Journal of Neurology, Neurosurgery, and Psychiatry最新文献

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Observational study of changes to glucocorticosteroid prescribing patterns in duchenne muscular dystrophy in the UK over the last decade. 对过去十年英国杜氏肌营养不良症患者糖皮质激素处方模式变化的观察研究。
IF 8.7 1区 医学
Journal of Neurology, Neurosurgery, and Psychiatry Pub Date : 2025-02-12 DOI: 10.1136/jnnp-2024-335223
Gregory Landon, Georgia Stimpson, Michela Guglieri, Anna Sarkozy, Adnan Y Manzur, Francesco Muntoni, Giovanni Baranello
{"title":"Observational study of changes to glucocorticosteroid prescribing patterns in duchenne muscular dystrophy in the UK over the last decade.","authors":"Gregory Landon, Georgia Stimpson, Michela Guglieri, Anna Sarkozy, Adnan Y Manzur, Francesco Muntoni, Giovanni Baranello","doi":"10.1136/jnnp-2024-335223","DOIUrl":"https://doi.org/10.1136/jnnp-2024-335223","url":null,"abstract":"<p><strong>Background: </strong>Glucocorticosteroids (GC) are standard-of-care treatment for most boys with duchenne muscular dystrophy (DMD). GC use has changed over time with evolving evidence, and we describe GC patterns, dosing and side-effects in the UK over 11 years.</p><p><strong>Method: </strong>NorthStar data from 2012 to 2022 were analysed to understand GC type, regime and starting age. GC dose with age, patterns of GC switching and side-effect profiles by type and regime were also analysed. Participants attributed to 'other' regimes were queried and details were included.</p><p><strong>Results: </strong>Data on GC usage were available for 1117 boys, across 6905 observations, with 74% of boys GC treated. Prednisolone was the most common regime in the period (65% of assessments) but deflazacort prescription has increased (17% in 2012 and 43% in 2022). Daily regimes were more common (66% of assessments), and the incidence of intermittent (10 days on/10 days off) regimes has declined (46% in 2012 and 26% in 2022). Older participants were more commonly on less than recommended doses, and this was more common in those on deflazacort or daily regimes. Gastrointestinal symptoms and cushingoid features were more common in those on deflazacort than prednisolone, while increased appetite, cushingoid features, gastrointestinal symptoms and insomnia were more common in those on daily than intermittent regimes.</p><p><strong>Conclusions: </strong>The use of deflazacort and daily regimes has steadily increased across the UK North Star Network in the last decade. This study provides one of the largest up-to-date real-world set of data of evolution in prescription patterns and the occurrence of side-effects in different groups of GC-treated DMD.</p>","PeriodicalId":16418,"journal":{"name":"Journal of Neurology, Neurosurgery, and Psychiatry","volume":" ","pages":""},"PeriodicalIF":8.7,"publicationDate":"2025-02-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143408706","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"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
Efficacy and safety of preoperative embolization in surgical treatment of brain arteriovenous malformations: a multicentre study with propensity score matching. 术前栓塞治疗脑动静脉畸形的疗效和安全性:一项倾向评分匹配的多中心研究。
IF 8.7 1区 医学
Journal of Neurology, Neurosurgery, and Psychiatry Pub Date : 2025-02-06 DOI: 10.1136/jnnp-2024-334974
Hamza Salim, Dawoud Hamdan, Nimer Adeeb, Sandeep Kandregula, Assala Aslan, Basel Musmar, Christopher S Ogilvy, Adam A Dmytriw, Ahmed Abdelsalam, Cagdas Ataoglu, Ufuk Erginoglu, Douglas Kondziolka, Kareem El Naamani, Jason Sheehan, Natasha Ironside, Deepak Kumbhare, Sanjeev Gummadi, Muhammed Amir Essibayi, Salem M Tos, Abdullah Keles, Sandeep Muram, Daniel Sconzo, Arwin Rezai, Omar Alwakaa, Johannes Pöppe, Rajeev D Sen, Mustafa K Baskaya, Christoph J Griessenauer, Pascal Jabbour, Stavropoula I Tjoumakaris, Elias Atallah, Howard Riina, Abdallah Abushehab, Christian Swaid, Jan-Karl Burkhardt, Robert M Starke, Laligam N Sekhar, Michael R Levitt, David J Altschul, Neil Haranhalli, Malia McAvoy, Adib Abla, Christopher Stapleton, Matthew J Koch, Visish M Srinivasan, Peng Roc Chen, Spiros Blackburn, Joseph Cochran, Omar Choudhri, Bryan Pukenas, Darren B Orbach, Edward R Smith, Markus Moehlenbruch, Pascal J Mosimann, Ali Alaraj, Mohammad Ali Aziz-Sultan, Aman B Patel, Vivek Yedavalli, Max Wintermark, Amey Savardekar, Hugo H Cuellar, Michael T Lawton, Jacques J Morcos, Bharat Guthikonda
