Lancet Neurology最新文献

{"title":"Angela Vincent: a pioneer of immune-mediated disease.","authors":"Jules Morgan","doi":"10.1016/S1474-4422(24)00125-X","DOIUrl":"10.1016/S1474-4422(24)00125-X","url":null,"abstract":"","PeriodicalId":17989,"journal":{"name":"Lancet Neurology","volume":" ","pages":"671"},"PeriodicalIF":48.0,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140194034","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}

{"title":"Multiple sclerosis diagnosis: a biologically driven perspective.","authors":"Massimo Filippi, Monica Margoni, Maria A Rocca","doi":"10.1016/S1474-4422(24)00207-2","DOIUrl":"10.1016/S1474-4422(24)00207-2","url":null,"abstract":"","PeriodicalId":17989,"journal":{"name":"Lancet Neurology","volume":"23 7","pages":"668-669"},"PeriodicalIF":46.5,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141321134","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}

{"title":"Safety and efficacy of stent retrievers plus contact aspiration in patients with acute ischaemic anterior circulation stroke and positive susceptibility vessel sign in France (VECTOR): a randomised, single-blind trial.","authors":"Romain Bourcier, Gaultier Marnat, Cyril Dargazanli, François Zhu, Arturo Consoli, Eimad Shotar, Kevin Premat, François Eugene, Kevin Janot, Vincent L'Allinec, Julien Ognard, Jean-Philippe Desilles, Raphael Blanc, Jean-Christophe Gentric, Frédéric Bourdain, Julien Labreuche, Liang Liao, Frédéric Clarençon, Xavier Barreau, Héloïse Ifergan, Jean-François Hak, Basile Kerleroux, Raoul Pop, Sébastien Soize, Nicolas Bricout, Jildaz Caroff, Johann Sebastian Richter, Hubert Desal, Bertrand Lapergue, Aymeric Rouchaud","doi":"10.1016/S1474-4422(24)00165-0","DOIUrl":"10.1016/S1474-4422(24)00165-0","url":null,"abstract":"<p><strong>Background: </strong>Positive susceptibility vessel sign (SVS) in patients with acute ischaemic stroke has been associated with friable red blood cell-rich clots and more effective recanalisation using stent retrievers versus contact aspiration. We compared the safety and efficacy of stent retrievers plus contact aspiration (combined technique) versus contact aspiration alone as the first-line thrombectomy technique in patients with acute ischaemic anterior circulation stroke and SVS-positive occlusions.</p><p><strong>Methods: </strong>Adaptive Endovascular Strategy to the Clot MRI in Large Intracranial Vessel Occlusion (VECTOR) was a prospective, randomised, open-label study with blinded evaluation. Patients with SVS-positive anterior circulation occlusions on pretreatment MRI and arterial puncture within 24 h of symptom onset were enrolled from 22 centres in France. A centralised web-based method was used by interventional neuroradiologists for dynamic randomisation by minimisation. Patients were randomly assigned 1:1 to the combined technique or contact aspiration alone. The primary outcome was expanded Thrombolysis in Cerebral Infarction (eTICI) grade 2c or 3 reperfusion after three or fewer passes on post-treatment angiogram, adjudicated by a blinded independent central imaging core laboratory. The intention-to-treat population was used to assess the primary and secondary outcomes. This trial is registered with ClinicalTrials.gov (NCT04139486) and is complete.</p><p><strong>Findings: </strong>Between Nov 26, 2019, and Feb 14, 2022, 526 patients were enrolled, of whom 521 constituted the intention-to-treat population (combined technique, n=263; contact aspiration alone, n=258). The median age of participants was 74·9 years (IQR 64·4-83·3); 284 (55%) were female and 237 (45%) male. The primary outcome did not differ significantly between groups (152 [58%] of 263 patients for the combined technique vs 135 [52%] of 258 for contact aspiration; odds ratio [OR] 1·27; 95% CI 0·88-1·83; p=0·19). Procedure-related adverse events occurred in 32 (12%) of 263 patients in the combined technique group and 27 (11%) of 257 in the contact aspiration group (OR 1·14; 0·65-2·00; p=0·65). The most common adverse event was intracerebral haemorrhage (146 [56%] of 259 patients for the combined technique vs 123 [49%] of 251 for contact aspiration; OR 1·32; 0·91-1·90; p=0·13). All-cause mortality at 3 months occurred in 57 (23%) of 251 patients in the combined technique group and 48 (19%) of 247 in the contact aspiration group (OR 1·19; 0·76-1·86; p=0·45), none of which was treatment-related.</p><p><strong>Interpretation: </strong>The results of the VECTOR trial do not show superiority of the combined stent retriever plus contact aspiration technique over contact aspiration alone in patients with SVS-positive occlusion with respect to achieving eTICI 2c-3 within three passes. These findings support the use of either the combined technique or contact aspirat","PeriodicalId":17989,"journal":{"name":"Lancet Neurology","volume":"23 7","pages":"700-711"},"PeriodicalIF":46.5,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141321137","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}

