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Diagnosis and Management of Infectious Encephalitis in Adults: Guidelines From the European Academy of Neurology and European Society of Clinical Microbiology and Infectious Diseases 成人传染性脑炎的诊断和管理:来自欧洲神经病学学会和欧洲临床微生物学和传染病学会的指南
IF 4.3 2区 医学
European Journal of Neurology Pub Date : 2026-10-09 DOI: 10.1111/ene.70647
Julia Granerod, Johann Sellner, Jean-Paul Stahl, Sylviane Defres, Alexandra Mailles, Erich Schmutzhard, Elisabeth Aurelius, Thomas Whitfield, Luca Massacesi, Maarten J. Titulaer, Pille Taba, Benedict D. Michael, Tehmina Bharucha, Lance Turtle, Brendan Sargent, Arina Tamborska, Jonathan Cattrall, Yun Huang, Eva Maria Hodel, Suzannah Lant, Glynn Webb, Rajish S. K. Shil, Shalley Dhar, Diederik van de Beek, Marie Studahl, Matthijs C. Brouwer, Uta Meyding-Lamade, Ava Easton, Bhagteshwar Singh, Tom Solomon
{"title":"Diagnosis and Management of Infectious Encephalitis in Adults: Guidelines From the European Academy of Neurology and European Society of Clinical Microbiology and Infectious Diseases","authors":"Julia Granerod,&nbsp;Johann Sellner,&nbsp;Jean-Paul Stahl,&nbsp;Sylviane Defres,&nbsp;Alexandra Mailles,&nbsp;Erich Schmutzhard,&nbsp;Elisabeth Aurelius,&nbsp;Thomas Whitfield,&nbsp;Luca Massacesi,&nbsp;Maarten J. Titulaer,&nbsp;Pille Taba,&nbsp;Benedict D. Michael,&nbsp;Tehmina Bharucha,&nbsp;Lance Turtle,&nbsp;Brendan Sargent,&nbsp;Arina Tamborska,&nbsp;Jonathan Cattrall,&nbsp;Yun Huang,&nbsp;Eva Maria Hodel,&nbsp;Suzannah Lant,&nbsp;Glynn Webb,&nbsp;Rajish S. K. Shil,&nbsp;Shalley Dhar,&nbsp;Diederik van de Beek,&nbsp;Marie Studahl,&nbsp;Matthijs C. Brouwer,&nbsp;Uta Meyding-Lamade,&nbsp;Ava Easton,&nbsp;Bhagteshwar Singh,&nbsp;Tom Solomon","doi":"10.1111/ene.70647","DOIUrl":"10.1111/ene.70647","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Encephalitis is difficult to diagnose and manage, posing a challenge to clinicians. Since the last European guidelines were published in 2010, novel causes have emerged, diagnostic processes have evolved and treatments assessed which need to be reflected in updated guidelines.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>We aimed to produce recommendations for the diagnosis and management of infectious encephalitis in adults based on current evidence to equip clinicians to improve patient outcomes.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>These guidelines were developed jointly with the support of two European specialist medical organisations. Clinical questions were prioritised and formulated in PICO format. A systematic literature search was conducted for each question, and the quality of evidence was assessed using standardised tools. Recommendations were developed according to the Grading of Recommendations, Assessment, Development and Evaluations (GRADE) approach. Where only indirect evidence was available, a good practice statement was made.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Sixteen questions were considered, with two recommendations reached: for initiation of intravenous aciclovir as soon as possible upon suspicion of encephalitis and against replacing pathogen-specific polymerase chain reaction (PCR) testing of cerebrospinal fluid (CSF) with multiplex CSF PCR panels for diagnosis of adult patients with suspected infectious encephalitis. For adults with proven HSV encephalitis, we were unable to recommend for or against the use of corticosteroids. A further 13 good practice statements regarding when to clinically suspect infectious encephalitis, microbiological investigations, additional investigations, treatment and ongoing care and rehabilitation were agreed.