Clinical Neurophysiology最新文献_第2页

Granularity of EEG reactivity in response to stimulation: “Atypical” reactivity in non-sedated and sedated patients 脑电对刺激反应的粒度：非镇静和镇静患者的“非典型”反应

IF 3.6 3区医学

Clinical Neurophysiology Pub Date : 2025-08-31 DOI: 10.1016/j.clinph.2025.2110996

Jaeho Hwang , Emily L. Johnson , Sung-Min Cho , Romergryko Geocadin , Christa W. Habela , Eva K. Ritzl

{"title":"Granularity of EEG reactivity in response to stimulation: “Atypical” reactivity in non-sedated and sedated patients","authors":"Jaeho Hwang , Emily L. Johnson , Sung-Min Cho , Romergryko Geocadin , Christa W. Habela , Eva K. Ritzl","doi":"10.1016/j.clinph.2025.2110996","DOIUrl":"10.1016/j.clinph.2025.2110996","url":null,"abstract":"<div><h3>Objective</h3><div>We describe the EEG phenomenon of increased arrhythmic delta-frequencies upon stimulation without sedating medications (“atypical reactivity”) and characterize patients with this pattern. This is currently not differentiated from the expected reactivity pattern of increased faster-frequencies, according to the American Clinical Neurophysiology Society’s (ACNS) critical care EEG terminology.</div></div><div><h3>Methods</h3><div>This was a retrospective single-center study of adult patients (2015–2022) with EEG reactivity defined by arrhythmic delta-frequencies emerging with stimulation. Raw EEGs were reviewed. Patient demographics, medical conditions, and outcome data were extracted.</div></div><div><h3>Results</h3><div>Ninety patients with atypical reactivity were identified: 70 without confounding sedating medications (non-sedation group), and 20 receiving sedating medications (sedation group). Atypical reactivities were marked by increased arrhythmic delta-activity compared to pre-stimulation baseline, often with decreased faster-frequencies. Nearly all patients in both groups (81%, 100%) had toxic-metabolic etiologies of encephalopathy regardless of sedation. Other encephalopathic EEG patterns were commonly present in both groups (57%, 55%). Survival at 30-days and hospital discharge were overall fair.</div></div><div><h3>Conclusions</h3><div>We propose an addition to the ACNS critical care EEG terminology, called atypical reactivity. Reversible toxic-metabolic encephalopathies are highly prevalent in patients with atypical reactivity.</div></div><div><h3>Significance</h3><div>Identifying atypical reactivity may help differentiate the etiologies and prognoses of patients with encephalopathy.</div></div>","PeriodicalId":10671,"journal":{"name":"Clinical Neurophysiology","volume":"179 ","pages":"Article 2110996"},"PeriodicalIF":3.6,"publicationDate":"2025-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145045379","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

Functional cortical mapping and structural subcortical anatomy predicts intra-operative speech arrest: a nTMS-tractography study 功能皮质映射和结构皮质下解剖预测术中言语骤停：一项ntms神经束造影研究

IF 3.6 3区医学

Clinical Neurophysiology Pub Date : 2025-08-30 DOI: 10.1016/j.clinph.2025.2110995

Josephine Jung , Kapil Rajwani , Ana Mirallave-Pescador , Sally Ann Price , Sabina Patel , Jahard Aliaga-Arias , Hilary Wren , Richard Gullan , Ranjeev Bhangoo , Keyoumars Ashkan , Francesco Vergani , José Pedro Lavrador

