Pub Date : 2024-10-01Epub Date: 2024-05-09DOI: 10.1007/s10072-024-07569-7
Alessandro Cocuzza, Giulio Bertani, Giorgio Conte, Edoardo Nicolò Aiello, Barbara Zarino, Teresa Difonzo, Stefano Zago, Leonardo Tariciotti, Claudia Gendarini, Elena Baratelli, Federico Verde, Barbara Poletti, Nicola Ticozzi, Mauro Pluderi, Marco Locatelli, Giacomo Pietro Comi, Maria Cristina Saetti
Introduction: Learning is a long-term memory process heavily influenced by the control processes implemented by working memory, including recognition of semantic properties of items by which subjects generate a semantic structure of engrams.
Aim: The aim of this study is to investigate the verbal learning strategies of patients affected by a tumor in the left frontal lobe to highlight the role of area 9.
Method: Ten patients with frontal low-grade gliomas and ten healthy control subjects, matched for age, sex and education, were recruited and then evaluated with a two-part verbal learning test: multi-trial word list learning in free recall, and multi-trial word list learning preceded by an explicit semantic strategy cue. Frontal patients were divided into two groups: those either with frontal lesions involving or sparing area 9.
Results: In comparison to healthy control subjects, frontal patients with lesions involving area 9 memorized fewer words and displayed difficulty in using semantic strategies. When the strategy was suggested by the examiner, their performance improved, but to a lesser extent than the healthy control. Conversely, frontal patients with lesions sparing area 9 showed similar results to healthy control subjects.
Conclusion: The results suggested that, while the identification of the categorical criterion requires the integrity of the entire dorsolateral prefrontal area, only area 9, and not the surrounding areas, could be responsible for the effective use of semantic strategies in learning tasks.
{"title":"Verbal learning in frontal patients: area 9 is critical for employing semantic strategies.","authors":"Alessandro Cocuzza, Giulio Bertani, Giorgio Conte, Edoardo Nicolò Aiello, Barbara Zarino, Teresa Difonzo, Stefano Zago, Leonardo Tariciotti, Claudia Gendarini, Elena Baratelli, Federico Verde, Barbara Poletti, Nicola Ticozzi, Mauro Pluderi, Marco Locatelli, Giacomo Pietro Comi, Maria Cristina Saetti","doi":"10.1007/s10072-024-07569-7","DOIUrl":"10.1007/s10072-024-07569-7","url":null,"abstract":"<p><strong>Introduction: </strong>Learning is a long-term memory process heavily influenced by the control processes implemented by working memory, including recognition of semantic properties of items by which subjects generate a semantic structure of engrams.</p><p><strong>Aim: </strong>The aim of this study is to investigate the verbal learning strategies of patients affected by a tumor in the left frontal lobe to highlight the role of area 9.</p><p><strong>Method: </strong>Ten patients with frontal low-grade gliomas and ten healthy control subjects, matched for age, sex and education, were recruited and then evaluated with a two-part verbal learning test: multi-trial word list learning in free recall, and multi-trial word list learning preceded by an explicit semantic strategy cue. Frontal patients were divided into two groups: those either with frontal lesions involving or sparing area 9.</p><p><strong>Results: </strong>In comparison to healthy control subjects, frontal patients with lesions involving area 9 memorized fewer words and displayed difficulty in using semantic strategies. When the strategy was suggested by the examiner, their performance improved, but to a lesser extent than the healthy control. Conversely, frontal patients with lesions sparing area 9 showed similar results to healthy control subjects.</p><p><strong>Conclusion: </strong>The results suggested that, while the identification of the categorical criterion requires the integrity of the entire dorsolateral prefrontal area, only area 9, and not the surrounding areas, could be responsible for the effective use of semantic strategies in learning tasks.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11422476/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140898832","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}
Pub Date : 2024-10-01Epub Date: 2024-05-17DOI: 10.1007/s10072-024-07593-7
Monica Ricci, Carmela Gerace, Massimiliano Ruggeri, Carlo Blundo
{"title":"Various faces of the Rey Auditory Verbal Learning Test (RAVLT): comments to the manuscript \"Normative data beyond the total scores: a process score analysis of the Rey's 15 words test in healthy aging and Alzheimer's Disease\" by Gasparini and colleagues.","authors":"Monica Ricci, Carmela Gerace, Massimiliano Ruggeri, Carlo Blundo","doi":"10.1007/s10072-024-07593-7","DOIUrl":"10.1007/s10072-024-07593-7","url":null,"abstract":"","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140958018","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}
Pub Date : 2024-10-01Epub Date: 2024-05-21DOI: 10.1007/s10072-024-07560-2
Alvin S Das, Akashleena Mallick, Samantha A Mora, Sophia Keins, Jessica R Abramson, Juan Pablo Castello, Marco Pasi, Christina E Kourkoulis, Axana Rodriguez-Torres, Andrew D Warren, Elif Gökçal, Anand Viswanathan, Steven M Greenberg, Christopher D Anderson, Jonathan Rosand, Alessandro Biffi, M Edip Gurol
Introduction: Intracerebral hemorrhage (ICH) is attributable to cerebral small vessel disease (cSVD), which includes cerebral amyloid angiopathy (CAA) and hypertensive-cSVD (HTN-cSVD). HTN-cSVD includes patients with strictly deep ICH/microbleeds and mixed location ICH/microbleeds, the latter representing a more severe form of HTN-cSVD. We test the hypothesis that more severe forms of HTN-cSVD are related to worse hypertension control in long-term follow-up after ICH.
