Neurology最新文献

{"title":"Perspectives of People With Multiple Sclerosis Regarding Data Linkage and Sharing.","authors":"Ruth Ann Marrie, Mudita Sharma, Gary R Cutter, Robert J Fox, Amber Salter","doi":"10.1212/WNL.0000000000213587","DOIUrl":"https://doi.org/10.1212/WNL.0000000000213587","url":null,"abstract":"<p><strong>Objectives: </strong>Linkage of clinical trial data to other data such as administrative data could enhance understanding of long-term outcomes. We investigated attitudes of people with multiple sclerosis (MS) regarding external linkage of clinical trial data.</p><p><strong>Methods: </strong>In a cross-sectional survey, North American Research Committee on Multiple Sclerosis registry participants reported willingness to share identifiers to support linkage of their clinical trial data to administrative health databases and preferences for long-term trial follow-up. Polytomous regression tested factors associated with agreeing to administrative data linkage.</p><p><strong>Results: </strong>Of 6,998 potential participants, 4,980 (71.2%) responded. Of 4,662 respondents meeting eligibility criteria, 3,524 participants (75.6%) indicated that they would agree or might agree to allow administrative data access. Participants were most willing to share their initials (43.7% definitely, 26.8% perhaps). Higher education (odds ratio [OR] 1.51; 1.19-1.93) and income (≥$100,000 vs <$50,000 OR 1.74; 1.17-2.59), alcohol consumption (OR range 1.77-2.27), and previous trial participation (yes/no, OR 1.89; 1.44-2.49) were associated with willingness to allow data access while Black race was associated with unwillingness (0.41; 0.20-0.82).</p><p><strong>Discussion: </strong>A substantial proportion of people with MS would potentially agree to data sharing and linkage to support clinical trials. Future studies should establish generalizability of these findings.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 9","pages":"e213587"},"PeriodicalIF":7.7,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143811781","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":"Having Second Thoughts About Parkinson Diagnosis.","authors":"Ronald B Postuma","doi":"10.1212/WNL.0000000000213594","DOIUrl":"https://doi.org/10.1212/WNL.0000000000213594","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 9","pages":"e213594"},"PeriodicalIF":7.7,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143784400","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":"Vertebrobasilar Dolichoectasia Presenting With Multiple Cranial Nerve Involvement.","authors":"Aurélien Freiherr von Seckendorff, Romain Deschamps, Augustin Lecler","doi":"10.1212/WNL.0000000000213541","DOIUrl":"https://doi.org/10.1212/WNL.0000000000213541","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 9","pages":"e213541"},"PeriodicalIF":7.7,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143772809","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":"Connected Speech Alterations and Progression in Patients With Primary Progressive Aphasia Variants.","authors":"Elisa Canu, Federica Agosta, Laura Lumaca, Silvia Basaia, Veronica Castelnovo, Sofia Santicioli, Stefano Pisano, Elena Gatti, Alessandra Lamanuzzi, Edoardo Gioele Spinelli, Giordano Cecchetti, Francesca Caso, Giuseppe Magnani, Paola Caroppo, Sara Prioni, Cristina Villa, Stefano F Cappa, Massimo Filippi","doi":"10.1212/WNL.0000000000213524","DOIUrl":"10.1212/WNL.0000000000213524","url":null,"abstract":"<p><strong>Background and objectives: </strong>Diagnosing the different variants of primary progressive aphasia (PPA) is challenging, but more accurate characterization can improve patient management and treatment outcomes. This study aimed to identify the following: (1) which speech features, alone or combined with language assessment and gray matter volumes (GMVs), best distinguish PPA variants and (2) how connected speech evolves in PPA.</p><p><strong>Methods: </strong>This prospective study was conducted at IRCCS San Raffaele Hospital in Milan, Italy, between 2010 and 2021. We included patients with PPA who underwent neuropsychological assessments, including standard evaluation of language and the \"Picnic Scene\" speech test, and, when available, brain structural MRI. Clinical and language assessments were also performed at follow-up in a subgroup. Sequential feature selection models identified speech parameters that best differentiated groups, incorporating age, sex, education, standard language tests, and GMVs. In each PPA group, linear mixed-effect models analyzed speech changes over time.</p><p><strong>Results: </strong>We included 95 patients with PPA (mean age 69 ± 9 years, 55 women [58%]; 40 with nonfluent variant PPA [nfvPPA], 35 with semantic variant PPA [svPPA], 20 with logopenic variant PPA [lvPPA]), of whom 82 underwent brain MRI and 34 had a follow-up visit after 10.2 months. Each model distinguished svPPA from the other PPA groups with high accuracy (<i>R</i>² range 0.93-1.00; <i>p</i> < 0.001). No differences in accuracy were observed among models for this distinction. In differentiating nfvPPA and lvPPA groups, the models incorporating speech parameters (<i>R</i>² = 0.92; <i>p</i> < 0.001), GMVs (<i>R</i>² = 0.95; <i>p</i> < 0.001), and their combination (speech + GMVs; <i>R</i>² = 0.97; <i>p</i> < 0.001) outperformed those using only standard language scores (<i>R</i>² = 0.75; <i>p</i> = 0.01). Over time, patients with nfvPPA showed more phonological errors, the svPPA group exhibited more semantic and morphosyntactic errors along with difficulties in naming and syntax production, and patients with lvPPA exhibited reduced number of words per second and fewer words per sentence.