Neurology最新文献

{"title":"\"Jack-Knife\" Dystonia in Pantothenate Kinase-Associated Neurodegeneration.","authors":"Manali Chaudhari, Koustubh Bavdhankar, Shailina Ali, Shruti Agrawal, Mayur Thakkar, Neeraj Jain, Sangeeta Ravat, Pankaj Agarwal","doi":"10.1212/WNL.0000000000213482","DOIUrl":"https://doi.org/10.1212/WNL.0000000000213482","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 7","pages":"e213482"},"PeriodicalIF":7.7,"publicationDate":"2025-04-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143605845","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":"Uncovering Subclinical Neural Alterations in Sport-Related Concussion: The Added Value of Longitudinal Neuroimaging.","authors":"Aurore Thibaut, Géraldine Martens","doi":"10.1212/WNL.0000000000213513","DOIUrl":"https://doi.org/10.1212/WNL.0000000000213513","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 7","pages":"e213513"},"PeriodicalIF":7.7,"publicationDate":"2025-04-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143616545","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":"Underestimation of Follow-Up Infarct Volume by Acute CT Perfusion Imaging.","authors":"Jelle Demeestere, Benjamin F J Verhaaren, Soren Christensen, Anke Wouters, Gregory W Albers, Maarten G Lansberg, Robin Lemmens","doi":"10.1212/WNL.0000000000213439","DOIUrl":"10.1212/WNL.0000000000213439","url":null,"abstract":"<p><strong>Background and objectives: </strong>It is unknown whether acute CT perfusion (CTP) core imaging may underestimate the follow-up infarct. We hypothesize that infarct underestimation occurs especially in late-presenting patients and that underestimated infarct can partially be detected on baseline noncontrast CT (NCCT).</p><p><strong>Methods: </strong>We included patients with acute anterior circulation ischemic stroke who underwent baseline NCCT and CTP imaging, complete endovascular reperfusion, and follow-up MRI from the Endovascular Therapy Following Imaging Evaluation for Ischemic Stroke (DEFUSE 3) trial and a consecutive, monocenter cohort. We divided patients into early (<6 hours) and late (6-24 hours) presenters. We performed semiautomated segmentations of the acute ischemic lesion on NCCT using 5% relative density difference (<i>rNCCT</i>_<i>>5%</i>) and used the relative cerebral blood flow <30% to segment the CTP core. On coregistered images, we performed volumetric and voxel-based analyses to compare infarct estimations by imaging modality. Spatial accuracy for the follow-up infarct was assessed using the Dice similarity coefficient (DSC) and balanced accuracy.</p><p><strong>Results: </strong>We included 109 patients with a median age of 70 (interquartile range [IQR] 31-93) years of whom 52% were female. The follow-up infarct was underestimated by the CTP core (mean absolute volume difference [MAVD] = 14 mL [SD 36], <i>p</i> < 0.001), but not by the union lesion (MAVD = 3 mL [SD 32], <i>p</i> = 0.76). Infarct underestimation was greater in late presenters (median 17 mL [IQR 7-33] vs 7 mL [IQR 4-25] in early presenters, <i>p</i> < 0.01) and in patients with poor collaterals (median 20 mL [IQR 8-56] vs 8 mL [IQR 4-20] in patients with good collaterals, <i>p</i> < 0.01). Median 25% of the infarct missed by the CTP core could be detected on baseline rNCCT in late presenters (vs. median 3% in early presenters). The combined <i>rNCCT</i>_<i>>5%</i> and CTP core lesion more accurately detected the follow-up infarct compared with the CTP core alone (median DSC 0.37 [IQR 0.06-0.55] vs 0.18 [IQR 0-0.42] and median balanced accuracy 0.67 [IQR 0.53-0.75] vs 0.56 [IQR 0.50-0.67], <i>p</i> < 0.001 for both).</p><p><strong>Discussion: </strong>Underestimation of follow-up infarct by CTP is substantial and the follow-up infarct can partially be detected by baseline NCCT, especially in patients with stroke with delayed presentation. Combining <i>rNCCT</i>_<i>>5%</i> and CTP increases the accuracy for predicting the follow-up infarct.