Methods: From consecutive non-traumatic ICH patients admitted to a tertiary care center, we classified the ICH as CAA, strictly deep ICH/microbleeds, and mixed-location ICH/microbleeds. CSVD burden was quantified using a validated MRI-based score (range: 0-6 points). We created a multivariable (linear mixed effects) model adjusting for age, sex, race, year of inclusion, hypertension, and antihypertensive medication usage to investigate the association of average systolic blood pressure (SBP) during follow-up with cSVD etiology/severity.
Results: 796 ICH survivors were followed for a median of 48.8 months (IQR 41.5-60.4). CAA-related ICH survivors (n = 373) displayed a lower median SBP (138 mmHg, IQR 133-142 mmHg) compared to those of strictly deep ICH (n = 222, 141 mmHg, IQR 136-143 mmHg, p = 0.04), and mixed location ICH/microbleeds (n = 201, 142 mmHg, IQR 135-144 mmHg, p = 0.02). In the multivariable analysis, mixed location ICH/microbleeds (effect: + 3.8 mmHg, SE: 1.3 mmHg, p = 0.01) and increasing cSVD severity (+ 1.8 mmHg per score point, SE: 0.8 mmHg, p = 0.03) were associated with higher SBP in follow-up.
Conclusion: CSVD severity and subtype predicts long-term hypertension control in ICH patients.
{"title":"Hypertension control after intracerebral hemorrhage among varying small vessel disease etiologies.","authors":"Alvin S Das, Akashleena Mallick, Samantha A Mora, Sophia Keins, Jessica R Abramson, Juan Pablo Castello, Marco Pasi, Christina E Kourkoulis, Axana Rodriguez-Torres, Andrew D Warren, Elif Gökçal, Anand Viswanathan, Steven M Greenberg, Christopher D Anderson, Jonathan Rosand, Alessandro Biffi, M Edip Gurol","doi":"10.1007/s10072-024-07560-2","DOIUrl":"10.1007/s10072-024-07560-2","url":null,"abstract":"<p><strong>Introduction: </strong>Intracerebral hemorrhage (ICH) is attributable to cerebral small vessel disease (cSVD), which includes cerebral amyloid angiopathy (CAA) and hypertensive-cSVD (HTN-cSVD). HTN-cSVD includes patients with strictly deep ICH/microbleeds and mixed location ICH/microbleeds, the latter representing a more severe form of HTN-cSVD. We test the hypothesis that more severe forms of HTN-cSVD are related to worse hypertension control in long-term follow-up after ICH.</p><p><strong>Methods: </strong>From consecutive non-traumatic ICH patients admitted to a tertiary care center, we classified the ICH as CAA, strictly deep ICH/microbleeds, and mixed-location ICH/microbleeds. CSVD burden was quantified using a validated MRI-based score (range: 0-6 points). We created a multivariable (linear mixed effects) model adjusting for age, sex, race, year of inclusion, hypertension, and antihypertensive medication usage to investigate the association of average systolic blood pressure (SBP) during follow-up with cSVD etiology/severity.</p><p><strong>Results: </strong>796 ICH survivors were followed for a median of 48.8 months (IQR 41.5-60.4). CAA-related ICH survivors (n = 373) displayed a lower median SBP (138 mmHg, IQR 133-142 mmHg) compared to those of strictly deep ICH (n = 222, 141 mmHg, IQR 136-143 mmHg, p = 0.04), and mixed location ICH/microbleeds (n = 201, 142 mmHg, IQR 135-144 mmHg, p = 0.02). In the multivariable analysis, mixed location ICH/microbleeds (effect: + 3.8 mmHg, SE: 1.3 mmHg, p = 0.01) and increasing cSVD severity (+ 1.8 mmHg per score point, SE: 0.8 mmHg, p = 0.03) were associated with higher SBP in follow-up.</p><p><strong>Conclusion: </strong>CSVD severity and subtype predicts long-term hypertension control in ICH patients.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141076314","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}
Pub Date : 2024-10-01Epub Date: 2024-04-27DOI: 10.1007/s10072-024-07539-z
Fuliang Yi, Yang Gao, Xin Liu, Yujuan Ying, Qiaojin Xie, You You, Qian Zha, Canjing Luo, Min Ni, Qiuping Wang, Yuanfang Zhu
Background: Hypertension is an established risk factor for mild cognitive impairment (MCI) in elderly individuals. Nevertheless, the impact of different levels of blood pressure on the progression of MCI remains uncertain. This study aims to investigate the non-linear relationship between blood pressure and MCI in the elderly and detect the critical blood pressure threshold, thus, improving blood pressure management for individuals at high risk of MCI.
