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“Fascicular twitch” on muscle ultrasonography in neuromuscular disorders: quantitative video-image analysis 神经肌肉疾病的肌肉超声“束性抽搐”:定量视频图像分析
IF 3.6 3区 医学 Q1 CLINICAL NEUROLOGY Pub Date : 2025-11-24 DOI: 10.1016/j.clinph.2025.2111447
Shun Matoba , Kenji Sekiguchi , Yoshikatsu Noda , Ryosuke Sugisawa , Hirotomo Suehiro , Katsuya Nishida , Riki Matsumoto

Objective

In contrast to needle electromyography, the diagnostic performance of muscle ultrasound (MUS) for differentiating neurogenic from myopathic disorders remains unclear. We examined the clinical utility of “fascicular twitch,” a visible movement of muscle bundles during isometric contraction, and evaluated its ability to differentiate neurogenic from myopathic changes.

Methods

We quantified fascicular twitches on MUS during isometric contraction as normalized twitch pixels (nTP) using an original image analysis method based on background subtraction. We analyzed echogenicity and nTP values in the biceps brachii muscle of 89 patients with neuromuscular disorders and 42 controls.

Results

The neurogenic group demonstrated substantially higher nTP values than the myopathic group and controls, achieving a sensitivity of 90.5% and specificity of 88.1% at an nTP cutoff of 0.04. Although echogenicity distinguished patients with neuromuscular disorders from controls, it failed to discriminate between the neurogenic and myopathic groups.

Conclusions

Quantification of fascicular twitch via nTP effectively differentiates neurogenic from myopathic disorders, thereby demonstrating that MUS is a valuable, noninvasive diagnostic method for assessing neuromuscular diseases.

Significance

This study introduces fascicular twitch quantification as a novel ultrasound biomarker that uniquely discriminates neurogenic from myopathic pathology, offering a high‑performance, noninvasive approach to neuromuscular diagnosis.
目的与针肌电图相比,肌肉超声(MUS)在神经源性和肌病性疾病的诊断价值尚不清楚。我们检查了“束性抽搐”的临床应用,这是一种肌肉束在等距收缩时的可见运动,并评估了其区分神经源性和肌病性变化的能力。方法采用基于背景减法的原始图像分析方法,将MUS在等距收缩过程中的肌束抽搐量化为归一化抽搐像素(nTP)。我们分析了89例神经肌肉疾病患者和42例对照者肱二头肌的回声性和nTP值。结果神经源性组的nTP值明显高于肌病组和对照组,在nTP截止值为0.04时,敏感性为90.5%,特异性为88.1%。虽然回声能区分神经肌肉疾病患者和对照组,但不能区分神经源性和肌病组。结论:通过nTP定量测量肌束抽搐可以有效区分神经源性和肌病性疾病,从而证明MUS是一种有价值的、无创的神经肌肉疾病诊断方法。本研究将束肌抽动量化作为一种新的超声生物标志物,独特地区分神经源性和肌病病理学,为神经肌肉诊断提供了一种高性能、无创的方法。
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引用次数: 0
Low intensity transcranial electric stimulation: Safety, ethical, legal regulatory and application guidelines (2017-2025: An update) - endorsed by the European Society for Brain Stimulation (ESBS) and by the International Federation for Clinical Neurophysiology (IFCN). 低强度经颅电刺激:安全性,伦理,法律法规和应用指南(2017-2025:更新)-由欧洲脑刺激学会(ESBS)和国际临床神经生理学联合会(IFCN)认可。
IF 3.6 3区 医学 Q1 CLINICAL NEUROLOGY Pub Date : 2025-11-23 DOI: 10.1016/j.clinph.2025.2111436
Andrea Antal, Jovana Bjekić, Ana Ganho-Ávila, Ivan Alekseichuk, Sara Assecondi, Til Ole Bergmann, Marom Bikson, Jerome Brunelin, Andre R Brunoni, Leigh Charvet, Robert Chen, Roi Cohen Kadosh, Lukas Diedrich, Giordano D'Urso, Roberta Ferrucci, Saša R Filipović, Paul B Fitzgerald, Agnes Flöel, Flavio Fröhlich, Mark S George, Roy H Hamilton, Jens Haueisen, Mark Hallett, Christoph S Herrmann, Friedhelm C Hummel, Shapour Jaberzadeh, Berthold Langguth, Michal Lavidor, Jean-Pascal Lefaucheur, Carlo Miniussi, Vera Moliadze, Mika Nikander, Stevan Nikolin, Michael A Nitsche, Alexander Opitz, Jacinta O'Shea, Frank Padberg, Christian Plewnia, Alberto Priori, Perianen Ramasawmy, Lais B Razza, Simone Rossi, John Rothwell, Maria A Rueger, Giulio Ruffini, Alexander T Sack, Ricardo Salvador, Klaus Schellhorn, Teresa Schuhmann, Yuichiro Shirota, Hartwig Roman Siebner, Axel Thielscher, Yoshikazu Ugawa, Susanne Uusitalo, Anna Wexler, Walter Paulus, Marie-Anne Vanderhasselt, Vincent Van Waes, Maximilian J Wessel, Miles Wischnewski, Chris Baeken, Ulf Ziemann

