Neurology最新文献

{"title":"Late-Onset Seizures: Etiology and Demographics in US Tertiary Care Epilepsy Centers.","authors":"Leah J Blank, Emily L Johnson, Kyle R Pellerin, Rani A Sarkis, Tyler E Gaston, Mouhsin M Shafi, Rodrigo Zepeda, Maria Camitan, Tania Anderson, Alexis Hankerson, Aseel Jan, Katie Jung, Maddie Leake, M Brandon Westover, Alice D Lam","doi":"10.1212/WNL.0000000000214948","DOIUrl":"https://doi.org/10.1212/WNL.0000000000214948","url":null,"abstract":"<p><strong>Background and objectives: </strong>Adults older than age 55 years have the highest incidence rate and are the fastest-growing population among people with epilepsy. The aim of this study was to characterize the etiologies of new-onset seizures in older adults and to examine how seizure etiology varies across demographic groups. We used data from 7 US epilepsy centers from 2021 to 2025 and compared findings with those of previous population-based studies, providing an updated view and highlighting opportunities for prevention and improved risk stratification.</p><p><strong>Methods: </strong>We retrospectively reviewed medical charts of 2,052 patients aged ≥55 years at the time of a first seizure, who were evaluated at 7 epilepsy centers between 2021 and 2025. We categorized seizures by etiology as follows: ischemic stroke, hemorrhagic stroke, tumor, neurodegeneration, provoked seizures, traumatic brain injury, and unknown. We examined differences in etiology by demographic strata (age, sex, race, and primary language) using chi-square tests, Kruskal-Wallis tests, analysis of variance, and Cuzick tests.</p><p><strong>Results: </strong>The most frequent seizure etiologies among older adults were unknown (29.9%), ischemic stroke (15.4%), and provoked seizures (14.9%). Neurodegenerative disease was the etiology for 5.3% of cases overall but increased in prevalence with age, accounting for 18.5% among patients aged 85-89 years. Seizure etiologies also differed by sex and race. Men more commonly had seizures caused by cerebrovascular disease and traumatic brain injury, while women more commonly had seizures due to neurodegenerative disease. Black patients had higher proportions of ischemic stroke and neurodegenerative disease, while unexplained epilepsy was more common among White patients.</p><p><strong>Discussion: </strong>The causes of late-onset seizures vary based on age, sex, and race. Nearly one-third of cases of epilepsy in older adults remain unexplained despite advances in imaging techniques, underscoring the need for further research on the mechanisms and health implications of late-onset unexplained epilepsy. Improved prevention of cerebrovascular disease and optimized management of provoked seizures may reduce the growing burden of epilepsy in older adults.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"106 11","pages":"e214948"},"PeriodicalIF":8.5,"publicationDate":"2026-06-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147840949","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":"Glaucoma Risk Associated With Calcitonin Gene-Related Peptide Inhibitor Use in Migraine: A Multinational Cohort Study.","authors":"Chien-Chih Chou, Jr-Wei Wu, Hui-Ju Lin, I-Jong Wang, Ssu-Yu Pan, Chien-Hsiang Weng","doi":"10.1212/WNL.0000000000218035","DOIUrl":"https://doi.org/10.1212/WNL.0000000000218035","url":null,"abstract":"<p><strong>Background and objectives: </strong>Migraine has been linked to an increased risk of glaucoma, and our study aims to assess the temporal association between calcitonin gene-related peptide inhibitors (CGRPi) and the risks of glaucoma in individuals with migraine.</p><p><strong>Methods: </strong>This multinational retrospective cohort study included adults diagnosed with migraine who received migraine preventive medications in 2018-2024. Participants were categorized into the CGRPi group (erenumab, fremanezumab, galcanezumab, eptinezumab, atogepant, or rimegepant) and non-CGRPi group (valproate, topiramate, flunarizine, candesartan, lisinopril, metoprolol, propranolol, nadolol, amitriptyline, and venlafaxine). Crossovers were not allowed, and the non-CGRPi group included only individuals who never used CGRPi. The CGRPi and non-CGRPi groups were propensity score matched in a 1:1 ratio and were followed for up to 3 years to monitor the occurrence of incident glaucoma. The Cox proportional hazards model was used to compare the risk of glaucoma between CGRPi and non-CGRPi users, with results reported as hazard ratios (HRs) and 95% CIs.