Methods: Data was obtained from the Chinese Longitudinal Healthy Longevity Survey (CLHLS) cohort. We chose normal cognitive elderly individuals who entered the cohort in 2014 for a 5-year follow-up to observe the progression of MCI. Subsequently, we utilized the Cox regression model to identify risk factors for MCI and conducted a Cox-based restricted cubic spline regression (RCS) model to examine the non-linear relationship between systolic blood pressure (SBP) and diastolic blood pressure (DBP) with MCI, determining the critical blood pressure threshold for MCI progression.
Results: In the elderly population, female (HR = 1.489, 95% CI: 1.017-2.180), lacking of exercise in the past (HR = 1.714, 95% CI: 1.108-2.653), preferring animal fats (HR = 2.340, 95% CI: 1.348-4.061), increased age (HR = 1.061, 95% CI: 1.038-1.084), increased SBP (HR = 1.036, 95% CI: 1.024-1.048), and increased DBP (HR = 1.056, 95% CI: 1.031-1.081) were associated with MCI progression. After adjusting factors such as gender, exercise, preferred types of fats, and age, both SBP (P non-linear < 0.001) and DBP (P non-linear < 0.001) in elderly individuals exhibited a non-linear association with MCI. The risk of MCI rose when SBP exceeded 135 mmHg and DBP was in the range of 80-88 mmHg. However, when DBP exceeded 88 mmHg, there was a declining trend in MCI progression, although the HR remained above 1. The identified critical blood pressure management threshold for MCI was 135/80 mmHg.
Conclusion: In this study, we discovered that risk factors affecting the progression of MCI in elderly individuals comprise gender (female), preferring to use animal fat, lack of exercise in the past, increased age, increased SBP, and increased DBP. Additionally, a non-linear relationship between blood pressure levels and MCI progression was confirmed, with the critical blood pressure management threshold for MCI onset falling within the prehypertensive range.
