Neurological Sciences最新文献

{"title":"Risk factors for poor response to initial first-line immunotherapy and subsequent immunotherapy on prognosis in pediatric anti-NMDA receptor encephalitis.","authors":"Yuhang Li, Hanyu Luo, Zhiwei Yu, Jiaxin Yang, Yan Jiang, Xiujuan Li, Jiannan Ma, Li Jiang","doi":"10.1007/s10072-025-08210-x","DOIUrl":"https://doi.org/10.1007/s10072-025-08210-x","url":null,"abstract":"<p><strong>Objectives: </strong>To identify factors associated with poor response to initial first-line immunotherapy in pediatric patients with anti-NMDAR encephalitis.</p><p><strong>Methods: </strong>This monocentric prospective cohort study included pediatric anti-NMDAR encephalitis between January 2017 and December 2021. The modified Rankin Scale (mRS) score was used to assess neurological severity.</p><p><strong>Results: </strong>This study included 152 patients, 74 of them (48.7%) had a poor response to initial first-line immunotherapy. Decreased consciousness (p = 0.001, OR = 6.889), autonomic dysfunction/central hypoventilation (p = 0.003, OR = 4.704), speech dysfunction (p = 0.007, OR = 4.272), mRS score before immunotherapy > = 4 points (p < 0.001, OR = 10.968), and age < = 3 years (p = 0.044, OR = 5.169) significantly affected the response to initial first-line immunotherapy. At 12 months, the good responders demonstrated a significantly better outcome than did the poor responders (100% vs. 74.3%, p < 0.001), although the recurrence rate was comparable between the two group (11.5% vs. 14.9%, p = 0.544). Of the 74 poor responders, 45 patients underwent subsequent immunotherapy, and they exhibited a greater proportion of favorable outcomes compared to the group without subsequent immunotherapy at 12 months.</p><p><strong>Conclusion: </strong>Pediatric anti-NMDAR encephalitis with decreased consciousness, autonomic dysfunction/central hypoventilation, speech dysfunction, mRS score > = 4 points before immunotherapy, and age < = 3 years had a greater risk of poor response to initial first-line immunotherapy, and poorer prognosis. Subsequent immunotherapy can improve long-term prognosis for poor responders.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-05-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144079158","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Letter to the Editor regarding \"The effects of transcranial magnetic stimulation in motor symptoms of Parkinson's disease: an overview of systematic reviews with meta-analysis\".","authors":"Ehsan Alimohammadi","doi":"10.1007/s10072-025-08241-4","DOIUrl":"https://doi.org/10.1007/s10072-025-08241-4","url":null,"abstract":"","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-05-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144086538","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Low-dose rituximab for the treatment of anti-NMDAR encephalitis in children: a case series study.","authors":"Haiyan Wu, Fenfen Xu, Yuan Chen, Shanshan Ding, Wei Li, Qiuyue Li, Na Chen, Lin Tang","doi":"10.1007/s10072-025-08228-1","DOIUrl":"https://doi.org/10.1007/s10072-025-08228-1","url":null,"abstract":"<p><strong>Background and purpose: </strong>Rituximab is a widely used second-line immune drug for the treatment of anti-NMDAR encephalitis in patients who fail to improve approximately 2 weeks after the initiation of first-line therapies (corticosteroids, intravenous immunoglobulin, plasma exchange). The aim of this study was to investigate the efficacy and safety of low-dose rituximab in the treatment of autoimmune encephalitis in children.</p><p><strong>Methods: </strong>This case series included 6 hospitalized children without tumors with anti-NMDAR encephalitis from January 2018 to December 2020. These children were treated with low-dose rituximab (100 mg, once weekly, according to peripheral blood CD19 + B-cell levels, 3-4 cycles, until CD19 + B cells in peripheral blood were cleared) approximately 2 weeks after the initiation of 2 or more first-line immunotherapies.</p><p><strong>Results: </strong>After 3-4 cycles of low-dose rituximab (100 mg) treatment, the mRS score and CD19 + B-cell count decreased significantly, and the patients maintained stable neurological function (mRS ≤ 2). No adverse events resulting from infusion or other adverse reactions occurred in any patient.