</p><p><strong>Discussion: </strong>All models were equally effective in distinguishing the svPPA group from the other 2 PPA subtypes. However, compared with using standard measures alone, incorporating speech measures from the \"Picnic Scene\" speech test, GMVs, or their combination into the models significantly improved accuracy in differentiating nfvPPA and lvPPA groups. The PPA variants showed distinct speech trajectories. These variables can aid in understanding disease progression, predicting patient outcomes, and planning speech therapy interventions in clinical practice.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 9","pages":"e213524"},"PeriodicalIF":7.7,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11974258/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143803898","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":"Pearls & Oy-Sters: Tiny Frontal Lesion Presenting With Generalized Epilepsy.","authors":"Michael Ginevra, Linda J Dalic, Kristian Bulluss, Aaron E L Warren, John S Archer","doi":"10.1212/WNL.0000000000213567","DOIUrl":"https://doi.org/10.1212/WNL.0000000000213567","url":null,"abstract":"<p><p>A 19-year-old woman presented with drug-refractory epilepsy since age 7 years. She had bilateral tonic seizures, generalized paroxysmal fast activity, and trains of anteriorly predominant <2.5 Hz generalized spike-wave discharges, but no intellectual disability. Structural MRI demonstrated a solitary 1 cm nodule of subcortical heterotopia in the right frontal pole that had been felt to be unrelated because of the \"generalized\" field of epileptiform discharges. A sEEG study targeting the nodule and adjacent frontal lobe demonstrated almost continuous epileptiform discharges from the nodule with more than 60 electrographic seizures arising broadly from the frontal electrodes. Radiofrequency thermocoagulation to the nodule resulted in more than 3 years of seizure freedom. This case demonstrates that highly focal lesions can produce a generalized electroclinical phenotype.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 9","pages":"e213567"},"PeriodicalIF":7.7,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143811779","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":"Corrections to Null Hypothesis Articles.","authors":"","doi":"10.1212/WNL.0000000000213475","DOIUrl":"https://doi.org/10.1212/WNL.0000000000213475","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 9","pages":"e213475"},"PeriodicalIF":7.7,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143784440","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":"Triple Antihypertensive Medication Prediction Score After Intracerebral Hemorrhage (the TRICH Score).","authors":"Ching Hei So, Charming Yeung, Ryan Wui-Hang Ho, Qing Hua Hou, Christopher H F Sum, William Leung, Yuen Kwun Wong, K C Roxanna Liu, Hon Hang Kwan, Joshua Fok, Edwin Kin-Keung Yip, Bun Sheng, Desmond Yat-Hin Yap, Gilberto K K Leung, Koon Ho Chan, Gary Kui Kai Lau, Kay Cheong Teo","doi":"10.1212/WNL.0000000000213560","DOIUrl":"10.1212/WNL.0000000000213560","url":null,"abstract":"<p><strong>Background and objectives: </strong>Poor long-term blood pressure (BP) control due to undertreatment of hypertension is not uncommon after intracerebral hemorrhage (ICH). It heightens the risk of ICH recurrence and subsequent stroke, which is the highest within the first year. Promptly achieving BP targets would significantly reduce these risks. To accomplish this, upfront triple antihypertensive medications could be prescribed soon after ICH because many ICH survivors require ≥3 antihypertensives. However, not all would suit this approach, particularly those with cerebral amyloid angiopathy (CAA), where elevated admission BP may be due to acute hypertensive response rather than underlying hypertension. In addition, overtreatment and excessive BP lowering would cause more side effects and have been associated with increased mortality in older patients. Hence, to facilitate individualized treatment, we aimed to develop a score (TRICH) to predict the need for ≥3 antihypertensives at 3 months after ICH.</p><p><strong>Methods: </strong>We developed the score using data from the University of Hong Kong prospective ICH registry (2011-2022) and validated it in 3 hospitals (2020-2022) locally. Consecutive patients with spontaneous ICH who survived >90 days and had follow-up BP 3 months after ICH were included. Predictors for needing ≥3 antihypertensive medications at 3 months were identified using multivariate logistic regression, and the score was created using the β-coefficients.</p><p><strong>Results: </strong>The TRICH score was developed from 462 patients (mean age 66.6 ± 14.3 years, 60% male) and validated in 203 patients (mean age 66.3 ± 14.6 years, 62% male). The 9-point score (age younger than 60 years = 1, male = 1, ischemic heart disease = 1, admission estimated glomerular filtration rate <60 mL/min/1.73 m² = 2, admission systolic BP 190-230 mm Hg = 2 while >230 mm Hg = 4) has a <i>c</i>-statistic (95% CI) of 0.79 (0.75-0.83) in the development cohort and 0.76 (0.69-0.82) in validation. A dichotomized score (≥3 points) predicted the need for ≥3 antihypertensives with 0.73 (95% CI 0.67-0.80) sensitivity and 0.76 (95% CI 0.70-0.81) specificity. The score performed better in patients with untreated/uncontrolled hypertension before ICH than in controlled patients (<i>c</i>-statistic [95% CI] 0.81 [0.77-0.86] vs 0.74 [0.69-0.80], <i>p</i> = 0.037) but showed no difference between patients with CAA and non-CAA patients.