European Journal of Neurology最新文献

{"title":"Clinical Variability and Genotype-Driven Outcomes in CHRND-Related Congenital Myasthenic Syndrome","authors":"David Muhmann,&nbsp;Göknur Haliloğlu,&nbsp;Maria Antonia Grimalt,&nbsp;Damjan Osredkar,&nbsp;Ana Vesperinas Castro,&nbsp;Silvia Cerezo Corredera,&nbsp;Angela Abicht,&nbsp;Adalet Elçin Yıldız,&nbsp;Johann Böhm,&nbsp;Ulrike Schara-Schmidt,&nbsp;Anja Troha Gergeli,&nbsp;Berta Estevez-Arias,&nbsp;Elena Cortes Vicente,&nbsp;Andres Nascimento,&nbsp;Adela Della Marina,&nbsp;Daniel Natera-de Benito,&nbsp;Andreas Roos","doi":"10.1111/ene.70742","DOIUrl":"https://doi.org/10.1111/ene.70742","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Congenital myasthenic syndromes (CMS) caused by pathogenic variants in <i>CHRND</i>, encoding the δ-subunit of the nicotinic acetylcholine receptor (AChR), are rare, and data on genotype–phenotype correlations and long-term outcomes are limited.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We performed a retrospective, multicenter study of nine patients with genetically confirmed <i>CHRND</i>-related CMS from specialized neuromuscular centers. Clinical, electrophysiological, genetic, and therapeutic data were systematically collected. All diagnoses were established by exome sequencing during routine clinical work-up.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Eight patients were compound heterozygous and one was homozygous for pathogenic <i>CHRND</i> variants, including nonsense, missense, splice-site variants, and one microdeletion. Disease onset ranged from the neonatal period (<i>n</i> = 7) to adolescence (<i>n</i> = 2). Three patients were followed longitudinally for 22–43 years. Ocular involvement, particularly ptosis and ophthalmoparesis, was present in all patients. Generalized fatigable weakness was common, whereas bulbar and respiratory involvement occurred in a subset and reflected overall disease severity. Genotypes including a null allele or a homozygous missense variant tended to be associated with more severe phenotypes, while compound heterozygous missense variants were linked to a broader and generally milder spectrum, sometimes limited to ocular symptoms. Long-term outcomes ranged from minimal symptoms under therapy to severe motor impairment with respiratory insufficiency, highlighting substantial interindividual variability.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>This study expands the phenotypic and genotypic spectrum of <i>CHRND</i>-related CMS and underscores the critical role of genotype in determining disease severity. Comprehensive genetic testing, longitudinal phenotyping, and genotype-informed management are essential for optimal diagnosis and care in this rare disorder.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 9","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/ene.70742","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148816528","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":"Shared Spectral Parameters Between EEG and Basal Ganglia LFP Distinguish Sleep-Specific Oscillations in Parkinson's Disease","authors":"Lisa Hirt,&nbsp;Ruby Martini,&nbsp;Siqun Tang,&nbsp;Michael Summers,&nbsp;Lesile West,&nbsp;Clete Kushida,&nbsp;Stephen V. Gliske,&nbsp;Dulce Maroni,&nbsp;Younghoon Nho,&nbsp;Casey H. Halpern,&nbsp;Aviva Abosch,&nbsp;John A. Thompson","doi":"10.1111/ene.70733","DOIUrl":"https://doi.org/10.1111/ene.70733","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>In Parkinson's disease (PD), sleep-related oscillatory dynamics between basal ganglia and cortical nodes may inform sleep dysfunction. The subthalamic nucleus (STN) is highly interconnected with human sleep circuitry and is a current target of interest for sleep neuromodulation.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>To investigate correlated cortical–subcortical activity, we recorded simultaneous PSG and STN local field potentials (LFP) in 19 patients undergoing deep brain stimulation (DBS) for PD. We evaluated the correlation in spectral power between STN-LFP and EEG during polysomnogram (PSG)-defined stages of sleep. In addition, we analyzed aperiodic 1/f spectral parameters (exponent and offset), which have been identified as markers of arousal across sleep stages. Finally, we trained classifiers on aperiodic features to test whether 1/f structure alone could recover PSG-defined sleep stages from EEG and STN-LFP.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Results revealed a significant interaction between recording modality and sleep stage. During wakefulness and REM sleep, STN-LFP exhibited a significantly steeper spectral slope compared to scalp EEG. However, this dissociation diminished with sleep depth; slopes appeared more similar during N2 and reversed during N3, where EEG became steeper than LFP. Across subjects, the aperiodic component showed robust state dependence and modality differences with STN-LFP maintaining higher exponents overall. STN-LFP aperiodic features alone classified sleep stages above chance and outperformed scalp EEG, approaching the combined-modality ceiling.