Clinical Neurophysiology Practice最新文献_第9页

Intraoperative neurophysiology monitoring in posterior spinal fusion: insight from a Ghanaian patient with Brown-Séquard Syndrome 后路脊柱融合术中神经生理监测：来自加纳布朗- ssamquard综合征患者的见解

IF 2 Q3 NEUROSCIENCES

Clinical Neurophysiology Practice

Pub Date : 2025-01-01 DOI: 10.1016/j.cnp.2025.11.002

Bright Worlanyo Aklamanu, Clement Okyere Sefa, Ebenezer Kodji Tetteh, Kwadzo Poku Yankey

引用次数: 0

Rapid recovery after four months of near-tetraplegia: A case report of pan-neurofascin nodopathy and a brief review of emerging questions 近四肢瘫痪4个月后快速恢复：一例泛神经束蛋白病理报告和对新出现问题的简要回顾

IF 2 Q3 NEUROSCIENCES

Clinical Neurophysiology Practice

Pub Date : 2025-01-01 DOI: 10.1016/j.cnp.2025.09.005

Dániel Milanovich, Zsolt Mezei, Anna Katalin Iljicsov, János Bíró, Zsuzsanna Arányi, Magdolna Simó

Objective

Pan-neurofascin nodopathy is a rare form of dysimmune neuropathies, mediated by antibodies against the common epitope of the 140, 155 and 186 neurofascin isoforms. These autoantibodies cause the defect of saltatory action potential spreading, disorganization of the nodal-paranodal ultrastructure, and axonal loss of variable degree.

Methods

A 32-year old male presented with the typical symptom of acute onset severe tetraparesis. Electrophysiological findings indicated severe conduction failure, but no appreciable axonal loss. High-resolution ultrasound showed diffuse, but patchy pathology, including mild enlargement and abnormal fascicular structure in proximal arm nerves and the brachial plexus. Symptoms worsened despite plasma exchange, and showed only mild and transient improvement upon repeated intravenous immunoglobulin treatment.

Results

After the verification of pan-neurofascin antibodies, rituximab treatment was applied and we observed rapid improvement within weeks, leading to complete remission at 6 weeks.

Conclusions

A young male with pan-neurofascin nodopathy presenting as acute-onset symptoms resembling Guillain–Barré syndrome, became asymptomatic following 4 months of near-tetraplegia as a result of rituximab therapy.

Significance

In pan-neurofascin nodopathy, rituximab treatment can lead to complete reversal of symptoms, even in cases characterized by prolonged and severe clinical manifestations. We also provide one of the first ultrasound descriptions of a pan-neurofascin case.

目的泛神经束蛋白结节病是一种罕见的免疫功能障碍神经病，由针对140,155和186神经束蛋白亚型共同表位的抗体介导。这些自身抗体导致跳跃式动作电位扩散缺陷，淋巴结-副淋巴结超微结构紊乱，以及不同程度的轴突损失。方法1例32岁男性，表现为急性发作型重度四肢麻痹的典型症状。电生理结果显示严重的传导障碍，但未见明显的轴突损失。高分辨率超声显示弥漫性，但斑片状病理，包括手臂近端神经和臂丛的轻度扩大和异常束状结构。血浆置换后症状恶化，反复静脉注射免疫球蛋白治疗后仅出现轻微和短暂的改善。结果在验证泛神经束蛋白抗体后，应用利妥昔单抗治疗，我们在几周内观察到快速改善，6周时完全缓解。结论1例年轻男性泛神经束蛋白结节病表现为类似格林-巴利综合征的急性发作症状，在接受利妥昔单抗治疗4个月后几乎四肢瘫痪，无症状。在泛神经束蛋白结节病中，利妥昔单抗治疗可导致症状完全逆转，即使在以长期和严重临床表现为特征的病例中也是如此。我们也提供了一个泛神经束蛋白病例的第一个超声描述。

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引用次数: 0

Impact of clinical neurophysiological assessment on diagnosis and management of tremor disorders 临床神经生理学评估对震颤障碍诊断和治疗的影响

IF 2 Q3 NEUROSCIENCES

Clinical Neurophysiology Practice

Pub Date : 2025-01-01 DOI: 10.1016/j.cnp.2025.05.003

Katherine Longardner , Yasoda Satpathy , Irene Litvan , Dietrich Haubenberger

Objective

To assess the clinical utility of a standardized, non-invasive electrodiagnostic testing protocol in refining the diagnosis and management of patients referred for tremor evaluation.