</p><p><strong>Discussion: </strong>The TRICH score identifies patients with ICH who need ≥3 antihypertensive medications 3 months after ICH with good discrimination ability. It may guide upfront triple antihypertensive prescription, but further research is warranted, particularly in non-Han Chinese populations.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 9","pages":"e213560"},"PeriodicalIF":7.7,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11970930/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143784402","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 of Social Determinants of Health With Genetic Test Request and Completion Rates in Children With Neurologic Disorders.","authors":"Jordan Janae Cole, Jonathan P Williams, Angela D Sellitto, Laura Rosa Baratta, Julia B Huecker, Dustin Baldridge, Thomas Kannampallil, Christina A Gurnett, Joyce E Balls-Berry","doi":"10.1212/WNL.0000000000213583","DOIUrl":"https://doi.org/10.1212/WNL.0000000000213583","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 9","pages":"e213583"},"PeriodicalIF":7.7,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143784438","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":"Diagnosis and Management of Children With Atypical Neuroinflammation.","authors":"Laura Saucier, Thomas Rossor, Mark P Gorman, Jonathan D Santoro, Yael Hacohen","doi":"10.1212/WNL.0000000000213537","DOIUrl":"https://doi.org/10.1212/WNL.0000000000213537","url":null,"abstract":"<p><p>Pediatric neuroimmune disorders comprise a heterogeneous group of immune-mediated CNS inflammatory conditions. Some, such as multiple sclerosis, are well defined by validated diagnostic criteria. Others, such as anti-NMDA receptor encephalitis, can be diagnosed with detection of specific autoantibodies. This review addresses neuroimmune disorders that neither feature a diagnosis-defining autoantibody nor meet criteria for a distinct clinicopathologic entity. A broad differential in these cases should include CNS infection, noninflammatory genetic disorders, toxic exposures, metabolic disturbances, and primary psychiatric disorders. Neuroimmune considerations addressed in this review include seronegative autoimmune encephalitis, seronegative demyelinating disorders such as neuromyelitis optica spectrum disorder, and genetic disorders of immune dysregulation or secondary neuroinflammation. In such cases, we recommend a broad diagnostic workup to support the presence of neuroinflammation, exclude non-neuroimmune disorders, detect autoantibodies and other biomarkers of known diseases, identify any potential genetic drivers of neuroinflammation, and provide case-specific insights into pathophysiologic mechanisms of inappropriate immune pathway activation or dysregulation. This review includes an extensive list of useful diagnostic tests and potential implications thereof, as well as a proposed algorithm for the diagnosis and management of the pediatric patient with atypical neuroimmune disorders. In general, first-line acute treatment of neuroimmune disorders begins with steroids, along with consideration of plasmapheresis or IV immunoglobulin. Selection of second-line or maintenance therapy is challenging without a definite, specific diagnosis and the associated benefit of established evidence-based treatment options. Immunotherapies may be considered based on the suspected mechanism of neuroinflammation and the likelihood of relapse. For example, rituximab may be considered for possible antibody-mediated or B-cell-mediated inflammation while anti-interleukin (IL)-6 agents, anti-IL-1 agents, or JAK inhibitors may be considered for certain cases of cytokine-mediated inflammation or innate immune system dysregulation. Care should be taken to monitor response and disease activity, revisit the differential diagnosis in the case of unexpected findings or poor treatment response, and weigh the risks of immunotherapy with the benefits of empiric treatment. Over time, further advancements in biomarker identification and omics research may define specific new clinicopathologic diagnoses and thus obviate the need for \"n of 1\" approaches to what are currently heterogeneous groups of atypical seronegative neuroimmune disorders.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 9","pages":"e213537"},"PeriodicalIF":7.7,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143784442","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":"Stroke in the Young: Intracranial Internal Carotid Artery Dissection.","authors":"Abhinay Kumar Gattu, Subhendu Parida, Vishnu Vardhan Anamalla, Jagarlapudi M K Murthy","doi":"10.1212/WNL.0000000000213543","DOIUrl":"https://doi.org/10.1212/WNL.0000000000213543","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 9","pages":"e213543"},"PeriodicalIF":7.7,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143772778","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}