Neurology最新文献

{"title":"Functional Outcome in Patients With Carotid Artery Dissection Undergoing Thrombectomy or Standard Medical Treatment.","authors":"Marek Sykora, Sven Poli, Michael Giannakakis, Joshua Mbroh, Alexandra Gomez Exposito, Stefan Krebs, Alexandra Posekany, Mira Katan, Susanne Wegener, Gian Marco De Marchis, Thomas Gattringer, Hannes A Deutschmann, Lukas Mayer-Suess, Jens Fiehler, Ulrike Ernemann, Florian Hennersdorf, Tomas Dobrocky, Zsolt Kulcsár, Pasquale Mordasini, Marios Psychogios, Christian Loewe, Elke R Gizewski, Christian H Nolte, Christian Neumann, Urs Fischer, Julia Ferrari","doi":"10.1212/WNL.0000000000213825","DOIUrl":"https://doi.org/10.1212/WNL.0000000000213825","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 12","pages":"e213825"},"PeriodicalIF":7.7,"publicationDate":"2025-06-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144128221","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":"Accelerated Aging in Children With Multiple Sclerosis: Can We Stop the Clock?","authors":"E Ann Yeh","doi":"10.1212/WNL.0000000000213840","DOIUrl":"https://doi.org/10.1212/WNL.0000000000213840","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 12","pages":"e213840"},"PeriodicalIF":7.7,"publicationDate":"2025-06-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144216410","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":"Teaching NeuroImage: Cortical DWI Hyperintensity, Cerebellar Atrophy, and Presentation With Prolonged Postictal Hemiparesis Suggest CACNA1A-Related Disorder.","authors":"Iacopo Chiavacci,Adam Gareth Thomas,Krupa Torne,Gabriela Jones","doi":"10.1212/wnl.0000000000213872","DOIUrl":"https://doi.org/10.1212/wnl.0000000000213872","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"16 1","pages":"e213872"},"PeriodicalIF":9.9,"publicationDate":"2025-06-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144370201","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 60-Year-Old Man With Rapidly Progressive Left Hemibody Weakness and Vision Loss.","authors":"Matthew Rock,Shannon C Shipley,Jessica N Little,Joseph R Berger,Denise J Xu","doi":"10.1212/wnl.0000000000213869","DOIUrl":"https://doi.org/10.1212/wnl.0000000000213869","url":null,"abstract":"A 60-year-old man presented with 2 weeks of progressive left-sided weakness, dysarthria, and an incongruous left homonymous hemianopsia, with MRI brain showing a lesion involving the right cerebral peduncle, optic radiation, and dorsal midbrain. Serum and CSF testing was largely unremarkable. His weakness worsened rapidly, and short-interval repeat imaging demonstrated expansion of the brainstem abnormality. A methodical investigation of inflammatory, infectious, neoplastic, and paraneoplastic causes ultimately yielded the diagnosis. This case provides a comprehensive differential for atypical brainstem lesions, which can pose a diagnostic dilemma, particularly when CSF studies are unrevealing and the location is not amenable to biopsy. In such situations, a systematic approach, including whole-body imaging, can aid in diagnosis.","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"1 1","pages":"e213869"},"PeriodicalIF":9.9,"publicationDate":"2025-06-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144370202","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":"Pearls & Oy-sters: Arterial Wall Enhancements and Rapid Clinical Progression in Noninflammatory Cerebral Amyloid Angiopathy.","authors":"João Moura,Vasco Abreu,João Pedro Filipe,Ricardo Taipa,Luís F Maia","doi":"10.1212/wnl.0000000000213849","DOIUrl":"https://doi.org/10.1212/wnl.0000000000213849","url":null,"abstract":"Cerebral amyloid angiopathy (CAA) is a common cause of lobar intracerebral hemorrhage (ICH) and cognitive impairment for which diagnostic criteria have been recently revised. A subset of CAA cases have superimposed inflammation in the form of CAA-related inflammation, which may result in a more severe clinical course. We present the case of a 61-year-old man who presented with subacute behavioral changes, motor aphasia, and right hemiparesis due to an ICH in the left superior frontal gyrus. Brain MRI revealed a small chronic cortico-subcortical right occipital hemorrhage and multiple lobar microbleeds, fulfilling the criteria for probable CAA. One year later, he developed acute psychosis with aggressive behavior. β-Amyloid (Aβ) 1-40 and 1-42 levels were reduced in the CSF, with normal total tau and phosphorylated tau. He remained clinically stable during the following years. At age 68, he showed a rapid cognitive deterioration over 6 months, atypical for CAA. Repeat brain MRI showed multiple cortico-subcortical microbleeds and microinfarctions. High-resolution vessel wall MRI showed concentric wall enhancement in multiple arterial segments. These findings raised concerns for an inflammatory process, in the form of either inflammatory CAA or vasculitis. The neuropathologic findings were consistent with severe CAA without vessel wall inflammation. This case highlights the periods of steep progression that may occur in CAA and that Aβ accumulation alone, without inflammation, may be associated with arterial wall enhancement, mimicking a vasculitic or amyloid-related inflammatory process. The value of this neuroimaging feature for patient stratification or prognosis requires further