Methods

In this prospective observational study, patients with tremulous limb movements with indeterminate clinical diagnoses involving tremor as a cardinal symptom were referred by movement disorders neurologists. Participants underwent standardized phenotyping and electrodiagnostic studies for tremor analysis including four-channel surface electromyography polygraphy and two-channel accelerometry.

Results

Clinical and electrophysiological data from 31 consecutive individuals were analyzed. Electrodiagnostic testing refined the differential diagnosis in 25/31 (80.6 %) participants and changed therapy in 14/29 (48.3 %). Changes included adjusting pharmacotherapy (n = 10), undergoing deep brain stimulation surgery (n = 2), or avoiding invasive procedures (n = 2).

Conclusions

We propose that electrodiagnostic testing is a clinically valuable tool that can narrow the differential diagnosis and impact treatment of tremor.

Significance

Clinical evaluation alone may be insensitive in diagnosing the tremor type when findings are subtle or when multiple movement disorders coexist. This may lead to inaccurate diagnosis and management, increasing cost and patient burden, and prolonging or preventing a successful journey towards adequate treatment. Clinical neurophysiology is a useful diagnostic procedure that can detect and quantify movements that may be otherwise indistinguishable by visual observation.

目的探讨一种标准化的、无创的电诊断测试方案在改进震颤评估患者诊断和管理中的临床应用价值。方法在这项前瞻性观察研究中，以震颤为主要症状的不确定临床诊断的震颤性肢体运动患者由运动障碍神经科医生转诊。参与者进行了标准化的表型和电诊断研究，以进行震颤分析，包括四通道表面肌电图和双通道加速度测量。结果对31例患者的临床和电生理资料进行分析。电诊断试验改善了25/31（80.6%）参与者的鉴别诊断，改变了14/29（48.3%）参与者的治疗方法。改变包括调整药物治疗（n = 10），接受深部脑刺激手术（n = 2），或避免侵入性手术（n = 2）。结论电诊断是一种有临床价值的工具，可以缩小震颤的鉴别诊断范围和影响治疗。意义当症状不明显或多种运动障碍共存时，单纯的临床评估可能对诊断震颤类型不敏感。这可能导致不准确的诊断和管理，增加费用和患者负担，并延长或阻止获得适当治疗的成功旅程。临床神经生理学是一种有用的诊断方法，可以检测和量化视觉观察无法区分的运动。

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引用次数: 0

Neurophysiology and muscle histopathology in ICU-acquired muscle weakness: Lessons learned from COVID-19 重症监护下获得性肌肉无力的神经生理学和肌肉组织病理学：从COVID-19中吸取的教训

IF 2 Q3 NEUROSCIENCES

Clinical Neurophysiology Practice

Pub Date : 2025-01-01 DOI: 10.1016/j.cnp.2025.05.001

Eva K. Hejbøl , Atle V. Lomstein , Henrik D. Schrøder , Benjamin Khan , Thomas Harbo , Hatice Tankisi

Objective

To describe different electrophysiological, histopathological, and ultrastructural patterns of muscle pathology in COVID-19-associated intensive care unit acquired weakness (ICUAW) and raise the question of whether COVID-19-associated critical illness myopathy (CIM) is a distinct entity or is similar to CIM of other causes.

Methods

A series of three patients with COVID-19-associated ICUAW were presented.

Clinical examination, electrophysiological testing, and muscle pathology with light and electron microscopy were reported systematically.

Results

All three patients were clinically affected with severe proximal and distal weakness of upper and lower extremities, increased plasma levels of muscle enzymes, and had myopathic electromyography. Furthermore, in two patients, electrophysiological signs of inflammatory myopathy with profuse denervation activity were present. Muscle pathologies were prominent but very diverse. One patient had signs of CIM, another showed severe inflammatory myopathy, and the main finding in the third patient was mitochondrial changes.

Conclusion

Although the three cases showed similar clinical and electrophysiological patterns, muscle pathology revealed distinct underlying features. This spectrum of muscle disease among patients with severe COVID-19 includes CIM, autoimmune response to the COVID-19 infection, and mitochondrial dysfunction.

Significance

Electrophysiology and histopathology complement each other and are important for determining the etiology, as well as guiding treatment and prognosis.

