European Journal of Neurology最新文献

{"title":"Correction to \"Re: \"Palliative Care in Europe: Safeguarding Compassion Amidst Changing End-Of-Life Policies and Expanding Access to Medically Assisted Dying Position Statement on Behalf of the European Academy of Neurology, the European Federation of Neurological Associations and OneNeurology\" by Rukavina Et al\".","authors":"","doi":"10.1111/ene.70794","DOIUrl":"https://doi.org/10.1111/ene.70794","url":null,"abstract":"","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 10","pages":"e70794"},"PeriodicalIF":4.3,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149227397","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}

{"title":"Re: “Palliative Care in Europe: Safeguarding Compassion Amidst Changing End-of-Life Policies and Expanding Access to Medically Assisted Dying Position Statement on Behalf of the European Academy of Neurology, the European Federation of Neurological Associations and OneNeurology”","authors":"Katarina Rukavina, Marianne de Visser, Elena Moro, Kailash Bhatia, Paul Boon, Jana Midelfart-Hoff, Matilde Leonardi, Irena Rektorova, Gereon R. Fink, Nathalie Nasr, Alicia Gonzalez-Martinez, Claudio L. A. Bassetti, Ulf Kallweit, Michael Crean, Patrick Cras, Ludo Vanopdenbosch, Astri Arnesen, Joke Jaarsma, Alexander Bernhard Kowski, Simone Veronese, David Oliver, the European Academy of Neurology, the European Federation of Neurological Associations, OneNeurology","doi":"10.1111/ene.70765","DOIUrl":"10.1111/ene.70765","url":null,"abstract":"<p>We would like to thank Pucci et al. for their interest in our position statement, in which we strongly advocate for timely, unrestricted access to high-quality palliative care for people living with long-term neurological conditions (PwLTNC) across Europe. In our statement, we emphasize that medically assisted dying (MAD) must never be introduced in response to deficiencies in care provision or as an alternative to comprehensive, multidisciplinary palliative care [<span>1</span>]. The joint development of this statement by the European Academy of Neurology (EAN) and two patient organizations (the European Federation of Neurological Associations, and OneNeurology) is particularly important, as this partnership ensured that patient perspectives were meaningfully and equitably represented, alongside those of clinicans [<span>1</span>].</p><p>Palliative care provides essential relief from suffering, supports patients and their families in an integrated manner, and helps preserve dignity and quality of life throughout the disease trajectory. Yet, it remains poorly integrated in the care for PwLTNC. In a recent survey, European Neurologists expressed their dissatisfaction with the availability of long-term healthcare, including palliative care, and in particular psychosocial and spiritual support offered to PwLTNC, their families and carers and the resources to provide healthcare for PwLTNC in medical emergencies [<span>2</span>]. Timely access to comprehensive, multidisciplinary palliative care across Europe remains a key unmet need.</p><p>We are grateful to the authors for providing the context of current Italian legislation. Nevertheless, it is important to emphasize that legal frameworks regulating MAD vary substantially across European countries. As a pan-European scientific society, the EAN does not seek to evaluate or validate national legal frameworks, but rather to emphasize the clinical, ethical, and patient-centered principles that should guide care for PwLTNC.</p><p>LTNC are well known to substantially impair quality of life for affected individuals and their families. PwLTNC may face profound threats to personhood and autonomy, often in the context of an uncertain prognosis [<span>3, 4</span>]. Indeed, LTNC are frequently represented among the underlying diagnoses in requests for medically assisted dying [<span>3, 5</span>]. Therefore, we fully agree with Pucci et al. that neurologists need to be appropriately equipped to lead such conversations with competence, sensitivity, and compassion, and to provide patients with balanced and comprehensive information about all available options, while holistically managing their physical, psychological, social, and spiritual needs. We thank Pucci et al. for contributing to this important discussion.