Essential Tremor

原发性震颤
  • 文章类型: Journal Article
    目的:在德国和欧洲临床指南中,经颅脑实质超声检查(TCS)已被推荐为帕金森病(PD)的早期和鉴别诊断工具。尽管如此,没有详细说明诊断问题需要检查的大脑结构以及成为合格研究者的要求.现在,这些问题已在德国神经病学会(DGN)2023年更新的PD临床指南中得到解决。
    方法:建议基于PRISMA(系统评价和荟萃分析的首选报告项目)指南的系统文献综述。
    结果:定义了三个诊断问题:(1)TCS在PD与非典型和继发性帕金森综合征的鉴别诊断中的准确性如何?(2)TCS在PD与特发性震颤的鉴别诊断中的准确性如何?(3)TCS在具有典型早期症状的人中诊断PD的准确性如何,与临床随访确定的诊断相比?针对这些问题制定了要评估的大脑结构和推荐水平。被视为合格的TCS研究者的培训要求由负责任的医学学会(德国医学超声学会,德国临床神经生理学和功能成像学会,DGKN)。最后,这些诊断问题的建议在指南委员会达成了强烈共识(各≥97%).这里,详细的审查和建议。
    结论:更新的指南阐明了TCS在PD中的诊断用途和局限性。
    OBJECTIVE: Transcranial brain parenchyma sonography (TCS) has been recommended as a tool for the early and differential diagnosis of Parkinson\'s disease (PD) in German and European clinical guidelines. Still, the brain structures to be examined for the diagnostic questions and the requirements for being a qualified investigator were not specified in detail. These issues have now been addressed in the 2023 update of the clinical guideline on PD by the German Society of Neurology (DGN).
    METHODS: The recommendations were based on a systematic literature review following PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines.
    RESULTS: Three diagnostic questions were defined: (1) What is the accuracy of TCS in the differential diagnosis of PD versus atypical and secondary Parkinsonian syndromes? (2) What is the accuracy of TCS in the differential diagnosis of PD versus essential tremor? (3) What is the accuracy of TCS in the diagnosis of PD in persons with typical early symptoms, compared with the diagnosis established by clinical follow-up? The brain structures to be assessed and the level of recommendation were formulated for these questions. The training requirements for being regarded as qualified TCS investigator were stipulated by the responsible medical societies (German Society of Ultrasound in Medicine, DEGUM; German Society for Clinical Neurophysiology and Functional Imaging, DGKN). Finally, the recommendations for these diagnostic questions reached strong consensus (each ≥ 97%) of the guideline committee. Here, the details of review and recommendations are presented.
    CONCLUSIONS: The updated guideline clarifies the diagnostic uses and limitations of TCS in PD.
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  • 文章类型: Journal Article
    原发性震颤(ET)是最常见的运动障碍,病因知之甚少。一些神经影像学研究报告小脑受累,而另一些则没有。这种差异可能源于动力不足的研究,磁共振成像(MRI)采集和处理中统计建模或变异的差异。为了解决这个问题,我们使用由PPMI和ADNI的匹配对照增强的局部高级ET数据集,调查了小脑结构差异.我们使用三种神经影像学生物标志物测试了小脑受累的假设:VBM,灰质/白质容积和小叶容积。此外,我们评估了统计模型和细分管道对结果的影响.结果表明,检测到的小脑结构变化随方法而异。右侧小脑灰质的显著减少和左侧小脑白质的增加是通过多种方法一致鉴定的仅有的两种生物标志物。结果还显示出基于SUIT的分割的大量体积高估-部分解释了以前的文献差异。这项研究表明,在MRI研究中可能过分强调了小脑参与ET的当前估计,并强调了方法敏感性分析对结果解释的重要性。需要具有大样本量和复制研究的ET数据集,以提高我们对小脑参与ET的区域特异性的理解。协议注册:本注册报告的第一阶段协议于2022年3月21日原则上被接受。协议,被杂志接受,可以找到:https://doi.org/10.6084/m9。图19697776。
    Essential tremor (ET) is the most prevalent movement disorder with poorly understood etiology. Some neuroimaging studies report cerebellar involvement whereas others do not. This discrepancy may stem from underpowered studies, differences in statistical modeling or variation in magnetic resonance imaging (MRI) acquisition and processing. To resolve this, we investigated the cerebellar structural differences using a local advanced ET dataset augmented by matched controls from PPMI and ADNI. We tested the hypothesis of cerebellar involvement using three neuroimaging biomarkers: VBM, gray/white matter volumetry and lobular volumetry. Furthermore, we assessed the impacts of statistical models and segmentation pipelines on results. Results indicate that the detected cerebellar structural changes vary with methodology. Significant reduction of right cerebellar gray matter and increase of the left cerebellar white matter were the only two biomarkers consistently identified by multiple methods. Results also show substantial volumetric overestimation from SUIT-based segmentation-partially explaining previous literature discrepancies. This study suggests that current estimation of cerebellar involvement in ET may be overemphasized in MRI studies and highlights the importance of methods sensitivity analysis on results interpretation. ET datasets with large sample size and replication studies are required to improve our understanding of regional specificity of cerebellum involvement in ET. PROTOCOL REGISTRATION: The stage 1 protocol for this Registered Report was accepted in principle on 21 March 2022. The protocol, as accepted by the journal, can be found at: https://doi.org/10.6084/m9.figshare.19697776 .
