Journal of Neurology最新文献

{"title":"Dizziness and spatial disorientation in patients with transient global amnesia: clinical and vestibular correlates.","authors":"Ileok Jung, Jin-Man Jung, Moon-Ho Park, Ji-Soo Kim","doi":"10.1007/s00415-025-13336-1","DOIUrl":"https://doi.org/10.1007/s00415-025-13336-1","url":null,"abstract":"<p><strong>Objective: </strong>To determine the prevalence and clinical significance of dizziness and spatial disorientation in patients with transient global amnesia (TGA), and to examine their relationships with vestibular function and imaging findings.</p><p><strong>Methods: </strong>We retrospective analyzed 64 TGA episodes from 63 patients [46 (71.9%) women, mean age ± SD = 58.6 ± 9.4]. Vestibular and ocular motor function test [including vestibular-evoked myogenic potentials (VEMPs)], neuropsychological assessments, and brain imaging were evaluated. Logistic regression was used to identify factors associated with dizziness and spatial disorientation.</p><p><strong>Results: </strong>Dizziness was reported in 30.6% (19/62 episodes), and spatial disorientation in 25.0% (16/64 episodes). In multivariable analysis, dizziness was independently associated with headache [odds ratio (OR) = 6.94; 95% confidence interval (CI) 1.91-25.25; p = 0.003] and Valsalva-like provoking events (OR = 4.19; 95% CI 1.14-15.38; p = 0.031). Abnormalities in ocular vestibular-evoked myogenic potentials (VEMPs) were significantly association with dizziness in univariate analysis (OR = 4.00; 95% CI 1.13-14.09; p = 0.031), but not in the multivariable analysis. In relation to spatial disorientation, abnormal cervical VEMPs showed a significant difference in chi-square test (p = 0.043), and a trend toward association in multivariable analysis (OR = 3.28; 95% CI 0.885-12.19; p = 0.076), suggesting otolithic vestibular dysfunction. There was a trend-level association between spatial disorientation and lower Korea-Montreal Cognitive Assessment scores (p = 0.066). Interestingly, patients in the spatial disorientation group showed better performance in visual memory immediate recall (p = 0.061), although this did not reach statistical significance. Hippocampal lesions were identified in 75.0% of TGA episodes, most commonly involving the hippocampal head. However, lesion laterality (left, right, or bilateral) was not associated with dizziness, spatial disorientation, or vestibular abnormalities.</p><p><strong>Conclusion: </strong>Dizziness and spatial disorientation are common and clinically meaningful symptoms in TGA, likely reflecting transient dysfunction of specific vestibular pathways. These findings suggest broader network involvement in TGA in addition to the hippocampus, particularly implicating otolith-ocular and vestibulo-spinal pathways.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 9","pages":"594"},"PeriodicalIF":4.6,"publicationDate":"2025-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12391237/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144957746","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":"Not for everyone: examining predictors of cognitive rehabilitation success in multiple sclerosis.","authors":"Anne Kever, Nayeon Kang, Cecilia Meza, Marta Pogacar, Robin Lui, Kelly Hennessy, Michelle Williams, Jiwon Oh, Anthony Feinstein","doi":"10.1007/s00415-025-13345-0","DOIUrl":"https://doi.org/10.1007/s00415-025-13345-0","url":null,"abstract":"","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 9","pages":"593"},"PeriodicalIF":4.6,"publicationDate":"2025-08-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144957816","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":"Cerebrospinal fluid levels of chitinase 3-like 1 and interleukin-6 can predict response to platform therapies in relapsing multiple sclerosis.","authors":"Guillaume Mathey, Jonathan Epstein, Tom Alix, Melissa Julien, Estelle Nisse, Sophie Pittion-Vouyovitch, Chloé Prunis, Catherine Malaplate","doi":"10.1007/s00415-025-13321-8","DOIUrl":"https://doi.org/10.1007/s00415-025-13321-8","url":null,"abstract":"<p><strong>Background: </strong>Platform therapies, such as dimethylfumarate, teriflunomide, and interferons beta, are insufficient to control relapsing-remitting multiple sclerosis in many patients. Having biomarkers available to stratify patients as good or poor responders before starting treatment would represent a considerable advance.