neurological disorders

神经系统疾病
  • 文章类型: Journal Article
    没有有效的创伤性脑病综合征(TES)的诊断标准。在20世纪早期和中期,TES被描述为一些高暴露拳击手所经历的临床状况,并且被认为反映了慢性创伤性脑损伤。TES诊断的共识标准于2021年公布。我们将TES的共识标准回顾性地应用于20世纪发表的文章中描述的拳击手慢性脑损伤的病例,这些文章是从叙事和系统评价中获得的。样本包括1929年至1999年间发表的21篇文章中确定的157名拳击手。两位作者回顾了每个病例描述,并编码了TES的标准。对于核心临床特征,认知障碍占63.1%,在28.7%的病例中,这个人的认知功能似乎大致正常。25.5%存在神经行为失调。三分之一(34.4%)被确定为进步的,30.6%不是进步的,35.0%的病程无法明确确定。总的来说,29.9%符合TES共识标准,28.0%没有,42.0%的人没有足够的信息来做出诊断决定。TES,在20世纪,被描述为神经系统疾病,不是精神疾病-这支持了2021年共识小组的决定,即从核心诊断特征中删除原发性和继发性精神病诊断。需要未来的研究来确定是否,或者在多大程度上,以TES为特征的认知障碍或神经行为失调与慢性创伤性脑病神经病理学改变有关。
    There are no validated diagnostic criteria for traumatic encephalopathy syndrome (TES). During the early and middle 20th century, TES was described as a clinical condition that was experienced by some high-exposure boxers-and it was believed to reflect chronic traumatic brain injury. Consensus criteria for the diagnosis of TES were published in 2021. We applied the consensus criteria for TES retrospectively to cases of chronic brain damage in boxers described in articles published in the 20th century that were obtained from narrative and systematic reviews. The sample included 157 boxers identified in 21 articles published between 1929 and 1999. Two authors reviewed each case description and coded the criteria for TES. For the core clinical features, cognitive impairment was noted in 63.1%, and in 28.7% of cases the person\'s cognitive functioning appeared to be broadly normal. Neurobehavioral dysregulation was present in 25.5%. One third (34.4%) were identified as progressive, 30.6% were not progressive, and the course could not be clearly determined in 35.0%. In total, 29.9% met the TES consensus criteria, 28.0% did not, and 42.0% had insufficient information to make a diagnostic determination. TES, in the 20th century, was described as a neurological condition, not a psychiatric disorder-and this supports the decision of the 2021 consensus group to remove primary and secondary psychiatric diagnoses from being a core diagnostic feature. Future research is needed to determine whether, or the extent to which, cognitive impairment or neurobehavioral dysregulation described as characterizing TES are associated with chronic traumatic encephalopathy neuropathological change.
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  • 文章类型: Journal Article
    目的:大多数神经疾病患者需要终身医疗。欧洲泌尿外科协会(EAU)定期更新这些患者的诊断和治疗指南。本综述的目的是提供2024年更新的EAU神经学指南的摘要。
    方法:针对指南更新进行了涵盖2021-2023年时间段的结构化文献综述。根据文献数据为每个建议分配了证据水平和强度等级。
    神经系统疾病显著影响泌尿,性,和肠道功能,并且需要对神经科患者进行终身管理,以维持他们的生活质量并防止尿路恶化。早期诊断和有效治疗是关键,和全面的临床评估,包括尿动力学,是至关重要的。管理应根据个人需求进行定制,并应采用多学科方法,并解决性行为和生育问题。终身监测和随访强调了对神经科患者进行持续护理的重要性。
    结论:2024EAU神经病理学指南提供了最新的诊断证据概述,治疗,以及对神经科患者的随访。
    结果:神经系统疾病经常影响下尿路、性功能和肠道功能,患者需要终身治疗。我们总结了最新的欧洲泌尿外科协会关于泌尿外科的指南,为患者和护理人员提供最佳医疗保健支持的最新见解。
    OBJECTIVE: Most patients with neurourological disorders require lifelong medical care. The European Association of Urology (EAU) regularly updates guidelines for diagnosis and treatment of these patients. The objective of this review is to provide a summary of the 2024 updated EAU guidelines on neurourology.
    METHODS: A structured literature review covering the timeframe 2021-2023 was conducted for the guideline update. A level of evidence and a strength rating were assigned for each recommendation on the basis of the literature data.