</p><p><b>Katarina Rukavina:</b> conceptualization, writing – original draft, writing – review and editing. <b>Marianne de Visser:</b> writing – review and editing. <b>Elena Moro:</b> writing – review ","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 10","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13624699/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149079916","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":"Dissociating Fatigue and Fatigability in Multiple Sclerosis: The Role of Cognitive Processing Speed","authors":"Jeromy Hrabovecky,&nbsp;Sophie Elands,&nbsp;Guillermo Borragán,&nbsp;Antonin Rovai,&nbsp;Nicolas Gaspard,&nbsp;Philippe Peigneux,&nbsp;Xavier De Tiège,&nbsp;Hichem Slama,&nbsp;Mélanie Strauss","doi":"10.1111/ene.70769","DOIUrl":"10.1111/ene.70769","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Fatigue in people with multiple sclerosis (PwMS) is common and disabling, yet poorly understood. This study aimed to clarify the distinction between long-term fatigue and task-induced fatigability and their respective pathophysiological processes, and to examine the contribution of decreased cognitive processing speed (CPS) to the relationship between structural brain damage, fatigue, and fatigability.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Fatigue was induced in 34 PwMS (18–49 years) and 32 age−/sex-matched healthy controls (HCs, 24–44 years) using an individually calibrated task based on each participant's CPS. Perceived task-induced fatigue was assessed from pre- to post-task change in self-reported fatigue, and objective fatigability from within-task changes in performance, vigilance, and pupil diameter. Brain volumes and lesions were quantified on structural 3 T MRI.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>PwMS showed higher long-term fatigue and slower CPS (<i>p</i> &lt; 0.001). Once cognitive load was calibrated to individual CPS, objective markers of fatigability (performance, vigilance, pupil changes) no longer differed from HCs (all <i>p</i> &gt; 0.05), whereas perceived task-induced fatigue remained elevated. Both CPS and long-term fatigue—but not fatigability—were associated with putamen atrophy and lesion burden. Reduced CPS mediated the associations between putamen atrophy and long-term fatigue (indirect effect, 95% CI [−54.48, −0.97]).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>In PwMS, the association between structural brain damage and long-term fatigue is mediated by reduced CPS, whereas objective fatigability normalizes once cognitive load is calibrated to individual CPS. These findings support adapting cognitive load to each patient's processing capacity rather than reducing task demands uniformly to help mitigate the daily-life impact of fatigue.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 10","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-09-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13620844/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149073860","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":"Continuous Versus Routine EEG in Critically Ill Patients Without Primary Neurologic Condition: Analysis of a Randomized Controlled Trial","authors":"Gregory Lepeu,&nbsp;Jan Novy,&nbsp;Vincent Alvarez,&nbsp;Kaspar A. Schindler,&nbsp;Stephan Rüegg,&nbsp;A. O. Rossetti","doi":"10.1111/ene.70768","DOIUrl":"10.1111/ene.70768","url":null,"abstract":"<div>\u0000 <section>\u0000 <h3> Introduction</h3>\u0000 <p>Continuous EEG (cEEG) is increasingly used in critically ill patients, driven by its superior seizure detection sensitivity over routine EEG (rEEG). However, cEEG is resource-demanding, and whether its use translates into improved clinical outcomes in patients without primary neurologic conditions remains unknown.</p>\u0000 </section>\u0000 <section>\u0000 <h3> Methods</h3>\u0000 <p>This is a post hoc analysis of the multicenter randomized CERTA trial, in which critically-ill adults were randomized 1:1 to cEEG or two rEEG recordings. Six-month mortality represented the primary outcome, while exploratory outcomes included functional outcome (Cerebral Performance Categories, CPC), detection of seizures/status epilepticus, ictal–interictal continuum features, medication changes, and infections within 4 weeks. Analysis was restricted to patients without primary neurologic injury.