JAMA neurology最新文献

{"title":"Menopause Impact on Multiple Sclerosis Disability Progression.","authors":"Francesca Bridge,Paul G Sanfilippo,Chao Zhu,Olga Skibina,Ai-Lan Nguyen,Tomas Kalincik,Katherine Buzzard,Bruce V Taylor,Jennifer MacIntyre,Lesley-Ann Hall,Mark Slee,Richard Macdonell,Vicki Maltby,Jeannette Lechner-Scott,Pamela McCombe,Helmut Butzkueven,Anneke van der Walt,Vilija G Jokubaitis","doi":"10.1001/jamaneurol.2025.3538","DOIUrl":"https://doi.org/10.1001/jamaneurol.2025.3538","url":null,"abstract":"ImportanceMost women with multiple sclerosis (MS) will experience menopause while living with MS. Despite this, the impact of menopause on MS disease trajectory remains unclear.ObjectiveTo assess whether menopause modifies the risk of disability progression for women with relapse-onset MS.Design, Setting, and ParticipantsThis retrospective cohort study used prospective clinical data that were collected within the MSBase Registry. Data were extracted from MSBase on July 1, 2023. These data were analyzed from January 2023 through February 2025. Female participants were recruited from 8 Australian neuroimmunology specialist centers (1 private practice and 7 tertiary referral centers) from 2018 through 2021. Participants included 1468 women aged 18 years or older who completed dedicated retrospective women's health surveys. Of these, 987 women with relapse-onset MS, 3 or more Expanded Disability Status Scale (EDSS) measurements recorded, and reported menopausal status were included in the primary analysis. The secondary analysis included 209 women with 1 or more EDSS measurements recorded in the MSBase database predate and postdate of menopause onset.ExposureCrude and adjusted Cox proportional hazards models were used to assess the impact of menopause, modeled as a time-varying covariate, on progressive disability milestones. Analyses were adjusted for age at MS onset, baseline disease duration, baseline EDSS score, baseline relapse, and exposure to high-efficacy disease-modifying therapy modeled as a time-varying covariate.Main Outcome MeasuresIn the primary analysis, the main outcome was time to 6-month confirmed disability progression (CDP). The secondary outcome was time to secondary progressive MS (SPMS). The secondary inflection point analysis examined longitudinal changes in EDSS in women who were followed up with throughout their menopausal transition.ResultsPrimary analysis included 583 premenopausal and 404 postmenopausal women with MS. The median age at menopause was 48.5 years. Following multivariable adjustment, menopause was not associated with an increased risk of CDP or SPMS (hazard ratio, 0.95; 95% CI, 0.70-1.29; P = .70 and hazard ratio, 1.00; 95% CI, 0.60-1.67; P = 1.00), respectively. In the secondary analysis, menopause did not represent an inflection point in EDSS worsening following multivariable adjustment.Conclusions and RelevanceWhile reproductive aging may be additive to the effects of somatic aging, these results do not support menopause as the leading factor for disability progression in older women with MS.","PeriodicalId":14677,"journal":{"name":"JAMA neurology","volume":"99 1","pages":""},"PeriodicalIF":29.0,"publicationDate":"2025-09-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145182590","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":"Rethinking the Menopause Transition and Multiple Sclerosis Progression.","authors":"Jennifer S Graves,Mar Tintoré","doi":"10.1001/jamaneurol.2025.2419","DOIUrl":"https://doi.org/10.1001/jamaneurol.2025.2419","url":null,"abstract":"","PeriodicalId":14677,"journal":{"name":"JAMA neurology","volume":"96 1","pages":""},"PeriodicalIF":29.0,"publicationDate":"2025-09-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145182623","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":"Concurrent FHL1-Related Myopathy and CMT1A Unveiled by Persistent Neuropathic Feature.","authors":"Xiaoqing Lv,Guiguan Yang,Pengfei Lin","doi":"10.1001/jamaneurol.2025.3433","DOIUrl":"https://doi.org/10.1001/jamaneurol.2025.3433","url":null,"abstract":"","PeriodicalId":14677,"journal":{"name":"JAMA neurology","volume":"66 1","pages":""},"PeriodicalIF":29.0,"publicationDate":"2025-09-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145182591","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":"More