European Journal of Neurology最新文献

{"title":"Long-term natural history in type II and III spinal muscular atrophy: a 4-year international study on the Hammersmith Functional Motor Scale Expanded","authors":"Giorgia Coratti,&nbsp;Francesca Bovis,&nbsp;Maria Carmela Pera,&nbsp;Matthew Civitello,&nbsp;Annemarie Rohwer,&nbsp;Francesca Salmin,&nbsp;Allan M. Glanzman,&nbsp;Jacqueline Montes,&nbsp;Amy Pasternak,&nbsp;Roberto De Sanctis,&nbsp;Sally Dunaway Young,&nbsp;Tina Duong,&nbsp;Irene Mizzoni,&nbsp;Evelin Milev,&nbsp;Maria Sframeli,&nbsp;Simone Morando,&nbsp;Emilio Albamonte,&nbsp;Adele D'Amico,&nbsp;Michela Catteruccia,&nbsp;Noemi Brolatti,&nbsp;Marika Pane,&nbsp;Mariacristina Scoto,&nbsp;Sonia Messina,&nbsp;Jesica Exposito Escudero,&nbsp;Liesbeth De Waele,&nbsp;Michio Hirano,&nbsp;Zarazuela Zolkipli-Cunningham,&nbsp;Basil T. Darras,&nbsp;Enrico Bertini,&nbsp;Andres Nascimiento Osorio,&nbsp;Claudio Bruno,&nbsp;Natalie Goemans,&nbsp;Valeria A. Sansone,&nbsp;John Day,&nbsp;Giovanni Baranello,&nbsp;Francesco Muntoni,&nbsp;Richard Finkel,&nbsp;Eugenio Mercuri,&nbsp;the ISMAC/international SMA consortium","doi":"10.1111/ene.16517","DOIUrl":"10.1111/ene.16517","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and purpose</h3>\u0000 \u0000 <p>Spinal muscular atrophy (SMA) is a genetic disorder caused by <i>SMN1</i> gene mutations. Although studies on available disease-modifying treatments have reported their efficacy and safety, long-term natural history data are lacking for comparison. The aim of this prospective study was to report 4-year changes on the Hammersmith Functional Motor Scale Expanded (HFMSE) in type II and III SMA in relation to several variables such as age, functional status and <i>SMN2</i> copy number.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>The study involves retrospective analysis of prospectively collected data from international datasets (Belgium, Italy, Spain, USA, UK). HFMSE longitudinal changes were analyzed using linear mixed effect models, examining annualized HFMSE change and its association with variables such as age at baseline, sex, motor function, <i>SMN2</i> copy number.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>In SMA type II (<i>n</i> = 226), the 4-year mean change was −2.20 points. The largest mean changes were observed in sitters aged 5–14 years and the lowest in those who lost the ability to sit unsupported. In SMA type III (<i>n</i> = 162), the 4-year mean change was −2.75 points. The largest mean changes were in those aged 7–15 years, whilst the lowest were in those below 7 and in the SMA type IIIa subgroup over 15. Age and score at baseline were predictive of 4-year changes.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Our findings provide natural history reference data for comparison with long-term follow-up of clinical trials or real-world data, highlighting the need to define patterns of changes in smaller SMA subgroups instead of reporting mean changes across an entire SMA cohort.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"31 12","pages":""},"PeriodicalIF":4.5,"publicationDate":"2024-10-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11555017/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142399769","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":"Pre-hospital transdermal glyceryl trinitrate for transient ischaemic attack: Data from the RIGHT-2 trial","authors":"Jason P. Appleton,&nbsp;Mark Dixon,&nbsp;Lisa J. Woodhouse,&nbsp;Craig S. Anderson,&nbsp;Sandeep Ankolekar,&nbsp;Lesley Cala,&nbsp;Timothy J. England,&nbsp;Peter J. Godolphin,&nbsp;Kailash Krishnan,&nbsp;Grant Mair,&nbsp;Keith W. Muir,&nbsp;John Potter,&nbsp;Chris I. Price,&nbsp;Marc Randall,&nbsp;Thompson G. Robinson,&nbsp;Christine Roffe,&nbsp;Peter M. Rothwell,&nbsp;Else Charlotte Sandset,&nbsp;Jeffrey L. Saver,&nbsp;A. Niroshan Siriwardena,&nbsp;Joanna M. Wardlaw,&nbsp;Nikola Sprigg,&nbsp;Philip M. Bath,&nbsp;the RIGHT-2 Investigators","doi":"10.1111/ene.16502","DOIUrl":"10.1111/ene.16502","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and purpose</h3>\u0000 \u0000 <p>Ambulance trials assessing interventions in suspected stroke patients will recruit patients with currently active symptoms that will resolve into transient ischaemic attack (TIA). The safety and efficacy of glyceryl trinitrate (GTN) in the pre-specified subgroup of patients with TIA in the Rapid Intervention with Glyceryl Trinitrate in Hypertensive Stroke Trial 2 (RIGHT-2) was assessed.