</p><p><strong>Conclusion: </strong>In children with autoimmune encephalitis without tumors for whom first-line immunotherapy is ineffective, low-dose rituximab effectively reduces peripheral blood CD19 + B-cell counts, improves the clinical symptoms of patients and reduces hospitalization expenses. RCTs with sufficient sample sizes are needed to firmly establish the value of low-dose rituximab therapy for treating anti-NMDAR encephalitis in children.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-05-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144078903","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Letter to the Editor: comment on article 'Fear of falling impairs spatiotemporal gait parameters, mobility, and quality of life in Parkinson's disease: a cross-sectional study'.","authors":"Bradley Lonergan, Benjamin H L Harris, Vincent E S Allott, Michael B Fertleman, Louis J Koizia","doi":"10.1007/s10072-025-08220-9","DOIUrl":"https://doi.org/10.1007/s10072-025-08220-9","url":null,"abstract":"","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-05-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144078829","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Exploring the relationship between dystonia and STN-DBS in Parkinson's disease: insights from a single-centre cohort.","authors":"Luigi G Remore, Delia Gagliardi, Linda Borellini, Alfonso Fasano, Valeria Lo Faso, Filippo Cogiamanian, Enrico Mailand, Gloria Valcamonica, Elena Pirola, Luigi Schisano, Antonella M Ampollini, Giulio A Bertani, Giorgio Fiore, Antonio D'Ammando, Leonardo Tariciotti, Giovanni Marfia, Stefania Elena Navone, Sergio Barbieri, Marco Locatelli","doi":"10.1007/s10072-025-08230-7","DOIUrl":"https://doi.org/10.1007/s10072-025-08230-7","url":null,"abstract":"<p><strong>Introduction: </strong>Motor side effects may emerge after deep brain stimulation (DBS) of the subthalamic nucleus (STN) in Parkinson's disease (PD) patients. Out of 60 PD patients, we observed 16 patients displaying de novo dystonic symptoms after the implantation and 11 dystonic PD patients without benefit from the stimulation. We hypothesized that a common neural pathway may cause dystonia in both conditions. Our study aims to investigate the clinical and connectivity substrates of dystonia after STN-DBS.</p><p><strong>Methods: </strong>We divided our cohort into four groups: 16 patients displaying dystonia after STN-DBS, 11 patients with previously known dystonia not improving after surgery, 14 patients with dystonic symptoms relieved by the stimulation and 19 controls who never experienced dystonia. MANOVA was used to compare clinical data and the distance of the active contact center from the STN border among the four groups. Finally, we reconstructed the \"sour\" spots for dystonic symptoms and the associated structural and functional connectivity using a Parkinsonian normative connectome.</p><p><strong>Results: </strong>De novo dystonic and not-improved dystonic patients had a statistically significant longer PD duration before surgery (p = 0.001) and a greater active contact-STN distance (p < 0.001). Moreover, the \"sour\" spots were similar in both groups and structural and functional connectivity profiles were associated with brain areas correlated with dystonia pathophysiology (cerebellum, midbrain, parietal and temporal cortices).</p><p><strong>Conclusions: </strong>We formulated a two-hit model for dystonia after STN-DBS: a clinical feature of Parkinsonian patients causes predisposing altered plasticity contributing to dystonic symptoms development when coupled with the stimulation of dystonia-related subcortical and cortical structures.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-05-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144078821","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Tele-assessment in limb-girdle muscular dystrophy: feasibility and reliability of patient-led asynchronous method.","authors":"Muhammed Baris, Halil Ibrahim Celik, Aynur Ayse Karaduman","doi":"10.1007/s10072-025-08225-4","DOIUrl":"https://doi.org/10.1007/s10072-025-08225-4","url":null,"abstract":"<p><strong>Objective: </strong>This study aimed to investigate the feasibility and reliability of the six assessment tools commonly used in limb-girdle muscular dystrophy (LGMD) for patient-led asynchronous method: Brooke Scale, Vignos Scale, Fatigue Severity Scale (FSS), Numeric Rating Scale for Pain (NRS-Pain), Modified Barthel Index (MBI), and Nottingham Health Profile (NHP).