Journal of Neurology最新文献

{"title":"Efficacy and safety of endovascular coiling compared to microsurgical clipping in aneurysmal subarachnoid hemorrhage: a systematic review and meta-analysis of randomized trials.","authors":"Martin Vychopen, Karl-Titus Hoffmann, Erdem Güresir, Johannes Wach","doi":"10.1007/s00415-026-14150-z","DOIUrl":"10.1007/s00415-026-14150-z","url":null,"abstract":"<p><strong>Background: </strong>Aneurysmal subarachnoid hemorrhage (aSAH) carries high morbidity and mortality despite advances in aneurysm repair. Microsurgical clipping and endovascular coiling are established treatment modalities, yet their relative benefits regarding functional outcome and long-term aneurysm occlusion remain debated. This study evaluates treatment effects stratified by aneurysm location and clinical grade.</p><p><strong>Methods: </strong>We identified RCTs comparing clipping and coiling for ruptured intracranial aneurysms. Primary outcomes were favorable functional outcome at 1-year [modified Rankin Scale (mRS), and complete aneurysm occlusion at 1-year]. Secondary outcomes included location-specific analyses, WFNS grade-specific analyses, and all-cause mortality.</p><p><strong>Results: </strong>Six RCTs involving up to 3044 patients were included. Coiling was associated with higher odds of favorable outcome at 1 year using mRS 0-2 (OR 1.60, 95% CI 1.36-1.88; p < 0.00001), but not when using mRS 0-3 (OR 1.19, p = 0.12). Location-specific analyses showed better outcomes after coiling for internal carotid artery aneurysms (OR 0.44, p < 0.00001), no difference for anterior communicating, anterior cerebral, middle cerebral, or posterior circulation aneurysms. Complete occlusion at 12 months favored clipping overall (OR 0.35, 95% CI 0.28-0.44; p < 0.00001; I² = 0%), driven by superiority in middle cerebral artery aneurysms (OR 5.21, p = 0.007). Mortality did not differ (OR 0.89, p = 0.35).</p><p><strong>Conclusions: </strong>Coiling offers superior 1-year functional outcomes when assessed with mRS 0-2, while clipping achieves higher complete occlusion rates, predominantly in middle cerebral artery aneurysms. No mortality difference was found.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"273 10","pages":""},"PeriodicalIF":5.4,"publicationDate":"2026-10-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13649945/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149137105","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Efficacy and safety of PCSK9 inhibitors combined with statins in acute ischemic stroke: a systematic review and meta-analysis.","authors":"Xiaozuo Lin, Rongxin He, Xiaohan Zhang, Yinan Zeng, Tian Wang, Simin Chen, Linkun Chen, Man Luo","doi":"10.1007/s00415-026-14199-w","DOIUrl":"10.1007/s00415-026-14199-w","url":null,"abstract":"<p><strong>Background: </strong>Acute ischemic stroke (AIS) imposes a substantial global burden of mortality and long-term disability. As novel lipid-modulating agents, proprotein convertase subtilisin/kexin type 9 inhibitors (PCSK9i) have demonstrated therapeutic benefits across a wide range of cardiovascular patient groups, but dedicated evidence in AIS cohorts has not been quantitatively synthesized. We therefore conducted this meta-analysis to compare the efficacy and safety of PCSK9i combined with statin therapy versus statin monotherapy among patients with AIS.</p><p><strong>Methods: </strong>We searched seven databases from inception to May 31, 2026 for randomized controlled trials (RCTs). Two reviewers independently performed study screening, data extraction, and risk-of-bias assessment. Dichotomous and continuous outcomes were synthesized using odds ratios (OR) and mean differences (MD), respectively. Heterogeneity was assessed via the I² statistic and Cochran's Q test, with leave-one-out sensitivity analysis and model switching to explore heterogeneity and test robustness. We evaluated the certainty of evidence using the GRADE approach.