{"title":"Safety and efficacy of arimoclomol in patients with early amyotrophic lateral sclerosis (ORARIALS-01): a randomised, double-blind, placebo-controlled, multicentre, phase 3 trial.","authors":"Michael Benatar, Thomas Hansen, Dror Rom, Marie A Geist, Thomas Blaettler, William Camu, Magdalena Kuzma-Kozakiewicz, Leonard H van den Berg, Raul Juntas Morales, Adriano Chio, Peter M Andersen, Pierre-Francois Pradat, Dale Lange, Philip Van Damme, Gabriele Mora, Mariusz Grudniak, Matthew Elliott, Susanne Petri, Nicholas Olney, Shafeeq Ladha, Namita A Goyal, Thomas Meyer, Michael G Hanna, Colin Quinn, Angela Genge, Lorne Zinman, Duaa Jabari, Christen Shoesmith, Albert C Ludolph, Christoph Neuwirth, Sharon Nations, Jeremy M Shefner, Martin R Turner, Joanne Wuu, Richard Bennett, Hoang Dang, Claus Sundgreen","doi":"10.1016/S1474-4422(24)00134-0","DOIUrl":"10.1016/S1474-4422(24)00134-0","url":null,"abstract":"<p><strong>Background: </strong>Amyotrophic lateral sclerosis is a progressive neurodegenerative disorder leading to muscle weakness and respiratory failure. Arimoclomol, a heat-shock protein-70 (HSP70) co-inducer, is neuroprotective in animal models of amyotrophic lateral sclerosis, with multiple mechanisms of action, including clearance of protein aggregates, a pathological hallmark of sporadic and familial amyotrophic lateral sclerosis. We aimed to evaluate the safety and efficacy of arimoclomol in patients with amyotrophic lateral sclerosis.</p><p><strong>Methods: </strong>ORARIALS-01 was a multinational, randomised, double-blind, placebo-controlled, parallel-group trial done at 29 centres in 12 countries in Europe and North America. Patients were eligible if they were aged 18 years or older and met El Escorial criteria for clinically possible, probable, probable laboratory-supported, definite, or familial amyotrophic lateral sclerosis; had an ALS Functional Rating Scale-Revised score of 35 or more; and had slow vital capacity at 70% or more of the value predicted on the basis of the participant's age, height, and sex. Patients were randomly assigned (2:1) in blocks of 6, stratified by use of a stable dose of riluzole or no riluzole use, to receive oral arimoclomol citrate 1200 mg/day (400 mg three times per day) or placebo. The Randomisation sequence was computer generated centrally. Investigators, study personnel, and study participants were masked to treatment allocation. The primary outcome was the Combined Assessment of Function and Survival (CAFS) rank score over 76 weeks of treatment. The primary outcome and safety were analysed in the modified intention-to-treat population. This trial is registered with ClinicalTrials.gov, NCT03491462, and is completed.</p><p><strong>Findings: </strong>Between July 31, 2018, and July 17, 2019, 287 patients were screened, 245 of whom were enrolled in the trial and randomly assigned. The modified intention-to-treat population comprised 239 patients (160 in the arimoclomol group and 79 in the placebo group): 151 (63%) were male and 88 (37%) were female; mean age was 57·6 years (SD 10·9). CAFS score over 76 weeks did not differ between groups (mean 0·51 [SD 0·29] in the arimoclomol group vs 0·49 [0·28] in the placebo group; p=0·62). Cliff's delta comparing the two groups was 0·039 (95% CI -0·116 to 0·194). Proportions of participants who died were similar between the treatment groups: 29 (18%) of 160 patients in the arimoclomol group and 18 (23%) of 79 patients in the placebo group. Most deaths were due to disease progression. The most common adverse events were gastrointestinal. Adverse events were more often deemed treatment-related in the arimoclomol group (104 [65%]) than in the placebo group (41 [52%]) and more often led to treatment discontinuation in the arimoclomol group (26 [16%]) than in the placebo group (four [5%]).</p><p><strong>Interpretation: </strong>Arimoclomol did not improve efficacy ou","PeriodicalId":17989,"journal":{"name":"Lancet Neurology","volume":" ","pages":"687-699"},"PeriodicalIF":46.5,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141088081","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}