目的描述新冠肺炎相关重症监护室获得性虚弱（ICUAW）患者肌肉病理的不同电生理、组织病理学和超微结构模式，并提出新冠肺炎相关危重病肌病（CIM）是一种不同的实体还是与其他原因的CIM相似的问题。方法对3例新型冠状病毒感染症（covid -19）相关ICUAW患者进行分析。系统地报道了临床检查、电生理检查和肌肉病理光镜和电镜。结果3例患者临床均表现为严重的上肢和下肢近端和远端无力，血浆肌酶水平升高，肌电图病变。此外，在两名患者中，存在炎症性肌病的电生理体征，伴有大量的去神经活动。肌肉病变很突出但也很多样。一名患者有CIM的迹象，另一名患者表现出严重的炎症性肌病，第三名患者的主要发现是线粒体改变。结论虽然3例患者表现出相似的临床和电生理模式，但肌肉病理表现出不同的潜在特征。严重COVID-19患者的肌肉疾病谱系包括CIM、对COVID-19感染的自身免疫反应和线粒体功能障碍。意义电生理与组织病理学相辅相成，对确定病因、指导治疗和预后具有重要意义。

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引用次数: 0

Comparison between sleep-deprived, and melatonin-induced sleep electroencephalography in children of different ages: a randomized controlled trial 不同年龄儿童睡眠剥夺和褪黑素诱发睡眠脑电图的比较：一项随机对照试验

IF 2 Q3 NEUROSCIENCES

Clinical Neurophysiology Practice

Pub Date : 2025-01-01 DOI: 10.1016/j.cnp.2025.09.002

Greta Gustafsson , Martin Ulander , Cornelia Lauermann , Johanna Thegerström , Kathe Dahlbom , Anders Broström , Eva Svanborg , Magnus Vrethem

Objective

To evaluate possible age-specific differences in the diagnostic yield of sleep-deprived, and melatonin-induced pediatric sleep EEGs. The multicenter study was performed prospectively.

Methods

Children referred to sleep EEG (n = 169), were stratified into three age groups (2–5; 6–11; 12–17 years) and randomized to sleep induction by melatonin or partial sleep deprivation. Epileptiform activity, sleep, adverse effects, and technical recording quality were compared between study arms and age categories.

Results

Epileptiform activity occurred in 36% of the children, without significant differences between melatonin and sleep-deprived EEGs. In 6–11-year-old children, epileptiform abnormalities occurred in 49%, significantly higher than in the other age groups independent of the sleep-inducing method. Sleep, but not the sleep induction method, was significantly associated with the presence of epileptiform activity (OR 9.16). The occurrence of sleep was significantly higher (97%) after melatonin induction compared to sleep deprivation (86%) without age differences. No serious adverse effects were registered.

Conclusions

There were no differences concerning the occurrence of epileptiform activity in EEG after melatonin induction or sleep deprivation in children of any age category. Melatonin increased the likelihood of sleep.

Significance

Melatonin as a premedication for sleep EEG should be considered safe and efficient in terms of diagnostic yield.

目的评价睡眠剥夺和褪黑激素诱发的儿童睡眠脑电图的诊断率可能存在的年龄特异性差异。多中心研究是前瞻性的。方法169例睡眠脑电图患儿按年龄分为2 ~ 5岁、6 ~ 11岁、12 ~ 17岁3组，随机分为褪黑素诱导组和部分剥夺睡眠组。癫痫样活动、睡眠、不良反应和技术记录质量在研究组和年龄组之间进行比较。结果36%的儿童出现癫痫样活动，褪黑激素与睡眠剥夺的脑电图无显著差异。在6 - 11岁儿童中，癫痫样异常发生率为49%，显著高于独立于睡眠诱导方法的其他年龄组。睡眠，而不是睡眠诱导法，与癫痫样活动的存在显著相关（OR 9.16）。褪黑激素诱导后的睡眠发生率（97%）明显高于睡眠剥夺（86%），没有年龄差异。未发现严重的不良反应。结论不同年龄组儿童在褪黑素诱导和睡眠剥夺后脑电图癫痫样活动的发生无明显差异。褪黑素增加了睡眠的可能性。就诊断率而言，褪黑素作为睡眠脑电图的前用药应被认为是安全有效的。

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引用次数: 0

Clinical neurophysiology of REM parasomnias: Diagnostic aspects and insights into pathophysiology 快速动眼期寄生虫的临床神经生理学：诊断方面和病理生理学见解

IF 1.7 Q3 NEUROSCIENCES

Clinical Neurophysiology Practice

Pub Date : 2024-01-01 DOI: 10.1016/j.cnp.2023.10.003

Melanie Bergmann , Birgit Högl , Ambra Stefani

Parasomnias are due to a transient unstable state dissociation during entry into sleep, within sleep, or during arousal from sleep, and manifest with abnormal sleep related behaviors, perceptions, emotions, dreams, and autonomic nervous system activity.