This guideline summarizes updated safety data (2017-2025) and provides expert recommendations on the use of low intensity transcranial electrical stimulation (tES) in humans. tES encompasses several techniques including transcranial direct current stimulation (tDCS), oscillatory transcranial direct current stimulation (otDCS), transcranial alternating current stimulation (tACS), transcranial random noise stimulation (tRNS), transcranial temporal interference stimulation (tTIS), and their combinations or variations. Across over 300,000 sessions involving healthy individuals, patients with neuropsychiatric conditions, and other clinical populations, no tES-related serious adverse events (AEs) have been reported. Moderate AEs are rare and limited to a small range of specific applications. Mild AEs are common and include transient symptoms such as localized sensations (e.g., tingling or burning), headaches, and fatigue. Similar mild AEs are also reported by individuals receiving placebo stimulation. The frequency, magnitude, and type of AEs are comparable across healthy, clinical, and vulnerable groups, including children, elderly, or pregnant women. Combined interventions (e.g., co-application with EEG, TMS, or neuroimaging) have not shown increased safety risks. Safety is well-established for both bipolar and multichannel tES when applied up to 4 mA and up to 60 min per day. Higher intensities and longer stimulation durations may also be safe. Nevertheless, the number of studies using intensities above 4 mA or stimulating longer than 60 min is low. Home-based use of treatments is growing rapidly, leveraging remote supervision to provide patients with greater access and enable repeated, sustained dosing paradigms. We recommend using screening and AE questionnaires in future controlled studies, in particular when planning to extend the stimulation parameters applied. We discuss recent regulatory and ethical issues.

本指南总结了最新的安全数据(2017-2025),并提供了关于在人类中使用低强度经颅电刺激(tES)的专家建议。tES包括几种技术,包括经颅直流电刺激(tDCS)、振荡式经颅直流电刺激(otDCS)、经颅交流电刺激(tACS)、经颅随机噪声刺激(tRNS)、经颅颞干扰刺激(tTIS)及其组合或变化。在涉及健康个体、神经精神疾病患者和其他临床人群的30多万次会议中,未报告tes相关的严重不良事件(ae)。中度ae很少见,仅限于小范围的特定应用。轻度ae很常见,包括短暂症状,如局部感觉(如刺痛或灼烧)、头痛和疲劳。接受安慰剂刺激的个体也报告了类似的轻度ae。在健康人群、临床人群和弱势群体(包括儿童、老年人或孕妇)中,ae的频率、程度和类型具有可比性。联合干预(如与脑电图、经颅磁刺激或神经成像联合应用)未显示出增加的安全风险。对于双极和多通道te,当每天使用高达4毫安、长达60分钟时,安全性是公认的。更高强度和更长的刺激持续时间也可能是安全的。然而,使用超过4毫安的强度或刺激时间超过60分钟的研究数量很少。以家庭为基础的治疗正在迅速增长,利用远程监督为患者提供更多的获取途径,并实现重复、持续的给药模式。我们建议在未来的对照研究中使用筛选和AE问卷,特别是当计划扩展刺激参数时。我们讨论了最近的监管和道德问题。
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引用次数: 0
Potassium channel dysfunction and distinct sensory axonal profiles in painful type 2 diabetes 疼痛性2型糖尿病的钾通道功能障碍和不同的感觉轴突分布
IF 3.6 3区 医学 Q1 CLINICAL NEUROLOGY Pub Date : 2025-11-21 DOI: 10.1016/j.clinph.2025.2111450
Cindy Shin-Yi Lin , Jowy Tani , Ting Wei Hsu , Yi-Chen Lin , Yun-Ru Liu , Jia-Ying Sung M.D. PhD.

