Essential Tremor

原发性震颤
  • 文章类型: Journal Article
    震颤,定义为“非自愿,有节奏的,身体部位的振荡运动“,是许多神经系统疾病的关键特征,包括帕金森病和特发性震颤。临床评估继续通过视觉观察进行,并在临床量表上进行量化。客观量化震颤的方法很有希望,但在各个中心仍未标准化。我们的中心进行全身行为测试与3D运动捕捉为临床和研究目的帕金森病患者,特发性震颤,和其他条件。这项研究的目的是评估几种候选处理管道在确认运动障碍患者的运动学数据中识别是否存在震颤的能力,并将其与运动障碍专家的专家评级进行比较。我们从我们中心收集了2272个独立的运动学数据记录的数据库,运动医生同时将其注释为存在或不存在的震颤。我们比较了六个独立的处理管道根据F1评分重新创建临床医生评级的能力,除了准确性,精度,和回忆。跨算法的性能通常是可比的。平均F1评分为0.84±0.02(平均值±SD;范围0.81-0.87)。第二性能最高的算法(交叉验证的F1=0.87)是混合的,其使用从具有现代支持向量机分类器的长期临床使用的算法改编的工程特征。一起来看,我们的研究结果表明,有可能更新传统的临床决策支持系统,以整合现代机器学习分类器,从而创建性能更好的工具.
    Tremor, defined as an \"involuntary, rhythmic, oscillatory movement of a body part\", is a key feature of many neurological conditions including Parkinson\'s disease and essential tremor. Clinical assessment continues to be performed by visual observation with quantification on clinical scales. Methodologies for objectively quantifying tremor are promising but remain non-standardized across centers. Our center performs full-body behavioral testing with 3D motion capture for clinical and research purposes in patients with Parkinson\'s disease, essential tremor, and other conditions. The objective of this study was to assess the ability of several candidate processing pipelines to identify the presence or absence of tremor in kinematic data from patients with confirmed movement disorders and compare them to expert ratings from movement disorders specialists. We curated a database of 2272 separate kinematic data recordings from our center, each of which was contemporaneously annotated as tremor present or absent by a movement physician. We compared the ability of six separate processing pipelines to recreate clinician ratings based on F1 score, in addition to accuracy, precision, and recall. The performance across algorithms was generally comparable. The average F1 score was 0.84±0.02 (mean ± SD; range 0.81-0.87). The second highest performing algorithm (cross-validated F1=0.87) was a hybrid that used engineered features adapted from an algorithm in longstanding clinical use with a modern Support Vector Machine classifier. Taken together, our results suggest the potential to update legacy clinical decision support systems to incorporate modern machine learning classifiers to create better-performing tools.
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  • 文章类型: Journal Article
    本文旨在根据主观自我报告绘制被诊断为特发性震颤(ET)的人的日常功能特征。此外,我们提供了一个客观的测量杯酒的任务。这项研究涉及20名被诊断为ET的参与者,他们完成了哥伦比亚大学原发性震颤残疾评估(CADET)问卷,其中包括与我们撰写的数字设备操作相关的五项额外任务。参与者还描述了他们实施的任务绩效修改。要创建客观的个人绩效配置文件,他们在使用传感器测量系统进行监控的同时执行了饮用杯子的任务。CADET的主观自我报告结果表明,参与者报告为难以修改或需要修改的最普遍的任务是写作,穿针,拿着一个杯子,用勺子,浇筑,并在手机上拍摄照片或视频。对参与者修改的分析表明,用两只手或一只手支撑另一只手握住物体是最普遍的类型。CADET总分与杯饮客观指标之间没有显着相关性。捕捉患者对其功能残疾的看法,除了客观的绩效衡量标准,设想为开发与个人概况相一致的定制干预措施做出贡献,即,基于患者/智能医疗。
    This paper aims to map the daily functional characteristics of people diagnosed with essential tremor (ET) based on their subjective self-reports. In addition, we provide objective measurements of a cup-drinking task. This study involved 20 participants diagnosed with ET who completed the Columbia University Assessment of Disability in Essential Tremor (CADET) questionnaire that included five additional tasks related to digital equipment operation we wrote. Participants also described task-performance modifications they implemented. To create objective personal performance profiles, they performed a cup-drinking task while being monitored using a sensor measurement system. The CADET\'s subjective self-report results indicate that the most prevalent tasks participants reported as having difficulty with or requiring modifications were writing, threading a needle, carrying a cup, using a spoon, pouring, and taking a photo or video on a mobile phone. Analysis of participants\' modifications revealed that holding the object with two hands or with one hand supporting the other were the most prevalent types. No significant correlation was found between the CADET total scores and the cup drinking objective measures. Capturing patients\' perspectives on their functional disability, alongside objective performance measures, is envisioned to contribute to the development of custom-tailored interventions aligned with individual profiles, i.e., patient-based/smart healthcare.
