European Journal of Neurology最新文献

{"title":"A Network Meta-Analysis of Hereditary Transthyretin Amyloidosis With Polyneuropathy Supports a Class Effect, Not a Ranking of Individual Drugs","authors":"Nicholas Streicher, Michelle Mezei, Marco Luigetti","doi":"10.1111/ene.70727","DOIUrl":"10.1111/ene.70727","url":null,"abstract":"<p>Duarte and colleagues [<span>1</span>] have synthesized the randomized evidence in ATTRv-PN with care, and their central finding is sound: gene silencers outperform stabilizers. The effect is large, consistent across both efficacy outcomes, and biologically coherent.</p><p>Our concern is the finer claim: that the individual gene silencers can be ranked against one another, and in particular that the RNAi agents outperform the antisense oligonucleotides. It is a different statement, resting on different evidence, and that evidence does not support it. The authors grade the key comparisons as low certainty, then present a definite ranking. The abstract and rankings read as established fact; the evidence behind them does not.</p><p>The ranking rests on a single transformed outcome. The network compares each trial against the others, and it contains only two direct head-to-head comparisons: the two RNAi agents against each other, and the two antisense oligonucleotides against each other. In the authors' own league table, neither shows a difference. Every comparison between an RNAi agent and an antisense agent is indirect, and the authors grade these low, downgraded for risk of bias and imprecision.</p><p>The ranking also appears on only one outcome. On Norfolk-QoL-DN, reported on a single scale, vutrisiran, patisiran, and eplontersen do not separate. The ordering appears only on mNIS+7, where two versions of the composite scale, differing in range and component weighting, had to be pooled using standardized mean differences.</p><p>The eplontersen comparison carries a further, structural problem. In NEURO-TTRansform, inotersen patients crossed to eplontersen at Week 35 [<span>2</span>], after which no inotersen comparator remains, so the eplontersen-versus-inotersen contrast derives only from that interim. Patisiran and vutrisiran enter the network at 18 months [<span>3, 4</span>]. Silencer effects grow with continued treatment, so a comparison anchored early understates the drug measured late. The network has no closed loop, so this bias cannot be detected or corrected.</p><p>For the patisiran-versus-eplontersen comparison, the authors downgrade for imprecision, noting credible intervals that “include widely disparate conclusions.” The abstract nevertheless states that vutrisiran and patisiran “demonstrated improvements compared with diflunisal, eplontersen, and inotersen.” A comparison the authors flag as spanning opposite conclusions cannot at the same time establish a ranking. A standardized mean difference rescales range but does not reconcile different constructs; Samjoo et al. [<span>5</span>] found that even the two mNIS+7 versions weight their components differently, yet the analysis pools them.</p><p>Three entries in the baseline-characteristics table of Duarte et al. do not change the main finding but affect whether the trials are similar enough to be compared fairly. NEURO-TTR is listed as 57%/43% stage 1/2, whereas the trial reports 67","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 8","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-08-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13435096/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148668784","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}

{"title":"The Diagnostic Accuracy of the Second Revision of the European Academy of Neurology/Peripheral Nerve Society Guideline for Diagnosing Chronic Inflammatory Demyelinating Polyradiculoneuropathy","authors":"Iris N. van Doorn,&nbsp;Adája E. Baars,&nbsp;Luuk Wieske,&nbsp;Bart C. Jacobs,&nbsp;Krista Kuitwaard,&nbsp;W. Ludo van der Pol,&nbsp;Pieter A. van Doorn,&nbsp;Ivo N. van Schaik,&nbsp;Filip Eftimov,&nbsp;H. Stephan Goedee,&nbsp;Judith Drenthen,&nbsp;Camiel Verhamme","doi":"10.1111/ene.70726","DOIUrl":"10.1111/ene.70726","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Diagnosing chronic inflammatory demyelinating polyneuropathy (CIDP) relies on clinical and electrodiagnostic features. In 2021, the European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) revised the CIDP guideline, superseding the 2010 European Federation of Neurological Societies (EFNS)/PNS guideline. This study compares the diagnostic accuracy of both guidelines and evaluates the impact of specific criterion changes and nerve conduction study (NCS) extensiveness.