    UNASSIGNED: Neurological conditions significantly affect urinary, sexual, and bowel function, and lifelong management is required for neurourological patients to maintain their quality of life and prevent urinary tract deterioration. Early diagnosis and effective treatment are key, and comprehensive clinical assessments, including urodynamics, are crucial. Management should be customised to individual needs and should involve a multidisciplinary approach and address sexuality and fertility. Lifelong monitoring and follow-up highlight the importance of continuous care for neurourological patients.
    CONCLUSIONS: The 2024 EAU guidelines on neurourology provide an up-to-date overview of available evidence on diagnosis, treatment, and follow-up for neurourological patients.
    RESULTS: Neurological disorders very frequently affect the lower urinary tract and sexual and bowel function and patients need lifelong management. We summarise the updated European Association of Urology guidelines on neurourology to provide patients and caregivers with the latest insights for optimal health care support.
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  • 文章类型: Journal Article
    背景:4岁以上儿童的慢性流口水率为0.5%,但在神经系统疾病患者中,这一比例上升到60%。身体和社会心理后果导致受影响患者的生活质量(QoL)下降;然而,这个问题仍然认识不足和处理不足。我们通过改良的Delphi调查进行了意大利共识,以讨论患有神经系统疾病的儿科患者流口水的当前治疗范式。
    方法:在查阅文献后,由10名专家组成的委员会定义了一些声明,这些声明将通过在线加密平台管理给多学科小组。问题的答案基于1-5李克特量表(1=强烈不同意;5=强烈同意)。得分分为1-2(不同意)和4-5(同意),而3被丢弃。当分歧或协议的总和≥75%时,就达成了共识。
    结果:15个陈述涵盖了三个主要主题,即临床表现和QoL,流口水的量化,和治疗策略。所有陈述均达成共识(≥75%同意)。55名意大利专家一致认为,应该对所有有复杂需求的儿童进行流口水评估,对生活质量有重大影响。应注意调查后唾液分泌过多,这是经常被忽视,但可能导致重要的临床后果。鉴于流口水的严重程度随着时间的推移而波动,其管理应以患者当前的需求为指导。此外,相对缺乏经过验证和通用的流口水量化量表限制了对治疗反应的评估.最后,共享的治疗范式是渐进的,在药物治疗之前进行保守治疗,仅对选定的病例保留手术。
    结论:这项研究证明了多学科方法对流口水的管理至关重要。各国专家一致认为,渐进式治疗可以减少并发症的发生,改善患者和护理人员的QoL,节省医疗资源。最后,这项研究强调了如何根据市场上现有的药物重新考虑治疗策略,症状的进展,和病人的需要。
    BACKGROUND: The rate of chronic drooling in children older than 4 years is 0.5%, but it rises to 60% in those with neurological disorders. Physical and psychosocial consequences lead to a reduction in the quality of Life (QoL) of affected patients; however, the problem remains under-recognized and under-treated. We conducted an Italian consensus through a modified Delphi survey to discuss the current treatment paradigm of drooling in pediatric patients with neurological disorders.
    METHODS: After reviewing the literature, a board of 10 experts defined some statements to be administered to a multidisciplinary panel through an online encrypted platform. The answers to the questions were based on a 1-5 Likert scale (1 = strongly disagree; 5 = strongly agree). The scores were grouped into 1-2 (disagreement) and 4-5 (agreement), while 3 was discarded. The consensus was reached when the sum of the disagreement or agreement was ≥75%.
    RESULTS: Fifteen statements covered three main topics, namely clinical manifestations and QoL, quantification of drooling, and treatment strategies. All statements reached consensus (≥75% agreement). The 55 Italian experts agreed that drooling should be assessed in all children with complex needs, having a major impact on the QoL. Attention should be paid to investigating posterior hypersalivation, which is often neglected but may lead to important clinical consequences. Given that the severity of drooling fluctuates over time, its management should be guided by the patients\' current needs. Furthermore, the relative lack of validated and universal scales for drooling quantification limits the evaluation of the response to treatment. Finally, the shared therapeutic paradigm is progressive, with conservative treatments preceding the pharmacological ones and reserving surgery only for selected cases.
    CONCLUSIONS: This study demonstrates the pivotal importance of a multidisciplinary approach to the management of drooling. National experts agree that progressive treatment can reduce the incidence of complications, improve the QoL of patients and caregivers, and save healthcare resources. Finally, this study highlights how the therapeutic strategy should be reconsidered over time according to the available drugs on the market, the progression of symptoms, and the patients\' needs.