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>RIGHT-2 was a pre-hospital-initiated multicentre randomized sham-controlled blinded-endpoint trial that randomized patients with presumed ultra-acute stroke within 4 h of symptom onset to transdermal GTN or sham. Final diagnosis was determined by site investigators. The primary outcome was a shift in modified Rankin Scale (mRS) scores at 90 days analysed using ordinal logistic regression reported as adjusted common odds ratio with 95% confidence intervals (CIs). Secondary outcomes included death or dependence (mRS &gt;2).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>In all, 109 of 1149 (9.5%) patients had a final diagnosis of TIA (GTN 57, sham 52) with mean age 73 (SD 13) years, 19 (17.4%) had pre-morbid mRS &gt;2, and onset to randomization was 80 min (interquartile range 49, 105). GTN lowered blood pressure by 7.4/5.2 mmHg compared with sham by hospital arrival. At day 90, GTN had no effect on shift in mRS scores (common odds ratio for increased dependence 1.47, 95% CI 0.70–3.11) but was associated with increased death or dependence (mRS &gt;2): GTN 29 (51.8%) versus sham 23 (46.9%), odds ratio 3.86 (95% CI 1.09–13.59).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Pre-hospital ultra-acute transdermal GTN did not improve overall functional outcome in patients with investigator-diagnosed TIA compared with sham treatment.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"31 12","pages":""},"PeriodicalIF":4.5,"publicationDate":"2024-10-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11554870/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142399770","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":"Characteristics and temporal evolution of asymptomatic diffusion-weighted imaging lesions in patients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL)","authors":"Ying-Chi Shen,&nbsp;Ya-Fang Chen,&nbsp;Yu-Wen Cheng,&nbsp;Chih-Hao Chen,&nbsp;Jiann-Shing Jeng,&nbsp;Sung-Chun Tang","doi":"10.1111/ene.16519","DOIUrl":"10.1111/ene.16519","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and Purpose</h3>\u0000 \u0000 <p>The role of asymptomatic diffusion-weighted imaging-positive (aDWI+) lesions in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) patients remains unclear, and their radiographic features may differ from those of symptomatic diffusion-weighted imaging-positive (sDWI+) lesions. We aimed to investigate the clinicoradiographic characteristics of aDWI+ lesions in CADASIL patients.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We conducted a retrospective analysis using data from the Taiwan CADASIL Registry. aDWI+ lesions were defined as incidentally detected DWI+ lesions without corresponding acute neurological deficits. We compared the baseline clinical characteristics of patients with and without aDWI+ lesions and analyzed their radiological features and evolution in relation to sDWI+ lesions.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Among 154 enrolled patients (mean age 62 ± 10 years), 17 (11%) had aDWI+ lesions. Baseline clinical characteristics were similar in the two groups, but those with aDWI+ lesions had more lacunes (median 8 vs. 2), multiple cerebral microbleeds (CMBs; 85% vs. 40%), and anterior temporal white matter hyperintensity (WMH; 47% vs. 14%). Multivariable analysis showed that aDWI+ lesions were associated with anterior temporal WMH (odds ratio 5.7, 95% confidence interval 1.5–21.0) after adjusting for multiple lacunes, multiple CMBs, and total WMH score. Compared to sDWI+ lesions, aDWI+ lesions were more often small infarcts (&lt;1 cm; 89% vs. 23%) and less likely to involve the corticospinal tract (11% vs. 96%). Among the 11 aDWI+ lesions with follow-up magnetic resonance imaging, seven became microinfarcts, three became lacunes, and one disappeared.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>aDWI+ lesions in CADASIL are not uncommon and are associated with higher burdens of small vessel disease and anterior temporal WMH. Further research is needed to assess their long-term impact on CADASIL.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"31 12","pages":""},"PeriodicalIF":4.5,"publicationDate":"2024-10-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11554854/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142399768","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":"Autonomic nervous system education in Europe: EAN/EFAS/INUS survey on curricula and skills in autonomic medicine of European neurology residents and consultants","authors":"Diogo Reis-Carneiro,&nbsp;Magdalena Krbot Skoric,&nbsp;Mario Habek,&nbsp;Ivan Adamec,&nbsp;Giovanna Calandra-Buonaura,&nbsp;Pietro Cortelli,&nbsp;J. Gert van Dijk,&nbsp;Cristian Falup-Pecurariu,&nbsp;Pietro Guaraldi,&nbsp;Max J. Hilz,&nbsp;Valeria Iodice,&nbsp;Jens Jordan,&nbsp;Isabel Rocha,&nbsp;Walter Struhal,&nbsp;Astrid Juhl Terkelsen,&nbsp;Roland Thijs,&nbsp;Beatriz Tijero,&nbsp;Thomas Berger,&nbsp;Irena Rektorova,&nbsp;Elena Moro,&nbsp;Anne Pavy-Le Traon,&nbsp;Gregor Wenning,&nbsp;Jalesh N. Panicker,&nbsp;Alessandra Fanciulli","doi":"10.1111/ene.16515","DOIUrl":"10.1111/ene.16515","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and purpose</h3>\u0000 \u0000 <p>Centers for training in autonomic nervous system (ANS) disorders are not widely available and the recent coronavirus 2019 pandemic temporarily reduced training opportunities in autonomic medicine across European countries. Here we evaluated the current state of education, clinical skills and postgraduate educational preferences on ANS disorders of European neurology residents and consultants.