Journal of Neurology最新文献

{"title":"Antibody-positive paraneoplastic neurological syndromes associated with immune checkpoint inhibitors: a systematic review.","authors":"Le Zhang, Siyuan Fan, Jiawei Wang, Haitao Ren, Hongzhi Guan","doi":"10.1007/s00415-025-12992-7","DOIUrl":"https://doi.org/10.1007/s00415-025-12992-7","url":null,"abstract":"<p><strong>Background and objectives: </strong>This study aimed to describe the clinical and prognostic characteristics of antibody-positive paraneoplastic neurological syndrome (PNS) associated with immune checkpoint inhibitors (ICIs).</p><p><strong>Methods: </strong>We conducted a systematic review of relevant publications in PubMed and Embase from inception to December 2023. Patients with positive anti-neuronal antibodies who had a definite, probable, or possible diagnosis of PNS based on the 2021 PNS-Care Score criteria were included.</p><p><strong>Results: </strong>A total of 76 records with 108 antibody-positive ICI-PNS patients were included in this systematic review. According to the updated 2021 criteria, 60.2% of patients were classified as definite PNS, 29.6% as probable PNS, and 10.2% as possible PNS. The median age was 66 years (range: 26-82), and 56.5% of patients were male. The most frequently associated tumors included lung cancer, melanoma, and Merkel cell carcinoma, and 72.2% of patients developed neurological symptoms within 6 months after ICIs treatment. The most common clinical phenotypes were limbic encephalitis (35.2%), rapidly progressive cerebellar syndrome (19.4%), and Lambert-Eaton myasthenic syndrome (13.0%), while the most common autoantibodies were anti-Hu (34.3%), anti-Ma2 (16.7%), and anti-P/Q VGCC (14.8%) antibodies. CSF inflammation was observed in 63.0% patients, predominantly lymphocytic. Corticosteroids were the mainstay of immunotherapy (90.9%), followed by intravenous immunoglobulin (IVIG) and plasma exchange. Outcome information was reported for 103 patients. The median follow-up was 4 months (IQR: 2, 10), and 56.3% of patients showed improvement, while 37.0% of patients died at the last follow-up. Patients with anti-Hu or  anti-Ma2 antibodies had a higher proportion of deterioration and mortality (P < 0.05).</p><p><strong>Conclusion: </strong>Limbic encephalitis and anti-Hu antibody are relatively common in antibody-positive ICI-PNS, and most patients present with CSF inflammation. Discontinuation of ICIs and corticosteroids are the main treatments. High-risk antibodies may be a risk factor for an unfavorable prognosis, particularly anti-Hu and anti-Ma2 antibodies.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 3","pages":"249"},"PeriodicalIF":4.8,"publicationDate":"2025-03-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143557224","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":"Separation of stroke from vestibular neuritis using the video head impulse test: machine learning models versus expert clinicians.","authors":"Chao Wang, Jeevan Sreerama, Benjamin Nham, Nicole Reid, Nese Ozalp, James O Thomas, Cecilia Cappelen-Smith, Zeljka Calic, Andrew P Bradshaw, Sally M Rosengren, Gülden Akdal, G Michael Halmagyi, Deborah A Black, David Burke, Mukesh Prasad, Gnana K Bharathy, Miriam S Welgampola","doi":"10.1007/s00415-025-12918-3","DOIUrl":"https://doi.org/10.1007/s00415-025-12918-3","url":null,"abstract":"<p><strong>Background: </strong>Acute vestibular syndrome usually represents either vestibular neuritis (VN), an innocuous viral illness, or posterior circulation stroke (PCS), a potentially life-threatening event. The video head impulse test (VHIT) is a quantitative measure of the vestibulo-ocular reflex that can distinguish between these two diagnoses. It can be rapidly performed at the bedside by any trained healthcare professional but requires interpretation by an expert clinician. We developed machine learning models to differentiate between PCS and VN using only the VHIT.</p><p><strong>Methods: </strong>We trained machine learning classification models using unedited head- and eye-velocity data from acute VHIT performed in an Emergency Room on patients presenting with acute vestibular syndrome and whose final diagnosis was VN or PCS. The models were validated using an independent test dataset collected at a second institution. We compared the performance of the models against expert clinicians as well as a widely used VHIT metric: the gain cutoff value.