validation.","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"22 1","pages":"e213849"},"PeriodicalIF":9.9,"publicationDate":"2025-06-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144370313","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":"Sudden Prehospital Deaths From Brain Arteriovenous Malformations: A Population-Based Study.","authors":"Anni Pohjola,Aleksanteri Asikainen,Jaakko Kaprio,Ilari Matias Rautalin,Mika Niemelä,Aki Laakso,Miikka Korja","doi":"10.1212/wnl.0000000000213818","DOIUrl":"https://doi.org/10.1212/wnl.0000000000213818","url":null,"abstract":"BACKGROUND AND OBJECTIVESThe number of unexpected prehospital deaths from brain arteriovenous malformations (AVMs) is unknown because existing mortality data are derived from hospital-based cohorts. In this study, we aimed to determine the rate and causes of AVM-related deaths in a large population-based cohort, both in and outside hospitals. We hypothesized that AVMs infrequently cause unexpected prehospital deaths, and when they do, it is typically due to hemorrhages rather than epileptic seizures.METHODSIn this population-based cross-sectional study, we examined the AVM-related deaths within the Helsinki University Hospital (HUH) region during 1998-2015, including both diagnosed patients and undiagnosed individuals with prehospital deaths identified at autopsy. Autopsies are legally mandatory in Finland for all unexpected and sudden deaths. Prehospital deaths were identified through autopsy reports, which were obtained from the National Cause of Death Register (using the ICD-10 codes I60.8, I61.0-I61.9, or Q28.0-Q28.3), and the patients who were already diagnosed were identified from the Helsinki AVM Register. Age-standardized detection rates were calculated using the 2013 European Standard Population (ESP)-derived age-specific weights, with 95% CIs derived from the normal approximation of binomial distribution.RESULTSBetween 1998 and 2015, we identified 4 unexpected prehospital AVM deaths in undiagnosed individuals (median age 41 years, 50% female) in the HUH region from the National Cause of Death Register data. For the same period, the HUH AVM database revealed 284 newly diagnosed patients with AVM (mean age at diagnosis 41.1 years [SD 18.0 years], 48% female). Altogether, there were 6 AVM-related prehospital deaths, 2 in previously diagnosed patients. Four of the 6 prehospital deaths were caused by AVM-related epileptic seizures and 2 by AVM hemorrhage. Among individuals experiencing their first AVM-related hemorrhage (n = 168), 1.2% experienced prehospital deaths due to hemorrhage. Similarly, of those diagnosed with AVM-related epileptic seizures (n = 69), 5.8% experienced prehospital deaths from seizures. For people with unruptured and ruptured AVMs, 6-month case fatality rates (CIs) from detection were 5.8% (1.7%-10.8%) and 11.9% (7.1%-17.3%), respectively. The crude and ESP-standardized detection rates of AVMs (including prehospital deaths) were 0.80 (0.71-0.89) and 0.78 (0.69-0.87) per 100,000 person-years.DISCUSSIONAVM-related prehospital deaths are rare and often caused by epileptic seizures. Most AVM-related deaths occur in hospitals.","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"19 1","pages":"e213818"},"PeriodicalIF":9.9,"publicationDate":"2025-06-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144370307","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: Acute and Progressive Cognitive Decline in a 38-Year-Old Man.","authors":"Lei Wu,Wei Jiang,RongRong Du,YuYing Liu,Sai Gao,Dehui Huang","doi":"10.1212/wnl.0000000000213871","DOIUrl":"https://doi.org/10.1212/wnl.0000000000213871","url":null,"abstract":"The differential diagnosis of multiple intracranial lesions is often perplexing and highly challenging, especially in the early stages. We present the case of a young man who initially presented with an acute headache and memory decline, which rapidly progressed to significant cognitive deterioration. Based on acute clinical manifestations, elevated CSF protein levels, and atypical MRI findings, the patient was initially misdiagnosed with idiopathic inflammatory demyelinating disease of the CNS. However, the rapid progression of symptoms and the enlargement of lesions on MRI raised further suspicion of cerebral venous sinus thrombosis, followed by primary angiitis of the CNS. Ultimately, digital subtraction angiography identified a rare intracranial vasculopathy, guiding appropriate management. The case highlights the importance of increased awareness in broadening the differential diagnosis, particularly in young patients presenting with acute cognitive decline and atypical imaging findings.","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"44 1","pages":"e213871"},"PeriodicalIF":9.9,"publicationDate":"2025-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144328622","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":"Scratching Beneath the Cortex: Superficial White Matter and Alzheimer Disease.","authors":"Mario Tranfa,Tiago Gil Oliveira","doi":"10.1212/wnl.0000000000213830","DOIUrl":"https://doi.org/10.1212/wnl.0000000000213830","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"236 1","pages":"e213830"},"PeriodicalIF":9.9,"publicationDate":"2025-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144328668","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":"Association of