Neurology最新文献

{"title":"Acute Kidney Injury and Risk of Adverse Neurocognitive Outcomes: A Systematic Review and Meta-Analysis.","authors":"Dearbhla M Kelly, Eoin M Kelleher, Peter M Rothwell","doi":"10.1212/WNL.0000000000218031","DOIUrl":"https://doi.org/10.1212/WNL.0000000000218031","url":null,"abstract":"<p><strong>Background and objectives: </strong>Chronic kidney disease is a recognized risk factor for adverse neurocognitive outcomes, but the effect of acute kidney injury (AKI) on brain health remains less well defined. We conducted a systematic review and meta-analysis to evaluate associations between AKI and subsequent risk of stroke, delirium, and dementia.</p><p><strong>Methods: </strong>Eligible studies were identified by searching Ovid MEDLINE and Embase from inception (Ovid: January 1946; Embase: January 1970) until April 2025. Studies were included if they reported quantitative estimates with measures of precision for the association between AKI and delirium, stroke, or dementia in adult populations. Two reviewers independently screened and extracted data, and study quality was assessed using standardized criteria. Study characteristics, participant demographics, and adjusted effect estimates (hazard ratios [HRs] or odds ratios [ORs]) with 95% CIs were extracted. Pooled HRs and ORs with 95% CIs were calculated using random-effects models. Heterogeneity was evaluated with the χ² test and <i>I</i>² statistic, and sources of heterogeneity were explored through prespecified subgroup analyses and meta-regression.</p><p><strong>Results: </strong>We identified 49 studies comprising 11,253,825 participants with 1,279,145 events. Individuals with AKI were at increased risk of stroke (pooled adjusted HR 1.35, 95% CI 1.20-1.52), delirium (pooled adjusted OR 1.76; 1.42-2.17), and dementia (pooled adjusted HR 1.64, 1.41-1.89). A gradient of risk across increasing AKI stages was demonstrated for stroke (stage 1: HR 1.11; 1.00-1.23; combined stages 2 and 3: HR 1.57; 1.35-1.81). AKI was also associated with higher in-hospital and 90-day mortality poststroke (pooled HR 2.13, 1.56-2.90, and 4.81, 2.55-9.08, respectively) and with 90-day disability (pooled adjusted OR 1.47, 1.22-1.76). Associations between AKI and all outcomes were directionally consistent across sensitivity analyses and pooled propensity score-matched studies.</p><p><strong>Discussion: </strong>In this systematic review and meta-analysis, AKI was consistently associated with increased short-term and long-term neurocognitive risk, including stroke, delirium, and dementia. These findings suggest that AKI may identify individuals vulnerable to both acute and chronic brain injury. Further studies are needed to clarify mechanisms linking AKI to brain injury and to identify strategies to mitigate neurocognitive risk in this high-risk population.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"106 11","pages":"e218031"},"PeriodicalIF":8.5,"publicationDate":"2026-06-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147840956","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: Stroke Recurrence After PFO Closure Uncovers Cerebrotendinous Xanthomatosis.","authors":"Lanyu Liu, Ying Wang, Xiujia Chen, Yiquan Li, Yicheng Fang, Cai Li","doi":"10.1212/WNL.0000000000218061","DOIUrl":"https://doi.org/10.1212/WNL.0000000000218061","url":null,"abstract":"<p><p>Cerebrotendinous xanthomatosis (CTX) is a rare autosomal recessive metabolic disorder that can cause premature atherosclerosis and ischemic stroke in young adults. We report a 23-year-old man who had recurrent strokes even after patent foramen ovale (PFO) closure. Initial evaluation for his first cryptogenic stroke revealed a PFO with a high Risk of Paradoxical Embolism (RoPE) score, leading to closure. Three years later, he experienced a recurrent stroke with right M1 segment occlusion. High-resolution magnetic resonance vessel wall imaging demonstrated intracranial atherosclerotic plaque, prompting reconsideration of the etiology. Further history taking revealed chronic diarrhea since infancy and juvenile cataracts. Examination showed borderline intellectual functioning, bilateral ankle clonus, and Achilles tendon xanthomas. Brain MRI exhibited characteristic bilateral dentate nucleus hyperintensities. Genetic testing confirmed CYP27A1 pathogenic variants, consistent with a diagnosis of CTX. Through highlighting CTX underlying premature atherosclerosis and stroke in a young man, this case emphasizes the importance of rare metabolic disorders in stroke, even when a common cause such as PFO is identified. Early recognition enables targeted therapy and improves outcomes.