</p><p><strong>Results: </strong>The training and test datasets comprised 252 and 49 patients, respectively. In the test dataset, the best machine learning model identified VN with 87.8% (95% CI 77.6%-95.9%) accuracy. Model performance was not significantly different (p = 0.56) from that of blinded expert clinicians who achieved 85.7% accuracy (75.5%-93.9%) and was superior (p = 0.01) to that of the optimal gain cutoff value (75.5% accuracy (63.8%-85.7%)).</p><p><strong>Conclusion: </strong>Machine learning models can effectively differentiate PCS from VN using only VHIT data, with comparable accuracy to expert clinicians. They hold promise as a tool to assist Emergency Room clinicians evaluating patients with acute vestibular syndrome.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 3","pages":"248"},"PeriodicalIF":4.8,"publicationDate":"2025-03-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143557167","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":"Heterogeneity of cognitive progression and clinical predictors in Parkinson's disease-subjective cognitive decline.","authors":"Jon Rodríguez-Antigüedad, Saül Martínez-Horta, Arnau Puig-Davi, Andrea Horta-Barba, Javier Pagonabarraga, Teresa de Deus Fonticoba, Silvia Jesús, Marina Cosgaya, Juan García Caldentey, María Asunción Ávila-Rivera, Nuria Caballol, Inés Legarda, Jorge Hernández Vara, Iria Cabo, Lydia López Manzanares, Isabel González Aramburu, Víctor Gómez Mayordomo, Jessica González Ardura, Julio Dotor García-Soto, Carmen Borrué, Berta Solano Vila, María Álvarez Sauco, Lydia Vela, Sonia Escalante, Esther Cubo, Zebenzui Mendoza, Isabel Pareés, Pilar Sánchez Alonso, María G Alonso Losada, Nuria López Ariztegui, Itziar Gastón, Javier Ruíz Martínez, María Teresa Buongiorno, Carlos Ordás, Caridad Valero, Víctor Puente, Mónica Kurtis, Marta Blázquez Estrada, Pablo Martínez-Martín, Pablo Mir, Diego Santos-García, Jaime Kulisevsky","doi":"10.1007/s00415-024-12808-0","DOIUrl":"https://doi.org/10.1007/s00415-024-12808-0","url":null,"abstract":"","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 3","pages":"246"},"PeriodicalIF":4.8,"publicationDate":"2025-03-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143557243","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":"Overlapping presence of β-amyloid, tau, p-tau, and α-synuclein in skin nerve fibers in Alzheimer's disease.","authors":"Emilie Buchholz, Marie-Luise Machule, Maria Buthut, Leon Stefanovski, Rosa Rössling, Harald Prüss","doi":"10.1007/s00415-025-12994-5","DOIUrl":"https://doi.org/10.1007/s00415-025-12994-5","url":null,"abstract":"<p><strong>Objective: </strong>Skin nerve fiber deposition of proteins can be strongly associated with neurodegenerative diseases, such as phosphorylated α-synuclein (p-SN) in synucleinopathies. Little is known about other neurodegenerative proteins, such as tau or β-amyloid, in skin nerve fibers of patients with Alzheimer's disease (AD) and their link to underlying neurodegeneration. We therefore aimed for describing the presence and distribution of these proteins in the skin of patients with AD and non-AD controls.</p><p><strong>Methods: </strong>Skin biopsies were taken from 45 patients with AD (n = 23) and non-AD controls (n = 22). Nerve fibers were identified using antibodies against protein gene product 9.5 (PGP9.5), and protein deposits were evaluated with double-immunostaining of β-amyloid 1-42 (Aβ1-42), p-SN, tau, and phospho-tau (p-tau).</p><p><strong>Results: </strong>Skin nerve fiber Aβ1-42 was present in 7/23 (30.4%) patients with AD and 7/22 (31.8%) controls. p-tau was detected in 12/23 (52.2%) patients with AD and 9/22 (40.9%) controls. Tau was present in 19/23 (82.6%) patients with AD and 16/22 (72.7%) controls. p-SN was detected in 12/23 (52.2%) patients with AD and 8/22 (36.4%) controls. Frequencies of deposits were not significantly different between groups and protein frequency did not correlate with severity of cognitive impairment.</p><p><strong>Interpretation: </strong>Deposits of β-amyloid 1-42, p-SN, tau, and p-tau were detected in skin nerve fibers in both patient groups; however, qualitative assessment did not discriminate between AD and non-AD patients at this sample size. Future analyses of protein distribution and spreading in peripheral nerves may give new insights into the pathophysiology of neurodegenerative diseases, but may require quantitative detection.