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  • 文章类型: Journal Article
    这篇共识论文的目的是讨论小脑在人类步态中的作用,以及它的评估和治疗。小脑疣对姿势控制至关重要。小脑确保将感觉信息映射到时间相关的运动命令中。步态的心理意象涉及包含小脑的内在联系的额顶叶网络。小脑患者的肌肉活动显示放电时间受损,影响协同作用的模式。姿态/步态共济失调是小脑疾病如退行性共济失调中的第一小脑缺陷之一,并且是具有高跌倒风险的致残症状。长时间的放电和增加的肌肉共激活可能与代偿机制和增强的身体摇摆有关,分别。原发性震颤常伴有轻度步态共济失调。越来越多的证据表明小脑皮层在特发性震颤的发病机理中起着重要作用。在多发性硬化症中,由于小脑和脊髓受累,平衡和步态受到影响,由于播散性脱髓鞘和神经变性损害本体感觉。在直立性震颤中,患者常表现为轻度至中度肢体和步态共济失调。震颤发生器可能位于后颅窝。串联步态在小脑疾病的早期阶段受损,可能在评估进行性共济失调的共济失调前期特别有用。诸如加速度计的可穿戴设备可以掌握步态时间和动力学参数的受损的关节间协调和增强的可变性。Kinect是一种有前途的低成本技术,可以获得可靠的步态测量和远程评估。正在开发深度学习方法,以帮助临床医生进行诊断和决策。小脑患者的运动适应受损。协调培训旨在提高跨步的协调策略和脚部位置,小脑患者受益于强烈的康复治疗。机器人训练是补充小脑常规康复和神经调节的一种有前途的方法。可穿戴动态矫形器代表了辅助步态的潜在辅助。专家小组一致认为,对小脑对步态控制的贡献的理解将导致更好地管理小脑共济失调,并可能有助于将步态参数用作未来临床试验的可靠生物标志物。
    The aim of this consensus paper is to discuss the roles of the cerebellum in human gait, as well as its assessment and therapy. Cerebellar vermis is critical for postural control. The cerebellum ensures the mapping of sensory information into temporally relevant motor commands. Mental imagery of gait involves intrinsically connected fronto-parietal networks comprising the cerebellum. Muscular activities in cerebellar patients show impaired timing of discharges, affecting the patterning of the synergies subserving locomotion. Ataxia of stance/gait is amongst the first cerebellar deficits in cerebellar disorders such as degenerative ataxias and is a disabling symptom with a high risk of falls. Prolonged discharges and increased muscle coactivation may be related to compensatory mechanisms and enhanced body sway, respectively. Essential tremor is frequently associated with mild gait ataxia. There is growing evidence for an important role of the cerebellar cortex in the pathogenesis of essential tremor. In multiple sclerosis, balance and gait are affected due to cerebellar and spinal cord involvement, as a result of disseminated demyelination and neurodegeneration impairing proprioception. In orthostatic tremor, patients often show mild-to-moderate limb and gait ataxia. The tremor generator is likely located in the posterior fossa. Tandem gait is impaired in the early stages of cerebellar disorders and may be particularly useful in the evaluation of pre-ataxic stages of progressive ataxias. Impaired inter-joint coordination and enhanced variability of gait temporal and kinetic parameters can be grasped by wearable devices such as accelerometers. Kinect is a promising low cost technology to obtain reliable measurements and remote assessments of gait. Deep learning methods are being developed in order to help clinicians in the diagnosis and decision-making process. Locomotor adaptation is impaired in cerebellar patients. Coordinative training aims to improve the coordinative strategy and foot placements across strides, cerebellar patients benefiting from intense rehabilitation therapies. Robotic training is a promising approach to complement conventional rehabilitation and neuromodulation of the cerebellum. Wearable dynamic orthoses represent a potential aid to assist gait. The panel of experts agree that the understanding of the cerebellar contribution to gait control will lead to a better management of cerebellar ataxias in general and will likely contribute to use gait parameters as robust biomarkers of future clinical trials.