{"title":"Functional cortical mapping and structural subcortical anatomy predicts intra-operative speech arrest: a nTMS-tractography study","authors":"Josephine Jung , Kapil Rajwani , Ana Mirallave-Pescador , Sally Ann Price , Sabina Patel , Jahard Aliaga-Arias , Hilary Wren , Richard Gullan , Ranjeev Bhangoo , Keyoumars Ashkan , Francesco Vergani , José Pedro Lavrador","doi":"10.1016/j.clinph.2025.2110995","DOIUrl":"10.1016/j.clinph.2025.2110995","url":null,"abstract":"<div><h3>Objective</h3><div>Cortical speech mapping using navigated Transcranial Magnetic Stimulation (nTMS) has a variable positive predictive value (PPV) when compared with intraoperative direct electrical stimulation.</div></div><div><h3>Methods</h3><div>This is a single centre prospective study of all patients undergoing pre-operative nTMS and tractography (frontal aslant tract (FAT) and arcuate fasciculus (AF)) for awake surgery between October 2018 and November 2023. We reviewed operative notes for speech arrest, collected data on demographics, histopathology and pre-/post-operative language assessment.</div></div><div><h3>Results</h3><div>57 patients were included (27 female, 30 male) with a mean age of 46 years. 89.5 % were right-handed. 59.6 % of patients had surgery for isocitrate dehydrogenase (IDH)-mutant glioma (n = 34). Intra-operative speech arrest was located at opIFG (n = 32), vPrG (n = 19), pMFG (n = 5) and mPoG (n = 1). In 87.7 % of cases (n = 50) intra-operative speech arrest was colocated with nTMS and tractography. This was statistically significant especially in tumours involving opIFG (n = 21) and improved accuracy of locating intra-operative speech arrest when compared with using nTMS or tractography alone (p < 0.01). This was also true for IDH-wildtype tumours (triple colocation n = 14, other modalities n = 6, p < 0.05).</div></div><div><h3>Conclusions</h3><div>Preoperative cortical-subcortical integration is paramount for the prediction of intra-operative speech arrest.</div></div><div><h3>Significance</h3><div>nTMS-FAT-AF trilocation is a useful tool in predicting intra-operative speech arrest.</div></div>","PeriodicalId":10671,"journal":{"name":"Clinical Neurophysiology","volume":"179 ","pages":"Article 2110995"},"PeriodicalIF":3.6,"publicationDate":"2025-08-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144933198","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

Applying high-density surface EMG to the study of neuromuscular disorders: a systematic review 应用高密度表面肌电图研究神经肌肉疾病：系统综述

IF 3.6 3区医学

Clinical Neurophysiology Pub Date : 2025-08-29 DOI: 10.1016/j.clinph.2025.2110983

Ehsan Shandiz , Asbjorn Gaskell , Matthew Ingram , Gabriel S. Trajano , Gabriel L. Fernandes , Joao S. Henkin , Pamela A. McCombe , Robert D. Henderson

{"title":"Applying high-density surface EMG to the study of neuromuscular disorders: a systematic review","authors":"Ehsan Shandiz , Asbjorn Gaskell , Matthew Ingram , Gabriel S. Trajano , Gabriel L. Fernandes , Joao S. Henkin , Pamela A. McCombe , Robert D. Henderson","doi":"10.1016/j.clinph.2025.2110983","DOIUrl":"10.1016/j.clinph.2025.2110983","url":null,"abstract":"<div><h3>Objective</h3><div>High-density surface electromyography (HD-sEMG) is a non-invasive and quantitative tool for studying neuromuscular disorders, enabling assessments of muscle excitation, motor unit (MU) characteristics and firing patterns. This systematic review reports the published evidence on the clinical applications of HD-sEMG across neuromuscular disorders, identifying the range of disorders studied, indexes utilized, and gaps in the literature.</div></div><div><h3>Methods</h3><div>Systematic searches in PubMed and Scopus identified 200 studies, of which 55 met the inclusion criteria. The included articles were grouped into anterior horn cell disorders, muscle disorders and peripheral neuropathies.</div></div><div><h3>Results</h3><div>Commonly studied muscles included the biceps brachii, tibialis anterior, vastus lateralis and the thenar muscles. In anterior horn cell disorders, HD-sEMG has studied fasciculation potentials, MU firing patterns and motor neuron excitability. For peripheral neuropathies, HD-sEMG revealed altered MU recruitment patterns and muscle fibre conduction velocity. In muscle disorders, HD-sEMG demonstrated disrupted MU propagation and reduced MU amplitudes.</div></div><div><h3>Conclusions</h3><div>Our systematic review demonstrated that HD-sEMG is a promising tool in assessing neuromuscular disorders as it can effectively distinguish individuals with neuromuscular disorders from healthy subjects.</div></div><div><h3>Significance</h3><div>However, the adoption of HD-sEMG in clinical practice is hindered by the lack of standardized protocols, complex data analysis and limited research on underexplored conditions.</div></div>","PeriodicalId":10671,"journal":{"name":"Clinical Neurophysiology","volume":"179 ","pages":"Article 2110983"},"PeriodicalIF":3.6,"publicationDate":"2025-08-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144996134","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