Objective

This study aims to characterize sensory axonal changes in patients with non-painful and painful type 2 diabetes mellitus (T2DM) to explore the mechanisms underlying diabetic neuropathic pain in relation to pregabalin treatment.

Methods

Clinical evaluations, including nerve conduction studies, nerve excitability testing (NET) and visual analogue scale (VAS), were conducted on 200 T2DM, resulting 131 qualifying for inclusion. Cohort stratification utilized VAS scores: 43 had VAS ≥ 4 (painful), and 88 had VAS < 4 (non-painful). The painful cohort was categorized into low pain (4 ≤ VAS < 7) and high pain (VAS ≥ 7). A subgroup receiving pregabalin (15) was assessed to investigate neurophysiological differences.

Results

The painful cohort differed significantly from the non-painful cohort in sensory nerve excitability parameters including stimulus for 50% maximum amplitude, refractoriness, superexcitability, subexcitability, and depolarization thresholds. Higher VAS scores correlated with reduced TEd40(Accom) and TEh(overshoot). Pregabalin-treated patients demonstrated improved stimulus–response slope, S2 accommodation and TEd40(Accom) compared to untreated patients.

Conclusions

Our findings suggest that diabetic neuropathic pain may be associated with axonal hyperpolarization and accommodative properties with implications to potassium channel dysfunction.

Significance

These findings elucidate mechanisms of diabetic pain pathophysiology, highlighting the clinical significance of sensory NET and the role of potassium channel modulation in T2DM pain management.
目的观察非疼痛性和疼痛性2型糖尿病(T2DM)患者感觉轴突的变化,探讨普瑞巴林治疗对糖尿病神经性疼痛的影响机制。方法对200例T2DM患者进行神经传导、神经兴奋性测试(NET)和视觉模拟评分(VAS)等临床评价,其中131例符合纳入标准。队列分层采用VAS评分:43例VAS≥4(疼痛),88例VAS <; 4(无疼痛)。疼痛组分为低疼痛组(4≤VAS < 7)和高疼痛组(VAS≥7)。接受普瑞巴林治疗的亚组(15)评估神经生理差异。结果疼痛组与非疼痛组的感觉神经兴奋性参数有显著差异,包括50%最大振幅的刺激、难阻性、超兴奋性、亚兴奋性和去极化阈值。较高的VAS评分与降低的TEd40(Accom)和TEh(超调)相关。与未治疗的患者相比,接受普瑞巴林治疗的患者表现出更好的刺激反应斜率、S2调节和TEd40(Accom)。结论糖尿病神经性疼痛可能与轴突超极化和调节特性有关,并与钾通道功能障碍有关。这些发现阐明了糖尿病疼痛的病理生理机制,强调了感觉NET的临床意义和钾通道调节在T2DM疼痛管理中的作用。
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引用次数: 0
Routine EEG with quantitative analysis to detect delayed cerebral ischemia in aneurysmal subarachnoid hemorrhage 常规脑电图定量分析检测动脉瘤性蛛网膜下腔出血迟发性脑缺血。
IF 3.6 3区 医学 Q1 CLINICAL NEUROLOGY Pub Date : 2025-11-20 DOI: 10.1016/j.clinph.2025.2111449
Marina S. Cherchi , Miguel A. Hernández-Hernández , Javier Hernández-Cabello , Eduardo Torres Díez , Pedro Orizaola , Tetyana Stakhurska-Bilynska , Rubén Martín-Láez , José L. Fernández-Torre

Objective

To assess the ability of routine electroencephalography (rEEG) to predict delayed cerebral ischemia (DCI) in aneurysmal subarachnoid hemorrhage (aSAH).

