Current Opinion in Neurology最新文献

Emerging targeted therapies in meningiomas. 新兴的脑膜瘤靶向治疗。

IF 4.4 2区医学

Current Opinion in Neurology Pub Date : 2025-10-09 DOI: 10.1097/WCO.0000000000001430

Erika Yamazawa, Emily Sullivan, Hiroaki Wakimoto, Priscilla K Brastianos

{"title":"Emerging targeted therapies in meningiomas.","authors":"Erika Yamazawa, Emily Sullivan, Hiroaki Wakimoto, Priscilla K Brastianos","doi":"10.1097/WCO.0000000000001430","DOIUrl":"https://doi.org/10.1097/WCO.0000000000001430","url":null,"abstract":"Purpose of review: Patients with grade 2 and 3 meningioma have high recurrence rates and limited treatment options after failure of radiation and surgery. Recent advances in molecular profiling of these tumors have enabled the investigation of novel targeted therapeutic approaches.Recent findings: Innovative treatment strategies under investigation for recurrent high-grade meningiomas include targeted therapies, immunotherapy, and radionuclide-based approaches. Inhibition of angiogenesis, histone deacetylases, FAK, mTOR, and CDK4/6 pathways has shown early signs of activity in small clinical trials of patients with recurrent meningiomas. Immunotherapy, such as immune checkpoint inhibition (ICI), has also demonstrated prolonged disease control in a subset of patients. Larger randomized studies are needed for further investigation of the efficacy and safety of these newer therapies in patients with high-grade and recurrent meningioma.Summary: Emerging molecularly driven treatment strategies show promise for the treatment of patients with high-grade meningiomas. Larger trials that incorporate molecular testing are warranted to fully evaluate their therapeutic potential.","PeriodicalId":11059,"journal":{"name":"Current Opinion in Neurology","volume":" ","pages":""},"PeriodicalIF":4.4,"publicationDate":"2025-10-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145257619","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Electrical and optogenetic spinal cord stimulation for movement recovery after spinal cord injury. 电和光遗传脊髓刺激对脊髓损伤后运动恢复的影响。

IF 4.4 2区医学

Current Opinion in Neurology Pub Date : 2025-10-06 DOI: 10.1097/WCO.0000000000001431

Richard P Henderson, Sarah E Mondello, Chet T Moritz

{"title":"Electrical and optogenetic spinal cord stimulation for movement recovery after spinal cord injury.","authors":"Richard P Henderson, Sarah E Mondello, Chet T Moritz","doi":"10.1097/WCO.0000000000001431","DOIUrl":"https://doi.org/10.1097/WCO.0000000000001431","url":null,"abstract":"Purpose of review: In this review, we discuss electrical and optogenetic technologies for stimulating the spinal cord to improve movement after spinal cord injury (SCI).Recent findings: Paralysis or paresis following SCI severely impairs control and movement of the extremities. Restoring movement in the upper and lower extremities is a top priority for this population. Invasive and noninvasive electrical stimulation of the spinal cord can modulate the activity of spinal circuits, resulting in improvements in motor and sensory function. More recently, optogenetic stimulation has emerged as another technique capable of modulating spinal circuity to facilitate movement recovery in animal models. Recent studies are offering new insights into the effects of parameter selection, multisite stimulation, and the combined effects of stimulation and wearable robotic exoskeletons, all with the goal of restoring movement after SCI.Summary: Modulating the activity of the spinal cord via electrical and optogenetic stimulation is a promising intervention for improving movement after SCI. Future studies should determine optimal stimulation parameters, synergistic effects when combined with wearable robotics, and the safety of optogenetics in the human spinal cord. Such work will best position these emerging technologies for clinical translation.","PeriodicalId":11059,"journal":{"name":"Current Opinion in Neurology","volume":" ","pages":""},"PeriodicalIF":4.4,"publicationDate":"2025-10-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145257679","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Update on statin-associated myopathy symptoms in the view of new clinical management strategies. 从新的临床管理策略来看，他汀类药物相关肌病症状的最新进展

IF 4.4 2区医学

Current Opinion in Neurology Pub Date : 2025-10-01 Epub Date: 2025-06-12 DOI: 10.1097/WCO.0000000000001405