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 7","pages":"e213439"},"PeriodicalIF":7.7,"publicationDate":"2025-04-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11908649/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143605936","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":"Cost Trends of New-To-Market Neurologic Medications: An Insurance Claims Database Analysis.","authors":"Amanda V Gusovsky Chevalier, Chun Chieh Lin, Kevin Kerber, Evan Lee Reynolds, Brian C Callaghan, James F Burke","doi":"10.1212/WNL.0000000000213428","DOIUrl":"10.1212/WNL.0000000000213428","url":null,"abstract":"<p><strong>Background and objectives: </strong>Costs for neurologic medications have increased considerably in recent years. Since 2014, more than 30 neurologic medications have been approved by the US Food and Drug Administration (FDA) for neurologic conditions. This study aims to characterize recent trends in annual costs and aggregate spending from 2012 to 2021 for new-to-market (NTM) medications for 9 neurologic conditions.</p><p><strong>Methods: </strong>We used the Merative MarketScan commercial and Medicare supplemental databases to observe patients seen by a neurologist with neurologic diseases with newly FDA-approved medications from 2014 to 2021: amyotrophic lateral sclerosis (ALS), transthyretin amyloidosis (ATTR), Duchenne muscular dystrophy (DMD), Huntington disease (HD), myasthenia gravis (MG), migraine, orthostatic hypotension (OH), tardive dyskinesia (TD), and spinal muscular atrophy (SMA). Patients were included if they had ≥1 disease-related prescription medication fill from 2012 to 2021. NTM (medications approved from 2014 to 2021) and older evidence-based guideline-supported medications were observed annually. Outcomes examined were annual and aggregate out-of-pocket (OOP) and total medication costs.</p><p><strong>Results: </strong>We identified 2,687 unique individuals with ALS, 38 with ATTR, 69 with DMD, 884 with HD, 9,984 with MG, 441,099 with migraine, 4,723 with OH, 1,266 with TD, and 17 with SMA. The youngest population was DMD (mean = 25 years [SD = 7]), and the oldest was TD (mean = 66 years [SD = 14]). For DMD, the population was 99% male and for migraine, the population was 84% female, and the other conditions had more relatively even sex divides. Collectively, migraine medications had the largest increase in aggregate costs (1993%) and had a substantial increase in OOP costs on average by 234% ($86-$288). Eculizumab for MG was an extreme outlier, with OOP costs increasing by 4,099% ($413-$17,359) and aggregate OOP costs by 7,005% ($5,375-$381,894). OOP costs of edaravone ($304-$5,707) and deutetrabenazine ($670-$7,170) sharply increased by 1,775% and 971%, respectively.</p><p><strong>Discussion: </strong>NTM medications for neurologic conditions have substantial and increasing individual and societal costs, which was not observed for older generic medications. These data suggest a need for policies to limit the financial burden of NTM medications on patients with neurologic conditions.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 6","pages":"e213428"},"PeriodicalIF":7.7,"publicationDate":"2025-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11874732/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143523921","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":"Teaching NeuroImage: Isolated Rosai-Dorfman Disease Resembling a Staghorn in the Spine.","authors":"Siyuan Pang, Zhimin Li, Yang Zhang, Yongning Li, Jun Gao","doi":"10.1212/WNL.0000000000213432","DOIUrl":"https://doi.org/10.1212/WNL.0000000000213432","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 6","pages":"e213432"},"PeriodicalIF":7.7,"publicationDate":"2025-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143458731","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":"Clinical Reasoning: A 26-Year-Old Woman With Headache and Eosinophilia.","authors":"Wilson Guo Wei Goh, Isaac Kah Siang Ng, Marcus Kai Xuan Tan, Clare Yoke Kum Fong, Christopher Yuan Kit Chua, Gabriel Zherong Yan, Jean-Marc Chavatte, Derek Tuck Loong Soon, Andrew Che-Fai Hui, Paul Ananth Tambyah, May Zin Myint","doi":"10.1212/WNL.0000000000213434","DOIUrl":"https://doi.org/10.1212/WNL.0000000000213434","url":null,"abstract":"<p><p>Headache accompanied by eosinophilia in blood or CSF presents a complex clinical challenge. We present a case of a 26-year-old woman with headache, peripheral eosinophilia with meningeal enhancement, and intracranial vasculopathy on imaging. This case illustrates a systematic approach to a patient with meningitis, peripheral eosinophilia, and cerebral vasculitis, which includes comprehensive clinical history taking and appropriate investigative workup to differentiate between infectious and noninfectious causes and timely tissue sampling to conclusively determine the causative agent.