{"title":"A non-linear relationship between blood pressure and mild cognitive impairment in elderly individuals: A cohort study based on the Chinese longitudinal healthy longevity survey (CLHLS).","authors":"Fuliang Yi, Yang Gao, Xin Liu, Yujuan Ying, Qiaojin Xie, You You, Qian Zha, Canjing Luo, Min Ni, Qiuping Wang, Yuanfang Zhu","doi":"10.1007/s10072-024-07539-z","DOIUrl":"10.1007/s10072-024-07539-z","url":null,"abstract":"<p><strong>Background: </strong>Hypertension is an established risk factor for mild cognitive impairment (MCI) in elderly individuals. Nevertheless, the impact of different levels of blood pressure on the progression of MCI remains uncertain. This study aims to investigate the non-linear relationship between blood pressure and MCI in the elderly and detect the critical blood pressure threshold, thus, improving blood pressure management for individuals at high risk of MCI.</p><p><strong>Methods: </strong>Data was obtained from the Chinese Longitudinal Healthy Longevity Survey (CLHLS) cohort. We chose normal cognitive elderly individuals who entered the cohort in 2014 for a 5-year follow-up to observe the progression of MCI. Subsequently, we utilized the Cox regression model to identify risk factors for MCI and conducted a Cox-based restricted cubic spline regression (RCS) model to examine the non-linear relationship between systolic blood pressure (SBP) and diastolic blood pressure (DBP) with MCI, determining the critical blood pressure threshold for MCI progression.</p><p><strong>Results: </strong>In the elderly population, female (HR = 1.489, 95% CI: 1.017-2.180), lacking of exercise in the past (HR = 1.714, 95% CI: 1.108-2.653), preferring animal fats (HR = 2.340, 95% CI: 1.348-4.061), increased age (HR = 1.061, 95% CI: 1.038-1.084), increased SBP (HR = 1.036, 95% CI: 1.024-1.048), and increased DBP (HR = 1.056, 95% CI: 1.031-1.081) were associated with MCI progression. After adjusting factors such as gender, exercise, preferred types of fats, and age, both SBP (P <sub>non-linear</sub> < 0.001) and DBP (P <sub>non-linear</sub> < 0.001) in elderly individuals exhibited a non-linear association with MCI. The risk of MCI rose when SBP exceeded 135 mmHg and DBP was in the range of 80-88 mmHg. However, when DBP exceeded 88 mmHg, there was a declining trend in MCI progression, although the HR remained above 1. The identified critical blood pressure management threshold for MCI was 135/80 mmHg.</p><p><strong>Conclusion: </strong>In this study, we discovered that risk factors affecting the progression of MCI in elderly individuals comprise gender (female), preferring to use animal fat, lack of exercise in the past, increased age, increased SBP, and increased DBP. Additionally, a non-linear relationship between blood pressure levels and MCI progression was confirmed, with the critical blood pressure management threshold for MCI onset falling within the prehypertensive range.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140852309","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}
Variations in the UBQLN2 gene are associated with a group of diseases with X-linked dominant inheritance and clinical phenotypes of amyotrophic lateral sclerosis (ALS) and/or frontal temporal lobe dementia (FTD). Cases with UBQLN2 variations have been rarely reported worldwide. The reported cases exhibit strong clinical heterogeneity. Here, we report two adult-onset cases with UBQLN2 variations in Han Chinese. Whole exome sequencing revealed the hemizygous P506S (c.1516C > T) and the heterozygous P509S variation (c.1525C > T), both of which were located within the hotspot mutation region. The patient with the P506S variation was a 24-year-old male. The clinical feature was spastic paraplegia without lower motor neuron damage. The patient's mother was an asymptomatic heterozygote carrier with skewed X-chromosome inactivation. The patient with the P509S variation was a 63-year-old female. Clinical features included ALS and parkinsonism. 18F-fluorodopa PET-CT revealed presynaptic dopaminergic deficits in bilateral posterior putamen. These cases further highlight the clinical heterogeneity of UBQLN2 cases.
UBQLN2 基因变异与一组 X 连锁显性遗传疾病有关,临床表现为肌萎缩侧索硬化症(ALS)和/或额颞叶痴呆症(FTD)。UBQLN2 变异的病例在世界范围内鲜有报道。已报道的病例表现出很强的临床异质性。在此,我们报告了两例汉族成人发病的UBQLN2变异病例。全外显子测序发现了半杂合子P506S(c.1516C > T)和杂合子P509S变异(c.1525C > T),这两个变异均位于热点突变区。P506S变异患者是一名24岁的男性。临床特征为痉挛性截瘫,但无下运动神经元损伤。患者的母亲是无症状的杂合子携带者,X染色体失活偏斜。P509S变异患者是一名63岁的女性。临床特征包括 ALS 和帕金森病。18F- 氟多巴 PET-CT 显示双侧后部普他门突触前多巴胺能缺陷。这些病例进一步凸显了 UBQLN2 病例的临床异质性。
{"title":"Two new cases with the UBQLN2 gene mutation in Han Chinese.","authors":"Shuang He, Xin-Xin He, Hong-Qi Yang, Jie-Wen Zhang, Shuai Chen","doi":"10.1007/s10072-024-07674-7","DOIUrl":"10.1007/s10072-024-07674-7","url":null,"abstract":"<p><p>Variations in the UBQLN2 gene are associated with a group of diseases with X-linked dominant inheritance and clinical phenotypes of amyotrophic lateral sclerosis (ALS) and/or frontal temporal lobe dementia (FTD). Cases with UBQLN2 variations have been rarely reported worldwide. The reported cases exhibit strong clinical heterogeneity. Here, we report two adult-onset cases with UBQLN2 variations in Han Chinese. Whole exome sequencing revealed the hemizygous P506S (c.1516C > T) and the heterozygous P509S variation (c.1525C > T), both of which were located within the hotspot mutation region. The patient with the P506S variation was a 24-year-old male. The clinical feature was spastic paraplegia without lower motor neuron damage. The patient's mother was an asymptomatic heterozygote carrier with skewed X-chromosome inactivation. The patient with the P509S variation was a 63-year-old female. Clinical features included ALS and parkinsonism. <sup>18</sup>F-fluorodopa PET-CT revealed presynaptic dopaminergic deficits in bilateral posterior putamen. These cases further highlight the clinical heterogeneity of UBQLN2 cases.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141469702","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}
Pub Date : 2024-10-01Epub Date: 2024-05-04DOI: 10.1007/s10072-024-07506-8
Ming Ke, Xiaofei Luo, Yi Guo, Juli Zhang, Xupeng Ren, Guangyao Liu
Background: Juvenile myoclonic epilepsy (JME) is characterized by altered patterns of brain functional connectivity (FC). However, the nature and extent of alterations in the spatiotemporal characteristics of dynamic FC in JME patients remain elusive. Dynamic networks effectively encapsulate temporal variations in brain imaging data, offering insights into brain network abnormalities and contributing to our understanding of the seizure mechanisms and origins.
