</p><p><strong>Objectives: </strong>To developed a TMT-incorporated model to predict 6-month adverse outcomes in acute ischemic stroke (AIS).</p><p><strong>Design: </strong>In this retrospective study, 479 AIS patients were divided into training (n=283), test (n=120), and external validation cohorts (n=76).</p><p><strong>Methods: </strong>A combined model was constructed to predict adverse outcomes in the training and test cohorts using LASSO regression analysis. Model performance was assessed via calculating accuracy, sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV) and F1 score.</p><p><strong>Results: </strong>The proportion of patients with an adverse outcomes in the training and test sets was 18.02% vs 17.50%, respectively (<i>P</i> = 0.90). Ischemic stroke event, admission NIHSS score, BI score, TMT were used to construct the prediction model. The combined model presented good discriminatory potential in the training and test sets (AUC = 0.929 and 0.930, respectively), which was verified in the external validation cohort (AUC = 0.902). For the combined model, the <i>P</i> values of the Hosmer-Lemeshow test in the training set, the test set, and the external validation were < 0.001 (χ2 = 44.007), 0.472 (χ2 = 7.611), and <0.001 (χ2 = 4919.666), respectively. The combined model showed good calibration and discrimination. The clinical usefulness of the model was confirmed by decision curve analysis.</p><p><strong>Conclusion: </strong>This study developed a combined model incorporating ischemic stroke event, admission NIHSS score, BI score, TMT and infarct volume to predict 6-month adverse outcomes in AIS patients, providing clinicians with a practical tool for treatment decisions and prognosis assessment.</p>","PeriodicalId":15218,"journal":{"name":"Journal of Central Nervous System Disease","volume":"18 ","pages":"11795735261466349"},"PeriodicalIF":2.4,"publicationDate":"2026-07-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13334075/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148436570","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Correlation Is Not Prediction: Reassessing Predictive MRI Evidence in Guidelines for Persons With Relapsing-Remitting Multiple Sclerosis. 相关性不是预测:重新评估复发缓解型多发性硬化症患者指南中的预测性MRI证据。
IF 2.4
Journal of Central Nervous System Disease Pub Date : 2026-06-25 eCollection Date: 2026-01-01 DOI: 10.1177/11795735261453967
Dulat Minas, Stefan Buchka, Joachim Havla, Ulrich Mansmann
{"title":"Correlation Is Not Prediction: Reassessing Predictive MRI Evidence in Guidelines for Persons With Relapsing-Remitting Multiple Sclerosis.","authors":"Dulat Minas, Stefan Buchka, Joachim Havla, Ulrich Mansmann","doi":"10.1177/11795735261453967","DOIUrl":"10.1177/11795735261453967","url":null,"abstract":"<p><strong>Background: </strong>Effective treatment monitoring and treatment decisions in relapsing-remitting multiple sclerosis (RRMS) require accurate and individualized prediction of future disease courses. Guidelines from the Magnetic Resonance Imaging in Multiple Sclerosis (MAGNIMS) group and the Canadian Multiple Sclerosis Working Group (CMSWG) frequently cite MRI outcomes as predictive, but the methodological quality of this evidence is uncertain.</p><p><strong>Objectives: </strong>This study aims to critically assess the methodological standards underlying predictive claims about MRI outcomes in four major relevant MS guidelines.</p><p><strong>Design: </strong>We conducted a content review of citations in the MAGNIMS 2015 and 2021 and the CMSWG 2013 and 2020 guideline publications.</p><p><strong>Methods: </strong>Each source was evaluated for whether it reported quantitative predictive evidence: either predictive values with confidence intervals, Kaplan-Meier-based risk estimates, or externally validated models that provide accurate risk estimates (good calibration) and correctly separate high- from low-risk patients (good discrimination); We also checked if measures such as correlations, odds ratios, hazard ratios, Prentice criteria, or likelihood ratio tests were used.