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>These findings highlight distinct subcortical electrophysiological signatures in sleep-dependent regulation of neural arousal across brain networks in PD. These insights offer potential biomarkers for both invasive and non-invasive forms of closed-loop neuromodulation strategies targeting sleep dysfunction.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Trial Registration</h3>\u0000 \u0000 <p>ClinicalTrials.gov identifier: NCT04620551</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 9","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-08-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/ene.70733","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148816451","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":"Climate Change, Neurological Disorders and Brain Health: To-Know Concepts and Future Actions by the EAN Environmental Influences in Neurology Task Force","authors":"Edoardo Caronna,&nbsp;Sanjay M. Sisodiya,&nbsp;Patricia Pozo-Rosich,&nbsp;Antonia Boesch,&nbsp;Jacques Reis,&nbsp;Anita Arsovska,&nbsp;Elisa Baldin,&nbsp;Cristian Falup-Pecurariu,&nbsp;Thomas Foiadelli,&nbsp;Maria Gogou,&nbsp;Matilde Leonardi,&nbsp;Nathalie Nasr,&nbsp;Justyna Paprocka,&nbsp;Mohamed Salama,&nbsp;Ambra Stefani,&nbsp;Antonio Toscano,&nbsp;Claudio L. A. Bassetti,&nbsp;Paul Boon,&nbsp;Elena Moro,&nbsp;Ulf Kallweit","doi":"10.1111/ene.70687","DOIUrl":"https://doi.org/10.1111/ene.70687","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and Purpose</h3>\u0000 \u0000 <p>While the estimation of the climate change effects is dependent on the models used, there is a growing consensus that emissions of greenhouse gases (GHG) cause global warming, resulting in the acceleration of climate change. The degree to which climate change is going to affect the nervous system of healthy individuals and how it may lead to an increase in the prevalence and burden of neurological disorders is presently incompletely understood and studied. It is imperative that the neurological community increases its awareness about climate change and strives for a clear evidence-based consensus on the relationship between climate change and the negative consequences on brain health and neurological disorders.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Part I provides a narrative overview of climate change in neurology, whereas Part II focuses on climate change action in neurology.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>We provide clinicians and neuroscience stakeholders with tools to understand key concepts at the interface between climate change, neurological disorders, and brain health. Moreover, this paper states priorities in this field for the European Academy of Neurology, through the work of the Environmental Influences in Neurology Task Force, presenting 10 operational areas adapted from the WHO <i>operational framework for building climate-resilient and low-carbon health systems</i>.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>In the frame of its Brain Health Mission, the European Academy of Neurology is committed to supporting a neurology that is able to understand the impact of climate change and become a climate-resilient neurology to promote health in general and brain health specifically.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 8","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-08-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/ene.70687","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148785192","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":"SOD1 Variants in Patients With Amyotrophic Lateral Sclerosis in Central Eastern Europe: From Genetic Testing to SOD1 Targeted Therapy","authors":"Magui Khazaal,&nbsp;Paula Stretavská,&nbsp;Magdalena Kuźma-Kozakiewicz,&nbsp;Krzysztof Nieporęcki,&nbsp;Adam Betík,&nbsp;Eva Vlčková,&nbsp;Monika Turčanová Koprušáková,&nbsp;Robert Petrovic,&nbsp;Hakan Cetin,&nbsp;Omar Keritam,&nbsp;Wolfgang Löscher,&nbsp;Christian Eggers,&nbsp;Stephan Iglseder,&nbsp;Radoslav Orenčák,&nbsp;Mária Judit Molnár,&nbsp;Zoltán Grosz,&nbsp;Tamás Szlepák,&nbsp;Blaž Koritnik,&nbsp;Magdalena Anna Koszewicz,&nbsp;Daniel Baumgartner,&nbsp;Lenka Slachtova","doi":"10.1111/ene.70717","DOIUrl":"https://doi.org/10.1111/ene.70717","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Amyotrophic lateral sclerosis (ALS) is one of the most devastating fatal motor neuron diseases, characterized by progressive degeneration of motor neurons in the brain and spinal cord. A significant advance in ALS therapy was achieved with the recent European Medicines Agency approval of Tofersen, the first antisense oligonucleotide (ASO) specifically targeting SOD1 mRNA, a key genetic determinant of the disease. Yet, despite its clinical relevance, data on <i>SOD1</i>-ALS in Central Eastern Europe remain scarce.