Neurological Sciences最新文献

{"title":"Review of atypical optic neuritis.","authors":"Lepša Žorić, Emina Čolak","doi":"10.1007/s10072-024-07895-w","DOIUrl":"10.1007/s10072-024-07895-w","url":null,"abstract":"<p><p>Optic neuritis (ON), an inflammatory optic neuropathy, is among the most common causes of visual loss. In its initial clinical appearance, ON may have unilateral or bilateral presentation, and anterior (papillitis) or retrobulbar localization. Traditionally, cases are divided into typical and atypical ON. In the Western hemisphere, most typical cases of optic nerve inflammation are associated with multiple sclerosis (MS). However, ON may also be associated with a series of disorders of known or initially undetected origin. Atypical ON has a somewhat different clinical picture from typical ON, and encompasses neuromyelitis optica spectrum disease (NMOSD), myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), idiopathic recurrent neuroretinitis (NR), chronic relapsing inflammatory ON (CRION), ON within systemic autoimmune diseases, paraneoplastic and neuritis during or after infectious diseases or vaccination. The causes should be meticulously worked up, to address the therapeutic and prognostic challenges posed by these conditions. Here, we provide a brief overview of atypical ON, as encountered in our clinical practice, and additionally discuss the possible occurrence of optic neuropathies other than inflammatory and other ocular diseases within these disorders.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":"1555-1564"},"PeriodicalIF":2.7,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11920306/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142847055","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Pons metabolite alterations in narcolepsy type 1.","authors":"Micaela Mitolo, Fabio Pizza, David Neil Manners, Lucia Guidi, Annalena Venneri, Luca Morandi, Caterina Tonon, Giuseppe Plazzi, Raffaele Lodi","doi":"10.1007/s10072-025-08009-w","DOIUrl":"10.1007/s10072-025-08009-w","url":null,"abstract":"<p><strong>Introduction: </strong>Narcolepsy type 1 (NT1) is a rare central sleep disorder characterized by a selective loss of hypocretin/orexin (hcrt)-producing neurons in the postero-lateral hypothalamus that project to widespread areas of the brain and brainstem. The aim of this study was to explore in a group of NT1 patients the metabolic alterations in the pons and their associations with disease features.</p><p><strong>Methods: </strong>Twenty-one NT1 patients (16 M) and twenty age-matched healthy controls (10 M) underwent a brain ¹H MRS on a 1.5 T GE Medical Systems scanner. Metabolite content of N-acetyl-aspartate (NAA), choline (Cho), and myo-inositol (mI) were estimated relative to creatine (Cr), using LCModel 6.3. Clinical data were also collected with validated questionnaires, polysomnography, the Multiple Sleep Latency Test (MSLT), Cerebrospinal fluid hypocretin-1 (CSF hcrt-1) concentration and genetic markers.</p><p><strong>Results: </strong>NT1 patients compared with healthy controls showed lower NAA/Cr ratio (p = 0.007) and NAA/mI ratio (p = 0.011) in the pons. The Epworth Sleepiness Scale score showed a significant negative correlation with NAA/Cr content (p = 0.023), MSLT sleep latency a negative correlation with the mI/Cr ratio (p = 0.008), and sleep onset REM periods a positive correlation with the mI/Cr ratio (p = 0.027). CSF hcrt-1 levels were positively correlated with the NAA/Cr ratio (p = 0.039) and negatively with the mI/Cr ratio (p = 0.045) and the Cho/Cr ratio (p = 0.026).</p><p><strong>Conclusion: </strong>The metabolic alterations found in the pons of NT1 patients using the MR Spectroscopy technique were associated with subjective and objective disease severity measures, highlighting the crucial role of this biomarker in the pathophysiology of the disease.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":"1905-1909"},"PeriodicalIF":2.7,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11920375/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143414694","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Normative values of the brief international cognitive assessment for multiple sclerosis (BICAMS) in an Italian young adolescent population: the influence of age, sex, and education.","authors":"Fabrizia Falco, Federica Lamagna, Martina Eliano, Cristina di Monaco, Luigi Trojano, Giacomo Lus, Marcello Moccia, Francesca Lauro, Tiziana Liccardo, Alessandro Chiodi, Antonio Carotenuto, Vincenzo Brescia Morra, Roberta Lanzillo","doi":"10.1007/s10072-024-07900-2","DOIUrl":"10.1007/s10072-024-07900-2","url":null,"abstract":"<p><strong>Background: </strong>The Brief International Cognitive Assessment for Multiple Sclerosis (BICAMS) is the most widely used in clinical practice and the least time-consuming battery to estimate cognitive function in adults with Multiple Sclerosis (MS), while it has been included in few studies on young MS, also because of the absence of normative values.