Methods

Single-center prospective study including adult patients with aSAH. Two rEEG recordings with quantitative analysis and transcranial Doppler sonography (TCD) were performed, within the first 72 h (EEG1 and TCD1) and between days 4–7 (EEG2 and TCD2). Multivariate logistic regression was performed to identify DCI predictors.

Results

Seventeen (25.4 %) of 67 patients developed DCI. In qualitative analysis, no patient with a normal EEG1 had DCI, while in EEG2, the absence of bilateral posterior alpha rhythm was more frequently observed in patients with DCI (52.9 % vs 20 %; p = 0.009). Quantitative EEG2 analysis revealed significantly higher total, delta, and theta power in DCI patients, with lower alpha–beta/theta-delta ratio and edge. Delta power ≥ 4.5 μV in EEG2 showed higher sensitivity than vasospasm on TCD2 but lower specificity. Age (OR 1.08; p = 0.04), vasospasm on TCD2 (OR 41.01; p = 0.003) and delta power ≥ 4.5 μV in EEG2 (OR 4.30; p = 0.06) were independent predictors of DCI.

Conclusions

rEEG with quantitative analysis is a useful tool for predicting DCI.

Significance

Integration of rEEG and TCD may improve detection of DCI in aSAH patients.
目的:探讨常规脑电图(rEEG)对动脉瘤性蛛网膜下腔出血(aSAH)迟发性脑缺血(DCI)的预测价值。方法:纳入成年aSAH患者的单中心前瞻性研究。在头72小时(EEG1和TCD1)和第4-7天(EEG2和TCD2)进行2次rEEG定量分析和经颅多普勒超声(TCD)记录。采用多元逻辑回归来确定DCI预测因子。结果:67例患者中17例(25.4%)发生DCI。在定性分析中,EEG1正常的患者没有DCI,而在EEG2中,DCI患者更常观察到双侧后α节律缺失(52.9% vs 20%; p = 0.009)。定量EEG2分析显示,DCI患者的总功率、δ和θ功率显著较高,α - β / δ - δ比值和边缘较低。EEG2 δ功率≥4.5 μV对TCD2的敏感性高于血管痉挛,但特异性较低。年龄(OR 1.08; p = 0.04)、TCD2血管痉挛(OR 41.01; p = 0.003)和EEG2 δ功率≥4.5 μV (OR 4.30; p = 0.06)是DCI的独立预测因子。结论:rEEG结合定量分析是预测DCI的有效工具。意义:结合rEEG和TCD可提高aSAH患者DCI的检出率。
{"title":"Routine EEG with quantitative analysis to detect delayed cerebral ischemia in aneurysmal subarachnoid hemorrhage","authors":"Marina S. Cherchi ,&nbsp;Miguel A. Hernández-Hernández ,&nbsp;Javier Hernández-Cabello ,&nbsp;Eduardo Torres Díez ,&nbsp;Pedro Orizaola ,&nbsp;Tetyana Stakhurska-Bilynska ,&nbsp;Rubén Martín-Láez ,&nbsp;José L. Fernández-Torre","doi":"10.1016/j.clinph.2025.2111449","DOIUrl":"10.1016/j.clinph.2025.2111449","url":null,"abstract":"<div><h3>Objective</h3><div>To assess the ability of routine electroencephalography (rEEG) to predict delayed cerebral ischemia (DCI) in aneurysmal subarachnoid hemorrhage (aSAH).</div></div><div><h3>Methods</h3><div>Single-center prospective study including adult patients with aSAH. Two rEEG recordings with quantitative analysis and transcranial Doppler sonography (TCD) were performed, within the first 72 h (EEG1 and TCD1) and between days 4–7 (EEG2 and TCD2). Multivariate logistic regression was performed to identify DCI predictors.