</p><p><strong>Methods: </strong>We tested serum and cerebrospinal fluid (CSF) levels of chitinase 3-like 1 (c and s-CHI3L1), soluble trigger receptor expressed on myeloid cells (sTREM2) and neuronal pentraxin 2 (NPTX2), and CSF levels of neurofilament light chains and of interleukin-6 (c-IL6) collected before treatment start in 70 patients in Eastern France. We explored associations with the status responder or non-responder after 12 months of efficient treatment. Non-responders were defined as patients with sign of activity, or confirmed increase of disability, or treatment cessation due to « inefficacy» according to the treating physician.</p><p><strong>Results: </strong>39 patients were non-responders (55.7%). c-CHI3L1 taken as a binary variable (under or above 164.6 ng/mL) and c-IL6 either as a binary variable (detectable or not) were associated with being non-responders. The positive predictive value of being non-responder in case of c-CHI3L1 higher than 164.6 ng/mL was 0.63 (95 confidence interval 95CI 0.43-0.82). No responder patients had detectable c-IL6, 12 non-responders (46.2%) had detectable c-IL6. Having high values of c-CHI3L1 and/or detectable c-IL6 was associated with an 8.33 higher risk of being non-responder after 1 year of effective platform treatment (95CI 1.44-33.33; p = 0.001).</p><p><strong>Conclusions: </strong>CHI3L1 and IL6 in CSF could predict the first-year response to platform therapies.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 9","pages":"592"},"PeriodicalIF":4.6,"publicationDate":"2025-08-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144957690","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":"Can memory mapping via stereo-electroencephalography stimulation predict post-operative memory outcomes? A case report.","authors":"Enric Bellido-Castillo, Erik J Kaestner, Donald J Bearden, Carrie R McDonald, Manuel Toledo, Elena Fonseca","doi":"10.1007/s00415-025-13307-6","DOIUrl":"https://doi.org/10.1007/s00415-025-13307-6","url":null,"abstract":"","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 9","pages":"591"},"PeriodicalIF":4.6,"publicationDate":"2025-08-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144957723","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":"Functional exercise training in persons with multiple sclerosis: a systematic review.","authors":"Frederike Adammek, Weronika Gralla, Marie Kupjetz, Annette Rademacher, Philipp Zimmer, Eduard Isenmann, Niklas Joisten","doi":"10.1007/s00415-025-13311-w","DOIUrl":"https://doi.org/10.1007/s00415-025-13311-w","url":null,"abstract":"<p><p>Functional exercise training focuses on enhancing the body's capacity for everyday movements by targeting a combination of strength, stability, balance, coordination, and flexibility. More flexible and less standardized exercise approaches, such as functional exercise training, may offer further benefits on improving symptoms in persons with MS (pwMS), but they have been less investigated. This review aims to provide a comprehensive overview of the existing literature on the effects of functional exercise training on functional outcomes (mobility, walking capacity, balance, and proprioception), physical (strength and endurance) and physiological performance measures, as well as patient-reported outcomes (Quality of Life, Fatigue) in pwMS. The systematic review was conducted according to the PRISMA guidelines. Literature searches were conducted in Medline (PubMed) and Cochrane CENTRAL (inception to 19th February 2025). We included studies investigating the effects of functional exercise training on any outcome in adult pwMS. The quality and reporting were evaluated using the Tool for the assEssment of Study qualiTy and reporting in EXercise (TESTEX). A total of 19 studies were included in the review, ranging from smaller pilot studies to larger randomized controlled trials. Interventions lasted between six and 16 weeks. Results of the studies included in this review were highly heterogeneous. Some of the included studies have demonstrated improvements in mobility, short and long walking capacity, balance, strength, and endurance measures as well as on quality of life and fatigue. Adherence to training was poorly reported. Functional exercise training for pwMS is a comprehensive approach to achieving functional improvements, strength gains, and improved endurance simultaneously, thereby promoting beneficial health effects. In future, a clear definition of functional exercise training is required, research should focus on the consistent reporting of treatment adherence, and investigate the long-term effects of functional exercise training in pwMS.PROSPERO registration number: CRD42023448077.