{"title":"Efficacy and safety of preoperative embolization in surgical treatment of brain arteriovenous malformations: a multicentre study with propensity score matching.","authors":"Hamza Salim, Dawoud Hamdan, Nimer Adeeb, Sandeep Kandregula, Assala Aslan, Basel Musmar, Christopher S Ogilvy, Adam A Dmytriw, Ahmed Abdelsalam, Cagdas Ataoglu, Ufuk Erginoglu, Douglas Kondziolka, Kareem El Naamani, Jason Sheehan, Natasha Ironside, Deepak Kumbhare, Sanjeev Gummadi, Muhammed Amir Essibayi, Salem M Tos, Abdullah Keles, Sandeep Muram, Daniel Sconzo, Arwin Rezai, Omar Alwakaa, Johannes Pöppe, Rajeev D Sen, Mustafa K Baskaya, Christoph J Griessenauer, Pascal Jabbour, Stavropoula I Tjoumakaris, Elias Atallah, Howard Riina, Abdallah Abushehab, Christian Swaid, Jan-Karl Burkhardt, Robert M Starke, Laligam N Sekhar, Michael R Levitt, David J Altschul, Neil Haranhalli, Malia McAvoy, Adib Abla, Christopher Stapleton, Matthew J Koch, Visish M Srinivasan, Peng Roc Chen, Spiros Blackburn, Joseph Cochran, Omar Choudhri, Bryan Pukenas, Darren B Orbach, Edward R Smith, Markus Moehlenbruch, Pascal J Mosimann, Ali Alaraj, Mohammad Ali Aziz-Sultan, Aman B Patel, Vivek Yedavalli, Max Wintermark, Amey Savardekar, Hugo H Cuellar, Michael T Lawton, Jacques J Morcos, Bharat Guthikonda","doi":"10.1136/jnnp-2024-334974","DOIUrl":"https://doi.org/10.1136/jnnp-2024-334974","url":null,"abstract":"<p><strong>Background: </strong>Brain arteriovenous malformations (AVMs) are abnormal connections between feeding arteries and draining veins, associated with significant risks of haemorrhage, seizures and other neurological deficits. Preoperative embolization is commonly used as an adjunct to microsurgical resection, with the aim of reducing intraoperative complications and improving outcomes. However, the efficacy and safety of this approach remain controversial.</p><p><strong>Methods: </strong>This study is a subanalysis of the Multicenter International Study for Treatment of Brain AVMs consortium. We retrospectively analysed 486 patients with brain AVMs treated with microsurgical resection between January 2010 and December 2023. Patients were divided into two groups: those who underwent microsurgery alone (n=245) and those who received preoperative embolization, followed by microsurgery (n=241). Propensity score matching was employed, resulting in 288 matched patients (144 in each group). The primary outcomes were rates of complete AVM obliteration and functional outcomes (measured by the modified Rankin Scale (mRS)). Secondary outcomes included complication rates, mortality, hospital length of stay and postsurgical rupture.</p><p><strong>Results: </strong>After matching, the complete obliteration rate was 97% with no significant difference between the microsurgery-only group and the preoperative embolization group (p=0.12). The proportion of patients with an mRS score of 0-2 at the last follow-up was similar in both groups (83% vs 84%; p=0.67). The median hospital stay was significantly longer for the embolisation group (9 days vs 7 days; p=0.017). Complication rates (24% vs 22%; p=0.57) and mortality rates (4.9% vs 2.1%; p=0.20) were comparable between the two groups. No significant differences were observed in postsurgical rupture, recurrence or retreatment rates.</p><p><strong>Conclusions: </strong>In this large multicentre study, preoperative embolization did not significantly improve AVM obliteration rates, functional outcomes or reduce complications compared with microsurgery alone.</p>","PeriodicalId":16418,"journal":{"name":"Journal of Neurology, Neurosurgery, and Psychiatry","volume":" ","pages":""},"PeriodicalIF":8.7,"publicationDate":"2025-02-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143365090","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"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
Guidance for clinical management of pathogenic variant carriers at elevated genetic risk for ALS/FTD. ALS/FTD 遗传风险较高的致病变异携带者临床管理指南。
IF 8.7 1区 医学
Journal of Neurology, Neurosurgery, and Psychiatry Pub Date : 2025-01-31 DOI: 10.1136/jnnp-2024-334339