</p><p><strong>Results: </strong>Across all four guidelines, most predictive statements relied on secondary citations and association-based measures. Odds ratios, hazard ratios, correlations, or Prentice criteria were commonly reported. Some studies reported predictive values, but confidence intervals were frequently not provided. Only isolated examples of properly validated prediction models were cited, and only one had undergone full external validation. Advanced methods, such as the likelihood reduction factor, were absent.</p><p><strong>Conclusion: </strong>Current guideline statements on MRI prediction in RRMS often rely on associations rather than validated individualized predictions. They do not quantify individual risk or provide evidence for accuracy, calibration, discrimination, or robustness (reliability of predictions across different patients and settings). To ensure trustworthy and actionable evidence, future guidelines should require prospective risk estimates with confidence intervals, externally validated models with calibration and discrimination, predefined thresholds for predictive usefulness, and evaluation of clinical utility (e.g., decision curve analysis).</p>","PeriodicalId":15218,"journal":{"name":"Journal of Central Nervous System Disease","volume":"18 ","pages":"11795735261453967"},"PeriodicalIF":2.4,"publicationDate":"2026-06-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13305512/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148345404","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Beyond Time Is Brain: The Time, Tissue, and Systems Framework for Acute Stroke. 超越时间的是大脑:急性中风的时间、组织和系统框架。
IF 2.4
Journal of Central Nervous System Disease Pub Date : 2026-06-14 eCollection Date: 2026-01-01 DOI: 10.1177/11795735261451033
Sonu Bhaskar
{"title":"Beyond Time Is Brain: The Time, Tissue, and Systems Framework for Acute Stroke.","authors":"Sonu Bhaskar","doi":"10.1177/11795735261451033","DOIUrl":"10.1177/11795735261451033","url":null,"abstract":"<p><p>Acute ischaemic stroke care has long been guided by the maxim \"time is brain,\" yet outcome variability suggests that clock time alone is insufficient. We propose the Time, Tissue, and Systems (TTS) framework, a quantitative model integrating temporal progression, tissue vulnerability, and health-system performance. Biological injury is conceptualized as accelerated neuronal loss scaled by tissue phenotype and systems friction, and clinical decision-making is reframed as net utility optimization. Illustrative modeling across four archetypal stroke profiles demonstrates how identical clock times yield divergent biological and functional trajectories. The TTS framework provides a coherent structure for precision stroke care, aligning biological time with system time.</p>","PeriodicalId":15218,"journal":{"name":"Journal of Central Nervous System Disease","volume":"18 ","pages":"11795735261451033"},"PeriodicalIF":2.4,"publicationDate":"2026-06-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13269984/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148271720","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Heterogeneity of Microglia in Ischemic Stroke from the Perspective of Single-Cell RNA Sequencing: Subset Characteristics, Mechanisms and Therapeutic Potential. 从单细胞RNA测序的角度看缺血性脑卒中小胶质细胞的异质性:亚群特征、机制和治疗潜力。
IF 2.4
Journal of Central Nervous System Disease Pub Date : 2026-05-16 eCollection Date: 2026-01-01 DOI: 10.1177/11795735261452744
Daman Tian, Jiao Yang, Zhifeng Wang, Junfeng Lan, Ting Shi, Shuangfeng Xu, Manyan Zhao, Pengfen He, Jian Wang, Liwei Xing