Methods: Resting-state functional magnetic resonance imaging (rs-fMRI) data were procured from 37 JME patients and 37 healthy counterparts. Forty-seven network nodes were identified by group-independent component analysis (ICA) to construct the dynamic network. Ultimately, patients' and controls' spatiotemporal characteristics, encompassing temporal clustering and variability, were contrasted at the whole-brain, large-scale network, and regional levels.
Results: Our findings reveal a marked reduction in temporal clustering and an elevation in temporal variability in JME patients at the whole-brain echelon. Perturbations were notably pronounced in the default mode network (DMN) and visual network (VN) at the large-scale level. Nodes exhibiting anomalous were predominantly situated within the DMN and VN. Additionally, there was a significant correlation between the severity of JME symptoms and the temporal clustering of the VN.
Conclusions: Our findings suggest that excessive temporal changes in brain FC may affect the temporal structure of dynamic brain networks, leading to disturbances in brain function in patients with JME. The DMN and VN play an important role in the dynamics of brain networks in patients, and their abnormal spatiotemporal properties may underlie abnormal brain function in patients with JME in the early stages of the disease.
{"title":"Alterations in spatiotemporal characteristics of dynamic networks in juvenile myoclonic epilepsy.","authors":"Ming Ke, Xiaofei Luo, Yi Guo, Juli Zhang, Xupeng Ren, Guangyao Liu","doi":"10.1007/s10072-024-07506-8","DOIUrl":"10.1007/s10072-024-07506-8","url":null,"abstract":"<p><strong>Background: </strong>Juvenile myoclonic epilepsy (JME) is characterized by altered patterns of brain functional connectivity (FC). However, the nature and extent of alterations in the spatiotemporal characteristics of dynamic FC in JME patients remain elusive. Dynamic networks effectively encapsulate temporal variations in brain imaging data, offering insights into brain network abnormalities and contributing to our understanding of the seizure mechanisms and origins.</p><p><strong>Methods: </strong>Resting-state functional magnetic resonance imaging (rs-fMRI) data were procured from 37 JME patients and 37 healthy counterparts. Forty-seven network nodes were identified by group-independent component analysis (ICA) to construct the dynamic network. Ultimately, patients' and controls' spatiotemporal characteristics, encompassing temporal clustering and variability, were contrasted at the whole-brain, large-scale network, and regional levels.</p><p><strong>Results: </strong>Our findings reveal a marked reduction in temporal clustering and an elevation in temporal variability in JME patients at the whole-brain echelon. Perturbations were notably pronounced in the default mode network (DMN) and visual network (VN) at the large-scale level. Nodes exhibiting anomalous were predominantly situated within the DMN and VN. Additionally, there was a significant correlation between the severity of JME symptoms and the temporal clustering of the VN.</p><p><strong>Conclusions: </strong>Our findings suggest that excessive temporal changes in brain FC may affect the temporal structure of dynamic brain networks, leading to disturbances in brain function in patients with JME. The DMN and VN play an important role in the dynamics of brain networks in patients, and their abnormal spatiotemporal properties may underlie abnormal brain function in patients with JME in the early stages of the disease.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140861031","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}
Pub Date : 2024-10-01Epub Date: 2024-05-20DOI: 10.1007/s10072-024-07576-8
Gabriel Marinheiro, Julyana Medeiros Dantas, Antonio Mutarelli, Artur Menegaz de Almeida, Gabriel de Almeida Monteiro, Djinane Spinosa Zerlotto, João Paulo Mota Telles
