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Electrodiagnostic criteria for neuromuscular transmission disorders suggested by a European consensus group 欧洲共识小组提出的神经肌肉传递障碍的电诊断标准
IF 2 Q3 NEUROSCIENCES Pub Date : 2025-01-01 DOI: 10.1016/j.cnp.2025.02.011
H. Tankisi , K. Pugdahl , B. Johnsen , J.P. Camdessanché , M. de Carvalho , P. Fawcett , A. Labarre-Vila , R. Liguori , W. Nix , I. Schofield , A. Fuglsang-Frederiksen

Objective

Electrodiagnostic testing plays an important role in diagnosing disorders of neuromuscular transmission (NMT), especially in seronegative myasthenia gravis. However, electrodiagnostic criteria for the diagnosis are sparse. This study aimed at inferring evidence-based recommendations for the electrodiagnostic examination of NMT disorders.

Methods

A total of 164 cases with a consensus diagnosis of NMT disorder obtained by peer review by eight experienced neurophysiologists were analysed for differences in examination strategy, the sensitivity of different tests, and inferring minimal criteria. The diagnostic performance of the suggested criteria was validated on 24 MG patients and 50 patients with neuropathy (17), myopathy (15), or fatigue (18).

Results

We recommend as minimal electrodiagnostic criteria for NMT disorders, either (a) 2 abnormal repetitive nerve stimulation (RNS), (b) 1 abnormal RNS and 1 abnormal single fiber electromyography (SFEMG) or (c) 2 abnormal SFEMG. These showed a good diagnostic performance with a sensitivity of 87.5 % and a specificity of 100 %.

Conclusion

Recommendations with high diagnostic sensitivity and specificity for the minimum number of RNS and SFEMG studies to diagnose NMT disorders developed by an international consensus group are suggested.

Significance

The suggested electrodiagnostic recommendations for diagnosing NMT disorders are reliable and suitable for use at different centres.
目的电诊断在诊断神经肌肉传递障碍(NMT),特别是血清阴性重症肌无力中具有重要意义。然而,诊断的电诊断标准是稀疏的。本研究旨在推断NMT疾病电诊断检查的循证建议。方法对经8位经验丰富的神经生理学家同行评议的164例一致诊断为NMT障碍的病例进行分析,分析其检查策略、不同检查方法的敏感性以及推断最小标准的差异。建议的诊断标准在24名MG患者和50名神经病变(17名)、肌病(15名)或疲劳(18名)患者中得到了验证。结果我们推荐NMT障碍的最低电诊断标准为:(a) 2个异常的重复神经刺激(RNS), (b) 1个异常的重复神经刺激(RNS)和1个异常的单纤维肌电图(SFEMG)或(c) 2个异常的SFEMG。结果表明,该方法具有良好的诊断性能,敏感性为87.5%,特异性为100%。结论国际共识小组提出了诊断NMT疾病的高诊断敏感性和特异性的RNS和SFEMG研究的最少数量的建议。建议的诊断NMT疾病的电诊断建议是可靠的,适合在不同的中心使用。
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引用次数: 0
Asymptomatic tarsal tunnel syndrome in rheumatoid arthritis: An electrophysiological perspective with Insights into clinical and Laboratory correlates 类风湿关节炎的无症状跗骨隧道综合征:电生理观点与临床和实验室相关的见解
IF 2 Q3 NEUROSCIENCES Pub Date : 2025-01-01 DOI: 10.1016/j.cnp.2025.07.003
Hosna Elshony , Mohamed H. Aly , Abdelgaffar Mohammed , Abdulrahman M. Hassan , Abdulrahman A. Alshehri , Mohamed Hedak , Rakan Almuhanna , Abdulaziz Al-Ghamdi , Rasha Elsaadawy

Objective

To evaluate the prevalence of asymptomatic tarsal tunnel syndrome (TTS) in rheumatoid arthritis (RA) patients and its association with disease activity, inflammation, and electrophysiological changes.

Methods

Forty RA patients and 40 age- and sex-matched controls underwent nerve conduction studies assessing medial plantar, lateral plantar, and posterior tibial nerves. Disease activity (DAS28, RASS), inflammatory markers (ESR, CRP), and autoantibodies (RF, anti-CCP) were recorded. Comparisons used t-tests; correlations assessed associations.

Results

RA patients showed significantly prolonged sensory latencies and reduced amplitudes and nerve conduction velocities, especially in medial and lateral plantar nerves. Sensory latencies had 100% sensitivity for subclinical TTS; motor parameters demonstrated high specificity. Higher DAS28, longer disease duration, elevated ESR/CRP, joint deformities, and seropositivity predicted TTS.

Conclusions

Subclinical TTS is highly prevalent in RA and linked to systemic inflammation, disease severity, and structural damage.

