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Debamestrocel multimodal effects on biomarker pathways in amyotrophic lateral sclerosis are linked to clinical outcomes Muscle Nerve (IF 3.4) Pub Date : 2024-04-09 Stacy R. Lindborg, Namita A. Goyal, Jonathan Katz, Matthew Burford, Jenny Li, Haggai Kaspi, Natalie Abramov, Bruno Boulanger, James D. Berry, Katharine Nicholson, Tahseen Mozaffar, Robert Miller, Liberty Jenkins, Robert H. Baloh, Richard Lewis, Nathan P. Staff, Margaret Ayo Owegi, Bob Dagher, Netta R. Blondheim‐Shraga, Yael Gothelf, Yossef S. Levy, Ralph Kern, Revital Aricha, Anthony J. Windebank,
Introduction/AimsBiomarkers have shown promise in amyotrophic lateral sclerosis (ALS) research, but the quest for reliable biomarkers remains active. This study evaluates the effect of debamestrocel on cerebrospinal fluid (CSF) biomarkers, an exploratory endpoint.MethodsA total of 196 participants randomly received debamestrocel or placebo. Seven CSF samples were to be collected from all participants
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Feasibility of virtual reality and comparison of its effectiveness to biofeedback in children with Duchenne and Becker muscular dystrophies Muscle Nerve (IF 3.4) Pub Date : 2024-04-01 Merve Kurt‐Aydin, Dilan Savaş‐Kalender, Tülay Tarsuslu, Uluç Yis
Introduction/AimsThe utilization of virtual reality (VR) and biofeedback training, while effective in diverse populations, remains limited in the treatment of Duchenne and Becker muscular dystrophies (D/BMD). This study aimed to determine the feasibility of VR in children with D/BMD and compare the effectiveness of VR and biofeedback in children with D/BMD.MethodsThe study included 25 children with
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Pseudoexon activation by deep intronic variation in GNE myopathy with thrombocytopenia Muscle Nerve (IF 3.4) Pub Date : 2024-04-01 Kexin Jiao, Nachuan Cheng, Xiao Huan, Jialong Zhang, Yu Ding, Xinghua Luan, LingChun Liu, Xilu Wang, Bochen Zhu, Kunzhao Du, Jiale Fan, Mingshi Gao, Xingyu Xia, Ningning Wang, Tao Wang, Jianying Xi, Sushan Luo, Jiahong Lu, Chongbo Zhao, Dongyue Yue, Wenhua Zhu
Introduction/AimsGNE myopathy is a rare autosomal recessive disorder caused by pathogenic variants in the GNE gene, which is essential for the sialic acid biosynthesis pathway. Although over 300 GNE variants have been reported, some patients remain undiagnosed with monoallelic pathogenic variants. This study aims to analyze the entire GNE genomic region to identify novel pathogenic variants.MethodsPatients
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FKRP‐related muscular dystrophy responsive to immunotherapy Muscle Nerve (IF 3.4) Pub Date : 2024-04-01 S. Waller, J. Stockwell, T. Tay, R. Pamphlett, S. Beuzeville, W. Huynh
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Cross‐sectional study of patients with VCP multisystem proteinopathy 1 using dual‐energy x‐ray absorptiometry Muscle Nerve (IF 3.4) Pub Date : 2024-03-29 Rod Carlo Agram Columbres, Vu Luu, Minh Nguyen, Virginia Kimonis
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Hyperexcitability of the asymptomatic motor cortex in a case of Mills' syndrome Muscle Nerve (IF 3.4) Pub Date : 2024-03-29 Martin T. Conwill, James J. P. Alix, Pamela J. Shaw
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Motor unit number estimation via MScanFit MUNE in spinal muscular atrophy Muscle Nerve (IF 3.4) Pub Date : 2024-03-29 Veria Vacchiano, Francesca Morabito, Claudia Faini, Giovanna Nocera, Riccardo Not, Gaia Scarpini, Martina Romagnoli, Antonella Pini, Rocco Liguori
Introduction/AimsMScanFit MUNE (MScanFit) is a novel tool to derive motor unit number estimates (MUNEs) from compound muscle action potential (CMAP) scans. Few studies have explored its utility in 5q spinal muscular atrophy (SMA5q) patients, assessing only the abductor pollicis brevis (APB) muscle. We aimed to assess different distal muscles in pediatric and adult SMA5q patients, further evaluating
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Serial electrodiagnostic testing: Utility and indications in adult neurological disorders Muscle Nerve (IF 3.4) Pub Date : 2024-03-29 Sandra L. Hearn, Amro Maher Stino, Ileana M. Howard, Gautam Malhotra, Lawrence Robinson
