Neurological Sciences最新文献

{"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}

{"title":"Neurologic features in hospitalized patients with COVID-19: a prospective cohort in a catalan hospital.","authors":"Oriol Barrachina-Esteve, A Anguita, A Reverter, J Espinosa, C Lafuente, M Rubio-Roy, M Crosas, C Vila-Sala, C Acero, M Navarro, D Cánovas, G Ribera, M Jodar, J Estela","doi":"10.1007/s10072-025-08031-y","DOIUrl":"10.1007/s10072-025-08031-y","url":null,"abstract":"<p><strong>Objectives: </strong>To study the prevalence and timing of neurological manifestations, including cognitive involvement, in patients hospitalized for Coronavirus disease 2019 (COVID-19). To analyze the pathogenic mechanisms and any association they have with disease severity.</p><p><strong>Methods: </strong>Longitudinal cohort study with prospective follow-up of patients who required hospitalization. Patients under 65 who had no pre-existing cognitive impairment and did not require an ICU stay were evaluated 3 and 12 months after discharge using a battery of neuropsychological tests.</p><p><strong>Results: </strong>Of 205 patients hospitalized for COVID-19, 153 (74.6%) presented with neurological manifestations. The most frequent were myalgia (32.7%), headache (31.7%), dysgeusia (29.2%), and anosmia (24.9%). Patients with more severe illness at the time of hospitalization presented fewer neurological manifestations. Of the 62 patients who underwent neuropsychological examination 3 months after discharge, 22.6% had impaired attention, 19.4% impaired working memory, 16.1% impaired learning and retrieval, 9.7% impaired executive functions, and 8.2% impaired processing speed. Patients with anosmia also presented with more headache (OR 5.45; p < 0.001) and greater risk of working memory impairment (OR 5.87; p 0.03). At follow-up 12 months after hospital discharge, 14.3% of patients still showed impaired attention, 2.4% impaired working memory, 2.5% impaired executive functions, and 2.5% impaired processing speed.</p><p><strong>Discussion: </strong>Neurological manifestations are common in patients hospitalized for COVID-19 regardless of severity. The high prevalence of anosmia and its association with headache and working memory impairment at 3 months, suggest potential direct or indirect damage to the prefrontal cortex via invasion of the olfactory bulb by COVID-19.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":"1477-1488"},"PeriodicalIF":2.7,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11920300/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143414536","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":"Trigeminal neuralgia in multiple sclerosis: proposal of surgical flowchart and long-term outcome evaluation in a mono-istitutional cohort.","authors":"Elio Mazzapicchi, Morgan Broggi, Francesco Restelli, Michela Eloisa Moretti, Marco Paolo Schiariti, Jacopo Falco, Paolo Confalonieri, Laura Brambilla, Sebastiano Giuseppe Crisafulli, Paolo Ferroli, Francesco Acerbi","doi":"10.1007/s10072-024-07909-7","DOIUrl":"10.1007/s10072-024-07909-7","url":null,"abstract":"<p><strong>Background: </strong>Trigeminal neuralgia (TN) is a chronic pain syndrome more prevalent in patients with multiple sclerosis (MS), often presenting with earlier onset and more intense pain compared to non-MS patients. The management of TN in MS is complex due to the interaction between demyelination and neurovascular factors.</p><p><strong>Methods: </strong>A retrospective analysis of 35 MS patients treated for TN with 65 procedures between 2010 and 2023 was conducted. Patients underwent surgical treatments guided by a flowchart and based on the presence of NVC and patient risk factors. Procedures included MVD, PBC, SRS and TRZ. Outcomes were assessed using BNI pain and hypoesthesia scales, focusing on pain recurrence and changes in antineuralgic medication.</p><p><strong>Results: </strong>After first treatment, MVD (13 procedures) achieved a 100% success rate with a mean pain-free interval of 59.4 months, and 77% of patients reduced or stopped medication. PBC (16) had a 93.8% success rate and a recurrence time of 34 months. SRS showed an 80% success rate with a recurrence time of 7.4 months, while TRZ had a 50% success rate with 24 months of pain-free duration. After the first surgical intervention, 30.5% of patients were pain-free, increasing to 40% after the third treatment. At last follow-up (77,0 ± 68.7 months) it was demonstrated high pain-free rates (61.1%) and good long-term pain control.</p><p><strong>Conclusion: </strong>The use of a tailored flowchart significantly improves outcomes in TN-MS patients. MVD provides the longest pain-free intervals when NVC is present, while PBC, SRS, and TRZ remain viable alternatives for those with contraindications. Personalized approaches enhance pain control and reduce recurrence.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":"1789-1798"},"PeriodicalIF":2.7,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142786326","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":"Multidisciplinary treatment of a rare rapidly progressive intracranial myxoid mesenchymal tumor of uncertain differentiation FET-CREB fusion-negative.","authors":"Flavio Panico, Andrea Bianconi, Luca Bertero, Rosa Palmiero, Pietro Zeppa, Alessia Andrea Ricci, Luca Mangherini, Fabio Cofano, Roberta Rudà, Diego Garbossa, Francesco Zenga","doi":"10.1007/s10072-024-07907-9","DOIUrl":"10.1007/s10072-024-07907-9","url":null,"abstract":"<p><strong>Background: </strong>Non-meningothelial intracranial mesenchymal tumors are a heterogeneous group of central nervous system neoplasms endowed with great variability clinically and histologically. For this precise reason, significant difficulties exist in specifically cataloguing tumor entities with such distant characteristics and such uncertain clinical course.