</div></div><div><h3>Results</h3><div>Seventeen (25.4 %) of 67 patients developed DCI. In qualitative analysis, no patient with a normal EEG1 had DCI, while in EEG2, the absence of bilateral posterior alpha rhythm was more frequently observed in patients with DCI (52.9 % vs 20 %; p = 0.009). Quantitative EEG2 analysis revealed significantly higher total, delta, and theta power in DCI patients, with lower alpha–beta/theta-delta ratio and edge. Delta power ≥ 4.5 μV in EEG2 showed higher sensitivity than vasospasm on TCD2 but lower specificity. Age (OR 1.08; p = 0.04), vasospasm on TCD2 (OR 41.01; p = 0.003) and delta power ≥ 4.5 μV in EEG2 (OR 4.30; p = 0.06) were independent predictors of DCI.</div></div><div><h3>Conclusions</h3><div>rEEG with quantitative analysis is a useful tool for predicting DCI.</div></div><div><h3>Significance</h3><div>Integration of rEEG and TCD may improve detection of DCI in aSAH patients.</div></div>","PeriodicalId":10671,"journal":{"name":"Clinical Neurophysiology","volume":"182 ","pages":"Article 2111449"},"PeriodicalIF":3.6,"publicationDate":"2025-11-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145602517","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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
Trigemino-cervical reflex can be recorded simultaneously with other trigeminal reflexes after V3 stimulation under general anesthesia 全身麻醉V3刺激后三叉-颈反射可与其他三叉反射同时记录。
IF 3.6 3区 医学 Q1 CLINICAL NEUROLOGY Pub Date : 2025-11-20 DOI: 10.1016/j.clinph.2025.2111448
Denise Lima Medeiros de Melo, Dayanne Rodrigues da Cunha Alves Bento Oliveira, Patricia Lago dos Santos Madureira
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引用次数: 0
Deriving reference limits from historical data – A comparison of four novel methods 从历史数据中得出参考限度——四种新方法的比较
IF 3.6 3区 医学 Q1 CLINICAL NEUROLOGY Pub Date : 2025-11-20 DOI: 10.1016/j.clinph.2025.2111451
Tomasz Szymon Szczepanski , Petter Moe Omland , Øystein Dunker , Trond Sand , Martijn Tannemaat , Robert Reijntjes , Anis Yazidi , Kerstin Bach , John Anker Zwart , Joe Jabre , Kristian Bernhard Nilsen

Objective

To compare four novel methods – extrapolated norms (E-norms), extrapolated reference values (E-Ref), multivariable extrapolated reference values (MeRef) and mixture model clustering (MMC) – for obtaining reference limits for nerve conduction studies (NCS) from historical data containing both normal and abnormal studies.

Methods

Reference limits for 29 commonly clinically used NCS measurements were calculated from a historical database containing measurements from 24 618 patients using E-norms, E-ref, MeRef and MMC. The resulting reference limits were compared to reference limits for NCS calculated from 680 healthy subjects using Youden’s J statistics.

Results

Except for distal latencies, E-norms produced reference limits with the highest Youden’s J statistics with higher sensitivity, but lower specificity. E-Ref, MeRef and MMC produced reference limits with high specificity, but lower sensitivity than E-norms.

Conclusions

There are substantial differences in the performance of E-norms, E-Ref, MeRef and MMC. A dynamic approach, where the methods used are changed based on the type of NCS measurement and the amount of historical data available, may yield the highest accuracy.