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Patients with CIDP were included if they fulfilled the 2010 EFNS/PNS electrodiagnostic criteria for at least possible CIDP or had a presumptive clinical CIDP diagnosis with objective treatment response. Matched controls were patients with suspected chronic immune-mediated neuropathy who received a diagnosis other than CIDP. Diagnostic accuracy was evaluated using two thresholds: one including possible CIDP and one restricted to (probable/definite) CIDP. The impact of electrodiagnostic criteria changes and NCS extensiveness on diagnostic performance was assessed.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>We included 339 patients and 339 matched controls. Sensitivity of the 2010 EFNS/PNS guideline was 92% when including possible CIDP and 91% for probable/definite CIDP, with specificities of 67% and 78%, respectively. For the 2021 EAN/PNS guideline, sensitivity was 94% when including possible CIDP and 83% for CIDP, with specificities of 66% and 86%, respectively. Differences were due to sensory criteria and changes in conduction block criteria. Unilateral NCS would miss 77 possible CIDP diagnoses.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>Sensitivity and specificity were comparable when including possible CIDP, but the 2021 EAN/PNS guideline showed lower sensitivity and higher specificity for CIDP alone. Modifying criteria, including removal of sensory abnormalities, and more extensive NCS enhanced accuracy.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 8","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-08-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13434837/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148668746","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}

{"title":"Federating European REgistries for Stroke: Principles, Data Model and Future Perspectives for a Federated Analysis of Multiple Prospective Stroke Registries","authors":"Alexander Salerno,&nbsp;Georges Melissargos,&nbsp;Mira Katan,&nbsp;Michael Knoflach,&nbsp;Stefan Kiechl,&nbsp;Joseph Harbison,&nbsp;Leonardo Renieri,&nbsp;Danilo Toni,&nbsp;Lina Palaiodimou,&nbsp;Georgios Tsivigoulis,&nbsp;Andreas Ktenidis,&nbsp;Theofilos Mailis,&nbsp;Yannis Ioannidis,&nbsp;Patrik Michel,&nbsp;Philippe Ryvlin,&nbsp;the FERES collaborators","doi":"10.1111/ene.70715","DOIUrl":"10.1111/ene.70715","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Acute ischemic stroke is a leading cause of death and disability. Despite strong evidence supporting reperfusion therapies and Stroke Unit care, access and quality of stroke services remain heterogeneous across Europe. Although national stroke registries provide valuable real-world data, fragmentation, limited interoperability, and data protection constraints have restricted multinational analyses and benchmarking.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>The Federating European REgistries for Stroke (FERES) initiative establishes a GDPR-compliant federated framework for secondary use of stroke registry data. FERES harmonizes heterogeneous datasets through a stroke-specific Common Data Elements (CDE) model and performs analyses locally within each registry using the Medical Informatics Platform, sharing only aggregated, non-identifiable outputs. To validate the framework, a predefined showcase analysis comparing anterior versus posterior circulation acute ischemic stroke was executed in both centralized and federated modes using an identical harmonized dataset.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>FERES connected five national registries from Austria, Greece, Ireland, Italy, and Switzerland within a unified federated infrastructure. At the time of analysis, 149,772 patient events from two registries were accessible for federated querying, with three additional registries technically integrated and in advanced onboarding. The harmonized ontology comprised 945 standardized variables spanning the stroke care pathway. Federated execution reproduced centralized pooled-data results across descriptive statistics, hypothesis testing, effect sizes, and multivariable regression models with only minimal numerical discrepancies.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>FERES demonstrates that large-scale, multinational stroke research and benchmarking can be conducted in Europe using a privacy-preserving federated approach, providing a scalable foundation for cross-border real-world evidence generation and quality improvement.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 8","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-08-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13436129/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148668767","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}

