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Synapse and primary cilia dysfunctions in Autism Spectrum Disorders. Avenues to normalize these functions. 自闭症谱系障碍中的突触和初级纤毛功能障碍。使这些功能正常化的途径。
IF 2.8 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-06-25 DOI: 10.1016/j.neurol.2024.06.002
J-J Hauw, C Hausser-Hauw, C Barthélémy

Aim: An update on the plasticity of the brain networks involved in autism (autism spectrum disorders [ASD]), and the increasing role of their synapses and primary non-motile cilia.

Methods: Data from PubMed and Google on this subject, published until February 2024, were analyzed.

Results: Structural and functional brain characteristics and genetic particularities involving synapses and cilia that modify neuronal circuits are observed in ASD, such as reduced pruning of dendrites, minicolumnar pathology, or persistence of connections usually doomed to disappear. Proteins involved in synapse functions (such as neuroligins and neurexins), in the postsynaptic architectural scaffolding (such as Shank proteins) or in cilia functions (such as IFT-independent kinesins) are often abnormal. There is an increase in glutaminergic transmission and a decrease in GABA inhibition. ASD may occur in genetic ciliopathies. The means of modulating these specificities, when deemed useful, are described.

Interpretation: The wide range of clinical manifestations of ASD is strongly associated with abnormalities in the morphology, functions, and plasticity of brain networks, involving their synapses and non-motile cilia. Their modulation offers important research perspectives on treatments when needed, especially since brain plasticity persists much later than previously thought. Improved early detection of ASD and additional studies on synapses and primary cilia are needed.

目的:更新自闭症(自闭症谱系障碍 [ASD])相关大脑网络的可塑性,以及其突触和初级非运动性纤毛所起的日益重要的作用:方法:分析了PubMed和Google上截至2024年2月发表的有关这一主题的数据:结果:在ASD患者中观察到了涉及突触和纤毛的大脑结构和功能特征以及改变神经元回路的遗传特异性,如树突修剪减少、小柱病理或通常注定消失的连接持续存在。参与突触功能的蛋白质(如神经ligins 和 neurexins)、参与突触后结构支架的蛋白质(如 Shank 蛋白)或参与纤毛功能的蛋白质(如不依赖于 IFT 的驱动蛋白)常常出现异常。谷氨酸传递增加,GABA 抑制减少。遗传性纤毛疾病也可能导致 ASD。在认为有用时,还介绍了调节这些特异性的方法:ASD的临床表现多种多样,与大脑网络的形态、功能和可塑性异常密切相关,涉及其突触和非运动性纤毛。对它们的调节为必要时的治疗提供了重要的研究视角,特别是因为大脑可塑性的持续时间比以前认为的要晚得多。我们需要加强对自闭症的早期检测,并对突触和初级纤毛进行更多的研究。
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引用次数: 0
Remote neurological evaluation reliably identifies patients eligible to endovascular therapy while non-eligible to intravenous thrombolysis. 远程神经评估能可靠地识别出符合血管内治疗条件但不符合静脉溶栓条件的患者。
IF 2.8 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-06-24 DOI: 10.1016/j.neurol.2024.05.006
L Lucas, A Georget, L Rouxel, P Briau, M Couture, J-S Liegey, S Debruxelles, M Poli, S Sagnier, P Renou, S Olindo, F Rouanet, X Maurin, A Benard, I Sibon

Introduction/background: Early identification of suspected stroke patients who might be eligible for a reperfusion strategy is a daily challenge in the management of patient referrals. The aim of this study was to evaluate the performance of a remote medical assessment in identifying patients eligible for endovascular therapy (EVT) while not eligible for intravenous thrombolysis (IVT), compared with a decision based on bedside clinico-radiological data.

Methods: Patients admitted to the emergency department for acute neurological symptoms lasting for less than 24h were prospectively included. Assessment of the clinical severity and medical history was performed simultaneously by two vascular neurologists (VNs), one remotely using a mobile telemedicine solution (NOMADEEC), the other at the bedside. RACE score was calculated from the NIHSS score. At the end of the evaluation, both VNs quoted their treatment convictions (IVT/EVT). Final therapeutic decision following brain and vascular imaging was recorded and compared to remote and bedside predictions. The performances of three different conditions were evaluated: complete medical evaluation (NIHSS+medical history), NIHSS score alone, and RACE score alone. Remote and bedside performances were also compared. Diagnostic accuracy parameters (sensitivity, specificity, positive and negative predictive values) of each condition were estimated, along with their two-sided 95% binomial confidence intervals.