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Here, we present a multicentric study across six countries—Austria, Czechia, Poland, Hungary, Slovakia, and Slovenia—representing approximately 16% of the European Union's population. We report all pathogenic, likely pathogenic, and uncertain <i>SOD1</i> variants, along with the phenotypic features, including heritability, age, site of onset, and survival. We also assessed the availability of genetic testing, counseling, and access to Tofersen therapy across the region.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Out of 1200 patients with confirmed ALS, we identified 24 distinct pathogenic <i>SOD1</i> variants in a total of 67 patients (median age at onset 47 [40–55] years), of whom 65.7% had familial ALS (fALS) and 34.3% had sporadic ALS (sALS). We characterized the associated phenotypes and reported that 42 patients are currently receiving Tofersen therapy.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>This study provides the first comprehensive overview of <i>SOD1</i>-ALS in Central Eastern Europe. Our findings underscore the importance of genetic testing and counseling, as well as equitable access to targeted therapies such as Tofersen to advance patient-specific care in this region.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 8","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-08-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/ene.70717","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148784096","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":"Association Between PM2.5 Components and Cerebral Hypoperfusion: A Mediation Role of Inflammatory Biomarkers","authors":"Xingyan Xu,&nbsp;Xinyue Lu,&nbsp;Nan Wang,&nbsp;Liuyan Zheng,&nbsp;Jianhui Guo,&nbsp;Tongjin Xu,&nbsp;Yiming Guo,&nbsp;Hao Li,&nbsp;Yi Yang,&nbsp;Le Yang","doi":"10.1111/ene.70716","DOIUrl":"10.1111/ene.70716","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Cerebral hypoperfusion, an acute manifestation of intracranial arterial stenosis (ICAS), is particularly concerning among older East Asian adults given its high prevalence. Systemic inflammation triggered by PM_2.5 exposure has been linked to cerebrovascular diseases, yet evidence on its specific components of hypoperfusion in this vulnerable group remains limited. We explore associations between PM_2.5 and its components exposure with cerebral hypoperfusion, and assess mediation by inflammatory indicators.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We analyzed 967 patients with symptomatic ICAS at the First Affiliated Hospital of Soochow University. Hypoperfusion was evaluated using multimodal CT imaging and quantified with MIStar software. Three-year exposure to PM_2.5 and its components was estimated using the TAP database. Associations were evaluated using generalized additive models and restricted cubic spline regression, while weighted quantile sum regression assessed mixture effects. Mediation analysis quantified inflammatory markers' roles in these relationships.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>PM_2.5 was significantly association with cerebral hypoperfusion (OR = 4.344, 95% CI: 3.323–5.734), with black carbon (BC) showing the strongest association (OR = 4.396, 95% CI: 3.327–5.869), followed by sulfate (SO₄²⁻) (OR = 4.359), organic matter (OM) (OR = 4.278), nitrate (NO₃⁻) (OR = 3.552), and ammonium (NH₄⁺) (OR = 3.313). OM (44.2%) and BC (42.2%) contributed 86% of the total mixture effect. Neutrophils mediated 5.37% of the total effect between PM_2.5 and cerebral hypoperfusion, whereas leukocytes (4.30%) and monocytes (2.73%) demonstrated significant mediation effects.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>Prolonged exposure to PM_2.5 and its components, particularly OM and BC, was associated with increased cerebral hypoperfusion risk, partially mediated by inflammatory responses. These findings highlight the need for component-targeted air quality policies to reduce cerebrovascular risk in vulnerable populations.