{"title":"Update on the Incidence of Paediatric Multiple Sclerosis, Myelin Oligodendrocyte Glycoprotein Antibody–Associated Disease, Aquaporin-4 Antibody–Positive Neuromyelitis Optica Spectrum Disorder, and Other Acquired Demyelinating Syndromes in the Netherlands, 2017–2025, Including the COVID-19 Pandemic","authors":"Sandy Molenaar,&nbsp;Michiel S. J. Buijze,&nbsp;Arlette L. Bruijstens,&nbsp;Yu Yi M. Wong,&nbsp;Maud A. Quist,&nbsp;C. Louk de Mol,&nbsp;Beatrijs H. A. Wokke,&nbsp;Rinze F. Neuteboom,&nbsp;Dutch Study Group for Paediatric Multiple Sclerosis and Acute Disseminated Encephalomyelitis","doi":"10.1111/ene.70723","DOIUrl":"10.1111/ene.70723","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Acquired demyelinating syndromes (ADS) in children include multiple sclerosis (MS), myelin oligodendrocyte glycoprotein antibody–associated disease (MOGAD), aquaporin 4 antibody-positive neuromyelitis optica spectrum disorder (AQP4+ NMOSD), and other seronegative disorders. We previously reported an increased incidence of overall paediatric ADS and MS between 2007 to 2010 and 2011–2016 (from 0.66 to 0.80 and 0.15 to 0.26 per 100,000 person-years, respectively). Environmental factors, including viral exposure, may influence these rates. Comparing a recent period that includes the COVID-19 pandemic provides an opportunity to study temporal patterns.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Children &lt; 18 years with a first demyelinating event between January 2017 and June 2025 were prospectively included in the nationwide PROUDkids 2.0 study or referred to the Dutch Paediatric MS Centre. Diagnoses followed international criteria, and incidence rates were calculated using national population data.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Among 257 included children, 111 (43%) had MS, 93 (36%) MOGAD, 5 (2%) AQP4+ NMOSD, and 48 (19%) seronegative ADS. Overall ADS incidence was 0.91 per 100,000 person-years, with MS 0.40, MOGAD 0.33, AQP4+ NMOSD 0.02, and seronegative ADS 0.16 per 100,000 person-years. Compared with 2011–2016 (0.26 per 100,000), MS incidence increased, whereas other ADS rates remained stable. MS incidence declined after COVID-19 restrictions, particularly among adolescents, while MOGAD incidence rose modestly in younger children during the COVID restrictions.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Paediatric MS incidence continues to rise in the Netherlands, whereas the incidence of other ADS remains stable. Temporal variations in the incidence of MS and MOGAD during and after the COVID-19 pandemic may reflect the influence of viral exposure in their pathogenesis.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 8","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-08-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13431134/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148663720","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}

{"title":"Neuropsychological Sub-Phenotypes in Amyotrophic Lateral Sclerosis","authors":"Barbara Poletti,&nbsp;Edoardo Nicolò Aiello,&nbsp;Monica Consonni,&nbsp;Barbara Iazzolino,&nbsp;Silvia Torre,&nbsp;Veronica Faltracco,&nbsp;Alessandra Telesca,&nbsp;Francesca Palumbo,&nbsp;Beatrice Curti,&nbsp;Giulia De Luca,&nbsp;Arianna Moreschi,&nbsp;Francesca Frisco,&nbsp;Eleonora Dalla Bella,&nbsp;Enrica Bersano,&nbsp;Nilo Riva,&nbsp;Federico Verde,&nbsp;Stefano Messina,&nbsp;Alberto Doretti,&nbsp;Alessio Maranzano,&nbsp;Claudia Morelli,&nbsp;Stefano Francesco Cappa,&nbsp;Andrea Calvo,&nbsp;Michael J. Strong,&nbsp;Vincenzo Silani,&nbsp;Giuseppe Lauria,&nbsp;Adriano Chiò,&nbsp;Nicola Ticozzi","doi":"10.1111/ene.70706","DOIUrl":"10.1111/ene.70706","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>This study aimed at identifying neuropsychological sub-phenotypes in amyotrophic lateral sclerosis (ALS) within the mild cognitive impairment (MCI) and mild behavioral impairment (MBI) frameworks.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We used individual task−/item-level data from the cognitive and behavioral sections of the Edinburgh Cognitive and Behavioral ALS Screen (ECAS) from 901 non-demented ALS to derive neuropsychological sub-phenotypes pursuant to classical MCI and MBI frameworks and in accordance with an expanded version of Strong's criteria, which also addressed memory and visuo-spatial measures.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>The prevalence of MCI and MBI was 39% and 37%, respectively in this retrospective review. The following MCI sub-phenotypes were identified: dysexecutive MCI—single- and multiple-domain (dMCI-sd: 63%; dMCI-md: 24%, respectively); non-dysexecutive MCI—single- and multiple-domain (ndMCI-sd: 12%; ndMCI-md: 1%, respectively). MBI was classified as follows: apathetic MBI—single- and multiple-domain (aMBI-sd: 40%; aMBI-md: 20%, respectively); apathetic-disinihibited/perseverative MBI—multiple domain (ad/pMBI-md: 21%); disinihibited/perseverative MBI—multiple domain (d/pMBI-md: 7%); psychotic MBI—single- and multiple-domain (psyMBI-sd: 2%; psyMBI-md: 3%, respectively); unclassifiable MBI—multiple domain (uMBI-md: 1%). 