Rapid eye movement (REM) parasomnias include REM sleep behavior disorder (RBD), isolated recurrent sleep paralysis and nightmare disorder. Neurophysiology is key for diagnosing these disorders and provides insights into their pathophysiology.

RBD is very well characterized from a neurophysiological point of view, also thank to the fact that polysomnography is needed for the diagnosis. Diagnostic criteria are provided by the American Academy of Sleep Medicine and video-polysomnography guidelines for the diagnosis by the International REM Sleep Behavior Disorder Study Group. Differences between the two sets of criteria are presented and discussed. Availability of polysomnography in RBD provides data on sleep electroencephalography (EEG), electrooculography (EOG) and electromyography (EMG). Sleep EEG in RBD shows e.g. changes in delta and theta power, in sleep spindles and K complexes. EMG during REM sleep is essential for RBD diagnosis and is an important neurodegeneration biomarker. RBD patients present alterations also in wake EEG, autonomic function, evoked potentials, and transcranial magnetic stimulation.

Clinical neurophysiological data on recurrent isolated sleep paralysis and nightmare disorder are scant. The few available data provide insights into the pathophysiology of these disorders, demonstrating a state dissociation in recurrent isolated sleep paralysis and suggesting alterations in sleep macro- and microstructure as well as autonomic changes in nightmare disorder.

快速眼动（REM）寄生虫病包括快速眼动睡眠行为障碍（RBD）、孤立的复发性睡眠瘫痪和噩梦障碍。从神经生理学的角度来看，快速眼动睡眠障碍的特征非常明显，这也得益于诊断时需要进行多导睡眠图检查。诊断标准由美国睡眠医学学会制定，视频多导睡眠图诊断指南由国际快速眼动睡眠行为障碍研究小组制定。本文介绍并讨论了这两套标准之间的差异。RBD中的多导睡眠图可提供睡眠脑电图（EEG）、脑电图（EOG）和肌电图（EMG）数据。RBD 患者的睡眠脑电图可显示出 delta 和 theta 功率、睡眠纺锤体和 K 复合体等的变化。快速眼动睡眠时的肌电图是诊断 RBD 的重要依据，也是重要的神经变性生物标志物。RBD 患者在清醒时的脑电图、自主神经功能、诱发电位和经颅磁刺激也会发生改变。现有的少量数据为这些疾病的病理生理学提供了深入的见解，显示复发性孤立性睡眠瘫痪的状态分离，并表明睡眠宏观和微观结构的改变以及梦魇症的自主神经变化。

{"title":"Clinical neurophysiology of REM parasomnias: Diagnostic aspects and insights into pathophysiology","authors":"Melanie Bergmann , Birgit Högl , Ambra Stefani","doi":"10.1016/j.cnp.2023.10.003","DOIUrl":"10.1016/j.cnp.2023.10.003","url":null,"abstract":"<div><p>Parasomnias are due to a transient unstable state dissociation during entry into sleep, within sleep, or during arousal from sleep, and manifest with abnormal sleep related behaviors, perceptions, emotions, dreams, and autonomic nervous system activity.</p><p>Rapid eye movement (REM) parasomnias include REM sleep behavior disorder (RBD), isolated recurrent sleep paralysis and nightmare disorder. Neurophysiology is key for diagnosing these disorders and provides insights into their pathophysiology.</p><p>RBD is very well characterized from a neurophysiological point of view, also thank to the fact that polysomnography is needed for the diagnosis. Diagnostic criteria are provided by the American Academy of Sleep Medicine and video-polysomnography guidelines for the diagnosis by the International REM Sleep Behavior Disorder Study Group. Differences between the two sets of criteria are presented and discussed. Availability of polysomnography in RBD provides data on sleep electroencephalography (EEG), electrooculography (EOG) and electromyography (EMG). Sleep EEG in RBD shows e.g. changes in delta and theta power, in sleep spindles and K complexes. EMG during REM sleep is essential for RBD diagnosis and is an important neurodegeneration biomarker. RBD patients present alterations also in wake EEG, autonomic function, evoked potentials, and transcranial magnetic stimulation.</p><p>Clinical neurophysiological data on recurrent isolated sleep paralysis and nightmare disorder are scant. The few available data provide insights into the pathophysiology of these disorders, demonstrating a state dissociation in recurrent isolated sleep paralysis and suggesting alterations in sleep macro- and microstructure as well as autonomic changes in nightmare disorder.</p></div>","PeriodicalId":45697,"journal":{"name":"Clinical Neurophysiology Practice","volume":"9 ","pages":"Pages 53-62"},"PeriodicalIF":1.7,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2467981X24000027/pdfft?md5=284f181c314e115bc2779fb68c7c51dd&pid=1-s2.0-S2467981X24000027-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139455170","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Sensory nerve conduction studies in infants, children and teenagers – An update 婴儿、儿童和青少年的感觉神经传导研究--最新进展