Although existing guidelines address electrodiagnostic (EDX) testing in identifying neuromuscular conditions, guidance regarding the uses and limitations of serial (or repeat) EDX testing is limited. By assessing neurophysiological change longitudinally across time, serial electrodiagnosis can clarify a diagnosis and potentially provide valuable prognostic information. This monograph presents four
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Redundant nerve roots on magnetic resonance imaging can predict ongoing denervation in patients with lumbar spinal stenosis Muscle Nerve (IF 3.4) Pub Date : 2024-03-28 Seoyeong Park, Sung Hwan Hong, Sun Gun Chung, Keewon Kim
Introduction/AimsRedundant nerve roots (RNRs) are abnormally elongated and tortuous nerve roots that develop secondary to degenerative spinal stenosis. RNRs have been associated with poorer clinical outcomes after decompression surgery; however, studies on their clinical characteristics are limited. This study aimed to investigate the association between RNRs and denervation potentials, that is, abnormal
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Hereditary motor sensory neuropathy with proximal involvement (HMSN‐P) associated with TFG p.Pro285Leu variant in an Italian family with a motor neuron disease‐like clinical picture Muscle Nerve (IF 3.4) Pub Date : 2024-03-27 Sara Cabras, Francesca Di Pede, Antonio Canosa, Maurizio Grassano, Tiziana Enrica Mongini, Giulio Gadaleta, Andrea Calvo, Adriano Chiò, Cristina Moglia, Salvatore Gallone
CONFLICT OF INTEREST STATEMENT None of the authors has any conflict of interest to disclose.
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The utility of electrodiagnostic testing in unprovoked rhabdomyolysis in the era of next‐generation sequencing Muscle Nerve (IF 3.4) Pub Date : 2024-03-27 Michael P. Skolka, Margherita Milone, William J. Litchy, Ruple S. Laughlin, Devon I. Rubin, Teerin Liewluck
Introduction/AimsRhabdomyolysis is an etiologically heterogeneous, acute necrosis of myofibers characterized by transient marked creatine kinase (CK) elevation associated with myalgia, muscle edema, and/or weakness. The study aimed to determine the role of electrodiagnostic (EDX) testing relative to genetic testing and muscle biopsy in patients with unprovoked rhabdomyolysis in identifying an underlying
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Botulinum toxin injections for the treatment of neurogenic thoracic outlet syndrome: A systematic review Muscle Nerve (IF 3.4) Pub Date : 2024-03-26 Tyler T. Woodworth, Austin Le, Campbell Miller, Aaron Conger, Mark A. Mahan, Daniel M. Cushman
Botulinum toxin (BTX) injections into the musculature surrounding the brachial plexus have been examined as a potential treatment for neurogenic thoracic outlet syndrome (nTOS). This systematic review identified 15 publications, of which one was a randomized controlled trial. BTX injections performed with ultrasound or electromyographic guidance, and with the inclusion of the pectoralis minor muscle
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Quantitative ultrasonography reveals skeletal muscle abnormalities in carriers of DMD pathogenic variants Muscle Nerve (IF 3.4) Pub Date : 2024-03-22 Bruna Melo Coelho Loureiro, Mariana Rabelo de Brito, Cristina Iwabe, Sergio San Juan Dertkigil, Marcondes C. França
Introduction/AimsCarriers of DMD pathogenic variants may become symptomatic and develop muscle‐related manifestations. Despite that, few studies have attempted to characterize changes in the muscles of these carriers using imaging tools, particularly muscle ultrasound (MUS). The aim of this study was to compare lower limb MUS findings in carriers of DMD pathogenic variants (cDMD) vs healthy controls
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Cardiopulmonary exercise testing on adaptive equipment in children and adults with Friedreich ataxia Muscle Nerve (IF 3.4) Pub Date : 2024-03-21 Nicolette A. Cilenti, Jaclyn G. Tamaroff, Christopher J. Capiola, Walter Faig, Michael G. McBride, Stephen M. Paridon, Shannon O'Malley, Jonathan B. Edelson, David R. Lynch, Shana E. McCormack, Kimberly Y. Lin