Lower-limb express visuomotor responses are spared in Parkinson’s disease during step initiation from a stable position 帕金森氏病在从稳定位置开始的步进过程中，下肢表达视觉运动反应得以保留

IF 3.6 3区医学

Clinical Neurophysiology Pub Date : 2025-08-27 DOI: 10.1016/j.clinph.2025.2110988

Lucas S. Billen , Jorik Nonnekes , Brian D. Corneil , Vivian Weerdesteyn

{"title":"Lower-limb express visuomotor responses are spared in Parkinson’s disease during step initiation from a stable position","authors":"Lucas S. Billen , Jorik Nonnekes , Brian D. Corneil , Vivian Weerdesteyn","doi":"10.1016/j.clinph.2025.2110988","DOIUrl":"10.1016/j.clinph.2025.2110988","url":null,"abstract":"<div><h3>Objective</h3><div>While motor impairments in Parkinson’s Disease are well-studied, less is known about how people with Parkinson’s Disease (PwPD) can nevertheless rapidly transform vision into action. These transformations can be studied by measuring express visuomotor responses (EVRs), which are stimulus-directed bursts of muscle activity thought to originate from the superior colliculus, reaching the periphery via the tecto-reticulospinal pathway.</div></div><div><h3>Methods</h3><div>We examined EVRs in the lower limbs during goal-directed step initiation in 20 PwPD and 20 healthy controls (HC). As lower-limb EVRs in the young have been shown to interact with postural control – which are often affected in PwPD − we manipulated postural demands by varying stance width and target location.</div></div><div><h3>Results</h3><div>Under low postural demand, both groups expressed consistent EVRs. EVR magnitudes were significantly higher in PwPD, yet decreased with greater disease severity. Under high postural demands, EVRs were suppressed and followed by strong anticipatory postural adjustments, which were smaller in PwPD compared to HC.</div></div><div><h3>Conclusions</h3><div>The circuit mediating EVRs may be upregulated in early PD to compensate for motor deficits experienced in daily life, but becomes progressively impaired as PD advances.</div></div><div><h3>Significance</h3><div>These findings provide novel insight into the neural underpinnings of rapid stepping in health and disease.</div></div>","PeriodicalId":10671,"journal":{"name":"Clinical Neurophysiology","volume":"179 ","pages":"Article 2110988"},"PeriodicalIF":3.6,"publicationDate":"2025-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144989243","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

Intermuscular coherence from muscle pairs in the leg as a biomarker for amyotrophic lateral sclerosis 腿部肌肉对肌间一致性作为肌萎缩性侧索硬化症的生物标志物

IF 3.6 3区医学

Clinical Neurophysiology Pub Date : 2025-08-26 DOI: 10.1016/j.clinph.2025.2110986

Naoum P. Issa , Serdar Aydin , Elena Badillo Goicoechea , Nathan Carberry , Mark A. Garret , Sean Smith , Ali A. Habib , Betty Soliven , Kourosh Rezania