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Given the clinical severity and public health importance of this condition, the paucity of high-quality evidence is striking. Transnational, large-scale research in infective encephalitis is encouraged.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 10","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-10-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/ene.70647","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149135540","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Letter to the Editor Regarding Panvino et al. “Pharmacological Treatment of Cerebellar Ataxia in Pediatric Ataxia-Telangiectasia: A Systematic Review” 关于Panvino等人给编辑的信。儿童共济失调-毛细血管扩张症小脑共济失调的药物治疗:系统综述。
IF 4.3 2区 医学
European Journal of Neurology Pub Date : 2026-10-08 DOI: 10.1111/ene.70780
Fabiola Panvino, Serena Galosi, Carlotta Spagnoli, Vincenzo Leuzzi, Francesco Pisani
{"title":"Letter to the Editor Regarding Panvino et al. “Pharmacological Treatment of Cerebellar Ataxia in Pediatric Ataxia-Telangiectasia: A Systematic Review”","authors":"Fabiola Panvino,&nbsp;Serena Galosi,&nbsp;Carlotta Spagnoli,&nbsp;Vincenzo Leuzzi,&nbsp;Francesco Pisani","doi":"10.1111/ene.70780","DOIUrl":"10.1111/ene.70780","url":null,"abstract":"&lt;p&gt;We recently published a systematic review titled “Pharmacological Treatment of Cerebellar Ataxia in Pediatric Ataxia-Telangiectasia: A Systematic Review,” including 13 studies published to December 2025 [&lt;span&gt;1&lt;/span&gt;]. In this Letter to the Editor, we would like to provide an update to our review and further discuss its findings in light of recently published evidence on ataxia-telangiectasia (AT).&lt;/p&gt;&lt;p&gt;At the time of our search and publication, the pivotal phase 3 IB1001-303 trial of N-acetyl-L-leucine (NALL) in AT had not yet been published [&lt;span&gt;2&lt;/span&gt;]. We therefore referred to preliminary gray literature, but could not include it in the synthesis or extract pediatric-specific data from the mixed-age sample.&lt;/p&gt;&lt;p&gt;We wish to clarify that the trial was inadvertently identified as IB1001-301 rather than IB1001-303 on Page 11 of our review [&lt;span&gt;1&lt;/span&gt;], although the cited reference corresponded to the AT trial. This typographical error does not affect our synthesis. Reference 39 was cited separately to note that US Food and Drug Administration approval of NALL for neurological manifestations of Niemann–Pick disease type C [&lt;span&gt;3&lt;/span&gt;].&lt;/p&gt;&lt;p&gt;Since publication of our review, the full results of the IB1001-303 trial were published by Martakis et al. in &lt;i&gt;The Lancet Neurology&lt;/i&gt; in July 2026 [&lt;span&gt;2&lt;/span&gt;].&lt;/p&gt;&lt;p&gt;The randomized, double-blind, placebo-controlled crossover trial enrolled 73 participants with genetically confirmed AT, including 47 pediatric participants (4–18 years) and 26 adults. Participants were assigned to orally administered NALL then placebo (&lt;i&gt;n&lt;/i&gt; = 36), or the reverse (&lt;i&gt;n&lt;/i&gt; = 37), over two consecutive 12-week periods. All 73 participants receiving at least one dose were included in the analysis; three dropped out due to unrelated serious adverse events. The study met its primary endpoint, with a mean reduction of 1.92 points in the Scale for the Assessment and Rating of Ataxia (SARA) total score after 12 weeks of NALL compared with a 0.14-point reduction with placebo (treatment effect, −1.88 points; 95% CI, −2.70 to −1.06; 𝑝 &lt; 0.0001). The age subgroup analysis showed a larger improvement in pediatric participants (&lt;i&gt;n&lt;/i&gt; = 45; estimated mean difference, −2.26 points; 95% CI, −3.19 to −1.32) than in adults (&lt;i&gt;n&lt;/i&gt; = 25; estimated mean difference, −0.86 points; 95% CI, −1.62 to −0.10). These results suggest a benefit of NALL in both age groups; however, subgroup-specific &lt;i&gt;p&lt;/i&gt; values and formal age-by-treatment interaction analyses were not reported. These findings are in contrast with the earlier study by Beyraghi-Tousi et al. [&lt;span&gt;4&lt;/span&gt;], which contributed to the mixed evidence in our review [&lt;span&gt;1&lt;/span&gt;].