</p>\u0000 </section>\u0000 <section>\u0000 <h3> Results</h3>\u0000 <p>Among 364 enrolled patients, 76 lacked a primary neurologic condition (cEEG: <i>n</i> = 34, rEEG: <i>n</i> = 42). Continuous EEG was not associated with mortality (<i>p</i> = 0.970) or functional outcome (CPC 1–2; <i>p</i> = 0.893). No seizures or status epilepticus were detected in either group. Rates of EEG periodic patterns, ictal–interictal continuum features, medication changes, and infections were comparable between groups.</p>\u0000 </section>\u0000 <section>\u0000 <h3> Conclusion</h3>\u0000 <p>In critically ill adults without a primary neurologic condition, cEEG was not associated with measurable benefits in survival, neurological recovery, or epileptiform activity detection compared with repeated rEEG, in a cohort without detected electrographic seizures or status epilepticus. Pending larger, ideally prospective comparative studies, repeated routine EEG may represent a reasonable alternative in resource-limited settings.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 10","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13617542/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149069567","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":"Serum GFAP and Age Accurately Distinguish AQP4-IgG Positive and Double Seronegative NMOSD From MOGAD After a Recent Attack","authors":"Manon Rival,&nbsp;Fabien Rollot,&nbsp;Sabine Laurent-Chabalier,&nbsp;Romain Casey,&nbsp;Illiasse El Bahi,&nbsp;Guillaume Brocard,&nbsp;Romain Marignier,&nbsp;Sophie Trouillet-Assant,&nbsp;Sandra Vukusic,&nbsp;Agnès Fromont,&nbsp;David Axel Laplaud,&nbsp;Guillaume Mathey,&nbsp;Elisabeth Maillart,&nbsp;Laure Michel,&nbsp;Aurélie Ruet,&nbsp;Pierre Labauge,&nbsp;Xavier Ayrignac,&nbsp;Hélène Zephir,&nbsp;Jonathan Ciron,&nbsp;Jérôme de Sèze,&nbsp;Arnaud Kwiatkowski,&nbsp;Caroline Papeix,&nbsp;Pierre Branger,&nbsp;Christine Lebrun-Frenay,&nbsp;Eric Berger,&nbsp;Pierre Clavelou,&nbsp;Olivier Heinzlef,&nbsp;Jean Pelletier,&nbsp;Abdullatif Al-Khedr,&nbsp;Olivier Casez,&nbsp;Bertrand Bourre,&nbsp;Alain Créange,&nbsp;Thomas David,&nbsp;Laurent Magy,&nbsp;Jean-Philippe Camdessanche,&nbsp;Inès Doghri,&nbsp;Amélie Dos Santos,&nbsp;Thibault Mura,&nbsp;Hanane Agherbi,&nbsp;Eric Thouvenot,&nbsp;OFSEP Group and NOMADMUS Investigators","doi":"10.1111/ene.70767","DOIUrl":"https://doi.org/10.1111/ene.70767","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Serum neurofilament-light chain (sNfL) and glial fibrillary acidic protein (sGFAP) are associated with multiple sclerosis (MS) activity. This study aimed to identify patient- and disease-level determinants of sNfL and sGFAP and to evaluate their ability to support differential diagnosis in neuromyelitis optica (NMO).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>sNFL and sGFAP levels were measured in 640 patients from the OFSEP cohort (88 NMO spectrum disorder (NMOSD) with anti-aquaporin-4 antibodies [AQP4-NMOSD], 28 double-seronegative NMOSD [DN-NMOSD], 61 myelin oligodendrocyte glycoprotein antibody–associated disease [MOGAD], 64 clinically isolated syndrome [CIS], 237 relapsing–remitting MS [RRMS], and 162 primary progressive MS [PPMS]). The association between log[sNFL] and log[sGFAP] and age, sex, time since relapse, EDSS, oligoclonal bands, and MRI characteristics was analyzed by multiple linear regression. The diagnostic performance of sNfL and sGFAP in discriminating between NMOSD and MS subgroups was evaluated using multivariable logistic regression and area under the curve (AUC).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>For each group, specific associations of sNFL and sGFAP levels with patient characteristics were observed. Clearest associations were observed in AQP4-NMOSD patients. After a recent attack (≤ 90 days), multivariate analysis revealed that sGFAP and age discriminated AQP4-NMOSD from MOGAD (AUC = 0.939 [0.910;0.982]), AQP4-NMOSD and DN-NMOSD from MOGAD (AUC = 0.863 [0.776;0.977]), and AQP4-NMOSD from all other groups (AUC = 0.950 [0.918;0.987]). Diagnostic probability heatmaps based on sGFAP and age illustrate the probability of each diagnosis relative to others for bedside interpretation.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>After an acute attack of NMOSD, risk score based on sGFAP and age may guide the management of patients before autoantibody status is available.