Superficial White Matter Microstructure With Cortical Pathology Deposition Across Early Stages of the AD Continuum.","authors":"Shuyue Wang,Fan Zhang,Qingze Zeng,Hui Hong,Yao Zhang,Linyun Xie,Miao Lin,Yeerfan Jiaerken,Xinfeng Yu,Ruiting Zhang,Xiao Luo,Kaicheng Li,Xiaopei Xu,Shiva Hassanzadeh-Behbahani,Bin Lin,Jarrett Rushmore,Chao Wang,Yogesh Rathi,Nikos Makris,Peiyu Huang,Minming Zhang,Jianzhong Sun,Lauren O'Donnell,","doi":"10.1212/wnl.0000000000213666","DOIUrl":"https://doi.org/10.1212/wnl.0000000000213666","url":null,"abstract":"BACKGROUND AND OBJECTIVESβ-amyloid (Aβ) and tau, 2 prominent pathologies of Alzheimer disease (AD), originate in cortical regions and primarily affect, and even spread along, the white matter tracts directly connected to these cortical regions. Superficial white matter (SWM), containing short-range association connections beneath the cortex, has been affected in mild cognitive impairment and AD, with gaps in understanding the disease's early stages. We perform a detailed investigation of individual SWM connections with cortical pathology deposition and cognition in the early stages of the AD continuum.METHODSWe enroll participants with Aβ PET, tau PET, diffusion MRI, and cognitive status from the Alzheimer's Disease Neuroimaging Initiative (ADNI) and Harvard Aging Brain Study (HABS). We stratify participants into disease stages following the Aβ/tau (AT) framework. We use diffusion MRI tractography to analyze SWM fiber clusters and assess their microstructure through free-water modeling, identifying significant differences between pathologically staged groups. We investigate associations of diffusion measures in SWM fiber clusters with regional pathology deposition (Aβ- and tau- PET uptake) and cognition.RESULTSThe study includes 150 ADNI participants (mean age 73.6 years, 61.3% female) and 175 HABS participants (mean age 75.6 years, 61.1% female). We find the following: (1) SWM microstructure differs along the early-stage Alzheimer disease continuum, with primary abnormalities in posterior brain regions; (2) there are significant free-water alterations in cognitively intact but pathology-positive individuals (p < 0.05, false discovery rate [FDR] corrected); (3) associations are identified between free water in SWM connections and proximal pathologic deposition, as well as between free water in several connections and memory function (standardized β coefficient = [-0.297 to -0.352], all p < 0.05, FDR corrected); and (iv) free water of a temporal SWM connection mediates the impact of temporal tau on memory (95% CI = [-0.146 to -0.002], accounts for 15.0% of the total effect).DISCUSSIONThe findings of this study suggest that the propagation of AD pathology and cognitive changes may involve SWM pathways even in the early stages, emphasizing the importance of SWM in developing AD therapies and early interventions.","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"37 1","pages":"e213666"},"PeriodicalIF":9.9,"publicationDate":"2025-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144328671","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":"CGRP-Targeted Migraine Therapies in Patients With Vascular Risk Factors or Stroke: A Review.","authors":"Michael Thomas Eller,Katarína Schwarzová,Lena Gufler,Anel Karisik,Katharina Kaltseis,Florian Frank,Gregor Broessner","doi":"10.1212/wnl.0000000000213852","DOIUrl":"https://doi.org/10.1212/wnl.0000000000213852","url":null,"abstract":"Calcitonin gene-related peptide (CGRP)-targeted therapies, including monoclonal antibodies (mAbs) and gepants, represent a major advancement in migraine prevention, offering greater efficacy and improved tolerability compared with traditional treatments. These agents selectively inhibit the CGRP pathway, a key mediator in migraine pathophysiology, and are increasingly used even as first-line options in selected patients. While clinical trials and real-world data suggest a favorable cardiovascular (CV) safety profile, particularly in patients without major risk factors, evidence remains limited for those with established vascular disease or recent vascular events. Concerns persist regarding long-term effects and the safety of CGRP blockade in high-risk populations. This narrative review focuses on the CV and cerebrovascular safety of CGRP-targeted migraine treatments-an area of growing clinical relevance. We compare these newer agents with traditional migraine preventives and highlight the paucity of data in patients with previous stroke, subarachnoid hemorrhage, myocardial infarction, or significant CV comorbidities. In addition, we discuss the emerging topic of dual CGRP pathway blockade (mAbs plus gepants), which has not previously been reviewed in the context of vascular risk. Based on currently available scientific evidence, we offer structured clinical considerations to guide the use of CGRP-targeted therapies in patients with vascular risk or cerebrovascular disease. Our aim is to support informed decision making in a population that has often been excluded from clinical trials but is becoming increasingly important in clinical practice.","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"14 1","pages":"e213852"},"PeriodicalIF":9.9,"publicationDate":"2025-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144328627","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}