European Journal of Neurology最新文献

{"title":"Thank You to Our Peer Reviewers in 2024","authors":"","doi":"10.1111/ene.70046","DOIUrl":"https://doi.org/10.1111/ene.70046","url":null,"abstract":"<p><b>Didier Leys</b> (<i>Editor-in-Chief</i>), <b>Claudia Sommer</b> (<i>Deputy Editor</i>), <b>Barbara Borroni</b>, <b>Andrew Chan, Mark Edwards, Alessandro Tessitore, and Kristl Vonck</b> (<i>Associate Editors</i>), <b>Paulo Caramelli</b> and <b>Isabel Martins</b> (<i>Guest Editor</i>s)</p><p>Thank all the reviewers from around the world who have provided expert advice to ensure the publication of the best quality articles in the <i>European Journal of Neurology</i> and the journal of the <i>European Academy of Neurology</i>. Their feedback is crucial to improve the quality of manuscripts and of neurological care provided to patients. The names of those who reviewed manuscripts for the <i>European Journal of Neurology</i> in 2024 are listed below. Our warmest gratitude goes to all of them.</p><p><i>*indicates those who reviewed four manuscripts in 2024</i>, <i>**indicates those who reviewed five to eight</i>, and <i>***indicates those who reviewed nine or more</i>.</p><p>Aamodt, Anne Hege</p><p>Abbadessa, Gianmarco</p><p>\u0000 <b>Abdelhalk, Ahmed*</b>\u0000 </p><p>Abu-Rumeileh, Samir</p><p>Accorroni, A.</p><p>Adhikary, Asit Baran</p><p>Afsin, Emine</p><p>Agarwal, Smriti</p><p>Aguglia, Umberto</p><p>Aguiar De Sousa, Diana</p><p>Aguirre-Vidal, Yoshajandith</p><p>Ahmadizar, Fariba</p><p>Aktas, O.</p><p>Alberici, Antonella</p><p>Albo, Zimbul</p><p>Alboini, Paolo</p><p>Algoet, Chloé</p><p>Alhusaini, Saud</p><p>\u0000 <b>Allen, Jeffrey A.*</b>\u0000 </p><p>Almqvist, Tove</p><p>Alonso, Angelika</p><p>Alpkvist, Peter</p><p>Altomare, Daniele</p><p>Alves, Pedro</p><p>Al-Zuhairy, Ali</p><p>Amaro, Sergio</p><p>Anagnostakou, Vania</p><p>Anagnostou, Evangelos</p><p>\u0000 <b>Andersen, Henning*</b>\u0000 </p><p>Anderson, Craig</p><p>Andrew, Melissa K.</p><p>Androdias, Géraldine</p><p>Angelini, Corrado</p><p>Appeltshauser, Luise</p><p>Araújo, Rui</p><p>Arba, Francesco</p><p>Arboix, Adria</p><p>Arsava, Ethem</p><p>Arshad, Qadeer</p><p>Arsovska, Anita</p><p>Ascherio, Alberto</p><p>Asseyer, Susanna</p><p>Aster, Hans-Christoph</p><p>\u0000 <b>Attarian, Shahram**</b>\u0000 </p><p>Audebert, Heinrich</p><p>Audoin, Bertrand</p><p>Bain, Peter</p><p>Baizabal-Carvallo, José</p><p>Baka, Panoraia</p><p>Balabanski, Anna</p><p>Balayssac, David</p><p>Baldelli, Luca</p><p>Ballester, Ly</p><p>Ballve, Alejandro</p><p>Banerjee, Soma</p><p>Bankstahl, Jens P.</p><p>Barber, Pa.</p><p>Barbera, Mariagnese</p><p>Barloese, Mads</p><p>Barnett, Carolina</p><p>Baron, Jean-Claude</p><p>Batla, Amit</p><p>Baum, Petra</p><p>Bax, Francesco</p><p>Baykan-Baykal, Betul</p><p>Beare, Richard</p><p>Beatriz Gago-Veiga, Ana</p><p>Bede, Peter</p><p>Beier, Christoph</p><p>Beier, Dagmar</p><p>Béjot, Yannick</p><p>Ben Assayag, Einor</p><p>Benakis, Corinne</p><p>Benbadis, Selim</p><p>Ben-Shabat, Ilan</p><p>Benussi, Alberto</p><p>Berciano, José</p><p>Bereczki, Dániel</p><p>Bergquist, Filip</p><p>Bernasconi, Neda</p><p>Berzero, Giulia</p><p>Bessi, Valentina</p","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"32 1","pages":""},"PeriodicalIF":4.5,"publicationDate":"2025-01-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/ene.70046","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143117358","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":"Cost-utility analysis of a coadjutant telemedicine intervention for fall prevention in Parkinson's disease","authors":"Esther Cubo,&nbsp;Mohammad Rohani,&nbsp;Negin Eissazade,&nbsp;Álvaro Garcia-Bustillo,&nbsp;José Miguel Ramírez-Sanz,&nbsp;José Luis Garrido-Labrador,&nbsp;Alicia Olivares-Gil,&nbsp;Florita Valiñas-Sieiro Rn,&nbsp;Marta Allende-Río Rn,&nbsp;Josefa Gonzalez-Santos,&nbsp;Jerónimo Javier Gonzalez-Bernal,&nbsp;José Trejo,&nbsp;Sara Calvo-Simal,&nbsp;José Francisco Diez-Pastor,&nbsp;David García-García,&nbsp;Álvar Arnaiz-González","doi":"10.1111/ene.16561","DOIUrl":"10.1111/ene.16561","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and purpose</h3>\u0000 \u0000 <p>Adopting telemedicine (TM) enables improved access to specialized care and reduces barriers. The aim was to assess the cost-utility of a coadjutant multidisciplinary TM programme for fall prevention compared to standard in-office visits for individuals with Parkinson's disease (PD).