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 9","pages":"590"},"PeriodicalIF":4.6,"publicationDate":"2025-08-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12374876/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144957742","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":"Short-term effects of air pollution and weather on reversible cerebral vasoconstriction syndrome: a retrospective bicenter study.","authors":"Pacôme Constant Dit Beaufils, Matilde Karakachoff, Frédéric Penven, Cédric Urbanczyck, Pierre-Antoine Gourraud, Solène de Gaalon","doi":"10.1007/s00415-025-13306-7","DOIUrl":"https://doi.org/10.1007/s00415-025-13306-7","url":null,"abstract":"<p><strong>Background: </strong>Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by thunderclap headaches and transient cerebral vasoconstriction. The influence of environmental factors, particularly air pollution and weather conditions, on RCVS remains poorly understood.</p><p><strong>Methods: </strong>From 2010 to 2022, clinical data from two hospitals were analyzed alongside air pollution and meteorological data provided by Air Pays de la Loire and Météo-France. A time-stratified case-crossover design was used to evaluate the association between RCVS onset and daily mean concentrations of O₃, PM₁₀, PM_2.5, and NO₂, using spatial modeling of residential exposure. Pollutant effects were examined over lag periods of up to three days, with adjustments for temperature and atmospheric pressure.</p><p><strong>Results: </strong>A total of 216 patients were included (mean age: 39.9 years; SD: 12.3; sex ratio: 1:1). In the three days preceding RCVS onset, only PM₁₀ concentrations remained significantly higher compared to the day of onset (19.03 vs. 17.99 µg/m³, P = 0.0029). A significant downward trend in PM₁₀ levels was observed over this period (slope -0.735, P = 0.0286), whereas no significant changes were found for PM_2.5, O₃, or NO₂. PM₁₀ was the only pollutant significantly associated with RCVS onset across lag days (Lag 0: OR 0.747, 95% CI: 0.579-0.962, P = 0.024; Lag 1: OR 0.770, 95% CI: 0.599-0.989, P = 0.041; Lag 2: OR 0.776, 95% CI: 0.604-0.997, P = 0.047; Lag 3: OR 0.768, 95% CI: 0.599-0.984, P = 0.037). After adjusting for atmospheric pressure and temperature, these associations were no longer statistically significant.</p><p><strong>Conclusion: </strong>This study suggests a short-term decrease in PM₁₀ levels prior to RCVS onset and a negative association between PM₁₀ concentration and occurrence. These findings contribute to understanding the potential environmental triggers of RCVS in the context of changing air quality and climate.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 9","pages":"589"},"PeriodicalIF":4.6,"publicationDate":"2025-08-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144957805","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":"Whole genome sequencing analysis in primary lateral sclerosis (PLS) patients reveals mutations in neurological diseases-causing genes.","authors":"Arianna Manini, Alberto Brusati, Maurizio Grassano, Giulia Scacciatella, Silvia Peverelli, Jacopo Spagliardi, Viviana Pensato, Alberto Doretti, Rosario Vasta, Umberto Manera, Antonio Canosa, Maura Brunetti, Davide Gentilini, Stefano Messina, Federico Verde, Cristina Moglia, Claudia Morelli, Eleonora Dalla Bella, Pamela J Keagle, John E Landers, Cinzia Gellera, Giuseppe Lauria Pinter, Adriano Chiò, Antonia Ratti, Andrea Calvo, Vincenzo Silani, Nicola Ticozzi","doi":"10.1007/s00415-025-13328-1","DOIUrl":"https://doi.org/10.1007/s00415-025-13328-1","url":null,"abstract":"<p><strong>Background: </strong>Primary Lateral Sclerosis (PLS) is a rare, adult-onset neurodegenerative disease that predominantly affects upper motor neurons. Despite being considered mostly sporadic, familial cases and rare genetic variants in genes associated with amyotrophic lateral sclerosis, hereditary spastic paraplegia and other neurological disorders have been reported in some PLS cases. Due to its rare prevalence among general population, large genetic studies of PLS are lacking.</p><p><strong>Methods: </strong>Fifty patients diagnosed with PLS based on consensus criteria were enrolled between 2013 and 2022 for comprehensive phenotypic and genotypic analysis using whole genome sequencing. We analyzed rare single nucleotide variants (SNVs), deemed pathogenic, likely pathogenic or of uncertain significance (VUS) based on the American College of Medical Genetics and Genomics criteria, and repeat expansions (REs) exceeding the pathogenic threshold, in 290 genes involved in neurological disorders.</p><p><strong>Results: </strong>We identified mutations in 7 patients (13.7%), specifically SNVs in CAPN1 (Spastic paraplegia 76), TBK1 (amyotrophic lateral sclerosis/frontotemporal dementia, ALS4/FTD), LITAF (Charcot-Marie-Tooth disease 1C), POLG (chronic progressive external ophthalmoplegia), APP (Alzheimer's disease) and OPTN (ALS12 ± FTD), and one RE in ATXN8OS (spinocerebellar ataxia 8). Additionally, two VUS were found in ANTXR2, a candidate gene for PLS recently identified via truncating variant collapsing analysis, but none of them was loss-of-function (one synonymous and one in-frame insertion).