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>A 23-item questionnaire was developed and distributed online amongst European neurology residents and consultants via mailing lists of the European Academy of Neurology. The questions assessed demographics, current training opportunities and learning preferences in ANS disorders. Six multiple-choice questions were used to self-evaluate knowledge of ANS disorders.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>In all, 285 individuals answered the survey (60% female, mostly 25–34 years of age). All respondents considered clinical autonomic skills necessary for good clinical neurological practice, and 92% would like to increase their ANS knowledge. Female respondents and those who trained in Southern/Eastern/Greater Europe more frequently judged ANS skills important for clinical practice than male respondents (<i>p</i> = 0.012) and respondents from Northern/Western Europe (<i>p</i> = 0.011). Female and younger respondents felt less confident in managing ANS disorders (<i>p</i> = 0.001 and <i>p</i> &lt; 0.001, respectively). Respondents below 45 years of age (<i>p</i> &lt; 0.001) and those with lower confidence in managing ANS disorders (<i>p</i> = 0.004) were more likely to recommend that ANS education is embedded in the residency curriculum.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Most European neurology residents and consultants reported a need for more autonomic education, with additional gender, age and regional differences. These findings underscore the importance of increasing the educational content on autonomic medicine in European medical and postgraduate curricula.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"31 12","pages":""},"PeriodicalIF":4.5,"publicationDate":"2024-10-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11555144/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142461074","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Serum liver enzymes and risk of stroke: Systematic review with meta-analyses and Mendelian randomization studies","authors":"Chun Li,&nbsp;Long Gu,&nbsp;Fu-Yi Shi,&nbsp;Shi-Ying Xiong,&nbsp;Gui-Sheng Wu,&nbsp;Jian-Hua Peng,&nbsp;Ruo-Lan Wang,&nbsp;Yuan Yuan,&nbsp;Yong Jiang,&nbsp;Chen Huang,&nbsp;Huai-Rong Luo","doi":"10.1111/ene.16506","DOIUrl":"10.1111/ene.16506","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and purpose</h3>\u0000 \u0000 <p>Previous observational studies have identified correlations between liver enzyme levels and stroke risk. However, the strength and consistency of these associations vary. To comprehensively evaluate the relationship between liver enzymes and stroke risk, we conducted meta-analyses complemented by Mendelian randomization (MR) analyses.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Following the PRISMA guidelines, we performed meta-analyses of prospective studies and conducted subgroup analyses stratified by sex and stroke subtype. Subsequently, adhering to the STROBE-MR guidelines, we performed two-sample bidirectional univariable MR (UVMR) and multivariable MR (MVMR) analyses using the largest genome-wide association studies summary data. Finally, the single-nucleotide polymorphisms associated with liver enzymes on sex differences underwent gene annotation, gene set enrichment, and tissue enrichment analyses.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>In the meta-analyses of 17 prospective studies, we found the relative risks for serum γ-glutamyl transferase (GGT) and alkaline phosphatase (ALP) were 1.23 (95% CI: 1.16–1.31) and 1.3 (95% CI: 1.19–1.43), respectively. Subgroup analyses revealed sex and stroke subtype differences in liver enzyme-related stroke risk. Bidirectional UVMR analyses confirmed that elevated GGT, alanine aminotransferase, and aspartate aminotransferase levels were associated with increased stroke occurrence. The primary results from the MVMR analyses revealed that higher ALP levels significantly increased the risk of stroke and ischemic stroke. Gene set and tissue enrichment analyses supported genetic differences in liver enzymes across sexes.