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Longitudinal data of serum creatine kinase levels and motor, pulmonary, and cardiac functions in 337 patients with Duchenne muscular dystrophy Muscle Nerve (IF 3.4) Pub Date : 2024-03-21 Hiroyuki Awano, Yoshinori Nambu, Chieko Itoh, Akihiro Kida, Tetsushi Yamamoto, Tomoko Lee, Yasuhiro Takeshima, Kandai Nozu, Masafumi Matsuo
Duchenne muscular dystrophy (DMD) presents with skeletal muscle weakness, followed by cardiorespiratory involvement. The need for longitudinal data regarding DMD that could serve as a control for determining treatment efficacy in clinical trials has increased notably. The present study examined the longitudinal data of Japanese DMD patients collectively and assessed individual patients with pathogenic
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Evaluating disease status in idiopathic inflammatory myopathies with quantitative muscle ultrasound Muscle Nerve (IF 3.4) Pub Date : 2024-03-15 Jie Ying Tan, Cheng Yin Tan, Mohd Azly Yahya, Nortina Shahrizaila, Khean Jin Goh
Muscle strength, functional status, and muscle enzymes are conventionally used to evaluate disease status in idiopathic inflammatory myopathies (IIM). This study aims to investigate the role of quantitative muscle ultrasound in evaluating disease status in IIM patients.
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Changes in the excitability of anterior horn cells in a mental rotation task of body parts Muscle Nerve (IF 3.4) Pub Date : 2024-03-15 Makoto Nomura, Yoichiro Aoyagi, Toshiaki Suzuki
Mental rotation (MR), a tool of implicit motor imagery, is the ability to rotate mental representations of two- or three-dimensional objects. Although many reports have described changes in brain activity during MR tasks, it is not clear whether the excitability of anterior horn cells in the spinal cord can be changed. In this study, we examined whether MR tasks of hand images affect the excitability
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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 Muscle Nerve (IF 3.4) Pub Date : 2024-03-14 Joel Iff, Nicolae Done, Edward Tuttle, Yi Zhong, Fangzhou Wei, Basil T. Darras, Craig M. McDonald, Eugenio Mercuri, Francesco Muntoni
Introduction/AimsEteplirsen, 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.MethodsUS patients with DMD receiving eteplirsen were followed
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Singer and songwriter: Enhancing the dual functions of the electrodiagnostic report Muscle Nerve (IF 3.4) Pub Date : 2024-03-13 Sandra L. Hearn, Zachary Simmons
See article on pages 620–625 in this issue.
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Neuromuscular consequences of spinal cord injury: New mechanistic insights and clinical considerations Muscle Nerve (IF 3.4) Pub Date : 2024-03-13 Mathew I. B. Debenham, Colin K. Franz, Michael J. Berger
The spinal cord facilitates communication between the brain and the body, containing intrinsic systems that work with lower motor neurons (LMNs) to manage movement. Spinal cord injuries (SCIs) can lead to partial paralysis and dysfunctions in muscles below the injury. While traditionally this paralysis has been attributed to disruptions in the corticospinal tract, a growing body of work demonstrates
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Reference values for nerve conduction studies of the peroneal, tibial, and sural nerve derived from a large population-based cohort: Associations with demographic and anthropometric characteristics—The Maastricht study Muscle Nerve (IF 3.4) Pub Date : 2024-03-09 Jurriaan J. A. Hodzelmans, Marcus L. F. Janssen, Jos P. H. Reulen, Paul J. Blijham, Annemarie Koster, Coen D. A. Stehouwer, Werner H. Mess, Nadia A. Sutedja
Nerve conduction studies (NCSs) are widely used to support the clinical diagnosis of neuromuscular disorders. The aims of this study were to obtain reference values for peroneal, tibial, and sural NCSs and to examine the associations with demographic and anthropometric factors.