{"title":"Heterogeneity of Microglia in Ischemic Stroke from the Perspective of Single-Cell RNA Sequencing: Subset Characteristics, Mechanisms and Therapeutic Potential.","authors":"Daman Tian, Jiao Yang, Zhifeng Wang, Junfeng Lan, Ting Shi, Shuangfeng Xu, Manyan Zhao, Pengfen He, Jian Wang, Liwei Xing","doi":"10.1177/11795735261452744","DOIUrl":"10.1177/11795735261452744","url":null,"abstract":"<p><p>Ischemic stroke (IS), a predominant cerebrovascular disorder contributing to global disability and mortality, characterizes by a complex, multi-tiered cascade of pathological processes. As the primary innate immune cells within the central nervous system (CNS), microglia exhibit dual functional characteristics following ischemic injury, switching dynamically between pro-inflammatory detrimental phenotypes and anti-inflammatory reparative phenotypes in response to temporal progression, cellular phenotypic transformation, and changes in the local microenvironment. Consequently, therapeutic strategies targeting microglia have garnered considerable research interest, challenging the traditional neuron-centric therapeutic approaches. Microglia display a wide range of phenotypes, and the traditional M1/M2 classification is overly simplistic, failing to capture the full spectrum of their functional diversity. In contrast, single-cell RNA sequencing (scRNA-seq) technology has surpassed the limitations of bulk sequencing, providing a robust tool for elucidating microglial heterogeneity. Recent studies utilizing animal models of stroke have identified several subsets distinct from the conventional M1/M2-like subsets, including but no limited to ischemic stroke-associated microglia (ISAM), <i>Prdx1</i> <sup>+</sup>SAM, <i>Spp1</i> <sup>+</sup>microglia and SAM-foamy. The mechanisms underlying microglial heterogeneity encompass innate programming influenced by genetic background, dynamic remodeling of epigenetic modifications, metabolic reprogramming in response to extrinsic microenvironmental stress, and intercellular interaction networks. This review systematically examines, from the perspective of scRNA-seq, the biological functions of MG, the heterogeneity observed throughout their CNS life cycle development and regional homeostasis, the characteristics and regulatory mechanisms of heterogeneous microglial subpopulations following ischemic stroke, and potential therapeutic strategies and associated challenges. The aim is to provide a reference for the development of precise therapeutic strategies for IS.</p>","PeriodicalId":15218,"journal":{"name":"Journal of Central Nervous System Disease","volume":"18 ","pages":"11795735261452744"},"PeriodicalIF":2.4,"publicationDate":"2026-05-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13180134/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147972726","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Machine Learning-Based Detection of Parkinson's Disease From Arabic Speech: A Cross-Linguistic Validation Study. 基于机器学习的帕金森病检测:一项跨语言验证研究。
IF 2.4
Journal of Central Nervous System Disease Pub Date : 2026-04-29 eCollection Date: 2026-01-01 DOI: 10.1177/11795735261448278
Ahmad B Hassanat, Ahmad S Tarawneh, Enas Al-Khlifeh, Alaa O Almahadin, Mansoor Alghamdi, Khalid Almohammadi, Malek Alrashidi, Lujain A Alhasanat, Ahmad H Al-Nawafleh
{"title":"Machine Learning-Based Detection of Parkinson's Disease From Arabic Speech: A Cross-Linguistic Validation Study.","authors":"Ahmad B Hassanat, Ahmad S Tarawneh, Enas Al-Khlifeh, Alaa O Almahadin, Mansoor Alghamdi, Khalid Almohammadi, Malek Alrashidi, Lujain A Alhasanat, Ahmad H Al-Nawafleh","doi":"10.1177/11795735261448278","DOIUrl":"10.1177/11795735261448278","url":null,"abstract":"<p><p>Early detection of Parkinson's disease (PD) through speech analysis offers significant clinical advantages, yet no validated tools exist for Arabic-speaking populations, representing a critical gap in global healthcare. Previous studies have relied on limited machine learning (ML) classifiers and voice attributes, which may introduce bias and hinder effective technique discovery. To address this, we developed an optimal PD prediction pipeline by testing multiple ML classifiers and feature extraction methods. We created the first Arabic PD speech dataset, comprising 40 subjects (17 with PD and 23 controls), and validated our methodology on an independent Spanish cohort of 100 subjects. Feature extraction included