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Our study provides evidence linking liver enzyme levels to stroke risk, suggesting liver enzymes as potential biomarkers for early identification of high-risk individuals. Personalized, sex-specific interventions targeting liver enzymes could offer new strategies for stroke prevention.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"31 12","pages":""},"PeriodicalIF":4.5,"publicationDate":"2024-10-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11555028/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142461080","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":"Economic and societal burden of myasthenia gravis in Denmark, Finland, and Sweden: A population-based registry study","authors":"Fredrik Piehl,&nbsp;John Vissing,&nbsp;Juha Mehtälä,&nbsp;Fredrik Berggren,&nbsp;Ingrid Lindberg-Schager,&nbsp;Didier Pitsi,&nbsp;Evangelos Tsitlakidis,&nbsp;Aino Vesikansa,&nbsp;Riina-Minna Väänänen,&nbsp;Tero Ylisaukko-oja,&nbsp;Sari Atula","doi":"10.1111/ene.16511","DOIUrl":"10.1111/ene.16511","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and purpose</h3>\u0000 \u0000 <p>Health care resource utilization (HCRU) and the economic burden of myasthenia gravis (MG) are significant, but existing studies rarely include comprehensive nationwide data. We examined HCRU and direct and indirect costs associated with MG overall and by disease severity in Denmark, Finland, and Sweden.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Data were collected retrospectively from nationwide health and social care registries. All individuals ≥18 years of age with ≥2 International Classification of Diseases diagnoses of MG between 2000 and 2020 were included. HCRU, direct (inpatient and outpatient contacts, medication) and indirect costs (early retirement, sick leave, death), and associated factors were calculated.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>The full study cohort comprised 8622 people with MG (pwMG). Mean annual numbers of all-cause secondary health care contacts for pwMG were 3.4 (SD = 8.3), 7.0 (SD = 12.3), and 2.9 (SD = 3.9), with mean annual total costs of €12,185, €9036, and €5997 per person in Denmark, Finland, and Sweden, respectively. Inpatient periods, involving 77%–89% of study participants in the three countries, contributed most to direct costs, whereas the majority of indirect costs resulted from early retirement in Denmark and Finland, and sick leave periods in Sweden. Mean annual total costs were highest with very severe MG (€19,570–€33,495 per person across the three countries). Female sex and comorbidities, such as mental and behavioral disorders and severe infections, were also associated with higher total costs.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>This population-based study shows a high level of HCRU and a significant direct and indirect economic burden of MG across three Nordic countries, especially for severe forms of MG.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"31 12","pages":""},"PeriodicalIF":4.5,"publicationDate":"2024-10-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11555013/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142389067","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":"Time to response with ravulizumab, a long-acting terminal complement inhibitor, in adults with anti-acetylcholine receptor antibody-positive generalized myasthenia gravis","authors":"Ali A. Habib,&nbsp;Michael Benatar,&nbsp;Tuan Vu,&nbsp;Andreas Meisel,&nbsp;Shahram Attarian,&nbsp;Masahisa Katsuno,&nbsp;Serena Liao,&nbsp;Kathleen N. Beasley,&nbsp;James F. Howard Jr","doi":"10.1111/ene.16490","DOIUrl":"10.1111/ene.16490","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and Purpose</h3>\u0000 \u0000 <p>The efficacy and safety of ravulizumab, a terminal complement C5 inhibitor, in adults with anti-acetylcholine receptor antibody-positive (AChR Ab+) generalized myasthenia gravis (gMG) were demonstrated in the CHAMPION MG study (NCT03920293). This analysis aimed to characterize the latency to onset of a clinically meaningful therapeutic effect for ravulizumab.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Post hoc analysis of data collected for up to 60 weeks from CHAMPION MG was performed to assess the timing of response to ravulizumab. Response was analyzed based on reductions of ≥2 and ≥3 points (minimal clinically important differences [MCIDs]) in Myasthenia Gravis–Activities of Daily Living (MG-ADL) and Quantitative Myasthenia Gravis (QMG) total scores, respectively, and on more rigorous reductions of ≥3 and ≥5 points, respectively. Time to first response was assessed using the Kaplan–Meier product-limit method.