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  • 文章类型: Journal Article
    背景:当相同的磁共振图像引导聚焦超声(MRgFUS)丘脑切开术目标用于原发性震颤(ET)和震颤为主的帕金森病(TDPD)时,临床结果仍然存在很大差异。
    目标:我们的目标是完善TDPD与ET的MRgFUS丘脑切开术目标。
    方法:我们对32名TDPD患者的多中心队列和先前发表的79名ET患者的队列,使用术后3-12个月的手震颤评分进行了逐体素疗效和结构连接图。和24小时T1加权后MRgFUS脑图像。我们使用统一的帕金森病评定量表第III部分评分对9名TDPD患者的独立队列进行了验证。
    结果:MRgFUS后临床改善为45.9%±35.9%,55.5%±36%,ET为46.1%±18.6%,多中心TDPD和验证TDPD队列,分别。TDPD和ET功效图差异显著(ppermute<0.05),在x=-13.5;y=-15.0;z=1.5,比ET目标前3毫米,背3毫米,TDPD改善峰值(87%)。判别连通性预测是针对TDPD中的运动和运动前区域,以及ET中的运动和体感区域。该疾病特异性逐体素功效图可用于高精度估计TDPD患者的预后(R=0.8;R2=0.64;P<0.0001)。该模型使用9名TDPD患者的独立队列进行了验证(R=0.73;R2=0.53;P=0.025-体素分析)。
    结论:我们证明了TDPD中最有效的MRgFUS丘脑切开术目标是在腹侧中间核/口腹后界区。这一发现为有助于震颤控制的丘脑区域提供了新的见解,对改善治疗结果具有重要意义。©2024作者(S)。由WileyPeriodicalsLLC代表国际帕金森症和运动障碍协会出版的运动障碍。
    BACKGROUND: There remains high variability in clinical outcomes when the same magnetic resonance image-guided focused ultrasound (MRgFUS) thalamotomy target is used for both essential tremor (ET) and tremor-dominant Parkinson\'s disease (TDPD).
    OBJECTIVE: Our goal is to refine the MRgFUS thalamotomy target for TDPD versus ET.
    METHODS: We retrospectively performed voxel-wise efficacy and structural connectivity mapping using 3-12-month post-procedure hand tremor scores for a multicenter cohort of 32 TDPD patients and a previously published cohort of 79 ET patients, and 24-hour T1-weighted post-MRgFUS brain images. We validated our findings using Unified Parkinson\'s Disease Rating Scale part III scores for an independent cohort of nine TDPD patients.
    RESULTS: The post-MRgFUS clinical improvements were 45.9% ± 35.9%, 55.5% ± 36%, and 46.1% ± 18.6% for ET, multicenter TDPD and validation TDPD cohorts, respectively. The TDPD and ET efficacy maps differed significantly (ppermute < 0.05), with peak TDPD improvement (87%) at x = -13.5; y = -15.0; z = 1.5, ~3.5 mm anterior and 3 mm dorsal to the ET target. Discriminative connectivity projections were to the motor and premotor regions in TDPD, and to the motor and somatosensory regions in ET. The disorder-specific voxel-wise efficacy map could be used to estimate outcome in TDPD patients with high accuracy (R = 0.8; R2 = 0.64; P < 0.0001). The model was validated using the independent cohort of nine TDPD patients (R = 0.73; R2 = 0.53; P = 0.025-voxel analysis).