traditional, audio-to-text, and deep voice features from a pre-trained Whisper model. We employed feature selection and dimensionality reduction techniques to refine the dataset dimensions. Final features were assessed using twelve classifiers with leave-one-out and k-fold cross-validation for robust performance evaluation. Shapley additive explanations (SHAP) were utilized to determine feature importance as vocal biomarkers. Linear Discriminant Analysis achieved optimal performance with 90% accuracy, precision, recall, and F1-score using leave-one-out cross-validation. Linear Support Vector Classification also performed well, achieving 87.7% precision and 87.5% recall. When tested on the independent Spanish dataset, our methodology attained 83% accuracy, confirming cross-linguistic generalizability. SHAP analysis indicated that audio-to-text features provide contextual insights on fluency and coherence, while traditional features effectively capture acoustic variations. This study establishes the first validated Arabic PD speech classification system and demonstrates its universal applicability, laying the groundwork for global speech-based PD screening.</p>","PeriodicalId":15218,"journal":{"name":"Journal of Central Nervous System Disease","volume":"18 ","pages":"11795735261448278"},"PeriodicalIF":2.4,"publicationDate":"2026-04-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13145026/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147838440","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Depression, Anxiety, and Stress After Acquired Brain Injury: The Role of Cognitive Reserve in Patients and Caregivers 'Symptoms. 获得性脑损伤后的抑郁、焦虑和压力:认知储备在患者和护理者症状中的作用
IF 2.4
Journal of Central Nervous System Disease Pub Date : 2026-04-16 eCollection Date: 2026-01-01 DOI: 10.1177/11795735261443603
Debora Bertoni, Benedetta Basagni, Sara Mondini, Antonio De Tanti, Elisa Di Rosa
{"title":"Depression, Anxiety, and Stress After Acquired Brain Injury: The Role of Cognitive Reserve in Patients and Caregivers 'Symptoms.","authors":"Debora Bertoni, Benedetta Basagni, Sara Mondini, Antonio De Tanti, Elisa Di Rosa","doi":"10.1177/11795735261443603","DOIUrl":"10.1177/11795735261443603","url":null,"abstract":"<p><strong>Objectives: </strong>Severe acquired brain injury (sABI) can impact the psychological health not only in patients, but also in their caregivers. The present study was aimed at examining depression, anxiety and stress symptoms in patients with sABI and in their caregivers, exploring differences and correlations between the two, and investigating the role of cognitive reserve as potential predictor of patients and caregiver's psychological wellbeing.</p><p><strong>Methods: </strong>Fifty-four individuals (27 patients-caregiver's dyads) took part in the present study. All patients had a severe injury and data were collected approximately five years post-injury. During a telemedicine assessment, the Depression Anxiety Stress Scale (DASS-21), the Caregiver Burden Inventory (CBI) and the Cognitive Reserve Index questionnaire (CRIq) were administered.</p><p><strong>Results: </strong>Results show higher levels of anxiety and perceived stress in caregivers, when compared with patients. Significant associations emerged between patients and caregivers' symptoms of depression and anxiety. Furthermore, cognitive reserve emerged as a significant modulator of caregivers' emotional burden and depressive symptoms.</p><p><strong>Conclusion: </strong>Findings highlight the interconnection between patients' and caregivers' psychological wellbeing and suggest that cognitive reserve might be considered a predictor of caregivers' mental health.</p>","PeriodicalId":15218,"journal":{"name":"Journal of Central Nervous System Disease","volume":"18 ","pages":"11795735261443603"},"PeriodicalIF":2.4,"publicationDate":"2026-04-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13087322/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147722703","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