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>The median (95% confidence interval) time to first response was 2.1 (2.1–2.6) and 4.1 (2.3–10.0) weeks for reductions of ≥2 and ≥3 points in MG-ADL total score, respectively (<i>n</i> = 139), and 4.1 (2.1–10.0) and 18.3 (11.0–33.4) weeks for reductions of ≥3 and ≥5 points in QMG total score, respectively (<i>n</i> = 134). Cumulative response rates at Week 60 (data cut-off) were 88% and 82% for ≥2- and ≥3-point MG-ADL score reductions, respectively, and 86% and 59% for ≥3- and ≥5-point QMG score reductions, respectively.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>The median times to MCID with ravulizumab treatment in patients with AChR Ab+ gMG were ~2 weeks and ~4 weeks based on MCID MG-ADL and QMG total score reductions, respectively.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"31 12","pages":""},"PeriodicalIF":4.5,"publicationDate":"2024-10-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11555155/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142380298","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 effect of influenza vaccination on the rate of dementia amongst older adults","authors":"Andreas Moses Appel,&nbsp;Janet Janbek,&nbsp;Christina Jensen-Dahm,&nbsp;Thomas Munk Laursen,&nbsp;Gunhild Waldemar","doi":"10.1111/ene.16489","DOIUrl":"10.1111/ene.16489","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and Purpose</h3>\u0000 \u0000 <p>Previous studies have reported conflicting results regarding the association between influenza vaccination and dementia. This association was investigated in a nationwide register-based cohort study.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Using nationwide registries, dementia-free adults aged ≥65 years in Denmark from 2002 to 2018 without previous influenza vaccinations were included. Poisson regression facilitated confounder-adjusted comparisons of dementia rates for ever versus never vaccinated, number of vaccinations and within/after 5 years from first vaccination. Sensitivity analyses included stratification on age and sex.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Vaccination during follow-up was associated with a slightly higher rate of dementia when adjusted for sociodemographic factors and comorbidities, both within and after the first 5 years from first vaccination (incidence rate ratio [IRR] 1.04; 95% confidence interval [CI] 1.03–1.05). The rate of dementia decreased with increasing number of vaccinations. The highest rate was amongst those with only one vaccination (IRR 1.14; 95% CI 1.12–1.17) and the rate of dementia was only decreased amongst those with six or more vaccinations (IRR 0.95; 95% CI 0.93–0.97). Applying the same models to control outcomes of hip fracture and cancer resulted in higher rates amongst vaccinated people of 6% and 7%, respectively. Vaccinated people also had a 10% higher mortality rate.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Discussion</h3>\u0000 \u0000 <p>Our results do not support the case for a preventive effect of influenza vaccination on the risk of dementia in the general population, as reported by some previous studies. However, the higher dementia rate amongst vaccinated people found in this study is probably due to residual confounding, indicated by a higher rate for control outcomes and mortality.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"31 12","pages":""},"PeriodicalIF":4.5,"publicationDate":"2024-10-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11554864/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142380297","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":"Cerebral venous thrombosis in elderly patients","authors":"Victor Garcia,&nbsp;Louise Bicart-Sée,&nbsp;Isabelle Crassard,&nbsp;Nicolas Legris,&nbsp;Mathieu Zuber,&nbsp;Fernando Pico,&nbsp;Céline Guidoux,&nbsp;Michael Obadia,&nbsp;Naouel Boulenoir,&nbsp;Didier Smadja,&nbsp;Mikael Mazighi,&nbsp;Cecile Lavenu-Bombled,&nbsp;Elodie Baudry,&nbsp;Bertrand Lapergue,&nbsp;Guillaume Turc,&nbsp;Philippe Tuppin,&nbsp;Christian Denier","doi":"10.1111/ene.16504","DOIUrl":"10.1111/ene.16504","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and purpose</h3>\u0000 \u0000 <p>We aimed to report the characteristics of cerebral venous thrombosis (CVT) in elderly people (aged ≥65 years).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>This multicenter retrospective cohort included elderly patients hospitalized for a first CVT in nine Paris–Ile-de-France hospitals between 2011 and 2021. The estimated incidence was compared to CVT recorded by the French health insurance data system. Lariboisière Hospital's CVT registry allowed comparisons of our elderly cohort with individuals younger than 65 years.