</p><p><strong>Results: </strong>Compared with statin monotherapy, PCSK9i combination therapy significantly improved functional independence (modified Rankin Scale (mRS) 0-2: OR = 2.17, 95% CI 1.29-3.64, P = 0.003, I² = 48%) and reduced recurrent ischemic stroke risk (OR = 0.33, 95% CI 0.16-0.69, P = 0.003, I² = 0%). LDL-C was substantially reduced in the combination group (MD = 0.66 mmol/L, 95% CI 0.45-0.87, P < 0.00001), with high heterogeneity (I² = 86%) partially explained by statin type (atorvastatin vs. rosuvastatin, P = 0.02). No significant difference was observed in National Institutes of Health Stroke Scale (NIHSS) scores (MD = 2.38, 95% CI - 0.63 to 5.39, P = 0.12, I² = 98%), with heterogeneity largely driven by one study. Safety outcomes, including abnormal liver function (OR = 0.67, 95% CI 0.35-1.28, P = 0.22), major adverse cardiovascular events(MACE) (OR = 0.56, 95% CI 0.18-1.75, P = 0.32), and total adverse events (OR = 0.85, 95% CI 0.49-1.45, P = 0.54), were comparable between groups. According to the GRADE assessment, evidence certainty was moderate for mRS, low for recurrent stroke, LDL-C, and safety outcomes, and very low for NIHSS.</p><p><strong>Conclusions: </strong>PCSK9i adjunctive therapy may improve functional recovery and reduce stroke recurrence in AIS patients receiving statins, with acceptable short-term safety; however, the current evidence remains preliminary and requires confirmation in larger, adequately powered randomized trials.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"273 10","pages":""},"PeriodicalIF":5.4,"publicationDate":"2026-10-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149137136","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Adherence to treatment recommendations in older adults with vertigo, dizziness, and balance disorders: the MobilE-TRA 2 cohort study.","authors":"Sophia P Schulze, Benedict Katzenberger, Daniela Koller, Doreen Huppert, Eva Grill, Ralf Strobl","doi":"10.1007/s00415-026-14195-0","DOIUrl":"10.1007/s00415-026-14195-0","url":null,"abstract":"<p><strong>Background: </strong>Specific exercises and lifestyle-related recommendations are used to manage vertigo, dizziness, and balance disorders (VDB), but evidence on adherence and its determinants in older adults is limited. This study examined adherence changes and associations with condition-related and patient-related factors from 3 to 12 months after therapeutic recommendations.</p><p><strong>Methods: </strong>We used data from the MobilE-TRA 2 prospective cohort of adults aged ≥60 years treated for VDB at a tertiary referral centre in Germany. The participants were assessed at baseline and followed up after 3 and 12 months. Self-reported adherence was measured using a five-point Likert scale and dichotomised at predefined cut-offs. Perceived dizziness-related handicap was assessed using the Dizziness Handicap Inventory (DHI). Longitudinal associations were analysed using logistic generalised linear mixed-effects models with random intercepts.</p><p><strong>Results: </strong>Overall, 337 participants were included (53% women; mean age 71 years). In adjusted analyses, adherence to specific exercises was lower at 12 than at 3 months (odds ratio (OR) 0.28; 95% confidence interval (CI) 0.12-0.67). Higher baseline DHI scores were significantly associated with higher adherence to specific exercises (OR 1.03; 95% CI 1.00-1.06), but not with lifestyle-related recommendations (OR 1.00; 95% CI 0.98-1.02). Male gender was associated with lower adherence to lifestyle-related recommendations (OR 0.37; 95% CI 0.16-0.88), while higher self-efficacy was associated with higher adherence (OR 1.65; 95%CI 1.02-2.66).</p><p><strong>Conclusions: </strong>Adherence differed by recommendation type, participant characteristics and over time in older adults with VDB. These findings may inform strategies to support adherence in VDB beyond initial follow-up.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"273 10","pages":""},"PeriodicalIF":5.4,"publicationDate":"2026-10-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13645870/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149127851","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Healthcare costs and burden of myasthenia gravis for patients and society: a systematic review.","authors":"Nils Erik Gilhus, Selma Conradi Melsom, Christoffer Bugge","doi":"10.1007/s00415-026-14111-6","DOIUrl":"10.1007/s00415-026-14111-6","url":null,"abstract":"<p><strong>Background and objectives: </strong>New and costly therapies for myasthenia gravis (MG) have been approved during the last years, and many more are in the pipeline. Cost-benefit analyses are needed as part of defining start and stop criteria for such treatments. In this systematic review, we examine healthcare costs associated with MG: METHODS: A systematic literature search combined the word \"myasthenia gravis\" with \"health resources\", and \"costs\". 