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>This was an 8-month single-blind randomized controlled trial. TM and control groups received in-office visits and standard management care at baseline, 4 and 8 months. In addition, the TM group received remote multidisciplinary visits for 4 months. Gait, motor and non-motor symptoms, daily living activities, balance and frailty were measured using PD-recommended rating scales and wearable sensors. Clinical characteristics were compared at each visit using baseline scores, gender and age as covariates. The incremental cost-effectiveness ratio (ICER) and quality-adjusted life years (QALYs) were calculated at each visit.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Fifty patients were included: 25 patients in the TM group (48% males, mean age 71.1 ± 9.0 years) and 25 patients in the control group (52% males, mean age 69.2 ± 9.4 years). Compared to controls, in the TM group similar QALYs were found but, in contrast, significant improvements in daily living activities, depression, apathy, freezing of gait, balance, quality of life and frailty (all <i>p</i> values &lt;0.05). The use of coadjutant TM intervention in addition to in-office visits was efficient for depression, apathy, freezing of gait, balance and frailty with ICERs, ranging from 91.55 € for non-motor symptoms to 1677.4 € for frailty.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Telemedicine could be considered an efficient coadjutant intervention for PD, especially for non-motor symptoms, enhancing health outcomes and accessibility.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"32 1","pages":""},"PeriodicalIF":4.5,"publicationDate":"2025-01-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11751255/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143002494","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":"Mitochondrial DNA (mtDNA) as fluid biomarker in neurodegenerative disorders: A systematic review","authors":"Barbara Risi,&nbsp;Alberto Imarisio,&nbsp;Giada Cuconato,&nbsp;Alessandro Padovani,&nbsp;Enza Maria Valente,&nbsp;Massimiliano Filosto","doi":"10.1111/ene.70014","DOIUrl":"10.1111/ene.70014","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Several studies evaluated peripheral and cerebrospinal fluid (CSF) mtDNA as a putative biomarker in neurodegenerative diseases, often yielding inconsistent findings. We systematically reviewed the current evidence assessing blood and CSF mtDNA levels and variant burden in Parkinson's disease (PD), Alzheimer's disease (AD) and amyotrophic lateral sclerosis (ALS). Multiple sclerosis (MS) was also included as a paradigm of chronic neuroinflammation-driven neurodegeneration.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Medline, Embase, Scopus and Web of Science were searched for articles published from inception until October 2023. Studies focused on mtDNA haplogroups or hereditary pathogenic variants were excluded. Critical appraisal was performed using the Quality Assessment for Diagnostic Accuracy Studies criteria.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Fifty-nine original studies met our a priori-defined inclusion criteria. The majority of CSF-focused studies showed (i) decreased mtDNA levels in PD and AD; (ii) increased levels in MS compared to controls. No studies evaluated CSF mtDNA in ALS. Results focused on blood cell-free and intracellular mtDNA were contradictory, even within studies evaluating the same disease. This poor reproducibility is likely due to the lack of consideration of the many factors known to affect mtDNA levels. mtDNA damage and methylation levels were increased and reduced in patients compared to controls, respectively. A few studies investigated the correlation between mtDNA and disease severity, with conflicting results.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Additional well-designed studies are needed to evaluate CSF and blood mtDNA profiles as putative biomarkers in neurodegenerative diseases. The identification of “mitochondrial subtypes” of disease may enable novel precision medicine strategies to counteract neurodegeneration.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"32 1","pages":""},"PeriodicalIF":4.5,"publicationDate":"2025-01-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11744304/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143002496","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The Brief International Cognitive Assessment in Multiple Sclerosis (BICAMS): Regression-based norms for German-speaking countries","authors":"I. K. Penner,&nbsp;J. Baijot,&nbsp;M. Filser,&nbsp;S. Bätge,&nbsp;L. Raithel,&nbsp;E. Toth,&nbsp;A. Renner,&nbsp;G. Nagels","doi":"10.1111/ene.16495","DOIUrl":"10.1111/ene.16495","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and purpose</h3>\u0000 \u0000 <p>Cognitive impairment in multiple sclerosis (MS) is common