Michael Benatar, Terry D Heiman-Patterson, Johnathan Cooper-Knock, Daniel Brickman, Kaitlin B Casaletto, Stephen A Goutman, Marco Vinceti, Laynie Dratch, Jalayne J Arias, Jean Swidler, Martin R Turner, Jeremy Shefner, Henk-Jan Westeneng, Leonard H van den Berg, Ammar Al-Chalabi
{"title":"Guidance for clinical management of pathogenic variant carriers at elevated genetic risk for ALS/FTD.","authors":"Michael Benatar, Terry D Heiman-Patterson, Johnathan Cooper-Knock, Daniel Brickman, Kaitlin B Casaletto, Stephen A Goutman, Marco Vinceti, Laynie Dratch, Jalayne J Arias, Jean Swidler, Martin R Turner, Jeremy Shefner, Henk-Jan Westeneng, Leonard H van den Berg, Ammar Al-Chalabi","doi":"10.1136/jnnp-2024-334339","DOIUrl":"10.1136/jnnp-2024-334339","url":null,"abstract":"<p><p>There is a growing understanding of the presymptomatic stages of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) and nascent efforts aiming to prevent these devastating neurodegenerative diseases have emerged. This progress is attributable, in no small part, to the altruism of people living with pathogenic variants at elevated genetic risk for ALS/FTD via their willingness to participate in natural history studies and disease prevention trials. Increasingly, this community has also highlighted the urgent need to develop paradigms for providing appropriate clinical care for those at elevated risk for ALS and FTD. This manuscript summarises recommendations emanating from a multi-stakeholder Workshop (Malvern, Pennsylvania, 2023) that aimed to develop guidance for at-risk carriers and their treating physicians. Clinical care recommendations span genetic testing (including counselling and sociolegal implications); monitoring for the emergence of early motor, cognitive and behavioural signs of disease; and the use of Food and Drug Administration-approved small molecule drugs and gene-targeting therapies. Lifestyle recommendations focus on exercise, smoking, statin use, supplement use, caffeine intake and head trauma, as well as occupational and environmental exposures. While the evidence base to inform clinical and lifestyle recommendations is limited, this guidance document aims to appraise carriers and clinicians of the issues and best available evidence, and also to define the research agenda that could yield more evidence-informed guidelines.</p>","PeriodicalId":16418,"journal":{"name":"Journal of Neurology, Neurosurgery, and Psychiatry","volume":" ","pages":""},"PeriodicalIF":8.7,"publicationDate":"2025-01-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12015018/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142687294","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"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
Cognitive changes in patients with relapse-free MS treated with high efficacy therapies: the predictive value of paramagnetic rim lesions. 接受高效治疗的无复发MS患者的认知变化:顺磁边缘病变的预测价值。
IF 8.7 1区 医学
Journal of Neurology, Neurosurgery, and Psychiatry Pub Date : 2025-01-31 DOI: 10.1136/jnnp-2024-335144
Vincenzo Daniele Boccia, Elisa Leveraro, Emilio Cipriano, Caterina Lapucci, Tommaso Sirito, Maria Cellerino, Giacomo Rebella, Lorenza Nasone, Giacomo Boffa, Matilde Inglese
{"title":"Cognitive changes in patients with relapse-free MS treated with high efficacy therapies: the predictive value of paramagnetic rim lesions.","authors":"Vincenzo Daniele Boccia, Elisa Leveraro, Emilio Cipriano, Caterina Lapucci, Tommaso Sirito, Maria Cellerino, Giacomo Rebella, Lorenza Nasone, Giacomo Boffa, Matilde Inglese","doi":"10.1136/jnnp-2024-335144","DOIUrl":"https://doi.org/10.1136/jnnp-2024-335144","url":null,"abstract":"<p><strong>Background: </strong>High-efficacy disease-modifying therapies (HETs) have substantially improved multiple sclerosis (MS) management, yet ongoing cognitive decline remains a concern. This study aims to assess Symbol Digit Modalities Test (SDMT) changes in patients with stable relapsing-remitting MS (RRMS) treated with HETs and to evaluate the role of baseline MRI biomarkers as predictors of SDMT changes.