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  • 文章类型: Journal Article
    一些癫痫综合征(睡眠相关的癫痫,SREs)与睡眠有很强的联系。合并症睡眠障碍在SRE患者中很常见,会对癫痫发作控制和生活质量产生负面影响。我们的目的是定义可能有SRE的患者的诊断途径的标准程序(方案1)以及SRE和睡眠障碍合并症患者的一般管理(方案2)。
    该项目是在欧洲神经病学会的主持下进行的,欧洲睡眠研究协会和欧洲国际抗癫痫联盟。该框架包含以下阶段:临床情景的概念;文献综述;关于标准程序的陈述。对于文献检索,采用了从系统评价到初步研究的逐步方法。已发表的研究来自美国国家医学图书馆的MEDLINE数据库和Cochrane图书馆。
    情况1:尽管证据质量低,针对特定的SRE,提供了有关记忆记忆评估的建议以及在家中或实验室捕获事件的工具。情况2:早期诊断和治疗SRE患者的睡眠障碍(尤其是呼吸系统疾病)可能有利于癫痫发作的控制。
    缺乏评估SRE患者的明确程序。提供的建议可能有助于标准化和改进特定SRE的诊断方法。强调了识别和治疗特定睡眠障碍对于SRE患者的管理和结果的重要性。
    Some epilepsy syndromes (sleep-related epilepsies, SREs) have a strong link with sleep. Comorbid sleep disorders are common in patients with SRE and can exert a negative impact on seizure control and quality of life. Our purpose was to define the standard procedures for the diagnostic pathway of patients with possible SRE (scenario 1) and the general management of patients with SRE and comorbidity with sleep disorders (scenario 2).
    The project was conducted under the auspices of the European Academy of Neurology, the European Sleep Research Society and the International League Against Epilepsy Europe. The framework entailed the following phases: conception of the clinical scenarios; literature review; statements regarding the standard procedures. For the literature search a stepwise approach starting from systematic reviews to primary studies was applied. Published studies were identified from the National Library of Medicine\'s MEDLINE database and Cochrane Library.
    Scenario 1: Despite a low quality of evidence, recommendations on anamnestic evaluation and tools for capturing the event at home or in the laboratory are provided for specific SREs. Scenario 2: Early diagnosis and treatment of sleep disorders (especially respiratory disorders) in patients with SRE are likely to be beneficial for seizure control.
    Definitive procedures for evaluating patients with SRE are lacking. Advice is provided that could be of help for standardizing and improving the diagnostic approach of specific SREs. The importance of identifying and treating specific sleep disorders for the management and outcome of patients with SRE is underlined.
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  • 文章类型: Journal Article
    经颅直流电刺激已显示出有希望的临床结果,导致对其临床效果进行循证审查的需求增加。
    我们召集了一组经颅直流电刺激专家,对超过1次刺激测试的临床试验进行系统回顾:疼痛,帕金森病运动功能与认知,中风运动功能和语言,癫痫,重度抑郁症,强迫症,Tourette综合征,精神分裂症,和毒瘾。
    专家被要求根据PRISMA指南的搜索方法进行系统评价。关于疗效的建议分为A级(绝对有效),B(可能有效),C(可能有效)或者没有推荐。我们评估了所有纳入研究的偏倚风险,以确认结果是否由潜在偏倚研究驱动。
    虽然大多数临床试验被设计为概念验证试验,本综述中分析的一些适应症可以被认为是绝对有效的(A级),比如抑郁症,并且可能有效(B级),比如神经性疼痛,纤维肌痛,偏头痛,术后患者自控镇痛和疼痛,帕金森病(运动和认知),行程(马达),癫痫,精神分裂症,酒精成瘾。偏倚评估表明,大多数研究的偏倚风险较低,偏倚的敏感性分析并未改变这些结果.效应大小从0.01到0.70不等,在大约8个条件下是显著的,效果最大的是术后急性疼痛,而中风运动恢复较小(与机器人疗法联合使用时无意义)。
    此处列出的所有建议均基于当前发布的PubMed索引数据。尽管在某些情况下有大量证据,必须强调的是,效果的大小和持续时间通常是有限的;因此,不同的研究设计需要进一步确定真正的临床影响.
    Transcranial direct current stimulation has shown promising clinical results, leading to increased demand for an evidence-based review on its clinical effects.
    We convened a team of transcranial direct current stimulation experts to conduct a systematic review of clinical trials with more than 1 session of stimulation testing: pain, Parkinson\'s disease motor function and cognition, stroke motor function and language, epilepsy, major depressive disorder, obsessive compulsive disorder, Tourette syndrome, schizophrenia, and drug addiction.
