{"title":"Relapses during treatment with monoclonal antibodies targeting B-cells in NMOSD.","authors":"Shugang Cao, Xingyue Zhou, Jing Du, Juanjuan Zhang, Yunfei Zhu, Ling Wei, Mingwu Xia, Qi Li, Yin Xu, Qun Xue, Yanghua Tian","doi":"10.1007/s00415-025-13118-9","DOIUrl":"10.1007/s00415-025-13118-9","url":null,"abstract":"<p><strong>Introduction: </strong>While sustained B-cell depletion has proven effective in maintaining a relapse-free state in patients with neuromyelitis optica spectrum disorder (NMOSD), B-cell-depleting therapies do not demonstrate efficacy in all cases. This study aims to investigate the risk factors associated with relapses during B-cell-targeting monoclonal antibody (mAb) treatment in patients with NMOSD.</p><p><strong>Methods: </strong>We retrospectively analyzed baseline clinical characteristics, B-cell test results, and relapse data from NMOSD patients who received B-cell-targeting mAb treatment at four medical centers from July 2014 to September 2024. The annualized relapse rates (ARR) before and after mAb treatment were compared, and potential risk factors for relapses during mAb treatment were evaluated using a Cox proportional hazard model.</p><p><strong>Results: </strong>This study included 101 NMOSD patients, comprising 14 patients treated with inebilizumab (INEB) and 87 with rituximab (RTX). The patients' mean age at mAb initiation was 49 years (range: 10-74), with a median follow-up duration of 21.5 months (9.4, 37.6). During the study period, two patients were lost to follow-up, one died, and 17 experienced relapses, yielding a relapse-free rate of 83.2%. The ARR showed a significant reduction from 0.3 (0, 0.9) before mAb initiation to 0 (0, 0) after treatment (p = 0.002). Kaplan-Meier curve analysis demonstrated that patients with clustered attacks had a significantly higher risk of relapses compared to those without clustered attacks before mAb initiation (p < 0.001). Similarly, patients with EDSS scores ≥ 6 showed a higher relapse risk than those with EDSS scores < 6 (p = 0.006). Cox proportional hazard models identified several significant risk factors for relapses during mAb treatment, including the total number of attacks (including 2-year, 1-year, and clustered attacks), disease duration, ARR, and EDSS scores ≥ 6 before mAb initiation. Furthermore, patients who experienced relapses with CD19<sup>+</sup> B-cell levels < 1% demonstrated a significantly higher number of attacks 1 year before mAb initiation (p = 0.015) and a greater proportion of clustered attacks compared to those with CD19<sup>+</sup> B-cell levels ≥ 1% at relapse (p = 0.035).</p><p><strong>Conclusion: </strong>Attack frequency and cumulative disability before mAb initiation are important predictors for relapses during targeted B-cell mAb treatment for NMOSD. Future therapeutic strategies should focus on preventing clustered attacks to reduce the likelihood of mAb treatment failure.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 6","pages":"406"},"PeriodicalIF":4.8,"publicationDate":"2025-05-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144094067","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":"MoCA and MMSE for the detection of post-stroke cognitive impairment: a comparative diagnostic test accuracy systematic review and meta‑analysis.","authors":"Xiaoqin Wei, Yuxiang Liu, Jia Li, Ying Zhu, Wenyuan Li, Yu Zhu, Longchun Hua, Jianxun Cao, Yuxia Ma","doi":"10.1007/s00415-025-13146-5","DOIUrl":"10.1007/s00415-025-13146-5","url":null,"abstract":"<p><strong>Background and purpose: </strong>Post-stroke cognitive impairment (PSCI) is one of the serious complications of stroke, which profoundly influences the quality of life of stroke survivors. The Montreal Cognitive Assessment (MoCA) and the Mini-Mental State Examination (MMSE) are the two cognitive screening tools most widely used in stroke settings. Previous studies investigated the diagnostic accuracy of MoCA and MMSE but yielded controversial results. We conducted this study to compare the diagnostic accuracy of MoCA with MMSE for PSCI.