Muscle & Nerve最新文献_第10页

Treatment response in patients with clinical and supportive laboratory features of chronic inflammatory demyelinating polyneuropathy without demyelinative findings on nerve conduction studies: A retrospective study. 具有慢性炎症性脱髓鞘性多发性神经病的临床和实验室支持性特征，但神经传导检查未发现脱髓鞘病变的患者的治疗反应：一项回顾性研究。

IF 2.8 3区医学

Muscle & Nerve Pub Date : 2024-07-03 DOI: 10.1002/mus.28198

Patrick Curry, David N Herrmann, Michael Stanton, Phillip Mongiovi, Chary Akmyradov, Eric Logigian

{"title":"Treatment response in patients with clinical and supportive laboratory features of chronic inflammatory demyelinating polyneuropathy without demyelinative findings on nerve conduction studies: A retrospective study.","authors":"Patrick Curry, David N Herrmann, Michael Stanton, Phillip Mongiovi, Chary Akmyradov, Eric Logigian","doi":"10.1002/mus.28198","DOIUrl":"https://doi.org/10.1002/mus.28198","url":null,"abstract":"Introduction/aims: Not all patients with chronic inflammatory demyelinating polyneuropathy (CIDP) have evidence of demyelination on nerve conduction studies (NCS). Patients with \"supportive\" evidence of CIDP on cerebrospinal fluid (CSF), magnetic resonance imaging (MRI), ultrasound (US), or nerve biopsy but not on NCS, often receive immunomodulating therapy. We evaluated the treatment response of patients with clinical and supportive features of CIDP lacking NCS evidence of demyelination.Methods: Retrospective chart review was conducted on 232 patients who met CIDP clinical criteria and were treated with disease-modifying therapy. Patients included did not have NCS criteria of demyelination, but did have supportive CSF, MRI, or US findings consistent with CIDP. A positive treatment response was defined as at least a one-point improvement in the modified Rankin scale (mRS), or a four-point increase in the Medical Research Council sum score (MRCSS).Results: Twenty patients met criteria: 17 of the 18 (94%) patients with CSF protein >45 mg/dL, 6 of the 14 (43%) with MRI lumbosacral root or plexus enhancement, and 4 of the 6 (67%) with enlarged proximal nerves on US. Eighteen patients received intravenous immunoglobulin, 10 corticosteroids, one plasma exchange, and six other immunomodulatory therapies. Twelve patients had a positive treatment response on the MRCSS or mRS. The presence of MRI lumbosacral root or plexus enhancement was associated with a positive treatment response.Discussion: A trial of immunomodulating treatment should be considered for patients with clinical features of CIDP in the absence of NCS evidence of demyelination, particularly when there is MRI lumbosacral root or plexus enhancement.","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":null,"pages":null},"PeriodicalIF":2.8,"publicationDate":"2024-07-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141492707","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

Utilizing nerve conduction studies to identify very early Guillain-Barré syndrome and distinguish it from mimics in emergency settings. 利用神经传导研究来识别极早期的吉兰-巴雷综合征，并将其与急诊环境中的模仿症状区分开来。