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Ultrahigh-frequency ultrasound of fascicles in the common fibular, superficial fibular, and sural nerves Muscle Nerve (IF 3.4) Pub Date : 2024-03-08 James B. Meiling, Nirmal Andrapalliyal, Marisa Barclay McGhee, Vanessa Baute Penry, Michael S. Cartwright, Rachana K. Gandhi Mehta
While ultrasound assessment of cross-sectional area and echogenicity has gained popularity as a biomarker for various neuropathies, there is a scarcity of data regarding fascicle count and density in neuropathies or even healthy controls. The aim of this study was to determine whether fascicles within select lower limb nerves (common fibular, superficial fibular, and sural nerves) can be counted in
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Epidemiology of seropositive myasthenia gravis in Sardinia: A population-based study in the district of Sassari Muscle Nerve (IF 3.4) Pub Date : 2024-03-08 Elia Sechi, Giovanni A. Deiana, Mariangela Puci, Pietro Zara, Enzo Ortu, Caterina Porcu, Nicola Carboni, Paola Chessa, Elisa Ruiu, Arianna Nieddu, Paolo Tacconi, Antonello Russo, Davide Manca, M. Margherita Sechi, Melania Guida, Roberta Ricciardi, Tommaso Ercoli, Marcello M. Mascia, Antonella Muroni, Paolo Profice, Valeria Saddi, Maurizio Melis, Eleonora Cocco, Gregorio Spagni, Raffaele Iorio, Valentina
The global incidence and prevalence of myasthenia gravis (MG) range between 6–31/million and 10–37/100,000, respectively. Sardinia is a high-risk region for different immune-mediated disorders, but the epidemiology of MG remains unclear. We determined the epidemiology of MG with acetylcholine receptor (AChR)-immunoglobulin G (IgG) and muscle-specific tyrosine kinase (MuSK)-IgG in the district of Sassari
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Outcome measures in pediatric chronic inflammatory demyelinating polyradiculoneuropathy Muscle Nerve (IF 3.4) Pub Date : 2024-03-04 Apoorva Guttikonda, Ghazal Ahmad, Parul Goyal, Yijin Xiang, Laura Michelle Johnson, Scott Gillespie, Kimberly T. Carvell, Robert Butera, Sumit Verma
Objective outcome measures in children undergoing treatment for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) are lacking. The aim of the study was to record serial grip strength and motor nerve conduction studies to assess interval change.
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Electrodiagnostic studies and new diagnostic modalities for evaluation of peripheral nerve disorders Muscle Nerve (IF 3.4) Pub Date : 2024-03-04 Andrew Hannaford, Elijah Paling, Matthew Silsby, Sanne Vincenten, Nens van Alfen, Neil G. Simon
Electrodiagnostic studies (EDx) are frequently performed in the diagnostic evaluation of peripheral nerve disorders. There is increasing interest in the use of newer, alternative diagnostic modalities, in particular imaging, either to complement or replace established EDx protocols. However, the evidence to support this approach has not been expansively reviewed. In this paper, diagnostic performance
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Cardiac atrial pathology in Duchenne muscular dystrophy Muscle Nerve (IF 3.4) Pub Date : 2024-03-01 Eleanor Greiner, Andrea Breaux, Jennifer Kasten, JangDong Seo, Nicholas J. Ollberding, David Spar, Thomas D. Ryan, Sean M. Lang, Cuixia Tian, Hemant Sawnani, Chet R. Villa
Duchenne muscular dystrophy (DMD) is characterized by fibrofatty replacement of muscle. This has been documented in the ventricular myocardium of DMD patients, but there is limited description of atrial involvement. The purpose of this study is to examine the arrhythmia and ectopy burden in patients with DMD and non-DMD dilated cardiomyopathy (DCM) and to characterize the cardiac histopathologic changes