    CONCLUSIONS: We demonstrated that the most effective MRgFUS thalamotomy target in TDPD is in the ventral intermediate nucleus/ventralis oralis posterior border region. This finding offers new insights into the thalamic regions instrumental in tremor control, with pivotal implications for improving treatment outcomes. © 2024 The Author(s). Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.
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  • 文章类型: Journal Article
    背景:特发性震颤(ET)是一种常见的衰弱状态,然而,目前的治疗往往不能提供令人满意的缓解。经皮脊髓电刺激(tSCS)已成为一种潜在的非侵入性神经调节技术,能够破坏震颤的振荡活动。
    目的:本研究旨在研究在ET患者队列中,tSCS以频率依赖性方式破坏震颤的潜力。
    方法:18名ET患者完成了研究。实验包括60秒的姿势震颤记录,在震颤频率的tSCS期间,在1Hz时,在21Hz时,没有刺激,和斜方肌刺激。分析并比较了不同条件下的震颤频率和振幅。
    结果:我们发现震颤频率刺激下的震颤幅度降低仅在刺激的后半期才显著。相同的刺激导致最高数量的反应者。1Hz的tSCS在刺激的后半期显示出震颤幅度降低的趋势。21Hz的tSCS没有产生任何明显的震颤改变,而斜方肌刺激加剧了它。值得注意的是,在震颤频率刺激期间,一组反应者在震颤阶段和传递的刺激之间表现出一致的同步,指示震颤夹带。
    结论:当在受试者的震颤频率下递送时,宫颈tSCS有望减轻ET患者的姿势性震颤。所观察到的震颤幅度的变化可能是由tSCS对脊髓回路的调制引起的,通过影响传入途径或脊柱反射来破坏对肌肉的振荡驱动。然而,不能忽视脊柱中心和脊柱上中心之间相互作用的可能性.©2024作者(S)。由WileyPeriodicalsLLC代表国际帕金森症和运动障碍协会出版的运动障碍。
    BACKGROUND: Essential tremor (ET) is a common debilitating condition, yet current treatments often fail to provide satisfactory relief. Transcutaneous spinal cord electrical stimulation (tSCS) has emerged as a potential noninvasive neuromodulation technique capable of disrupting the oscillatory activity underlying tremors.
    OBJECTIVE: This study aimed to investigate the potential of tSCS to disrupt tremor in a frequency-dependent manner in a cohort of patients with ET.
    METHODS: Eighteen patients with ET completed the study. The experiment consisted of 60-s postural tremor recording, during tSCS at tremor frequency, at 1 Hz, at 21 Hz, no stimulation, and trapezius stimulation. Tremor frequency and amplitude were analyzed and compared across the conditions.
    RESULTS: We found tremor amplitude reduction at tremor frequency stimulation significant only during the second half of the stimulation. The same stimulation resulted in the highest number of responders. tSCS at 1 Hz showed a trend toward decreased tremor amplitude in the latter half of stimulation. tSCS at 21 Hz did not produce any significant alterations in tremor, whereas trapezius stimulation exacerbated it. Notably, during tremor frequency stimulation, a subgroup of responders exhibited consistent synchronization between tremor phase and delivered stimulation, indicating tremor entrainment.
    CONCLUSIONS: Cervical tSCS holds promise for alleviating postural tremor in patients with ET when delivered at the subject\'s tremor frequency. The observed changes in tremor amplitude likely result from the modulation of spinal cord circuits by tSCS, which disrupts the oscillatory drive to muscles by affecting afferent pathways or spinal reflexes. However, the possibility of an interplay between spinal and supraspinal centers cannot be discounted. © 2024 The Author(s). Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.
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  • 文章类型: Journal Article
    ET,最常见的神经系统疾病之一,在残疾方面提出了重大挑战。尽管近年来人们越来越关注ET,一直缺乏全面的文献计量分析。
    本研究利用WebofScience(WOS)数据库深入研究了2013年至2023年期间的基本震颤研究。利用CiteSpace进行定量分析,它检查了一系列指标,包括年度出版趋势,国家和机构的捐款,作者模式,关键术语,和参考共同引用的模式。主要目标是使用CiteSpace对过去十年的文献进行详细的视觉探索,精确定位了基本震颤研究中不断变化的景观和重点领域,从而为未来的调查工作奠定了基础。
    总共有2224部文学作品。近年来,已发表作品的数量一直在稳步上升。其中,大多数来自美国,路易斯,ElanD.是大多数出版物的出版商(161篇文章),运动障碍是被引用次数最多的期刊。关键词投稿和共引文献表明,特发性震颤的生理病理机制是近年来研究的主要热点,脑深部电刺激(DBS)和手术经颅磁共振引导聚焦超声(MRgFUS)在特发性震颤外科治疗中的最佳目标的确定以及肉毒毒素给药方法的创新研究。
    UNASSIGNED: ET, one of the most prevalent neurological disorders, presents a significant challenge in terms of disability. Despite the growing focus on ET in recent years, comprehensive bibliometric analysis has been lacking.