IF 2.8 3区医学

Muscle & Nerve Pub Date : 2024-07-03 DOI: 10.1002/mus.28199

Thomas John, Asha Elizabeth Mathew

{"title":"Utilizing nerve conduction studies to identify very early Guillain-Barré syndrome and distinguish it from mimics in emergency settings.","authors":"Thomas John, Asha Elizabeth Mathew","doi":"10.1002/mus.28199","DOIUrl":"https://doi.org/10.1002/mus.28199","url":null,"abstract":"Introduction/aims: Accurately diagnosing Guillain-Barré syndrome (GBS) in its early stages and distinguishing it from mimics poses challenges. This study aimed to evaluate the utility of an existing electrodiagnostic criterion in very early GBS (VEGBS) for discerning mimics. Additionally, we explored specific electrophysiological abnormalities in VEGBS to design a new diagnostic criterion for more accurate VEGBS diagnosis.Methods: We retrospectively identified all patients with flaccid quadriparesis initially suspected of GBS who underwent nerve conduction studies (NCS) ≤4 days from symptom onset. We then retrieved their NCS data and applied an existing electrodiagnostic criterion for sensitivity and specificity analyses based on the final discharge diagnosis. Furthermore, we designed a new criterion based on the observed electrophysiological abnormalities that have maximum specificity and at least 50% sensitivity.Results: Among 70 patients suspected of VEGBS, 44 (63%) received a final diagnosis of GBS, while in 26 (37%), the GBS diagnosis was later refuted. Umapathi's definite criterion exhibited a sensitivity of 61.36% and a specificity of 92.31%. The probable and possible groups showed very high sensitivity (90.91% and 100%, respectively); however, specificity was low (57.69% and 30.77%, respectively) in the very early stage. Our proposed criterion demonstrated a sensitivity of 88.64% (CI: 75.44%-96.21%) and a specificity of 96.15% (CI: 80.36%-99.90%).Discussion: The criterion based on presumed electrophysiological correlates of specific early GBS pathophysiology proved more effective than the existing electrodiagnostic criterion in differentiating VEGBS from mimics.","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":null,"pages":null},"PeriodicalIF":2.8,"publicationDate":"2024-07-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141492708","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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IF 3.4 3区医学

Muscle & Nerve Pub Date : 2024-07-03 DOI: 10.1002/mus.27893

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IF 3.4 3区医学

Muscle & Nerve Pub Date : 2024-07-03 DOI: 10.1002/mus.27891

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Survival among patients receiving eteplirsen for up to 8 years for the treatment of Duchenne muscular dystrophy and contextualization with natural history controls. 接受依替普利森治疗长达 8 年的杜氏肌营养不良症患者的存活率以及与自然病史对照组的比较。

IF 3.4 3区医学

Muscle & Nerve Pub Date : 2024-07-01 Epub Date: 2024-03-14 DOI: 10.1002/mus.28075

Joel Iff, Nicolae Done, Edward Tuttle, Yi Zhong, Fangzhou Wei, Basil T Darras, Craig M McDonald, Eugenio Mercuri, Francesco Muntoni

{"title":"Survival among patients receiving eteplirsen for up to 8 years for the treatment of Duchenne muscular dystrophy and contextualization with natural history controls.","authors":"Joel Iff, Nicolae Done, Edward Tuttle, Yi Zhong, Fangzhou Wei, Basil T Darras, Craig M McDonald, Eugenio Mercuri, Francesco Muntoni","doi":"10.1002/mus.28075","DOIUrl":"10.1002/mus.28075","url":null,"abstract":"Introduction/aims: Eteplirsen, approved in the US for patients with Duchenne muscular dystrophy (DMD) with exon 51 skip-amenable variants, is associated with attenuated ambulatory/pulmonary decline versus DMD natural history (NH). We report overall survival in a US cohort receiving eteplirsen and contextualize these outcomes versus DMD NH.Methods: US patients with DMD receiving eteplirsen were followed through a patient support program, with data collected on ages at eteplirsen initiation and death/end of follow-up. Individual DMD NH data were extracted by digitizing Kaplan-Meier (KM) curves from published systematic and targeted literature reviews. Overall survival age was analyzed using KM curves and contextualized with DMD NH survival curves; subanalyses considered age groups and duration of eteplirsen exposure. Overall survival time from treatment initiation was also evaluated.Results: A total of 579 eteplirsen-treated patients were included. During a total follow-up of 2119 person-years, median survival age was 32.8 years. DMD NH survival curves extracted from four publications (follow-up for 1224 DMD NH controls) showed overall pooled median survival age of 27.4 years. Eteplirsen-treated patients had significantly longer survival from treatment initiation versus age-matched controls (age-adjusted hazard ratio [HR], 0.65; 95% confidence interval [CI], 0.44-0.98; p < .05). Longer treatment exposure was associated with improved survival (HR, 0.15; 95% CI, 0.05-0.41; p < .001). Comparisons using different DMD NH cohorts to address common risks of bias yielded consistent findings.Discussion: Data suggest eteplirsen may prolong survival in patients with DMD across a wide age range. As more data become available, the impact of eteplirsen on survival will be further elucidated.","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":null,"pages":null},"PeriodicalIF":3.4,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140120127","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

Survival in eteplirsen-treated Duchenne Muscular Dystrophy patients: Are there benefits beyond steroids? 接受依替普利森治疗的杜兴氏肌肉萎缩症患者的存活率：除类固醇外还有其他益处吗？