IF 1.7 Q3 NEUROSCIENCES

Clinical Neurophysiology Practice

Pub Date : 2024-01-01 DOI: 10.1016/j.cnp.2024.01.001

Tom Frenzel , Anne-Katrin Baum , Hardy Krause , Christoph Arens , Aiden Haghikia , Imke Galazky

Objective

Nerve conduction studies (NCS) in children remain technically challenging and depend on the cooperation of the child. Motor NCS are not compromised by analgosedation but data for sensory NCS are lacking. Here, we ask whether sensory NCS is influenced by analgosedation. We also compare the present data with NCS studies from the 1990s regarding anthropometric acceleration of the contemporary paediatric population.

Methods

Sensory NCS of the median nerve and sural nerve were performed in 182 healthy subjects aged 1 to 18 years during general anaesthesia and in 47 of them without analgosedation.

Results

Sensory NCS was not influenced by midazolam or propofol. The sensory nerve action potential (SNAP) amplitude and the nerve conduction velocity (NCV) of the sural nerve as well as the SNAP of the median nerve show no significant age dependence in age range 1–18 years. The sensory NCV of the median nerve increased age-dependent.

Conclusions

In clinical practice, analgosedation can be used for diagnostic NCS. Sensory NCS data show no relevant secular trend over the last 30 years. Differences due to technical inconsistency predominate.

Significance

Analgosedation can improve diagnostic quality of sensory NCS in children. Additionally, we provide sensory NCS values from a large pediatric cohort.

目标儿童神经传导研究（NCS）在技术上仍具有挑战性，并且依赖于儿童的合作。运动神经传导不受镇痛的影响，但感觉神经传导却缺乏相关数据。在此，我们想知道感觉神经NCS是否会受到镇痛的影响。方法对 182 名 1 至 18 岁的健康受试者在全身麻醉期间进行了正中神经和硬脊膜神经的感觉 NCS，其中 47 名受试者在未使用镇痛剂的情况下进行了 NCS。硬神经的感觉神经动作电位（SNAP）振幅和神经传导速度（NCV）以及正中神经的感觉神经动作电位（SNAP）在 1 至 18 岁年龄范围内没有明显的年龄依赖性。结论在临床实践中，镇痛可用于诊断性 NCS。结论在临床实践中，镇痛可用于诊断性 NCS。在过去 30 年中，感觉 NCS 数据未显示出相关的世俗趋势。意义镇痛可提高儿童感觉 NCS 的诊断质量。此外，我们还提供了一个大型儿科队列的感觉 NCS 值。

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引用次数: 0

Effects of sleep deprivation on cortical excitability: A threshold-tracking TMS study and review of the literature 剥夺睡眠对大脑皮层兴奋性的影响：阈值追踪 TMS 研究和文献综述

IF 1.7 Q3 NEUROSCIENCES

Clinical Neurophysiology Practice

Pub Date : 2024-01-01 DOI: 10.1016/j.cnp.2023.12.001

Magdalena Mroczek , Amedeo de Grado , Hossain Pia , Zahra Nochi , Hatice Tankisi

Objective

Insufficient sleep is linked to several health problems. Previous studies on the effects of sleep deprivation on cortical excitability using conventional transcranial magnetic stimulation (TMS) included a limited number of modalities, and few inter-stimulus intervals (ISIs) and showed conflicting results. This study aimed to investigate the effects of sleep deprivation on cortical excitability through threshold-tracking TMS, using a wide range of protocols at multiple ISIs.