Than a Quick Look.","authors":"Jo-Hsuan Wu","doi":"10.1001/jamaneurol.2025.3408","DOIUrl":"https://doi.org/10.1001/jamaneurol.2025.3408","url":null,"abstract":"","PeriodicalId":14677,"journal":{"name":"JAMA neurology","volume":" ","pages":""},"PeriodicalIF":21.3,"publicationDate":"2025-09-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145112962","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":"Long-Term Personalized Adaptive Deep Brain Stimulation in Parkinson Disease: A Nonrandomized Clinical Trial.","authors":"Helen M Bronte-Stewart,Martijn Beudel,Jill L Ostrem,Simon Little,Leonardo Almeida,Adolfo Ramirez-Zamora,Alfonso Fasano,Travis Hassell,Kyle T Mitchell,Elena Moro,Michal Gostkowski,Gaurav Chattree,Rob M A de Bie,Martijn de Neeling,Dan Piña-Fuentes,Bart Swinnen,Philip A Starr,Lauren H Hammer,Kelly D Foote,R Mark Richardson,Alice Flaherty,Alexandra Boogers,Qais Sa'di,Sara Meoni,Anna Castrioto,Scott Stanslaski,Rebekah L S Summers,Lisa Tonder,Ye Tan,Helen Berrier,Tim J Goble,Robert S Raike,Todd M Herrington, ","doi":"10.1001/jamaneurol.2025.2781","DOIUrl":"https://doi.org/10.1001/jamaneurol.2025.2781","url":null,"abstract":"ImportanceAdaptive deep brain stimulation (aDBS) automatically adjusts stimulation amplitude in response to changes in relevant neural activity in people with Parkinson disease (PD). Whether long-term at-home aDBS is safe and delivers effective therapy in people with PD remains unknown.ObjectiveTo determine the tolerability, efficacy, and safety of long-term aDBS in people with PD who were previously stable receiving continuous DBS (cDBS).Design, Setting, and ParticipantsThis international, open-label, prospective, pivotal trial enrolled participants from December 2020 to July 2022 in the US, Canada, and Europe. Referred participants with PD were first assessed while receiving stable cDBS and those who tolerated 2 aDBS modes were randomized and blinded to 30 days in each mode (single-blind crossover design); those who tolerated only 1 mode were assessed in that mode only; assessments completed holding medication stable. Participants were given the option to continue their selected mode of aDBS for long-term follow-up (10 months). Data used for analysis were from March 2024. Multiple imputation was used if more than 5% of data was missing for the primary or secondary end points. A referred sample of 68 participants with PD, stable while receiving cDBS and medication, was included.InterventionsTwo modes of aDBS controlled by an embedded closed-loop stimulation system: single threshold (ST-aDBS) and dual threshold (DT-aDBS).Main Outcomes and MeasuresThe primary end point required that at least 50% of participants meet a performance goal of on-time (ie, time when symptoms were well controlled) without troublesome dyskinesias with no less than 1-SD reduction (and post hoc threshold less than 2 hours per day reduction) reported during aDBS therapy compared to cDBS, determined from a self-reported motor diary. The secondary end point was total electrical energy delivered (TEED) compared between aDBS and cDBS. Safety assessments were conducted by characterizing adverse events (AEs), stimulation-related AEs, serious AEs, and device deficiencies.ResultsA total of 68 participants enrolled (mean [SD] age, 62.2 [8.4] years; 48 [70.6%] male); 40 and 35 were evaluated with DT-aDBS and ST-aDBS, respectively. The primary end point performance goal was met in the DT-aDBS group (91% of participants) and ST-aDBS (79% of participants) with the post hoc performance threshold; no difference between aDBS modes (χ21 = 1.0; P = .51). TEED was reduced during ST-aDBS compared to cDBS (mean change, -15%; nominal P = .01) and not different from DT-aDBS. All but 1 stimulation-related AE resolved during the aDBS setup and adjustment phase with no serious device AEs through long-term follow-up. Exploratory analyses suggested improvement in on-time without troublesome dyskinesias with DT-aDBS compared to cDBS.Conclusions and RelevanceIn this study, long-term aDBS was tolerable, effective, and safe in people with PD who were previously stable while receiving cDBS.Trial Registratio","PeriodicalId":14677,"journal":{"name":"JAMA neurology","volume":"17 