</p><p><strong>Results: </strong>A total of 73,644 individuals were included in the final analysis. Compared with non-CGRPi users, CGRPi users experience a lower risk of glaucoma development (HR 0.75; 95% CI 0.61-0.92) within 3 years since its first prescription. Individuals using CGRPi also exhibit a reduced risk of glaucoma compared with those using topiramate (HR 0.73; 95% CI 0.59-0.90), valproate (HR 0.54; 95% CI 0.35-0.83), propranolol (HR 0.76; 95% CI 0.59-0.98), metoprolol (HR 0.76; 95% CI 0.59-0.98), lisinopril (HR 0.49; 95% CI 0.38-0.62), amitriptyline (HR 0.69; 95% CI 0.54-0.89), and venlafaxine (HR 0.68; 95% CI 0.50-0.91). In the analysis that further classified participants based on the specific CGRPi used, only users of monoclonal antibody CGRPi show a reduced risk of glaucoma compared with non-CGRPi users (HR 0.77; 95% CI 0.61-0.98). The reduced risk of glaucoma associated with CGRPi is also observed in older adults, women, and those with chronic migraine or migraine without aura.</p><p><strong>Discussion: </strong>Among adults with migraine receiving preventive treatment, systemic use of CGRPi, particularly monoclonal antibody CGRPi, is associated with a reduced risk of glaucoma compared with the use of other migraine preventive medications.</p><p><strong>Classification of evidence: </strong>This is a Class II study demonstrating that CGRPi users with migraine have a lower risk of incident glaucoma when compared with non-CGRPi users with migraine.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"106 11","pages":"e218035"},"PeriodicalIF":8.5,"publicationDate":"2026-06-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147841040","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":"Malformations and Fetal Exposures to Antiseizure Medication Polytherapies.","authors":"Kimford J Meador","doi":"10.1212/WNL.0000000000218043","DOIUrl":"https://doi.org/10.1212/WNL.0000000000218043","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"106 11","pages":"e218043"},"PeriodicalIF":8.5,"publicationDate":"2026-06-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147840341","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 35-Year-Old Patient With Rapidly Progressive Ascending Weakness Leading to Loss of Brainstem Reflexes.","authors":"Hannah Lyons, Kelly Rath, Natalie Kreitzer, Daniel Kanter, Alisa Kanfi, Joseph LaPorta, Brandon Foreman","doi":"10.1212/WNL.0000000000218059","DOIUrl":"https://doi.org/10.1212/WNL.0000000000218059","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"106 11","pages":"e218059"},"PeriodicalIF":8.5,"publicationDate":"2026-06-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147856673","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":"Saltwater Medicine: The Healing Power of Tears in Neurology.","authors":"Lauren Bojarski","doi":"10.1212/WNL.0000000000218075","DOIUrl":"https://doi.org/10.1212/WNL.0000000000218075","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"106 11","pages":"e218075"},"PeriodicalIF":8.5,"publicationDate":"2026-06-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147856682","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: Bilateral Sturge-Weber Syndrome With Dyke-Davidoff-Masson Syndrome Changes.","authors":"Jie-Qiang Xia, Jian Fan, Longbiao Xie, Yan Lin, Hao Sun","doi":"10.1212/WNL.0000000000214951","DOIUrl":"https://doi.org/10.1212/WNL.0000000000214951","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"106 11","pages":"e214951"},"PeriodicalIF":8.5,"publicationDate":"2026-06-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147840706","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":"Sensitivity of Nerve and Skin Biopsy and Fat Aspirate for Amyloid in Symptomatic Hereditary ATTR Amyloidosis With Peripheral Neuropathy.","authors":"Alexa H Panrudkevich, Felipe