</p><p><strong>Methods: </strong>Consecutive patients with RRMS treated with HETs underwent clinical, SDMT and MRI assessment at baseline with SDMT and clinical re-evaluation after 24 months. Patients presenting relapses or MRI activity (new T2 and/or gadolinium-enhancing lesions) during follow-up were excluded. Cognitive changes were defined using the 90% CI regression-based reliable change index methodology accounting for sex, age, education and baseline score. Baseline MRI examination included three-dimensional-sagittal Fluid Attenuated Inversion Recovery (FLAIR), T1-Magnetization Prepared - RApid Gradient Echo (T1-MPRAGE) and quantitative susceptibility mapping (QSM) for paramagnetic rim lesions (PRLs) and QSM-isointense lesions (ISO) assessment. Univariate and multivariable regression analyses were performed to predict SDMT changes.</p><p><strong>Results: </strong>90 patients (mean age: 40.3 years, median Expanded Disability Status Scale: 2.0) were included. PRLs were present in 46 (51.1%) patients. After 24 months, 13 (14.4%) patients showed SDMT decline and 8 (8.9%) showed improvement. At multivariable analyses, PRLs were associated with higher risk of SDMT decline (β: 2.70, p: 0.02, OR: 14.82) while higher ISO lesion volumes were weakly associated with SDMT improvement (β: 0.07, p: 0.01, OR: 1.07).</p><p><strong>Conclusions: </strong>SDMT decline and improvement are detectable in patients with RRMS without clinical or MRI activity over 2 years. PRLs seem to predict SDMT decline in MS, underscoring the critical role of compartmentalised chronic inflammation in disease progression.</p>","PeriodicalId":16418,"journal":{"name":"Journal of Neurology, Neurosurgery, and Psychiatry","volume":" ","pages":""},"PeriodicalIF":8.7,"publicationDate":"2025-01-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143074858","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"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
Working years lost in people with epilepsy: a population-based cohort study. 癫痫患者工作时间减少:一项基于人群的队列研究。
IF 8.7 1区 医学
Journal of Neurology, Neurosurgery, and Psychiatry Pub Date : 2025-01-23 DOI: 10.1136/jnnp-2024-335220
Julie Werenberg Dreier, Betina B Trabjerg, Kasper Lolk, Oleguer Plana-Ripoll, Jakob Christensen
{"title":"Working years lost in people with epilepsy: a population-based cohort study.","authors":"Julie Werenberg Dreier, Betina B Trabjerg, Kasper Lolk, Oleguer Plana-Ripoll, Jakob Christensen","doi":"10.1136/jnnp-2024-335220","DOIUrl":"https://doi.org/10.1136/jnnp-2024-335220","url":null,"abstract":"<p><strong>Background: </strong>We quantify the loss of working years for people with epilepsy compared with the general population and consider variation by aetiology, psychiatric comorbidity, sex and age.</p><p><strong>Methods: </strong>This population-based cohort study included all individuals aged 18-65 years living in Denmark from 1995 to 2018. Using nationwide registers since 1977, we identified people with epilepsy and obtained information on the main source of income or employment for each year during follow-up from 1995 to 2020. The main outcome was number of working years lost in people with epilepsy compared with the general population of same sex and age, capturing both working life lost due to permanent (death, disability pension, early retirement) and temporary (unemployment, sick leave) factors.</p><p><strong>Results: </strong>The study comprised 5 466 140 individuals, including 74 980 (1.4%) with epilepsy. In people with epilepsy, the number of working years was on average reduced by 6.6 (95% CI: 6.5 to 6.7) years compared with the general population, largely due to disability pension (4.8 years, 95% CI: 4.7 to 4.9) and premature death (1.6 years, 95% CI: 1.6 to 1.7). Loss of working life was more pronounced in those with a presumed underlying aetiology (9.0 years (95% CI: 8.9 to 9.2) vs 5.4 years (95% CI: 5.2 to 5.5) in those with unknown aetiology), those with psychiatric comorbidity (14.5 years (95% CI: 14.2 to 14.7) vs 5.6 years (95% CI: 5.5 to 5.7) in those without), men (7.2 years (95% CI: 7.1 to 7.3) vs 5.9 (95% CI: 5.8 to 6.0) years in women) and people with early onset of epilepsy (eg, 11.5 years (95% CI: 11.3 to 11.7) among those with onset <20 years).</p><p><strong>Conclusions: </strong>Epilepsy was associated with significant loss of working life resulting from both disability and premature death.</p>","PeriodicalId":16418,"journal":{"name":"Journal of Neurology, Neurosurgery, and Psychiatry","volume":" ","pages":""},"PeriodicalIF":8.7,"publicationDate":"2025-01-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143029090","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"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