Results: Out of 215 enrolled patients, 186 had a complete evaluation, 91 (54.3%) were diagnosed with an ischemic stroke or transient ischemic attack and 46 (24.7%) had an intracranial occlusion. Considering the three conditions evaluated remotely, RACE score-based decision provided the best sensitivity 54.6% [95% CI 23.4; 83.2]/specificity 80.6% [73.9; 86.2] combination. However, the complete medical evaluation had the best specificity (88.6% [82.9; 92.9] compared to RACE scores alone (P=0.038). Remote and bedside performances did not differ (κ=0.68 [0.59; 0.77]).

Discussion/conclusion: This real-life study performed in the setting of emergency demonstrates that remote medical evaluations including recording of extensive medical information and NIHSS examination to address patient's eligibility to revascularization treatment is swiftly feasible and is as effective as bedside prediction to EVT and/or IVT. Remote standardized medical evaluation might improve the decision of patients' primary orientation and avoid overcrowding of comprehensive stroke centres.

导言/背景:早期识别可能符合再灌注策略的疑似卒中患者是患者转诊管理中的一项日常挑战。本研究旨在评估远程医疗评估在识别符合血管内治疗(EVT)条件但不符合静脉溶栓(IVT)条件的患者方面的性能,并与基于床旁临床放射学数据的决策进行比较:方法:前瞻性地纳入因急性神经症状持续时间少于 24 小时而入住急诊科的患者。临床严重程度和病史评估由两名血管神经学家(VN)同时进行,其中一名使用移动远程医疗解决方案(NOMADEEC)进行远程评估,另一名在床边进行评估。根据 NIHSS 评分计算 RACE 评分。在评估结束时,两位 VN 都会提出他们的治疗意见(IVT/EVT)。记录脑部和血管成像后的最终治疗决定,并与远程和床旁预测进行比较。评估了三种不同情况下的表现:完整的医学评估(NIHSS+病史)、单独的 NIHSS 评分和单独的 RACE 评分。同时还比较了远程和床旁预测的性能。对每种情况的诊断准确性参数(灵敏度、特异性、阳性预测值和阴性预测值)及其双侧 95% 二项式置信区间进行了估算:在 215 名登记患者中,186 人进行了完整的评估,91 人(54.3%)被诊断为缺血性中风或短暂性脑缺血发作,46 人(24.7%)患有颅内闭塞。考虑到远程评估的三种情况,基于 RACE 评分的决策提供了最佳的灵敏度 54.6% [95% CI 23.4; 83.2] / 特异性 80.6% [73.9; 86.2] 组合。然而,与单独的 RACE 评分相比,完整的医学评估具有最佳的特异性(88.6% [82.9; 92.9])(P=0.038)。远程和床边的表现没有差异(κ=0.68 [0.59; 0.77]):这项在急诊环境下进行的真实研究表明,远程医疗评估(包括记录大量医疗信息和 NIHSS 检查)可迅速确定患者接受血管再通治疗的资格,并且与床旁预测 EVT 和/或 IVT 一样有效。远程标准化医疗评估可改善患者的首要定位决策,避免综合卒中中心人满为患。
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引用次数: 0
Limb-girdle muscle weakness and muscle hypertrophy: Do not dismiss spinal muscular atrophy. 肢腰肌无力和肌肉肥大:不要忽视脊髓性肌萎缩症。
IF 3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-06-17 DOI: 10.1016/j.neurol.2024.06.001
T Nanda, S Nanda, A Manna, J Mukherjee, M Mandal, J Benito-León
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引用次数: 0
The pattern of cortical thickness associated with executive dysfunction in MCI and SCC: The MEMENTO cohort. 与 MCI 和 SCC 执行功能障碍相关的皮层厚度模式:MEMENTO 队列。
IF 3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-06-11 DOI: 10.1016/j.neurol.2024.02.394
D Andriuta, M Roussel, G Chene, C Fischer, J-F Mangin, B Dubois, B Vellas, F Pasquier, F Tison, F Blanc, O Hanon, C Paquet, A Gabelle, M Ceccaldi, C Annweiler, P Krolak-Salmon, R David, I Rouch-Leroyer, A Benetos, O Moreaud, F Sellal, I Jalenques, P Vandel, V Bouteloup, O Godefroy

Background: The association between the pattern of cortical thickness (CT) and executive dysfunction (ED) in mild cognitive impairment (MCI) and subjective cognitive complaints (SCC) is still poorly understood. We aimed to investigate the association between CT and ED in a large French cohort (MEMENTO) of 2323 participants with MCI or SCC.