IF 3.4 3区医学

Muscle & Nerve Pub Date : 2024-07-01 Epub Date: 2024-05-06 DOI: 10.1002/mus.28107

W Bryan Burnette

引用次数: 0

Hourglass-like constrictions on MRI are common in electromyography-confirmed cases of neuralgic amyotrophy (Parsonage-Turner syndrome): A tertiary referral center experience. 在肌电图确诊的神经性肌萎缩症（帕森纳-特纳综合征）病例中，核磁共振成像上的沙漏样收缩很常见：一家三级转诊中心的经验。

IF 3.4 3区医学

Muscle & Nerve Pub Date : 2024-07-01 Epub Date: 2023-08-23 DOI: 10.1002/mus.27961

Darryl B Sneag, Casey Urban, Tim Y Li, Philip G Colucci, Emily G Pedrick, Clare A Nimura, Joseph H Feinberg, Carlo J Milani, Ek T Tan

{"title":"Hourglass-like constrictions on MRI are common in electromyography-confirmed cases of neuralgic amyotrophy (Parsonage-Turner syndrome): A tertiary referral center experience.","authors":"Darryl B Sneag, Casey Urban, Tim Y Li, Philip G Colucci, Emily G Pedrick, Clare A Nimura, Joseph H Feinberg, Carlo J Milani, Ek T Tan","doi":"10.1002/mus.27961","DOIUrl":"10.1002/mus.27961","url":null,"abstract":"Introduction/aims: Hourglass-like constrictions (HGCs) of involved nerves in neuralgic amyotrophy (NA) (Parsonage-Turner syndrome) have been increasingly recognized with magnetic resonance neurography (MRN). This study sought to determine the sensitivity of HGCs, detected by MRN, among electromyography (EMG)-confirmed NA cases.Methods: This study retrospectively reviewed records of patients with the clinical diagnosis of NA, and with EMG confirmation, who underwent 3-Tesla MRN within 90 days of EMG at a single tertiary referral center between 2011 and 2021. \"Severe NA\" positive cases were defined by a clinical diagnosis and specific EMG criteria: fibrillation potentials or positive sharp waves, along with motor unit recruitment (MUR) grades of \"discrete\" or \"none.\" On MRN, one or more HGCs, defined as focally decreased nerve caliber or diffusely beaded appearance, was considered \"imaging-positive.\" Post hoc inter-rater reliability for HGCs was measured by comparing the original MRN report against subsequent blinded interpretation by a second radiologist.Results: A total of 123 NA patients with 3-Tesla MRN performed within 90 days of EMG were identified. HGCs were observed in 90.2% of all NA patients. In \"severe NA\" cases, based on the above EMG criteria, HGC detection resulted in a sensitivity of 91.9%. Nerve-by-nerve analysis (183 nerve-muscle pairs, nerves assessed by MRN, muscles assessed by EMG) showed a sensitivity of 91.0%. The second radiologist largely agreed with the original HGC evaluation, (94.3% by subjects, 91.8% by nerves), with no significant difference between evaluations (subjects: χ2 = 2.27, P = .132, nerves: χ2 = 0.98, P = .323).Discussion: MRN detection of HGCs is common in NA.","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":null,"pages":null},"PeriodicalIF":3.4,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10884353/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10054260","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Categorization of the amyotrophic lateral sclerosis population via the clinical determinant of post-onset ΔFS for study design and medical practice. 通过发病后ΔFS的临床决定因素对肌萎缩侧索硬化症患者进行分类，以利于研究设计和医疗实践。

IF 3.4 3区医学

Muscle & Nerve Pub Date : 2024-07-01 Epub Date: 2024-05-07 DOI: 10.1002/mus.28101

Albert C Ludolph, Philippe Corcia, Claude Desnuelle, Terry Heiman-Patterson, Jesus S Mora, Colin D Mansfield, Philippe Couratier