Olimpia Musumeci, Selene Francesca Anna Drago

{"title":"Update on statin-associated myopathy symptoms in the view of new clinical management strategies.","authors":"Olimpia Musumeci, Selene Francesca Anna Drago","doi":"10.1097/WCO.0000000000001405","DOIUrl":"10.1097/WCO.0000000000001405","url":null,"abstract":"Purpose of review: Purpose of this review is to highlight the recent findings in terms of clinical aspects, pathogenic mechanisms and managements of statin associated muscle symptoms (SAMS), and focusing on the use of novel therapeutic alternatives in clinical practice.Recent findings: While extensive research has been conducted on SAMS, the precise mechanisms remain unclear. Recent findings continue to explore various aspects, including potential risk factors, diagnostic approaches, and management strategies. Managing SAMS involves a careful assessment to confirm the diagnosis, a stepwise approach to treatment that may include dose adjustments, switching statins, considering alternate-day dosing, and exploring nonstatin therapies, all while prioritizing patient well being and cardiovascular risk reduction through shared decision-making and ongoing monitoring. In recent years, the therapeutic landscape has expanded with the introduction of several novel lipid-lowering agents, providing valuable alternatives for both statin-tolerant and statin-intolerant patients but their use in clinical practice is still limited because of high costs, regulatory limitations and type of administration.Summary: Given the increasing use of both traditional and emerging lipid-lowering therapies, a clear understanding of their comparative safety, particularly regarding musculoskeletal adverse effects, is essential for guiding clinical decision-making.","PeriodicalId":11059,"journal":{"name":"Current Opinion in Neurology","volume":" ","pages":"551-559"},"PeriodicalIF":4.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144274397","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Enzyme replacement therapies in adults with Pompe disease: from trials to real-world data. 成人庞贝病的酶替代疗法：从试验到实际数据。

IF 4.4 2区医学

Current Opinion in Neurology Pub Date : 2025-10-01 Epub Date: 2025-06-05 DOI: 10.1097/WCO.0000000000001385

Nadine A M E van der Beek, Lianne H Potters, Benedikt Schoser

{"title":"Enzyme replacement therapies in adults with Pompe disease: from trials to real-world data.","authors":"Nadine A M E van der Beek, Lianne H Potters, Benedikt Schoser","doi":"10.1097/WCO.0000000000001385","DOIUrl":"10.1097/WCO.0000000000001385","url":null,"abstract":"Purpose of review: To review the clinical trial results and emerging real-world data of two new enzyme replacement therapies (ERTs) for late-onset Pompe disease and to compare these effects in the context of what has been achieved over the last two decades in advancing care for Pompe disease.Recent findings: Randomized controlled trials (RCTs) of avalglucosidase alfa and cipaglucosidase alfa plus miglustat have demonstrated that both treatments are at least as efficacious as alglucosidase alfa and possess a comparable safety profile. Several post hoc analyses of the trial data have shown that these newer ERTs result in a greater percentage of patients achieving meaningful improvements and larger reductions in biomarker levels. The first real-world data on switching from alglucosidase alfa to avalglucosidase alfa has shown that the switch is safe and may alter individual disease trajectories.Summary: The advent of two next-generation enzyme replacement therapies marks a new era in treating patients diagnosed with Pompe disease. Clinical trials and early real-world data suggest that they may be superior to alglucosidase alfa, the standard of care for the past 20 years, although head-to-head comparisons between all three treatments are lacking. More data will become available over the next 5 years, leading to better guidelines for starting, stopping and switching therapies based on a more personalized assessment of outcomes.","PeriodicalId":11059,"journal":{"name":"Current Opinion in Neurology","volume":" ","pages":"538-545"},"PeriodicalIF":4.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12419020/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144233487","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Sleep alterations in amyotrophic lateral sclerosis. 肌萎缩侧索硬化症患者的睡眠改变。

IF 4.4 2区医学

Current Opinion in Neurology Pub Date : 2025-10-01 Epub Date: 2025-08-20 DOI: 10.1097/WCO.0000000000001424

Christina Lang

{"title":"Sleep alterations in amyotrophic lateral sclerosis.","authors":"Christina Lang","doi":"10.1097/WCO.0000000000001424","DOIUrl":"10.1097/WCO.0000000000001424","url":null,"abstract":"Purpose of review: This review summarizes recent evidence on sleep disturbances in amyotrophic lateral sclerosis (ALS), emphasizing their role as intrinsic features of the disease process rather than consequence of motor decline.Recent findings: Emerging data suggest that sleep disturbances such as sleep fragmentation, rapid eye movement sleep (REM) and non rapid eye movement sleep (NREM) alterations and circadian changes often precede classic motor symptoms. Structural and functional hypothalamic changes have been observed in early ALS, suggesting a direct role in sleep-wake dysregulation. In addition, impaired glymphatic clearance during sleep may contribute to neurodegeneration by impairing the removal of protein waste. Polysomnographic studies and cohort data support the presence of prodromal sleep abnormalities in both symptomatic patients and gene mutation carriers. Noninvasive ventilation has shown benefits not only in respiratory management but also in improving sleep quality and overall prognosis.Summary: Sleep alterations in ALS are increasingly recognized as early indicators and potential modulators of disease progression. The hypothalamus and the glymphatic system emerge as key contributors to these disturbances, highlighting sleep as a therapeutic target. Understanding the role of sleep in ALS pathophysiology may aid in earlier diagnosis and novel intervention strategies aimed at modifying disease course.","PeriodicalId":11059,"journal":{"name":"Current Opinion in Neurology","volume":" ","pages":"606-613"},"PeriodicalIF":4.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12435240/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144882406","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Agentic artificial intelligence in neuromuscular health: a new ecosystem for autonomous digital systems featuring multimodal integration, trial support, and real-world monitoring. 神经肌肉健康中的人工智能：自主数字系统的新生态系统，具有多模态集成，试验支持和现实世界监测。