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 8","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-08-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/ene.70716","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148722761","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":"Reply to: “A Network Meta-Analysis of Hereditary Transthyretin Amyloidosis With Polyneuropathy Supports a Class Effect, Not a Ranking of Individual Drugs”","authors":"Gonçalo Nuno da Silva Duarte,&nbsp;Tiago Luís Gonçalves Neves Machado,&nbsp;Filipe André Brogueira Rodrigues,&nbsp;Isabel Conceição,&nbsp;Davide Pareyson,&nbsp;João Nuno Marques Parracho Guerra da Costa,&nbsp;the task force of the EAN/PNS joint guideline for the management of neurological manifestations in hereditary transthyretin amyloidosis","doi":"10.1111/ene.70732","DOIUrl":"10.1111/ene.70732","url":null,"abstract":"&lt;p&gt;We thank Streicher and colleagues for their careful reading of our network meta-analysis [&lt;span&gt;1, 2&lt;/span&gt;] and welcome the opportunity to respond. We agree on the central message: transthyretin gene silencers were more efficacious than stabilizers, a large, consistent, and biologically coherent effect. We also agree that the randomized evidence does not establish a definitive hierarchy among the individual gene silencers.&lt;/p&gt;&lt;p&gt;Where we differ is in the inference drawn from that agreement. That a comparison is indirect and of low certainty is a reason to interpret a ranking cautiously, not to omit it. Treatment rankings, namely the SUCRA values and the full rank distributions in figures 1B–3B, are standard, recommended outputs of Bayesian network meta-analysis [&lt;span&gt;3&lt;/span&gt;], and reporting them with their uncertainty is more transparent, not less, than suppressing them. Throughout, we reported the overlapping ranking credible intervals, graded the key cross-drug comparisons as low certainty using GRADE, and framed the findings as hypothesis-generating. The ranking was not presented as established fact, but as a probabilistic ordering carrying explicit, and considerable, uncertainty; omitting it would discard information the randomized evidence legitimately provides.&lt;/p&gt;&lt;p&gt;The specific methodological points reinforce, rather than undermine, this cautious reading. The two primary outcomes diverge: on Norfolk-QoL-DN we found no significant differences between patisiran, vutrisiran, and eplontersen, and the ordering emerged only on mNIS+7. We reported both precisely so this would be visible. Harmonizing the two versions of the mNIS+7 through standardized mean differences is a pre-specified, conventional approach to related but non-identical instruments, as we noted in our review, for which we downgraded certainty for indirectness and imprecision. The timing of assessments is a genuine limitation, which we flagged; because silencer effects accrue with continued treatment, it would, if anything, understate a later-assessed agent.&lt;/p&gt;&lt;p&gt;We thank the correspondents for identifying three discrepancies in table 1 of our paper [&lt;span&gt;1&lt;/span&gt;]. Two are transcription errors: the NEURO-TTR disease-stage entry should read Stage 1, 67%; Stage 2, 33% [&lt;span&gt;4&lt;/span&gt;], and the NEURO-TTRansform entry inadvertently reported the polyneuropathy disability score rather than the Coutinho stage (correct values: Stage 1, 79%; Stage 2, 21%) [&lt;span&gt;5&lt;/span&gt;]. Both are confined to table 1; the transitivity assessment and meta-regressions used the correct values, so the estimates, rankings, GRADE assessments, and disease-stage heterogeneity analyses are unaffected, and a Correction has been published [&lt;span&gt;5&lt;/span&gt;]. The third figure, the disease-duration mean of 46.8 months, is not an error, but the mean duration from diagnosis reported in the eplontersen Clinical Study Report.&lt;/p&gt;&lt;p&gt;We agree that the choice among gene silencers awaits head-to-head trials. Our analy","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 8","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-08-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13454732/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148701105","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":"Beyond Venous Inflammation: Vessel Wall Imaging of Intracranial Arteries in Neuro-Behçet's Syndrome","authors":"Esra Kochan Kizilkilic,&nbsp;Bora Korkmazer,&nbsp;Nursena Erener,&nbsp;Ahmet Kursat Karaman,&nbsp;Abdullah Arcan,&nbsp;Serdar Arslan,&nbsp;Aksel Siva,&nbsp;Emire Seyahi,&nbsp;Ugur Uygunoglu,&nbsp;Osman Kizilkilic","doi":"10.1111/ene.70718","DOIUrl":"10.1111/ene.70718","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Neuro-Behçet syndrome (NBS) is a heterogeneous inflammatory disorder in which venous involvement is well recognized, while intracranial arterial pathology remains insufficiently characterized. Conventional luminal imaging methods are limited in detecting early or isolated arterial wall abnormalities. Vessel wall imaging (VWI) may provide additional insights into arterial involvement in NBS.