1374 records were identified initially, and 44 were included in the final review.</p><p><strong>Results: </strong>Direct costs include treatment and follow-up in the primary and specialist health care. They were estimated to €3 500-12 000 per MG patient per year in the Nordic countries, similar in other European countries, and higher in United States. Indirect costs include productivity loss, welfare payments, time loss due to medical follow-up, and productivity loss for caregivers. They were estimated to €2 500-8 700 per patient per year in the Nordic countries, the highest figures appearing when all indirect costs were accounted for. A marked reduction in working capacity and an increase in welfare payments due to MG have been reported. Intangible costs include value of lost life-years and reduced quality of life, which are rarely assessed, but have been estimated to be half of the total societal MG costs.</p><p><strong>Discussion: </strong>Total costs of MG need to be included together with the expected benefits from new treatments when performing cost-benefit analyses. Precise knowledge of such costs is essential for healthcare planning and to ensure the availability of optimal treatments.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"273 10","pages":""},"PeriodicalIF":5.4,"publicationDate":"2026-10-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13645850/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149127846","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cortical thickness signature of early-onset Alzheimer's disease across clinical stages and A/T1 biomarker profiles.","authors":"Alessandro Zilioli, Rosaleena Mohanty, Anna Rosenberg, Anna Matton, Tobias Granberg, Göran Hagman, Marco Spallazzi, Miia Kivipelto, Eric Westman","doi":"10.1007/s00415-026-14187-0","DOIUrl":"10.1007/s00415-026-14187-0","url":null,"abstract":"<p><strong>Background: </strong>The cortical thinning signature of early-onset Alzheimer's disease (EOAD) is well established in dementia, but its emergence during earlier disease stages remains unclear. We investigated when it becomes detectable and whether it differs according to fluid amyloid and phosphorylated-tau (A/T1) biomarker profiles.</p><p><strong>Methods: </strong>We studied 292 individuals <65 years who underwent 3T structural MRI and cerebrospinal fluid assessment of Aβ42/40 and p-tau181. Participants were classified as A-T1- subjective cognitive decline (SCD; reference group), A+T1-, or A+T1+. Cortical thickness and hippocampal volume were measured using FreeSurfer. EOAD signature regions were defined by comparing biomarker-confirmed AD dementia with the reference group and subsequently examined across A/T1 profiles in SCD and mild cognitive impairment (MCI). Brain-cognition associations were assessed.</p><p><strong>Results: </strong>The cohort comprised 107 reference and 185 amyloid-positive participants (76 A+T1- and 109 A+T1+). EOAD dementia was characterized by marked cortical thinning in posterior parietal and lateral temporal regions. In MCI, cortical thinning was focal in A+T1-, but more extensive in A+T1+ who also showed hippocampal atrophy. At the SCD stage, focal thinning of the EOAD signature, precuneus, and banks of the superior temporal sulcus was observed in A+T1+, whereas no structural alterations were detected in A+T1-. Cortical thinning was associated with episodic and visuospatial memory performance in MCI, while no brain-cognition associations were observed in SCD.</p><p><strong>Conclusion: </strong>EOAD-related cortical thinning is detectable before dementia and varies across A/T1 profiles, with combined amyloid and p-tau181 positivity associated with earlier and more extensive structural involvement.