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 6","pages":"e213434"},"PeriodicalIF":7.7,"publicationDate":"2025-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143472617","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":"Editors' Note: Association of Prenatal Exposure to Antiseizure Medications With Creative and Executive Function at Age 4.5 Years.","authors":"Ariane Lewis, Steven L Galetta","doi":"10.1212/WNL.0000000000213476","DOIUrl":"https://doi.org/10.1212/WNL.0000000000213476","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 6","pages":"e213476"},"PeriodicalIF":7.7,"publicationDate":"2025-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143449585","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":"Effect Modifiers of the Association of Blood Pressure With Brain Amyloid and Tau Pathology.","authors":"Wenjie Cai, Julia Neitzel, Lidia Glodzik, Deborah Blacker, Yuan Ma","doi":"10.1212/WNL.0000000000213441","DOIUrl":"10.1212/WNL.0000000000213441","url":null,"abstract":"<p><strong>Background and objectives: </strong>Hypertension is an important modifiable risk factor of Alzheimer disease (AD), but previous studies reported heterogeneous associations of late-life blood pressure (BP) with brain amyloid and tau pathologies. We investigated how the associations of BP with brain amyloid and tau vary by <i>APOE</i> ε4 carriership, age, cerebrovascular burden, and cognitive status.</p><p><strong>Methods: </strong>We performed analyses among participants with postmortem neuropathology measurements (2005-June 2022) from the National Alzheimer's Coordinating Center. The average systolic BP (SBP) of the first 3 annual visits was the primary exposure, and baseline hypertension status was the secondary exposure. Brain AD pathologies were assessed using Thal and Braak staging. Potential modifiers included <i>APOE</i> ε4 carriership, age, stroke history, and cognitive status. Multinomial logistic regressions with interaction terms were used to test effect modification, adjusting for age, sex, <i>APOE</i> ε4 carriership, education, antihypertensive medication use, and years to death.</p><p><strong>Results: </strong>Among 2,094 participants (baseline age: 75 ± 9.5 years; 51.4% women), the association of higher SBP with higher amyloid and tau burdens varied by stroke history and cognitive status while the effect modification by age or <i>APOE</i> ε4 carriership was less consistent. More pronounced associations of SBP with higher amyloid and tau burdens were observed in those with dementia (vs without dementia) and those with a history of stroke (vs without stroke) (All <i>p</i> interaction<0.05). The odds ratios (ORs) per 10-mm Hg increase in SBP in the stroke vs nonstroke subgroup were 1.58 (95% CI 1.04-2.41) vs 1.14 (1.03-1.27) for amyloid and 1.54 (1.00-2.36) vs 1.04 (0.96-1.12) for tau. When comparing dementia with cognitively normal subgroups, ORs were 1.39 (1.17-1.64) vs 1.12 (0.96-1.31) for amyloid and 1.24 (1.08-1.42) vs 0.98 (0.85-1.14) for tau. Similar findings were observed for baseline hypertension status.</p><p><strong>Discussion: </strong>Preexisting cerebrovascular burden and cognitive status might interact with elevated SBP in their association with higher brain amyloid and tau, which could help identify high-risk subgroups for BP management and AD prevention. These heterogeneous association patterns need to be confirmed in longitudinal studies with in vivo AD pathology assessments.