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Thirty-nine NBS patients (27 men, 12 women; mean age 38.52 ± 9.54 years) underwent intracranial VWI; 16 were imaged during the attack period and 23 during the attack-free period. A total of 1170 artery segments were systematically evaluated for wall thickening patterns and contrast enhancement. VWI findings were analyzed in relation to imaging timing, radiological subtype, MR angiography findings, and EDSS.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Vessel wall involvement was found in 16 patients (41%), mainly in the posterior circulation (81%). Concentric wall thickening without enhancement was the most common pattern (80.48%). Vessel wall involvement was significantly more frequent in patients imaged during the attack period compared with those imaged in the attack-free period (68.8% vs. 21.7%; <i>p</i> = 0.007). Vessel wall enhancement was present only in the attack period (<i>p</i> = 0.022). Notably, higher EDSS scores were significantly associated with multisegmental posterior circulation involvement, particularly when ≥ 3 segments were affected (<i>p</i> = 0.015; <i>p</i> = 0.024).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>Intracranial arterial wall involvement is detectable by VWI in a substantial proportion of NBS patients, predominantly in the posterior circulation with a concentric thickening pattern. These findings suggest that arterial wall pathology may be more common than previously recognized and that VWI provides complementary diagnostic information beyond conventional luminal imaging.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 8","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-08-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13448507/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148683714","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":"Decoding Prodromal Lewy Body Disease: Clinical Differences Between Isolated REM Sleep Behavior Disorder and Hyposmia With Dopamine Transporter Deficit","authors":"Luke Vikram Banerjee,&nbsp;Jacopo Pasquini,&nbsp;Robin Henderson,&nbsp;Nicola Pavese,&nbsp;Kirstie N. Anderson","doi":"10.1111/ene.70724","DOIUrl":"10.1111/ene.70724","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Lewy body diseases (LBDs) are heterogeneous, and this variability is already evident in the prodromal phase. Several frameworks propose that prodromal heterogeneity reflects distinct phenotypic and biological subtypes, supported by differences in clinical profiles, imaging, and α-synuclein biomarkers. We provide a detailed clinical characterization of two enriched prodromal cohorts: isolated REM Sleep Behavior Disorder (iRBD) and Hyposmia with Dopamine Transporter Deficit.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>This cross-sectional study included 360 participants with iRBD, 1101 with hyposmia and abnormal dopamine transporter imaging and 283 healthy controls from the Parkinson's Progression Markers Initiative. Using the earliest assessments available, we compared the Montreal Cognitive Assessment (MoCA), Scales for Outcomes in Parkinson's Disease Autonomic Dysfunction (SCOPA-AUT) and Movement Disorder Society Unified Parkinson's Disease Rating Scale (MDS-UPDRS) Parts I-III. Group differences were assessed using age and sex-adjusted permutation testing. Secondary descriptive analysis identified the individual items contributing most to between-group differences.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Both prodromal cohorts had significantly worse scores than healthy controls in all assessments. Compared with hyposmia, iRBD was associated with higher SCOPA-AUT and MDS-UPDRS Part I scores, driven mainly by constipation and urinary symptoms.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>In prodromal Lewy body disease, iRBD is associated with a statistically significant excess of autonomic symptom burden compared with hyposmia with dopamine transporter deficit, with the largest item-level differences in urinary and constipation measures.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 8","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-08-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13446031/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148677764","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":"Improvement in Quality of Life and Non-Motor Symptoms Associated With OFF Episodes in People With Parkinson's Disease Treated With Inhaled Levodopa","authors":"Diego Santos García,&nbsp;María Cerdán Sánchez,&nbsp;Rosa Yáñez Baña,&nbsp;Iria Cabo,&nbsp;Maria Icíar Cimas Hernando","doi":"10.1111/ene.70731","DOIUrl":"10.1111/ene.70731","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>CVT-301 (Inbrija) has demonstrated its effectiveness in treating OFF periods in people with Parkinson's disease (PwP). However, its effect on non-motor symptoms (NMS) and quality of life has not been researched yet. Our aim was to analyze the change observed in health-related quality of life (HRQoL) and, as a secondary exploratory analysis, NMS associated with OFF episodes in PwP treated with CVT-301.