</p><p><strong>Methods: </strong>Embase, PubMed, CINAHL, Web of Science, and The Cochrane Library were searched until August 17, 2024 for diagnostic accuracy studies comparing MoCA and MMSE. Data extraction was performed by two independent researchers. Risk of bias and applicability assessment was evaluated by the Quality Assessment of Diagnostic Accuracy Studies-2 tool. Coupled forest plots and hierarchical summary receiver operating characteristic (hsROC) curves were created in R statistical software.</p><p><strong>Results: </strong>9 studies with 1,135 patients were included in this review. Most studies were at high risk of bias. MoCA displayed a pooled sensitivity of 0.80 (95% CI 0.72 to 0.86) and specificity of 0.79 (95% CI 0.71 to 0.85). MMSE displayed a sensitivity and specificity of 0.76 (95% CI 0.71 to 0.81) and 0.78 (95% CI 0.73 to 0.83), respectively. No difference was shown between these modalities (SEN p = 0.36, SPE p = 0.80).</p><p><strong>Conclusion: </strong>No difference was observed between MoCA and MMSE in the detection of PSCI. We recommend both screeners be considered for the detection of PSCI based on the purpose of the test and by other metrics, such as acceptability and feasibility. Although it should be noted MoCA and MMSE were cognitive screening tools in stroke settings and not a substitute for detailed clinical assessment.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 6","pages":"407"},"PeriodicalIF":4.8,"publicationDate":"2025-05-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144094065","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}
Zhouzhou Wang, Hongmei Tan, Wenjuan Huang, Yuxin Fan, Liang Wang, Lei Zhou, Jingzi ZhangBao, Chao Quan
{"title":"Fluctuations in serum aquaporin-4 antibody titers: the clinical significance in neuromyelitis optica spectrum disorder.","authors":"Zhouzhou Wang, Hongmei Tan, Wenjuan Huang, Yuxin Fan, Liang Wang, Lei Zhou, Jingzi ZhangBao, Chao Quan","doi":"10.1007/s00415-025-13137-6","DOIUrl":"https://doi.org/10.1007/s00415-025-13137-6","url":null,"abstract":"<p><strong>Objectives: </strong>To evaluate the relationship between aquaporin-4 immunoglobulin G (AQP4-IgG) titer dynamics and relapse in neuromyelitis optica spectrum disorder (NMOSD), compare AQP4-IgG dynamics across different maintenance therapy strategies, and identify factors associated with AQP4-IgG titer seroreversion to negativity.</p><p><strong>Methods: </strong>Altogether 171 patients with ≥ 2 serum AQP4-IgG tests by fixed cell-based assay 30 days apart and at least once positive were included. Their clinical and treatment data were reviewed.</p><p><strong>Results: </strong>Among the 171 NMOSD patients with a median disease duration of 81.3 months, 44 (25.7%) became AQP4-IgG seronegative, accompanied by a reduction in annualized relapse rate (0.16 vs. 0.00, P < 0.001). Decline in serum AQP4-IgG titers emerged as the protective factor against relapse (HR 0.53, 95% CI 0.36-0.79, P = 0.002). Patients receiving monoclonal antibodies (mostly B cell-depleting therapies) as their initial and sole therapy throughout disease demonstrated higher likelihood of AQP4-IgG seroreversion compared to those treated with non-specific immunosuppressants (HR 3.01, 95% CI 1.23-7.34, P = 0.016) or those started with immunosuppressants and later switched to monoclonal antibodies (HR 6.17, 95% CI 2.13-17.54, P < 0.001). Male (OR 3.95, 95% CI 1.35-11.63, P = 0.012), baseline AQP4-IgG titer (OR 0.71, 95% CI 0.55-0.92, P = 0.008) and monoclonal antibody treatment throughout disease (OR 4.07, 95% CI 1.45-11.40, P = 0.008) were independently associated with AQP4-IgG seroreversion.</p><p><strong>Interpretation: </strong>Serum AQP4-IgG titer is related with relapse risk. Early initiation of monoclonal antibodies shows a superior suppressive effect on AQP4 autoimmunity.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 6","pages":"403"},"PeriodicalIF":4.8,"publicationDate":"2025-05-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144086273","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}