Methods: All participants with available CT and executive function data (verbal fluency and Trail Making Test [TMT]) were selected (n=1924). Linear regressions were performed to determine relationships between executive performance and the brain parenchymal fraction (BPF) and CT using FreeSurfer.

Results: The global executive function score was related to the BPF (sß: 0.091, P<0.001) and CT in the right supramarginal (sß: 0.060, P=0.041) and right isthmus cingulate (sß: 0.062, P=0.011) regions. Literal verbal fluency was related to the BPF (sß: 0.125, P<0.001) and CT in the left parsorbitalis region (sß: 0.045, P=0.045). Semantic verbal fluency was related to the BPF (sß: 0.101, P<0.001) and CT in the right supramarginal region (sß: 0.061, P=0.042). The time difference between the TMT parts B and A was related to the BPF (sß: 0.048, P=0.045) and CT in the right precuneus (sß: 0.073, P=0.019) and right isthmus cingulate region (sß: 0.054, P=0.032).

Conclusions: In a large clinically based cohort of participants presenting with either MCI or SCC (a potential early stage of Alzheimer's disease [AD]), ED was related to the BPF and CT in the left pars orbitalis, right precuneus, right supramarginal, and right isthmus cingulate regions. This pattern of lesions adds knowledge to the conventional anatomy of ED and could contribute to the early diagnosis of AD.

背景:人们对轻度认知障碍(MCI)和主观认知抱怨(SCC)患者皮层厚度(CT)模式与执行功能障碍(ED)之间的关系仍然知之甚少。我们的目的是在一个由 2323 名 MCI 或 SCC 患者组成的大型法国队列(MEMENTO)中调查 CT 与 ED 之间的关系:我们选取了所有具有 CT 和执行功能数据(言语流畅性和寻迹测试 [TMT])的参与者(n=1924)。使用FreeSurfer进行线性回归,以确定执行力表现与脑实质分数(BPF)和CT之间的关系:结果:总体执行功能得分与 BPF 相关(sß:0.091,PC结论:在一个以临床为基础的大型参与者队列中,在出现 MCI 或 SCC(阿尔茨海默病 [AD] 的潜在早期阶段)时,ED 与左侧眶旁区、右侧楔前区、右侧边际上区和右侧扣带回峡区的 BPF 和 CT 有关。这种病变模式增加了对 ED 传统解剖学的了解,有助于早期诊断 AD。
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引用次数: 0
Neuropsychological assessment in pediatric epilepsy surgery: A French procedure consensus 小儿癫痫手术的神经心理学评估:法国手术共识。
IF 3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-06-01 DOI: 10.1016/j.neurol.2023.08.019
V. Laguitton , M. Boutin , H. Brissart , D. Breuillard , M. Bilger , N. Forthoffer , V. Guinet , S. Hennion , C. Kleitz , H. Mirabel , C. Mosca , S. Pradier , S. Samson , V. Voltzenlogel , M. Planton , M. Denos , C. Bulteau

Neuropsychological assessment is a mandatory part of the pre- and post-operative evaluation in pediatric epilepsy surgery. The neuropsychology task force of the ILAE - French Chapter aims to define a neuropsychological procedure consensus based on literature review and adapted for French practice. They performed a systematic review of the literature published between 1950 and 2023 on cognitive evaluation of individuals undergoing presurgical work-up and post-surgery follow-up and focused on the pediatric population aged 6–16. They classified publications listed in the PubMed database according to their level of scientific evidence. The systematic literature review revealed no study with high statistical power and only four studies using neuropsychological scales in their French version. Afterwards, the experts defined a neuropsychological consensus strategy in pediatric epilepsy surgery according to the psychometric determinants of cognitive tests, specificity of epilepsy, surgery context, French culture and literature reports. A common French neuropsychological procedure dedicated to pediatric epilepsy surgery is now available. This procedure could serve as a guide for the pre- and post-surgical work-up in French centers with pediatric epilepsy surgery programs. The main goal is to anticipate the functional risks of surgery, to support the postoperative outcome beyond the seizure-related one, while taking into consideration the plasticity and vulnerability of the immature brain and allowing the possibility of collaborative studies.