Worldwide epidemiology of paediatric multiple sclerosis: data from the Multiple Sclerosis International Federation Atlas of MS, third edition. 小儿多发性硬化症的全球流行病学:数据来自多发性硬化症国际联盟图集,第三版。
IF 8.7 1区 医学
Journal of Neurology, Neurosurgery, and Psychiatry Pub Date : 2025-01-23 DOI: 10.1136/jnnp-2024-335175
Grace Gombolay, Laura Johnson, Rachel King, Madeleine Hebert, Brenda Banwell, Tanuja Chitnis, Anne Helme
{"title":"Worldwide epidemiology of paediatric multiple sclerosis: data from the Multiple Sclerosis International Federation Atlas of MS, third edition.","authors":"Grace Gombolay, Laura Johnson, Rachel King, Madeleine Hebert, Brenda Banwell, Tanuja Chitnis, Anne Helme","doi":"10.1136/jnnp-2024-335175","DOIUrl":"https://doi.org/10.1136/jnnp-2024-335175","url":null,"abstract":"<p><strong>Background: </strong>Limited data are available on the global rates of paediatric multiple sclerosis. Here, we report on the estimated worldwide prevalence of paediatric MS.</p><p><strong>Methods: </strong>We included paediatric prevalence data in 2020-2022 (Multiple Sclerosis International Federation Atlas of MS) and the prevalence of child neurologists (International Child Neurology Association). Data were split into prevalence bands per 100 000. Countries were classified by the WHO Region and World Bank Income. Descriptive analyses were performed. An estimated worldwide prevalence rate was calculated from the 2020-2022 paediatric prevalence data, which was adjusted to reduce outliers' impact and to reflect worldwide income distribution. The Atlas of MS data was obtained via survey of coordinators from the countries who use different tracking methods including national registries vs crude estimates.</p><p><strong>Results: </strong>Paediatric data were available in 24% (53/219) countries (38 higher and 15 lower income) with 31 420 total paediatric MS cases. In 2022, 67% (10/15) of lower income countries reported prevalence bands of '<1.0' compared with 34% (13/38) of higher income countries. Only 7% (1/15) of lower income countries reported prevalence bands '≥3.1'compared with 34% (13/38) of higher income countries. The rates of child neurologists positively correlated with the prevalence band. In 2020-2022, the estimated global prevalence (crude) was 2.53/100 000 (95% CI 2.51 to 2.56), with an adjusted prevalence rate of 1.48/100 000 (95% CI 1.45 to 1.51).</p><p><strong>Conclusions: </strong>Access to epidemiology data from resource-limited countries is challenging including surveillance for case ascertainment. Increased resources and standard methodologies will facilitate the understanding of rare disease epidemiology.</p>","PeriodicalId":16418,"journal":{"name":"Journal of Neurology, Neurosurgery, and Psychiatry","volume":" ","pages":""},"PeriodicalIF":8.7,"publicationDate":"2025-01-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143029091","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"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
Bridging thrombolysis with tenecteplase versus endovascular thrombectomy alone for large-vessel anterior circulation stroke: a target trial emulation analysis. 用tenecteplase桥接溶栓与单独血管内取栓治疗大血管前循环卒中:目标试验模拟分析。
IF 8.7 1区 医学
Journal of Neurology, Neurosurgery, and Psychiatry Pub Date : 2025-01-22 DOI: 10.1136/jnnp-2024-335325
Valerian L Altersberger, Johannes Kaesmacher, Leonid Churilov, Vignan Yogendrakumar, Jan Gralla, Daniel Strbian, David J Seiffge, Peter J Mitchell, Timothy J Kleinig, Bruce Cv Campbell, Urs Fischer