&lt;/p&gt;&lt;p&gt;Secondary outcomes supported a treatment benefit on the International Cooperative Ataxia Rating Scale and the Investigator's Clinical Global Impression of Improvement. These outcomes should be interpreted cautiously since no adjustment for multiple comparisons was prespec","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 10","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-10-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/ene.70780","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149135517","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Recalibration of the Frontal Behavioural Inventory Cut-Off for Behavioural Screening in Amyotrophic Lateral Sclerosis 肌萎缩性侧索硬化症行为筛查中额叶行为量表截点的重新校准。
IF 4.3 2区 医学
European Journal of Neurology Pub Date : 2026-10-07 DOI: 10.1111/ene.70778
Barbara Iazzolino, Francesca Palumbo, Antonio Canosa, Stefano Callegaro, Vita Passidomo, Umberto Manera, Rosario Vasta, Enrico Matteoni, Sara Cabras, Giorgio Pellegrino, Alessandra Maccabeo, Cristina Moglia, Andrea Calvo, Adriano Chiò
{"title":"Recalibration of the Frontal Behavioural Inventory Cut-Off for Behavioural Screening in Amyotrophic Lateral Sclerosis","authors":"Barbara Iazzolino,&nbsp;Francesca Palumbo,&nbsp;Antonio Canosa,&nbsp;Stefano Callegaro,&nbsp;Vita Passidomo,&nbsp;Umberto Manera,&nbsp;Rosario Vasta,&nbsp;Enrico Matteoni,&nbsp;Sara Cabras,&nbsp;Giorgio Pellegrino,&nbsp;Alessandra Maccabeo,&nbsp;Cristina Moglia,&nbsp;Andrea Calvo,&nbsp;Adriano Chiò","doi":"10.1111/ene.70778","DOIUrl":"10.1111/ene.70778","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>The Frontal Behavioural Inventory (FBI) was validated in behavioural-variant frontotemporal dementia (bvFTD) with a cut-off of 25. We tested whether this threshold is appropriate in amyotrophic lateral sclerosis (ALS), where behavioural change is milder and partly confounded by motor pathology.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We analysed 506 ALS patients with a valid FBI assessment. Behavioural impairment was defined using two complementary reference standards, to minimise dependence on any single behavioural instrument: a consensus rule (pathology on ≥ 2 of three independent instruments: Edinburgh Cognitive and Behavioural ALS Screen, Frontal Systems Behaviour Scale, Beaumont Behavioural Inventory) and Latent Class Analysis. ROC analysis identified the Youden-optimal cut-off; agreement was quantified by Cohen's <i>κ</i>; internal validity was assessed by stratified 5-fold cross-validation. Total and subscale scores were analysed separately.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Among 345 complete-case patients, 24.6% met criteria for behavioural impairment. The FBI total discriminated impairment well (AUC 0.855, 95% CI 0.806–0.898). The Youden-optimal cut-off was FBI ≥ 9 (sensitivity 0.87, specificity 0.74), whereas the legacy cut-off of 25 showed sensitivity 0.22. Subscale recalibration yielded cut-offs of FBI-Apathy ≥ 7 (AUC 0.808) and FBI-Disinhibition ≥ 4 (AUC 0.851).