</p><p><strong>Objective: </strong>The aim of this study is to evaluate the impact of age, sex and education on BICAMS scores in a young adolescent population.</p><p><strong>Methods: </strong>We administered the BICAMS to 169, 11-to-18-year-old, healthy subjects. Linear regression models were used to assess the impact of age, sex, and education on sub-test scores. When statistically significant (p < 0.05), we used the regression coefficient to correct the raw scores.</p><p><strong>Results: </strong>younger age was associated with worse performance on SDMT (β = 1.76; p < 0.05), CVLT-II (β = 3.33; p < 0.05) and BVMT-R (β = 0.62; p < 0.05). Female sex was associated SDMT (β = 2.75 (p < 0.05) and CVLT-II (β = 2.51 (p < 0.05). Educational attainment was associated with better performance on SDMT (β = 1.79 (p = < 0.05) and BVMT-R (β = 0.61; p < 0.05). Cut-off points were suggested at the 5th lowest percentile.</p><p><strong>Conclusion: </strong>Age, sex, and education must be accounted for when applying the BICAMS to young population. Its use in everyday assessment of patients with Pediatric Onset Multiple Sclerosis (POMS) could help to compare and combine data across centers, identifying patients requiring a comprehensive evaluation and ad hoc cognitive stimulation programs.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":"1777-1782"},"PeriodicalIF":2.7,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142739918","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":"Nanomedicine in the treatment of Alzheimer's disease: bypassing the blood-brain barrier with cutting-edge nanotechnology.","authors":"Nana Dong, Payam Ali-Khiavi, Nima Ghavamikia, SeyedAbbas Pakmehr, Farzaneh Sotoudegan, Ahmed Hjazi, Morad Kohandel Gargari, Homa Kohandel Gargari, Parisa Behnamrad, Mohammadreza Rajabi, Anis Elhami, Hossein Saffarfar, Mehrdad Nourizadeh","doi":"10.1007/s10072-024-07871-4","DOIUrl":"10.1007/s10072-024-07871-4","url":null,"abstract":"<p><p>Alzheimer's disease (AD) remains a formidable challenge in the field of neurodegenerative disorders, necessitating innovative therapeutic strategies. Nanomedicine, leveraging nanomaterials, has emerged as a promising avenue for AD treatment, with a key emphasis on overcoming the blood-brain barrier (BBB) to enhance drug delivery efficiency. This review provides a comprehensive analysis of recent advancements in the application of nanomaterials for AD therapy, highlighting their unique properties and functions. The blood-brain barrier, a complex physiological barrier, poses a significant hurdle for traditional drug delivery to the brain. Nanomedicine addresses this challenge by utilizing various nanomaterials such as liposomes, polymeric nanoparticles, and metal nanoparticles. These nanocarriers enable improved drug bioavailability, sustained release, and targeted delivery to specific brain regions affected by AD pathology. The review discusses the diverse range of nanomaterials employed in AD treatment, exploring their capacity to encapsulate therapeutic agents, modulate drug release kinetics, and enhance drug stability. Additionally, the multifunctionality of nanomaterials allows for simultaneous imaging and therapy, facilitating early diagnosis and intervention. Key aspects covered include the interaction of nanomaterials with Aβ aggregates, the role of antioxidants in mitigating oxidative stress, and the potential of nanomedicine in alleviating neuroinflammation associated with AD. Furthermore, the safety, biocompatibility, and toxicity profiles of various nanomaterials are scrutinized to ensure their clinical applicability. In conclusion, this review underscores the pivotal role of nanomedicine and nanomaterials in revolutionizing AD treatment strategies. By specifically addressing BBB challenges, these innovative approaches offer new avenues for targeted drug delivery and improved therapeutic outcomes in the complex landscape of Alzheimer's disease.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":"1489-1507"},"PeriodicalIF":2.7,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142786324","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":"Anti-JCV antibody index seroconversion in Turkish multiple sclerosis patients treated with natalizumab.","authors":"Edanur Şahin, Tuncay Gündüz, Ahmed Serkan Emekli, Mazlum Ercanoğlu, Sevda Öztürk Erden, Murat Kürtüncü","doi":"10.1007/s10072-024-07929-3","DOIUrl":"10.1007/s10072-024-07929-3","url":null,"abstract":"<p><strong>Background: </strong>The anti-JCV antibody index is widely used to monitor multiple sclerosis (MS) patients receiving natalizumab, as seroconversion is linked to an increased risk of progressive multifocal leukoencephalopathy. This study aimed to evaluate the prevalence and risk factors of anti-JCV antibody seroconversion in patients treated with natalizumab.