    Experts were asked to conduct this systematic review according to the search methodology from PRISMA guidelines. Recommendations on efficacy were categorized into Levels A (definitely effective), B (probably effective), C (possibly effective), or no recommendation. We assessed risk of bias for all included studies to confirm whether results were driven by potentially biased studies.
    Although most of the clinical trials have been designed as proof-of-concept trials, some of the indications analyzed in this review can be considered as definitely effective (Level A), such as depression, and probably effective (Level B), such as neuropathic pain, fibromyalgia, migraine, post-operative patient-controlled analgesia and pain, Parkinson\'s disease (motor and cognition), stroke (motor), epilepsy, schizophrenia, and alcohol addiction. Assessment of bias showed that most of the studies had low risk of biases, and sensitivity analysis for bias did not change these results. Effect sizes vary from 0.01 to 0.70 and were significant in about 8 conditions, with the largest effect size being in postoperative acute pain and smaller in stroke motor recovery (nonsignificant when combined with robotic therapy).
    All recommendations listed here are based on current published PubMed-indexed data. Despite high levels of evidence in some conditions, it must be underscored that effect sizes and duration of effects are often limited; thus, real clinical impact needs to be further determined with different study designs.
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  • 文章类型: Journal Article
    The use of Next Generation Sequencing such as Whole Genome Sequencing (WGS) is a promising step towards a better understanding and treatment of neurological diseases. WGS can result into unexpected information (incidental findings, IFs), and information with uncertain clinical significance. In the context of a Genome Canada project on \'Personalized Medicine in the Treatment of Epilepsy\', we intended to address these challenges surveying neurologists\' opinions about the type of results that should be returned, and their professional responsibility toward recontacting patients regarding new discovered mutations.
    Potential participants were contacted through professional organizations or direct invitations.
    A total of 204 neurologists were recruited. Fifty nine percent indicated that to be conveyed, WGS results should have a demonstrated clinical utility for diagnosis, prognosis or treatment. Yet, 41% deemed appropriate to return results without clinical utility, when they could impact patients\' reproductive decisions, or on patients\' request. Current use of targeted genetic testing and age of patients influenced respondents\' answers. Respondents stated that analysis of genomics data resulting from WGS should be limited to the genes likely to be relevant for the patient\'s specific medical condition (69%), so as to limit IFs. Respondents felt responsible to recontact patients and inform them about newly discovered mutations related to the medical condition that triggered the test (75%) for as long as they are following up on the patient (55%). Finally, 53.5% of the respondents felt responsible to recontact and inform patients of clinically significant, newly discovered IFs.
    Our results show the importance of formulating professional guidelines sensitive to the various - and sometimes opposite - viewpoints that may prevail within a same community of practice, as well as flexible so as to be attuned to the characteristics of the neurological conditions that triggered a WGS.
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  • 文章类型: Journal Article
    戊型肝炎病毒(HEV)感染是发达国家和发展中国家急性肝炎的主要原因之一。这种传染病在欧洲有很高的患病率和发病率。HEV感染对脆弱人群有更大的临床影响,如免疫抑制患者,孕妇和潜在肝病患者。因此,病毒性肝炎研究小组(GrupodeEstudiodeHepatitisVíricas,GEHEP)西班牙传染病和临床微生物学学会(SociedadEspañoladeEnfermedades传染病和微生物,SEIMC)认为准备一份共识文件以帮助做出有关诊断的决策非常重要,临床和治疗管理,和预防HEV感染。
    Hepatitis E virus (HEV) infection is one of the main causes of acute hepatitis in both developed and developing countries. This infectious disease has a high prevalence and incidence in Europe. HEV infection has a greater clinical impact in vulnerable populations, such as immunosuppressed patients, pregnant women and patients with underlying liver disease. Therefore, the Study Group for Viral Hepatitis (Grupo de Estudio de Hepatitis Víricas, GEHEP) of the Spanish Society of Infectious Diseases and Clinical Microbiology (Sociedad Española de Enfermedades Infecciosas y Microbiología Clínica, SEIMC) believed it very important to prepare a consensus document to help in decision-making regarding diagnosis, clinical and therapeutic management, and prevention of HEV infection.