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"106 11","pages":"e218061"},"PeriodicalIF":8.5,"publicationDate":"2026-06-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147856689","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":"Striatal Dopamine Transporter and Rest Tremor in Parkinson Disease: A Clinical Validation.","authors":"Kalle J Niemi, Elina Jaakkola, Elina Maaria Myller, Mikael R E Eklund, Simo Nuuttila, Tuomas Mertsalmi, Kirsi-Marja Murtomäki, Reeta Levo, Tommi Noponen, Toni Ihalainen, Filip Scheperjans, Juho Joutsa, Valtteri Kaasinen","doi":"10.1212/WNL.0000000000218131","DOIUrl":"https://doi.org/10.1212/WNL.0000000000218131","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"106 11","pages":"e218131"},"PeriodicalIF":8.5,"publicationDate":"2026-06-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147856791","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: Cervical Myelopathy Due to Alveolar Spinal Echinococcosis Cysts.","authors":"Julian Ullrich, Marcomaria Meluso, Luca R Traini, Patrick Freund, Carl Moritz Zipser","doi":"10.1212/WNL.0000000000214950","DOIUrl":"https://doi.org/10.1212/WNL.0000000000214950","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"106 11","pages":"e214950"},"PeriodicalIF":8.5,"publicationDate":"2026-06-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147840653","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":"The Prevalence and Incidence of Cluster Headache: A Norwegian Population-Based Time-Trend Study.","authors":"Einar Naveen Møen, Marte-Helene Bjørk, Nils Erik Gilhus, Dagrun Slettebø Daltveit, Daniela Contreras, Knut Hagen, Mahendran Jayaraman, Andrej Netland Khanevski, Tore Wergeland, Anker Stubberud, Erling Tronvik, Jannicke Igland","doi":"10.1212/WNL.0000000000214862","DOIUrl":"https://doi.org/10.1212/WNL.0000000000214862","url":null,"abstract":"<p><strong>Background and objectives: </strong>Data on time trends in cluster headache epidemiology are sparse. The aim of this study was to report trends in prevalence and incidence of cluster headache in Norway over a 14-year period.</p><p><strong>Methods: </strong>We conducted a registry-based study using linked data from the Norwegian Registry for Primary Health Care, the Norwegian Control and Payment of Health Reimbursements Database, the Norwegian Patient Registry, the Norwegian Prescribed Drug Registry, and Statistics Norway from 2009 to 2022. Data included diagnostic codes, prescriptions, and education. Adults (age ≥18 years) were included. Cluster headache prevalence was defined as ≥2 contacts (clinical consults or prescriptions) for cluster headache in a 365-day period. Age-standardized trends in prevalence and incidence by sex and year, and interactions between education and year, were analyzed with negative binomial regression. We estimated prevalence rate ratio (PRR) and incidence rate ratio per calendar year with 95% CIs.</p><p><strong>Results: </strong>The number of patients with cluster headache increased from 1,029 in 2009 (median age 44 years; 39.7% women) to 1,833 patients in 2022 (median age 47 years; 50.1% women). The annual age-standardized prevalence rate increased from 27.0 to 42.5 per 100,000 in the same period. Women had a 3-fold higher annual increase of 6% (PRR 1.06, 95% CI 1.05-1.07) compared with 2% (PRR 1.02, 95% CI 1.02-1.03) in men. The prevalence rate was higher in women than in men by 2022 (43.4 vs 41.7 per 100,000). The annual prevalence of chronic cluster headache and refractory chronic cluster headache varied between 6%-7% and 1%-2% of all cluster headache cases, respectively. The annual age-standardized incidence rate of cluster headache increased in women, from 10.1 to 14.6 per 100,000 from 2012 to 2022 and decreased in men, from 13.5 to 11.0 per 100,000. Incidence and prevalence rates were higher among individuals with lower education.</p><p><strong>Discussion: </strong>Prevalence increased over 14 years, possibly reflecting improved diagnostic practices and awareness. These findings challenge previous reports of cluster headache predominantly affecting men, illustrating distinct shifts and trends in disease epidemiology. A limitation was the lack of clinical validation of cluster headache diagnostic codes in primary health care.