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"273 10","pages":""},"PeriodicalIF":5.4,"publicationDate":"2026-10-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13642493/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149110625","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"High-frequency vestibulo-ocular reflex (VOR) evaluation in patients with spontaneous downbeat nystagmus: a case-control study.","authors":"T J Odorico, J L De La Fuente, F G Urbina, A K Mohamad, A Levy, M G Sorbara, D A Yacovino","doi":"10.1007/s00415-026-14192-3","DOIUrl":"10.1007/s00415-026-14192-3","url":null,"abstract":"<p><strong>Introduction: </strong>Spontaneous downbeat nystagmus (DBN) is generally attributed to vestibulocerebellar dysfunction, although its exact pathophysiological mechanism remains unclear. The video head impulse test (vHIT) quantifies vestibulo-ocular reflex (VOR) gain in semicircular canals, allowing evaluation of VOR imbalance.</p><p><strong>Objective: </strong>To elucidate the role of VOR in spontaneous DBN of central origin we measured gain across semicircular canals and gain asymmetry between coplanar canals, compared with matched healthy subjects.</p><p><strong>Methods: </strong>Nested case-control study within a retrospective cohort. We included 30 patients with DBN and 30 healthy controls matched for age and sex. Gains of the right horizontal (RH), left horizontal (LH), left anterior (LA), right posterior (RP), right anterior (RA), and left posterior (LP) canals were analyzed, together with horizontal (RH-LH) and vertical (LA-RP and RA-LP) gain asymmetry. Mann-Whitney U test was used for all comparisons (α = 0.05).</p><p><strong>Results: </strong>Cases showed significantly lower gains in the RH (p = 0.009), LH (p = 0.002), RP (p = 0.004), and LP (p = 0.016) canals, and a significantly higher gain in the RA canal (p = 0.002). The LA canal showed no significant difference. The LARP (p < 0.001) and RALP (p < 0.001) asymmetries were significantly greater in cases.</p><p><strong>Conclusions: </strong>Patients with DBN showed a pattern of VOR impairment characterized by hypofunction of the horizontal and posterior canals, hyperfunction of the anterior canals, and marked asymmetries in the vertical LARP and RALP planes. These findings are preliminarily consistent with the hypothesis of asymmetric cerebellar modulation of the VOR.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"273 10","pages":""},"PeriodicalIF":5.4,"publicationDate":"2026-10-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149127892","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Sex-related phenotypic differences in myotonic dystrophy types 1 and 2: a nationwide registry study from Serbia.","authors":"Ana Azanjac Arsic, Nikola Andrejic, Vanja Viric, Ilija Gunjic, Svetlana Sreckovic, Sonja Rajic, Zita Jovin, Ivo Bozovic, Gordana Djordjevic, Ivana Basta, Stojan Peric","doi":"10.1007/s00415-026-14190-5","DOIUrl":"10.1007/s00415-026-14190-5","url":null,"abstract":"<p><strong>Background: </strong>Myotonic dystrophies (DM) are autosomal dominant, multisystem disorders with variable clinical expressions. DM1 and DM2 share overlapping features, such as myotonia and muscle weakness, but differ in age at onset, severity, progression and multisystem features. Emerging evidence suggests that biological sex influences disease manifestations, progression, and outcomes.</p><p><strong>Objective: </strong>To assess sex-specific differences among patients with genetically confirmed DM1 and DM2.</p><p><strong>Patients and methods: </strong>This cross-sectional study included 587 adult patients (391 DM1, 196 DM2) from the Serbian Registry for Myotonic Dystrophies. Comprehensive assessments included neuromuscular examination, electrophysiological testing, systemic evaluation (cardiac, respiratory, endocrine, skeletal, and ophthalmologic), laboratory analysis, and functional status. Muscle strength was measured using a modified Medical Research Council score. Statistical analyses compared male and female patients within each DM type. Given the exploratory nature of the study, no adjustment for multiple comparisons was applied.</p><p><strong>Results: </strong>In DM1, the male-to-female ratio was 1.1:1. Males exhibited more severe neuromuscular involvement, including less strength in distal upper limb and proximal lower limb, longer myotonia duration, higher prevalence of dysarthria, dysphagia, polyneuropathy, skeletal deformities, baldness, and infertility, as well as more common impairment on ECG. In DM2, females predominated (ratio 1.5:1) and showed more pronounced weakness in proximal lower limb, reduced respiratory capacity, and more common cataracts. DM2 males had longer myotonia, higher prevalence of polyneuropathy, baldness, infertility, hyperglycemia, and more frequent pacemaker implantation.