</p><p><strong>Conclusions: </strong>Our study demonstrates a notable genetic intersection between PLS and various neurological disorders, including motor neuron diseases, neuropathies, mitochondrial disorders, ataxias, and dementias. These findings underscore the relevance of further investigation in larger cohorts to fully elucidate PLS genetic architecture and highlight the need to reconsider the role of genetic testing in its diagnostic criteria.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 9","pages":"587"},"PeriodicalIF":4.6,"publicationDate":"2025-08-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144957854","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":"The HTT gene influences plasma neurofilament light chain and brain metabolism in prodromal Alzheimer's disease.","authors":"Salvatore Mazzeo, Chiara Crucitti, Michael Lassi, Assunta Ingannato, Valentina Berti, Matilde Nerattini, Giulia Giacomucci, Silvia Bagnoli, Valentina Moschini, Carmen Morinelli, Sonia Padiglioni, Giulia Galdo, Filippo Emiliani, Maria Salsone, Massimo Filippi, Sandro Sorbi, Alberto Mazzoni, Valentina Bessi, Benedetta Nacmias","doi":"10.1007/s00415-025-13312-9","DOIUrl":"https://doi.org/10.1007/s00415-025-13312-9","url":null,"abstract":"<p><strong>Objective: </strong>HTT, encoding a protein involved in axonal trafficking, contains a key region of CAG repeats. When expanded beyond 39 repeats, this region leads to Huntington's disease (HD). However, several studies have suggested that increasing the number of CAG repeats below the pathological threshold may confer functional advantages by enhancing HTT activity. In the present study, we aim to investigate the association between CAG repeat length below the pathological threshold and neurodegeneration biomarkers in prodromal Alzheimer's disease (AD).</p><p><strong>Methods: </strong>Ninety-five patients (36 with SCD and 59 with MCI) underwent blood collection for NfL measurement and HTT genetic analysis. Cerebrospinal fluid was collected for the measurement of Aβ₄₂, Aβ₄₀, total tau, and phosphorylated tau, and/or amyloid PET imaging was performed. Thirty-nine patients who were positive for both Aβ and phosphorylated tau biomarkers were classified as \"A+/T+\", while 56 patients who were either negative for both markers or positive for only one were classified as \"isolated Aβ/non-AD.\"</p><p><strong>Results: </strong>In the A+/T+ group, quadratic models described the association between CAG repeat length with NfL concentrations and 18F-FDG uptake. In particular, a concave curve was observed in the medial and middle frontal gyri, while a convex curve was found in the parahippocampal and fusiform gyri.</p><p><strong>Interpretation: </strong>Among individuals with SCD and MCI who show evidence of AD pathology, CAG repeat length in the HTT gene below the HD pathological threshold is associated with biomarkers of neurodegeneration in a region-specific and U-shaped manner. These findings suggest a modulatory role of HTT in prodromal AD.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 9","pages":"588"},"PeriodicalIF":4.6,"publicationDate":"2025-08-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12373535/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144957824","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":"AI-Based EMG Reporting: A Randomized Controlled Trial.","authors":"Alon Gorenshtein, Yana Weisblat, Mohamed Khateb, Gilad Kenan, Irina Tsirkin, Galina Fayn, Semion Geller, Shahar Shelly","doi":"10.1007/s00415-025-13261-3","DOIUrl":"https://doi.org/10.1007/s00415-025-13261-3","url":null,"abstract":"<p><strong>Background and objectives: </strong>Accurate interpretation of electrodiagnostic (EDX) studies is essential for the diagnosis and management of neuromuscular disorders. Artificial intelligence (AI) based tools may improve consistency and quality of EDX reporting and reduce workload. The aim of this study is to evaluate the performance of an AI-assisted, multi-agent framework (INSPIRE) in comparison with standard physician interpretation in a randomized controlled trial (RCT).