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 10","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/ene.70767","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149048509","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":"Cannabidiol in Adults With Lennox–Gastaut Syndrome: Real-World Experience","authors":"Pyae Aung,&nbsp;Debbie Miller,&nbsp;Emily Sewell,&nbsp;Laura Mantoan Ritter,&nbsp;Evangelia Theochari,&nbsp;Ioannis Stavropoulos,&nbsp;Robert Delamont,&nbsp;Mark P. Richardson,&nbsp;Joel S. Winston,&nbsp;Lina Nashef","doi":"10.1111/ene.70760","DOIUrl":"https://doi.org/10.1111/ene.70760","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>We report a single centre experience of cannabidiol use as adjunctive treatment in adults with Lennox–Gastaut Syndrome (LGS).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Retrospective review of adults with LGS treated with cannabidiol with clinical data collected from electronic records and caregiver standardised seizure diaries with seizure-related outcomes assessed at baseline, 6 months, and last follow-up.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Seventy-nine adults (median age 29 years; 40 male) were included. Median follow-up was 41 months. Four patients died unrelated to cannabidiol treatment. Sixty-seven (86%) remained on treatment at last follow-up. Across all seizure types, 59.5% achieved ≥ 50% reduction at 6 months and 67.1% at last follow-up. Among those with ‘Drop Seizures’ (<i>n</i> = 78), 50% achieved &gt; 50% reduction at 6 months and 61.5% at last follow-up. ‘Drop Seizure’ responder rates were numerically higher with clobazam &gt; 5 mg/day, but with no statistically significant dose–response relationship seen. Seizure-free days increased (baseline 5.7/month; last follow-up 12.5/month). Seizure-related hospital admissions and injuries declined. Cognitive or behavioural improvements were reported in 48 patients (60.8%); there was no statistically significant association with achieving ≥ 50% seizure reduction. Drowsiness (<i>n</i> = 33) was the most frequent adverse event often related to drug–drug interactions. Diarrhoea (<i>n</i> = 24) was more common than previously reported. Side-effects necessitated ASM adjustments in 51 patients. Fourteen were able to withdraw one or more ASMs.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>CBD was associated with sustained effectiveness and good tolerability in adults with LGS, with high retention and improvement in seizure and other clinically meaningful outcomes. Adverse effects, mainly interaction-related, were usually managed with concomitant medication adjustments.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 10","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/ene.70760","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149048510","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":"Neural Correlates of Apathy in Multiple System Atrophy: A Clinical and Perfusion Imaging Study","authors":"Yuki Nakagawa,&nbsp;Atsuhiko Sugiyama,&nbsp;Yoshikazu Nakano,&nbsp;Hajime Yokota,&nbsp;Shigeki Hirano,&nbsp;Masahiro Namiki,&nbsp;Tatsuya Yamamoto,&nbsp;Yoshitaka Yamanaka,&nbsp;Shoichi Ito,&nbsp;Masahiro Mori","doi":"10.1111/ene.70766","DOIUrl":"https://doi.org/10.1111/ene.70766","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Apathy is a frequent nonmotor symptom of multiple system atrophy (MSA) that worsens quality of life. Although frontostriatal dysfunction has been implicated in apathy, its neural basis in MSA remains insufficiently characterized.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Objectives</h3>\u0000 \u0000 <p>To identify the neural substrates of apathy associated with MSA-specific neurodegenerative processes.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>This retrospective cross-sectional study included 76 patients with cognitively preserved clinically established/probable MSA who underwent clinical and neuropsychological assessments, including the Apathy Scale and a separate depression measure, magnetic resonance imaging, and cerebral blood flow single-photon emission computed tomography. Clinical variables, gray matter volume, and voxel-wise perfusion were compared between apathy and non-apathy groups. Multivariate logistic regression was used to analyze the independent association of the significant perfusion cluster with apathy, and seed-based covariance analysis was used to explore network alterations.