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>In ALS, the bvFTD-derived threshold of 25 misses most patients with behavioural involvement. FBI ≥ 9 is a promising candidate ALS-specific screening threshold, with scores ≥ 15 indicating a higher probability of behavioural involvement; exploratory subscale thresholds of ≥ 7 for Apathy and ≥ 4 for Disinhibition are also proposed. These cut-offs substantially improve sensitivity while maintaining acceptable specificity, but require prospective external validation before replacing current practice.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 10","pages":""},"PeriodicalIF":4.3,"publicationDate":"2026-10-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13643373/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149106209","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Anti-CD20 Drugs and Natalizumab Confer Equal Protection Against Progression Independent of Relapse Activity (PIRA) in People With Multiple Sclerosis. 抗cd20药物和Natalizumab在多发性硬化症患者中具有相同的抗进展独立于复发活动(PIRA)的保护作用。
IF 4.3 2区 医学
European Journal of Neurology Pub Date : 2026-10-01 DOI: 10.1111/ene.70771
Leonardo Palazzo, Ferdinando Clarelli, Michele Surano, Antonino Giordano, Lucia Moiola, Maria A Rocca, Massimo Filippi, Federica Esposito
{"title":"Anti-CD20 Drugs and Natalizumab Confer Equal Protection Against Progression Independent of Relapse Activity (PIRA) in People With Multiple Sclerosis.","authors":"Leonardo Palazzo, Ferdinando Clarelli, Michele Surano, Antonino Giordano, Lucia Moiola, Maria A Rocca, Massimo Filippi, Federica Esposito","doi":"10.1111/ene.70771","DOIUrl":"https://doi.org/10.1111/ene.70771","url":null,"abstract":"<p><strong>Background: </strong>Highly effective (HE) disease-modifying therapies (DMTs) reduce relapse-associated worsening (RAW) and progression independent of relapse activity (PIRA). Natalizumab (NTZ) and anti-CD20 agents show similar efficacy on relapse suppression, but long-term comparative data on PIRA are limited. We aimed to compare PIRA accrual in relapsing-remitting MS (RRMS) patients treated with NTZ and anti-CD20 drugs over a long follow-up.</p><p><strong>Methods: </strong>We included RRMS patients followed at our centre and recorded in the Italian MS and Related Disorders Register with at least 5 years of continuous treatment with NTZ or anti-CD20 agents (Ocrelizumab, Rituximab, Ofatumumab), at least 3 Expanded Disability Status Scale (EDSS) assessments, and complete demographic and clinical data. Patients were matched for gender, age, EDSS, disease duration, MS phenotype at treatment initiation. Logistic regression and Cox proportional hazard models were applied.</p><p><strong>Results: </strong>Of 267 eligible patients (178 NTZ, 89 anti-CD20), optimal matching yielded 89 balanced pairs. A first PIRA event was observed in 27.0% of NTZ-treated and 28.1% of anti-CD20-treated patients. The total number of PIRA events was comparable between NTZ-treated and anti-CD20-treated patients (36 vs. 30; p = 0.451). Adjusted analyses revealed no significant differences in cumulative risk (RR: 1.48; 95% CI: 0.66-3.40; p = 0.333) or time to first PIRA event (HR: 1.51; 95% CI 0.83-2.77; p = 0.179).</p><p><strong>Conclusion: </strong>In this real-world cohort with long-term follow-up, NTZ and anti-CD20 therapies showed no significant differences in PIRA accrual or time to first PIRA, suggesting comparable long-term control of disability progression beyond relapse. Larger studies are warranted to confirm and refine these observations.</p>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 10","pages":"e70771"},"PeriodicalIF":4.3,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13660713/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149180230","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
A Data-Driven Operational Definition of Square-Wave Jerks and Its Diagnostic Utility in Progressive Supranuclear Palsy.