and is associated with problems in employment, driving ability, and quality of life. Since cognitive impairment at time of diagnosis is predictive of disability progression, early assessment and annual monitoring is recommended. The Brief International Cognitive Assessment in Multiple Sclerosis (BICAMS) was introduced as a time-efficient screening tool that can easily be applied in standard clinical care. However, besides application, tests need to be analysed and the raw values obtained must be interpreted accordingly. Since normative values have not been available for German-speaking countries, BICAMS has not been implemented in clinical routine. The aim of this study was to calculate German normative BICAMS data to improve the current diagnostic process for people with MS.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Healthy control subjects in different age categories were recruited to enable regression-based norm analysis. Raw scores for each BICAMS test were converted into scaled scores before they were regressed for age, age squared, gender, and education. The obtained scores were then normalised to a <i>z</i>-score by dividing the difference between the scaled score and the predicted score by the root mean squared error of the model.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>In all, 237 HCs were recruited (68.8% female, 31.2% male) and examined with BICAMS. Datasets entered the regression-based norms analysis and formed the basis for a final <i>z</i>-score calculation. To simplify handling in everyday clinical practice, nomograms and look-up tables were created which provide clinicians with age- and education-corrected norms.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>Our work fills the gaps between BICAMS application, evaluation and interpretation and will make a significant contribution to more regular cognitive assessment of MS patients.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"32 1","pages":""},"PeriodicalIF":4.5,"publicationDate":"2025-01-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11744042/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143002501","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":"Prognostic Value of Signal Abnormalities on Brain MRI in Post-Anoxic Super-Refractory Status Epilepticus: A Single-Center Retrospective Study","authors":"Susanna Diamanti,&nbsp;Francesco Pasini,&nbsp;Cristina Capraro,&nbsp;Mirko Patassini,&nbsp;Elisa Bianchi,&nbsp;Matteo Pozzi,&nbsp;Marco Normanno,&nbsp;Anna Coppo,&nbsp;Paolo Remida,&nbsp;Leonello Avalli,&nbsp;Carlo Ferrarese,&nbsp;Giuseppe Foti,&nbsp;Simone Beretta","doi":"10.1111/ene.70045","DOIUrl":"10.1111/ene.70045","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Epileptiform activity, including status epilepticus (SE), occurs in up to one-third of comatose survivors of cardiac arrest and may predict poor outcome. The relationship between SE and hypoxic–ischemic brain injury (HIBI) is not established.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>This is a single-center retrospective study on consecutive patients with post-anoxic super-refractory SE. HIBI was graded as non-widespread (group 1) or widespread (group 2) by qualitative analysis of DWI/ADC and T2w-FLAIR. Between-group differences in the rate of poor neurological outcome at 6 months (primary outcome), SE resolution and consciousness recovery before discharge, and mortality at 6 months (secondary outcomes) were investigated.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>From January 2011 to February 2023, 40 patients were included. HIBI was widespread in 45% of patients and non-widespread in 55%. The rate of poor neurological outcome at 6 months was 27% in group 1 and 83% in group 2 (OR 12.8, CI 95% [2.5–64.3], <i>p</i> = 0.002). The rate of consciousness recovery before discharge was 73% in group 1 versus 22% in group 2 (OR 8.8, CI 95% [1.9–40.3], <i>p</i> = 0.005). SE resolved in 95% of patients in group 1 versus 67% in group 2 (OR 10.5, CI 95% [1.1–97.9], <i>p</i> = 0.039). Mortality rate at 6 months was 27% in group 1 versus 50% in group 2 (OR 0.4, CI 95% [0.1–1.9], <i>p</i> = 0.303).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>Patients with widespread HIBI had higher odds of poor outcome at 6 months, lower probability of SE resolution and of consciousness recovery before discharge compared to those with non-widespread HIBI. Mortality at 6 months did not differ significantly between the two groups.