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Apathy was present in 55.3% of patients and did not differ between motor subtypes. Compared with the non-apathy group, the apathy group showed lower visuospatial scores and frequency of neurogenic orthostatic hypotension, similar depression scores and gray matter volume, and reduced perfusion in the medial prefrontal cortex on voxel-wise perfusion analysis. These findings remained independently associated with apathy after adjustment for clinical variables. Seed-based covariance analysis suggested reduced positive covariance between the medial prefrontal cortex and cerebellar regions, predominantly in the posterior lobe.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Apathy was common in MSA even with relatively preserved global cognition and distinct from depression. In addition to medial frontostriatal dysfunction, exploratory findings suggested that mechanisms related to cerebellar cognitive affective syndrome may contribute to its pathophysiology.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 10","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/ene.70766","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149048483","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":"Impact of a Specialized Care Pathway for ICU Patients With Disorders of Consciousness: Observational Insights From an Expert Center","authors":"Clément Caiazzo,&nbsp;Estelle Pruvost-Robieux,&nbsp;Ghazi Hmeydia,&nbsp;Eleonore Bouchereau,&nbsp;Camille Legouy,&nbsp;Charlotte Calligaris,&nbsp;Julie Lévi-Strauss,&nbsp;Catherine Oppenheim,&nbsp;Sarah Benghanem,&nbsp;Caroline Schimpf,&nbsp;Martine Gavaret,&nbsp;Tarek Sharshar,&nbsp;Bertrand Hermann","doi":"10.1111/ene.70536","DOIUrl":"https://doi.org/10.1111/ene.70536","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Management of lasting disorders of consciousness (DoC) is complex and sometimes requires referral of patients to an expert team. We aimed to evaluate the impact of such specialized expert assessment through the description of a single-center experience.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Method</h3>\u0000 \u0000 <p>Monocentric study including all patients with DoC referred to a tertiary neuroICU for multimodal clinical, electrophysiological, and MRI assessment allowing diagnostic and therapeutic proposals and multidisciplinary prognostic prediction. The primary endpoint was the patients' actual outcome, assessed by 3-month functional disability and 1-year mortality, according to the predicted prognosis.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Between July 2017 to June 2024, 124 patients (median age 61 [49–70] years, 67% of men) were referred from 43 departments of 37 primary hospitals. Most frequent etiologies were post-anoxic encephalopathy (62%) and secondary systemic brain injuries (15%). At a median of 23 [15–33] days after DoC onset, predicted prognosis was good in 20 (16%) patients, intermediate in 41 (33%), and poor in 63 (51%). Three-month GOS-E was 3 [1–3], 2 [1–3], and 1 [1] (<i>p</i> &lt; 0.001) and 1-year mortality was 30%, 41%, and 82% (<i>p</i> &lt; 0.001), respectively. Withdrawal of life-sustaining therapies increased with worsening predicted prognosis (38%, 41%, and 65%, respectively, <i>p</i> &lt; 0.001), but functional disability and mortality remained higher in the poor prognosis group (<i>p</i> &lt; 0.003 and <i>p</i> &lt; 0.039, respectively) even after excluding patients with those decisions.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>Specialized consciousness assessment impacts the ethical decisions made by primary centers and helps refine the prediction of recovery but fails to achieve highly accurate individual prediction in a population with overall poor survival and functional prognosis.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 10","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/ene.70536","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149048508","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":"Genetic ALS—Progressive Sleep Defects Precede the Onset of Clinical Disease","authors":"Christina Lang,&nbsp;Simon J. Guillot,&nbsp;Antje Knehr,&nbsp;Patrick Weydt,&nbsp;Johannes Dorst,&nbsp;Hayrettin Tumani,&nbsp;Albert C. Ludolph,&nbsp;Luc Dupuis,&nbsp;Matei Bolborea","doi":"10.1111/ene.70755","DOIUrl":"10.1111/ene.70755","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>The onset of amyotrophic lateral sclerosis (ALS) is preceded by non-motor signs detectable many years before motor symptom onset. We recently showed that early ALS patients and presymptomatic carriers of ALS-associated gene mutations display altered sleep macroarchitecture, characterised by increased wakefulness and decreased non-REM sleep, together with altered microarchitecture, particularly reduced slow oscillations and sleep spindles. Here we aimed at determining whether sleep alterations are progressive in premanifest carriers of ALS-associated gene mutations.