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  • 文章类型: Journal Article
    2018年,关于震颤分类的新共识声明,由国际帕金森运动障碍协会震颤工作队,已发布。到目前为止,这篇文章在同行评审的国际期刊上被引用了400多次,并且由于社区的热烈欢迎,在各种会议上经常进行辩论。与以前的共识声明(1998)相比,主要的创新点是:1)根据临床表现(轴1)和病因(轴2)对震颤进行分类,因此使用了一种综合方法;2)将原发性震颤定义为一种综合征;3)对新类别原发性震颤的识别加,这源于通常与特发性震颤相关的软神经体征的不确定意义。在本文中,我们总结并解释了震颤新分类的最重要方面,突出了主要的新奇事物,它们的相关性,以及在临床实践中的应用。此外,我们讨论其可能的弱点,并反思迄今为止所发表的批评意见。我们认为这种新的震颤分类是全面的,严谨,并且一致,考虑到我们目前对震颤综合征的了解,这是我们目前能做的最好的事情。本文是DanielD.Truong编辑的特刊“Tremor”的一部分,MarkHallett,还有AasefShaikh.
    In 2018, the new Consensus Statement on the Classification of Tremors, by the Task Force on Tremor of the International Parkinson Movement Disorder Society, was published. So far, the article has been cited more than 400 times in peer-reviewed international journals and commonly debated in conferences and meetings due to an enthusiastic welcome from the community. Compared to the previous Consensus Statement (1998), the main novelties are: 1) the classification of tremor according to clinical manifestation (Axis 1) and etiology (Axis 2), and therefore the use of a syndromic approach; 2) the definition of essential tremor as a syndrome; 3) the recognition of the new category essential tremor plus, that derives from the uncertain significance of the soft neurological signs often associated with essential tremor. In this paper, we summarise and explain the most important aspects of the new classification of tremors, highlighting the main novelties, their relevance, and application in clinical practice. Moreover, we discuss its possible weakness and reflect on the critical comments made so far. We believe that this new tremor classification is comprehensive, rigorous, and consistent and, considering our current knowledge of tremor syndromes, it is the best we can do at present. This article is part of the Special Issue \"Tremor\" edited by Daniel D. Truong, Mark Hallett, and Aasef Shaikh.
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  • 文章类型: Journal Article
    目的:本研究使用国际帕金森与运动障碍协会(IPMDS)发布的震颤分类标准,包括美国耳鼻咽喉头颈外科学会(AAO-HNS)的喉特征,对声带震颤(VT)患者的临床表型进行了表征。
    方法:从2017年至2019年的参与者病历中提取VT表型描述符。临床表型描述包括:(a)首次任命的主要投诉和纪律,(b)人口统计,(c)震颤体分布,条件,频率,和进步,(d)加剧/缓解因素,(e)治疗方法,和(g)神经合并症。进行了描述性统计。
    结果:在符合入选标准的179人中,2/3为女性;震颤发作受声音(43%)或四肢(32%)影响,有2/3的震颤持续时间为3年或更长时间。原发性室性心动过速者首先看到耳鼻咽喉科或语言病理学(59%),而患有原发性四肢/头部震颤的人首先看到神经病学(36%)。文献通常省略了震颤的临床特征,例如(a)观察到的震颤状况(64%),(b)喉部特征(64%),和(c)震颤频率(92%)。因此,室性心动过速分类是基于49%患者的合并症(即,特发性震颤(48%),肌张力障碍(72%),和帕金森病(100%)),32%的人没有足够的文件进行分类。
    结论:大多数室性心动过速患者无法根据记录的临床特征进行分类,强调需要对影响言语结构的震颤进行一致的多学科评估。震颤的主要部位决定了所见的第一个学科。最常见的室性心动过速分类包括特发性震颤(47%),肌张力障碍(28%),帕金森病(7%),和孤立性室性心动过速(19%)。
    方法:4.