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"32 1","pages":""},"PeriodicalIF":4.5,"publicationDate":"2025-01-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11736634/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143002499","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":"A roadmap toward promoting and improving brain health in Europe and closing the awareness and funding gap","authors":"Paul A. J. M. Boon,&nbsp;Thomas Berger,&nbsp;Matilde Leonardi,&nbsp;Tony Marson,&nbsp;Ulf Kallweit,&nbsp;Elena Moro,&nbsp;Antonio Toscano,&nbsp;Irena Rektorova,&nbsp;Alice Accorroni,&nbsp;Charlotte Scheerens,&nbsp;Antonia Boesch,&nbsp;Michael Crean,&nbsp;Anja Sander,&nbsp;Simon Lee,&nbsp;Claudio L. A. Bassetti","doi":"10.1111/ene.16589","DOIUrl":"10.1111/ene.16589","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and Purpose</h3>\u0000 \u0000 <p>The global burden of neurological diseases exceeds 43.1%, imposing a significant burden on patients, caregivers and society. This paper presents a roadmap to reduce this burden and improve brain health (BH) in Europe.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>The roadmap is based on the European Academy of Neurology's (EAN) five-pillar BH strategy: advancing a global BH approach (P1), supporting policymaking (P2), fostering research (P3), promoting education (P4), and raising awareness of prevention and treatment (P5). It reviews current efforts, collaborations and future directions aligned with the WHO Intersectoral Global Action Plan (iGAP) for Neurological Disorders and suggests future initiatives and call for action.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <div>\u0000 \u0000 <ol>\u0000 \u0000 \u0000 <li>P1: Support WHO-iGAP through defined action points, international collaborations, in particular, the WHO BH Unit, and the EAN Brain Health Mission.</li>\u0000 \u0000 \u0000 <li>P2: Collaborate with 48 national neurological societies to promote National Brain Plans (NBPs), addressing local needs, and improving access to care.</li>\u0000 \u0000 \u0000 <li>P3: Advocate for more research funding; identify determinants of BH; develop preventive measures.</li>\u0000 \u0000 \u0000 <li>P4: Provide educational opportunities for neurologists, public education programs, and advocacy training, including tools to educate the public.</li>\u0000 \u0000 \u0000 <li>P5: Spearhead global awareness campaigns, organize public educational activities, and train BH advocates to contribute toward sustainable and long-term public health campaigns and policy engagement.</li>\u0000 </ol>\u0000 </div>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>The paper highlights the importance of a unified approach, integrating international collaborations and local initiatives, to improve BH outcomes based on the WHO-iGAP, and support sustainable development goals, in particular SDG 3: Good Health and Well-being and SDG 4: Quality Education.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"32 1","pages":""},"PeriodicalIF":4.5,"publicationDate":"2025-01-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11735729/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143002490","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":"Lyme neuroborreliosis in Japan: Borrelia burgdorferi sensu lato as a cause of meningitis of previously undetermined etiology in hospitalized patients outside of the island of Hokkaido, 2010–2021","authors":"Masayuki Ohira,&nbsp;Ai Takano,&nbsp;Kentaro Yoshi,&nbsp;Akira Arai,&nbsp;Yashuhiro Aso,&nbsp;Rikiya Furutani,&nbsp;Tadanori Hamano,&nbsp;Ikuko Takahashi-Iwata,&nbsp;Chikako Kaneko,&nbsp;Tohru Matsuura,&nbsp;Norihisa Maeda,&nbsp;Hideto Nakajima,&nbsp;Katsuro Shindo,&nbsp;Toshihiko Suenaga,&nbsp;Kazuma Sugie,&nbsp;Yasuhiro Suzuki,&nbsp;Toru Yamashita,&nbsp;Frederick J. Angulo,&nbsp;Juanita Edwards,&nbsp;Cody Matthew Bender,&nbsp;Lisa R. Harper,&nbsp;Yoshikazu Nakayama,&nbsp;Shuhei Ito,&nbsp;Andreas Pilz,&nbsp;James H. Stark,&nbsp;Jennifer C. Moïsi,&nbsp;Hidehiro Mizusawa,&nbsp;Masaki Takao","doi":"10.1111/ene.70005","DOIUrl":"10.1111/ene.70005","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and Purpose</h3>\u0000 \u0000 <p>Clinical manifestations of Lyme borreliosis (LB), caused by <i>Borrelia burgdorferi</i> sensu lato (Bbsl), include erythema migrans, Lyme neuroborreliosis (LNB), carditis, and arthritis. LB is a notifiable disease in Japan with &lt;30 surveillance-reported LB cases annually, predominately from Hokkaido Prefecture. However, LB, including LNB, may be under-diagnosed in Japan since diagnostic tests are not readily available. We sought to determine if LNB could be a cause of previously undiagnosed encephalitis or meningitis in Japan.