IF 4.4 2区医学

Current Opinion in Neurology Pub Date : 2025-10-01 Epub Date: 2025-09-04 DOI: 10.1097/WCO.0000000000001417

Benedikt Schoser

引用次数: 0

Myofibrillar myopathy: towards a mechanism-based definition as a Z-disk-opathy. 肌原纤维肌病：迈向基于机制的z盘病定义。

IF 4.4 2区医学

Current Opinion in Neurology Pub Date : 2025-10-01 Epub Date: 2025-06-09 DOI: 10.1097/WCO.0000000000001397

Michio Inoue, Conrad C Weihl

{"title":"Myofibrillar myopathy: towards a mechanism-based definition as a Z-disk-opathy.","authors":"Michio Inoue, Conrad C Weihl","doi":"10.1097/WCO.0000000000001397","DOIUrl":"10.1097/WCO.0000000000001397","url":null,"abstract":"Purpose of review: Myofibrillar myopathies (MFMs) are traditionally defined by histopathology, but recent genetic discoveries have broadened the spectrum of causative genes beyond Z-disk components. This review aims to address the resulting terminological inconsistency by proposing a refined, mechanism-based definition of MFM centered on its identity as a \"Z-disk-opathy.\" This re-evaluation is timely and relevant for improving diagnostic clarity and guiding future research.Recent findings: The literature increasingly reports MFM-like pathology in conditions caused by mutations in genes not directly encoding Z-disk structural proteins or their interacting chaperones. This review highlights the pathogenic mechanisms distinguishing true MFMs, which involve disruption of Z-disk protein structure or Z-disk protein homeostasis, from \"myopathies with MFM pathology\" that share histological features but stem from different molecular etiologies. Key themes include the dominant nature of mutations in Z-disk structural proteins and the critical role of chaperone dysfunction in MFM pathogenesis.Summary: A refined definition classifying MFM as a \"Z-disk-opathy\" offers a clearer framework for diagnosis and mechanistic understanding. This distinction has significant implications for clinical practice, facilitating more accurate diagnosis, and for research, by supporting the development of targeted therapeutic strategies aimed at either restoring Z-disk proteostasis or mitigating the effects of aberrant protein accumulation.","PeriodicalId":11059,"journal":{"name":"Current Opinion in Neurology","volume":" ","pages":"513-523"},"PeriodicalIF":4.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12354002/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144246884","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Small fiber neuropathy: expanding diagnosis with unsettled etiology. 小纤维神经病：病因不明的扩展诊断。

IF 4.4 2区医学

Current Opinion in Neurology Pub Date : 2025-10-01 Epub Date: 2025-08-20 DOI: 10.1097/WCO.0000000000001418

Grazia Devigili, Margherita Marchi, Giuseppe Lauria

{"title":"Small fiber neuropathy: expanding diagnosis with unsettled etiology.","authors":"Grazia Devigili, Margherita Marchi, Giuseppe Lauria","doi":"10.1097/WCO.0000000000001418","DOIUrl":"10.1097/WCO.0000000000001418","url":null,"abstract":"Purpose of review: Small fiber neuropathies (SFN) are a heterogeneous group of disorders affecting the thinly myelinated Aδ and unmyelinated C-fibers. The clinical picture is dominated by neuropathic pain, often accompanied by autonomic symptoms of variable severity. The underlying causes encompass metabolic conditions like diabetes mellitus, immuno-mediated disorders, infection, exposure to toxins, and gain-of-function variants in the genes encoding the Nav1.7, Nav1.8, and Nav1.9 sodium channel subunits, though the list of associated diseases continues to grow. Recently, increased attention has focused on immune-mediated forms, which led to the identification of potentially treatable subgroups. These discoveries have advanced our understanding of pathophysiological mechanisms.Recent findings: Recent studies have broadened the spectrum of underlying conditions associated with SFN, including immune-mediated forms and links to SARS-CoV-2 infection and vaccines. Studies on genetic variants linked to unique clinical presentations have also yielded new insights. Furthermore, emerging perspectives highlighted disorders involving small fiber pathology that lacks typical clinical features of neuropathic pain, challenging traditional diagnostic criteria.Summary: Deepening our understanding of the causes underlying SFN advances the identification of potential therapeutic targets. The clinical presentation of SFN can vary significantly and may not consistently correlate with specific underlying conditions. Therefore, a systematic investigation of possible causes through a structured diagnostic assessment is critical to unveil additional contributing factors.","PeriodicalId":11059,"journal":{"name":"Current Opinion in Neurology","volume":" ","pages":"485-495"},"PeriodicalIF":4.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12419023/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144793688","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

