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Impact of weight loss and disease progression on survival in ALS: insights from a multidisciplinary care center. 体重减轻和疾病进展对ALS患者生存的影响:来自多学科护理中心的见解。
IF 1.6 4区 医学 Q4 NEUROSCIENCES Pub Date : 2025-10-01 Epub Date: 2025-10-15 DOI: 10.1055/s-0045-1812029
Mário Emílio Teixeira Dourado Junior, Laura Carvalheira Dourado, Glauciane Costa Santana, Sancha Helena de Lima Vale, Lucia Leite-Lais

Amyotrophic lateral sclerosis (ALS) is a multifaceted neurodegenerative disorder with a poor prognosis. Weight loss and malnutrition emerge as significant clinical features during disease progression.To explore how demographic and clinical characteristics relate to survival in ALS patients, emphasizing the role of weight loss percentage at the time of diagnosis.We conducted a retrospective study that used the database of a multidisciplinary ALS care center in the city of Natal, Brazil.A total of 132 patients were included in the study. The mean age of the participants at symptom onset was of 56.9 years, and most of them were male (59.8%). Older age, bulbar onset, and faster disease progression were associated with weight loss ≥ 10% at diagnosis. Among 132 patients, 72% experienced death or tracheostomy, with a median survival of 34 months. Survival was notably reduced in patients aged ≥ 60 years, those with significant weight loss, rapid disease progression, or those submitted to gastrostomy. Weight loss and the rate of disease progression were the strongest predictors of reduced survival. Potential factors relating gastrostomy with reduced survival are discussed.The present study highlights the critical impact of weight loss and disease progression on survival in ALS patients, emphasizing the importance of early nutritional and clinical interventions. These findings underscore the need for comprehensive, multidisciplinary care strategies to address key prognostic factors and improve outcomes in ALS patients.

肌萎缩性侧索硬化症(ALS)是一种预后不良的多方面神经退行性疾病。体重减轻和营养不良是疾病进展过程中的重要临床特征。探讨人口统计学和临床特征与ALS患者生存的关系,强调在诊断时体重减轻百分比的作用。我们进行了一项回顾性研究,该研究使用了巴西纳塔尔市一家多学科ALS护理中心的数据库。该研究共纳入132例患者。参与者出现症状时的平均年龄为56.9岁,男性居多(59.8%)。年龄较大、球部发病和疾病进展较快与诊断时体重减轻≥10%相关。在132例患者中,72%的患者死亡或气管切开术,中位生存期为34个月。年龄≥60岁、体重明显减轻、疾病进展迅速或接受胃造口术的患者的生存率明显降低。体重减轻和疾病进展速度是降低生存率的最强预测因子。探讨了胃造口术降低生存率的潜在因素。本研究强调了体重减轻和疾病进展对ALS患者生存的关键影响,强调了早期营养和临床干预的重要性。这些发现强调需要综合的、多学科的护理策略来解决关键的预后因素并改善ALS患者的预后。
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引用次数: 0
Headache and GLP-1 receptor agonists: when medications are therapeutic and when they contribute to the symptom. 头痛和GLP-1受体激动剂:当药物治疗和当它们有助于症状。
IF 1.6 4区 医学 Q4 NEUROSCIENCES Pub Date : 2025-10-01 Epub Date: 2025-10-27 DOI: 10.1055/s-0045-1812303
Erika Tavares Ferreira, Leidys Marina Pedrozo Garcia, Renata Gomes Londero

Obesity is a complex metabolic disorder with significant implications for both individual and public health. It has been strongly linked to chronic headache conditions, including migraines and idiopathic intracranial hypertension (IIH). Individuals with obesity who suffer from migraine are at increased risk of chronification, while weight reduction has been associated with improvement in IIH-related headaches, likely due to a decrease in cerebrospinal fluid pressure. These observations underscore the importance of weight management strategies as a therapeutic consideration in patients with obesity and headache disorders. Glucagon-like peptide-1 receptor agonists (GLP-1 RAs) are pharmacological agents that mimic the hormone's endogenous activity. Analysis of selected studies highlights that these agents have emerged as a promising therapeutic option. The aim of this narrative review is to examine the role of GLP-1 RAs in the management of headaches, particularly in the context of IIH, migraine, and the gut-brain axis. Additionally, this review addresses the challenges associated with the use of this pharmaceutical class, including the potential for headaches as adverse effect, and identifies existing knowledge gaps that may guide future research.