GLP-1 Receptor Agonists in Acute Ischemic Stroke and Secondary Stroke Prevention: A Narrative Review of Preclinical and Clinical Evidence. GLP-1受体激动剂在急性缺血性卒中和继发性卒中预防中的作用:临床前和临床证据的叙述性回顾。
IF 2.4
Journal of Central Nervous System Disease Pub Date : 2026-04-16 eCollection Date: 2026-01-01 DOI: 10.1177/11795735261444425
Valentini Samanidou, Konstantinos Tsamis, Alexandr Ceasovschih, Theocharis Koufakis, Dimitrios Patoulias, Evangelos C Rizos, Manfreddi Rizzo, Haralampos Milionis, Fotios Barkas
{"title":"GLP-1 Receptor Agonists in Acute Ischemic Stroke and Secondary Stroke Prevention: A Narrative Review of Preclinical and Clinical Evidence.","authors":"Valentini Samanidou, Konstantinos Tsamis, Alexandr Ceasovschih, Theocharis Koufakis, Dimitrios Patoulias, Evangelos C Rizos, Manfreddi Rizzo, Haralampos Milionis, Fotios Barkas","doi":"10.1177/11795735261444425","DOIUrl":"10.1177/11795735261444425","url":null,"abstract":"<p><strong>Background: </strong>Acute ischemic stroke (AIS) is a leading cause of death and disability. Glucagon-like peptide-1 receptor agonists (GLP-1RA) reduce atherosclerotic events in type 2 diabetes and obesity, and meta-analyses of cardiovascular outcome trials (CVOTs) suggest a modest reduction in incident stroke. Their safety and efficacy within acute AIS pathways remain uncertain.</p><p><strong>Methods: </strong>We conducted a narrative review by searching MEDLINE (PubMed), Cochrane CENTRAL, and ClinicalTrials.gov for studies through January 2026. We included in vitro and animal stroke models, observational studies, randomized controlled trials, and meta-analyses reporting AIS-related outcomes or stroke prevention endpoints with GLP-1RA.</p><p><strong>Results: </strong>Experimental models commonly show reduced infarct volume and improved neurological outcomes, with proposed mechanisms including attenuation of excitotoxicity, apoptosis, oxidative stress, neuroinflammation, and blood-brain barrier disruption, alongside signals of angiogenesis and neurogenesis. Translation is limited by heterogeneity of agents, timing, dosing, and routes, and by uncertainty over direct central nervous system versus systemic mediation. CVOTs and meta-analyses support long-term stroke risk reduction, whereas observational studies and small AIS trials mainly inform feasibility, metabolic control, and safety, with efficacy unproven. Ongoing stroke-dedicated trials should define patient selection, exposure-response relationships, and interactions with thrombolysis or thrombectomy, while prospectively incorporating imaging and biomarker endpoints to test mechanisms.</p><p><strong>Conclusions: </strong>Current evidence does not support routine GLP-1RA use as an acute neuroprotective therapy in AIS in humans. At present, GLP-1RA should be considered primarily for secondary prevention in patients with established indications, pending dedicated stroke trials clarifying acute safety, optimal timing/dosing, interactions with reperfusion therapies, and functional endpoints.</p>","PeriodicalId":15218,"journal":{"name":"Journal of Central Nervous System Disease","volume":"18 ","pages":"11795735261444425"},"PeriodicalIF":2.4,"publicationDate":"2026-04-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13087331/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147722727","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Ofatumumab Treatment Used in Pediatric MOGAD: A Retrospective Case Series. Ofatumumab治疗小儿MOGAD:回顾性病例系列
IF 2.4
Journal of Central Nervous System Disease Pub Date : 2026-04-08 eCollection Date: 2026-01-01 DOI: 10.1177/11795735261436889
Wenlin Wu, Haixia Zhu, Chi Hou, Yanping Ran, Wenxiao Wu, Wen-Xiong Chen, Xiaolan Mo, Kelu Zheng, Huiling Shen, Houliang Deng, Yulin Tang, Wei Liang, Yuanyuan Gao, Xiaojing Li