New insights in the immune treatment of Guillain-Barré syndrome. 格林-巴罗综合征免疫治疗的新见解。

IF 4.4 2区医学

Current Opinion in Neurology Pub Date : 2025-10-01 Epub Date: 2025-07-21 DOI: 10.1097/WCO.0000000000001408

Eveline J A Wiegers, Bart C Jacobs

{"title":"New insights in the immune treatment of Guillain-Barré syndrome.","authors":"Eveline J A Wiegers, Bart C Jacobs","doi":"10.1097/WCO.0000000000001408","DOIUrl":"10.1097/WCO.0000000000001408","url":null,"abstract":"Purpose of review: Guillain-Barré syndrome (GBS) is a severe but treatable form of immune-mediated neuropathy. The purpose of this review is to provide an update on current immune treatments for GBS, highlight challenges in clinical practice and research, and discuss new developments in therapies that focus on reducing inflammation and preventing further nerve damage.Recent findings: In 2023, a GRADE-based guideline was published on the diagnosis and treatment of GBS on behalf of EAN/PNS. Several clinical trials have been conducted in GBS recently, including studies with an observational comparative study design.Summary: Since 30 years, intravenous immunoglobulins and plasma exchange are the only proven effective immune treatments for GBS. Despite these treatments, a substantial proportion of patients recover incompletely and have residual disability or complaints with a high impact on quality of life. New treatment trials focus on reducing immunoglobulin G antibodies to nerves and inhibition of complement activation. Observational comparative studies based on extensive and well defined cohorts are an alternative method to evaluate the effect of treatments in GBS. Several novel study designs are discussed that aim to facilitate the conduct of future trials with more sustainable use of data.","PeriodicalId":11059,"journal":{"name":"Current Opinion in Neurology","volume":" ","pages":"471-477"},"PeriodicalIF":4.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12419018/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144759389","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Autoimmune nodopathies: emerging insights and clinical implications. 自身免疫性结节病：新见解和临床意义

IF 4.4 2区医学

Current Opinion in Neurology Pub Date : 2025-10-01 Epub Date: 2025-06-20 DOI: 10.1097/WCO.0000000000001399

Roger Collet-Vidiella, Alberto De Lorenzo, Luis Querol

{"title":"Autoimmune nodopathies: emerging insights and clinical implications.","authors":"Roger Collet-Vidiella, Alberto De Lorenzo, Luis Querol","doi":"10.1097/WCO.0000000000001399","DOIUrl":"10.1097/WCO.0000000000001399","url":null,"abstract":"Purpose of review: Autoimmune nodopathies (AN) are a recognized distinct group of immune-mediated peripheral neuropathies with unique immunopathological features and therapeutic implications. This review synthesizes recent advances in their pathogenesis, diagnosis, and management, which have refined their clinical classification and informed targeted treatment strategies.Recent findings: AN are characterized by autoantibodies targeting surface proteins in the nodal-paranodal area (anti-contactin-1, anti-contactin-associated protein 1, anti-neurofascin-155, anti-pan-neurofascin), predominantly of IgG4 subclass. Recent studies have delineated antibody subclass contributions to disease mechanisms and identified B-cell response patterns predictive of therapeutic outcomes. Despite clinical overlap with chronic inflammatory demyelinating polyradiculoneuropathy and Guillain-Barré syndrome, AN exhibit a distinct phenotype and a poor response to intravenous immunoglobulins. Multiple studies, including recent ones, report good response and long-term clinical remission with B-cell depleting therapies. Diagnostic assays such as cell-based assays and ELISA offer high accuracy, while biomarker-guided monitoring using antibody titers and serum neurofilament light chain supports individualized follow-up.Summary: Emerging evidence consolidates AN as a nosologically and therapeutically distinct entity. Integration of immunopathological insights with biomarker-driven strategies enables precision diagnostics and targeted immunotherapy, improving clinical outcomes.","PeriodicalId":11059,"journal":{"name":"Current Opinion in Neurology","volume":"38 5","pages":"452-458"},"PeriodicalIF":4.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145014110","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0