Methods

Fifteen healthy subjects (mean age ± SD: 36 ± 3.34 years) were included. The following tests were performed before and after 24 h of sleep deprivation using semi-automated threshold-tacking TMS protocols: short-interval intracortical inhibition (SICI) and intracortical facilitation (ICF) at 11 ISIs between 1 and 30 ms, short interval intracortical facilitation (SICF) at 14 ISIs between 1 and 4.9 ms, long interval intracortical inhibition (LICI) at 6 ISIs between 50 and 300 ms, and short-latency afferent inhibition (SAI) at 12 ISIs between 16 and 30 ms.

Results

No significant differences were observed between pre- and post-sleep deprivation measurements for SICI, ICF, SICF, or LICI at any ISIs (p < 0.05). As for SAI, we found a difference at 28 ms (p = 0.007) and 30 ms (p = 0.04) but not at other ISIs.

Conclusions

Sleep deprivation does not affect cortical excitability except for SAI.

Significance

This study confirms some of the previous studies while contradicting others.

目的睡眠不足与多种健康问题有关。以往使用传统经颅磁刺激（TMS）研究睡眠不足对大脑皮层兴奋性的影响时，采用的刺激模式和刺激间隔（ISI）有限，结果也相互矛盾。本研究旨在通过阈值追踪 TMS，在多个 ISIs 下使用多种方案，研究睡眠剥夺对大脑皮层兴奋性的影响。在剥夺睡眠 24 小时之前和之后，使用半自动阈值跟踪 TMS 方案进行了以下测试：1 至 30 毫秒之间 11 个 ISI 的短间隔皮层内抑制（SICI）和皮层内促进（ICF）、1 至 4.9 毫秒之间 14 个 ISI 的短间隔皮层内促进（SICF）、长间隔皮层内抑制（SICI）和皮层内促进（ICF）。结果在任何 ISIs 的 SICI、ICF、SICF 或 LICI 方面，睡眠剥夺前后的测量结果均未观察到显著差异（p < 0.05）。结论除 SAI 外，睡眠剥夺不会影响大脑皮层的兴奋性。

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引用次数: 0

Decreased diaphragm moving distance measured by ultrasound speckle tracking reflects poor prognosis in amyotrophic lateral sclerosis 超声斑点追踪技术测量的膈肌移动距离减少反映了肌萎缩侧索硬化症的不良预后

IF 2 Q3 NEUROSCIENCES

Clinical Neurophysiology Practice

Pub Date : 2024-01-01 DOI: 10.1016/j.cnp.2024.10.002

Shunsuke Watanabe , Kenji Sekiguchi , Hirotomo Suehiro , Masaaki Yoshikawa , Yoshikatsu Noda , Naohisa Kamiyama , Riki Matsumoto

Objective

Decreased cephalocaudal diaphragm movement may indicate respiratory dysfunction in amyotrophic lateral sclerosis (ALS). We aimed to evaluate diaphragm function in ALS using ultrasound speckle tracking, an image-analysis technology that follows similar pixel patterns.

Methods

We developed an offline application that tracks pixel patterns of recorded ultrasound video images using speckle-tracking methods. Ultrasonography of the diaphragm movement during spontaneous quiet respiration was performed on 19 ALS patients and 21 controls to measure the diaphragm moving distance (DMD) in the cephalocaudal direction during a single respiration. We compared respiratory function measures and analyzed the relationship between the clinical profiles and DMD.

Results

DMD was significantly lower in ALS patients than in the control group (0.6 ± 1.4 mm vs 2.2 ± 2.2 mm, p < 0.01) and positively correlated with phrenic nerve compound motor action potential amplitude (R = 0.63, p = 0.01). DMD was negatively correlated with the change in the ALS Functional Rating Scale-Revised scores per month after the exam (R = −0.61, p = 0.02), and those with a larger rate of decline had a significantly lower DMD (p = 0.03).

Conclusions

Diaphragm ultrasound speckle tracking enabled the detection of diaphragm dysfunction in ALS.

Significance

Diaphragm ultrasound speckle tracking may be useful for predicting prognosis.