{"title":"Bridging thrombolysis with tenecteplase versus endovascular thrombectomy alone for large-vessel anterior circulation stroke: a target trial emulation analysis.","authors":"Valerian L Altersberger, Johannes Kaesmacher, Leonid Churilov, Vignan Yogendrakumar, Jan Gralla, Daniel Strbian, David J Seiffge, Peter J Mitchell, Timothy J Kleinig, Bruce Cv Campbell, Urs Fischer","doi":"10.1136/jnnp-2024-335325","DOIUrl":"https://doi.org/10.1136/jnnp-2024-335325","url":null,"abstract":"<p><strong>Background: </strong>Whether bridging thrombolysis with tenecteplase is beneficial compared with thrombectomy alone in patients who had a stroke with large-vessel occlusion remains unclear.</p><p><strong>Methods: </strong>This is a causal inference study of observational data from the trials SWIFT DIRECT and EXTEND-IA TNK Parts 1 and 2 applying target trial emulation. We compared patients receiving thrombectomy alone to patients receiving tenecteplase 0.25 mg/kg or 0.40 mg/kg before thrombectomy. The primary outcome was functional independence (modified Rankin Scale (mRS) of 0-2) at 90 days. Secondary outcomes included improvement over the full ordinal mRS scale, freedom of disability (mRS 0-1), mortality and occurrence of symptomatic intracranial haemorrhage. The average causal treatment effect was estimated via inverse probability of treatment weighting and G-Computation. We calculated standardised risk differences (SRDs) and adjusted (common) ORs (a(c)ORs).</p><p><strong>Results: </strong>Of 377 patients included in the target trial, 187 received thrombectomy alone and 190 tenecteplase before thrombectomy. Tenecteplase before thrombectomy did not increase the probability of patients achieving functional independence (SRD 0.04 (95% CI -0.06 to 0.13)) but resulted in a significant improvement in the mRS overall (acOR 1.56 (95% CI 1.07 to 2.23)) and in a higher probability of freedom from disability (SRD 0.10 (95% CI 0.01 to 0.20)). The probability for improvement of functional outcomes was further increased in patients treated within 140 min after onset (ordinal mRS acOR 1.63 (95% CI 1.04 to 2.56)). No significant differences in safety outcomes were observed between the two groups.</p><p><strong>Conclusion: </strong>Tenecteplase before thrombectomy compared with thrombectomy alone did not increase the probability of functional independence but resulted in significant improvement over the full mRS scale. This improvement was most evident in patients treated early.</p>","PeriodicalId":16418,"journal":{"name":"Journal of Neurology, Neurosurgery, and Psychiatry","volume":" ","pages":""},"PeriodicalIF":8.7,"publicationDate":"2025-01-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143029085","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"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
Autoimmune encephalitis: recovery, residual symptoms and predictors of long-term sequelae. 自身免疫性脑炎:恢复、残留症状和长期后遗症的预测因素
IF 8.7 1区 医学
Journal of Neurology, Neurosurgery, and Psychiatry Pub Date : 2025-01-19 DOI: 10.1136/jnnp-2024-334957
Smathorn Thakolwiboon, Michael Gilligan, Emma Orozco, Jeffrey W Britton, Divyanshu Dubey, Eoin P Flanagan, A Sebastian Lopez-Chiriboga, Kelsey Smith, Cristina Valencia-Sanchez, Nicholas L Zalewski, Anastasia Zekeridou, Sean J Pittock, Andrew McKeon
{"title":"Autoimmune encephalitis: recovery, residual symptoms and predictors of long-term sequelae.","authors":"Smathorn Thakolwiboon, Michael Gilligan, Emma Orozco, Jeffrey W Britton, Divyanshu Dubey, Eoin P Flanagan, A Sebastian Lopez-Chiriboga, Kelsey Smith, Cristina Valencia-Sanchez, Nicholas L Zalewski, Anastasia Zekeridou, Sean J Pittock, Andrew McKeon","doi":"10.1136/jnnp-2024-334957","DOIUrl":"https://doi.org/10.1136/jnnp-2024-334957","url":null,"abstract":"<p><strong>Background: </strong>Data regarding long-term recovery from autoimmune encephalitis (AE) remain limited.</p><p><strong>Methods: </strong>This retrospective observational study investigated outcomes in 182 patients who met the 2016 criteria for definite AE. Recovery data were available in 172 patients. Follow-up data at ≥24 months post-attack were available for 119. Recovery trajectory, residual symptoms, outcome predictors and causes of post-AE death were assessed.