</p><p><strong>Methods: </strong>We included MS patients exposed to natalizumab treatment for at least one year, with a negative anti-JCV antibody index at baseline, and a minimum of two anti-JCV antibody assessments more than six months apart. We employed Kaplan-Meier survival analysis to assess the median time to seroconversion and the annual seroconversion rate, and univariate and multivariate Cox regression models to evaluate the covariates.</p><p><strong>Results: </strong>Among 96 patients followed for a median of 99 months, 29 (30.2%) patients had seroconversion. The median time to seroconversion was 8.3 years, with an annual rate of 6.1%. Seroconversion rates were higher in smokers (p = 0.02) and patients with a body mass index (BMI) over 25 kg/m² (p = 0.006). Patients who started natalizumab at age 35 or older had a shorter median time to seroconversion (p = 0.003), and most seroconversions occurred within the first three years. No significant associations were found with gender, prior immunosuppressive treatment, MS subtype, or MS age of onset.</p><p><strong>Conclusion: </strong>Anti-JCV seroconversion is more likely in patients who smoke, have a higher BMI, start natalizumab therapy after age 35, and within the first three years of treatment. For these high-risk patients, vigilant monitoring of anti-JCV antibodies is required.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":"1799-1805"},"PeriodicalIF":2.7,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142818684","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":"A randomized controlled trial investigating the impact of early goal-directed sedation dominated by dexmedetomidine on cerebral oxygen metabolism and inflammatory mediators in patients with severe brain injury.","authors":"Shitao Lu, Haiying Song, Yuxin Lin, Bo Song, Sheng Lin","doi":"10.1007/s10072-024-07916-8","DOIUrl":"10.1007/s10072-024-07916-8","url":null,"abstract":"<p><strong>Objective: </strong>The aim of this study is to assess the neuroprotective efficacy of early goal-directed sedation (EGDS) primarily governed by dexmedetomidine in patients experiencing severe traumatic brain injury, and to elucidate its potential underlying mechanisms.</p><p><strong>Data and methods: </strong>All participants were randomly allocated into two groups: the experimental group-dexmedetomidine-dominated EGDS group (group D, n = 30) and the control group-the standard propofol sedation group (group P, n = 30). Patients in the experimental group received sedation primarily with dexmedetomidine, while those in the control group received propofol sedation. Subsequently, retrograde catheterization of the internal jugular vein on the affected side was performed, blood gas analysis samples were collected, cerebral oxygen extraction rates were computed, and levels of interleukin 6 (IL-6) and interleukin 1β (IL-1β) were assessed. One-way ANOVA and Chi-square tests were used for statistical analysis.</p><p><strong>Results: </strong>In group D, significant reductions were observed in the duration of ventilator dependency (p < 0.05).Compared to those documented in group P, tracheostomy incidence, and pulmonary infection rates were no different (p > 0.05). On the second, third and the seventh day, the SjvO2 levels in group D exhibited a statistically significant elevation compared to group P, while the CERO₂ levels were notably lower in group D than in group P (p < 0.05). The GCS scores of patients in group D was significantly higher than that of the patients in group P and the baseline value on the seventh day and the time of discharge (p < 0.05). Additionally, the IL-6 levels in group D were significantly lower than those in group P and their corresponding baseline levels on the third and seventh days (p < 0.05). The IL-1β levels were no significant difference between the two groups.</p><p><strong>Conclusion: </strong>A predominance of dexmedetomidine in EGDS demonstrates efficacy in reducing the duration of ICU stay and ventilator dependency, enhancing cerebral oxygen metabolism, and attenuating the infiltration of inflammatory factors.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":"1741-1750"},"PeriodicalIF":2.7,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142822321","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":"AChR-blocking antibodies and complement system dynamics: evaluating their interplay and clinical implications in myasthenia gravis.","authors":"Florencia Aguirre, Mariano E Justo, Lucía Cialdella, Mariela L Paz","doi":"10.1007/s10072-024-07889-8","DOIUrl":"10.1007/s10072-024-07889-8","url":null,"abstract":"<p><strong>Background: </strong>Myasthenia gravis (MG) is an autoimmune disorder characterised by autoantibodies (abs) targeting proteins at the neuromuscular junction, primarily the acetylcholine receptor (AChR). While the role of AChR-binding abs is well-established, the pathogenicity and clinical relevance of AChR-blocking antibodies in MG, and their association with complement system, remain less understood.