IF 4.3 2区 医学
European Journal of Neurology Pub Date : 2026-10-01 DOI: 10.1111/ene.70790
Duy Duan Nguyen, Andrea Quattrone, Mark Paine, Amir Fazlollahi, Soohyun Lee, Baibin Ben Wang, Tanya Davidson, Felicia Coleman, John D O'Sullivan, Peter J Nestor
{"title":"A Data-Driven Operational Definition of Square-Wave Jerks and Its Diagnostic Utility in Progressive Supranuclear Palsy.","authors":"Duy Duan Nguyen, Andrea Quattrone, Mark Paine, Amir Fazlollahi, Soohyun Lee, Baibin Ben Wang, Tanya Davidson, Felicia Coleman, John D O'Sullivan, Peter J Nestor","doi":"10.1111/ene.70790","DOIUrl":"https://doi.org/10.1111/ene.70790","url":null,"abstract":"<p><strong>Background: </strong>The Movement Disorder Society diagnostic criteria for progressive supranuclear palsy (PSP) list frequent square-wave jerks (SWJ) as a suggestive oculomotor feature. A standardised operational definition of SWJ is however presently lacking.</p><p><strong>Objective: </strong>To create a data-driven optimal SWJ definition and to assess its diagnostic utility in PSP.</p><p><strong>Methods: </strong>We prospectively recruited PSP n = 25, Parkinson's disease (PD) n = 32, Alzheimer's disease (AD) n = 35, and controls n = 88. SWJ were recorded with a visual fixation task using video-oculography. Multiple candidate definitions of SWJ were generated by systematically varying morphological features, producing 32 candidate definitions. A nested cross-validation was used to obtain unbiased diagnostic performance and optimised SWJ definition. Results were then externally tested in a fully independent sample of n = 25 PSP, n = 41 PD and n = 25 controls.</p><p><strong>Results: </strong>The optimal selected SWJ definition consisted of saccade amplitude 1°-8°, intra-SWJ intervals 20-600 ms, directional opposition within ±45°, and amplitude similarity ≤ 0.25. Only SWJ rate, and not morphological features, was distinctive for PSP. SWJ rate gave out-of-fold AUCs of 0.81 for PSP versus controls, 0.71 for PSP versus AD, 0.63 for PSP versus PD, and 0.76 for PSP versus all non-PSP groups. Post hoc analyses using this data-driven definition on the external validation cohort yielded relatively comparable results. SWJ rate > 24/min had high specificity, but low sensitivity, for PSP when compared with PD.</p><p><strong>Conclusions: </strong>SWJ rate was significantly increased in PSP but with insufficient discriminant ability for use as a stand-alone diagnostic marker. This data-driven definition may serve as a reproducible operational definition for future research.</p>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 10","pages":"e70790"},"PeriodicalIF":4.3,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149227399","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Transition From Child to Adult Care in Paediatric Neurological Disorders in Europe-A Joint EAN-EPNS Survey of Current Practices for Healthcare Providers. 欧洲儿童神经系统疾病从儿童到成人护理的转变——EAN-EPNS对医疗保健提供者当前实践的联合调查。
IF 4.3 2区 医学
European Journal of Neurology Pub Date : 2026-10-01 DOI: 10.1111/ene.70787
Dana Craiu, Cristina Cazacu, Maria T Papadopoulou, Ming Lim, Kevin Rostasy, Anna C Jansen, Leena M Haataja, Ilona Anna Kopyta, Kristl G Claeys, Guido Rubboli, Chiara Ferrazzoli, Ronan McGinty, Maria Roberta Cilio, Maria Lolich, Dimitrios Zafeiriou, Nadia Bahi-Buisson, Elena Moro, Gereon R Fink, Thomas Berger
{"title":"Transition From Child to Adult Care in Paediatric Neurological Disorders in Europe-A Joint EAN-EPNS Survey of Current Practices for Healthcare Providers.","authors":"Dana Craiu, Cristina Cazacu, Maria T Papadopoulou, Ming Lim, Kevin Rostasy, Anna C Jansen, Leena M Haataja, Ilona Anna Kopyta, Kristl G Claeys, Guido Rubboli, Chiara Ferrazzoli, Ronan McGinty, Maria Roberta Cilio, Maria Lolich, Dimitrios Zafeiriou, Nadia Bahi-Buisson, Elena Moro, Gereon R Fink, Thomas Berger","doi":"10.1111/ene.70787","DOIUrl":"10.1111/ene.70787","url":null,"abstract":"<p><strong>Background: </strong>Transition from paediatric to adult care is essential for young people with paediatric-onset neurological disorders, yet remains inconsistently structured across Europe. This study aimed to characterise current transition practices, identify key barriers and explore perceived priorities for improving transition care.