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 3","pages":"247"},"PeriodicalIF":4.8,"publicationDate":"2025-03-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143557248","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":"Ultralong-term EEG: a loop recorder for the brain.","authors":"Earshad Mia, Khalid Hamandi","doi":"10.1007/s00415-025-12985-6","DOIUrl":"https://doi.org/10.1007/s00415-025-12985-6","url":null,"abstract":"","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 3","pages":"245"},"PeriodicalIF":4.8,"publicationDate":"2025-03-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143542351","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":"Prevalence of infratentorial superficial siderosis in a large general population sample from the UK Biobank.","authors":"N Kharytaniuk, D Hikmat, H Ozkan, E A Lim, D E Bamiou, P Cowley, H R Jäger, D J Werring","doi":"10.1007/s00415-025-12965-w","DOIUrl":"https://doi.org/10.1007/s00415-025-12965-w","url":null,"abstract":"<p><strong>Background: </strong>Classical infratentorial superficial siderosis (iSS) is considered a rare but potentially disabling neurological disorder. It results from slow chronic blood extravasation into the cerebrospinal fluid and deposition of the iron degradation product hemosiderin within the subpial layers of the central nervous system. Susceptibility-weighted (SW) magnetic resonance imaging (MRI) is the reference diagnostic modality. Few studies have described the population prevalence of cerebellar or infratentorial siderosis, and there are none from the UK population. The aim of this cross-sectional observational study was to estimate the prevalence of iSS in the UK Biobank sample using pre-defined radiological criteria.</p><p><strong>Methods: </strong>We reviewed SW MRIs of participants from the UK Biobank, looking for the radiological features of classical iSS: involvement of infratentorial structures (superior vermis, cerebellar folia, brainstem, or craniocervical junction). We calculated the point prevalence as the number of identified cases per total number of cases reviewed and 95% confidence intervals (CI) using Wilson's Score formula.</p><p><strong>Results: </strong>Of 10,305 SW MRIs reviewed, five cases with radiological features of iSS were identified demonstrating cerebellar/superior vermis involvement. The estimated prevalence of iSS was calculated as 48.5 (95%CI 20.7-113.5) cases per 100,000 population.</p><p><strong>Conclusions: </strong>This is the first study to estimate iSS prevalence in the UK population. The prevalence of iSS is higher than that reported for other rare neurological and neuro-otological disorders, suggesting an important unmet healthcare need for early diagnosis and targeted management strategies. Further studies are needed to determine the clinical associations and prognostic significance of radiologically defined iSS in the general population.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 3","pages":"242"},"PeriodicalIF":4.8,"publicationDate":"2025-03-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143542345","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":"Deep characterization of females with heterozygous Duchenne muscular dystrophy mutations.","authors":"Pietro Riguzzi, Daniele Sabbatini, Aurora Fusto, Sara Vianello, Beatrice Merlo, Vittoria Zangaro, Giuliana Capece, Domenico Gorgoglione, Gianni Sorarù, Riccardo Bariani, Chiara Calore, Barbara Bauce, Marika Martini, Anna Mutterle, Luca Bello, Elena Pegoraro","doi":"10.1007/s00415-025-12987-4","DOIUrl":"https://doi.org/10.1007/s00415-025-12987-4","url":null,"abstract":"<p><strong>Objective: </strong>Duchenne muscular dystrophy (DMD) is an X-linked muscular dystrophy due to null mutations in the DMD gene that predominantly affects males, while heterozygous females are usually asymptomatic carriers. In approximately 10-20% of cases, they may present with muscle weakness and/or cardiomyopathy. We aimed to describe clinical and molecular characteristics of DMD heterozygous females.</p><p><strong>Methods: </strong>A monocentric, observational, and cross-sectional study was designed. Clinical and molecular data were collected along with, when available, muscle biopsies. The pattern of X inactivation was determined in peripheral blood and the genotypes at SPP1, LTBP4 and CD40 modifier genes were established.