    UNASSIGNED: This study delves into essential tremor research covering the period from 2013 to 2023, utilizing the Web of Science (WOS) database. Employing CiteSpace for quantitative analysis, it examines an array of metrics including annual publication trends, contributions from countries and institutions, authorship patterns, key terminologies, and patterns of reference co-citation. The primary objective is to use CiteSpace for a detailed visual exploration of the literature over the last decade, pinpointing the evolving landscape and key areas of focus in essential tremor research, and thus providing a foundation for future investigative endeavors.
    UNASSIGNED: There were 2,224 literary works included in all. The amount of published works has been steadily rising in recent years. Of them, the majority originate from the United States, Louis, Elan D. is the publisher of the most publications (161 articles), and Movement Disorders is the journal that receives the most citations. The key words contribution and co-cited literatures suggest that the main research hotspots in recent years are the physiological and pathological mechanism of essential tremor, the determination of optimal targets for deep brain stimulation (DBS) and surgery transcranial magnetic resonance-guided focused ultrasound (MRgFUS) in the surgical management of essential tremor and the innovative research of botulinum toxin administration method.
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  • 文章类型: Case Reports
    腹核的深部脑刺激(VIM-DBS)被认为是治疗医学上顽固性特发性震颤(ET)的安全有效的治疗方法。然而,脑室肿大可以提供手术挑战,因为在手术过程中心室破裂的风险增加,具有脑室内出血和靶移位的潜在风险。
    在这种情况下,我们报道了1例72岁和69岁女性ET患者成功的双侧VIM-DBS,患者有显著的脑室增宽.VIM-DBS治疗提供了优异的震颤反应。五年后,由于Hakim-Adams三联征不完整,第一位患者植入了脑室-腹腔分流术,步态和认知的显著改善。
    据我们所知,我们首次报道了VIM-DBS在患有脑室增宽的ET患者中的应用,并阐明了VIM-DBS可以为患有难治性ET的患者提供良好的震颤反应。即使在明显的脑室增宽的情况下。
    UNASSIGNED: Deep brain stimulation of the nucleus ventralis intermedius (VIM-DBS) is considered a safe and effective treatment for medically intractable essential tremor (ET). However, ventriculomegaly can provide a surgical challenge, as there is an increased risk of breaching the ventricle during the procedure, with potential risk of intraventricular hemorrhage and target displacement.
    UNASSIGNED: In this case series, we report successful bilateral VIM-DBS in a 72-year-old and 69-year-old female ET patient with significant ventriculomegaly. VIM-DBS therapy provided an excellent tremor response. After 5 years, a ventriculoperitoneal shunt was implanted in the first patient due to an incomplete Hakim-Adams triad, with significant improvement in gait and cognition.
    UNASSIGNED: To the best of our knowledge, we present the first report on VIM-DBS in ET patients with ventriculomegaly and illustrate that VIM-DBS can provide an excellent tremor response in patients with medically intractable ET, even in the context of marked ventriculomegaly.