{"title":"Implementation research: an approach to overcoming the know-do gap.","authors":"Lesli E Skolarus, Linda S Williams","doi":"10.1016/S1474-4422(24)00219-9","DOIUrl":"10.1016/S1474-4422(24)00219-9","url":null,"abstract":"","PeriodicalId":17989,"journal":{"name":"Lancet Neurology","volume":"23 7","pages":"656-658"},"PeriodicalIF":46.5,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141321131","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}

{"title":"Amyotrophic lateral sclerosis: a lesson in translation.","authors":"Orla Hardiman","doi":"10.1016/S1474-4422(24)00223-0","DOIUrl":"10.1016/S1474-4422(24)00223-0","url":null,"abstract":"","PeriodicalId":17989,"journal":{"name":"Lancet Neurology","volume":" ","pages":"651-653"},"PeriodicalIF":48.0,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141088079","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}

{"title":"Speculation on the transmissibility of Alzheimer's disease.","authors":"Alifiya Kapasi, Julie A Schneider","doi":"10.1016/S1474-4422(24)00145-5","DOIUrl":"10.1016/S1474-4422(24)00145-5","url":null,"abstract":"","PeriodicalId":17989,"journal":{"name":"Lancet Neurology","volume":"23 6","pages":"555-556"},"PeriodicalIF":46.5,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140957995","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}

{"title":"Climate change and disorders of the nervous system.","authors":"Sanjay M Sisodiya, Medine I Gulcebi, Francesco Fortunato, James D Mills, Ethan Haynes, Elvira Bramon, Paul Chadwick, Olga Ciccarelli, Anthony S David, Kris De Meyer, Nick C Fox, Joanna Davan Wetton, Martin Koltzenburg, Dimitri M Kullmann, Manju A Kurian, Hadi Manji, Mark A Maslin, Manjit Matharu, Hugh Montgomery, Marina Romanello, David J Werring, Lisa Zhang, Karl J Friston, Michael G Hanna","doi":"10.1016/S1474-4422(24)00087-5","DOIUrl":"10.1016/S1474-4422(24)00087-5","url":null,"abstract":"<p><p>Anthropogenic climate change is affecting people's health, including those with neurological and psychiatric diseases. Currently, making inferences about the effect of climate change on neurological and psychiatric diseases is challenging because of an overall sparsity of data, differing study methods, paucity of detail regarding disease subtypes, little consideration of the effect of individual and population genetics, and widely differing geographical locations with the potential for regional influences. However, evidence suggests that the incidence, prevalence, and severity of many nervous system conditions (eg, stroke, neurological infections, and some mental health disorders) can be affected by climate change. The data show broad and complex adverse effects, especially of temperature extremes to which people are unaccustomed and wide diurnal temperature fluctuations. Protective measures might be possible through local forecasting. Few studies project the future effects of climate change on brain health, hindering policy developments. Robust studies on the threats from changing climate for people who have, or are at risk of developing, disorders of the nervous system are urgently needed.</p>","PeriodicalId":17989,"journal":{"name":"Lancet Neurology","volume":"23 6","pages":"636-648"},"PeriodicalIF":46.5,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140957760","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}

{"title":"Elucidating a new path of CSF transport in the CNS.","authors":"Helene Benveniste, Jean-Leon Thomas","doi":"10.1016/S1474-4422(24)00164-9","DOIUrl":"10.1016/S1474-4422(24)00164-9","url":null,"abstract":"","PeriodicalId":17989,"journal":{"name":"Lancet Neurology","volume":"23 6","pages":"553-554"},"PeriodicalIF":46.5,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11700482/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140957945","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}

