NeurologyPub Date : 2026-11-10Epub Date: 2026-10-13DOI: 10.1212/WNL.0000000000218557
David Reyes-Leiva, Álvaro Carbayo, Ana Vesperinas, Fernando Caballero-Martínez, Ricardo Rojas-Garcia, Janina Turon-Sans, Francesc Pla-Junca, Eduard Gallardo, Mar Pujades-Rodriguez, Elena Cortés-Vicente
{"title":"Disease Control in Generalized Myasthenia Gravis: Consensus Definition, Evolution, and Patient Characterization.","authors":"David Reyes-Leiva, Álvaro Carbayo, Ana Vesperinas, Fernando Caballero-Martínez, Ricardo Rojas-Garcia, Janina Turon-Sans, Francesc Pla-Junca, Eduard Gallardo, Mar Pujades-Rodriguez, Elena Cortés-Vicente","doi":"10.1212/WNL.0000000000218557","DOIUrl":"https://doi.org/10.1212/WNL.0000000000218557","url":null,"abstract":"<p><strong>Background and objectives: </strong>Generalized myasthenia gravis (gMG) is a chronic fluctuating neuromuscular disease. Some patients keep experiencing symptoms or adverse events (AEs) despite tailored therapeutic schemes. The aim of this study was to characterize adequately controlled gMG and describe patients' evolution and outcomes, including estimated direct health costs in a universal health care system.</p><p><strong>Methods: </strong>Spanish expert neurologists developed a definition of adequately controlled gMG using Delphi methodology. This was applied to an existing cohort of patients recently diagnosed with gMG during 1998-2020 in a Spanish referral neuromuscular diseases' unit. Control status, clinical outcomes, AEs, and health care resource utilization (HCRU) were described biennially.</p><p><strong>Results: </strong>We assessed 220 patients diagnosed with gMG (54.5% women; mean age at onset of 58 years; 84% anti-AChR, 6% anti-MuSK, 10% seronegative). Adequately controlled gMG was defined as achieving Myasthenia Gravis Foundation of America Post-Intervention Status classification of clinical stable remission, pharmacologic remission, or minimal manifestations stable for a year; having an MG-ADL score ≤2; experiencing no clinical exacerbations during the last year requiring a change in immunosuppressive therapy or rescue therapy; and having no or mild AE to therapies (100% of experts in agreement or total agreement). The proportion adequately controlled (AC) patients increased from 62.0% at 2 years after first visit to 81.9% after 8 years of follow-up. 47.3% of patients remained AC in every observation, and 17.7% remained not adequately controlled (NC) in every observation. No features were significantly associated with adequate control. AE-related drug interruption rates were higher in NC patients (ranging from 50% to 100% during the study). The mean cost per patient/year ranged from 2,718.6€ in the first biennial period to 843.1€ in the last biennial period for AC patients and from 10,190.1€ to 5,827.4€ for NC patients.</p><p><strong>Discussion: </strong>Based on the new gMG control consensus definition, almost 1 in 5 patients do not reach disease control even after 8 years of evolution. Those patients lacked specific clinical features, underscoring the unpredictability of the gMG course. Burden of AE-related drug interruptions, HCRU, and costs per patients/year were higher among those patients NC. However, our study is unicentric and included patients from a referral unit, with a possible overrepresentation of NC patients.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"107 9","pages":"e218557"},"PeriodicalIF":8.9,"publicationDate":"2026-11-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149183462","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}
NeurologyPub Date : 2026-11-10Epub Date: 2026-10-15DOI: 10.1212/WNL.0000000000218675
Andrea Morotti, Umberto Pensato, Jawed Nawabi, Andrea Dell'Orco, Federico Mazzacane, Qi Li, Giorgio Busto, Anna Cavallini, Raffaele Ornello, Mariarosaria Valente, Giovanni Merlino, Giovanni Baronchelli, Francesco Berinato, Emanuele Olivieri, Sebastiano Giacomozzi, Eugenio Magni, Mauro Gentile, Matteo Paolucci, Stefano Forlivesi, Maurizio Paciaroni, Michele Laudisi, Simona Sacco, Michele Romoli, Ilaria Casetta, Enrico Fainardi, Andrea Zini, Alessandro Padovani