</p><p><strong>Case description: </strong>In an attempt to increase the knowledge inherent in this type of central nervous system lesions we report a case of a rare and unusual myxoid mesenchymal tumor of difficult anatomopathological classification characterized by rapid progression and optimal therapeutic response after combined surgical and radiotherapy treatment, with histo-molecular definition and DNA methylation profile. In this case, multidisciplinary management led to timely surgical intervention based on the rapid clinical deterioration and radiological progression; after adjuvant therapy with hadron therapy, the patient has no signs of recurrence two years after the surgical procedure. No FET-CREB fusion was detected, and the DNA methylation profile suggested the presence of multiple chromosomal gains and losses.</p><p><strong>Conclusions: </strong>The molecular definition as well the optimal therapeutic regimen of these tumors is not clearly defined yet and analysis of larger series is strongly warranted.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":"1867-1873"},"PeriodicalIF":2.7,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142822332","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":"Clinical and genetic characteristics of RANBP2 mutations in children with acute necrotizing encephalopathy.","authors":"Chaonan Fan, Chanjuan Hao, Kechun Li, Liping Chen, Yeqing Wang, Hengmiao Gao, Wei Li, Suyun Qian","doi":"10.1007/s10072-024-07911-z","DOIUrl":"10.1007/s10072-024-07911-z","url":null,"abstract":"<p><strong>Background: </strong>This study investigated RANBP2 mutations in children with acute necrotizing encephalopathy (ANE) and conducted a systematic review of the differences in clinical characteristics between with or without RANBP2 mutations.</p><p><strong>Methods: </strong>Whole-exome sequencing was performed on 19 pediatric ANE patients at Beijing Children's Hospital affiliated to Capital Medical University between 2017 and 2020. A systematic literature review was also conducted on the clinical characteristics and spectrum analysis of RANBP2 mutations.</p><p><strong>Results: </strong>Besides the common mutation site c.1754 C > T, new mutation sites were identified, including c.7454G > T, c.7474 A > G, c.7807 C > T, c.7918 C > A, and c.872 A > G. These sites are highly conserved. Twenty-four publications describing 38 ANE children were reviewed, of which 22 cases had the RANBP2 mutations. When combined with our study, the data included 54 ANE children aged from 3 months to 120 months, and divided into RANBP2 mutation group (n = 26) and non-mutation group (n = 28). No significant differences were observed in initial presentations, neuroimaging, treatment, or outcomes between these two groups. However, children with RANBP2 mutations had slightly elevated blood ammonia levels and a broader etiological spectrum, especially involving non-influenza pathogens.</p><p><strong>Conclusion: </strong>This study highlights novel RANBP2 mutation sites in ANE children and associates these mutations with higher blood ammonia levels and diverse etiologies.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":"1817-1826"},"PeriodicalIF":2.7,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142877536","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":"Sleep disorders in functional neurological disorder - a systematic review and meta-analysis.","authors":"Siddarth Kannan, Anirban Dutta, Abhijit Das","doi":"10.1007/s10072-024-07931-9","DOIUrl":"10.1007/s10072-024-07931-9","url":null,"abstract":"<p><strong>Introduction: </strong>Functional neurological disorders (FND) are conditions marked by disruptions in brain network function without structural abnormalities. Sleep disturbances, though under-researched, are commonly observed in FND patients and may worsen symptoms and overall health.</p><p><strong>Methods: </strong>This systematic review had been registered prospectively in PROSPERO with the registration number: CRD42023446306. Search of PubMed, MEDLINE, Embase, and Cochrane databases identified 218 articles. After removing duplicates and applying exclusion criteria, 9 studies were included in the final analysis.</p><p><strong>Results: </strong>The analysis showed a significant prevalence of sleep disorders among FND patients, with 58% reporting sleep disturbances, similar to other neuropsychiatric conditions. Studies on psychogenic non-epileptic seizures (PNES) indicated poorer subjective sleep quality and higher insomnia rates compared to epilepsy controls. Limited data on specific measures such as wake after sleep onset (WASO) and Epworth Sleepiness Scale (ESS) scores prevented definitive conclusions.</p><p><strong>Discussion: </strong>This review is the first systematic examination of sleep disorders in FND. The findings reveal a high prevalence of sleep disturbances, especially among PNES patients, correlating with lower quality of life and increased symptom severity. However, the heterogeneity of studies and limited reporting of specific sleep metrics weaken these conclusions. Further research is needed to investigate the direct impact of sleep quality on FND pathogenesis and management.</p><p><strong>Conclusion: </strong>Sleep disturbances are prevalent in FND patients and can significantly affect their quality of life. Increased awareness and routine evaluation of sleep in FND patients are recommended. Future studies should explore the relationship between sleep deprivation and FND to develop targeted therapeutic interventions.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":"1573-1580"},"PeriodicalIF":2.7,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11920331/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142908870","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}