</p><p><strong>Results: </strong>We recruited 47 participants: 27 (57%) were asymptomatic and 20 (43%) manifested symptoms. Proximal muscles were prominently involved, as in male dystrophinopathies. Twenty % of carriers showed cardiac involvement. Creatine kinase (CK) values were in the normal range in ~ 20% of symptomatic and ~ 46% asymptomatic patients. In all muscle biopsies, a mosaic of dystrophin positive and negative fibers was observed that only marginally correlated to dystrophin amount. No correlation was found between X chromosome inactivation pattern and the severity of muscular involvement, nor any association with cardiomyopathy. No genotype-phenotype correlations were identified.</p><p><strong>Interpretation: </strong>Genotype/phenotype correlations in females heterozygous for DMD mutations are influenced by multiple mechanisms, of which better understanding will be crucial for future dystrophin gene replacement therapies. An earlier molecular identification is essential to lead to greater awareness of the potential cardiac complications, and hence the reinforcement of  appropriate cardiac follow-up.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 3","pages":"244"},"PeriodicalIF":4.8,"publicationDate":"2025-03-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143542341","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":"Predictors of achieving minimal manifestations or better status in ocular myasthenia gravis with immunotherapy.","authors":"Teppei Komatsu, Haruhiko Motegi, Masahiro Mimori, Motohiro Okumura, Marina Masui, Hiroyuki Kida, Masakazu Ozawa, Hiromasa Matsuno, Kenichi Sakuta, Keiko Bono, Kenichiro Sakai, Hidetaka Mitsumura, Yasuyuki Iguchi","doi":"10.1007/s00415-025-12993-6","DOIUrl":"https://doi.org/10.1007/s00415-025-12993-6","url":null,"abstract":"<p><strong>Background: </strong>Ocular myasthenia gravis (MG), while less severe than generalized MG, significantly impairs quality of life due to its impact on vision and daily activities. Immunotherapy is a key treatment, yet the optimal timing for initiation remains undefined.</p><p><strong>Objective: </strong>To identify predictive factors for achieving minimal manifestations (MM) or better status in ocular MG patients receiving immunotherapy, with a focus on the role of early intervention.</p><p><strong>Methods: </strong>We conducted a retrospective analysis of 48 ocular MG patients treated at our institution. Patients were categorized based on whether they achieved MM or better status. Clinical variables were analyzed using univariate, receiver-operating characteristic, and multivariate logistic regression analyses.</p><p><strong>Results: </strong>MM or better status was achieved in 34/48 patients (median onset age: 55 years). Time from symptom onset to immunotherapy initiation was significantly shorter in those who achieved MM or better status (median: 30 days vs. 1095 days; p < 0.001). Receiver-operating characteristic analysis determined a 255-day cutoff for immunotherapy initiation, yielding sensitivity of 92.9% and specificity of 91.2% (AUC = 0.986, 95% CI: 0.962-1.000, p < 0.001). Logistic regression confirmed early immunotherapy as an independent predictor.</p><p><strong>Conclusions: </strong>Early initiation of immunotherapy (< 255 days from onset) significantly improves the likelihood of achieving MM or better status in ocular MG. These findings support the need for prompt diagnosis and treatment to optimize outcomes.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 3","pages":"243"},"PeriodicalIF":4.8,"publicationDate":"2025-03-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143542343","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":"Tick-borne encephalitis in adults in Denmark: a nationwide prospective cohort study from 2015 to 2023.","authors":"Anna Maria Florescu, Thomas Bryrup, Carsten Schade Larsen, Lykke Larsen, Lothar Wiese, Hans Rudolf Lüttichau, Micha Phill Grønholm Jepsen, Birgitte Rønde Hansen, Christian Østergaard, Anja Vad Søndergaard, Peter H S Andersen, Lasse Skafte Vestergaard, Ria Lassaunière, Anders Fomsgaard, Bo Bødker Jensen, Jacob Bodilsen, Henrik Nielsen, Anne-Mette Lebech, Helene Mens","doi":"10.1007/s00415-025-12986-5","DOIUrl":"10.1007/s00415-025-12986-5","url":null,"abstract":"<p><strong>Background: </strong>Our aim was to characterize the clinical presentation and outcome in adults with tick-borne encephalitis (TBE) and to determine the incidence and test activity of TBE in Denmark.