{"title":"Ofatumumab Treatment Used in Pediatric MOGAD: A Retrospective Case Series.","authors":"Wenlin Wu, Haixia Zhu, Chi Hou, Yanping Ran, Wenxiao Wu, Wen-Xiong Chen, Xiaolan Mo, Kelu Zheng, Huiling Shen, Houliang Deng, Yulin Tang, Wei Liang, Yuanyuan Gao, Xiaojing Li","doi":"10.1177/11795735261436889","DOIUrl":"10.1177/11795735261436889","url":null,"abstract":"<p><strong>Background: </strong>Myelin oligodendrocyte glycoprotein antibody-associated disorder (MOGAD) is a rare central nervous system demyelinating disease with a variable course, including both monophasic and relapsing phenotypes. Effective treatment options, particularly for relapsing disease, remain limited. Ofatumumab (OFA), a subcutaneous anti-CD20 monoclonal antibody approved for multiple sclerosis, has not been studied in pediatric MOGAD. This study aims to assess its safety and efficacy in this population.</p><p><strong>Objective: </strong>To describe the safety and efficacy of off-label OFA use in pediatric MOGAD patients.</p><p><strong>Patients and methods: </strong>We conducted a retrospective case series of consecutive pediatric patients with relapsing MOGAD who received OFA. Clinical data, including relapse history, annualized relapse rate (ARR), laboratory findings, and adverse events, were collected and analyzed.</p><p><strong>Results: </strong>Three pediatric (1 female, 2 males) were included. Age at onset was 2.3, 6.1, and 2.5 years; disease duration before OFA was 6.3, 2.2, and 9.1 years; and age at OFA initiation was 8.6, 8.3, and 11.6 years. Two patients started OFA due to breakthrough relapses on mycophenolate mofetil (MMF); one switched from maintenance intravenous immunoglobulin (IVIG) for administrative convenience. Treatment durations were 12, 9, and 9 months. All patients achieved rapid B-cell depletion (CD19+ < 10 cells/μL), which was sustained in two. Serum MOG-IgG titers increased in two patients and became negative in one. Two patients remained relapse-free during OFA treatment; one experienced a single relapse at 3 months. ARR decreased in all three patients post-OFA initiation. OFA was well tolerated. Only one patient developed transient fever after the first injection, which resolved with symptomatic treatment and did not recur.</p><p><strong>Conclusion: </strong>In this small case series, OFA was generally well-tolerated and associated with a reduction in ARR, suggesting a potential role in combination therapy for relapsing pediatric MOGAD. Given the use of combination treatments in some cases, including OFA+MMF and IVIG, the role of OFA alone remains unclear. Its subcutaneous route provides practical advantages in terms of convenience. Owing to the small sample size and relapse observed in one patient, these findings remain preliminary and warrant validation in larger, prospective studies.</p>","PeriodicalId":15218,"journal":{"name":"Journal of Central Nervous System Disease","volume":"18 ","pages":"11795735261436889"},"PeriodicalIF":2.4,"publicationDate":"2026-04-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13065265/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147673721","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Multiple Sclerosis in Charcot-Marie-Tooth Disease Type 1A - A Case Report and Literature Review. 多发性硬化症1A - A型病例报告及文献复习。
IF 2.4
Journal of Central Nervous System Disease Pub Date : 2026-03-11 eCollection Date: 2026-01-01 DOI: 10.1177/11795735261431921
Wen Yang, Lei Zhou, Gavin P Reynolds, Xianwen Wei
{"title":"Multiple Sclerosis in Charcot-Marie-Tooth Disease Type 1A - A Case Report and Literature Review.","authors":"Wen Yang, Lei Zhou, Gavin P Reynolds, Xianwen Wei","doi":"10.1177/11795735261431921","DOIUrl":"10.1177/11795735261431921","url":null,"abstract":"<p><p>Central nervous system (CNS) demyelination is an uncommon observation in patients with Charcot-Marie-Tooth disease (CMT). Where it does occur, it is usually associated with X-linked CMT. We present a case of CMT type 1A with a likely de novo mutation who experienced initial symptoms, and subsequent exacerbation, of multiple sclerosis following respiratory infection. A review of the literature reveals that reports of CMT1A with CNS demyelination are rare. We propose that the mutations in the PMP22 gene result in an over-expression of PMP22 mRNA, which overcomes the normal suppression by miRNA species that occurs in the CNS. This abnormal expression of PMP22 protein may, in certain circumstances, exacerbate autoimmune responses to result eventually in CNS demyelination.