</p><p><strong>Results: </strong>Of 172 patients, 138 (80%) achieved good recovery (modified Rankin Scale (mRS) ≤2) with a median recovery time of 4 months (95% CI: 2 to 6 months). Recovery varied by associated neural antibody, with the best recovery observed in leucine-rich glioma-inactivated 1 (97% good recovery, median recovery time 0 (0 to 2) months). Paraneoplastic AE (p=0.007), severe attacks (eg, mRS ≥4 at attack, p=0.007) and cerebrospinal fluid pleocytosis (p=0.005) were associated with a lower likelihood of good recovery, while seizure presentation (p=0.026) was associated with better recovery. Despite good recovery, several residual symptoms persisted ≥24 months post-AE, including cognitive deficits (53%), seizures (26%), depression (23%), sleep disorders (25%), brainstem/cerebellar symptoms (13%), other movement disorders (14%) and autonomic symptoms (12%). Predictors of long-term sequelae included disabling cognitive deficit at onset and delayed immunotherapy for post AE-dementia, and medial temporal atrophy as well as escalation to cyclophosphamide therapy for both drug-resistant epilepsy and chronic depression. Of 182 patients, 20 (11%) died; the most common cause of death was progression of AE (6/20 (30%)).</p><p><strong>Conclusion: </strong>While the majority of patients achieved functional independence after AE, several residual symptoms persisted. Several clinical and paraclinical features were associated with long-term sequelae.</p>","PeriodicalId":16418,"journal":{"name":"Journal of Neurology, Neurosurgery, and Psychiatry","volume":" ","pages":""},"PeriodicalIF":8.7,"publicationDate":"2025-01-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143006776","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"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
Directional deep brain stimulation electrodes in Parkinson's disease: meta-analysis and systematic review of the literature. 帕金森病的定向脑深部刺激电极:荟萃分析和系统性文献综述。
IF 8.7 1区 医学
Journal of Neurology, Neurosurgery, and Psychiatry Pub Date : 2025-01-16 DOI: 10.1136/jnnp-2024-333947
Victor Hvingelby, Fareha Khalil, Flavia Massey, Alexander Hoyningen, San San Xu, Joseph Candelario-McKeown, Harith Akram, Thomas Foltynie, Patricia Limousin, Ludvic Zrinzo, Marie T Krüger
{"title":"Directional deep brain stimulation electrodes in Parkinson's disease: meta-analysis and systematic review of the literature.","authors":"Victor Hvingelby, Fareha Khalil, Flavia Massey, Alexander Hoyningen, San San Xu, Joseph Candelario-McKeown, Harith Akram, Thomas Foltynie, Patricia Limousin, Ludvic Zrinzo, Marie T Krüger","doi":"10.1136/jnnp-2024-333947","DOIUrl":"10.1136/jnnp-2024-333947","url":null,"abstract":"<p><strong>Background: </strong>Since their introduction in 2015, directional leads have practically replaced conventional leads for deep brain stimulation (DBS) in Parkinson's disease (PD). Yet, the benefits of directional DBS (dDBS) over omnidirectional DBS (oDBS) remain unclear. This meta-analysis and systematic review compares the literature on dDBS and oDBS for PD.</p><p><strong>Methods: </strong>Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines were followed. Database searches included Pubmed, Cochrane (CENTRAL) and EmBase, using relevant keywords such as 'directional', 'segmented', 'brain stimulation' and 'neuromodulation'. The screening was based on the title and abstract.</p><p><strong>Results: </strong>23 papers reporting on 1273 participants (1542 leads) were included. The therapeutic window was 0.70 mA wider when using dDBS (95% CI 0.13 to 1.26 mA, p=0.02), with a lower therapeutic current (0.41 mA, 95% CI 0.27 to 0.54 mA, p=0.01) and a higher side-effect threshold (0.56 mA, 95% CI 0.38 to 0.73 mA, p<0.01). However, there was no relevant difference in mean Unified Parkinson's Disease Rating Scale III change after dDBS (45.8%, 95% CI 30.7% to 60.9%) compared with oDBS (39.0%, 95% CI 36.9% to 41.2%, p=0.39), in the medication-OFF state. Median follow-up time for dDBS and oDBS studies was 6 months and 3 months, respectively (range 3-12 for both). The use of directionality often improves dyskinesia, dysarthria, dysesthesia and pyramidal side effects. Directionality was used in 55% of directional leads at 3-6 months, remaining stable over time (56% at a mean of 14.1 months).</p><p><strong>Conclusions: </strong>These findings suggest that stimulation parameters favour dDBS. However, these do not appear to have a significant impact on motor scores, and the availability of long-term data is limited. dDBS is widely accepted, but clinical data justifying its increased complexity and cost are currently sparse.</p><p><strong>Prospero registration number: </strong>CRD42023438056.</p>","PeriodicalId":16418,"journal":{"name":"Journal of Neurology, Neurosurgery, and Psychiatry","volume":" ","pages":"188-198"},"PeriodicalIF":8.7,"publicationDate":"2025-01-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142289434","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"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