肥胖是一种复杂的代谢紊乱,对个人和公众健康都有重大影响。它与慢性头痛疾病密切相关,包括偏头痛和特发性颅内高压(IIH)。患有偏头痛的肥胖个体慢性化的风险增加,而体重减轻与iih相关头痛的改善有关,可能是由于脑脊液压力降低。这些观察结果强调了体重管理策略作为肥胖和头痛疾病患者治疗考虑的重要性。胰高血糖素样肽-1受体激动剂(GLP-1 RAs)是模仿激素内源性活性的药理学药物。对选定研究的分析强调,这些药物已成为一种有希望的治疗选择。这篇叙述性综述的目的是研究GLP-1 RAs在头痛管理中的作用,特别是在IIH、偏头痛和肠脑轴的背景下。此外,本综述解决了与使用这类药物相关的挑战,包括潜在的头痛副作用,并确定了可能指导未来研究的现有知识差距。
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引用次数: 0
Causal links between mitochondrial genes, cerebrospinal fluid metabolites, and delirium: a mendelian randomization study. 线粒体基因、脑脊液代谢物和谵妄之间的因果关系:一项孟德尔随机研究。
IF 1.6 4区 医学 Q4 NEUROSCIENCES Pub Date : 2025-10-01 Epub Date: 2025-12-02 DOI: 10.1055/s-0045-1812892
Yafeng Wang, Jiaming Wu, Shiyang Wei, Yanyan Hu, Yalan Li

Mitochondrial dysfunction plays a crucial role in neuropsychiatric disorders, including delirium.To explore the causal links between mitochondrial-related druggable genes, cerebrospinal fluid metabolites, and delirium.Summary-level data on mitochondrial-related druggable genes, expression quantitative trait loci (eQTLs), 338 cerebrospinal fluid (CSF) metabolites, and delirium data were obtained from publicly accessible genome-wide association studies. A two-sample Mendelian randomization (MR) was applied to assess the causal effects of blood cis-eQTL of mitochondrial-related druggable genes on delirium. Sensitivity analyses were also undertaken to ensure the MR results' reliability. We assessed whether cerebrospinal fluid metabolites mediate the causal relationship between druggable mitochondrial genes and delirium.A total of 12 mitochondrial-related druggable genes (8 protective and 4 risk) were identified to be associated with delirium risk (p < 0.05). Furthermore, 20 CSF metabolites were significantly associated with delirium, 9 positively and 11 negatively. Sensitivity analyses showed no evidence of heterogeneity or horizontal pleiotropy. Mediation analysis indicated that 3-hydroxyoctanoate partially mediated the causal association between sterol carrier protein 2 (SCP2) and delirium, accounting for approximately 19.23% of the total effect.The present work reveals that mitochondrial-related genes and CSF metabolites may play causal roles in delirium and highlights SCP2-3-hydroxyoctanoate as a novel molecular axis. These findings expand current knowledge of delirium pathogenesis and offer a potential molecular target for diagnosis and therapy. Further experimental validation and population-diverse studies are needed to confirm these findings.

线粒体功能障碍在包括谵妄在内的神经精神疾病中起着至关重要的作用。探讨线粒体相关药物基因、脑脊液代谢物与谵妄之间的因果关系。从公开的全基因组关联研究中获得了线粒体相关药物基因、表达数量性状位点(eQTLs)、338脑脊液(CSF)代谢物和谵妄数据的汇总数据。采用双样本孟德尔随机化(MR)来评估血液中线粒体相关药物基因顺式- eqtl对谵妄的因果影响。敏感性分析也进行,以确保核磁共振结果的可靠性。我们评估了脑脊液代谢物是否介导可用药线粒体基因与谵妄之间的因果关系。共有12个线粒体相关药物基因(8个保护性基因和4个危险基因)被确定与谵妄风险相关(p
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引用次数: 0
From HITS to misses: aspirin's effect on TCD-detected cerebral microemboli in Chagas cardiomyopathy. 从HITS到未命中:阿司匹林对查加斯心肌病tcd检测的脑微栓塞的影响。
IF 1.6 4区 医学 Q4 NEUROSCIENCES Pub Date : 2025-10-01 Epub Date: 2025-12-02 DOI: 10.1055/s-0045-1812885
Brandon J Bond, Victoria Grau Kazmieczak, José Biller
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引用次数: 0
Z-drug abuse and dependence: clinical guideline of the Brazilian Academy of Neurology for diagnosis and management. z -药物滥用和依赖:巴西神经病学学会诊断和管理临床指南。
IF 1.6 4区 医学 Q4 NEUROSCIENCES Pub Date : 2025-10-01 Epub Date: 2025-11-04 DOI: 10.1055/s-0045-1812323
Fernando Gustavo Stelzer, Andrea Bacelar, Alan Luiz Éckeli, André Brooking Negrão, Carlos Maurício Oliveira Almeida, Clélia Maria Ribeiro Franco, Gabriel Natan Pires, Lívia Leite Goés Gitaí, Manoel Alves Sobreira-Neto, Márcia Assis, Paulo Afonso Mei, Rosa Hasan, Sandra Cristina Gonçalves Martinez, Tania Marchiori, Thiago M Fidalgo, Luciana L de Siqueira, Dalva Poyares