{"title":"Intermuscular coherence from muscle pairs in the leg as a biomarker for amyotrophic lateral sclerosis","authors":"Naoum P. Issa , Serdar Aydin , Elena Badillo Goicoechea , Nathan Carberry , Mark A. Garret , Sean Smith , Ali A. Habib , Betty Soliven , Kourosh Rezania","doi":"10.1016/j.clinph.2025.2110986","DOIUrl":"10.1016/j.clinph.2025.2110986","url":null,"abstract":"<div><h3>Objective</h3><div>To evaluate the diagnostic potential of intermuscular coherence (IMC) measured from leg muscle pairs as an early-stage biomarker for amyotrophic lateral sclerosis (ALS).</div></div><div><h3>Methods</h3><div>Surface electromyography (sEMG) was recorded in neurotypical subjects and patients with early-stage ALS from muscle pairs: gastrocnemius lateralis–gastrocnemius medialis (GLGM), tibialis anterior–extensor digitorum brevis (TAED), and vastus lateralis–vastus medialis (VLVM). IMC within the 20–40 Hz range (IMCβγ) and the imaginary component of coherency in the 20–40 Hz range (ICOHβγ) were calculated. Area under the receiver operating characteristic curves (AUC) was used to assess diagnostic performance.</div></div><div><h3>Results</h3><div>IMCβγ was lower in ALS patients than neurotypical subjects for all three muscle pairs (p < 0.05 in all cases). Diagnostic performance (AUC) ranged from 0.69 to 0.78 and was highest for TAED. Reducing the effect of volume conduction by using ICOHβγ tended to improve the diagnostic ability (AUC range 0.76 to 0.84).</div></div><div><h3>Conclusions</h3><div>IMCβγ from leg muscles, particularly TAED, can help differentiate early-stage ALS patients from neurotypical individuals. ICOHβγ further improves diagnostic performance by reducing volume conduction artifacts.</div></div><div><h3>Significance</h3><div>Leg muscle IMC measurements could aid in the early and accurate diagnosis of ALS.</div></div>","PeriodicalId":10671,"journal":{"name":"Clinical Neurophysiology","volume":"179 ","pages":"Article 2110986"},"PeriodicalIF":3.6,"publicationDate":"2025-08-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144922400","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

Electrophysiological features of anti-neurofascin-155 autoimmune nodopathy: Preferential involvement of distal and proximal segments 抗神经束蛋白-155自身免疫性结节病的电生理特征：远端和近端优先受累

IF 3.6 3区医学

Clinical Neurophysiology Pub Date : 2025-08-26 DOI: 10.1016/j.clinph.2025.2110990

Jinhee Kim , Yeon Hak Chung , MinGi Kim , Hee Jo Han , Hee-Jae Jung , Hyunjin Kim , Eun-Jae Lee , Young-min Lim , Byoung Joon Kim , Seung Woo Kim , Ha Young Shin

{"title":"Electrophysiological features of anti-neurofascin-155 autoimmune nodopathy: Preferential involvement of distal and proximal segments","authors":"Jinhee Kim , Yeon Hak Chung , MinGi Kim , Hee Jo Han , Hee-Jae Jung , Hyunjin Kim , Eun-Jae Lee , Young-min Lim , Byoung Joon Kim , Seung Woo Kim , Ha Young Shin","doi":"10.1016/j.clinph.2025.2110990","DOIUrl":"10.1016/j.clinph.2025.2110990","url":null,"abstract":"<div><h3>Objective</h3><div>Anti-neurofascin-155 (NF155) antibody-associated autoimmune nodopathy (NF155-AN) is associated with distinct clinical features, yet its electrophysiological characteristics remain unclear. This study aimed to identify electrophysiologic features of treatment-naïve NF155-AN.</div></div><div><h3>Methods</h3><div>We conducted a multicenter retrospective analysis of nerve conduction data from 10 treatment-naïve patients with NF155-AN and compared them with 22 patients with seronegative chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). The terminal latency index (TLI) was calculated to assess distal nerve involvement, while the intermediate segment F-ratio, a new parameter, was used to evaluate proximal nerve conduction.</div></div><div><h3>Results</h3><div>Patients with NF155-AN more often exhibited subacute onset, sensory ataxia, tremors, and cranial nerve involvement, and were classified as atypical CIDP with poor responses to intravenous immunoglobulin and corticosteroids. Electrophysiologically, NF155-AN patients had significantly longer distal latencies in the peroneal and tibial nerves. The TLI of the ulnar, peroneal, and tibial nerves was lower, while the intermediate segment F-ratio of the ulnar and tibial nerves was higher in the NF155-AN group.</div></div><div><h3>Conclusions</h3><div>NF155-AN patients exhibit prominent proximal and distal nerve slowing, suggesting unique patterns of nerve dysfunction distinct from seronegative CIDP.</div></div><div><h3>Significance</h3><div>These findings provide enhanced insight into the unique electrophysiological features of NF155-AN, offering clues to its pathophysiology and aiding in differentiation from CIDP.</div></div>","PeriodicalId":10671,"journal":{"name":"Clinical Neurophysiology","volume":"179 ","pages":"Article 2110990"},"PeriodicalIF":3.6,"publicationDate":"2025-08-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145045380","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