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Chronic glucocorticoid management in neuromuscular disease: A survey of neuromuscular neurologists Muscle Nerve (IF 3.4) Pub Date : 2024-02-27 Lora Stepanian, Ruple S. Laughlin, Corey Bacher, Aaron Izenberg, Victoria Hodgkinson, Adrienna Dyck, Ari Breiner, Charles D. Kassardjian
Introduction/AimsGlucocorticoids (GC) are first‐line therapy for many neuromuscular diseases. There is a lack of guidelines regarding the prevention and management of GC complications in the context of neuromuscular disease, introducing the potential for practice variation, that may compromise quality of care. Our aim was to evaluate the practice patterns among Canadian adult neuromuscular neurologists
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Neuromuscular ultrasound changes in unilateral symptomatic subacute lumbosacral radiculopathy: A prospective simple blinded cohort study Muscle Nerve (IF 3.4) Pub Date : 2024-02-23 David Cuendet, Daniele Valsecchi, Hugo Najberg, Gianluca Maestretti, Friedrich Medlin
Lumbosacral radiculopathy (LR) is a common disorder. Neuromuscular ultrasound (NMU) is a rapidly evolving technique for the investigation of peripheral nerve and muscle disorders, but studies using NMU in LR are lacking. The aim of the present study was to investigate ultrasonographic neuromuscular changes distant from root compression in patients with subacute to chronic compressive LR with motor
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Efficacy of ravulizumab in patients with generalized myasthenia gravis by time from diagnosis: A post hoc subgroup analysis of the CHAMPION MG study Muscle Nerve (IF 3.4) Pub Date : 2024-02-21 James F. Howard, Tuan Vu, Renato Mantegazza, Hani Kushlaf, Shigeaki Suzuki, Heinz Wiendl, Kathleen N. Beasley, Serena Liao, Andreas Meisel
The CHAMPION MG study demonstrated that ravulizumab significantly improved Myasthenia Gravis-Activities of Daily Living (MG-ADL) and Quantitative Myasthenia Gravis (QMG) total scores versus placebo in adults with acetylcholine receptor antibody-positive generalized myasthenia gravis (AChR+ gMG). This post hoc analysis aimed to assess these outcomes by time from MG diagnosis.
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Patterns and assessment of spastic hemiplegic gait Muscle Nerve (IF 3.4) Pub Date : 2024-02-19 Sheng Li
Hemiparetic gait disorders are common in stroke survivors. A circumductory gait is often considered the typical hemiparetic gait. In clinical practice, a wide spectrum of abnormal gait patterns is observed, depending on the severity of weakness and spasticity, and the anatomical distribution of spasticity. Muscle strength is the key determinant of gait disorders in hemiparetic stroke survivors. Spasticity
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Sports-related peripheral nerve injuries of the upper limb Muscle Nerve (IF 3.4) Pub Date : 2024-02-19 Rebecca A. Dutton, John Norbury, Berdale Colorado
Peripheral nerve injuries in athletes affect the upper limb more commonly than the lower limb. Common mechanisms include compression, traction, laceration, and ischemia. Specific sports can have unique mechanisms of injury and are more likely to be associated with certain neuropathies. Familiarity with these sport-specific variables and recognition of the common presentations of upper limb neuropathic
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Myxovirus resistance protein A (MxA) expression in myositides: Sarcoplasmic expression is common in both dermatomyositis and lupus myositis Muscle Nerve (IF 3.4) Pub Date : 2024-02-19 Changhong Xing, Jaya Trivedi, Nicole Bitencourt, Dennis K. Burns, Joan S. Reisch, Chunyu Cai
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Diagnosing CIDP: Can we look forward by looking back? Muscle Nerve (IF 3.4) Pub Date : 2024-02-17 Nens van Alfen
See article on pages 397–402 in this issue.