{"title":"Intracerebral Hemorrhage Expansion in Late Presenters: Proposed Target Expansion Criteria.","authors":"Andrea Morotti, Umberto Pensato, Jawed Nawabi, Andrea Dell'Orco, Federico Mazzacane, Qi Li, Giorgio Busto, Anna Cavallini, Raffaele Ornello, Mariarosaria Valente, Giovanni Merlino, Giovanni Baronchelli, Francesco Berinato, Emanuele Olivieri, Sebastiano Giacomozzi, Eugenio Magni, Mauro Gentile, Matteo Paolucci, Stefano Forlivesi, Maurizio Paciaroni, Michele Laudisi, Simona Sacco, Michele Romoli, Ilaria Casetta, Enrico Fainardi, Andrea Zini, Alessandro Padovani","doi":"10.1212/WNL.0000000000218675","DOIUrl":"10.1212/WNL.0000000000218675","url":null,"abstract":"<p><strong>Background and objectives: </strong>The benefit of therapies targeting hematoma expansion (HE) in acute intracerebral hemorrhage (ICH) is highly time dependent and greatest within the first 2 hours after onset. We aimed to develop imaging-based \"target expansion criteria\" to identify late presenters (2-12 hours from onset/last seen well) who remain at high risk of HE. To achieve this goal, a practical risk score (target expansion score) was developed.</p><p><strong>Methods: </strong>We conducted a multicenter, international, retrospective cohort study. To mirror a clinical trial population, we excluded patients with brainstem ICH, anticoagulation, and baseline ICH volume >100 mL. The primary outcome was HE (>33%/6 mL). The following imaging predictors of HE were analyzed: high ultra-early hematoma growth (uHG) (baseline volume/onset to imaging time >10 mL/h), hypodensities on noncontrast CT (NCCT), and spot sign on CT angiography (CTA). The study population was randomly divided into development and validation cohorts, and a weighted score was derived from estimates from multivariable logistic regression.</p><p><strong>Results: </strong>Overall, 492 patients were included: 345 (70.1%) in the development and 147 (29.9%) in the validation cohort. A 4-point score was created, based on independent predictors of HE (high uHG: 1 point, NCCT hypodensities: 1 point, and CTA spot sign: 2 points). The score showed good discrimination in the development and validation cohorts (area under the curve [AUC] 0.77, 95% CI 0.71-0.83, and AUC 0.79, 95% CI 0.71-0.87, respectively). We observed a nonlinear increase in the frequency of HE and mean absolute hematoma growth with higher scores: 9.8% and 0.9 mL (score 0), 17.1% and 3.9 mL (score 1), 48.3% and 11.5 mL (score 2), 70.8% and 13.0 mL (score 3), and 87.5% and 26.9 mL (score 4). These findings were confirmed in the validation cohort. A dichotomized score greater than or equal to 2 predicted HE with 0.61 sensitivity and 0.85 specificity in the development cohort and 0.64 sensitivity with 0.84 specificity in the validation cohort.</p><p><strong>Discussion: </strong>Imaging-based target expansion criteria, defined as a target expansion score ≥2, identified late-presenting patients with ICH at high risk of HE. These findings might inform future trials targeting HE and increase the pool of eligible patients.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"107 9","pages":"e218675"},"PeriodicalIF":8.9,"publicationDate":"2026-11-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149195402","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}
NeurologyPub Date : 2026-11-10Epub Date: 2026-10-16DOI: 10.1212/WNL.0000000000218584
Caterina Felici, Rebecca E Green, Charlotte Warren-Gash, Julia Butt, Tim Waterboer, Alun Hughes, Nishi Chaturvedi, Ashvini Keshavan, William Coath, Jonathan M Schott, Marcus Richards, Dylan M Williams
{"title":"Associations of Antibodies Against Several Infections With Alzheimer Disease Neuropathology: A Prospective Cohort Study Analysis.","authors":"Caterina Felici, Rebecca E Green, Charlotte Warren-Gash, Julia Butt, Tim Waterboer, Alun Hughes, Nishi Chaturvedi, Ashvini Keshavan, William Coath, Jonathan M Schott, Marcus Richards, Dylan M Williams","doi":"10.1212/WNL.0000000000218584","DOIUrl":"https://doi.org/10.1212/WNL.0000000000218584","url":null,"abstract":"<p><strong>Background and objectives: </strong>Associations of common infections with Alzheimer disease (AD) risk have been reported. A hypothesized mechanism for these is cerebral β-amyloid (Aβ) aggregation as a defense to infection. We studied whether serologic measures of several infections in the UK population are associated with measures of AD neuropathology.