143 (16%) of patients exhibited mild cognitive and behavioral impairment (MCBI).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>This study delivers a provisional, ECAS-based classification for the neuropsychological sub-phenotyping of non-demented ALS patients, which, with further validation, might be useful for both research and clinical purposes.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 8","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-08-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13431129/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148663718","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}

{"title":"Italian Delphi Consensus on Maternity Care in Multiple Sclerosis: Toward an Interdisciplinary Model of Care.","authors":"Emilio Portaccio, Paola Cavalla, Lucia Moiola, Francesco Patti, Luca Marozio, Paolo Palma, Martina Campobasso, Federica Balzani, Alessia Villani, Valentina Sara Barbiero, Daniela Facchinello, Raffaella Robello, Girolama Alessandra Marfia","doi":"10.1111/ene.70700","DOIUrl":"10.1111/ene.70700","url":null,"abstract":"<p><strong>Background: </strong>Reproductive health is a critical component of care for women with multiple sclerosis (MS), yet standardized interdisciplinary approaches remain limited.</p><p><strong>Objectives: </strong>This Delphi consensus aimed to develop an interdisciplinary, evidence-informed care model tailored to the Italian healthcare system.</p><p><strong>Results: </strong>A two-round Delphi process was conducted in Italy (June-July 2025), involving MS experts (Round 1: 107, Round 2: 95), including neurologists, gynecologists, pediatricians, nurses, and patient representatives. Participants evaluated key aspects of the reproductive care pathway, from preconception counseling to postpartum follow-up, to identify priorities and areas of agreement across disciplines. Strong consensus was achieved on the importance of early counseling, coordinated interdisciplinary management, and individualized treatment planning across the reproductive continuum. The central role of nurses in patient education was also emphasized. Divergence emerged regarding the continuation of specific therapies during pregnancy and the role of neonatal immunological monitoring following exposure to depleting agents, reflecting areas of ongoing uncertainty and limited evidence.</p><p><strong>Conclusions: </strong>This consensus proposes the first interdisciplinary framework for reproductive care in MS within the Italian context. It emphasizes patient-centered decision-making, collaboration across specialties, and the integration of reproductive health into routine MS management. By identifying both shared priorities and unresolved challenges, it provides a foundation for more harmonized, evidence-informed clinical practice and future research.</p>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 8","pages":"e70700"},"PeriodicalIF":4.3,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13445565/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148677116","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}

{"title":"The Human Intracerebral EEG Platform: A Cloud-Based Trusted Research Environment for Collaborative SEEG Research in Epilepsy and Neuroscience.","authors":"Carolina Ciumas, Florian Perrin, Florian Sipp, Nathalie Casati, Guy Spuhler, Anthony Boyer, Jonathan Haab, Viktoria Bastic Schmid, Birgit Schaffhauser, Georges Melissargos, Evan Hancock, Olivier David, Philippe Ryvlin","doi":"10.1111/ene.70702","DOIUrl":"https://doi.org/10.1111/ene.70702","url":null,"abstract":"<p><strong>Introduction: </strong>Lack of standardised methodologies and secure processing environments for intracerebral EEG (iEEG) data creates critical barriers to international collaboration and reproducibility in clinical neuroscience. Within the framework of the Human Brain Project and EBRAINS, we have developed a specific platform, the Human Intracerebral EEG Platform (HIP), to address this challenge.</p><p><strong>Methods: </strong>The specifications considered to develop the HIP included: (1) secure access to sensitive datasets; (2) standardised data organisation following the BIDS for iEEG (BIDS-iEEG) to ensure interoperability; (3) controlled access via a secure web-based interface; (4) provision of software needed to analyse iEEG; (5) a structured governance model enabling institutional participation through formal data sharing agreements; and (6) alignment with FAIR principles.