Significance

Routine electrophysiological screening may enable early detection and intervention, preserving nerve function and improving long-term mobility and quality of life in RA patients.

Trial Registration

The study was approved by the SFHM Institutional Review Board and registered under HAP-02-K-052 in August 2024.
目的探讨无症状跗骨隧道综合征(TTS)在类风湿关节炎(RA)患者中的患病率及其与疾病活动度、炎症和电生理变化的关系。方法40例RA患者和40例年龄和性别匹配的对照组接受了神经传导研究,评估了足底内侧神经、足底外侧神经和胫后神经。记录疾病活动性(DAS28、RASS)、炎症标志物(ESR、CRP)和自身抗体(RF、抗ccp)。比较采用t检验;相关性评估关联。结果ra患者感觉潜伏期明显延长,振幅和神经传导速度明显降低,尤以内侧和外侧足底神经明显。感觉潜伏期对亚临床TTS的敏感性为100%;电机参数具有高特异性。较高的DAS28、较长的病程、升高的ESR/CRP、关节畸形和血清阳性预测TTS。结论亚临床TTS在RA中非常普遍,并与全身性炎症、疾病严重程度和结构损伤有关。常规电生理筛查可以早期发现和干预,保护神经功能,改善RA患者的长期活动能力和生活质量。试验注册该研究已获得SFHM机构审查委员会的批准,并于2024年8月根据HAP-02-K-052注册。
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引用次数: 0
Novel approaches to EEG and MEG in motor neurone disease: IFCN Handbook Chapter 运动神经元疾病EEG和MEG的新方法:IFCN手册章节
IF 2 Q3 NEUROSCIENCES Pub Date : 2025-01-01 DOI: 10.1016/j.cnp.2025.07.001
Stefan Dukic , Rosanne Govaarts , Arjan Hillebrand , Marianne de Visser , Margitta Seeck , Roisin McMackin
Motor neurone diseases (MNDs) are increasingly being acknowledged as network disorders, with cortical dysfunction and degeneration extending beyond the motor cortex. Measures of this broader cortical pathophysiology are providing promising candidates in the search for diagnostic and prognostic biomarkers of the MNDs. Electroencephalography (EEG) and magnetoencephalography (MEG) offer a direct view of neural network activity by detecting changes in electromagnetic fields of the brain. Measurements based on EEG/MEG have often been overlooked in the search for MND biomarkers, largely due to their limited spatial resolution and the perceived challenges associated with noise in these signals. However, with recent developments in sensor technology and source reconstruction algorithms, alongside substantial improvement in pipelines that address noise, EEG/MEG-based measures can now be readily employed for spatiotemporally-precise, economical and non-invasive characterisation of cortical network pathophysiology in MNDs. Here, we provide an overview of how EEG/MEG signals have been employed to quantify neural network function in MND. We outline the advantages and limitations of these measurements, discuss the most clinically promising EEG/MEG studies of MNDs to date, and highlight future directions warranted to harness the full potential of these technologies for understanding and assessing MNDs.
运动神经元疾病(mnd)越来越多地被认为是一种网络疾病,其皮层功能障碍和退化延伸到运动皮层以外。这种更广泛的皮层病理生理学的测量为寻找mnd的诊断和预后生物标志物提供了有希望的候选物。脑电图(EEG)和脑磁图(MEG)通过检测大脑电磁场的变化提供了对神经网络活动的直接观察。在寻找MND生物标志物的过程中,基于EEG/MEG的测量常常被忽视,这主要是由于它们有限的空间分辨率和与这些信号中的噪声相关的感知挑战。然而,随着传感器技术和声源重建算法的最新发展,以及解决噪声的管道的实质性改进,基于EEG/ meg的测量现在可以很容易地用于大脑皮层网络病理生理的时空精确、经济和非侵入性表征。在这里,我们概述了如何使用EEG/MEG信号来量化MND中的神经网络功能。我们概述了这些测量的优点和局限性,讨论了迄今为止最有临床前景的脑电/脑磁图研究,并强调了未来的方向,保证利用这些技术的全部潜力来理解和评估脑磁图。
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引用次数: 0
Evaluating diaphragm motor response variability in electric and magnetic phrenic nerve stimulations during passive expiration 评估被动呼气时膈神经电和磁刺激下膈肌运动的反应变异性
IF 2 Q3 NEUROSCIENCES Pub Date : 2025-01-01 DOI: 10.1016/j.cnp.2025.11.003
Ivan Chakalov , Swen Hülsmann , Perianen Ramasawmy , Lukas Diedrich , Mathias Bähr , Leif Saager , Konrad Meissner , Onnen Moerer , Caspar Stephani , Andrea Antal

Objective

This study assessed variability in cervical electrical (CEPNS) and magnetic (CMPNS) phrenic nerve stimulation alongside transcranial magnetic stimulation (diTMS) of the diaphragm, focusing on motor response latency and amplitude at individually calculated suprathreshold intensities.