Investigating circadian patterns in electrochemical skin Conductance: A year-long retrospective analysis using home-based devices 研究电化学皮肤电导的昼夜节律模式：使用家用设备进行为期一年的回顾性分析

IF 3.6 3区医学

Clinical Neurophysiology Pub Date : 2025-08-26 DOI: 10.1016/j.clinph.2025.2110991

Benjamin Vittrant , Violaine Courrier , Sibylle Mauries , Pierre A. Geoffroy , Jean-pascal Lefaucheur , Dinesh Selvarajah

{"title":"Investigating circadian patterns in electrochemical skin Conductance: A year-long retrospective analysis using home-based devices","authors":"Benjamin Vittrant , Violaine Courrier , Sibylle Mauries , Pierre A. Geoffroy , Jean-pascal Lefaucheur , Dinesh Selvarajah","doi":"10.1016/j.clinph.2025.2110991","DOIUrl":"10.1016/j.clinph.2025.2110991","url":null,"abstract":"<div><h3>Objective</h3><div>This study presents a comprehensive analysis of electrochemical skin conductance (ESC) values collected from over 1.9 million measurements using Withings Body Scan and Body Comp scales. It aimed at establishing descriptive values for ESC while correcting for the influence of age, sex, and circadian and seasonal variations on sudomotor function.</div></div><div><h3>Methods</h3><div>We used nonlinear multivariate models and machine learning approaches to optimize the best set of covariate parameters associated with the ESC.</div></div><div><h3>Results</h3><div>Our findings reveal that ESC values exhibit a small, gradual decline with age and are potentially slightly lower in women compared to men. Notably, we identified significant daily and annual patterns in ESC values, suggesting the influence of chronobiological factors.</div></div><div><h3>Conclusions</h3><div>The study underscores the importance of considering these variations in clinical and home monitoring of ESC, offering new insights into peripheral neuronal aging and the potential for ESC as a biomarker of the sympathetic nervous system.</div></div><div><h3>Significance</h3><div>Our work establishes a crucial reference for interpreting ESC measurements, taking into account demographic, biological, and seasonal variations, and highlights the need for further research to explore the clinical implications of these findings.</div></div>","PeriodicalId":10671,"journal":{"name":"Clinical Neurophysiology","volume":"179 ","pages":"Article 2110991"},"PeriodicalIF":3.6,"publicationDate":"2025-08-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144996135","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

High-frequency nerve ultrasound in Guillain-Barré syndrome 格林-巴罗综合征的高频神经超声诊断

IF 3.6 3区医学

Clinical Neurophysiology Pub Date : 2025-08-26 DOI: 10.1016/j.clinph.2025.2110985

Jesper Helbo Storgaard , Erisela Qerama , Anders Stouge , Hatice Tankisi , Thomas Harbo , Henning Andersen , Lotte Levison