Benzodiazepine (BZD) receptor agonists, commonly known as Z-drugs, are non-BZD hypnotics primarily prescribed for the treatment of insomnia. Their use is recommended for no longer than four weeks to minimize the risk of adverse effects, including dependence and withdrawal. However, these guidelines are frequently disregarded, and the abuse of and dependence on Z-drugs has emerged as a growing public health concern in Brazil. The present article reviews the current evidence on Z-drug use disorder-including dependence and withdrawal-and proposes clinical guidelines for the management of discontinuation. The recommendations were developed based on a systematic review of the literature and refined using the Delphi methodology. The consensus was developed by a multidisciplinary task force, with coordination and voting led by a steering committee. An advisory committee, consisting of neurologists from the Brazilian Academy of Neurology (Academia Brasileira de Neurologia, ABN, in Portuguese) and psychiatrists specializing in substance-use disorders, contributed to the selection and organization of the scientific literature and took part in the voting process. Key recommendations were established: 1) prior to discontinuation, a comprehensive assessment of mental status, psychiatric and sleep comorbidities, and the degree of pharmacological dependence is essential; 2) gradual tapering is advised; 3) non-pharmacological interventions, such as cognitive behavioral therapy for insomnia, are recommended, and acceptance and commitment therapy, which is optional, may be incorporated; 4) for zolpidem withdrawal, adjunctive pharmacotherapy, which is optional, may include trazodone, other antidepressants, quetiapine or other antipsychotics, alpha-2-delta (α2δ) ligands, or alternative hypnotics (such as ramelteon, zopiclone, and eszopiclone); 5) for Z-drug discontinuation, intermediate- or long-acting BZDs are recommended; and 6) short- or ultra-short-acting BZDs and immediate-release melatonin are not recommended.

苯二氮卓(BZD)受体激动剂,通常被称为z药物,是一种非BZD催眠药,主要用于治疗失眠。建议使用不超过四周,以尽量减少不良反应的风险,包括依赖和戒断。然而,这些准则经常被忽视,滥用和依赖z -药物已成为巴西日益严重的公共卫生问题。本文回顾了目前关于z -药物使用障碍的证据,包括依赖和戒断,并提出了停药管理的临床指南。这些建议是在对文献进行系统回顾的基础上制定的,并使用德尔菲方法进行了改进。该共识是由一个多学科工作组制定的,由一个指导委员会领导协调和表决。由巴西神经病学学会(葡萄牙语:Academia Brasileira de Neurologia, ABN)的神经学家和专门研究药物使用障碍的精神病学家组成的咨询委员会为科学文献的选择和组织做出了贡献,并参与了投票过程。主要建议如下:1)停药前,应全面评估精神状态、精神和睡眠合并症以及药物依赖程度;2)建议逐步减持;3)推荐非药物干预,如认知行为治疗失眠,并可纳入可选的接受和承诺治疗;4)对于唑吡坦戒断,辅助药物治疗(可选)可包括曲唑酮、其他抗抑郁药、喹硫平或其他抗精神病药、α -2- δ (α2δ)配体或替代催眠药(如拉美替龙、佐匹克隆和艾司佐匹克隆);5)对于z型药物停药,推荐使用中效或长效BZDs;6)不建议使用短效或超短效BZDs和立即释放的褪黑激素。
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引用次数: 0
Rewriting the prognosis of multiple sclerosis in Brazil: a 25-year perspective on evolving diagnostic criteria. 改写巴西多发性硬化症的预后:25年来不断发展的诊断标准
IF 1.6 4区 医学 Q4 NEUROSCIENCES Pub Date : 2025-10-01 Epub Date: 2025-10-27 DOI: 10.1055/s-0045-1811725
Dagoberto Callegaro, Guilherme Diogo Silva
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引用次数: 0
Cervical radiculopathy for neurologists: the role of electrodiagnosis. 神经学家的颈神经根病:电诊断的作用。
IF 1.6 4区 医学 Q4 NEUROSCIENCES Pub Date : 2025-10-01 Epub Date: 2025-11-28 DOI: 10.1055/s-0045-1812893
Lucas Immich Gonçalves, Pedro Helder de Oliveira Junior, José Pedro Soares Baima

Cervical radiculopathy (CR) is a common condition encountered in the general population, usually related to a musculoskeletal degenerative condition. Conventional electroneuromyography (ENMG) consists of nerve conduction studies (NCS) and needle electromyography (EMG), and it is regarded as the most specific diagnostic evaluation in this scenario. Although CR is commonly encountered in clinical practice, ENMG as a diagnostic tool is not often discussed in neurology residency programs. Electromyography has demonstrated modest sensitivity (50-71%) but excellent specificity (approaching 100%) for the diagnosis of CR. It can also provide valuable information about lesion chronicity. In EMG, acute lesions typically present with denervation potentials and reduced recruitment, but with preserved motor unit action potential (MUAP) morphology. In contrast, chronic lesions are characterized by remodeling, with MUAPs showing increased duration, amplitude, and number of phases, in addition to reduced recruitment. The present review aims to provide an overview of the roles of NCS and EMG, while also introducing key terminology commonly encountered in the interpretation of these diagnostic modalities.