Significance

When combining the different novel methods, it is possible to create clinically useful reference limits using historical data.
目的比较外推规范(e -norm)、外推参考值(E-Ref)、多变量外推参考值(MeRef)和混合模型聚类(MMC)四种新方法从包含正常和异常研究的历史数据中获得神经传导研究(NCS)的参考限。方法采用e -norm、E-ref、MeRef和MMC,从包含24618例患者测量值的历史数据库中计算29种临床常用NCS测量值的参考限。使用Youden 's J统计方法将所得参考限值与680名健康受试者计算的NCS参考限值进行比较。结果除远端潜伏期外,e -规范产生的参考限具有最高的约登J统计量,敏感性较高,但特异性较低。E-Ref、MeRef和MMC的参考限特异性高,但灵敏度低于e -规范。结论e -norm、E-Ref、MeRef和MMC的表现存在显著差异。动态方法根据NCS测量的类型和可用的历史数据量改变所使用的方法,可能产生最高的准确性。当结合不同的新方法时,可以使用历史数据创建临床有用的参考界限。
{"title":"Deriving reference limits from historical data – A comparison of four novel methods","authors":"Tomasz Szymon Szczepanski ,&nbsp;Petter Moe Omland ,&nbsp;Øystein Dunker ,&nbsp;Trond Sand ,&nbsp;Martijn Tannemaat ,&nbsp;Robert Reijntjes ,&nbsp;Anis Yazidi ,&nbsp;Kerstin Bach ,&nbsp;John Anker Zwart ,&nbsp;Joe Jabre ,&nbsp;Kristian Bernhard Nilsen","doi":"10.1016/j.clinph.2025.2111451","DOIUrl":"10.1016/j.clinph.2025.2111451","url":null,"abstract":"<div><h3>Objective</h3><div>To compare four novel methods – extrapolated norms (E-norms), extrapolated reference values (E-Ref), multivariable extrapolated reference values (MeRef) and mixture model clustering (MMC) – for obtaining reference limits for nerve conduction studies (NCS) from historical data containing both normal and abnormal studies.</div></div><div><h3>Methods</h3><div>Reference limits for 29 commonly clinically used NCS measurements were calculated from a historical database containing measurements from 24<!--> <!-->618 patients using E-norms, E-ref, MeRef and MMC. The resulting reference limits were compared to reference limits for NCS calculated from 680 healthy subjects using Youden’s J statistics.</div></div><div><h3>Results</h3><div>Except for distal latencies, E-norms produced reference limits with the highest Youden’s J statistics with higher sensitivity, but lower specificity. E-Ref, MeRef and MMC produced reference limits with high specificity, but lower sensitivity than E-norms.</div></div><div><h3>Conclusions</h3><div>There are substantial differences in the performance of E-norms, E-Ref, MeRef and MMC. A dynamic approach, where the methods used are changed based on the type of NCS measurement and the amount of historical data available, may yield the highest accuracy.</div></div><div><h3>Significance</h3><div>When combining the different novel methods, it is possible to create clinically useful reference limits using historical data.</div></div>","PeriodicalId":10671,"journal":{"name":"Clinical Neurophysiology","volume":"182 ","pages":"Article 2111451"},"PeriodicalIF":3.6,"publicationDate":"2025-11-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145616692","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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
Nerve ultrasound in asymptomatic hereditary transthyretin amyloidosis carriers 无症状遗传性甲状腺转蛋白淀粉样变携带者的神经超声检查
IF 3.6 3区 医学 Q1 CLINICAL NEUROLOGY Pub Date : 2025-11-20 DOI: 10.1016/j.clinph.2025.2111446
Wilder-Smith E , Tournev I , Chamova T , Asenov O , Bohlhalter S , Antimov P , Mihaylova V

Objective

The aim of our study was to perform high-resolution nerve ultrasound in asymptomatic carriers of transthyretin (TTR) p.Glu89Gln (p.Glu109Gln) mutation and to evaluate its role as a complementary non-invasive diagnostic tool in early detection of amyloid deposits in peripheral nerves.

Methods

The study included 21 asymptomatic carriers of TTR p.Glu89Gln (p.Glu109Gln). History, neurological examination, nerve conduction studies, sympathetic skin response, SudoScan and high-resolution nerve ultrasound were performed.

Results

Ultrasound pattern sum score was abnormal in 70% of the carriers. Despite the younger age of male carriers compared to the female carriers the abnormal ultrasound findings were more frequently encountered in males. The latter also demonstrated more pronounced cross-sectional area enlargement of the peroneal nerve at the fibular head and had more frequently ultrasound evidence of median nerve swelling at the wrist. Sural nerves were not enlarged in contrast to radial and fibular superficial nerves which showed male preponderance.