Recent randomized controlled trials (RCTs) have shown a benefit of brexpiprazole in managing agitation in patients with Alzheimer's disease (AD). However, its efficacy and safety remain unclear. We systematically searched PubMed, Embase, and Cochrane Library for RCTs comparing brexpiprazole with placebo in patients with agitation and AD. Three studies comprising 1,048 patients were included. In patients with agitation and AD, brexpiprazole significantly improved the Cohen-Mansfield Agitation Inventory total score (CMAI) at any dose (MD -3.05; 95% CI -5.12, -0.98; p < 0.01; I2 = 19%) and at 2 mg (MD -4.36; 95% CI -7.02, -1.70; p < 0.01; I2 = 0%) over 12 weeks. Brexpiprazole at any dose and 2 mg also showed benefit in the Clinical Global Impression - Severity of illness (CGI-S) score as related to agitation over 12 weeks (MD -0.20; 95% CI -0.36, -0.05; p < 0.01; I2 = 35%). There is no significant difference between the groups in the incidence of at least one treatment-emergent adverse events (TEAEs; RR 1.14; 95% CI 0.95, 1.37; p = 0.16; I2 = 45%) and all-cause mortality (RR 1.99; 95% CI 0.37, 10.84; p = 0.42; I2 = 0%). Brexpiprazole at any dose significantly increased the Simpson-Angus Scale (SAS; MD 0.47; 95% CI 0.28, 0.66; p < 0.01). Our results suggest that brexpiprazole is more efficacious than placebo in the treatment of agitation in AD patients. Further studies are still necessary to confirm long-term effects of brexpiprazole.Prospero registry: CRD42023486694.
最近的随机对照试验(RCT)显示,布来哌唑在控制阿尔茨海默病患者(AD)的躁动方面有一定疗效。然而,其疗效和安全性仍不明确。我们系统地检索了 PubMed、Embase 和 Cochrane 图书馆中对躁动和 AD 患者进行布来哌唑与安慰剂比较的 RCT。共纳入了三项研究,包括 1,048 名患者。在任何剂量(MD -3.05;95% CI -5.12,-0.98;P 2 = 19%)和 2 毫克剂量(MD -4.36;95% CI -7.02,-1.70;P 2 = 0%)的躁动和注意力缺失症患者中,布雷克吡唑在 12 周内可显著改善 Cohen-Mansfield Agitation Inventory 总分(CMAI)。在12周内,任何剂量和2毫克的布雷哌唑也显示出与躁动相关的临床总体印象-疾病严重程度(CGI-S)评分的益处(MD -0.20;95% CI -0.36,-0.05;P 2 = 35%)。在至少一种治疗突发不良事件(TEAEs;RR 1.14;95% CI 0.95,1.37;P = 0.16;I2 = 45%)和全因死亡率(RR 1.99;95% CI 0.37,10.84;P = 0.42;I2 = 0%)的发生率方面,两组间无明显差异。任何剂量的布雷哌唑都能显著增加辛普森-安格斯量表(SAS;MD 0.47;95% CI 0.28,0.66;p
{"title":"Efficacy and safety of brexpiprazole for the treatment of agitation in Alzheimer's disease: a meta-analysis of randomized controlled trials.","authors":"Gabriel Marinheiro, Julyana Medeiros Dantas, Antonio Mutarelli, Artur Menegaz de Almeida, Gabriel de Almeida Monteiro, Djinane Spinosa Zerlotto, João Paulo Mota Telles","doi":"10.1007/s10072-024-07576-8","DOIUrl":"10.1007/s10072-024-07576-8","url":null,"abstract":"<p><p>Recent randomized controlled trials (RCTs) have shown a benefit of brexpiprazole in managing agitation in patients with Alzheimer's disease (AD). However, its efficacy and safety remain unclear. We systematically searched PubMed, Embase, and Cochrane Library for RCTs comparing brexpiprazole with placebo in patients with agitation and AD. Three studies comprising 1,048 patients were included. In patients with agitation and AD, brexpiprazole significantly improved the Cohen-Mansfield Agitation Inventory total score (CMAI) at any dose (MD -3.05; 95% CI -5.12, -0.98; p < 0.01; I<sup>2</sup> = 19%) and at 2 mg (MD -4.36; 95% CI -7.02, -1.70; p < 0.01; I<sup>2</sup> = 0%) over 12 weeks. Brexpiprazole at any dose and 2 mg also showed benefit in the Clinical Global Impression - Severity of illness (CGI-S) score as related to agitation over 12 weeks (MD -0.20; 95% CI -0.36, -0.05; p < 0.01; I<sup>2</sup> = 35%). There is no significant difference between the groups in the incidence of at least one treatment-emergent adverse events (TEAEs; RR 1.14; 95% CI 0.95, 1.37; p = 0.16; I<sup>2</sup> = 45%) and all-cause mortality (RR 1.99; 95% CI 0.37, 10.84; p = 0.42; I<sup>2</sup> = 0%). Brexpiprazole at any dose significantly increased the Simpson-Angus Scale (SAS; MD 0.47; 95% CI 0.28, 0.66; p < 0.01). Our results suggest that brexpiprazole is more efficacious than placebo in the treatment of agitation in AD patients. Further studies are still necessary to confirm long-term effects of brexpiprazole.Prospero registry: CRD42023486694.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141066034","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}