颈神经根病(CR)是一种常见病,通常与肌肉骨骼退行性疾病有关。传统的神经肌电图(ENMG)包括神经传导研究(NCS)和针肌电图(EMG),它被认为是这种情况下最具体的诊断评估。虽然CR在临床实践中经常遇到,但ENMG作为诊断工具在神经内科住院医师项目中很少被讨论。肌电图在诊断CR方面表现出适度的敏感性(50-71%),但极好的特异性(接近100%),它还可以提供有价值的病变慢性信息。在肌电图中,急性病变通常表现为去神经支配电位和募集减少,但运动单位动作电位(MUAP)形态保留。相比之下,慢性病变以重塑为特征,muap的持续时间、振幅和相数增加,同时再募集减少。本综述旨在概述NCS和EMG的作用,同时也介绍了在解释这些诊断模式时经常遇到的关键术语。
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引用次数: 0
The cerebellar involvement in essential tremor: the connecting roads. 小脑参与特发性震颤:连接道路。
IF 1.6 4区 医学 Q4 NEUROSCIENCES Pub Date : 2025-10-01 Epub Date: 2025-10-27 DOI: 10.1055/s-0045-1812324
Carlos Henrique Ferreira Camargo, Léo Coutinho, Luís Eduardo B M Zubko, Gustavo L Franklin, Hélio Afonso Ghizoni Teive

Essential tremor (ET) is the most prevalent movement disorder globally, affecting about 1% of the general population and 5% of those aged over 65 years. Characterized by involuntary, rhythmic oscillations, it primarily manifests as postural and kinetic tremors, predominantly in the upper limbs. Genetic studies, neuropathological examinations, neurophysiological assessments, and various neuroimaging techniques have demonstrated functional, neurotransmitter-related, and structural abnormalities within the cerebello-thalamo-cortical circuit. These findings collectively portray ET as a neurodegenerative syndrome with diverse etiologies and clinical manifestations, highlighting the involvement of the cerebellum.

特发性震颤(ET)是全球最普遍的运动障碍,影响约1%的总人口和5%的65岁以上老年人。它的特点是不自主的、有节奏的振荡,主要表现为体位性和运动性震颤,主要发生在上肢。遗传研究、神经病理学检查、神经生理学评估和各种神经成像技术已经证明,在小脑-丘脑-皮层回路中存在功能异常、神经递质相关和结构异常。这些发现共同描述ET是一种具有多种病因和临床表现的神经退行性综合征,突出了小脑的参与。
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引用次数: 0
Gilles de La Tourette syndrome: the contribution of Guinon. 图雷特氏症:吉农的贡献。
IF 1.6 4区 医学 Q4 NEUROSCIENCES Pub Date : 2025-10-01 Epub Date: 2025-10-27 DOI: 10.1055/s-0045-1812298
Hélio Afonso Ghizoni Teive, Francisco M Branco Germiniani, Marcus Vinícius Della Coletta, Carlos Henrique Ferreira Camargo, Léo Coutinho, Olivier Walusinski

We review the contributions of Georges Guinon, an eminent pupil of Professor Jean-Martin Charcot, to the clinical description of Gilles de La Tourette syndrome, including phenomenological definitions and the occurrence of psychiatric findings such as obsessive-compulsive behavior.

我们回顾了Jean-Martin Charcot教授的杰出学生Georges Guinon对Gilles de La Tourette综合征的临床描述的贡献,包括现象学定义和精神病学发现(如强迫症行为)的发生。
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引用次数: 0
Isolated complete oculomotor nerve palsy as a presentation of adult medulloblastoma. 成人成神经管细胞瘤表现为孤立的完全动眼神经麻痹。
IF 1.6 4区 医学 Q4 NEUROSCIENCES Pub Date : 2025-10-01 Epub Date: 2025-07-01 DOI: 10.1055/s-0045-1809883
Rafael Tuzino Leite Neves Maffei, Bruna Gutierres Gambirasio, Murillo Silva Catito, Sebastião Boanerges de Araujo Neto, Adrialdo José Santos
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引用次数: 0
期刊
Arquivos de neuro-psiquiatria
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