Conclusions

Our data suggest early ultrasound detection of amyloid deposition in presymptomatic TTR p. Glu89Gln (p.Glu109Gln) carriers and demonstrated sex related differences with earlier amyloid deposition in male carriers.

Significance

As a non-invasive tool nerve ultrasound may be added to the established follow up program for the asymptomatic TTR carriers.
目的对无症状转甲状腺素(TTR) p.Glu89Gln (p.Glu109Gln)突变携带者进行高分辨率神经超声检查,并评价其作为一种辅助的非侵入性诊断工具在早期检测周围神经淀粉样蛋白沉积中的作用。方法选取21例无症状TTR p.Glu89Gln (p.Glu109Gln)携带者。进行病史、神经学检查、神经传导研究、交感皮肤反应、SudoScan和高分辨率神经超声检查。结果70%的携带者超声模式综合评分异常。尽管男性携带者比女性携带者年龄小,但异常超声结果在男性中更常见。后者也表现出腓骨头腓神经更明显的横截面积扩大,并且在手腕处有更频繁的正中神经肿胀的超声证据。腓肠神经没有扩张,而桡骨和腓骨浅神经则以男性为主。结论早期超声可检出TTR p. Glu89Gln (p. glu109gln)症状前携带者的淀粉样蛋白沉积,且与男性携带者的早期淀粉样蛋白沉积存在性别差异。意义:神经超声作为一种无创工具,可加入到已建立的无症状TTR携带者随访计划中。
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引用次数: 0
Hemispheric asymmetry of the ipsilateral silent period following voluntary movement of the opposite hand 另一只手自主运动后同侧沉默期的半球不对称。
IF 3.6 3区 医学 Q1 CLINICAL NEUROLOGY Pub Date : 2025-11-19 DOI: 10.1016/j.clinph.2025.2111444
Sabira Alibhai-Najarali , John P. de Grosbois , Aaron E. Philipp-Muller , Jennifer Shao , Matthew J. Hawken , Matea Skenderija , Jed A. Meltzer

Objective

To investigate whether post-movement beta rebound (PMBR), a proposed marker of cortical inhibition, reflects interhemispheric inhibition as measured by the ipsilateral silent period (iSP) following voluntary movement and to assess hemispheric asymmetries.

Methods

Twenty right-handed participants underwent transcranial magnetic stimulation (TMS) to elicit iSPs at baseline (no button-press) and eight timepoints following movement of the opposite hand, iSPs and motor-evoked potentials (MEPs) were recorded for both hemispheres. Six participants completed magnetoencephalography (MEG) to characterize beta oscillatory activity during the task. Five participants completed the task without the TMS as a control.

Results

iSP magnitude did not significantly change during PMBR period. Instead, post-movement iSP increased in the right hemisphere and decreased in the left, yielding a significant hemispheric difference (p = 0.014). MEG data confirmed symmetrical PMBR across hemispheres. MEPs showed increased excitability post-movement, particularly at early timepoints. Movement alone, without TMS, did not account for hemispheric asymmetry.

Conclusions

PMBR and iSP appear to reflect distinct inhibitory mechanisms. The dissociation suggests PMBR does not index transcallosal inhibition (TCI). Hemispheric asymmetry in iSP suggests stronger TCI following non-dominant hand movements.