J S Jones, Kamal Shouman, Christopher J Klein, P James B Dyck, Sarah E Berini, Christopher J Lamb, Jaclyn Jacobi, Nathan P Staff, Divyanshu Dubey, Janean K Engelstad, Jennifer M Martinez-Thompson, Martha Grogan, Angela Dispenzieri, Julie A Khoury, Elizabeth Ann Mauricio, Michelle L Mauermann, Marcus V Pinto","doi":"10.1212/WNL.0000000000218033","DOIUrl":"https://doi.org/10.1212/WNL.0000000000218033","url":null,"abstract":"<p><strong>Objectives: </strong>The aim of this study was to compare the sensitivity of nerve biopsy, fat aspirate, and skin biopsy in detecting amyloid in symptomatic hereditary transthyretin amyloidosis with peripheral neuropathy (ATTRv-PN).</p><p><strong>Methods: </strong>We identified patients with ATTRv-PN (2012-2023) seen at one of the Mayo Clinic 3 sites in Minnesota, Florida, and Arizona. Patients with symptomatic PN confirmed by a neurologist or EMG, who underwent an abdominal fat aspiration, skin biopsy, or nerve biopsy, were included. All patients had a pathogenic <i>TTR</i> variant and either a positive tissue biopsy or a positive pyrophosphate cardiac scintigraphy scan. Demographic, clinical, laboratory, and pathologic data were reviewed.</p><p><strong>Results: </strong>Of the 195 patients with symptomatic ATTRv-PN seen, 111 were included. The median age was 64.2 years (interquartile range 56.9-70.1), and 74.8% were male. Fat aspirate was positive for amyloid in 50 of 103 patients (sensitivity 48.5%, 95% CI 0.39-0.59), skin biopsy in 17 of 32 (sensitivity 53.1%, 95% CI 0.35-0.71), and nerve biopsy in 20 of 21 (sensitivity 95%, 95% CI 0.76-1.0). Skin biopsy yielded a higher sensitivity (60% vs 24%; <i>p</i> = 0.02, n = 25) in patients who underwent skin biopsy and fat aspirate.</p><p><strong>Discussion: </strong>Nerve biopsies are more sensitive than fat aspirate or skin biopsy for tissue diagnosis in this retrospective cohort of patients with symptomatic ATTRv-PN. Skin biopsy was slightly more sensitive than fat aspirate.</p><p><strong>Classification of evidence: </strong>This study provides Class IV evidence that a nerve biopsy, fat aspirate, and skin biopsy can accurately diagnose hereditary transthyretin amyloidosis in patients presenting with polyneuropathy. Nerve biopsies are more accurate than fat aspirates or skin biopsies in identifying patients with hereditary transthyretin amyloidosis.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"106 11","pages":"e218033"},"PeriodicalIF":8.5,"publicationDate":"2026-06-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147840690","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":"Lung Function and Respiratory Muscle Strength in Symptomatic Children and Adults With Spinal Muscular Atrophy Treated With Nusinersen.","authors":"Femke Vercoelen, Lieke A P Arts, Marit E M de Bruijne, Ruben P A van Eijk, Lina M Vermeer, Renske I Wadman, Laura P Verweij-van den Oudenrijn, Ruben S Zoodsma, Inge Cuppen, Bart Bartels, Fay-Lynn Asselman, Camiel A Wijngaarde, W Ludo van der Pol, Esther S Veldhoen","doi":"10.1212/WNL.0000000000214982","DOIUrl":"https://doi.org/10.1212/WNL.0000000000214982","url":null,"abstract":"<p><strong>Background and objectives: </strong>Spinal muscular atrophy (SMA) causes progressive muscle weakness. Respiratory muscle weakness results in progressive lung function decline, scoliosis, and chest wall deformities that predispose to respiratory failure. The efficacy of survival motor neuron protein-augmenting therapies on motor function in patients with SMA has been reported extensively, but how respiratory function changes under treatment remains underexplored. The aim of this study was to evaluate longitudinal changes in lung function and respiratory muscle strength during nusinersen treatment.</p><p><strong>Methods: </strong>We conducted a national retrospective observational cohort study at the University Medical Center Utrecht. We included children and adults with SMA types 1, 2, and 3a, who symptomatically started treatment with nusinersen between May 2017 and July 2025 as part of the Dutch reimbursement arrangements. We retrieved all available data on lung function and respiratory muscle strength collected during routine care. We analyzed the differences in the annual rates of change of lung function and respiratory muscle strength before and after nusinersen treatment initiation, using linear mixed-effects models.