</p><p><strong>Methods: </strong>This prospective association study used data from the 1946 British birth cohort. Circulating antibodies against 14 pathogens, measured at age 60-64 years, were modelled as pathogen serostatus (indicating lifetime exposure to an agent), pathogen burden indices (measuring cumulative exposure to 2+ pathogens), and seroreactivity tertiles (indicating recent immunologic activity against pathogens). Associations of these were tested with plasma phosphorylated tau at threonine 217 (p-tau217) (primary outcome) and Aβ status (secondary outcome), measured with PET scans, at ages 69-71 years. Modelling used multivariable quantile and logistic regression. Model 1 adjusted for sex and ages at serology and outcome assessment, and models 2 and 3 additionally adjusted for <i>APOE</i> ε4 carriage and education, respectively. We also tested for interactions in associations by <i>APOE</i> ε4 carriage, education, and sex, and for interactions between herpes simplex virus 1 exposure with cytomegalovirus (CMV) and varicella zoster virus (VZV) exposure.</p><p><strong>Results: </strong>A total of 1,356 and 424 individuals had complete data for p-tau217 and Aβ-PET analyses, respectively. Mean age at p-tau217 was 69.9 years (SD 0.7), and 51.3% of participants were female. Median p-tau217 was 0.30 pg/mL (interquartile range 0.21-0.44). 28.3% had Aβ-positive scans. No notable associations were observed for serology measures with either outcome in main models, except for an unexpected association of herpes simplex virus 2 seropositivity with lower p-tau217 at the 75th quantile (-0.07 pg/mL in seropositive individuals; 95% CI -0.10 to -0.03). Odds of Aβ-PET positivity may have been higher for individuals seropositive for Epstein-Barr virus (odds ratio [OR] 2.84; 95% CI 0.95-8.52) and <i>Toxoplasma gondii</i> (OR 1.69; 95% CI 0.94-3.05). There were also potential interactions in p-tau217 associations by <i>APOE</i> ε4 carriage (for <i>Helicobacter pylori</i> and CMV) and educational attainment (for <i>H pylori</i>).</p><p><strong>Discussion: </strong>These findings do not imply associations between exposures to many common infections and AD neuropathologies. The possibility of pathogen interactions with <i>APOE</i> ε4 carriage and education in relation to AD neuropathology warrants further study.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"107 9","pages":"e218584"},"PeriodicalIF":8.9,"publicationDate":"2026-11-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149207684","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":"Child Neurology: Transient Global Amnesia Precipitated by a Valsalva Maneuver in an Adolescent","authors":"Emilie Groulx-Boivin,Leena Toban,Lucia Carpineta,Elisabeth Simard Tremblay","doi":"10.1212/wnl.0000000000218571","DOIUrl":"https://doi.org/10.1212/wnl.0000000000218571","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"56 1","pages":""},"PeriodicalIF":9.9,"publicationDate":"2026-10-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149048972","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}
NeurologyPub Date : 2026-10-27DOI: 10.1212/wnl.0000000000218553
Aviv Fineberg,Axel Petzold,Mark A. Hellmann,Iliya Simantov,Yamit Cohen-Tayar,Omer Y. Bialer,Hadas Stiebel-Kalish
{"title":"Clinical Reasoning: An Immunocompetent Young Adult With Acute Vision Loss","authors":"Aviv Fineberg,Axel Petzold,Mark A. Hellmann,Iliya Simantov,Yamit Cohen-Tayar,Omer Y. Bialer,Hadas Stiebel-Kalish","doi":"10.1212/wnl.0000000000218553","DOIUrl":"https://doi.org/10.1212/wnl.0000000000218553","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"22 1","pages":""},"PeriodicalIF":9.9,"publicationDate":"2026-10-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149048974","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}
NeurologyPub Date : 2026-10-27DOI: 10.1212/wnl.0000000000213440
Raed A. Joundi,Eric E. Smith,Aravind Ganesh,Moira K. Kapral
{"title":"Author Response: Magnitude and Time-Course of Dementia Risk in Stroke Survivors","authors":"Raed A. Joundi,Eric E. Smith,Aravind Ganesh,Moira K. Kapral","doi":"10.1212/wnl.0000000000213440","DOIUrl":"https://doi.org/10.1212/wnl.0000000000213440","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"7 1","pages":""},"PeriodicalIF":9.9,"publicationDate":"2026-10-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149048976","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}