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Correction to “Muscle MRI in periodic paralysis shows myopathy is common and correlates with intramuscular fat accumulation” Muscle Nerve (IF 3.4) Pub Date : 2024-02-15
Vivekanandam V, Suetterlin K, Matthews E, et al. Muscle MRI in periodic paralysis shows myopathy is common and correlates with intramuscular fat accumulation. Muscle Nerve 2023;68(4):439-450. doi:10.1002/mus.27947 In the above article, the second author's last name was misspelled as “Seutterlin.” The correct spelling is “Suetterlin.” The article has been corrected. We apologize for this error.
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Ultrasonographic evaluation of ulnar nerve morphology in patients with ulnar nerve instability Muscle Nerve (IF 3.4) Pub Date : 2024-02-15 Narmin Ahmadli, Kenan Akgun, Rana Terlemez, Tugce Ozekli Misirlioglu, Deniz Palamar
Ulnar nerve instability (UNI) in the retroepicondylar groove is described as nerve subluxation or dislocation. In this study, considering that instability may cause chronic ulnar nerve damage by increasing the friction risk, we aimed to examine the effects of UNI on nerve morphology ultrasonographically.
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Clinical prognostic factors predicting survival of motor neuron disease patients with gastrostomy: A retrospective analysis Muscle Nerve (IF 3.4) Pub Date : 2024-02-14 Jie Yang, Yun Zhao, Mario Soares, Merrilee Needham, Andrea Begley, Emily Calton
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Pilot study of a virtual weight management program for Duchenne muscular dystrophy Muscle Nerve (IF 3.4) Pub Date : 2024-02-14 Natassja Billich, Paula Bray, Helen Truby, Maureen Evans, Kate Carroll, Katy de Valle, Justine Adams, Rachel A. Kennedy, Daniella Villano, Andrew J. Kornberg, Eppie M. Yiu, Monique M. Ryan, Zoe E. Davidson
Young people with Duchenne muscular dystrophy (DMD) are at increased risk of obesity. Weight management is important to families; however, several barriers exist. This pilot study aimed to investigate the feasibility and acceptability of a co-designed weight management program for DMD.
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Exploring caregivers' attitudes and beliefs about nutrition and weight management for young people with Duchenne muscular dystrophy Muscle Nerve (IF 3.4) Pub Date : 2024-02-14 Natassja Billich, Paula Bray, Helen Truby, Maureen Evans, Monique M. Ryan, Kate Carroll, Katy de Valle, Daniella Villano, Andrew Kornberg, Bianca Sowerby, Michelle A. Farrar, Manoj P. Menezes, Sandra Holland, Rachel Lindeback, Anita Cairns, Zoe E. Davidson
Obesity disproportionately affects children and adolescents with Duchenne muscular dystrophy (DMD) and with adverse consequences for disease progression. This study aims to: explore barriers, enablers, attitudes, and beliefs about nutrition and weight management; and to obtain caregiver preferences for the design of a weight management program for DMD.
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The impact of myasthenia gravis severity on work and daily activities Muscle Nerve (IF 3.4) Pub Date : 2024-02-13 Jacqueline Pesa, Zia Choudhry, Jonathan de Courcy, Sophie Barlow, Emma Chatterton, Owen Thomas, Gregor Gibson, Bethan Hahn, Raghav Govindarajan
People with myasthenia gravis (MG) experience impaired quality of life. However, the impact of MG symptoms on work productivity has not been well-studied. We aimed to evaluate this impact and to examine associations between disease severity and the degree of impairment.