Methods

Diaphragm motor responses were elicited via CEPNS and CMPNS (measuring compound muscle action potentials, CMAPs) and diTMS (measuring diaphragm motor evoked potentials, diMEPs). Latency and amplitude were recorded. Statistical analyses compared methods, evaluated variability using coefficients of variation (CV), and explored associations with height and central motor conduction time.

Results

Among 25 participants (mean age 25 ± 4 years), CMPNS evoked CMAPs in 21 subjects, CEPNS in 16, with 12 responding to both. No significant latency or amplitude differences emerged between CEPNS and CMPNS responders. TMS evoked diMEPs in 24 participants. Latency was more consistent than amplitude across all methods; CMPNS exhibited the lowest amplitude variability. Height moderately correlated positively with CMAP latency and negatively with central motor conduction time.

Conclusions

Individually adapted suprathreshold CEPNS and CMPNS assessed phrenic nerve conduction with varying success rates. Latency was a more reliable measure than amplitude. CMPNS and diTMS surpassed CEPNS in response reliability, positioning magnetic stimulations as preferable for assessing phrenic nerve conduction. CEPNS might not be suitable for examining motor response amplitudes when not applied at supramaximal intensity. Height-related anatomical factors influencing conduction merit further study.

Significance

Latency of diaphragm motor responses elicited at individually estimated stimulation intensities offers a more consistent biomarker over amplitude for assessing phrenic nerve function.
目的:本研究评估膈神经经颅磁刺激(diTMS)与宫颈电刺激(CEPNS)和磁刺激(CMPNS)的可变性,重点关注运动反应潜伏期和阈上强度单独计算的振幅。方法通过CEPNS、CMPNS(测量复合肌动作电位,CMAPs)和diTMS(测量膈肌运动诱发电位,diMEPs)诱发膈肌运动反应。记录潜伏期和振幅。统计分析比较了各种方法,使用变异系数(CV)评估了变异率,并探讨了与身高和中枢运动传导时间的关系。结果25例参与者(平均年龄25±4岁)中,CMPNS诱发cmap的21例,CEPNS诱发cmap的16例,其中12例均有反应。CEPNS和CMPNS应答者之间没有明显的潜伏期或幅度差异。经颅磁刺激诱发24例diMEPs。在所有方法中,潜伏期比振幅更一致;CMPNS表现出最低幅度的变异性。身高与CMAP潜伏期呈正相关,与中枢运动传导时间呈负相关。结论单独应用阈上CEPNS和CMPNS评估膈神经传导成功率不同。潜伏期比振幅更可靠。CMPNS和diTMS在反应可靠性上优于CEPNS,定位磁刺激作为评估膈神经传导的首选。当不以最大强度应用时,CEPNS可能不适合检查运动反应幅度。与高度相关的影响传导的解剖学因素值得进一步研究。在单独估计的刺激强度下引起的膈肌运动反应的潜伏期为评估膈神经功能提供了一个更一致的生物标志物。
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引用次数: 0
Favorable outcomes of epilepsy with gait-induced seizures after resection of the unilateral supplementary motor area 切除单侧辅助运动区后伴有步态性癫痫发作的良好结果
IF 2 Q3 NEUROSCIENCES Pub Date : 2025-01-01 DOI: 10.1016/j.cnp.2025.10.004
Satoshi Kodama , Naoto Kunii , Yuichiro Shirota , Takusei Chou , Mizuho Kawai , Seijiro Shimada , Meiko Maeda , Hiroyuki Ishiura , Masashi Hamada , Masako Ikemura , Yuko Saito , Naoki Akamatsu , Taira Uehara , Nobuhito Saito , Tatsushi Toda

Background

Gait-induced seizures are a rare manifestation of reflex epilepsy. Pathophysiology of this phenomenon has not been fully understood.

Case presentation

A 28-year-old woman presented with a long history of “falls” following paroxysmal bilateral leg stiffness triggered by walking. Scalp electroencephalogram (EEG) revealed low-amplitude rhythmic beta activity, maximal at the Cz electrode, during these events. Magnetoencephalography demonstrated repetitive sharp waves source-localized to the right primary motor cortex. Multiple anti-seizure medications failed to improve her symptoms; however, the clinical manifestation was consistent with epilepsy with gait-induced seizures. Intracranial subdural EEG recording was performed and confirmed ictal activity originating from the right supplementary motor area. Resection of this area resulted in complete resolution of her symptoms.