1","pages":""},"PeriodicalIF":29.0,"publicationDate":"2025-09-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145103423","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":"Self-Diagnosed REM Sleep Behavior Disorder Using a Consumer Device.","authors":"Salonee Marwaha,Carlos H Schenck,Emmanuel H During","doi":"10.1001/jamaneurol.2025.3400","DOIUrl":"https://doi.org/10.1001/jamaneurol.2025.3400","url":null,"abstract":"","PeriodicalId":14677,"journal":{"name":"JAMA neurology","volume":"38 1","pages":""},"PeriodicalIF":29.0,"publicationDate":"2025-09-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145103424","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":"Revised Diagnostic Criteria for Vascular Cognitive Impairment and Dementia-The VasCog-2-WSO Criteria.","authors":" ,Perminder S Sachdev,Adam C Bentvelzen,Nicole A Kochan,Jiyang Jiang,Satoshi Hosoki,Rebecca Koncz,Russell J Chander,Danit Saks,Hugo P Aben,Daisy Acosta,Pia Andersen,Frédéric Assal,Hee-Joon Bae,Geert Jan Biessels,Deborah Blacker,Régis Bordet,Emily M Briceno,Henry Brodaty,Amy Brodtmann,Paulo Caramelli,Erico Castro-Costa,Hugues Chabriat,Christopher Chen,Una Clancy,Lucette Cysique,Charles DeCarli,Ding Ding,Marco Duering,Eliasz Engelhardt,Serge Gauthier,Fatemeh Geranmayeh,Olivier Godefroy,Philip Gorelick,Steven M Greenberg,Vesna Jelic,Hanna Jokinen,Raj N Kalaria,Murali Krishna,Kurt Lancaster,Frank-Erik de Leeuw,Jae-Sung Lim,Anna Marseglia,Javier Marta-Moreno,John T O'Brien,Leonardo Pantoni,Matthew P Pase,Sarah T Pendlebury,Gary Rosenberg,Behnam Sabayan,Emilia Salvadori,Katherine Samaras,Ivy Anne Sebastian,Sudha Seshadri,Eric E Smith,Velandai Srikanth,Kathryn Stokes,Felipe Kenji Sudo,Lukas Sveikata,Michael Valenzuela,Anders Wallin,Joanna M Wardlaw,Qun Xu","doi":"10.1001/jamaneurol.2025.3242","DOIUrl":"https://doi.org/10.1001/jamaneurol.2025.3242","url":null,"abstract":"ImportanceSeveral sets of diagnostic criteria have been proposed for vascular cognitive impairment and dementia (VCID). The International Society for Vascular Behavioural and Cognitive Disorders (VasCog) working group published comprehensive operationalized criteria in 2014. Considering subsequent advances in the field, a revision was needed.ObjectiveTo update the VasCog criteria to achieve consensus on diagnosis of VCID.Design, Setting, and ParticipantsVasCog criteria and other published diagnostic guidelines, aided by literature review of recent developments in VCID, were used as reference points for an online Delphi survey (minimum 3 rounds, ≥75% threshold for agreement), including operationalization of criteria and guidance on potential biomarkers. Seventy international experts from diverse international regions were invited to participate in 2023.ResultsThree survey rounds included 49 to 54 participants that agreed on VasCog-2 diagnostic criteria for preclinical, mild, and major dementia levels of vascular cognitive impairment (under the overarching term VCID). Research guidelines, including the use of novel neuroimaging and fluid biomarkers, were also agreed on. The World Stroke Organization (WSO) endorsed the criteria, hence named VasCog-2-WSO.Conclusions and RelevanceThe VasCog-2-WSO criteria update the VasCog criteria for the diagnosis of VCID, providing operationalization and additional guidance on potential neuroimaging and fluid biomarkers. VasCog-2-WSO should provide an international standard for VCID diagnosis, facilitating diagnostic consistency among clinicians and researchers.","PeriodicalId":14677,"journal":{"name":"JAMA neurology","volume":"19 1","pages":""},"PeriodicalIF":29.0,"publicationDate":"2025-09-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145068309","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":"Limitations of Categorical Diagnoses of CVD-Related Cognitive Impairment.","authors":"David S Knopman","doi":"10.1001/jamaneurol.2025.3234","DOIUrl":"https://doi.org/10.1001/jamaneurol.2025.3234","url":null,"abstract":"","PeriodicalId":14677,"journal":{"name":"JAMA neurology","volume":"72 1","pages":""},"PeriodicalIF":29.0,"publicationDate":"2025-09-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145068311","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":"Five-Year Outcomes from Deep Brain Stimulation of the Subthalamic Nucleus for Parkinson