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  • 文章类型: Journal Article
    Multiple sclerosis (MS) is a complex disease of the central nervous system. As new drugs are becoming available, knowledge on diagnosis and treatment must continuously evolve. There is therefore a need for a reference tool compiling current data on benefit and safety, to aid professionals in treatment decisions and use of resources across Europe. The European Committee of Treatment and Research in Multiple Sclerosis (ECTRIMS) and the European Academy of Neurology (EAN) have joined forces to meet this need. The objective was to develop an evidence-based clinical practice guideline for the pharmacological treatment of people with MS to guide healthcare professionals in the decision-making process.
    This guideline has been developed using the GRADE methodology and following the recently updated EAN recommendations for guideline development. Clinical questions were formulated in PICO format (patient, intervention, comparator, outcome) and outcomes were prioritized according to their relevance to clinical practice. An exhaustive literature search up to December 2016 was performed for each question and the evidence is presented narratively and, when possible, combined in a meta-analysis using a random-effects model. The quality of evidence for each outcome was rated into four categories - very high, high, low and very low - according to the risk of bias. GRADE evidence profiles were created using GRADEprofiler (GRADEpro) software (Version 3.6). The recommendations with assigned strength (strong, weak) were formulated based on the quality of evidence and the risk-benefit balance. Consensus between the panellists was reached by use of the modified nominal group technique.
    A total of 10 questions have been agreed, encompassing treatment efficacy, response criteria, strategies to address suboptimal response and safety concerns and treatment strategies in MS and pregnancy. The guideline takes into account all disease-modifying drugs approved by the European Medicine Agency at the time of publication. A total of 20 recommendations were agreed by the guideline working group members after three rounds of consensus.
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  • 文章类型: Journal Article
    To support clinical decision-making in central neurological disorders, a physical examination is used to assess responses to passive muscle stretch. However, what exactly is being assessed is expressed and interpreted in different ways. A clear diagnostic framework is lacking. Therefore, the aim was to arrive at unambiguous terminology about the concepts and measurement around pathophysiological neuromuscular response to passive muscle stretch.
    During two consensus meetings, 37 experts from 12 European countries filled online questionnaires based on a Delphi approach, followed by plenary discussion after rounds. Consensus was reached for agreement ≥75%.
    The term hyper-resistance should be used to describe the phenomenon of impaired neuromuscular response during passive stretch, instead of for example \'spasticity\' or \'hypertonia\'. From there, it is essential to distinguish non-neural (tissue-related) from neural (central nervous system related) contributions to hyper-resistance. Tissue contributions are elasticity, viscosity and muscle shortening. Neural contributions are velocity dependent stretch hyperreflexia and non-velocity dependent involuntary background activation. The term \'spasticity\' should only be used next to stretch hyperreflexia, and \'stiffness\' next to passive tissue contributions. When joint angle, moment and electromyography are recorded, components of hyper-resistance within the framework can be quantitatively assessed.
    A conceptual framework of pathophysiological responses to passive muscle stretch is defined. This framework can be used in clinical assessment of hyper-resistance and will improve communication between clinicians. Components within the framework are defined by objective parameters from instrumented assessment. These parameters need experimental validation in order to develop treatment algorithms based on the aetiology of the clinical phenomena.
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  • 文章类型: Journal Article
    背景:神经学家在临床实践中面临着各种急性和慢性(即神经退行性)疾病患者对干细胞方法的适用性的询问。挑战是为这些患者提供有关干细胞使用范围的准确信息,同时,赋予患者自主决定使用干细胞的能力。
    方法:印度神经病学学会委托召开专家组会议,为执业神经科医师提供咨询意见,为寻求有关干细胞方法的信息和建议的患者提供咨询。
    结论:在此类咨询过程中,应该强调的是,许多非专业网站提供的信息可能没有根据。此外,干细胞研究的标准建议,特别是,应严格遵守多层监督的应用,以确保干细胞在神经系统疾病中的安全和道德使用。
    BACKGROUND: Neurologists in their clinical practice are faced with inquiries about the suitability of stem cell approaches by patients with a variety of acute and chronic (namely neurodegenerative) disorders. The challenge is to provide these patients with accurate information about the scope of stem cell use as well as at the same time, empowering patients with the capacity to make an autonomous decision regarding the use of stem cells.
    METHODS: The Indian Academy of Neurology commissioned an Expert Group Meeting to formulate an advisory to practicing neurologists to counsel patients seeking information and advice about stem cell approaches.
    CONCLUSIONS: In the course of such counselling, it should be emphasized that the information provided by many lay websites might be unsubstantiated. Besides, standard recommendations for the stem cell research, in particular, the application of several layers of oversight should be strictly adhered in order to ensure safety and ethical use of stem cells in neurological disorders.
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