</p>","PeriodicalId":15218,"journal":{"name":"Journal of Central Nervous System Disease","volume":"18 ","pages":"11795735261431921"},"PeriodicalIF":2.4,"publicationDate":"2026-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12982849/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147468155","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Modulation of EEG Background Rhythms in Sturge-Weber Syndrome: Impact of Surgical Timing on the Unaffected Hemisphere. 斯特奇-韦伯综合征脑电图背景节律的调节:手术时机对未受影响半球的影响。
IF 2.4
Journal of Central Nervous System Disease Pub Date : 2026-03-04 eCollection Date: 2026-01-01 DOI: 10.1177/11795735261431415
Jing Wang, Kun Lv, Zeming Wang, Xiongfei Wang, Yuguang Guan, Jian Zhou, Tianfu Li, Mengyang Wang, Yujiao Yang, Guoming Luan
{"title":"Modulation of EEG Background Rhythms in Sturge-Weber Syndrome: Impact of Surgical Timing on the Unaffected Hemisphere.","authors":"Jing Wang, Kun Lv, Zeming Wang, Xiongfei Wang, Yuguang Guan, Jian Zhou, Tianfu Li, Mengyang Wang, Yujiao Yang, Guoming Luan","doi":"10.1177/11795735261431415","DOIUrl":"10.1177/11795735261431415","url":null,"abstract":"<p><strong>Background: </strong>Epilepsy surgery improves seizure outcomes in Sturge-Weber syndrome (SWS), yet the electrophysiological patterns of postoperative recovery remain poorly characterized. Previous studies suggest that early intervention may yield distinct clinical trajectories, but the associated changes in EEG background activity have not been systematically investigated.</p><p><strong>Objective: </strong>To investigate whether age of surgery influences the postoperative modulation of EEG background rhythms in children with unilateral SWS, and to evaluate the utility of preoperative EEG asymmetry for lateralizing the epileptogenic hemisphere.</p><p><strong>Design: </strong>Retrospective cohort study.</p><p><strong>Methods: </strong>We analyzed children with unilateral SWS who underwent epilepsy surgery, stratified by age at intervention (<2 vs. ≥2 years). Pre- and postoperative scalp EEGs were visually assessed to quantify posterior dominant alpha frequency and slow-wave (delta and theta bands) activity separately in the affected and unaffected hemispheres (AH, UH). Preoperative lateralization accuracy was also evaluated for both frequency bands.</p><p><strong>Results: </strong>A total of 99 patients were included. Seizure freedom rates were comparable between age groups. However, younger patients exhibited a significantly greater postoperative increase in alpha frequency, particularly in the UH (20 ± 20% vs. 4 ± 10%, <i>P</i> < 0.001). This effect was consistent across both focal resection (17±12% vs. 1 ± 8%, <i>P</i> < 0.001) and hemispheric surgery (22 ± 22% vs. 7 ± 11%, <i>P</i> = 0.006). In contrast, slow-wave modulation did not differ by age. Preoperative alpha asymmetry correctly lateralized the surgical hemisphere in 86.9% of cases (sensitivity 85.7%, specificity 88.0%, <i>κ</i> = 0.74), outperforming slow-wave asymmetry (accuracy 66.7%, <i>κ</i> = 0.33).</p><p><strong>Conclusion: </strong>Early epilepsy surgery in SWS is associated with enhanced postoperative modulation of alpha frequency in the UH, possibly reflecting greater neuroplastic capacity during early development. Preoperative alpha asymmetry offers robust lateralizing value. These findings support the clinical utility of background EEG analysis in surgical planning and postoperative monitoring.</p>","PeriodicalId":15218,"journal":{"name":"Journal of Central Nervous System Disease","volume":"18 ","pages":"11795735261431415"},"PeriodicalIF":2.4,"publicationDate":"2026-03-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12961107/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147377565","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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