Neurology最新文献

{"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":"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":"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}

{"title":"Retirement of Guidelines.","authors":"","doi":"10.1212/WNL.0000000000213572","DOIUrl":"https://doi.org/10.1212/WNL.0000000000213572","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 9","pages":"e213572"},"PeriodicalIF":7.7,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144022722","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":"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":"Persimmon Season.","authors":"Christopher Kim","doi":"10.1212/WNL.0000000000213602","DOIUrl":"https://doi.org/10.1212/WNL.0000000000213602","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 9","pages":"e213602"},"PeriodicalIF":7.7,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144033990","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":"Longitudinal Evolution of Posterior Cortical Atrophy: Diagnostic Delays, Overlapping Phenotypes, and Clinical Outcomes.","authors":"Dror Shir, Noah Lee, Stuart J McCarter, Vijay K Ramanan, Hugo Botha, David S Knopman, Ronald C Petersen, Bradley F Boeve, Gregory Scott Day, Neill R Graff-Radford, David T Jones, Melissa E Murray, Aivi T Nguyen, R Ross Reichard, Dennis W Dickson, Deena Tajfirouz, Mary M Machulda, Jennifer L Whitwell, Keith Anthony Josephs, Jonathan Graff-Radford","doi":"10.1212/WNL.0000000000213559","DOIUrl":"10.1212/WNL.0000000000213559","url":null,"abstract":"<p><strong>Background and objectives: </strong>Although several large studies have evaluated individuals with posterior cortical atrophy (PCA) cross-sectionally, its longitudinal progression remains poorly characterized. The objectives of this study were to determine the longitudinal trajectory of PCA, encompassing the temporal aspects of diagnosis, the spectrum of clinical manifestations, and patient outcomes.</p><p><strong>Methods: </strong>This retrospective study included participants evaluated and diagnosed with PCA at the Mayo Clinic, between 1995 and 2023. Clinical data (demographics, neurologic evaluations, and cognitive tests at initial presentation and late stage) were extracted from medical records. Initial clinical diagnoses during previous medical evaluations, including ophthalmologic assessments after onset of neurologic symptoms, were documented. Participants were retrospectively classified as PCA-pure if they solely met PCA criteria or as PCA-plus if they exhibited complex phenotypes also meeting criteria for other neurodegenerative syndromes. CSF analyses and neuropathology findings were documented.</p><p><strong>Results: </strong>The cohort of 558 participants (65% female) had a mean age at symptom onset of 61 ± 8 years, with 68% meeting early-onset criteria (younger than 65 years). The mean duration from symptom onset to diagnosis was 3.6 ± 2.5 years. Ophthalmologic/optometric evaluations (49%) and completion of ophthalmologic procedures (16%) were common before PCA diagnosis. Psychiatric diagnoses were made in 23% of participants before PCA diagnosis, particularly among younger women. Common initial symptoms included misplacement of items, difficulties with reading and driving, and concerns pertaining to basic visual processing. Notable signs were constructional apraxia, dyscalculia, simultanagnosia, and space perception deficits. CSF biomarkers were consistent with Alzheimer disease in 139 of 158 individuals (88%). Superimposed features of non-PCA clinical syndromes were observed in a quarter of the participants at presentation, with frequency of PCA-plus cases increasing longitudinally. Longitudinal analysis of Short Test of Mental Status scores predicted an initial rapid decline in cognitive function, with the rate of decline gradually slowing over 0-10 years (time coefficient [SE] = -4.20 [0.29], <i>p</i> < 0.001).</p><p><strong>Discussion: </strong>This study highlights the protracted time from symptom onset and frequent misdiagnoses/misattribution of symptoms in PCA. Ophthalmologic evaluations often preceded neurologic assessments. Psychiatric diagnoses were more frequent among younger women. These observations highlight the need to improve diagnostic processes and earlier recognition of PCA, which may enhance the effectiveness of emerging disease-modifying therapies.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 9","pages":"e213559"},"PeriodicalIF":7.7,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11984831/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143811154","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":"Prehospital Large-Vessel Occlusion Stroke Detection Scales: A Pooled Individual Patient Data Analysis of 2 Prospective Cohorts.","authors":"Luuk Dekker, Jasper D Daems, Mariam Ali, Martijne H C Duvekot, Truc My T Nguyen, Esmee Venema, Marcel D J Durieux, Erik W van Zwet, Walid Moudrous, Ido R van den Wijngaard, Henk Kerkhoff, Hester F Lingsma, Diederik W J Dippel, Marieke J H Wermer, Bob Roozenbeek, Nyika D Kruyt","doi":"10.1212/WNL.0000000000213570","DOIUrl":"10.1212/WNL.0000000000213570","url":null,"abstract":"<p><strong>Background and objectives: </strong>Various prehospital scales have been developed to detect patients with anterior-circulation large-vessel occlusion (aLVO) ischemic stroke to enable direct transportation to a thrombectomy-capable stroke center. To guide implementation, a head-to-head comparison of aLVO stroke detection scales is needed to determine which scale is most useful for prehospital triage in different regional contexts. We aimed to systematically identify and compare these scales.</p><p><strong>Methods: </strong>Published prehospital aLVO stroke scales were identified with a systematic literature search. Scales were reconstructed from individual patient data of 2 large prospective observational cohort studies conducted between 2018 and 2019, the Leiden Prehospital Stroke Study and PREhospital triage of patients with suspected STrOke symptoms study. Both studies included consecutive adult patients suspected by paramedics of having a stroke within 6 hours of symptom onset, from 4 Dutch ambulance regions, encompassing 15 stroke centers and serving 3.7 million people. All data used for the reconstruction of scales were acquired by paramedics in the field before hospital arrival. Scales' diagnostic performance to detect aLVO stroke was compared with the area under the receiver operating characteristic curve (AUROC) of the full scale and sensitivity and specificity at the scales' original cut-point. Decision curve analysis was used to evaluate harm-benefit trade-offs between delaying IV thrombolysis and expediting endovascular thrombectomy with direct transportation of patients to a thrombectomy-capable center.</p><p><strong>Results: </strong>We identified 63 aLVO scales, of which 14 could be reconstructed. Of 2,358 included patients (mean age 70 years; 47% female), 231 (9.8%) had aLVO stroke. The AUROC was highest for Rapid Arterial oCclusion Evaluation (RACE) (0.81, 95% CI 0.78-0.84), Los Angeles Motor Scale (LAMS) (0.80, 95% CI 0.77-0.83), Gaze-Face-Arm-Speech-Time (G-FAST) (0.80, 95% CI 0.77-0.83), and modified Gaze-Face-Arm-Speech-Time (mG-FAST) (0.79, 95% CI 0.76-0.82). The Emergency Medical Stroke Assessment had highest sensitivity (85%, 95% CI 80%-90%) but lowest specificity (58%, 95% CI 56%-61%) while Cincinnati Prehospital Stroke Scale with an adjusted cut-point of 3 + gaze had highest specificity (94%, 95% CI 93%-95%) but lowest sensitivity (35%, 95% CI 29%-41%). In decision curve analysis, RACE had the highest benefit across a clinically reasonable range of harm-benefit trade-offs.</p><p><strong>Discussion: </strong>RACE, LAMS, G-FAST, and mG-FAST are the best-performing scales, with RACE being preferred in most triage settings. Our findings may support policymakers with implementing a scale suitable for their region.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 9","pages":"e213570"},"PeriodicalIF":7.7,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11984832/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143811785","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}