Pub Date : 2024-10-01Epub Date: 2024-04-25DOI: 10.1007/s10072-024-07548-y
Fabienne Kreimer, Assem Aweimer, Ibrahim El-Battrawy, Adnan Labedi, Ruth Schneider, Arash Haghikia, Andreas Mügge, Michael Gotzmann
Background: In patients with embolic stroke of undetermined source (ESUS), underlying subclinical atrial fibrillation (AF) is often suspected. Previous studies identifying predictors of AF have been limited in their ability to diagnose episodes of AF. Implantable loop recorders enable prolonged, continuous, and therefore more reliable detection of AF. The aim of this study was to identify clinical and ECG parameters as predictors of AF in ESUS patients with implantable loop recorders.
Methods: 101 ESUS patients who received an implantable loop recorder between 2012 and 2020 were included in this study. Patients were followed up regularly on a three-monthly outpatient interval.
Results: During a mean follow-up of 647 ± 385 days, AF was detected in 26 patients (26%). Independent risk factors of AF were age ≥ 60 years (HR 2.753, CI 1.129-6.713, p = 0.026), P-wave amplitude in lead II ≤ 0.075 mV (HR 3.751, CI 1.606-8.761, p = 0.002), and P-wave duration ≥ 125 ms (HR 4.299, CI 1.844-10.021, p < 0.001). In patients without risk factors, the risk of developing AF was 16%. In the presence of one risk factor, the probability increased only slightly to 18%. With two or three risk factors, the risk of AF increased to 70%.
Conclusion: AF was detected in about one in four patients after ESUS in this study. A comprehensive evaluation involving multiple parameters and the existence of multiple risk factors yields the highest predictive accuracy for detecting AF in patients with ESUS.
{"title":"Predictors of atrial fibrillation after embolic stroke of undetermined source in patients with implantable loop recorders.","authors":"Fabienne Kreimer, Assem Aweimer, Ibrahim El-Battrawy, Adnan Labedi, Ruth Schneider, Arash Haghikia, Andreas Mügge, Michael Gotzmann","doi":"10.1007/s10072-024-07548-y","DOIUrl":"10.1007/s10072-024-07548-y","url":null,"abstract":"<p><strong>Background: </strong>In patients with embolic stroke of undetermined source (ESUS), underlying subclinical atrial fibrillation (AF) is often suspected. Previous studies identifying predictors of AF have been limited in their ability to diagnose episodes of AF. Implantable loop recorders enable prolonged, continuous, and therefore more reliable detection of AF. The aim of this study was to identify clinical and ECG parameters as predictors of AF in ESUS patients with implantable loop recorders.</p><p><strong>Methods: </strong>101 ESUS patients who received an implantable loop recorder between 2012 and 2020 were included in this study. Patients were followed up regularly on a three-monthly outpatient interval.</p><p><strong>Results: </strong>During a mean follow-up of 647 ± 385 days, AF was detected in 26 patients (26%). Independent risk factors of AF were age ≥ 60 years (HR 2.753, CI 1.129-6.713, p = 0.026), P-wave amplitude in lead II ≤ 0.075 mV (HR 3.751, CI 1.606-8.761, p = 0.002), and P-wave duration ≥ 125 ms (HR 4.299, CI 1.844-10.021, p < 0.001). In patients without risk factors, the risk of developing AF was 16%. In the presence of one risk factor, the probability increased only slightly to 18%. With two or three risk factors, the risk of AF increased to 70%.</p><p><strong>Conclusion: </strong>AF was detected in about one in four patients after ESUS in this study. A comprehensive evaluation involving multiple parameters and the existence of multiple risk factors yields the highest predictive accuracy for detecting AF in patients with ESUS.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11422254/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140865368","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}
Pub Date : 2024-10-01Epub Date: 2024-05-27DOI: 10.1007/s10072-024-07609-2