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 6","pages":"e213441"},"PeriodicalIF":7.7,"publicationDate":"2025-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11874733/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143523924","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":"Association Between Self-Perceived Stress and Cryptogenic Ischemic Stroke in Young Adults: A Case-Control Study.","authors":"Shakar Kutal, Lauri Juhani Tulkki, Tomi Sarkanen, Petra Redfors, Katarina Jood, Annika Nordanstig, Nilüfer Yeşilot, Mine Sezgin, Pauli Ylikotila, Marialuisa Zedde, Ulla Junttola, Annette Fromm, Kristina Ryliskiene, Radim Licenik, Phillip Ferdinand, Dalius Jatužis, Liisa Kõrv, Janika Kõrv, Alessandro Pezzini, Juha Sinisalo, Mika Lehto, Eva Gerdts, Jaana Autere, Ana Catarina Fonseca, Ulrike Waje-Andreassen, Bettina Von Sarnowski, Tiina Sairanen, Turgut Tatlisumak, Juha Huhtakangas, Pekka Jäkälä, Jukka Putaala, Nicolas Martinez-Majander","doi":"10.1212/WNL.0000000000213369","DOIUrl":"10.1212/WNL.0000000000213369","url":null,"abstract":"<p><strong>Background and objectives: </strong>Psychosocial stress is a potentially modifiable risk factor of early-onset ischemic stroke, with limited evidence suggesting a stronger association between stress and cryptogenic ischemic stroke (CIS) compared with strokes of known etiology. We aimed to explore the association between self-perceived stress and CIS, with subgroup analyses stratified by sex and age.</p><p><strong>Methods: </strong>Young patients aged 18-49 years with a first-ever CIS and sex-matched and age-matched stroke-free controls from 19 European centers were included. Self-perceived stress was assessed using a modified version of the Perceived Stress Scale (PSS). Scores were categorized into low (0-13), moderate (14-26), and high (27-40) perceived stress. Conditional logistic regression-adjusted for age, level of education, traditional risk factors (hypertension, cardiovascular diseases, diabetes mellitus, heavy alcohol consumption, current smoking, obesity, diet, depression, and physical inactivity), and migraine with aura (MA)-was used to assess independent association between self-perceived stress and CIS.</p><p><strong>Results: </strong>Altogether, 426 patients (median age 41 years; 47.7% women) and 426 controls were included. Patients were more often at least moderately stressed compared with controls (46.2% vs 33.3%, <i>p</i> < 0.001). In the entire study population, higher self-perceived stress as a discrete measure was independently associated with CIS: adjusted odds ratio (OR) 1.04 per point increase; 95% CI 1.01-1.07. Categorical PSS score analysis showed an independent association between moderate stress and CIS (OR 1.47; 95% CI 1.00-2.14), but not with high stress (2.62; 0.81-8.45). In sex-specific analysis, higher stress as a discrete measure was associated with CIS in women (1.06; 1.02-1.11), but not in men (1.02; 0.97-1.07). Moderate stress was associated with CIS in women (1.78; 1.07-2.96), but not in men (1.06; 0.58-1.96). When stratified by age, higher stress as a discrete measure was significantly associated with CIS only in patients aged 18-39 years (1.06; 1.00-1.11).</p><p><strong>Discussion: </strong>Self-perceived stress was strongly correlated with an increased risk of early-onset CIS, even after robust adjustment for cardiovascular risk factors and MA. These findings highlight the need for further investigation into the mechanisms by which stress may contribute to the risk of CIS. Possibility of recall bias should be considered when interpreting the results.</p><p><strong>Trial registration information: </strong>Clinical trial registration number: NCT01934725.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 6","pages":"e213369"},"PeriodicalIF":7.7,"publicationDate":"2025-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11894670/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143567807","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":"Teaching NeuroImage: An Unusual Infectious Cause of Pseudotumoral CNS Lesions.","authors":"Ana João Marques, Mariana Vargas, Andreia Veiga, Ricardo Taipa, João Paulo Gabriel","doi":"10.1212/WNL.0000000000213430","DOIUrl":"https://doi.org/10.1212/WNL.0000000000213430","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 6","pages":"e213430"},"PeriodicalIF":7.7,"publicationDate":"2025-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143441379","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}