{"title":"Categorization of the amyotrophic lateral sclerosis population via the clinical determinant of post-onset ΔFS for study design and medical practice.","authors":"Albert C Ludolph, Philippe Corcia, Claude Desnuelle, Terry Heiman-Patterson, Jesus S Mora, Colin D Mansfield, Philippe Couratier","doi":"10.1002/mus.28101","DOIUrl":"10.1002/mus.28101","url":null,"abstract":"The amyotrophic lateral sclerosis (ALS) functional rating scale-revised (ALSFRS-R) has become the most widely utilized measure of disease severity in patients with ALS, with change in ALSFRS-R from baseline being a trusted primary outcome measure in ALS clinical trials. This is despite the scale having several established limitations, and although alternative scales have been proposed, it is unlikely that these will displace ALSFRS-R in the foreseeable future. Here, we discuss the merits of delta FS (ΔFS), the slope or rate of ALSFRS-R decline over time, as a relevant tool for innovative ALS study design, with an as yet untapped potential for optimization of drug effectiveness and patient management. In our view, categorization of the ALS population via the clinical determinant of post-onset ΔFS is an important study design consideration. It serves not only as a critical stratification factor and basis for patient enrichment but also as a tool to explore differences in treatment response across the overall population; thereby, facilitating identification of responder subgroups. Moreover, because post-onset ΔFS is derived from information routinely collected as part of standard patient care and monitoring, it provides a suitable patient selection tool for treating physicians. Overall, post-onset ΔFS is a very attractive enrichment tool that is, can and should be regularly incorporated into ALS trial design.","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":null,"pages":null},"PeriodicalIF":3.4,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140855533","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

Understanding the role of the lower motor neuron in spinal cord injury and its impact on electrodiagnostic assessment. 了解下运动神经元在脊髓损伤中的作用及其对电诊断评估的影响。

IF 3.4 3区医学

Muscle & Nerve Pub Date : 2024-07-01 Epub Date: 2024-05-07 DOI: 10.1002/mus.28108

Lawrence R Robinson, Jana Dengler

引用次数: 0

Oral and topical analgesia in pediatric electrodiagnostic studies. 小儿电诊断研究中的口服和局部镇痛。

IF 2.8 3区医学

Muscle & Nerve Pub Date : 2024-07-01 Epub Date: 2024-05-08 DOI: 10.1002/mus.28105

Bisma Aziz, Sajid Hameed, Haris Hakeem, Fazal Ur Rehman, Marib Ghulam Rasool Malik, Saadia Sattar, Pinin Baig, Safoora Ibraheem Zuberi, Sara Khan

{"title":"Oral and topical analgesia in pediatric electrodiagnostic studies.","authors":"Bisma Aziz, Sajid Hameed, Haris Hakeem, Fazal Ur Rehman, Marib Ghulam Rasool Malik, Saadia Sattar, Pinin Baig, Safoora Ibraheem Zuberi, Sara Khan","doi":"10.1002/mus.28105","DOIUrl":"10.1002/mus.28105","url":null,"abstract":"Introduction/aims: Electrodiagnostic examinations, such as nerve conduction studies (NCS) and needle electromyography (EMG), are perceived as painful by children and their parents/guardians. Methods to reduce peri-procedural pain improve compliance and have neurocognitive and neuropsychiatric benefits. This study aimed to assess the efficacy of combined oral and topical analgesics (COTA), oral analgesics (OA), and placebo in reducing pain during NCS/EMG in children.Methods: We performed a double-blind, randomized, placebo-controlled trial on children presenting to our neurophysiology lab. Patients were stratified into two age groups (6M-6Y and 7Y-18Y) and randomized into three arms: COTA, OA, and placebo. Pain scores post-NCS/EMG were assessed using the Modified Behavioral Pain Scale (MBPS) and Faces Pain Scale-Revised (FPS-R).Results: One hundred thirteen participants were enrolled. A comparison of participants from both age groups combined revealed no significant differences in guardian FPS-R scores across all arms for NCS and EMG. A significant difference in the distribution of post-NCS FPS-R score severities in children aged 7Y-18Y was noted between OA and placebo (p = .007). EMG was more painful than NCS across all arms (p < .05). In children aged 6M-6Y undergoing at least 10 muscle samplings during EMG, those receiving COTA had significantly lower pain scores (p = .014).Discussion: This study reveals the complexity of pediatric pain perception during NCS/EMG and highlights that other methods to reduce experienced pain are required. Our findings suggest that procedural characteristics, such as number of muscles sampled, may influence the effectiveness of analgesia and serve as a foundation for future research aimed at optimizing pain management strategies.","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":null,"pages":null},"PeriodicalIF":2.8,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140876869","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