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Correction to “Femoral neuropathy: A clinical and electrodiagnostic review” Muscle Nerve (IF 3.4) Pub Date : 2024-02-09
Santilli, AR, Martinez-Thompson, JM, Speelziek, SJA, Staff, NP, Laughlin, RS. Femoral neuropathy: a clinical and electrodiagnostic review. Muscle Nerve 2023; 69(1): 64–71. In the footnote below the authors' names, the text “Presented as a poster at the 75th annual meeting of the American Association of Neurology, Boston, MA, April 2023” was incorrect. This should have read: “Presented as a poster at
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Multifocal motor neuropathy as a mimic of amyotrophic lateral sclerosis: Serum neurofilament light chain as a reliable diagnostic biomarker Muscle Nerve (IF 3.4) Pub Date : 2024-02-09 Vera E. A. Kleinveld, Omar Keritam, Corinne G. C. Horlings, Hakan Cetin, Julia Wanschitz, Anna Hotter, Laura S. Zirch, Fritz Zimprich, Raffi Topakian, Petra Müller, Dierk Oel, Stefan Quasthoff, Marcus Erdler, Helmut Rauschka, Susanne Grinzinger, Julia Jecel, Petra Gaulhofer, Barbara Castek, Klaus Stadler, Wolfgang N. Löscher
The clinical presentation of multifocal motor neuropathy (MMN) may mimic early amyotrophic lateral sclerosis (ALS) with predominant lower motor neuron (LMN) involvement, posing a diagnostic challenge. Both diseases have specific treatments and prognoses, highlighting the importance of early diagnosis. The aim of this study was to assess the diagnostic value of serum neurofilament light chain (NfL)
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Appropriate dosage, timing, and site of intramuscular injections of brain-derived neurotrophic factor (BDNF) promote motor recovery after facial nerve injury in rats Muscle Nerve (IF 3.4) Pub Date : 2024-02-08 Svenja Rink-Notzon, Jannika Reuscher, Laura Wollny, Levent Sarikcioglu, Süreyya Bilmen, Marilena Manthou, Tessa Gordon, Doychin N. Angelov
Daily intramuscular injections of fibroblast growth factor 2 (FGF2) but not of brain-derived neurotrophic factor (BDNF) significantly improve whisking behavior and mono-innervation of the rat levator labii superioris (LLS) muscle 56 days after buccal nerve transection and suture (buccal–buccal anastomosis, BBA). We explored the dose–response of BDNF, FGF2, and insulin growth factor 2 (IGF2) on the
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Neuromuscular ultrasound findings in gunshot wounds Muscle Nerve (IF 3.4) Pub Date : 2024-02-08 Patrick Fagan, Christopher D. Geiger, Gaurav Chenji, David C. Preston
A spectrum of peripheral nerve injuries is associated with gunshot wounds (GSWs). Due to Wallerian degeneration, distal nerve lesions may go undetected on electrodiagnostic (EDX) testing. In patients with GSW undergoing high-resolution ultrasound (HRUS) for evaluation of neurological deficits, we have observed distal nerve morphological changes, but these have not been systematically studied. The aim
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Needle electromyography does not meaningfully impact findings in MR-neurography/−myography Muscle Nerve (IF 3.4) Pub Date : 2024-02-07 Stefan Sondermann, Tobias Boppel, Katharina Fieseler, Peter Schramm, Tobias Bäumer, Peter Trillenberg
Magnetic resonance neurography (MRN) and myography (MRM) are emerging imaging methods for detecting diseases of the peripheral nerve system (PNS). Most patients with PNS diseases also undergo needle electromyography (EMG). This study examined whether EMG led to lesions that were detectable using MRN/MRM and whether these lesions could impair image interpretation.
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Positive predictive value of myositis antibody line blot testing in patients with suspected idiopathic inflammatory myopathy Muscle Nerve (IF 3.4) Pub Date : 2024-02-06 Yiu-Chia Chang, Liju Yang, Adrian Budhram
Line blot (LB) is in widespread use for myositis antibody detection. Yet, studies of its positive predictive value (PPV) in patients with suspected idiopathic inflammatory myopathy (IIM), which would be of particular relevance to neuromuscular clinicians, are lacking. We aimed to determine the PPV of myositis antibody LB testing in patients with suspected IIM, and examine whether PPV was significantly
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Electrodiagnostic reporting preferences of referring physicians: An exploratory survey Muscle Nerve (IF 3.4) Pub Date : 2024-02-03 Kyung Joon Mun, Jordan Farag, Lawrence R. Robinson
Electrodiagnostic (EDX) studies play a crucial role in the evaluation of patients with peripheral nervous system disorders. Accurate and succinct communication of test results is critical to patient safety and clinical decision-making. The objective of this study was to explore EDX reporting preferences of referring physicians to improve quality of communication and patient care.