    OBJECTIVE: This study characterized the clinical phenotypes of individuals with vocal tremor (VT) using tremor classification criteria published by the International Parkinson and Movement Disorder Society (IPMDS) including laryngeal features from the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS).
    METHODS: VT phenotypic descriptors were extracted from participant medical records from 2017 to 2019. Clinical phenotype descriptors included the: (a) chief complaint and discipline for the first appointment, (b) demographics, (c) tremor body distribution, condition, frequency, and progression, (d) exacerbating/alleviating factors, (e) treatment approaches, and (g) neurologic comorbidities. Descriptive statistics were conducted.
    RESULTS: Of 179 meeting inclusion criteria, 2/3 were female; tremor onset affected voice (43%) or extremity (32%) and 2/3 were documented with tremor duration of 3 years or more. Those with primary VT first saw otolaryngology or speech language pathology (59%), whereas those with primary extremity/head tremor first saw neurology (36%). Documentation commonly omitted tremor clinical features such as (a) observed conditions of tremor (64%), (b) laryngeal features (64%), and (c) tremor frequency (92%). Thus, VT classification was based on comorbidity in 49% of patients (ie, essential tremor (48%), dystonia (72%), and Parkinson\'s disease (100%)) and 32% had inadequate documentation to classify.
    CONCLUSIONS: The majority of individuals with VT were unable to be classified based on documented clinical features highlighting the need for consistent multidisciplinary assessment of tremor affecting speech structures. The primary site of tremor determined the first discipline seen. Most commonly classified VT categories included essential tremor (47%), dystonia (28%), Parkinsonism (7%), and isolated VT (19%).
    METHODS: 4.
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  • 文章类型: Journal Article
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  • 文章类型: Journal Article
    BACKGROUND: This evidence-based guideline is an update of the 2005 American Academy of Neurology practice parameter on the treatment of essential tremor (ET).
    METHODS: A literature review using MEDLINE, EMBASE, Science Citation Index, and CINAHL was performed to identify clinical trials in patients with ET published between 2004 and April 2010.
    CONCLUSIONS: Conclusions and recommendations for the use of propranolol, primidone (Level A, established as effective); alprazolam, atenolol, gabapentin (monotherapy), sotalol, topiramate (Level B, probably effective); nadolol, nimodipine, clonazepam, botulinum toxin A, deep brain stimulation, thalamotomy (Level C, possibly effective); and gamma knife thalamotomy (Level U, insufficient evidence) are unchanged from the previous guideline. Changes to conclusions and recommendations from the previous guideline include the following: 1) levetiracetam and 3,4-diaminopyridine probably do not reduce limb tremor in ET and should not be considered (Level B); 2) flunarizine possibly has no effect in treating limb tremor in ET and may not be considered (Level C); and 3) there is insufficient evidence to support or refute the use of pregabalin, zonisamide, or clozapine as treatment for ET (Level U).
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  • 文章类型: English Abstract
    In Germany, deep brain stimulation (DBS) of the thalamic ventralis intermedius nucleus (VIM) is licensed for treatment of essential tremor in cases unresponsive to pharmacotherapy. Especially a bothersome hand tremor interfering with activities of daily living will improve, whereas head, tongue or vocal tremor shows less response. DBS was proven to be superior to lesional thalamotomy with better functional outcome and less adverse effects. The consensus statement presented here reflects the current recommendations of the German Deep Brain Stimulation Study Group for inclusion and exclusion criteria as well as for peri-, intra- and postoperative neurological management.
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