</p><p><strong>Methods: </strong>A cross-sectional web-based survey, developed by the European Paediatric Neurology Society/European Academy of Neurology Task Force on Transition from Childhood to Adulthood, was distributed to members of both societies between February and April 2025. The 22-item questionnaire assessed institutional characteristics, transition practices, perceived barriers and improvement measures. Responses were analysed descriptively and compared according to the reported presence of an institutional transition programme.</p><p><strong>Results: </strong>Overall, 328 responses were analysed. A transition programme was reported to be available in the country of practice by 123 respondents (37.5%). Respondents from institutions with transition programmes reported earlier transition initiation (median 16 vs. 17 years) and more frequent structured practices, including joint paediatric-adult consultations (73.7% vs. 27.1%). The highest-rated barriers were lack of multidisciplinary teams, limited adult neurology capacity and difficulty accessing adult neurology services. Key improvement priorities included joint consultations, national recommendations, specialised care networks and dedicated transition coordination. Satisfaction with current transition practice was low; only 7.9% of respondents reported complete satisfaction.</p><p><strong>Conclusions: </strong>Transition of care for paediatric-onset neurological disorders in Europe remains heterogeneous. Although core elements of effective transition are widely recognised, implementation is limited by organisational and system-level barriers. Coordinated strategies are needed to support structured, patient-centred transition pathways.</p>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 10","pages":"e70787"},"PeriodicalIF":4.3,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13675786/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149206724","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Nerve Ultrasound Features of Peripheral Nervous System Lymphoma and Non-Systemic Vasculitic Neuropathy. 周围神经系统淋巴瘤和非全身性血管性神经病的神经超声特征。
IF 4.3 2区 医学
European Journal of Neurology Pub Date : 2026-10-01 DOI: 10.1111/ene.70793
Huihong Tian, Nan Hu, Jianfeng Ding, Min Qian, Yiying Wang, Liying Cui, Jingwen Niu, Mingsheng Liu
{"title":"Nerve Ultrasound Features of Peripheral Nervous System Lymphoma and Non-Systemic Vasculitic Neuropathy.","authors":"Huihong Tian, Nan Hu, Jianfeng Ding, Min Qian, Yiying Wang, Liying Cui, Jingwen Niu, Mingsheng Liu","doi":"10.1111/ene.70793","DOIUrl":"https://doi.org/10.1111/ene.70793","url":null,"abstract":"<p><strong>Objective: </strong>To compare the clinical, electrophysiological, and nerve ultrasound characteristics of peripheral nervous system lymphoma (PNSL) and non-systemic vasculitic neuropathy (NSVN).</p><p><strong>Methods: </strong>Between July 2015 and May 2026, 9 PNSL patients and 7 NSVN patients were retrospectively enrolled. Clinical characteristics, electrophysiological studies, and nerve ultrasound data were collected. Cross-sectional area (CSA) was measured at predetermined sites of peripheral nerves.</p><p><strong>Results: </strong>The PNSL group showed significantly higher rates of cranial nerve involvement (66.7% vs. 0%, p = 0.011) and nerve root/plexus involvement (88.9% vs. 0%, p = 0.001) than the NSVN group. Although CSA at most median and ulnar nerve sites did not differ significantly between groups, the PNSL group showed significantly higher CSA of the cervical roots and brachial plexus than the NSVN group (C5, C6, upper trunk, middle trunk, and lower trunk; p = 0.001-0.018) except C7 root. In the PNSL group, the CSA was significantly greater on the initially affected side than on the contralateral side (p < 0.001), whereas in the NSVN group the CSA was significantly greater on the contralateral side than on the onset side (p = 0.005). The maximum-CSA ratio between bilateral homonymous nerves (affected onset/contralateral) was significantly higher in PNSL than in NSVN (1.32 vs. 0.88, p < 0.001).</p><p><strong>Conclusions: </strong>PNSL shows persistent severe focal enlargement favoring the initially-affected side and frequent cranial/root involvement, whereas NSVN exhibits mild-to-moderate, variable enlargement with rare cranial/root involvement. Nerve ultrasound may serve as a noninvasive screening tool prior to PET/CT and biopsy.</p>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 10","pages":"e70793"},"PeriodicalIF":4.3,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149223641","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Prospective, Population-Based Evaluation of Costs and Outcomes of Status Epilepticus According to the New Definition.