</p><p><strong>Methods: </strong>This study included 40 individuals with LGMD (55% female; mean age = 30.53 ± 9.68 years). The assessment tools used in the study were initially completed by the participants using the patient-led asynchronous method and one week later using the clinician-led synchronous method. Google Forms was utilized for the patient-led asynchronous method, and ZoomTM was used for the clinician-led synchronous method. Furthermore, a questionnaire created by the researchers was administered to assess the satisfaction and usefulness of the procedures of both tele-assessment methods.</p><p><strong>Results: </strong>There was no significant difference between the total scores of the six assessment tools obtained by both methods (p's > 0.05). Intraclass correlation coefficients (ICC) exhibited excellent reliability for the total scores of the FSS (ICC = 0.91), MBI (ICC = 0.92), and NHP (ICC = 0.87). Weighted kappa (κw) showed excellent reliability for the Brooke Scale (κw = 0.94) and Vignos Scale (κw = 0.94), and good reliability for the NRS-Pain (κw = 0.63). The questionnaire conducted on the satisfaction and usefulness of the procedure of tele-assessment methods showed significantly greater overall satisfaction with the clinician-led synchronous method (p = 0.009).</p><p><strong>Conclusion: </strong>In individuals with LGMD, the six assessment tools were feasible and reliable when utilized with the patient-led asynchronous method.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-05-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143995494","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Neurological findings in a cohort of adults with down syndrome.","authors":"Nicola Grotteschi, Magali Jane Rochat, Virginia Pollarini, Alessandro Ghezzo, Camilla Pellegrini, Giovanna Calandra-Buonaura, Raffaele Lodi, Caterina Tonon, Pietro Cortelli, Maria Giulia Bacalini, Gian Luca Pirazzoli, Luisa Sambati","doi":"10.1007/s10072-025-08195-7","DOIUrl":"https://doi.org/10.1007/s10072-025-08195-7","url":null,"abstract":"<p><p>The aim of this study is to describe the results of a comprehensive neurological assessment conducted on a cohort of 70 adults with Down syndrome (DS), aged 21 to 74 years, recruited in Bologna, Italy. Neurocognitive disorder (NcD) was identified in 28.6% of participants and showed a significant association with psychiatric disorders (p=0.03). Psychiatric conditions were present in 40% of the cohort, while Down Syndrome Regression Disorder (DSRD) was diagnosed in 7.1%. Transient loss of consciousness affected 28.6% of individuals. Epilepsy, observed in 7.1%, was significantly associated with NcD (p=0.02). Neurological examination revealed that stereotypic movements correlated with DSRD (p<0.01), tics with obsessive-compulsive disorders (p=0.01), and hypokinetic movement disorders with psychotic conditions (p=0.03). Additionally, nystagmus and cerebellar signs were significantly associated with elevated serum bilirubin levels (p<0.01). These findings underscore the high prevalence and complexity of neurological comorbidities in adults with DS, emphasizing the need for specialized, multidisciplinary care.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144020314","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Predictive factors for the progression of multiple sclerosis: a meta-analysis.","authors":"Yun Huang","doi":"10.1007/s10072-025-08142-6","DOIUrl":"https://doi.org/10.1007/s10072-025-08142-6","url":null,"abstract":"<p><strong>Introduction: </strong>This meta-analysis systematically explored predictive factors for the progression of multiple sclerosis (MS).