Discussion

This is the first reported case of successful resective surgery for epilepsy with gait-induced seizure. Brain networks involving cortical regions responsible for the initiation or execution of walking presumably played a key role in the generation of gait-induced seizures. Careful assessment using non-invasive neurophysiological studies facilitated accurate diagnosis, successful intracranial recordings, and effective resective surgery.
背景:步态诱发癫痫是反射性癫痫的一种罕见表现。这一现象的病理生理学尚未完全了解。病例介绍:一名28岁女性,因行走引起阵发性双侧腿僵硬,长期出现“跌倒”病史。在这些事件中,头皮脑电图(EEG)显示低振幅节律性β活动,在Cz电极处最大。脑磁图显示重复的尖波源定位于右侧初级运动皮层。多种抗癫痫药物未能改善她的症状;但临床表现符合癫痫伴步态性发作。进行颅内硬脑膜下脑电图记录,证实脑电图活动起源于右侧辅助运动区。切除该区域使她的症状完全消失。讨论:这是首例成功切除癫痫伴步态性癫痫发作的病例。涉及皮层区域的大脑网络负责开始或执行行走,可能在步态诱发癫痫发作的产生中发挥了关键作用。使用非侵入性神经生理学研究的仔细评估有助于准确诊断,成功的颅内记录和有效的切除手术。
{"title":"Favorable outcomes of epilepsy with gait-induced seizures after resection of the unilateral supplementary motor area","authors":"Satoshi Kodama ,&nbsp;Naoto Kunii ,&nbsp;Yuichiro Shirota ,&nbsp;Takusei Chou ,&nbsp;Mizuho Kawai ,&nbsp;Seijiro Shimada ,&nbsp;Meiko Maeda ,&nbsp;Hiroyuki Ishiura ,&nbsp;Masashi Hamada ,&nbsp;Masako Ikemura ,&nbsp;Yuko Saito ,&nbsp;Naoki Akamatsu ,&nbsp;Taira Uehara ,&nbsp;Nobuhito Saito ,&nbsp;Tatsushi Toda","doi":"10.1016/j.cnp.2025.10.004","DOIUrl":"10.1016/j.cnp.2025.10.004","url":null,"abstract":"<div><h3>Background</h3><div>Gait-induced seizures are a rare manifestation of reflex epilepsy. Pathophysiology of this phenomenon has not been fully understood.</div></div><div><h3>Case presentation</h3><div>A 28-year-old woman presented with a long history of “falls” following paroxysmal bilateral leg stiffness triggered by walking. Scalp electroencephalogram (EEG) revealed low-amplitude rhythmic beta activity, maximal at the Cz electrode, during these events. Magnetoencephalography demonstrated repetitive sharp waves source-localized to the right primary motor cortex. Multiple anti-seizure medications failed to improve her symptoms; however, the clinical manifestation was consistent with epilepsy with gait-induced seizures. Intracranial subdural EEG recording was performed and confirmed ictal activity originating from the right supplementary motor area. Resection of this area resulted in complete resolution of her symptoms.</div></div><div><h3>Discussion</h3><div>This is the first reported case of successful resective surgery for epilepsy with gait-induced seizure. Brain networks involving cortical regions responsible for the initiation or execution of walking presumably played a key role in the generation of gait-induced seizures. Careful assessment using non-invasive neurophysiological studies facilitated accurate diagnosis, successful intracranial recordings, and effective resective surgery.</div></div>","PeriodicalId":45697,"journal":{"name":"Clinical Neurophysiology Practice","volume":"10 ","pages":"Pages 489-492"},"PeriodicalIF":2.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145415147","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
COMPRESSIVE DATA STORAGE FOR LONG-TERM EEG: VALIDATION BY VISUAL ANALYSIS 长期脑电图压缩数据存储:视觉分析验证
IF 2 Q3 NEUROSCIENCES Pub Date : 2025-01-01 DOI: 10.1016/j.cnp.2025.07.005
Giridhar P. Kalamangalam , Subeikshanan Venkatesan , Maria-Jose Bruzzone , Yue Wang , Carolina B. Maciel , Sotiris Mitropanopoulos , Jean Cibula , Kajal Patel , Abbas Babajani-Feremi

Objectives

Long-term EEG monitoring (LTM) in acute neurology generates massive data volumes. We investigated whether data-analytic techniques could reduce LTM data size yet conserve their visual diagnostic features.

Methods

LTM exemplars from 50 patients underwent singular value decomposition (SVD). High-variance SVD components were transformed using discrete cosine transform (DCT), and significant elements run-length encoded. Two regimes were tested: (I) SVD and DCT compression ratio (CR) of 1.7 and 12, and (II) CR of 3.7 and 5.7; each achieved an overall CR of ≈20. Compressed data were reconstructed alongside uncompressed originals, to create a total of 200 recordings that were scored by two blinded reviewers. Scores of original and reconstructed data were statistically analyzed.