{"title":"High-frequency nerve ultrasound in Guillain-Barré syndrome","authors":"Jesper Helbo Storgaard , Erisela Qerama , Anders Stouge , Hatice Tankisi , Thomas Harbo , Henning Andersen , Lotte Levison","doi":"10.1016/j.clinph.2025.2110985","DOIUrl":"10.1016/j.clinph.2025.2110985","url":null,"abstract":"<div><h3>Objective</h3><div>To explore the utility of high-frequency nerve ultrasound (HNUS) of peripheral nerves in patients with Guillain-Barré syndrome (pwGBS) at time of diagnosis and the following 6 months.</div></div><div><h3>Methods</h3><div>Cross-sectional area (CSA) of brachial plexus nerves and upper limb peripheral nerves (20 sites total) were determined with HNUS in 21 pwGBS and 19 healthy subjects. PwGBS were examined 11 (median, interquartile range 6–14) days from motor symptom onset, with follow-up examinations 4 and 26 weeks later. Additionally, Medical Research Council sum-score and GBS Disability Score were assessed.</div></div><div><h3>Results</h3><div>At baseline, pwGBS had increased CSA at 16 out of 20 sites compared to healthy subjects. Sum-score of cervical roots C5–C7 decreased from baseline to 4 weeks (−0.042 (CI:−0.075; −0.008) cm<sup>2</sup>; p = 0.017) and further decreased to week 26 (−0.039 (CI: −0.067; −0.012) cm<sup>2</sup>; p = 0.008). In the interscalene passage, C5–C7 sum-score decreased from baseline to 26 weeks (−0.073 (CI: −0.127; −0.018) cm<sup>2</sup>; p = 0.012). Sum-score of proximal limb nerve CSA decreased from week 4 to week 26 (−0.038 (CI: −0.076; -0.001) cm<sup>2</sup>; p = 0.047). HNUS did not consistently correlate to clinical severity.</div></div><div><h3>Conclusions</h3><div>HNUS enables detection of widespread acute nerve enlargements. Follow-up showed variable regression of nerve swellings at anatomical sites.</div></div><div><h3>Significance</h3><div>HNUS may add information in diagnosis and monitoring of GBS.</div></div>","PeriodicalId":10671,"journal":{"name":"Clinical Neurophysiology","volume":"179 ","pages":"Article 2110985"},"PeriodicalIF":3.6,"publicationDate":"2025-08-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144922397","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

High resolution nerve ultrasound in neurofibromatosis type 1: a prospective and descriptive study 1型神经纤维瘤病的高分辨率神经超声：一项前瞻性和描述性研究

IF 3.6 3区医学

Clinical Neurophysiology Pub Date : 2025-08-26 DOI: 10.1016/j.clinph.2025.2110992

D. Christine Noordhoek , Tessa A. Ennik , Sarah A. van Dijk , Eline Mann , Sophie van der Vlist , Judith Drenthen , Walter Taal

{"title":"High resolution nerve ultrasound in neurofibromatosis type 1: a prospective and descriptive study","authors":"D. Christine Noordhoek , Tessa A. Ennik , Sarah A. van Dijk , Eline Mann , Sophie van der Vlist , Judith Drenthen , Walter Taal","doi":"10.1016/j.clinph.2025.2110992","DOIUrl":"10.1016/j.clinph.2025.2110992","url":null,"abstract":"<div><h3>Objective</h3><div>To explore the value of high resolution nerve ultrasound (HRUS)<span><span><sup>3</sup></span></span> and nerve conduction studies (NCS)<span><span><sup>4</sup></span></span> in screening for plexiform neurofibromas (PNs)<span><span><sup>5</sup></span></span> in neurofibromatosis type 1 (NF1).<span><span><sup>6</sup></span></span></div></div><div><h3>Methods</h3><div>Adult patients with NF1 were eligible. Patients were divided into two groups: with peripheral nervous system related symptoms (PNS<span><span><sup>7</sup></span></span> group) and without PNS-related symptoms (non-PNS group). Study visit included neurological examination, NCS and HRUS.</div></div><div><h3>Results</h3><div>Sixty patients were enrolled, 37 in the PNS group and 23 in the non-PNS group. HRUS was abnormal in 52 patients (87 %), 34 in the PNS group (92 %) and 18 in the non-PNS group (78 %, p = 0.24). Patients with continuous nerve enlargements (high PN tumor load) were easily distinguished with HRUS. 85 % of patients with normal NCS have nerve enlargements on HRUS.</div></div><div><h3>Conclusions</h3><div>HRUS helps to assess PN tumor burden in NF1. NCS did not have additional value.</div></div><div><h3>Significance</h3><div>HRUS might be useful as a screening tool for PN in NF1, especially in settings with less MRI availability.</div></div>","PeriodicalId":10671,"journal":{"name":"Clinical Neurophysiology","volume":"179 ","pages":"Article 2110992"},"PeriodicalIF":3.6,"publicationDate":"2025-08-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144922401","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