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Identification of gene fusions associated with amyotrophic lateral sclerosis Muscle Nerve (IF 3.4) Pub Date : 2024-02-02 Yogindra Raghav, Allison A. Dilliott, Tiziana Petrozziello, Spencer E. Kim, James D. Berry, Merit E. Cudkowicz, Khashayar Vakili, , Ernest Fraenkel, Sali M. K. Farhan, Ghazaleh Sadri-Vakili
Genetics is an important risk factor for amyotrophic lateral sclerosis (ALS), a neurodegenerative disease affecting motor neurons. Recent findings demonstrate that in addition to specific genetic mutations, structural variants caused by genetic instability can also play a causative role in ALS. Genomic instability can lead to deletions, duplications, insertions, inversions, and translocations in the
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The CAPN3 p.Lys 254del variant is not always associated with dominant CAPN3-related muscular dystrophy Muscle Nerve (IF 3.4) Pub Date : 2024-02-01 Andrea Valls, Gerardo Gutiérrez-Gutiérrez, Agustín Martínez, Cristina Ruiz-Roldán, Pilar Camaño, Adolfo López de Munain, Amets Sáenz
Limb-girdle muscular dystrophy R1 (LGMDR1) calpain 3-related usually presents as a recessively transmitted weakness of proximal limb-girdle muscles due to pathogenic variants in the CAPN3 gene. Pathogenic variants in this gene have also been found in patients with an autosomal dominantly inherited transmission pattern (LGMDD4). The mechanism underlying this difference in transmission patterns has not
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Segmental infralesional pathological spontaneous activity in subacute traumatic spinal cord injury Muscle Nerve (IF 3.4) Pub Date : 2024-01-31 Michael J. Berger, Adenike A. Adewuyi, Christopher Doherty, Amy K. Hanlan, Cynthia Morin, Russ O'Connor, Radhika Sharma, Shannon Sproule, Kevin N. Swong, Harvey Wu, Colin K. Franz, Erin Brown
There is a dearth of knowledge regarding the status of infralesional lower motor neurons (LMNs) in individuals with traumatic cervical spinal cord injury (SCI), yet there is a growing need to understand how the spinal lesion impacts LMNs caudal to the lesion epicenter, especially in the context of nerve transfer surgery to restore several key upper limb functions. Our objective was to determine the
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Protective effect of alpha-lipoic acid and epalrestat on oxaliplatin-induced peripheral neuropathy in zebrafish Muscle Nerve (IF 3.4) Pub Date : 2024-01-31 Dong-Won Lee, Hae-Chul Park, Dong Hwee Kim
Oxaliplatin is a platinum-based anti-cancer drug widely used in colorectal cancer patients, but it may cause peripheral neuropathy. As one of the main causes of oxaliplatin-induced peripheral neuropathy (OPN) is oxidative stress, which is also a key factor causing diabetic peripheral neuropathy (DPN), the aim of this study was to evaluate the preventive effects of alpha-lipoic acid (ALA) and epalrestat
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Retraction: Utility of phrenic nerve conduction studies for identification of patients with neuromuscular diseases requiring invasive mechanical ventilation Muscle Nerve (IF 3.4) Pub Date : 2024-01-29
Retraction: Utility of phrenic nerve conduction studies for identification of patients with neuromuscular diseases requiring invasive mechanical ventilation. S. Narukawa, K. Ishizuka, K. Sugimoto, K. Nomura. Muscle & Nerve 65, 211-216. https://doi.org/10.1002/mus.27449.
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Retrospective review of patients with myasthenia gravis switched from plasma exchange therapy to efgartigimod treatment Muscle Nerve (IF 3.4) Pub Date : 2024-01-29 Anahit Mehrabyan, Rebecca E. Traub
Therapeutic plasma exchange (TPE) is sometimes used as maintenance therapy for the treatment of myasthenia gravis (MG). Efgartigimod is a newly approved monoclonal antibody targeting the neonatal Fc receptor, effectively reducing immunoglobulin G levels in the treatment of MG. The aim of this study was to describe the clinical experience of switching patients from maintenance TPE treatment to efgartigimod