</p><p><strong>Methods: </strong>Computerized searches were conducted in Pubmed, OVID, Web of science, Willey Library, and EMbase from inception to August 2023. Patients with MS were divided into the deterioration group, defined as those with an increase in the Expanded Disability Status Scale (EDSS) score of ≥ 1.0 for a baseline EDSS ≤ 5.5 or ≥ 0.5 for a baseline EDSS > 5.5 during the follow-up period, and the non-deterioration group, comprising patients who exhibited no significant changes in EDSS over the same period. This study analyzed the impact of specific predictors, including age at onset, sex (male), smoking status, disease duration, number of relapses, EDSS score, prodromal neurological symptoms, and the use of disease-modifying therapies (DMTs) on MS progression.</p><p><strong>Results: </strong>Of 3647 retrieved, 14 studies were finally included. Meta-analysis showed that sex (male) [odds ratio (OR) = 1.15, 95% confidence interval (CI): 1.05-1.26, P < 0.01], smoking [OR = 1.92, 95%CI: 1.41-2.60, P < 0.01], relapses frequency [OR = 1.05, 95%CI: 1.02-1.08, P < 0.01], EDSS score [OR = 1.54, 95%CI: 1.10-2.15, P = 0.01], and DMTs use [OR = 0.38, 95%CI: 0.26-0.55, P < 0.01] significant influenced MS progression, while age at onset, disease duration, and prodromal neurological symptoms were not significant.</p><p><strong>Conclusion: </strong>Sex (male), smoking, a higher number of relapses, EDSS score, and DMTs are predictors of MS deterioration. Hence, particular attention should be given to these risk factors when assessing MS deterioration.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144021640","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Augusto Tamburini (1848-1918) and Jean-Martin Charcot (1825-1893): the struggle for a scientific approach to Hysteria.","authors":"Francesco Brigo","doi":"10.1007/s10072-025-08235-2","DOIUrl":"https://doi.org/10.1007/s10072-025-08235-2","url":null,"abstract":"<p><p>This article explores the scientific relationship between Augusto Tamburini (1848-1918) and Jean-Martin Charcot (1825-1893) through a detailed analysis of archival documents. It also investigates Tamburini's conception of hypnotism based on the complex interplay between the Salpêtrière and Nancy schools of hypnotism, which is emblematic of Charcot's influence on Italian medicine at the turn of the nineteenth century. Tamburini, a leading Italian psychiatrist, was instrumental in modernizing asylum care, integrating experimental psychology into clinical practice, and advancing psychiatric research. We examine Tamburini's connection with Charcot and present two significant historical documents: the account of Charcot's 1881 visit to the San Lazzaro asylum, directed by Tamburini, and the obituary he wrote in 1893 to honor the French master. Charcot's visit reinforced San Lazzaro's prominence in psychiatric research, particularly in hysteria and hypnosis, and affirmed Tamburini's methodological alignment with the French neurologist. Both scholars utilized photography as a tool for psychiatric documentation, reflecting their commitment to empirical inquiry. Initially following Charcot's neuropathological model of hypnosis, Tamburini later proposed a more integrative theory, incorporating elements of the psychological perspective proposed by Hippolyte Bernheim (1840-1919). His evolving stance illustrated the broader scientific debates of the time while maintaining a strong foundation in experimental methods. Tamburini's obituary for Charcot emphasized his admiration for the French neurologist's scientific rigor and lasting contributions to neurology and psychiatry. Ultimately, Tamburini's work exemplifies how Italian psychiatry, while influenced by Charcot, established its own distinct and respected identity.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144042536","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Correction To: Felbamate as a therapeutic alternative to drug-resistant genetic generalized epilepsy: a systematic review and meta-analysis.","authors":"Yitao Ma, Matthew Kaminski, Robert Crutcher","doi":"10.1007/s10072-025-08229-0","DOIUrl":"https://doi.org/10.1007/s10072-025-08229-0","url":null,"abstract":"","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-05-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143973559","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}