神经心理学评估是小儿癫痫手术术前和术后评估的强制性组成部分。ILAE-法国分会的神经心理学工作组旨在根据文献综述和法国实践,定义一种神经心理学程序共识。他们对1950年至2023年间发表的关于接受术前检查和术后随访的个体认知评估的文献进行了系统综述,重点关注6-16岁的儿科人群。他们根据科学证据的水平对PubMed数据库中列出的出版物进行了分类。系统文献综述显示,没有一项研究具有较高的统计能力,只有四项研究使用了法语版的神经心理学量表。之后,专家们根据认知测试的心理测量决定因素、癫痫的特异性、手术背景、法国文化和文献报道,在儿童癫痫手术中定义了一种神经心理学共识策略。法国一种常见的专门用于儿童癫痫手术的神经心理学程序现已问世。该程序可作为法国儿童癫痫手术中心手术前和手术后检查的指南。主要目标是预测手术的功能风险,支持癫痫相关的术后结果,同时考虑到未成熟大脑的可塑性和脆弱性,并允许进行合作研究。
{"title":"Neuropsychological assessment in pediatric epilepsy surgery: A French procedure consensus","authors":"V. Laguitton ,&nbsp;M. Boutin ,&nbsp;H. Brissart ,&nbsp;D. Breuillard ,&nbsp;M. Bilger ,&nbsp;N. Forthoffer ,&nbsp;V. Guinet ,&nbsp;S. Hennion ,&nbsp;C. Kleitz ,&nbsp;H. Mirabel ,&nbsp;C. Mosca ,&nbsp;S. Pradier ,&nbsp;S. Samson ,&nbsp;V. Voltzenlogel ,&nbsp;M. Planton ,&nbsp;M. Denos ,&nbsp;C. Bulteau","doi":"10.1016/j.neurol.2023.08.019","DOIUrl":"10.1016/j.neurol.2023.08.019","url":null,"abstract":"<div><p>Neuropsychological assessment is a mandatory part of the pre- and post-operative evaluation in pediatric epilepsy surgery. The neuropsychology task force of the ILAE - French Chapter aims to define a neuropsychological procedure consensus based on literature review and adapted for French practice. They performed a systematic review of the literature published between 1950 and 2023 on cognitive evaluation of individuals undergoing presurgical work-up and post-surgery follow-up and focused on the pediatric population aged 6–16. They classified publications listed in the PubMed database according to their level of scientific evidence. The systematic literature review revealed no study with high statistical power and only four studies using neuropsychological scales in their French version. Afterwards, the experts defined a neuropsychological consensus strategy in pediatric epilepsy surgery according to the psychometric determinants of cognitive tests, specificity of epilepsy, surgery context, French culture and literature reports. A common French neuropsychological procedure dedicated to pediatric epilepsy surgery is now available. This procedure could serve as a guide for the pre- and post-surgical work-up in French centers with pediatric epilepsy surgery programs. The main goal is to anticipate the functional risks of surgery, to support the postoperative outcome beyond the seizure-related one, while taking into consideration the plasticity and vulnerability of the immature brain and allowing the possibility of collaborative studies.</p></div>","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":"180 6","pages":"Pages 494-506"},"PeriodicalIF":3.0,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"72210613","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}
引用次数: 0
Spreading of motor neuron degeneration in ALS is not so random 肌萎缩性脊髓侧索硬化症运动神经元变性的扩散并不是随机的。
IF 3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-06-01 DOI: 10.1016/j.neurol.2024.02.384
P. Corcia , P. Couratier
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引用次数: 0
Emerging autophagic endo-lysosomal targets in the management of Parkinson's disease 治疗帕金森病的新自噬内溶酶体靶点
IF 3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-06-01 DOI: 10.1016/j.neurol.2023.07.007
T. Siddiqui, L.K. Bhatt

Synucleopathies, specifically Parkinson's disease, are still incurable and available therapeutic options are scarce and symptomatic. The autophagy-lysosomal-endosomal system is an indigenous mechanism to manage the proteome. Excess/misfolded protein accumulation activates this system, which degrades the undesired proteins via lysosomes. Cells also eliminate these proteins by releasing them into the extracellular space via exosomes. However, the sutophagy-lysosomal-endosomal system becomes unfunctional in Parkinson's disease and there is accumulation and spread of pathogenic alpha-synuclein. Neuronal degeneration results Owing to pathogenic alpha-synuclein. Thus, the autophagy-lysosomal-endosomal system could be a promising target for neuroprotection. In the present review, we discuss the autophagy-lysosomal-endosomal system as an emerging target for the management of Parkinson's disease. Modulation of these targets associated with the autophagy-lysosomal-endosomal system can aid in clearing pathogenic alpha-synuclein and prevent the degeneration of neurons.