Results

Score differences between original recordings were smaller than comparisons involving reconstructions using the first regime but did not differ significantly from reconstructions using the second regime.

Conclusions

Raw LTM EEG has sufficient redundancy to undergo extreme (20-fold) data compression without compromising visual diagnostic information. A balanced mix of SVD and DCT appears to be a suitable data-analytic pipeline for achieving such compression.

Significance

Dimension reduction is a significant goal in managing big biomedical data. Our results suggest a pathway for archival of meaningful representations of entire LTM datasets. The latent space suggests new lines of data-scientific inquiry of the EEG in acute neurological illness.
目的急性神经内科长期脑电图监测(LTM)产生海量数据。我们研究了数据分析技术是否可以减少LTM数据大小,同时保留其视觉诊断特征。方法对50例患者的sltm样本进行奇异值分解(SVD)。采用离散余弦变换(DCT)对高方差SVD分量进行变换,并对重要元素进行游程编码。测试两种方案:(I) SVD和DCT压缩比(CR)分别为1.7和12,(II) CR分别为3.7和5.7;每个细胞的总CR≈20。压缩后的数据与未压缩的原始数据一起重建,共创建200个录音,由两位盲法评论者评分。对原始数据和重建数据的评分进行统计分析。结果原始记录之间的评分差异小于使用第一种制度重建的比较,但与使用第二种制度重建的比较没有显著差异。结论原始LTM脑电图具有足够的冗余,可以在不影响视觉诊断信息的情况下进行极端(20倍)的数据压缩。SVD和DCT的平衡混合似乎是实现这种压缩的合适数据分析管道。降维是生物医学大数据管理的重要目标。我们的研究结果为整个LTM数据集的有意义表示的存档提供了一条途径。这一潜在的空间为脑电图在急性神经系统疾病中的数据科学探究提供了新的思路。
{"title":"COMPRESSIVE DATA STORAGE FOR LONG-TERM EEG: VALIDATION BY VISUAL ANALYSIS","authors":"Giridhar P. Kalamangalam ,&nbsp;Subeikshanan Venkatesan ,&nbsp;Maria-Jose Bruzzone ,&nbsp;Yue Wang ,&nbsp;Carolina B. Maciel ,&nbsp;Sotiris Mitropanopoulos ,&nbsp;Jean Cibula ,&nbsp;Kajal Patel ,&nbsp;Abbas Babajani-Feremi","doi":"10.1016/j.cnp.2025.07.005","DOIUrl":"10.1016/j.cnp.2025.07.005","url":null,"abstract":"<div><h3>Objectives</h3><div>Long-term EEG monitoring (LTM) in acute neurology generates massive data volumes. We investigated whether data-analytic techniques could reduce LTM data size yet conserve their visual diagnostic features.</div></div><div><h3>Methods</h3><div>LTM exemplars from 50 patients underwent singular value decomposition (SVD). High-variance SVD components were transformed using discrete cosine transform (DCT), and significant elements run-length encoded. Two regimes were tested: (I) SVD and DCT compression ratio (CR) of 1.7 and 12, and (II) CR of 3.7 and 5.7; each achieved an overall CR of ≈20. Compressed data were reconstructed alongside uncompressed originals, to create a total of 200 recordings that were scored by two blinded reviewers. Scores of original and reconstructed data were statistically analyzed.</div></div><div><h3>Results</h3><div>Score differences between original recordings were smaller than comparisons involving reconstructions using the first regime but did not differ significantly from reconstructions using the second regime.</div></div><div><h3>Conclusions</h3><div>Raw LTM EEG has sufficient redundancy to undergo extreme (20-fold) data compression without compromising visual diagnostic information. A balanced mix of SVD and DCT appears to be a suitable data-analytic pipeline for achieving such compression.</div></div><div><h3>Significance</h3><div>Dimension reduction is a significant goal in managing big biomedical data. Our results suggest a pathway for archival of meaningful representations of entire LTM datasets. The latent space suggests new lines of data-scientific inquiry of the EEG in acute neurological illness.</div></div>","PeriodicalId":45697,"journal":{"name":"Clinical Neurophysiology Practice","volume":"10 ","pages":"Pages 331-339"},"PeriodicalIF":2.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144779854","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Tibial to ulnar nerve amplitude ratio as a marker of length-dependent neuropathy 胫尺神经振幅比作为长度依赖性神经病变的标志
IF 2 Q3 NEUROSCIENCES Pub Date : 2025-01-01 DOI: 10.1016/j.cnp.2025.10.006
Chikashi Yano , Tomonori Nakamura , Masahiro Ando , Yujiro Higuchi , Jun-Hui Yuan , Akiko Yoshimura , Takahiro Hobara , Fumikazu Kojima , Yu Hiramatsu , Satoshi Nozuma , Yusuke Sakiyama , Akihiro Hashiguchi , Raymond L. Rosales , Kimiyoshi Arimura , Hiroshi Takashima

Objective

To evaluate the utility of nerve conduction studies as a marker of length-dependent neuropathy.