Erica Biassoni, Margherita Bellucci, Elisa Micalizzi, Marco Miggino, Enzo Andorno, Elisa Porcile, Martina Resaz, Luca Roccatagliata, Lucio Castellan, Flavio Villani, Alessandra Ferrari
Epileptic seizures are frequently associated with liver dysfunction and alcoholism. Subacute encephalopathy with seizures in chronic alcoholics (SESA) is an underrecognized condition with peculiar clinical, EEG and neuroradiological features.We report the case of a 58-year-old man with previous alcohol use disorder (AUD) and acute-on chronic liver failure on alcohol-related cirrhosis, referred for urgent Orthotopic Liver Transplantation evaluation. The patient presented with delirium, aphasia and progressive deterioration of consciousness leading to intensive care unit admission. EEG showed slow activity with superimposed lateralized periodic discharges (LPDs) over the left temporo-occipital regions and ictal discharges with focal motor phenomena, consistent with focal status epilepticus. Antiseizure treatment with lacosamide and levetiracetam was administered with progressive improvement of consciousness.Brain MRI disclosed T2/FLAIR areas of hyperintensity in the left pulvinar and T2/FLAIR hyperintensity with corresponding DWI hyperintensity in the left hippocampal cortex, suggestive of post/peri-ictal excitotoxic changes with anatomical correspondence to focal LPDs distribution. SWI demonstrated decreased prominence of cortical veins in the left temporo-occipital region consistent with increased venous blood oxygenation in compensatory hyperperfusion.In conclusion, SESA should be suspected in the differential diagnosis of patients with AUD presenting with focal neurological deficits, seizures and focal EEG abnormalities. In this context, EEG and brain MRI represent useful tools with both diagnostic and prognostic value.
{"title":"The role of EEG and neuroimaging in the diagnosis of non-convulsive status epilepticus in Subacute Encephalopathy with Seizures in Alcoholics (SESA syndrome): a case report and overview of the literature.","authors":"Erica Biassoni, Margherita Bellucci, Elisa Micalizzi, Marco Miggino, Enzo Andorno, Elisa Porcile, Martina Resaz, Luca Roccatagliata, Lucio Castellan, Flavio Villani, Alessandra Ferrari","doi":"10.1007/s10072-024-07609-2","DOIUrl":"10.1007/s10072-024-07609-2","url":null,"abstract":"<p><p>Epileptic seizures are frequently associated with liver dysfunction and alcoholism. Subacute encephalopathy with seizures in chronic alcoholics (SESA) is an underrecognized condition with peculiar clinical, EEG and neuroradiological features.We report the case of a 58-year-old man with previous alcohol use disorder (AUD) and acute-on chronic liver failure on alcohol-related cirrhosis, referred for urgent Orthotopic Liver Transplantation evaluation. The patient presented with delirium, aphasia and progressive deterioration of consciousness leading to intensive care unit admission. EEG showed slow activity with superimposed lateralized periodic discharges (LPDs) over the left temporo-occipital regions and ictal discharges with focal motor phenomena, consistent with focal status epilepticus. Antiseizure treatment with lacosamide and levetiracetam was administered with progressive improvement of consciousness.Brain MRI disclosed T2/FLAIR areas of hyperintensity in the left pulvinar and T2/FLAIR hyperintensity with corresponding DWI hyperintensity in the left hippocampal cortex, suggestive of post/peri-ictal excitotoxic changes with anatomical correspondence to focal LPDs distribution. SWI demonstrated decreased prominence of cortical veins in the left temporo-occipital region consistent with increased venous blood oxygenation in compensatory hyperperfusion.In conclusion, SESA should be suspected in the differential diagnosis of patients with AUD presenting with focal neurological deficits, seizures and focal EEG abnormalities. In this context, EEG and brain MRI represent useful tools with both diagnostic and prognostic value.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141158881","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}