Cumulative motor index in spinal muscular atrophy after gene therapy: baseline predicts maximal recovery 基因治疗后脊髓性肌萎缩的累积运动指数：基线预测最大恢复

IF 3.6 3区医学

Clinical Neurophysiology Pub Date : 2025-08-26 DOI: 10.1016/j.clinph.2025.2110987

Rémi Barrois , Christine Barnerias , Anaïs Hervé , Elodie Deladrière , Brigitte Fauroux , Lucie Griffon , Sonia Khirani , Clément Poirault , Isabelle Desguerre , Cyril Gitiaux

{"title":"Cumulative motor index in spinal muscular atrophy after gene therapy: baseline predicts maximal recovery","authors":"Rémi Barrois , Christine Barnerias , Anaïs Hervé , Elodie Deladrière , Brigitte Fauroux , Lucie Griffon , Sonia Khirani , Clément Poirault , Isabelle Desguerre , Cyril Gitiaux","doi":"10.1016/j.clinph.2025.2110987","DOIUrl":"10.1016/j.clinph.2025.2110987","url":null,"abstract":"<div><h3>Objective</h3><div>Higher compound muscle action potentials (CMAP) amplitudes are associated with motor milestones acquisitions in patients with symptomatic early onset spinal muscular atrophy (SMA) after gene therapy (GT). This study aimed to propose a predictive model for the evolution of CMAP amplitudes over 36 months in these patients.</div></div><div><h3>Methods</h3><div>Nineteen SMA patients (mean age 8.5 months; 12 with two SMN2 copies, 7 with three) were prospectively assessed for motor scores and CMAP amplitudes (median, ulnar, fibular, tibial nerves). A cumulative motor index (CMI, sum of CMAP amplitudes) was calculated.</div></div><div><h3>Results</h3><div>Post-GT, CMAP amplitudes and CMI increased significantly (p < 0.05) but plateaued at low pathological values after 24 months. The plateau occurred earlier and peaked lower in patients with two <em>SMN2</em> copies, correlating also with a clinical motor plateau. In these patients, baseline CMI strongly predicted the maximal plateau value at 36 months (<span><math><mrow><msub><mrow><mi>C</mi><mi>M</mi><mi>I</mi></mrow><mrow><mi>M</mi><mn>36</mn></mrow></msub><mo>=</mo><mn>2.67</mn><mo>×</mo><msub><mrow><mi>C</mi><mi>M</mi><mi>I</mi></mrow><mrow><mi>M</mi><mn>0</mn></mrow></msub><mo>+</mo><mn>1.92</mn></mrow></math></span>; R<sup>2</sup> = 0.97). Patients with three <em>SMN2</em> copies plateaued at ∼10 mV, regardless of baseline CMI (R<sup>2</sup> = 0.70).</div></div><div><h3>Conclusion</h3><div>The baseline CMI value emerged as a strong predictor of its maximal value after GT, along with the number of copies of <em>SMN2</em>.</div></div><div><h3>Significance</h3><div>These results support the validation of CMI as a guide for optimal patient selection and therapeutic management.</div></div>","PeriodicalId":10671,"journal":{"name":"Clinical Neurophysiology","volume":"179 ","pages":"Article 2110987"},"PeriodicalIF":3.6,"publicationDate":"2025-08-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144922399","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}

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