Methods

We conducted a retrospective study of 44 chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) patients and 365 genetically confirmed Charcot-Marie-Tooth disease (CMT) patients, including those with PMP22 duplications or mutations in GJB1, MFN2, MPZ, and MME. Nerve conduction study parameters were compared, with subgroup analyses of CIDP-mimicking CMT (genetically confirmed CMT with a prior clinical diagnosis of CIDP) and gene-based classifications. Receiver operating characteristic (ROC) analysis assessed the sensitivity and specificity of these parameters.

Results

The tibial to ulnar nerve distal compound muscle action potential (T/U CMAP) amplitude ratio was significantly higher in CIDP patients compared to those with genetically confirmed CMT, CIDP-mimicking CMT, and gene-based subgroups. This ratio yielded the highest area under the curve (AUC: 0.95) among all evaluated parameters, with a cutoff value of 0.385 demonstrating high diagnostic sensitivity (95.5%) and specificity (85.5%). In CIDP-mimicking CMT group, a similar sensitivity and specificity were observed.

Conclusions

The T/U CMAP amplitude ratio is a simple, robust electrophysiological index of length-dependent neuropathy.

Significance

This marker offers a reliable and accessible way to distinguish between acquired and inherited neuropathies, improving diagnostic accuracy and helping prioritize genetic testing.
目的评价神经传导检查作为长度依赖性神经病变标志物的应用价值。方法对44例慢性炎症性脱髓鞘性多根神经病变(CIDP)患者和365例遗传确诊的沙克-玛丽-图斯病(CMT)患者进行回顾性研究,包括GJB1、MFN2、MPZ和mme中PMP22重复或突变的患者。神经传导研究参数进行比较,并对模拟CIDP的CMT(既往临床诊断为CIDP的遗传确诊CMT)进行亚组分析和基于基因的分类。受试者工作特征(ROC)分析评估这些参数的敏感性和特异性。结果cdp患者胫尺神经远端复合肌动作电位(T/U CMAP)振幅比明显高于遗传确诊CMT、cdp模拟CMT和基因亚组。该比值在所有评估参数中产生最高的曲线下面积(AUC: 0.95),截止值为0.385,具有较高的诊断敏感性(95.5%)和特异性(85.5%)。在模拟CMT组中,观察到相似的敏感性和特异性。结论T/U CMAP振幅比是一种简单、可靠的长度依赖性神经病变电生理指标。该标记提供了一种可靠且易于获取的方法来区分获得性和遗传性神经病变,提高诊断准确性并帮助优先进行基因检测。
{"title":"Tibial to ulnar nerve amplitude ratio as a marker of length-dependent neuropathy","authors":"Chikashi Yano ,&nbsp;Tomonori Nakamura ,&nbsp;Masahiro Ando ,&nbsp;Yujiro Higuchi ,&nbsp;Jun-Hui Yuan ,&nbsp;Akiko Yoshimura ,&nbsp;Takahiro Hobara ,&nbsp;Fumikazu Kojima ,&nbsp;Yu Hiramatsu ,&nbsp;Satoshi Nozuma ,&nbsp;Yusuke Sakiyama ,&nbsp;Akihiro Hashiguchi ,&nbsp;Raymond L. Rosales ,&nbsp;Kimiyoshi Arimura ,&nbsp;Hiroshi Takashima","doi":"10.1016/j.cnp.2025.10.006","DOIUrl":"10.1016/j.cnp.2025.10.006","url":null,"abstract":"<div><h3>Objective</h3><div>To evaluate the utility of nerve conduction studies as a marker of length-dependent neuropathy.</div></div><div><h3>Methods</h3><div>We conducted a retrospective study of 44 chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) patients and 365 genetically confirmed Charcot-Marie-Tooth disease (CMT) patients, including those with <em>PMP22</em> duplications or mutations in <em>GJB1</em>, <em>MFN2</em>, <em>MPZ</em>, and <em>MME</em>. Nerve conduction study parameters were compared, with subgroup analyses of CIDP-mimicking CMT (genetically confirmed CMT with a prior clinical diagnosis of CIDP) and gene-based classifications. Receiver operating characteristic (ROC) analysis assessed the sensitivity and specificity of these parameters.</div></div><div><h3>Results</h3><div>The tibial to ulnar nerve distal compound muscle action potential (T/U CMAP) amplitude ratio was significantly higher in CIDP patients compared to those with genetically confirmed CMT, CIDP-mimicking CMT, and gene-based subgroups. This ratio yielded the highest area under the curve (AUC: 0.95) among all evaluated parameters, with a cutoff value of 0.385 demonstrating high diagnostic sensitivity (95.5%) and specificity (85.5%). In CIDP-mimicking CMT group, a similar sensitivity and specificity were observed.</div></div><div><h3>Conclusions</h3><div>The T/U CMAP amplitude ratio is a simple, robust electrophysiological index of length-dependent neuropathy.</div></div><div><h3>Significance</h3><div>This marker offers a reliable and accessible way to distinguish between acquired and inherited neuropathies, improving diagnostic accuracy and helping prioritize genetic testing.</div></div>","PeriodicalId":45697,"journal":{"name":"Clinical Neurophysiology Practice","volume":"10 ","pages":"Pages 499-506"},"PeriodicalIF":2.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145473741","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Challenging the electrophysiological interpretation of mirror visual feedback in stroke 挑战脑卒中中镜像视觉反馈的电生理解释
IF 2 Q3 NEUROSCIENCES Pub Date : 2025-01-01 DOI: 10.1016/j.cnp.2025.08.008
Brijesh Sathian , Javed Iqbal , Hanadi Al Hamad
{"title":"Challenging the electrophysiological interpretation of mirror visual feedback in stroke","authors":"Brijesh Sathian ,&nbsp;Javed Iqbal ,&nbsp;Hanadi Al Hamad","doi":"10.1016/j.cnp.2025.08.008","DOIUrl":"10.1016/j.cnp.2025.08.008","url":null,"abstract":"","PeriodicalId":45697,"journal":{"name":"Clinical Neurophysiology Practice","volume":"10 ","pages":"Pages 378-379"},"PeriodicalIF":2.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145048259","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Optimising electrical vestibular stimulation (EVS) for assessing vestibular function 优化前庭电刺激(EVS)评估前庭功能
IF 2 Q3 NEUROSCIENCES Pub Date : 2025-01-01 DOI: 10.1016/j.cnp.2025.08.006
Raphael Hamel , Peter Gaskell , Sophie Prosser , Darren Whelan , Richard Irving , Raymond Francis Reynolds