突触核病,特别是帕金森病,仍然无法治愈,现有的治疗方案很少,而且症状不明显。自噬-溶酶体-内体系统是一种管理蛋白质组的本地机制。过量/折叠不良蛋白质的积累会激活该系统,通过溶酶体降解不需要的蛋白质。细胞还会通过外泌体将这些蛋白质释放到细胞外空间,从而消除这些蛋白质。然而,在帕金森病中,溶酶体-内泌体系统功能失调,致病性α-突触核蛋白积累和扩散。致病性α-突触核蛋白导致神经元变性。因此,自噬-溶酶体-内体系统可能是一个很有前景的神经保护靶点。在本综述中,我们将讨论自噬-溶酶体-内体系统作为治疗帕金森病的一个新兴靶点。调节这些与自噬-溶酶体-内体系统相关的靶点有助于清除致病性α-突触核蛋白并防止神经元变性。
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引用次数: 0
In young patients with stroke of undetermined etiology, large vessel occlusions are less frequent in the group with high-risk patent foramen ovale 在病因不明的中风年轻患者中,大血管闭塞在高危的卵圆孔未闭组中发生率较低
IF 3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-06-01 DOI: 10.1016/j.neurol.2023.11.002
A. Ter Schiphorst , A. Lippi , L. Corti , I. Mourand , P. Prin , A. Agullo , F. Cagnazzo , J.-C. Macia , C. Arquizan

Introduction

Patent foramen ovale (PFO) is present in a significant proportion of young patients with stroke of undetermined etiology, but is not always causal. Therefore, classifications (RoPE, PASCAL) have been developed to determine the probability that PFO is the stroke cause. However, the presence of an initial arterial occlusion as a prediction factor was not studied when these classifications were built. Our aim was to evaluate the presence of arterial occlusion in young patients with stroke of undetermined etiology with/without high-risk PFO.

Methods

From a prospectively-built monocentric database, we identified patients aged  18 to < 60-years with strokes of undetermined etiology and complete etiological work-up, including transesophageal echocardiography. We divided patients in two groups: (i) with high-risk PFO [i.e. PFO with large interatrial shunt (> 30 microbubbles) or associated with atrial septal aneurysm] and (ii) with low-risk/without PFO. We recorded the presence of arterial occlusion and large vessel occlusion (LVO) in the acute phase.

Results

We included 96 patients; 55 (57%) had high-risk PFO. Their median age was 48 (40–52) years, and 28 (29%) were women. The percentages of patients with arterial occlusion and with LVO were lower in the high-risk PFO group than in the low-risk/without PFO group: 11 (20%) versus 19 (46%) (P = 0.008), and 5 (9%) versus 15 (37%) (P = 0.002), respectively. There was no difference in the median RoPE score between groups (P = 0.30).

Conclusion

The presence of LVO could represent a “red flag” of PFO causality in stroke of undetermined etiology, and could be implemented in future PFO-related stroke classifications.

导言在病因未明的卒中年轻患者中,有相当一部分存在闭孔 (PFO),但并不总是因果关系。因此,已开发出一些分类方法(RoPE、PASCAL)来确定 PFO 是卒中病因的可能性。然而,在建立这些分类时,并未将是否存在初始动脉闭塞作为预测因素进行研究。我们的目的是评估病因不明、伴有/不伴有高危 PFO 的年轻中风患者中是否存在动脉闭塞。方法我们从一个前瞻性建立的单中心数据库中确定了年龄≥ 18 至 60 岁、病因不明的中风患者,并进行了完整的病因学检查,包括经食道超声心动图检查。我们将患者分为两组:(i) 高危 PFO 患者[即 PFO 伴有较大的房室间分流(30 微气泡)或伴有房间隔动脉瘤];(ii) 低危/无 PFO 患者。我们记录了急性期动脉闭塞和大血管闭塞(LVO)的情况。他们的中位年龄为 48(40-52)岁,28(29%)人为女性。高危 PFO 组中动脉闭塞和 LVO 患者的比例低于低危/无 PFO 组:分别为 11 (20%) 对 19 (46%) (P = 0.008) 和 5 (9%) 对 15 (37%) (P = 0.002)。结论 LVO 的存在可作为病因未确定的卒中中 PFO 病因的 "红旗",可在未来 PFO 相关卒中分类中应用。
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引用次数: 0
Tribute to Maurice Collard (1931–2024) 向莫里斯-科拉尔德(1931-2024 年)致敬。
IF 3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-06-01 DOI: 10.1016/j.neurol.2024.05.002
F. Sellal
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引用次数: 0
Neurodystrophic hand dermatitis – Sannino-Barduagni syndrome 神经营养不良性手部皮炎--桑尼诺-巴杜亚尼综合征
IF 3 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-06-01 DOI: 10.1016/j.neurol.2023.11.007
A. Demas , T. Apard , A.-L. Bedat-Millet , T. Maisonobe
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引用次数: 0
期刊
Revue neurologique
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