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Investigators at 15 hospitals in 10 prefectures throughout Japan retrieved serum and/or cerebrospinal fluid (CSF) samples collected in 2010–2021 from 517 patients hospitalized with encephalitis or meningitis which had an etiology that had not been determined. Samples were tested for Bbsl-specific antibodies using ELISA and Western blot tests. In alignment with the European Union LNB case definition, a confirmed LNB case had CSF pleocytosis and intrathecal production of Bbsl-specific antibodies and a probable LNB case had a CSF sample with pleocytosis and Bbsl-specific antibodies.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>LNB was identified in three hospitalized patients with meningitis of previously undetermined etiology: a male resident of Aomori Prefecture was a confirmed LNB case, and two female residents of Oita Prefecture were probable LNB cases. None of the patients with confirmed or probable LNB had traveled in the month prior to symptom onset and none had samples previously tested for LB.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>The identification of previously undiagnosed LNB cases indicates a need for enhanced disease awareness in Japan, particularly beyond Hokkaido Island, and more readily available LB diagnostic testing.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"32 1","pages":""},"PeriodicalIF":4.5,"publicationDate":"2025-01-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11729742/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142978018","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":"Factors associated with true-positive and false-positive diagnoses of behavioural variant frontotemporal dementia in 100 consecutive referrals from specialist physicians","authors":"Joshua Flavell,&nbsp;Emily G. M. Ahern,&nbsp;Benignus Logan,&nbsp;Thomas B. Shaw,&nbsp;Robert J. Adam,&nbsp;Caitlin A. T. McElligott,&nbsp;Peter J. Nestor","doi":"10.1111/ene.70036","DOIUrl":"10.1111/ene.70036","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>The behavioural variant of frontotemporal dementia (bvFTD) is a challenging diagnosis due to overlapping symptoms with psychiatric and other neurological conditions. Accordingly, misdiagnosis is common. The present study aimed to identify clinical factors contributing to misdiagnoses of bvFTD by specialist physicians.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We retrospectively analysed 100 consecutive referrals by specialist physicians (primarily psychiatrists, neurologists and geriatricians) to a tertiary cognitive disorders clinic specializing in frontotemporal lobar degenerative disorders. Patients were included if the referring specialist suspected bvFTD or if bvFTD was confirmed as the final diagnosis. Diagnostic factors were assessed by comparing the initial referral information with final clinical diagnoses.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Of the 100 patients, 34 were true-positive and 66 were false-positive for bvFTD. False-positive diagnoses were often based on misinterpretation of neuroimaging, particularly nuclear imaging (FDG-PET and HMPAO-SPECT), where subjective interpretation errors led to incorrect bvFTD diagnoses in 32 patients. Cognitive testing also contributed to misdiagnosis, with formal neuropsychological testing incorrectly leading to a bvFTD diagnosis in 20 patients. Patients with prior psychiatric histories were more likely to be misdiagnosed. Observable behavioural features of bvFTD and physical neurological signs were significantly more prevalent in true-positive patients.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Misinterpretation of neuroimaging and cognitive testing, in particular formal neuropsychological testing, significantly contributed to false-positive bvFTD diagnoses. Physicians should be cautious not to over-interpret neuroimaging and neuropsychology studies and be wary of patients with prior psychiatric histories. In contrast, greater weight should be placed on objective clinical observations of behavioural signs of bvFTD and the emergence of physical neurological signs.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"32 1","pages":""},"PeriodicalIF":4.5,"publicationDate":"2025-01-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11733081/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142983181","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 Sex on Clinical Outcomes of Tandem Occlusion in Acute Ischemic Stroke Patients Treated With Mechanical Thrombectomy. A Propensity-Matched Analysis","authors":"Lucio D'Anna,&nbsp;Matteo Foschi,&nbsp;Mariarosaria Valente,&nbsp;Liqun Zhang,&nbsp;Simona Sacco,&nbsp;Raffaele Ornello,&nbsp;Nina Mansoor,&nbsp;Matthew Fallon,&nbsp;Adelaida Gartner Jaramillo,&nbsp;Massimo Sponza,&nbsp;Vladimir Gavrilovic,&nbsp;Kyriakos Lobotesis,&nbsp;Gian Luigi Gigli,&nbsp;Soma Banerjee,&nbsp;Giovanni Merlino","doi":"10.1111/ene.70044","DOIUrl":"10.1111/ene.70044","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Although mechanical thrombectomy (MT) represents the standard of care for ischemic stroke due to large-vessel occlusion (LVO), the impact of sex on outcomes in tandem occlusions remains unclear. We investigated sex-based differences in outcomes after MT for tandem occlusions.