Charlotte Bert-Marcaz, Étienne Fortanier, Antoine Briantais, Benoit Faucher, Rim Bourguiba, Laure Swiader, Nicolas Schleinitz, Giovanni Corazza, Rodolphe Jean, Adrien Bigot, Paola Marianetti-Guingel, Marie Kostine, Roderau Outh, Yannick Dieudonné, Estibaliz Lazaro, Guillaume Vial, Sylvain Palat, Simon Frachet, Sébastien De Almeida Chaves, Stéphane Vinzio, Karim Sacré, Marie Robert, Thilbault Comont, Jérémie Dion, Pierre Girardie, Valentin Lacombe, Vincent Langlois, Vincent Jachiet, Paul Decker, Thomas Moulinet, Sylvie Grosleron, Jonathan Broner, Philippe Guilpain, Maxime Samson, Benjamin Terrier, Sophie Georgin-Lavialle, Sharham Attarian, Arsène Mekinian, Emilien Delmont, Mikael Ebbo
{"title":"Correction: Neurological manifestations in patients with VEXAS syndrome.","authors":"Charlotte Bert-Marcaz, Étienne Fortanier, Antoine Briantais, Benoit Faucher, Rim Bourguiba, Laure Swiader, Nicolas Schleinitz, Giovanni Corazza, Rodolphe Jean, Adrien Bigot, Paola Marianetti-Guingel, Marie Kostine, Roderau Outh, Yannick Dieudonné, Estibaliz Lazaro, Guillaume Vial, Sylvain Palat, Simon Frachet, Sébastien De Almeida Chaves, Stéphane Vinzio, Karim Sacré, Marie Robert, Thilbault Comont, Jérémie Dion, Pierre Girardie, Valentin Lacombe, Vincent Langlois, Vincent Jachiet, Paul Decker, Thomas Moulinet, Sylvie Grosleron, Jonathan Broner, Philippe Guilpain, Maxime Samson, Benjamin Terrier, Sophie Georgin-Lavialle, Sharham Attarian, Arsène Mekinian, Emilien Delmont, Mikael Ebbo","doi":"10.1007/s00415-025-13047-7","DOIUrl":"10.1007/s00415-025-13047-7","url":null,"abstract":"","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 6","pages":"401"},"PeriodicalIF":4.8,"publicationDate":"2025-05-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144078519","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}
Benjamin A Levett, Avinash Chandra, Jessica Jiang, Nehzat Koohi, Dale Sharrad, Lucy B Core, Jeremy C S Johnson, Madison Tutton, Tim Green, Dona M P Jayakody, Jin-Tai Yu, Iracema Leroi, Charles R Marshall, Doris-Eva Bamiou, Chris J D Hardy, Jason D Warren
{"title":"Hearing impairment and dementia: cause, catalyst or consequence?","authors":"Benjamin A Levett, Avinash Chandra, Jessica Jiang, Nehzat Koohi, Dale Sharrad, Lucy B Core, Jeremy C S Johnson, Madison Tutton, Tim Green, Dona M P Jayakody, Jin-Tai Yu, Iracema Leroi, Charles R Marshall, Doris-Eva Bamiou, Chris J D Hardy, Jason D Warren","doi":"10.1007/s00415-025-13140-x","DOIUrl":"10.1007/s00415-025-13140-x","url":null,"abstract":"<p><p>The relationship between hearing impairment and dementia has attracted significant attention, the 2024 Lancet Commission report identifying hearing loss as the largest modifiable risk factor for dementia from mid-life. The nature of this linkage between dementia and hearing remains unclear and is likely to be complex. In principle, hearing impairment could cause (directly promote), catalyze (amplify) or be a consequence of neurodegenerative pathology and cognitive decline. Here we use this framework to examine different lines of evidence for the association between hearing impairment and dementia, and consider how this evidence speaks to potential mechanisms and treatment implications. We conclude by considering practical clinical implications for management of patients with hearing impairment and dementia, the potential role for central hearing tests as 'auditory biomarkers' of dementia, and the need for further collaborative and mechanistically motivated research in this area.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 6","pages":"402"},"PeriodicalIF":4.8,"publicationDate":"2025-05-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12084262/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144078524","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}