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  • 文章类型: Journal Article
    几项研究表明,原发性震颤(ET)存在非运动症状,包括REM睡眠行为障碍(RBD)。RBD是前驱帕金森病(PPD)的基本标准,暗示ET和PD之间有联系.我们的目的是评估患有RBD和PDD的ET患者的患病率和特征。RBD是通过问卷调查诊断的,其次是多导睡眠图。用结构化方案评估PPD危险因素和前驱标志物。患者的特征在于震颤特征。将有RBD(ET-RBD)和PPD(ET-PPD)的ET患者与无RBD(ET-nonRBD)和无PPD(ET-nonPPD)的患者进行比较,分别。还比较了ET-RBD患者与一组孤立的RBD(iRBD)的PPD特征。我们总共评估了64例ET患者。5人(8.3%)和4人(6.3%)有RBD和PPD的标准,分别。ET-RBD患者与ET-nonRBD患者没有差异,只是PPD的患病率较高。ET-RBD组和iRBD组(n=12)之间无显著差异。与ET-nonPPD相比,ET-PPD的DaT-Scans和RBD阳性患病率更高。3例ET-RBD患者有PPD,3例ET-PPD患者有RBD。ET患者的RBD和PPD均比一般老年人群更常见,但与特定的震颤特征无关。ET-RBD患者与iRBD患者无显著差异,容易发展为PD的群体。这些数据表明ET和PD之间存在联系,并且与显示ET人群中lewy体病理学和PD发生率增加的研究一致。
    Several studies suggested the presence of non-motor symptoms in Essential Tremor (ET), including REM sleep behavioral disorder (RBD). RBD is an essential criterion for Prodromal Parkinson\'s Disease (PPD), suggesting a link between ET and PD. Our objective was to assess the prevalence and features of ET patients with RBD and PDD. RBD was diagnosed by questionnaire screening, followed by polysomnography. PPD risk factors and prodromic markers were assessed with a structured protocol. Patients were characterized regarding tremor features. ET patients with RBD (ET-RBD) and PPD (ET-PPD) were compared to patients without RBD (ET-nonRBD) and without PPD (ET-nonPPD), respectively. ET-RBD patients were also compared with a group of isolated RBD (iRBD) regarding PPD features. We assessed a total of 64 ET patients. Five (8.3 %) and 4 (6.3 %) had criteria for RBD and PPD, respectively. ET-RBD patients did not differ from ET-nonRBD except for a higher prevalence of PPD. There were no significant differences between ET-RBD and iRBD (n = 12) groups. ET-PPD had a higher prevalence of positive DaT-Scans and RBD compared to ET-nonPPD. Three ET-RBD patients had PPD and 3 ET-PPD had RBD. Both RBD and PPD are more frequent in ET patients than in general aged population but not related with specific tremor features. ET-RBD patients did not differ significantly from iRBD patients, a group prone to develop PD. These data suggest a link between ET and PD and are in accordance with studies showing an increase incidence of lewy-body pathology and PD in ET populations.
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  • 文章类型: Journal Article
    已知帕金森病(PD)和特发性震颤(ET)的震颤具有重叠的特征,这使得临床医生难以区分它们。虽然深度学习在检测人类不明显的特征方面是强大的,不透明的训练模型在临床场景中是不切实际的,因为训练数据中的巧合相关性可能被模型用来进行分类,这可能导致误诊。这项工作旨在通过引入具有可解释AI(XAI)的多层BiLSTM网络来克服深度学习模型的上述挑战,该网络可以更好地解释颤抖特征并量化震颤分化中各自发现的重要区域。拟议的网络对PD进行了分类,ET,和饮酒过程中的正常震颤,并从震颤特征中获得贡献,(即,时间,频率,振幅,和动作)在分类任务中使用。分析表明,XAI-BiLSTM标记具有高震颤振幅的区域在分类中很重要,通过相关性分布与震颤位移幅度之间的高度相关性来验证。XAI-BiLSTM发现,从手臂休息到抬起(在饮酒周期中)的过渡阶段是对震颤进行分类的最重要动作。此外,XAI-BiLSTM揭示了仅有助于一种震颤类别分类的频率范围,这可能是克服重叠频率问题的潜在独特特征。通过揭示PD和ET震颤特有的关键时间和频率模式,这个提出的XAI-BiLSTM模型使临床医生能够做出更明智的分类,有可能降低误分类率并改善治疗结果.