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>One hundred fourteen patients were included in this study (mean age = 74.2 years, range = 65–93, 61% female). The CVT annual incidence in Ile-de-France was 5.9–7.1 per million elderly individuals versus 8.5 per million nationwide. Headaches and focal deficits were the most common initial clinical features (50% and 51%, respectively), followed by seizures and confusion (40% and 27%). Treatment included anticoagulation (93%) and, rarely, endovascular procedure (2%) or craniectomy (1%). Compared with adult patients aged &lt;65 years (younger adults), elderly patients presented fewer headaches (50% vs. 96%, <i>p</i> &lt; 0.01) and intracranial hypertension (7% vs. 22%, <i>p</i> &lt; 0.01) but more seizures and focal deficits (40% vs. 27% and 51% vs. 38%, respectively, <i>p</i> &lt; 0.01). Underlying cancer, hemopathy, and locoregional infections were more frequent in elderly patients than among younger adults (<i>p</i> &lt; 0.01). The prognosis of patients from our elderly cohort was poorer than that of younger adults; 8% died in the acute phase, and 73% had a favorable outcome at 1 year (vs. 1.7% and 87%, respectively, <i>p</i> &lt; 0.01).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>CVT in elderly patients has a specific clinical presentation, epidemiology, and risk factors such as cancer or hemopathy, justifying specialized management.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"31 12","pages":""},"PeriodicalIF":4.5,"publicationDate":"2024-10-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11554858/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142375313","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":"Immune triggers preceding neuralgic amyotrophy","authors":"Davide Sparasci,&nbsp;Lenka Schilg-Hafer,&nbsp;Bettina Schreiner,&nbsp;Olivier Scheidegger,&nbsp;Anne-Kathrin Peyer,&nbsp;Agustina Maria Lascano,&nbsp;Alex Vicino,&nbsp;Bernhard Friedrich Décard,&nbsp;Pinelopi Tsouni,&nbsp;Andrea Monika Humm,&nbsp;Enea Pianezzi,&nbsp;Giulia Zezza,&nbsp;Thomas Hundsberger,&nbsp;Anelia Dietmann,&nbsp;Hans H. Jung,&nbsp;Thierry Kuntzer,&nbsp;Einar Wilder-Smith,&nbsp;Gladys Martinetti-Lucchini,&nbsp;Orlando Petrini,&nbsp;Stefano Fontana,&nbsp;Peter Gowland,&nbsp;Christoph Niederhauser,&nbsp;Claudio Gobbi,&nbsp;Paolo Ripellino","doi":"10.1111/ene.16462","DOIUrl":"10.1111/ene.16462","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and purpose</h3>\u0000 \u0000 <p>Infections and vaccinations have been identified as potential immunological triggers of neuralgic amyotrophy (NA), but the exact type and frequency of the preceding agents is unknown.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>This was a multicentre, prospective, observational, matched case–control study. NA was diagnosed by neuromuscular experts according to validated clinical criteria and electrodiagnostic studies. Clinical data and biological samples of NA patients were collected within 90 days from disease onset between June 2018 and December 2023. All NA patients were asked about prior infection and vaccination in the month before disease onset. Serological tests for hepatitis E virus, human immunodeficiency virus, severe acute respiratory syndrome coronavirus 2, Epstein–Barr virus, cytomegalovirus, parvovirus B19, varicella-zoster virus, <i>Borrelia burgdorferi</i>, <i>Mycoplasma pneumoniae</i> and <i>Bartonella henselae</i> were performed in a central laboratory. Each case was matched with a healthy control for age, sex, place of residence and time of blood collection.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Fifty-seven patients and corresponding controls were included. The mean age was 45 years for both groups. NA onset was preceded by a symptomatic infectious trigger confirmed by microbiological tests in 15/57 (26.3%) patients. Coronavirus disease 2019 vaccination was considered a potential trigger in 7/57 (12.3%) subjects. An acute viral infection was associated with a bilateral involvement of the brachial plexus (<i>p</i> = 0.003, Cramèr's <i>V</i> = 0.43).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Confirmed immune triggers (infection or vaccination) preceded disease onset in 22/57 (38.6%) NA cases. We suggest to test NA patients in the acute phase for intracellular antigens, especially in the case of concomitant bilateral involvement and hepatitis.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11954,"journal":{"name":"European Journal of Neurology","volume":"31 12","pages":""},"PeriodicalIF":4.5,"publicationDate":"2024-10-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11554871/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142371281","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}