Association between heavy alcohol consumption and cryptogenic ischaemic stroke in young adults: a case-control study. 年轻人大量饮酒与隐源性缺血性中风之间的关系:一项病例对照研究。
IF 8.7 1区 医学
Journal of Neurology, Neurosurgery, and Psychiatry Pub Date : 2025-01-16 DOI: 10.1136/jnnp-2024-333759
Nicolas Martinez-Majander, Shakar Kutal, Pauli Ylikotila, Nilufer Yesilot, Lauri Tulkki, Marialuisa Zedde, Tomi Sarkanen, Ulla Junttola, Annika Nordanstig, Annette Fromm, Kristina Ryliskiene, Radim Licenik, Phillip Ferdinand, Dalius Jatuzis, Liisa Kõrv, Janika Kõrv, Alessandro Pezzini, Suvi Tuohinen, Juha Sinisalo, Mika Lehto, Eva Gerdts, Essi Ryödi, Jaana Autere, Marja Hedman, Ana Catarina Fonseca, Ulrike Waje-Andreassen, Bettina von Sarnowski, Petra Redfors, Tiina Sairanen, Turgut Tatlisumak, Risto O Roine, Juha Huhtakangas, Heikki Numminen, Pekka Jäkälä, Jukka Putaala
{"title":"Association between heavy alcohol consumption and cryptogenic ischaemic stroke in young adults: a case-control study.","authors":"Nicolas Martinez-Majander, Shakar Kutal, Pauli Ylikotila, Nilufer Yesilot, Lauri Tulkki, Marialuisa Zedde, Tomi Sarkanen, Ulla Junttola, Annika Nordanstig, Annette Fromm, Kristina Ryliskiene, Radim Licenik, Phillip Ferdinand, Dalius Jatuzis, Liisa Kõrv, Janika Kõrv, Alessandro Pezzini, Suvi Tuohinen, Juha Sinisalo, Mika Lehto, Eva Gerdts, Essi Ryödi, Jaana Autere, Marja Hedman, Ana Catarina Fonseca, Ulrike Waje-Andreassen, Bettina von Sarnowski, Petra Redfors, Tiina Sairanen, Turgut Tatlisumak, Risto O Roine, Juha Huhtakangas, Heikki Numminen, Pekka Jäkälä, Jukka Putaala","doi":"10.1136/jnnp-2024-333759","DOIUrl":"10.1136/jnnp-2024-333759","url":null,"abstract":"<p><strong>Background: </strong>The underlying risk factors for young-onset cryptogenic ischaemic stroke (CIS) remain unclear. This multicentre study aimed to explore the association between heavy alcohol consumption and CIS with subgroup analyses stratified by sex and age.</p><p><strong>Methods: </strong>Altogether, 540 patients aged 18-49 years (median age 41; 47.2% women) with a recent CIS and 540 sex-matched and age-matched stroke-free controls were included. Heavy alcohol consumption was defined as >7 (women) and >14 (men) units per week or at least an average of two times per month ≥5 (women) and ≥7 (men) units per instance (binge drinking). A conditional logistic regression adjusting for age, sex, education, hypertension, cardiovascular diseases, diabetes, hypercholesterolaemia, current smoking, obesity, diet and physical inactivity was used to assess the independent association between alcohol consumption and CIS.</p><p><strong>Results: </strong>Patients were twice as more often heavy alcohol users compared with controls (13.7% vs 6.7%, p<0.001), were more likely to have hypertension and they were more often current smokers, overweight and physically inactive. In the entire study population, heavy alcohol consumption was independently associated with CIS (adjusted OR 2.11; 95% CI 1.22 to 3.63). In sex-specific analysis, heavy alcohol consumption was associated with CIS in men (2.72; 95% CI 1.25 to 5.92), but not in women (1.56; 95% CI 0.71 to 3.41). When exploring the association with binge drinking alone, a significant association was shown in the entire cohort (2.43; 95% CI 1.31 to 4.53) and in men (3.36; 95% CI 1.44 to 7.84), but not in women.</p><p><strong>Conclusions: </strong>Heavy alcohol consumption, particularly binge drinking, appears to be an independent risk factor in young men with CIS.</p>","PeriodicalId":16418,"journal":{"name":"Journal of Neurology, Neurosurgery, and Psychiatry","volume":" ","pages":"114-121"},"PeriodicalIF":8.7,"publicationDate":"2025-01-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11877115/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141436985","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"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
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