IF 4.3 2区 医学
European Journal of Neurology Pub Date : 2026-10-01 DOI: 10.1111/ene.70777
Clara Jünemann, Meike Menche, Jens-Peter Reese, Felix Rosenow, Martin Jünemann, Christoph Best, Michael Teepker, Christian Roth, Ingrid Sünkeler, Elisabeth Pryss, Leona Möller, Katja Menzler, Lars Timmermann, Susanne Knake, Adam Strzelczyk
{"title":"Prospective, Population-Based Evaluation of Costs and Outcomes of Status Epilepticus According to the New Definition.","authors":"Clara Jünemann, Meike Menche, Jens-Peter Reese, Felix Rosenow, Martin Jünemann, Christoph Best, Michael Teepker, Christian Roth, Ingrid Sünkeler, Elisabeth Pryss, Leona Möller, Katja Menzler, Lars Timmermann, Susanne Knake, Adam Strzelczyk","doi":"10.1111/ene.70777","DOIUrl":"https://doi.org/10.1111/ene.70777","url":null,"abstract":"<p><strong>Background: </strong>To address the paucity of data on outcomes and costs of status epilepticus (SE) following the updated 2015 International League Against Epilepsy (ILAE) definition and changes in clinical practice guidelines in Germany.</p><p><strong>Methods: </strong>This prospective population-based study (October 2018-March 2020) included adult patients from postal code area prefix 35, representative of Germany, diagnosed with SE according to the 2015 ILAE definition. Costs were calculated using German Diagnosis-Related Groups (G-DRG).</p><p><strong>Results: </strong>In total, 181 patients were included. Mean inpatient costs were €10,543 (median €6376; range €845-€133,267), corresponding to €939 per hospital day (median €580.8; range €76.8-€12,115). Mean length of stay (LOS) was 11 days (median 9; range 1-67). In univariable analyses, super-refractory SE was associated with higher costs and longer LOS (both p < 0.001), while acute symptomatic etiology was associated with longer LOS (p = 0.006). On multivariable analysis, mechanical ventilation was the strongest independent predictor of costs (Exp(B) = 2.918, 95% CI 1.753-4.857; p < 0.001), and etiology was overall significantly associated with cost variation (p = 0.022), with acute symptomatic SE showing the highest adjusted costs. Discharge destination correlated with SE severity, with most patients with non-refractory SE discharged home (p = 0.028).</p><p><strong>Conclusions: </strong>This study provides population-based data on costs and outcomes of SE in Germany, serving as a basis for future cost-effectiveness analyses. Compared with earlier studies, lower direct costs and shorter LOS were observed. Identification of high-risk constellations may support clinical decision-making and optimize monitoring, treatment escalation, and allocation of intensive care resources.</p>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 10","pages":"e70777"},"PeriodicalIF":4.3,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149227450","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Diagnostic Impact of Intracranial Vessel Wall MRI on Ischemic Stroke Etiology in Young Adults.
IF 4.3 2区 医学
European Journal of Neurology Pub Date : 2026-10-01 DOI: 10.1111/ene.70795
Juhan Reimand, Siim Schneider, Riina Vibo, Liisa Kõrv, Dagmar Loorits, Karl Jõgi, Teele Tuularu, Nele Taba, Kateriine Orav, Janika Kõrv
{"title":"Diagnostic Impact of Intracranial Vessel Wall MRI on Ischemic Stroke Etiology in Young Adults.","authors":"Juhan Reimand, Siim Schneider, Riina Vibo, Liisa Kõrv, Dagmar Loorits, Karl Jõgi, Teele Tuularu, Nele Taba, Kateriine Orav, Janika Kõrv","doi":"10.1111/ene.70795","DOIUrl":"https://doi.org/10.1111/ene.70795","url":null,"abstract":"<p><strong>Background: </strong>Intracranial vessel wall imaging (VWI) enables visualization of arterial wall pathology and may improve etiologic classification of ischemic stroke, but existing evidence is largely based on retrospective studies. We aimed to prospectively evaluate the diagnostic impact of VWI in young stroke patients.</p><p><strong>Methods: </strong>In this two-centre study, consecutive patients aged 18-59 years with acute ischemic stroke in the middle cerebral or anterior choroidal artery territory underwent contrast-enhanced VWI within 2 weeks of stroke onset. Stroke etiology was assessed using the Trial of ORG 10172 in Acute Stroke Treatment (TOAST) and the Causative Classification of Stroke (CCS) classifications before and after incorporating VWI findings. Changes in etiologic classification and predictors of diagnostic change were analyzed.