</p><p><strong>Conclusions: </strong>Sex is associated with distinct phenotypic profiles in DM1 and DM2. Greater neuromuscular burden was seen in DM1 males and DM2 females. On the other hand, male patients generally exhibit more cardiologic and endocrinological/metabolic burden in both DM1 and DM2. Incorporating sex as a variable in clinical evaluation, risk stratification, and therapeutic planning may be essential for personalized management of DM patients in future.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"273 10","pages":""},"PeriodicalIF":5.4,"publicationDate":"2026-10-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149111077","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"TRICALS risk score augments but cannot replace neurofilament as a prognostic biomarker in ALS.","authors":"Ahmad Al Khleifat, Andrea Malaspina, Janine Kirby, Timothy Tree, Pamela J Shaw, P Nigel Leigh, Ammar Al-Chalabi","doi":"10.1007/s00415-026-14040-4","DOIUrl":"10.1007/s00415-026-14040-4","url":null,"abstract":"<p><strong>Background: </strong>Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder with substantial clinical heterogeneity, complicating the design and interpretation of clinical trials. Two strategies have been proposed to address this variability: phenotype-based prognostic modelling and biomarker-based stratification using neurofilaments. We aimed to determine whether a widely used phenotype-based method, the TRICALS risk score, could substitute for neurofilament biomarkers in a clinical trial.</p><p><strong>Methods: </strong>We used data from the MIROCALS phase 2b randomised controlled trial of low-dose interleukin-2 in ALS (n = 220). The TRICALS risk score was calculated for each participant. The published biomarker model includes CSF phosphorylated neurofilament heavy chain (pNFH) and a pNFH × treatment interaction. We analysed the effects of replacing CSF pNFH with the TRICALS risk score and of adding TRICALS score as an additional covariate to the original model. Model performance was assessed using likelihood ratio χ² statistics and - 2 log likelihood (- 2LL). The primary outcome was survival.</p><p><strong>Results: </strong>TRICALS risk score was moderately correlated with CSF pNFH (r = 0.357), explaining 12.7% of shared variance. Substituting CSF pNFH with TRICALS score reduced model performance (- 2LL 928.25 vs 793.36). The TRICALS × treatment interaction was statistically significant (hazard ratio [HR] 0.72, 95% CI 0.54-0.96 p = 0.025), but contributed modestly to model fit (Δχ² = 4.76), compared with the pNFH × treatment interaction (Δχ² = 14.24). Incorporating the pNFH × treatment interaction significantly improved model fit and identified a treatment effect (HR 0.28, 95% CI 0.12-0.63; p = 0.002).</p><p><strong>Conclusions: </strong>The TRICALS risk score does not substitute for CSF neurofilament biomarkers as a prognostic or stratification variable but provides complementary information.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"273 10","pages":""},"PeriodicalIF":5.4,"publicationDate":"2026-10-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13642438/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149111051","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Associations between duration of status epilepticus and clinical outcomes in adults: a systematic review.","authors":"Fabio Iannaccone, Claudia Scarpitta, Elisa De Rossi, Chiara Milano, Francesco Turco, Enrica Bonanni, Francesco Fornai, Roberto Ceravolo, Chiara Pizzanelli","doi":"10.1007/s00415-026-14179-0","DOIUrl":"10.1007/s00415-026-14179-0","url":null,"abstract":"<p><strong>Objective: </strong>Duration of status epilepticus (SE) is widely considered a key determinant of outcome severity, but its prognostic value is hard to interpret because definitions of seizure onset, termination, and recurrence, as well as analytical handling, differ substantially across studies. We reviewed its association with mortality, functional outcome (motor and/or cognitive impairment), and refractoriness in adults.</p><p><strong>Methods: </strong>We searched MEDLINE (via PubMed), Cochrane CENTRAL, and Scopus for studies examining SE duration in relation to clinical outcomes in adults. We extracted study design, SE phenotype, duration definitions, analytical strategy, and temporal thresholds. Risk of bias was assessed with the QUIPS tool. Outcome-specific associations were synthesized narratively.