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>This multicenter observational study included consecutive patients with tandem occlusion treated with MT across three stroke centers (2021–2023). Propensity score matching was performed. Primary outcomes were the 90-day favorable functional outcome (mRS 0–2) and mRS score shift. Secondary outcomes included favorable recanalization, 24-h early neurological improvement, and NIHSS median score. Safety outcomes were post-MT intracerebral hemorrhage and 90-day mortality.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Of 635 patients (46.8% women), 289 women were matched to 289 men. There were no significant differences in primary, secondary, or safety outcomes between sexes. Subgroup analysis showed a lower rate of favorable 90-day mRS scores in women with diabetes compared to men. Women not receiving emergent carotid treatment had higher rates of favourable outcomes. No significant sex differences were found in other subgroups.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Women with anterior circulation tandem occlusions treated with MT have similar outcomes to men. However, women with diabetes and those treated with intracranial MT alone exhibited sex-specific differences. Further studies are needed to explore underlying mechanisms.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"32 1","pages":""},"PeriodicalIF":4.5,"publicationDate":"2025-01-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11726627/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142970224","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":"Vacuolar myopathy with monoclonal gammopathy and stiffness (VAMMGAS)","authors":"Katia Staedler,&nbsp;Yves Allenbach,&nbsp;Emmanuelle Salort-Campana,&nbsp;Edoardo Malfatti,&nbsp;Aude Rigolet,&nbsp;Shahram Attarian,&nbsp;André Maues de Paula,&nbsp;Sarah Léonard-Louis,&nbsp;Olivier Benveniste,&nbsp;Tanya Stojkovic","doi":"10.1111/ene.70026","DOIUrl":"10.1111/ene.70026","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Monoclonal gammopathy (MG) has been reported in association with numerous neurological disorders but the spectrum of MG-associated myopathies remains poorly described.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>To report a newly acquired myopathy associated with MG.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Three adult patients with the same phenotype from two French referral centers were prospectively analyzed. Clinical, electrophysiological, muscle biopsy data, and patients' outcomes under treatment are reported.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>The patients, aged 37, 46, and 56 years, presented progressive weakness with subacute worsening and stiffness, in the context of severe weight loss. The weakness mainly involved the proximal limbs and axial muscles. Creatine kinase levels were 1400–2900 IU/L and electromyography revealed a myopathic pattern with spontaneous complex repetitive discharges. Muscle biopsies showed vacuoles containing glycogen and autophagic material along with the presence of sarcolemmal complement membrane attack complex deposits. There was no evidence of a genetic glycogen metabolic disorder. IgGκ monoclonal gammopathy was identified in all cases, without signs of lymphoplasmocytic proliferation. All patients improved with a treatment combining corticosteroids, intravenous immunoglobulins, and immunosuppressants, and two patients recovered walking ability.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion and Relevance</h3>\u0000 \u0000 <p>We report a new muscle disease defined by a vacuolar myopathy characterized by axial and proximal muscle weakness with prominent stiffness and high frequency discharges on electromyography associated with monoclonal gammopathy, defined under the acronym VAMMGAS.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"32 1","pages":""},"PeriodicalIF":4.5,"publicationDate":"2025-01-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11726623/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143055886","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}