    The tremors of Parkinson\'s disease (PD) and essential tremor (ET) are known to have overlapping characteristics that make it complicated for clinicians to distinguish them. While deep learning is robust in detecting features unnoticeable to humans, an opaque trained model is impractical in clinical scenarios as coincidental correlations in the training data may be used by the model to make classifications, which may result in misdiagnosis. This work aims to overcome the aforementioned challenge of deep learning models by introducing a multilayer BiLSTM network with explainable AI (XAI) that can better explain tremulous characteristics and quantify the respective discovered important regions in tremor differentiation. The proposed network classifies PD, ET, and normal tremors during drinking actions and derives the contribution from tremor characteristics, (i.e., time, frequency, amplitude, and actions) utilized in the classification task. The analysis shows that the XAI-BiLSTM marks the regions with high tremor amplitude as important in classification, which is verified by a high correlation between relevance distribution and tremor displacement amplitude. The XAI-BiLSTM discovered that the transition phases from arm resting to lifting (during the drinking cycle) is the most important action to classify tremors. Additionally, the XAI-BiLSTM reveals frequency ranges that only contribute to the classification of one tremor class, which may be the potential distinctive feature to overcome the overlapping frequencies problem. By revealing critical timing and frequency patterns unique to PD and ET tremors, this proposed XAI-BiLSTM model enables clinicians to make more informed classifications, potentially reducing misclassification rates and improving treatment outcomes.
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  • 文章类型: Journal Article
    磁共振引导聚焦超声(MRgFUS)是一种非侵入性损伤技术,用于治疗运动障碍,如特发性震颤(ET),帕金森病(PD),和X连锁肌张力障碍-帕金森病(XDP)。我们想报告我们在建立和发展MRgFUS计划和初步结果方面的经验。成人患者以震颤为主的PD(TDPD),ET,和XDP被考虑用于初步筛查(神经系统评估,颅骨密度比[SDR]测定)。符合条件的患者接受二次筛查(神经外科和神经心理学评估,精神病学和医学许可)。在手术过程中,神经麻醉师和神经科医生也在场,以监测患者并进行神经系统评估,分别。临床随访安排在治疗后2周,然后每三个月。共有30例患者接受MRgFUS治疗:22TDPD,6XDP,2ET平均年龄为55.7岁,大多数为男性(86.7%)。平均病程为8.6年。平均SDR为0.46。TDPD和ET的目标是丘脑对侧腹侧中间核;对于XDP,那是苍白丘脑道。平均最高温度为59.8oC;超声检查次数,7.3;和治疗时间,64.6min.大多数患者在手术后好转。暂时性术中不良事件(头痛,头晕)在20%的患者中报告,而术后事件(轻度虚弱,麻木)见于16.7%。只有26.7%的患者有随访数据。尽管遇到了独特的挑战,MRgFUS治疗在资源有限的环境中是可行的。必须采取其他步骤来开发和改进该计划。
    Magnetic resonance-guided focused ultrasound (MRgFUS) is a non-invasive lesioning technique used to treat movement disorders such as essential tremor (ET), Parkinson\'s disease (PD), and X-linked dystonia-parkinsonism (XDP). We would like to report our experience in establishing and developing our MRgFUS program and preliminary results. Adult patients with tremor-dominant PD (TDPD), ET, and XDP were considered for initial screening (neurologic evaluation, skull density ratio [SDR] determination). Eligible patients underwent secondary screening (neurosurgical and neuropsychological evaluation, psychiatric and medical clearance). During the procedure, a neuro-anesthesiologist and neurologist were also present to monitor the patient and perform neurologic evaluation, respectively. Clinical follow-up was scheduled at 2 weeks post-treatment, then at every 3 months. A total of 30 patients underwent MRgFUS treatment: 22 TDPD, 6 XDP, and 2 ET. The mean age was 55.7 years, and majority were male (86.7%). Mean disease duration was 8.6 years. Mean SDR was 0.46. The targets for TDPD and ET were the contralateral ventral intermediate nucleus of the thalamus; for XDP, it was the pallidothalamic tract. The mean maximum temperature was 59.8oC; number of sonocations, 7.3; and treatment time, 64.6 min. Majority of patients improved after the procedure. Transient intraprocedural adverse events (headache, dizziness) were reported in 20% of patients while post-procedural events (mild weakness, numbness) were seen in 16.7%. Only 26.7% of patients had follow-up data. Despite the unique challenges encountered, MRgFUS treatment is feasible in resource-limited settings. Additional steps would have to be made to develop and improve the program.
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  • 文章类型: Journal Article
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