Significance

The dissociation between iSP and PMBR underscores the complexity of motor control, informs understanding lateralized motor function and rehabilitation.
目的:研究运动后β反弹(PMBR),一种被提出的皮层抑制标志物,是否反映了自主运动后同侧沉默期(iSP)测量的半球间抑制,并评估半球不对称性。方法:采用经颅磁刺激(TMS)诱发20名右撇子参与者在基线(不按按钮)和对侧手运动后8个时间点的iSPs,记录双脑的iSPs和运动诱发电位(MEPs)。六名参与者完成了脑磁图(MEG)来表征任务期间的β振荡活动。五名参与者在没有经颅磁刺激作为对照的情况下完成了任务。结果:PMBR期间iSP大小无明显变化。相反,运动后的iSP在右半球增加,在左半球减少,产生显著的半球差异(p = 0.014)。脑磁图数据证实了跨半球的对称PMBR。mep在运动后表现出兴奋性增强,尤其是在早期。单独运动,不经颅磁刺激,不能解释半球不对称。结论:PMBR和iSP似乎反映了不同的抑制机制。分离表明PMBR不能反映经胼胝体抑制(TCI)。iSP的半球不对称表明非优势手运动后TCI增强。意义:iSP和PMBR之间的分离强调了运动控制的复杂性,有助于理解侧化运动功能和康复。
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引用次数: 0
Youth with essential tremor differ from age-matched controls in multiple aspects of visually-guided reaching movements 患有特发性震颤的青少年在视觉引导的伸手动作的多个方面与年龄匹配的对照组不同
IF 3.6 3区 医学 Q1 CLINICAL NEUROLOGY Pub Date : 2025-11-19 DOI: 10.1016/j.clinph.2025.2111445
Nicholas Cothros , Catherine Lowrey , Stephen H. Scott , Alex Medina Escobar , Sean Dukelow , Davide Martino , Tamara Pringsheim

Objective

Essential tremor (ET) likely includes deficits outside tremor, though this is under-examined in youth. We tested the hypothesis that reaching movements in youth with ET are characterized by greater incoordination.

Methods

Fifteen youth with ET (ages 11 to 17) recruited from the Calgary Tourette and Pediatric Movement Disorders Clinic completed a visually-guided reaching task using the Kinarm Exoskeleton Lab. Performance in several kinematic parameters was compared with age-matched controls.

Results

Youth with ET differed from controls in multiple parameters. In the dominant arm, the initial portion of reaching movements was slower and smaller-amplitude, with longer reaction time and greater posture speed at movement end when the hand should be at rest. In the non-dominant arm, those with ET differed from controls in a larger number of parameters. All but three with ET were impaired in at least one task parameter, scoring outside age-predicted norms.

Conclusion

Our study supports the notion of ET as a disorder with abnormalities beyond tremor, and that motoric abnormalities are demonstrable in youth with ET.

Significance

This is the first study showing youth with ET differ from age-matched controls in multiple aspects of reaching movements, including initial trajectory control, movement smoothness, overall trajectory, and endpoint control.
目的特发性震颤(ET)可能包括震颤以外的缺陷,尽管这在青少年中尚未得到充分研究。我们测试了一个假设,即ET青少年的伸展运动的特点是更大的不协调。方法从卡尔加里抽动秽语和儿童运动障碍诊所招募了15名ET青少年(11至17岁),使用Kinarm外骨骼实验室完成了一项视觉引导的到达任务。将几个运动学参数的表现与年龄匹配的对照组进行比较。结果ET患儿与对照组在多个参数上存在差异。优势臂到达动作的起始部分速度较慢,幅度较小,动作结束时反应时间较长,动作结束时姿态速度较大。在非优势组中,ET患者在许多参数上与对照组不同。除了三个外,所有ET患者在至少一个任务参数上都受损,得分超出了年龄预测标准。结论:我们的研究支持ET是一种除震颤外的异常疾病的观点,并且运动异常在ET的青少年中是明显的。意义:这是第一个研究表明ET的青少年在到达运动的多个方面与年龄匹配的对照组不同,包括初始轨迹控制、运动平滑度、总体轨迹和终点控制。
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引用次数: 0
Between Mind and Movement: Electrophysiological insights into functional dystonia 在意识和运动之间:功能性肌张力障碍的电生理学见解
IF 3.6 3区 医学 Q1 CLINICAL NEUROLOGY Pub Date : 2025-11-17 DOI: 10.1016/j.clinph.2025.2111442
Alexey Sedov , Ulia Semenova , Anna Gamaleya , Alexey Tomskiy , Aasef G. Shaikh
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引用次数: 0
期刊
Clinical Neurophysiology
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