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"106 11","pages":"e214862"},"PeriodicalIF":8.5,"publicationDate":"2026-06-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147840880","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 Video NeuroImage: Eating-Related Cataplexy in Prader-Willi Syndrome.","authors":"Lorenzo Chiarella, Ramona Cordani, Marco Veneruso, Antonella Barbieri, Flavia Napoli, Mohamad Maghnie, Lino Nobili","doi":"10.1212/WNL.0000000000218016","DOIUrl":"https://doi.org/10.1212/WNL.0000000000218016","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"106 11","pages":"e218016"},"PeriodicalIF":8.5,"publicationDate":"2026-06-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147840930","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":"Posterior Epidural Migrated Disc Mimicking a Tumor.","authors":"Guohui Zheng, Haijiang Li, Yunlei Zhai, Haiyang Yu","doi":"10.1212/WNL.0000000000218030","DOIUrl":"https://doi.org/10.1212/WNL.0000000000218030","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"106 11","pages":"e218030"},"PeriodicalIF":8.5,"publicationDate":"2026-06-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147840374","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":"Multiple Sclerosis and Outpatient Physician Care in the Last Years of Life: A Population-Based Study.","authors":"WooJin Kim, Christine L Watt, Stephen G Fung, Samantha Yoo, Colleen Webber, Dalia Rotstein, Michelle Howard, Peter Tanuseputro, Usha Ramanathan","doi":"10.1212/WNL.0000000000218067","DOIUrl":"https://doi.org/10.1212/WNL.0000000000218067","url":null,"abstract":"<p><strong>Background and objectives: </strong>Patients with multiple sclerosis (MS) require increasing health care support, particularly in the last year of life. Despite the benefits of palliative care in addressing symptom burden and improving quality of life, its integration into MS care remains inconsistent. This study aimed to characterize health care utilization and palliative care access among people with MS in the last years of life and to identify key factors associated with hospital death and receipt of palliative care.</p><p><strong>Methods: </strong>A retrospective cohort study was conducted using linked health administrative databases in Ontario, Canada. The cohort consisted of all decedents aged 18 years or older who died in Ontario between April 2016 and March 2020, and we compared health care utilization in decedents with MS to the rest of the cohort. The identification of MS cases was based on a validated algorithm. Demographics, health care utilization, palliative care involvement, and medical interventions were analyzed. Predictors of palliative care receipt and hospital deaths were evaluated using multivariable logistic regression models.</p><p><strong>Results: </strong>MS decedents (n = 1,975; mean age 68 years; 66.4% female) were younger and had fewer comorbidities than non-MS decedents (n = 500,904; mean age 77). High percentage of MS decedents had outpatient neurology visits in the last 5 years of life (49.4%) but a steep decline closer to death (2.7% in the last month). Palliative care use was similar (58.0% vs 56.9%) but occurred earlier in MS (61.9% vs 59.6% in the last 5 years). Multivariable regression showed that rural residence was associated with increased odds of hospital death (odds ratio [OR] 1.81, 95% CI 1.21-2.70) and lower odds of receiving palliative care (OR 0.74, 95% CI 0.55-0.99), while receiving palliative care within the last 5 years of life reduced the odds of hospital death (OR 0.47, 95% CI 0.37-0.60). Higher comorbidity and increased outpatient visits to urology were associated with a greater odd of receiving palliative care (OR 3.64, 95% CI 2.50-5.29 and OR 1.47, 95% CI 1.20-1.80).</p><p><strong>Discussion: </strong>Although palliative care receipt was comparable between MS and non-MS decedents, rural disparities and high hospital deaths persist. Earlier palliative integration, particularly through neuropalliative care, could improve quality of life and reduce hospitalizations.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"106 11","pages":"e218067"},"PeriodicalIF":8.5,"publicationDate":"2026-06-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147856617","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":"Bridging the Gap: Baló Concentric Sclerosis-Like Leukoencephalopathy in Chronic Cocaine Use: A 1-Year Clinical and Imaging Follow-Up of 2 Cases.","authors":"Maria Lanzante, Alfonso Cerase, Anna de Mauro, Azzurra Forti, Paola Gullino, Francesco Sicurelli, Maria Laura Stromillo, Ivano Chiarotti, Sergio Antonio Tripodi, Nicola De Stefano, Carla Battisti, Rosa Cortese","doi":"10.1212/WNL.0000000000218068","DOIUrl":"https://doi.org/10.1212/WNL.0000000000218068","url":null,"abstract":"<p><p>Cocaine use is occasionally associated with development of acute leukoencephalopathy but only rarely do the magnetic resonance imaging (MRI) features resemble Baló concentric sclerosis, a demyelinating disorder traditionally considered within the multiple sclerosis spectrum. Here, we report 2 patients with history of cocaine use living in the same geographic area who were referred for acute multifocal leukoencephalopathy with a Baló-like pattern.Patient 1, a 31-year-old woman originally from the Philippines with