Mark S Freedman, Ahmed Abdelhak, Mohit K Bhutani, Jason Freeman, Sharmilee Gnanapavan, Salman Hussain, Sheshank Madiraju, Friedemann Paul
{"title":"The role of serum neurofilament light (sNfL) as a biomarker in multiple sclerosis: insights from a systematic review.","authors":"Mark S Freedman, Ahmed Abdelhak, Mohit K Bhutani, Jason Freeman, Sharmilee Gnanapavan, Salman Hussain, Sheshank Madiraju, Friedemann Paul","doi":"10.1007/s00415-025-13093-1","DOIUrl":"10.1007/s00415-025-13093-1","url":null,"abstract":"<p><strong>Objective: </strong>This systematic literature review (SLR) was conducted to explore the role of serum neurofilament light chain (sNfL) as a biomarker in multiple sclerosis (MS) disease management.</p><p><strong>Methods: </strong>The review was conducted in accordance with the recommendation laid by the Cochrane Handbook for Systematic Reviews. A comprehensive literature search was performed in key biomedical databases (EMBASE<sup>®</sup>, MEDLINE<sup>®</sup>, MEDLINE<sup>®</sup>-In-Process, and all Evidence-Based Medicine [EBM] Reviews databases) to retrieve studies reporting the association between sNfL and disease activity in patients with MS. Additional evidence was also identified through hand searching of key conference proceedings and gray literature.</p><p><strong>Results: </strong>Following review of 1831 records, 75 studies from 180 publications were included in the review. The studies included in the SLR consistently demonstrated an association between higher sNfL levels and an increased risk of future relapses within 2 years and MS disease progression. Higher levels of sNfL were also linked to an increased likelihood of experiencing gadolinium-enhancing T1 and T2 lesions. Patients with lower sNfL levels had a higher likelihood of achieving no evidence of disease activity status. Furthermore, an inverse correlation was observed between sNfL levels and cognitive impairment as assessed via the Symbol Digit Modalities Test performance and Timed 25-Foot Walk scores.</p><p><strong>Conclusion: </strong>This SLR demonstrates the significance of sNfL as a sensitive biomarker for monitoring MS progression. Convenient and reliable sNfL measurement could benefit routine clinical practice, providing clinicians with a simple and effective tool to monitor disease and treatment response.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 6","pages":"400"},"PeriodicalIF":4.8,"publicationDate":"2025-05-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12081536/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144078451","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}
Yoon Seob Kim, In Ja Shin, Don Gueu Park, Jung Han Yoon
{"title":"Hashimoto's encephalopathy with diffuse leukoencephalopathy: serial MRI and gait analysis.","authors":"Yoon Seob Kim, In Ja Shin, Don Gueu Park, Jung Han Yoon","doi":"10.1007/s00415-025-13132-x","DOIUrl":"https://doi.org/10.1007/s00415-025-13132-x","url":null,"abstract":"","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 6","pages":"399"},"PeriodicalIF":4.8,"publicationDate":"2025-05-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144078521","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}
Victor Soto-Insuga, David Conejo Moreno, Elena González-Alguacil, Angel Aledo Serrano, Virginia Navarro Abia, Anna Gretel Pinzón-Acevedo, Nuria Lamagrande Casanova, Anna Duat Rodríguez, Verónica Cantarín Extremera, Juan José García Peñas