Objective

Electrical vestibular stimulation (EVS) exhibits similar diagnostic performance to caloric irrigation for assessing interaural vestibular asymmetry. To optimise EVS for clinical uses, we investigated the influence of EVS stimulus parameters upon absolute torsional eye response amplitude and asymmetry assessments.

Methods

In young healthy adults (n = 72), monoaural sinusoidal EVS was delivered to each mastoid process for 40 s whilst ocular torsion was assessed. Ambient lights, electrode montage, and stimulus frequency were systematically manipulated.

Results

Ambient lights (250 lx) attenuated torsional responses by 20 % compared to darkness. When the reference electrode was next to C7 (i.e., mastoid-C7 montage), responses to monoaural stimuli were 50 % those of binaural stimulation. Sinusoidal stimuli at 0.5 Hz evoked torsional responses 250 % larger than at 2 Hz but required 300 % more time to estimate asymmetry (12sec vs 4sec). Perceived discomfort was lower at 0.5 Hz than at 2 Hz. Pooled results (n = 53) suggest a normative asymmetry range of 0 ± 25 % for assessing pathological asymmetry.

Conclusions

Darkness and lower stimulus frequencies optimise absolute torsional eye response amplitude. Higher stimulus frequencies accelerate asymmetry assessments. To activate a single vestibular end organ, a mastoid-C7 (or mastoid-acromioclavicular) montage should be used.

Significance

These results extend the clinical potential of EVS for assessing vestibular function.
目的前庭电刺激(EVS)在评估耳间前庭不对称方面表现出与热灌相似的诊断性能。为了优化EVS的临床应用,我们研究了EVS刺激参数对绝对扭眼反应振幅和不对称性评估的影响。方法对72例年轻健康成人进行单耳正弦EVS注射,观察眼扭转情况,观察其对乳突的影响。环境光、电极蒙太奇和刺激频率被系统地操纵。结果与黑暗相比,环境光(250 lx)可使扭转响应减弱20%。当参考电极靠近C7(即乳突-C7蒙太奇)时,对单耳刺激的反应是双耳刺激的50%。0.5 Hz的正弦刺激引起的扭转反应比2 Hz大250%,但需要多300%的时间来估计不对称性(12秒比4秒)。0.5 Hz时的感觉不适感低于2 Hz。合并结果(n = 53)表明,评估病理不对称性的标准不对称性范围为0±25%。结论黑暗和较低的刺激频率可优化绝对扭眼反应幅度。较高的刺激频率加速了非对称性评估。要激活单个前庭末端器官,应使用乳突- c7(或乳突-肩锁)蒙太奇。意义这些结果拓展了EVS在评估前庭功能方面的临床应用潜力。
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引用次数: 0
Evaluating pediatric peripheral neuromuscular disorders using deep neural networks on electrodiagnostic data 基于电诊断数据的深度神经网络评估小儿周围神经肌肉疾病
IF 2 Q3 NEUROSCIENCES Pub Date : 2025-01-01 DOI: 10.1016/j.cnp.2025.08.001
G.K. Cooray , L. Nastasi , D. Motan , J. Deeb

Objective:

This study aims to assess application of deep neural networks to predict neuromuscular disorders in patients based on electrodiagnostic data and compare with clinical assessment.