</p><p><strong>Methods: </strong>We prospectively enrolled 200 patients (out of 363 assessed for eligibility) referred for EDX. Patients were randomly assigned to either a control group (physician-only interpretation) or an intervention group (physician-AI interpretation). Three board-certified physicians, rotated across both arms. In the intervention group, an AI-generated preliminary report was combined with the physician's independent findings (human-AI integration). The primary outcome was EDX report quality using score we developed named AI-Generated EMG Report Score (AIGERS; score range, 0-1, with higher scores indicating more accurate or complete reports). Secondary outcomes included a physician-reported AI integration rating score (PAIR) and a compliance survey evaluating ease of AI adoption.</p><p><strong>Results: </strong>Of the 200 enrolled patients, 100 were allocated to AI-assisted interpretation and 100 to physician-only reporting. While AI-generated preliminary reports offered moderate consistency on the AIGERS metric, the integrated (physician-AI) approach did not significantly outperform physician-only methods. Despite some anecdotal advantages such as efficiency in suggesting standardized terminology quantitatively, the AIGERS scores for physician-AI integration was nearly the same as those in the physician-only arm and did not reach statistical significance (p > 0.05 for all comparisons). Physicians reported variable acceptance of AI suggestions, expressing concerns about the interpretability of AI outputs and workflow interruptions. Physician-AI collaboration scores showed moderate trust in the AI's suggestions (mean 3.7/5) but rated efficiency (2.0/5), ease of use (1.7/5), and workload reduction (1.7/5) as poor, indicating usability challenges and workflow interruptions.</p><p><strong>Discussion: </strong>In this single-center, randomized trial, AI-assisted EDX interpretation did not demonstrate a significant advantage over conventional physician-only interpretation. Nevertheless, the AI framework may help reduce workload and documentation burdens by handling simpler, routine EDX tests freeing physicians to focus on more complex cases that require greater expertise.</p><p><strong>Trial registration: </strong>ClinicalTrials.gov Identifier: NCT06902675.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 9","pages":"586"},"PeriodicalIF":4.6,"publicationDate":"2025-08-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12373542/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144957729","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":"Paramagnetic rim lesions are associated with choroid plexus inflammation and expansion over 5 years in people with multiple sclerosis.","authors":"Jack A Reeves, Ashley Tranquille, Alexander Bartnik, Maryam Mohebbi, Fahad Salman, Dejan Jakimovski, Ferdinand Schweser, Bianca Weinstock-Guttman, Michael G Dwyer, Eleonora Tavazzi, Robert Zivadinov, Niels Bergsland","doi":"10.1007/s00415-025-13309-4","DOIUrl":"https://doi.org/10.1007/s00415-025-13309-4","url":null,"abstract":"<p><strong>Background: </strong>Chronic active white matter inflammation is linked with multiple sclerosis (MS) clinical severity and is likely integral in MS progression. It has also been associated with choroid plexus (CP) inflammation in vivo, pointing to a potential pathophysiological link between the two phenomena. However, how these aspects of the disease co-evolve over time remains poorly understood nor has their relationship been specifically assessed in people with progressive MS (pwPMS).</p><p><strong>Methods: </strong>In this retrospective analysis of a longitudinal study, 129 people with MS (pwMS; 86 people with relapsing remitting (pwRRMS) and 43 pwPMS) were imaged with 3 T MRI at baseline and after 5.4 years of follow-up. CP volume and CP pseudo-T2 (pT2) were calculated as measures reflecting CP inflammation in MS. Paramagnetic rim lesions (PRLs), a marker of chronic active white matter inflammation, were assessed at baseline for the whole cohort, and longitudinally in 96 pwMS with available data (66 pwRRMS, 32 pwPMS). Baseline and longitudinal associations between the CP and PRLs, including interactions with disease course, were assessed, adjusted for covariates including age and sex.</p><p><strong>Results: </strong>PwMS with PRLs had significantly larger CP volume at baseline than pwMS without PRLs after correcting for age, sex, and disease duration (mean difference: 0.3 ± 1.2 mL; p = 0.042). Longitudinally, baseline PRL number was associated with increased CP volume in pwPMS (B = 0.22 mL/mL; p < 0.001), but not people with relapsing-remitting MS (B = - 0.01 mL/mL; p > 0.7).</p><p><strong>Conclusion: </strong>Increased CP volume is related to chronic white matter inflammation, particularly in pwPMS.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 9","pages":"585"},"PeriodicalIF":4.6,"publicationDate":"2025-08-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12370838/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144957880","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}