{"title":"Fenfluramine: an effective treatment for developmental epileptic encephalopathies beyond Dravet and Lennox-Gastaut Syndromes.","authors":"Victor Soto-Insuga, David Conejo Moreno, Elena González-Alguacil, Angel Aledo Serrano, Virginia Navarro Abia, Anna Gretel Pinzón-Acevedo, Nuria Lamagrande Casanova, Anna Duat Rodríguez, Verónica Cantarín Extremera, Juan José García Peñas","doi":"10.1007/s00415-025-13135-8","DOIUrl":"10.1007/s00415-025-13135-8","url":null,"abstract":"<p><strong>Background: </strong>Fenfluramine (FFA) is an antiseizure medication (ASM) with effectiveness in Dravet Syndrome (DS) and Lennox-Gastaut syndrome (LGS), but unknown effectiveness in other developmental epileptic encephalopathies (DEEs).</p><p><strong>Methods: </strong>This multicenter, retrospective study evaluated the efficacy and tolerability of FFA in children with DS, LGS and other DEEs within clinical practice. Data were extracted from patients' charts before and up to 6 months after treatment.</p><p><strong>Results: </strong>Fifty-four patients (median age 10 years; 67% male) with DS (n = 17), LGS (n = 20), or other DEE (n = 17) were included. At three months following FFA treatment, the proportion of responders (≥ 50% reduction in seizure frequency) was significantly higher in patients with DS (94%) compared with LGS (50%; p = 0.003) and other DEEs (47%; p = 0.003). No significant difference in responder rates was observed between the LGS and other DEE groups. FFA efficacy was independent of dosage, concomitant ASMs, epilepsy duration, etiology, or specific comorbidities. FFA demonstrated effectiveness across all seizure types, with particular efficacy in tonic-clonic seizures. Responders experienced improvements in physician-assessed seizure intensity; 56-91% showed improvements in other Clinical Global Impression domains, including cognition, behavior, sleep, and seizure severity. Adverse events occurred in 56% of patients and were predominantly mild, with somnolence, anorexia, and irritability the most common. Treatment discontinuation due to AEs occurred in three patients (1 LGS, 2 other DEEs).</p><p><strong>Conclusion: </strong>FFA demonstrates effectiveness and tolerability in patients with DEEs in a real-world setting, and has potential as a broad-spectrum ASM, effective across a wide range of DEEs, seizure types, and patient profiles.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 6","pages":"397"},"PeriodicalIF":4.8,"publicationDate":"2025-05-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144023708","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}
Elena Rossini, Vincenzo Di Stefano, Raffaele Iorio, Francesco Habetswallner, Michelangelo Maestri, Claudia Vinciguerra, Elena Maria Pennisi, Giuseppe Di Martino, Nicasio Rini, Silvia Falso, Sofia Marini, Dario Ricciardi, Melania Guida, Stefania Morino, Matteo Garibaldi, Luca Leonardi, Demetrio Marando, Laura Tufano, Giovanni Antonini, Laura Fionda
{"title":"Ravulizumab for generalized Myasthenia Gravis: a multicenter real-life experience.","authors":"Elena Rossini, Vincenzo Di Stefano, Raffaele Iorio, Francesco Habetswallner, Michelangelo Maestri, Claudia Vinciguerra, Elena Maria Pennisi, Giuseppe Di Martino, Nicasio Rini, Silvia Falso, Sofia Marini, Dario Ricciardi, Melania Guida, Stefania Morino, Matteo Garibaldi, Luca Leonardi, Demetrio Marando, Laura Tufano, Giovanni Antonini, Laura Fionda","doi":"10.1007/s00415-025-13127-8","DOIUrl":"10.1007/s00415-025-13127-8","url":null,"abstract":"<p><strong>Introduction: </strong>Ravulizumab, a monoclonal antibody targeting C5, was recently approved for the treatment of anti-AChR positive generalized myasthenia gravis (gMG) patients. The objective of this study is to present the Italian multicenter real-world experience evaluating the safety and efficacy of ravulizumab in gMG within the context of the Expanded Early Access Program (EAP).</p><p><strong>Methods: </strong>We conducted a retrospective study in 7 gMG referral centres in Italy. Demographic and clinical characteristics were recorded at baseline and during follow-up through clinical scale changes including Myasthenia Gravis-Activities of Daily Living (MG-ADL), Quantitative Myasthenia Gravis (QMG) and Myasthenia Gravis Composite (MGC). Frequency of minimal symptom expression (MSE) and changes in concomitant medications were also evaluated.