</p><p><strong>Results: </strong>HIP was implemented as a cloud-based Trusted Research Environment (TRE), with a first operational release in 2023. It provides private spaces for each participating institution and researchers, as well as collaborative spaces to share data. The HIP operates many software packages, including 3DSlicer, Brainstorm, HiBoP, etc., and CiCLONE, a tool specifically designed to coregister and visualise electrodes and recording leads on MRI and brain atlas. So far, the HIP hosts 34 institutions and 10 ongoing projects, including that on heartbeat potentials that show promising findings toward a better understanding of central autonomic dysfunction in persons with epilepsy.</p><p><strong>Conclusions: </strong>The HIP addresses key barriers to large-scale iEEG research by combining secure governance, standardised data management, and collaborative analysis within a dedicated TRE, facilitating reproducible multi-centre research.</p>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 8","pages":"e70702"},"PeriodicalIF":4.3,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13495059/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148789896","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}

{"title":"Refining Electrophysiological Criteria in CIDP and MMN: Toward a Unified Definition of Motor Conduction Block and Proximal Temporal Dispersion.","authors":"Chiara Gallo, Carla Fasano, Angelo Fonseca, Yuri Falzone, Dario Cocito, Fiore Manganelli, Sabrina Matà, Giuseppe Cosentino, Vincenzo Di Stefano, Massimiliano Filosto, Luca Leonardi, Dario Ricciardi, Anna Mazzeo, Luana Benedetti, Marco Luigetti, Paolo Solla, Maurizio Inghilleri, Francesco Habetswallner, Alessandro Barilaro, Fabrizio Canale, Alberto De Lorenzo, Adele Ratti, Emanuele Cassano, Maddalena Spalletti, Elisa Vegezzi, Filippo Brighina, Barbara Risi, Francesca Forcina, Alessandra Fasolino, Luca Gentile, Giacomo Iabichella, Alessandro Beronio, Francesca Vitali, Federica Moret, Stefano Gazzina, Corrado Cabona, Vincenzo Todisco, Eduardo Nobile-Orazio, Pietro Emiliano Doneddu","doi":"10.1111/ene.70739","DOIUrl":"10.1111/ene.70739","url":null,"abstract":"<p><strong>Background: </strong>The definition of motor conduction block (CB) and proximal temporal dispersion (TD) for the diagnosis of CIDP and MMN remains debated, particularly regarding whether CB should be based on amplitude or area reduction, and whether proximal TD is best assessed using negative peak or total CMAP duration.</p><p><strong>Methods: </strong>Nerve conduction waveforms from 106 CIDP, 87 MMN, and 234 controls were analyzed. Motor CB was defined by ≥ 30%, ≥ 40%, or ≥ 50% amplitude or area reduction. Proximal TD was defined as a ≥ 30% increase in negative peak duration, total CMAP duration, or either criterion. Sensitivity, specificity, and inter-rater reliability were analyzed.</p><p><strong>Results: </strong>Across both CIDP and MMN, a ≥ 30% reduction in CMAP amplitude showed diagnostic performance comparable to area-based CB definitions and outperformed higher cutoff values. Requiring CB in multiple nerves increased specificity but reduced sensitivity. For proximal TD, negative peak duration was the most robust single parameter. Total CMAP duration identified additional abnormalities and, when combined with negative peak duration, improved diagnostic sensitivity in both disorders while maintaining high specificity. Total CMAP duration demonstrated good inter-rater agreement at the patient level. CMAP morphology showed substantial overlap between CIDP and MMN and limited discriminatory value.</p><p><strong>Conclusions: </strong>A ≥ 30% reduction in CMAP amplitude can be used as a universal definition of motor CB in both CIDP and MMN, providing diagnostic performance comparable to area-based criteria with greater simplicity. In addition, proximal TD assessed using total CMAP duration may represent a complementary electrophysiological criterion in both disorders.</p>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 8","pages":"e70739"},"PeriodicalIF":4.3,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13486628/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148789887","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}