</p><p><strong>Aims: </strong>This study aims to provide comprehensive insights into the prevalence and interplay of AChR-blocking antibodies and the complement system in an Argentinian MG cohort, investigating their relationships with disease activity.</p><p><strong>Methods: </strong>We studied 75 MG patients with detectable AChR-binding abs, assessing the presence of AChR-blocking abs and complement components C3, C4, and C5a. We also examined clinical severity using the Activities of Daily Living and MG Composite scores. Correlation analyses were made to elucidate associations.</p><p><strong>Results: </strong>AChR-blocking abs were detected in 49.3% of the patients. An inverse correlation was found between AChR-blocking abs titres and disease severity, with a higher titre associated with milder symptoms. Complement analysis revealed higher C4 levels in the AChR-blocking abs positive group, indicating reduced complement activation.</p><p><strong>Conclusion: </strong>Our study provides valuable insights into the prevalence of AChR-blocking antibodies. Higher AChR-blocking abs titres were associated with less severe MG and reduced complement system activation, indicating a potential protective mechanism for those abs. These findings suggest that AChR-blocking abs could serve as a potential biomarker for a milder disease course and highlight the need for further research to understand their role in MG pathology, which will improve strategies for clinical management and diagnosis.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":"1827-1832"},"PeriodicalIF":2.7,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142829091","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":"Bradyarrhythmias in patients with embolic stroke of undetermined source: a tight relationship with atrial cardiomyopathy.","authors":"Alberto Comuzzi, Ilaria Armani, Sara De Giovanni, Ruggero Tomei, Cecilia Zivelonghi, Bruna Bolzan, Elena Franchi, Francesca Vassanelli, Sofia Capocci, Manuel Cappellari, Luca Tomasi, Flavio Luciano Ribichini, Giacomo Mugnai","doi":"10.1007/s10072-024-07959-x","DOIUrl":"10.1007/s10072-024-07959-x","url":null,"abstract":"<p><strong>Background: </strong>Implantable loop recorders (ILRs) have been shown to significantly improve the detection of atrial fibrillation (AF) in patients with embolic stroke of undetermined source (ESUS). The incidence and characterization of bradyarrhythmias in this subset of patients is still unknown.</p><p><strong>Methods: </strong>All consecutive patients who received ILRs, after an ESUS, between March 2015 and December 2022 in our Center were retrospectively enrolled and analyzed. Bradyarrhythmias were defined as: (1) sinus node dysfunction defined as sinus bradycardia, pause or arrest, exit block; (2) second-degree heart block or complete atrioventricular block. The primary endpoint was to analyze the incidence and characterization of bradyarrhythmias; the secondary endpoint was the detection of possible risk factors for bradyarrythmias. All patients were followed through the remote monitoring.</p><p><strong>Results: </strong>A total of 150 consecutive patients (mean age 70.4 ± 10.3 years old, 52.7% males) were analyzed. Bradyarrythmias were identified in 13 patients (8.7%). On multivariable analysis the presence of AF and first degree AV block were independently associated with the occurrence of bradyarrhythmias (respectively, OR 4.95, 95% CI 1.12-21.89, p = 0.0.03 and OR 3.77, 95% CI 1.08-13.14, p = 0.04).</p><p><strong>Conclusions: </strong>The incidence of bradyarrhythmias detected by ILRs in patients with ESUS was 8.7%. Atrial fibrillation, left atrial enlargement and first degree AV block due to prolonged P wave duration were associated with the occurrence of bradyarrhythmias during the follow up.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":"1707-1713"},"PeriodicalIF":2.7,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142872485","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":"Central hand syndrome.","authors":"Akiyuki Hiraga","doi":"10.1007/s10072-024-07913-x","DOIUrl":"10.1007/s10072-024-07913-x","url":null,"abstract":"","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":"1915-1916"},"PeriodicalIF":2.7,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142769906","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":"Case Report: Novel homozygous MAN1B1mutation in two Italians patients with Rafiq syndrome.","authors":"Maria Sofia Cotelli, Alessandro Draghi, Ilaria Bestetti, Maria Francesca Bedeschi, Barbara Borroni","doi":"10.1007/s10072-024-07893-y","DOIUrl":"10.1007/s10072-024-07893-y","url":null,"abstract":"","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":"1937-1939"},"PeriodicalIF":2.7,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142801839","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}