chronic cocaine use history, presented with 1 week of rapidly worsening nonfluent aphasia, neglect, severe right hemiparesis, and numbness on the same side. Two months later, patient 2, a 25-year-old man originally from Morocco with chronic cocaine use history, presented with acute left leg weakness, confusion, and neglect. In both patients, brain MRI revealed multiple T2-hyperintense rounded lesions in the white matter, characterized by a concentric target-like appearance and gadolinium-enhanced incomplete ring pattern. In patient 1, a stereotactic biopsy confirmed a demyelinating inflammatory process with microcystic degeneration, increased cellularity, and macrophage/CD4+ lymphocyte infiltration. Both patients were treated with high-dose IV corticosteroid, resulting in significant clinical and radiologic improvement sustained at 1-year follow-up. Although corticosteroids yield promising outcomes, long-term prognosis hinges on sustained treatment adherence and abstinence from cocaine.The temporal and geographic proximity of these cases, occurring within 2 months in the same Italian province (covering an area of approximately 70 miles), raises the possibility of a shared toxic exposure which might explain the unique pathology and provide clues to the immune mechanisms that drive Baló-like demyelination.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"106 11","pages":"e218068"},"PeriodicalIF":8.5,"publicationDate":"2026-06-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147856708","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":"Antiseizure Medication Polytherapies During Pregnancy and the Risk of Congenital Malformations.","authors":"Sharon C W Ng, Sonia Hernandez-Diaz, Moira Quinn, Jason R Ward, Susan Conant, Amy Lyons, Frances A High","doi":"10.1212/WNL.0000000000214984","DOIUrl":"https://doi.org/10.1212/WNL.0000000000214984","url":null,"abstract":"<p><strong>Background and objectives: </strong>The use of antiseizure medication (ASM) polytherapy has become increasingly common. However, it is known that there is an elevated risk of major congenital malformations (MCMs) in newborns after maternal use of select ASMs. This study characterizes the risk of MCMs in infants born to women using common ASM polytherapies during the first trimester.</p><p><strong>Methods: </strong>The study population was pregnant women enrolled in the North American AED Pregnancy Registry between 1997 and 2024. Phone interviews at enrollment, 7 months' gestation, and postpartum collected data on ASM use and maternal characteristics. ASM monotherapy or polytherapy exposure is defined by ASM or ASM combination use during the first 12 weeks of pregnancy. MCMs were confirmed by medical records and adjudicated by a blinded teratologist. The risk of MCMs was calculated among infants exposed to a specific ASM polytherapy during the first trimester of pregnancy and those exposed to lamotrigine (LTG) monotherapy. RRs and 95% CIs were estimated using logistic regression.</p><p><strong>Results: </strong>In a population of 15,318 pregnant women taking ASM at conception, 2,348 (15.3%) used 2 or more ASMs during the first trimester, with 796 polytherapy-exposed pregnancies eligible for analysis (mean age 30 years). The most common monotherapy was lamotrigine (LTG) (n = 2,582, mean age 31 years). The most common polytherapy combination was levetiracetam-lamotrigine (LEV-LTG) (38.4%). Indication of analyzed ASM polytherapy use was largely epilepsy (98.5%). The prevalence of MCMs was 2.0% for the reference LTG monotherapy group. Compared with LTG monotherapy, the adjusted RR of MCM was 1.63 (95% CI 0.69-3.40) for levetiracetam-LTG, 0.78 (0.04-3.78) for levetiracetam-carbamazepine, 0.99 (0.05-5.29) for levetiracetam-lacosamide, 1.40 (0.22-4.97) for levetiracetam-topiramate, 1.21 (0.07-6.22) for levetiracetam-zonisamide, 3.25 (0.74-9.98) for LTG-carbamazepine, 4.54 (1.61-11.07) for LTG-topiramate, and 3.22 (0.74-9.79) for LTG-zonisamide. Among infants exposed to any ASM polytherapy, the most common MCMs were hypospadias (5 cases), ventricular septal defect (5 cases), and cleft lip/palate (4 cases). All cleft lip/palate cases occurred in combinations with either topiramate or zonisamide.</p><p><strong>Discussion: </strong>Our findings suggest an increased risk of MCM associated with LTG-topiramate duotherapy compared with LTG monotherapy. Relative risk estimates were too imprecise for us to conclude on the teratogenicity of other analyzed polytherapy combinations.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"106 11","pages":"e214984"},"PeriodicalIF":8.5,"publicationDate":"2026-06-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147840995","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}