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>INLEVO-LIFE PD (an open-label study of the effect of INhaled LEVOdopa on quality of LIFE and non-motor symptoms in Parkinson's Disease) is a prospective open-label study conducted in five centers in Spain. The change from baseline (V0) to the end of the observational period (12 ± 2 weeks) (V12w) in the 39-item Parkinson's Disease Quality of Life Questionnaire (PDQ-39) total score was the primary efficacy measure.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Forty PD patients (age 62.3 ± 9.2 years; 55% males) were included between March/2024 and November/2025. At 12 weeks, 34 patients were using CVT-301 and completed the follow-up (85%). The PDQ39 total score decreased from 43.6 ± 24.2 at V0 to 31.4 ± 20.6 at V12w (<i>p</i> &lt; 0.0001). A significant decrease was observed in the mean score of 4 domains of the PDQ39. NMS burden was reduced at v12w from the OFF state to 30, 60, and 90 min after using CVT-301 (<i>p</i> &lt; 0.0001). Mean daily OFF time decreased by 1.6 ± 1.9 h (<i>p</i> &lt; 0.0001). Adverse events related to CVT-301 were reported in 35% of the patients.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>HRQoL and NMS associated with OFF episodes improved in PwP after 12 weeks using CVT-301.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 8","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-08-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13446143/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148676755","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":"Migraine in Young Stroke Patients—Better Outcomes Despite Distinct Risk Profiles: Insights From the STROKE-CARD-Study","authors":"Katharina Kaltseis,&nbsp;Silvia Felicetti,&nbsp;Benjamin Dejakum,&nbsp;Lena Weber,&nbsp;Samuel Labrecque,&nbsp;Anel Karisik,&nbsp;Kurt Moelgg,&nbsp;Christian Boehme,&nbsp;Thomas Toell,&nbsp;Lukas Mayer-Suess,&nbsp;Michael Thomas Eller,&nbsp;Florian Frank,&nbsp;Alois Schiefecker,&nbsp;Stefan Kiechl,&nbsp;Michael Knoflach,&nbsp;Gregor Broessner,&nbsp;STROKE-CARD Study Group","doi":"10.1111/ene.70709","DOIUrl":"10.1111/ene.70709","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Introduction</h3>\u0000 \u0000 <p>Migraine is a common neurological disorder and a non-traditional risk factor for ischaemic stroke. Its relationship with specific stroke subtypes and post-stroke outcomes remains unclear. We evaluated the association between migraine (with and without aura), stroke aetiology, functional outcomes, and cardiovascular risk profiles in young patients with ischaemic stroke or TIA.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Patients aged ≤ 55 years enrolled in the STROKE-CARD long-term follow-up study underwent structured face-to-face headache interviews by headache specialists using ICHD-3 criteria. A predefined protocol collected demographic, clinical, neuroimaging, aetiological, and cardiovascular risk data.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Among 289 young stroke patients (median age 48.0 [41–52] years; 36.3% women), 92 (31.8%) had a history of migraine (51.1% with aura). Migraine was more common in women, associated with younger age at stroke onset and more frequent headache at stroke onset, but not with traditional vascular risk factors. PFO was more prevalent in patients with migraine with aura (68.9% vs. 36.9% no migraine, <i>p</i> &lt; 0.001). No significant differences were observed in stroke territory or stroke pattern. Stroke severity (NIHSS) and functional outcome (mRS) at discharge were more favourable in migraineurs (<i>p</i> ≤ 0.001). In multivariable analysis, migraine without aura was independently associated with good functional outcome (OR 7.21, 95% CI 1.93–26.93, <i>p</i> = 0.003).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>In young adults with ischaemic stroke, migraine—particularly with aura—was associated with younger age at stroke onset and a higher prevalence of PFO, while traditional vascular risk factors and stroke characteristics were similar across groups. Despite comparable stroke patterns, patients with migraine experienced more favourable short-term outcomes.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Trial Registration</h3>\u0000 \u0000 <p>Post-Stroke Disease Management—Stroke Card: NCT02156778; Stroke Card Long-term Follow-Up NCT04205006</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 8","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-08-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13445783/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148677118","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}