Disease.","authors":"Philip A Starr,Rajat S Shivacharan,Edward Goldberg,Alexander I Tröster,Paul A House,Monique L Giroux,Adam O Hebb,Donald M Whiting,Timothy A Leichliter,Jill L Ostrem,Leo Verhagen Metman,Sepehr Sani,Jessica A Karl,Mustafa S Siddiqui,Stephen B Tatter,Ihtsham Ul Haq,Andre G Machado,Michal Gostkowski,Michele Tagliati,Adam N Mamelak,Michael S Okun,Kelly D Foote,Guillermo Moguel-Cobos,Francisco A Ponce,Rajesh Pahwa,Kelly Lyons,Cathrin M Buetefisch,Robert E Gross,Corneliu C Luca,Jonathan R Jagid,Gonzalo J Revuelta,Istvan Takacs,Michael H Pourfar,Alon Y Mogilner,Andrew P Duker,George T Mandybur,Joshua M Rosenow,Cindy Zadikoff,Suketu M Khandhar,Mark Sedrak,Fenna T Phibbs,Joseph Neimat,Jennifer Durphy,Adolfo Ramirez-Zamora,Julie G Pilitsis,Ryan J Uitti,Robert Wharen,Michael C Park,Jerrold L Vitek, ","doi":"10.1001/jamaneurol.2025.3373","DOIUrl":"https://doi.org/10.1001/jamaneurol.2025.3373","url":null,"abstract":"ImportanceThe Implantable Neurostimulator for the Treatment of Parkinson's Disease (INTREPID) trial was a randomized, double-blind, sham-controlled study of subthalamic nucleus (STN) deep brain stimulation (DBS) for the treatment of Parkinson disease (PD).ObjectiveTo evaluate the long-term (5-year) outcomes and safety of STN-DBS for PD.Design, Setting, and ParticipantsThis was a prospective, randomized (3:1), 12-week double-blind sham-controlled study at 23 movement disorder centers across the US with an open-label 5-year follow-up. Patients were implanted and followed up with the Vercise DBS system from May 2013 to December 2022. Eligibility required diagnosis of bilateral idiopathic PD with more than 5 years of motor symptoms, more than 6 hours per day of poor motor function, modified Hoehn and Yahr Scale scores higher than 2, Unified Parkinson's Disease Rating Scale (UPDRS-III) score of 30 or higher (medication-off state), and 33% or higher improvement in UPDRS-III medication-on score.InterventionBilateral STN-DBS for moderate to advanced PD.Main Outcomes and MeasuresPrimary outcomes included changes in UPDRS and dyskinesia scores, quality-of-life measures, and safety assessments. Exploratory analyses included medication reduction and DBS association with motor signs.ResultsA total of 313 patients were enrolled with 191 receiving the DBS system, and 137 participants (72%) completed the study. The study population had a mean (SD) age of 60 (7.9) years, with 139 (73%) male participants. Motor function without medication as measured by UPDRS-III improved from a mean (SD) of 42.8 (9.4) to 21.1 (10.6) at year 1 (51%; 95% CI, 49%-53%; P < .001) and 27.6 (11.6) at year 5 (36%; 95% CI, 33%-38%; P < .001). Activities of daily living without medication as measured by UPDRS-III improved from a mean (SD) of 20.6 (6.0) to 12.4 (6.1) at year 1 (41%; 95% CI, 38%-42%; P < .001) and 16.4 (6.5) at year 5 (22%; 95% CI, 18%-23%; P < .001). Dyskinesia scores decreased from 4.0 (5.1) to 1.0 (2.1) at year 1 (75%; 95% CI, 73%-75%; P < .001) and to 1.2 (2.1) at year 5 (70%; 95% CI, 63%-75%; P < .001). The levodopa equivalent dose was reduced by 28% at year 1, remaining stable at year 5 (28%; 95% CI, 26%-31%; P < .001). The most common serious adverse event was infection (9 participants). Ten deaths were reported, none related to the study.Conclusions and RelevanceAlthough STN-DBS outcomes declined slightly, possibly due to the progressive nature of the disease, patients with PD sustained significant improvement in motor and activities of daily living scores, along with a stable reduction in anti-parkinsonian medication over the 5-year follow-up period.","PeriodicalId":14677,"journal":{"name":"JAMA neurology","volume":"1 1","pages":""},"PeriodicalIF":29.0,"publicationDate":"2025-09-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145058942","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":"Error in Figures.","authors":"","doi":"10.1001/jamaneurol.2025.3563","DOIUrl":"10.1001/jamaneurol.2025.3563","url":null,"abstract":"","PeriodicalId":14677,"journal":{"name":"JAMA neurology","volume":" ","pages":""},"PeriodicalIF":21.3,"publicationDate":"2025-09-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12439179/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145064498","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}