{"title":"RAB32 Ser71Arg in autosomal dominant Parkinson's disease: linkage, association, and functional analyses.","authors":"Emil K Gustavsson, Jordan Follett, Joanne Trinh, Sandeep K Barodia, Raquel Real, Zhiyong Liu, Melissa Grant-Peters, Jesse D Fox, Silke Appel-Cresswell, A Jon Stoessl, Alex Rajput, Ali H Rajput, Roland Auer, Russel Tilney, Marc Sturm, Tobias B Haack, Suzanne Lesage, Christelle Tesson, Alexis Brice, Carles Vilariño-Güell, Mina Ryten, Matthew S Goldberg, Andrew B West, Michele T Hu, Huw R Morris, Manu Sharma, Ziv Gan-Or, Bedia Samanci, Pawel Lis, Maria Teresa Periñan, Rim Amouri, Samia Ben Sassi, Faycel Hentati, Francesca Tonelli, Dario R Alessi, Matthew J Farrer","doi":"10.1016/S1474-4422(24)00121-2","DOIUrl":"10.1016/S1474-4422(24)00121-2","url":null,"abstract":"&lt;p&gt;&lt;strong&gt;Background: &lt;/strong&gt;Parkinson's disease is a progressive neurodegenerative disorder with multifactorial causes, among which genetic risk factors play a part. The RAB GTPases are regulators and substrates of LRRK2, and variants in the LRRK2 gene are important risk factors for Parkinson's disease. We aimed to explore genetic variability in RAB GTPases within cases of familial Parkinson's disease.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Methods: &lt;/strong&gt;We did whole-exome sequencing in probands from families in Canada and Tunisia with Parkinson's disease without a genetic cause, who were recruited from the Centre for Applied Neurogenetics (Vancouver, BC, Canada), an international consortium that includes people with Parkinson's disease from 36 sites in 24 countries. 61 RAB GTPases were genetically screened, and candidate variants were genotyped in relatives of the probands to assess disease segregation by linkage analysis. Genotyping was also done to assess variant frequencies in individuals with idiopathic Parkinson's disease and controls, matched for age and sex, who were also from the Centre for Applied Neurogenetics but unrelated to the probands or each other. All participants were aged 18 years or older. The sequencing and genotyping findings were validated by case-control association analyses using bioinformatic data obtained from publicly available clinicogenomic databases (AMP-PD, GP2, and 100 000 Genomes Project) and a private German clinical diagnostic database (University of Tübingen). Clinical and pathological findings were summarised and haplotypes were determined. In-vitro studies were done to investigate protein interactions and enzyme activities.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Findings: &lt;/strong&gt;Between June 1, 2010, and May 31, 2017, 130 probands from Canada and Tunisia (47 [36%] female and 83 [64%] male; mean age 72·7 years [SD 11·7; range 38-96]; 109 White European ancestry, 18 north African, two east Asian, and one Hispanic] underwent whole-exome sequencing. 15 variants in RAB GTPase genes were identified, of which the RAB32 variant c.213C&gt;G (Ser71Arg) cosegregated with autosomal dominant Parkinson's disease in three families (nine affected individuals; non-parametric linkage Z score=1·95; p=0·03). 2604 unrelated individuals with Parkinson's disease and 344 matched controls were additionally genotyped, and five more people originating from five countries (Canada, Italy, Poland, Turkey, and Tunisia) were identified with the RAB32 variant. From the database searches, in which 6043 individuals with Parkinson's disease and 62 549 controls were included, another eight individuals were identified with the RAB32 variant from four countries (Canada, Germany, UK, and USA). Overall, the association of RAB32 c.213C&gt;G (Ser71Arg) with Parkinson's disease was significant (odds ratio [OR] 13·17, 95% CI 2·15-87·23; p=0·0055; I&lt;sup&gt;2&lt;/sup&gt;=99·96%). In the people who had the variant, Parkinson's disease presented at age 54·6 years (SD 12·75, range 31-81, n=16), and two-thirds ","PeriodicalId":17989,"journal":{"name":"Lancet Neurology","volume":" ","pages":"603-614"},"PeriodicalIF":46.5,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11096864/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140868002","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}