目的头尾膈肌运动减少可能预示着肌萎缩侧索硬化症（ALS）患者的呼吸功能障碍。我们的目的是利用超声斑点追踪技术评估 ALS 的膈肌功能，该技术是一种图像分析技术，可跟踪类似的像素模式。方法我们开发了一种离线应用程序，可利用斑点追踪方法跟踪记录的超声视频图像的像素模式。我们对 19 名 ALS 患者和 21 名对照组患者进行了自发安静呼吸时横膈膜运动的超声波成像，以测量单次呼吸时横膈膜在头尾方向的移动距离 (DMD)。结果 ALS 患者的膈肌移动距离明显低于对照组（0.6 ± 1.4 mm vs 2.2 ± 2.2 mm，p < 0.01），且与膈神经复合运动动作电位振幅呈正相关（R = 0.63，p = 0.01）。DMD与检查后每月ALS功能评定量表-修订版评分的变化呈负相关（R = -0.61，p = 0.02），下降率较大者的DMD显著较低（p = 0.03）。结论膈肌超声斑点追踪技术可检测ALS患者的膈肌功能障碍。

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引用次数: 0

The speed of completion of the decremental responses on repetitive nerve stimulation 重复神经刺激时递减反应的完成速度

IF 2 Q3 NEUROSCIENCES

Clinical Neurophysiology Practice

Pub Date : 2024-01-01 DOI: 10.1016/j.cnp.2024.06.003

Yuki Ueta , Takamichi Kanbayashi , Yosuke Miyaji , Yuki Hatanaka , Keisuke Tachiyama , Kazusa Takahashi , Hiroo Terashi , Hitoshi Aizawa , Masahiro Sonoo

Objective

It is generally believed that the decremental response in repetitive nerve stimulation (RNS) stabilizes at the fourth or fifth response. We have a preliminary impression that the decremental response approaches a plateau earlier in proximal muscles than in distal muscles. We investigated the speed of the completion of the decremental response in different muscles.

Methods

The “decrement completion ratio (DCR)” in the second or third response (DCR2 or DCR3) was defined as the ratio of the decremental percentage of the second or third response to that of the fourth response. Patients showing more than 10% decremental response both in the abductor pollicis (APB) and deltoid muscles were retrospectively extracted from our EMG database. The DCR2 and DCR3 were compared between two muscles in patients with myasthenia gravis (MG) and amyotrophic lateral sclerosis (ALS).

Results

Identified subjects consisted of 11patients with MG and 11 patients with ALS. Multiple regression analysis revealed that only the difference of muscle influenced on DCR2 and DCR3, with no contribution from the different disorder (MG or ALS) or the initial amplitude of the compound muscle action potential (CMAP). Both DCR2 and DCR3 were significantly higher in deltoid than in APB. In ALS, the normalized CMAP amplitude was not different between APB and deltoid whereas the decremental percentage was significantly higher in deltoid, suggesting a lower safety factor of the neuromuscular transmission in proximal muscles.

Conclusions

The decremental response completed more rapidly in deltoid than in APB which may be related to the lower safety factor also documented by this study.

Significance

Unexpected early completion of the decrement such as at the second response in RNS is not a technical error but may be an extreme of the rapid completion in deltoid, a proximal muscle.

目的一般认为，重复性神经刺激（RNS）的递减反应在第四次或第五次反应时趋于稳定。我们的初步印象是，近端肌肉的递减反应比远端肌肉更早趋于稳定。我们研究了不同肌肉的递减反应完成速度。方法第二或第三次反应的 "递减完成比（DCR）"（DCR2 或 DCR3）定义为第二或第三次反应的递减百分比与第四次反应的递减百分比之比。我们从肌电图数据库中回顾性地提取了脊柱外展肌（APB）和三角肌反应递减均超过10%的患者。比较了重症肌无力（MG）和肌萎缩性脊髓侧索硬化症（ALS）患者两块肌肉的 DCR2 和 DCR3。多元回归分析表明，只有肌肉的差异会对 DCR2 和 DCR3 产生影响，而不同的疾病（MG 或 ALS）或复合肌动作电位（CMAP）的初始振幅不会对其产生影响。三角肌的 DCR2 和 DCR3 都明显高于 APB。在 ALS 中，APB 和三角肌的归一化 CMAP 振幅没有差异，而三角肌的递减百分比明显更高，这表明近端肌肉的神经肌肉传递安全系数更低。

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