</p><p><strong>Results: </strong>Forty-three studies (1994-2025) were included. The definitions and measurement of SE duration, as well as analytical approaches (continuous vs. categorical, with heterogeneous thresholds), varied widely. Mortality was assessed in 31 studies, functional outcome in 26, and refractoriness in 7. Longer duration was more consistently and independently associated with worse functional outcome than with mortality; the weaker mortality signal likely reflects competing determinants such as etiology, premorbid vulnerability, and systemic severity. The evidence on refractoriness was limited but consistent with a link between prolonged seizures and treatment resistance.</p><p><strong>Conclusions: </strong>SE duration should be interpreted as a context-dependent prognostic marker rather than as an absolute threshold. Its meaning appears to be shaped by the specific outcome, electroclinical phenotype, etiology, and diagnostic approach. Standardized onset and termination criteria, explicit recurrence rules, and harmonized modeling would improve cross-study comparability and strengthen prognostic assessment.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"273 10","pages":""},"PeriodicalIF":5.4,"publicationDate":"2026-10-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149110579","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"External validation of an integrative model of functional impairment in early-stage behavioral variant frontotemporal dementia (bvFTD).","authors":"Electra Chatzidimitriou, Despina Moraitou, Panagiotis Ioannidis, Eleni Aretouli, Yu Chen, Howard J Rosen, Maria Luisa Gorno-Tempini, William W Seeley, Bruce L Miller, Katherine P Rankin","doi":"10.1007/s00415-026-14182-5","DOIUrl":"10.1007/s00415-026-14182-5","url":null,"abstract":"<p><strong>Background/objectives: </strong>Functional decline in behavioral variant frontotemporal dementia (bvFTD) emerges early and significantly disrupts quality of life, yet its determinants remain incompletely understood. Integrative models capturing key disease manifestations may better explain its complexity but require validation in independent samples. This study aimed to examine whether a previously proposed model of functional impairment could be supported in a larger cohort and characterize the interrelationships among its cognitive, behavioral, personality, and neuroanatomical components.</p><p><strong>Methods: </strong>A total of 146 individuals with mild-stage bvFTD underwent structural MRI, neuropsychological assessment, and informant-based evaluation of behavioral symptoms, personality traits, and functional status. Penalized Least Absolute Shrinkage and Selection Operator (LASSO) regression identified the strongest correlates of functional impairment (Functional Activities Questionnaire/FAQ), while structural equation modeling examined their interrelationships.</p><p><strong>Results: </strong>LASSO identified conscientiousness, apathy, global cognition, executive functioning, sarcasm perception, and semantic verbal fluency as significant correlates of functional status. The resulting path model demonstrated excellent fit and supported both neural-cognitive and behavioral-personality pathways. Reduced right superior frontal gyrus volume was associated with poorer executive and global cognitive performance, and through these variables, with greater functional impairment. In parallel, apathy demonstrated both direct and indirect associations with functional decline through conscientiousness.</p><p><strong>Conclusions: </strong>Two partially distinct yet complementary processes appear to contribute to functional impairment in bvFTD: one reflecting the impact of frontal neurodegeneration on cognitive abilities essential for independent functioning, and another involving motivational and personality changes that directly undermine everyday engagement, thereby providing support for a multidimensional model of functional disability in bvFTD.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"273 10","pages":""},"PeriodicalIF":5.4,"publicationDate":"2026-10-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13638733/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149110773","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}