</p><p><strong>Results: </strong>We included 120 patients (60 female individuals, 50%), of whom 78 were children (median age 7.3 years, range 0.7, 17.7) and 42 adults (median age 34.4 years, range 18.1, 76.1) at nusinersen initiation. In children, we observed a slower decline in lung function and a higher expiratory muscle strength after starting nusinersen. The absolute mean slope differences were 4.1% (95% CI 2.2-6.0) for forced vital capacity, 3.8% (95% CI 1.4-6.2) for forced expiratory volume in 1 second (FEV₁), 4.2% (95% CI 0.3-8.0) for peak expiratory flow, and 5.1% (95% CI 0.7-7.6) for maximal expiratory pressure after initiation of nusinersen. In adults, we only observed a change in FEV₁ after starting nusinersen. At treatment initiation, 30 patients required ventilatory support. None were able to discontinue ventilation, and 6 patients started noninvasive ventilation during treatment.</p><p><strong>Discussion: </strong>This large retrospective cohort study found slower deterioration of lung function in symptomatic children during nusinersen treatment and improvements in respiratory muscle strength. In adult patients treated with nusinersen, we only observed a change in FEV₁.</p><p><strong>Classification of evidence: </strong>This study provides Class IV evidence that nusinersen slows deterioration of lung function and improves respiratory muscle strength in symptomatic children with SMA.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"106 11","pages":"e214982"},"PeriodicalIF":8.5,"publicationDate":"2026-06-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147840993","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 28-Year-Old Man With Seizures and Thalamic Lesions.","authors":"Ana B Santos-Rojo, Anthony Hong-Lo, Georgina Toledo-Rosello, Christopher Eckstein, Suma Shah, Nicholas Turner, Anne F Buckley, Cherylee W J Chang, Gabriel Torrealba-Acosta","doi":"10.1212/WNL.0000000000218065","DOIUrl":"https://doi.org/10.1212/WNL.0000000000218065","url":null,"abstract":"<p><p>This case involves a 28-year-old Black man who presented with new-onset seizures and bilateral medial temporal lobe hyperintensities on brain MRI, initially raising concern for viral encephalitis. CSF analysis revealed mild lymphocytic pleocytosis, but extensive infectious testing was negative. One year later, he developed acute right-sided weakness, and an MRI showed an enhancing FLAIR/T2 hyperintense mass-like lesion in the left thalamus. Stereotactic biopsy demonstrated reactive changes and Periodic acid-Schiff diastase (PASD)-positive macrophages, prompting consideration of atypical infectious or inflammatory etiologies. Despite empiric antibiotic treatment, his symptoms recurred 8 months later with new T2/FLAIR hyperintense lesions in the bilateral thalami and internal capsule, accompanied by mucocutaneous findings. He responded well to immunosuppressive therapy, with significant clinical and radiographic improvement. This case highlights the challenges of diagnosing relapsing neurologic syndromes with overlapping infectious, inflammatory, and neoplastic features and underscores the importance of integrating imaging, histopathology, and clinical evolution to reach a definitive diagnosis.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"106 11","pages":"e218065"},"PeriodicalIF":8.5,"publicationDate":"2026-06-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147856626","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 NeuroImages: Topless Optic Disc or Superior Segmental Optic Nerve Hypoplasia.","authors":"M Tariq Bhatti, Dipika B Joshi","doi":"10.1212/WNL.0000000000218051","DOIUrl":"https://doi.org/10.1212/WNL.0000000000218051","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"106 11","pages":"e218051"},"PeriodicalIF":8.5,"publicationDate":"2026-06-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147840847","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}