</p><p><strong>Results: </strong>Twenty-four gMG patients (10/24 females) aged between 24 and 82 years (Median 60.5, IQR 52.5-67.5), were included. Fifteen patients had undergone thymectomy, and 14 had a thymoma. Median follow-up duration was 26 weeks (range 10-74, IQR 26-42). MG-ADL and QMG scores showed a significant decrease with respect to baseline (p < 0.001). MSE was achieved by 37.5% patients at the last available follow-up. Tapering of prednisone daily dosage was possible in 76% of patients. Thymoma was significantly associated with QMG score reduction and the frequency of QMG responders at week 2 (p = 0.03). Three patients discontinued treatment. One patient experienced a myasthenic exacerbation and needed rescue therapy. Infectious adverse events were reported in 5/24 patients, and a Stevens-Johnson syndrome in one patient.</p><p><strong>Conclusions: </strong>Real-world data confirm the effectiveness, safety, and prednisone-sparing effect of ravulizumab in patients with gMG, especially in those with thymoma.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 6","pages":"396"},"PeriodicalIF":4.8,"publicationDate":"2025-05-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12078421/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144017706","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}
Daniela Taranu, Luisa T Balz, Jill Holbrook, Visal Tumani, Herbert Schreiber, Hayrettin Tumani, Ingo Uttner
{"title":"Cognitive impairment, mood, and fatigue in various multiple sclerosis subtypes: a one-year follow-up study.","authors":"Daniela Taranu, Luisa T Balz, Jill Holbrook, Visal Tumani, Herbert Schreiber, Hayrettin Tumani, Ingo Uttner","doi":"10.1007/s00415-025-13115-y","DOIUrl":"10.1007/s00415-025-13115-y","url":null,"abstract":"<p><strong>Background: </strong>Multiple sclerosis (MS) subtypes-relapsing-remitting (RRMS), secondary-progressive (SPMS), and primary-progressive (PPMS) - have been associated with distinct cognitive impairment profiles, with progressive subtypes, in contrast to RRMS, showing additional deficits in more widespread domains. Research has largely focused on RRMS, leaving SPMS and PPMS underexplored due to their lower prevalence and limited therapeutic targeting. Data on the interplay between cognitive impairment, mood, and fatigue over time are also scarce. This study examined cognition, fatigue, and psychopathology over a period of one year to identify subtype-specific impairments and progression trajectories.</p><p><strong>Methods: </strong>Sixty-six MS patients (22 each with RRMS, SPMS, and PPMS) and 22 healthy controls (HC) were assessed using neuropsychological tests for attention, memory, processing speed, working memory, fluency and visuospatial functions. Patient-reported outcomes for depression, anxiety, and fatigue were also collected. Analyses included correlations, within-group comparisons (paired t-tests), and between-group comparisons (ANOVAs/ANCOVAs).</p><p><strong>Results: </strong>Progressive MS subtypes exhibited more severe cognitive impairments, fatigue, and mood disturbances than RRMS. Over one year, treated RRMS patients improved in various cognitive domains, while PPMS patients showed gains only in visuospatial abilities. On the other hand, SPMS patients exhibited no significant changes, suggesting more pronounced cognitive deficits.</p><p><strong>Conclusions: </strong>Cognitive impairments differed significantly across MS subtypes. While RRMS patients improved over one year and PPMS patients showed selective gains in one domain, SPMS showed no significant changes, indicating reduced cognitive reserve. These between-group differences suggest different cognitive trajectories. The findings underscore the need for tailored, holistic interventions for different MS subtypes.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 6","pages":"398"},"PeriodicalIF":4.8,"publicationDate":"2025-05-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12078348/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143988687","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}