</p><p><strong>Methods: </strong>A nationwide prospective cohort study of all adults hospitalized with TBE at departments of infectious diseases in Denmark from 2015 to 2023. An age- and sex-matched cohort of herpes simplex virus type 1 (HSV-1) encephalitis patients was included to compare outcome.</p><p><strong>Results: </strong>Fifty-two patients with TBE were included. Median age was 50 years, 32/52 (62%) were men, 1/52 (2%) was fully vaccinated against TBE at the time of infection, 29/52 (56%) were infected in Denmark. Upon admission 25/52 (48%) had meningitis, 27/52 (52%) encephalitis, three of the latter 3/52 (6%) with additional myelitis or radiculitis. Admission to the intensive care unit 6/52 (12%) and death 2/52 (4%) were associated with pre-existing comorbidities and older age. At 3-month follow-up, 16/50 (32%) had an unfavorable outcome (Glasgow Outcome Scale score 1-4) compared to 39/52 (75%) in the HSV-1 cohort. The most common residual symptoms at 6-month follow-up or later were headache, cognitive impairment, and fatigue. The TBE incidence increased from 0.03/100,000 in 2015 to 0.48/100,000 in 2023, and the test rate from 5.5/100,000 in 2015 to 14.4/100,000 in 2023, with a positivity rate of 0.6% in 2015 and 3.3% in 2023.</p><p><strong>Conclusion: </strong>The incidence of TBE in Denmark increased in the study period, with clinical characteristics and outcome of adult patients comparable to reports from other European countries.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 3","pages":"241"},"PeriodicalIF":4.8,"publicationDate":"2025-03-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143542348","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":"Longitudinal analysis of retinal and choriocapillaris in patients with multiple sclerosis: a 3-year study.","authors":"Mihai Bostan, Chi Li, Jia Wei Cheong, Damon Wing Kee Wong, Bingyao Tan, Munirah Binte Ismail, Gerhard Garhöfer, Ruxandra Coroleuca, Leopold Schmetterer, Alina Popa-Cherecheanu, Jacqueline Chua","doi":"10.1007/s00415-025-12930-7","DOIUrl":"10.1007/s00415-025-12930-7","url":null,"abstract":"<p><strong>Objectives: </strong>This study investigated the longitudinal progression of retinal structure and microvasculature over 3 years in patients with relapsing-remitting multiple sclerosis (RRMS) using optical coherence tomography (OCT) and OCT angiography (OCTA). It also explored the correlation between these changes and the Expanded Disability Status Scale (EDSS) scores.</p><p><strong>Methods: </strong>In this prospective, longitudinal study, we enrolled 66 patients with RRMS without history of optic neuritis and 124 healthy controls. All participants underwent full ophthalmological examination, OCT/OCTA scans, and disability scoring (EDSS) at baseline and after 12 and 24 months. OCT data were analyzed for retinal layer thickness, while OCTA assessed microvascular perfusion in the retinal capillary plexuses and choriocapillaris. Statistical models evaluated yearly rates of change and their association with EDSS scores.</p><p><strong>Results: </strong>The patients with RRMS exhibited 3.6 times faster thinning of the inner plexiform layer (IPL; - 0.47 µm per year, P = 0.001) compared to controls over 3 years. Additionally, superficial retinal capillary layer perfusion density decreased more rapidly at - 0.44% per year (P = 0.006) in patients with MS. A strong correlation was found between worsening EDSS scores and accelerated ONL thinning (estimated coefficient: - 1.62 µm/per unit change of EDSS score, P = 0.004).</p><p><strong>Discussion: </strong>This study demonstrates progressive retinal neurodegeneration and microvascular dysfunction in patients with RRMS without a history of optic neuritis. The association between ONL thinning and increased disability supports the potential of OCT/OCTA as valuable tools for monitoring disease progression and severity in RRMS.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 3","pages":"237"},"PeriodicalIF":4.8,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11872746/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143537290","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}