{"title":"Long-Term Cancer and Mortality After Thymectomy","authors":"Shahar Shelly,&nbsp;Mor Ani,&nbsp;Christopher Klein,&nbsp;Yuebing Li,&nbsp;Henry Kaminski,&nbsp;Gil I. Wolfe,&nbsp;Keren Miron,&nbsp;Roni Ramon-Gonen","doi":"10.1111/ene.70721","DOIUrl":"https://doi.org/10.1111/ene.70721","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Long-term health effects of thymectomy in adults remain uncertain. Previous reports suggested associations with increased mortality, cancer incidence, and autoimmune disease, although these findings may reflect differences in patient selection, surgical indication, or follow-up duration. We aimed to examine long-term outcomes following adult thymectomy.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We performed a propensity score-matched retrospective study of patients who underwent thymectomy or comparator cardiothoracic procedures, with follow-up extending to 10 years. Primary outcomes were all-cause mortality, new-onset malignancy, and autoimmune disease. Findings were compared with those reported in a recent study that has raised caution regarding thymic excision.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>We identified 425 patients who underwent thymectomy and 5401 controls who underwent coronary artery bypass or valve surgery. After matching, 398 patients remained in each group. Thymectomy was not associated with increased mortality at 5 years (HR = 0.63; 95% CI, 0.38–1.04) or 10 years (HR = 0.78; 95% CI, 0.54–1.13). Excluding reclassified thymic cancers, 5-year extrathymic malignancy incidence was 2.3% versus 1.8% (<i>p</i> = 0.54); no significant differences were observed in immune-related disease. Mortality was higher among patients with thymic malignancy. Among MG patients, only one event occurred, precluding reliable estimation.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Thymectomy was not associated with excess mortality or malignancy at 10 years compared with matched cardiothoracic controls. Indication-stratified analyses within the thymectomy cohort showed higher mortality for thymic malignancy, with insufficient events to assess outcomes in MG patients. These findings do not support a generalized survival penalty from thymectomy, though caution is warranted for incidental thymic removal.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 8","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/ene.70721","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148647659","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}

{"title":"3D Radiomic Texture Analysis of Quantitative Muscle MRI Enhances the Distinction Between Myotonic Dystrophy Type 1 and Charcot–Marie-Tooth Neuropathy Type 1A: A Proof-of-Concept Study","authors":"Louise Iterbeke,&nbsp;Lotte Huysmans,&nbsp;Kobe Bamps,&nbsp;Ronald Peeters,&nbsp;Veerle Goosens,&nbsp;Frederik Maes,&nbsp;Patrick Dupont,&nbsp;Kristl G. Claeys","doi":"10.1111/ene.70719","DOIUrl":"https://doi.org/10.1111/ene.70719","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Differentiating myogenic from neurogenic neuromuscular diseases (NMDs) can be clinically challenging. While quantitative muscle MRI (qMRI) with proton density fat fraction (PDFF,%) quantifies fat replacement, it misses micro-spatial patterns linked to underlying pathology. This study investigates whether qMRI with 3D radiomic texture analysis (TA) might improve differentiation between myogenic and neurogenic diseases, using myotonic dystrophy type 1 (DM1) and Charcot–Marie-Tooth neuropathy type 1A (CMT1A) as proof-of-concept models.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Thirty-three adults with DM1, 33 with CMT1A, and 33 matched healthy controls were included. qMRI on a 3T Philips Achieva system using a 6-point Dixon sequence generated PDFF(%) maps of the lower limbs, and a convolutional neural network performed 3D segmentation of 28 lower limb muscles. We extracted macroscopic features, including muscle volume, asymmetry, and disto-proximal gradients, alongside micro-spatial radiomic features (entropy, contrast, homogeneity) to quantify tissue heterogeneity.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Both patient cohorts exhibited higher PDFF(%) in all lower limb muscles compared to controls (<i>p</i> &lt; 0.001). DM1 predominantly involved the posterior compartment, while CMT1A targeted the anterolateral compartment with significantly steeper disto-proximal fat gradients (<i>p</i> &lt; 0.05). TA revealed higher entropy and contrast, and lower homogeneity in CMT1A compared to DM1, reflecting a more reticular pattern of fat infiltration vs. the confluent pattern in DM1.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>In this proof-of-concept study, 3D radiomic texture analysis of PDFF(%) maps revealed distinct spatial patterns of fat replacement in DM1 and CMT1A. Integrating radiomic and conventional qMRI features may enhance the non-invasive distinction between DM1 and CMT1A and warrants further investigation in a broader range of NMDs.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 8","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/ene.70719","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148647699","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}