</p><p><strong>Results: </strong>Sixty-nine patients were included (median age 53 years, 72% male). Incorporation of VWI findings resulted in a change in etiologic classification in 9/69 (13%) patients according to TOAST and 10/69 (14%) according to CCS. For TOAST classification, the proportion of large artery atherosclerosis increased from 17% to 28%, while cryptogenic stroke decreased from 22% to 14%. Baseline cryptogenic stroke was associated with etiologic reclassification after adjustment for age and sex (OR 7.8, 95% CI: 1.2-49.7; p = 0.029).</p><p><strong>Conclusions: </strong>VWI frequently identified intracranial atherosclerotic plaques not evident on luminal imaging, leading to etiologic reclassification predominantly toward large artery atherosclerosis. These findings suggest incremental diagnostic value in the etiologic assessment of young stroke, particularly in cryptogenic cases. Larger prospective studies are warranted to determine its role in routine diagnostic algorithms.</p>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 10","pages":"e70795"},"PeriodicalIF":4.3,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149227457","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Real-World Patient Eligibility for Alzheimer's Disease Anti-Amyloid Therapy in Seven Swedish Memory Clinics. 在七个瑞典记忆诊所,阿尔茨海默病抗淀粉样蛋白治疗的现实世界患者资格。
IF 4.3 2区 医学
European Journal of Neurology Pub Date : 2026-10-01 DOI: 10.1111/ene.70786
Anna Rosenberg, Anna Rennie, Urban Ekman, Makrina Daniilidou, Anette Hall, Anna Matton, Ari Solomon, Lars-Olof Wahlund, Miia Kivipelto, Eric Westman
{"title":"Real-World Patient Eligibility for Alzheimer's Disease Anti-Amyloid Therapy in Seven Swedish Memory Clinics.","authors":"Anna Rosenberg, Anna Rennie, Urban Ekman, Makrina Daniilidou, Anette Hall, Anna Matton, Ari Solomon, Lars-Olof Wahlund, Miia Kivipelto, Eric Westman","doi":"10.1111/ene.70786","DOIUrl":"https://doi.org/10.1111/ene.70786","url":null,"abstract":"<p><strong>Background: </strong>Anti-amyloid therapies lecanemab and donanemab are the first potentially disease-modifying treatments for Alzheimer's disease (AD) approved in Europe. We estimated real-world eligibility for these treatments in a large multicenter memory clinic patient cohort in Sweden (MemClin).</p><p><strong>Methods: </strong>MemClin included patients with a first diagnostic visit between 2016 and 2022 at seven specialist memory clinics in Stockholm, Sweden. To estimate eligibility for anti-amyloid therapy, we applied key inclusion/exclusion criteria from the lecanemab and donanemab appropriate use recommendations. Eligible patients were required to have (1) a clinical diagnosis of AD-type dementia or mild cognitive impairment (MCI), (2) confirmed Aβ pathology (abnormal CSF Aβ42) and (3) no apparent medical exclusion criteria. Eligibility was calculated for different scenarios to provide both upper-bound estimates (main analysis) and more conservative, implementation-ready estimates of near-term treatable patients (sensitivity analysis).</p><p><strong>Results: </strong>Cohort consisted of 2126 patients (mean age 77.1 ± 6.6 years; 61.8% MCI, 24.2% dementia, 13.7% subjective cognitive impairment). In the main analysis, 11.5% of the whole cohort was estimated to be eligible for either treatment (lecanemab 10.6%, donanemab 11.5%). Most eligible patients had early-stage disease (61.9% MCI vs. 38.1% dementia) and an A + T- biomarker profile, that is, normal biomarker of tau (57.9%). Eligibility was markedly lower in sensitivity analyses (e.g., 2.7% when all patients without MRI were assumed ineligible).</p><p><strong>Conclusions: </strong>Even upper-bound estimates indicate that a minority of real-world memory clinic patients may currently qualify for anti-amyloid therapy. These findings provide timely evidence to inform the ongoing debate on the implementation of anti-amyloid therapy in public healthcare.</p>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"33 10","pages":"e70786"},"PeriodicalIF":4.3,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149223602","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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