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>This longitudinal observational cohort study included presymptomatic first-degree relatives of ALS patients carrying pathogenic gene mutations (<i>n</i> = 18) and noncarrier family members (<i>n</i> = 3). Sleep macroarchitecture and sleep microarchitecture were assessed using standardised polysomnography and electroencephalographic analyses at baseline and in a follow-up examination after 2.3 ± 1.5 years.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>In all carriers, sleep alterations were more severe at follow-up compared to the initial examination, for both macro- and microarchitecture. The progression of sleep alterations was seemingly independent of proximity to motor onset, whether evaluated by increased neurofilament levels or phenoconversion during the study period.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Sleep macro- and microarchitectural abnormalities progressively worsen during the premanifest phase of ALS. These findings suggest that polysomnography-derived sleep measures and their neurochemical correlates may serve as sensitive, noninvasive surrogate biomarkers of disease progression in clinically silent ALS. This could support disease prevention, for example, by the antisense oligonucleotide tofersen in patients carrying SOD1 mutations.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 9","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-09-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13591987/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149012277","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":"Chitinases in Tear Fluid of Patients With Amyotrophic Lateral Sclerosis","authors":"Lara Wenz,&nbsp;Lena-Sophie Scholl,&nbsp;Nya Reinhardt,&nbsp;Ricarda von Heynitz,&nbsp;Vincent Gmeiner,&nbsp;Petra Rau,&nbsp;Philipp Jie Müller,&nbsp;Andreas W. Wolff,&nbsp;Emily Feneberg,&nbsp;Antonia F. Demleitner,&nbsp;Paul Lingor","doi":"10.1111/ene.70757","DOIUrl":"https://doi.org/10.1111/ene.70757","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Chitinases, including chitotriosidase (CHIT1) and chitinase-3-like protein 1 (CHI3L1), are markers of neuroinflammation, a key process in amyotrophic lateral sclerosis (ALS). Tear fluid (TF) can be collected non-invasively and may represent a promising alternative to CSF or blood to study chitinases.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>TF was collected from 50 ALS patients and 50 control subjects using Schirmer strips. CHIT1 and CHI3L1 levels in TF, serum, and CSF were quantified using ELISA. Serum NfL was measured using SIMOA. The frequency of a 24 bp-duplication polymorphism in the CHIT1 gene influencing CHIT1 expression was assessed by PCR.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>No group differences in the distribution of the CHIT1 polymorphism were detected. Carriers of the polymorphism in both ALS and controls showed lower CHIT1 levels in serum and TF. CHI3L1 levels in TF were higher in ALS patients compared to controls (<i>p</i> = 0.007), consistent with changes in CSF but not serum. In ALS, males showed higher TF CHIT1 values compared to females (<i>p</i> = 0.009). Combining TF chitinase values with serum NfL values improved discrimination between ALS and controls.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Chitinases are detectable in TF, and CHI3L1 levels recapitulate changes observed in CSF, highlighting its potential for non-invasive longitudinal assessment. Furthermore, chitinase values in TF, together with serum NfL, may act complementarily by capturing distinct aspects of the disease, neuroinflammation and axonal damage. These results suggest TF chitinases and serum NfL could complementarily contribute to the diagnosis and monitoring of the disease, and call for further evaluation of TF as a biomarker source in ALS.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 9","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-09-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/ene.70757","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148994250","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}