Methods:

Patients evaluated with electrodiagnostic tests in intensive care over a 10-year period were included in this study. The data set contained both electrodiagnostic and clinical information. Based on the final diagnosis, patients were classified into six groups: non-primary neuromuscular disorders, neuropathy, motor-neuronopathy, myopathy, neuromuscular-junction disorders, and critical-illness neuromyopathy. The neural network was trained on the data.

Results:

The data set was small, allowing training of the neural network on a standard laptop. The validation results were promising, with an accuracy of 0.92, an ROC-AUC of 0.99, and a precision recall AUC of 0.97. The confusion and positive predictive value matrix demonstrated high performance, with diagonal values exceeding 0.82.

Conclusion:

This study demonstrates the efficacy of neural networks in predicting neuromuscular disorders using electrodiagnostic tests. The performance of the model was comparable to clinical assessment. These findings suggest that with more extensive datasets, neural networks can provide reliable estimates of neuromuscular diagnoses.

Significance:

Incorporating neural networks into diagnostic workflows could enhance decision-making, especially in scenarios requiring reassessment or complementary investigations.
目的:探讨基于电诊断数据的深度神经网络在患者神经肌肉疾病预测中的应用,并与临床评估进行比较。方法:在10年的重症监护期间接受电诊断试验评估的患者纳入本研究。数据集包含电诊断和临床信息。根据最终诊断,将患者分为6组:非原发性神经肌肉疾病、神经病变、运动神经病变、肌病、神经肌肉连接疾病和危重症神经肌病。神经网络在数据上进行训练。结果:数据集很小,允许在标准笔记本电脑上训练神经网络。验证结果令人满意,准确度为0.92,ROC-AUC为0.99,精密度召回AUC为0.97。混淆和阳性预测值矩阵表现出良好的性能,对角线值超过0.82。结论:本研究证明了神经网络在电诊断测试中预测神经肌肉疾病的有效性。该模型的性能与临床评估相当。这些发现表明,有了更广泛的数据集,神经网络可以提供神经肌肉诊断的可靠估计。意义:将神经网络纳入诊断工作流程可以提高决策能力,特别是在需要重新评估或补充调查的情况下。
{"title":"Evaluating pediatric peripheral neuromuscular disorders using deep neural networks on electrodiagnostic data","authors":"G.K. Cooray ,&nbsp;L. Nastasi ,&nbsp;D. Motan ,&nbsp;J. Deeb","doi":"10.1016/j.cnp.2025.08.001","DOIUrl":"10.1016/j.cnp.2025.08.001","url":null,"abstract":"<div><h3>Objective:</h3><div>This study aims to assess application of deep neural networks to predict neuromuscular disorders in patients based on electrodiagnostic data and compare with clinical assessment.</div></div><div><h3>Methods:</h3><div>Patients evaluated with electrodiagnostic tests in intensive care over a 10-year period were included in this study. The data set contained both electrodiagnostic and clinical information. Based on the final diagnosis, patients were classified into six groups: non-primary neuromuscular disorders, neuropathy, motor-neuronopathy, myopathy, neuromuscular-junction disorders, and critical-illness neuromyopathy. The neural network was trained on the data.</div></div><div><h3>Results:</h3><div>The data set was small, allowing training of the neural network on a standard laptop. The validation results were promising, with an accuracy of 0.92, an ROC-AUC of 0.99, and a precision recall AUC of 0.97. The confusion and positive predictive value matrix demonstrated high performance, with diagonal values exceeding 0.82.</div></div><div><h3>Conclusion:</h3><div>This study demonstrates the efficacy of neural networks in predicting neuromuscular disorders using electrodiagnostic tests. The performance of the model was comparable to clinical assessment. These findings suggest that with more extensive datasets, neural networks can provide reliable estimates of neuromuscular diagnoses.</div></div><div><h3>Significance:</h3><div>Incorporating neural networks into diagnostic workflows could enhance decision-making, especially in scenarios requiring reassessment or complementary investigations.</div></div>","PeriodicalId":45697,"journal":{"name":"Clinical Neurophysiology Practice","volume":"10 ","pages":"Pages 474-479"},"PeriodicalIF":2.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145319649","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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Clinical Neurophysiology Practice
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