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Evaluating disease status in idiopathic inflammatory myopathies with quantitative muscle ultrasound Muscle Nerve (IF 3.4) Pub Date : 2024-03-16 Jie Ying Tan, Cheng Yin Tan, Mohd Azly Yahya, Nortina Shahrizaila, Khean Jin Goh
Introduction/AimsMuscle 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.MethodsPatients with IIM, excluding inclusion body myositis, were recruited along with age‐ and sex‐matched healthy
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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 : 2024-03-16 Makoto Nomura, Yoichiro Aoyagi, Toshiaki Suzuki
Introduction/AimsMental 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
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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
CONFLICT OF INTEREST STATEMENT Dr. Hearn has no conflicts of interest to disclose. Dr. Simmons has received consultation fees from Amylyx, Biogen, Clene, and Corcept, and research support from Sanofi.
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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
Introduction/AimsNerve 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.MethodsIn 5099 participants (aged 40–79 years) without type 2 diabetes of The Maastricht Study, NCSs of peroneal
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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
Introduction/AimsWhile 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)
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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
Introduction/AimsThe 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
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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
Introduction/AimsObjective 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.MethodsThis was a retrospective review of 16 children (8 females and 8 males; median age, 9.7 years; interquartile range, 6–13
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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
Introduction/AimsDuchenne 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
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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
Introduction/AimsLumbosacral 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
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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
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Assessment of the upper limb function, strength, and mobility in treatment-naive children with spinal muscular atrophy Types 2 and 3 Muscle Nerve (IF 3.4) Pub Date : 2024-01-18 Evelin Milev, Victoria Selby, Amy Wolfe, Annemarie Rohwer, Ricarda Tillmann, Danielle Ramsey, Mario Iodice, Jean-Yves Hogrel, Giovanni Baranello, Mariacristina Scoto, Francesco Muntoni
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Segmental motor neuron dysfunction in amyotrophic lateral sclerosis: Insights from H reflex paradigms Muscle Nerve (IF 3.4) Pub Date : 2024-01-14 José Castro, Miguel Oliveira Santos, Michael Swash, Mamede de Carvalho
In amyotrophic lateral sclerosis (ALS), the role of spinal interneurons in ALS is underrecognized. We aimed to investigate pre- and post-synaptic modulation of spinal motor neuron excitability by studying the H reflex, to understand spinal interneuron function in ALS.
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Novel uses of complement inhibitors in myasthenia gravis—Two case reports Muscle Nerve (IF 3.4) Pub Date : 2024-01-11 Sean Zadeh, Hayley Price, Reed Drews, Marc A. Bouffard, Lucy H. Young, Pushpa Narayanaswami
Myasthenia gravis (MG) is a rare, life-threatening immune-related adverse effect (irAE) of immune checkpoint inhibitor (ICI) treatment. C5-complement inhibitors are effective treatments for acetylcholine receptor antibody (AChR ab) positive generalized MG. We describe the use of eculizumab/ravulizumab in two patients with MG receiving concomitant pembrolizumab.
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A longitudinal study of disease progression in facioscapulohumeral muscular dystrophy (FSHD) Muscle Nerve (IF 3.4) Pub Date : 2024-01-08 Anika Varma, Michael S. Todinca, Katy Eichinger, Susanne Heininger, Nuran Dilek, William Martens, Rabi Tawil, Jeffrey Statland, John T. Kissel, Michael P. McDermott, Chad Heatwole
In preparation for clinical trials, it is important to better understand how disease burden changes over time in facioscapulohumeral muscular dystrophy (FSHD) and to assess the capability of select metrics to detect these changes. This study aims to evaluate FSHD disease progression over 1 year and to examine the sensitivity of several outcome measures in detecting changes during this interval.
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The value of non-enhanced MRI in the evaluation of patients with suspected idiopathic inflammatory myopathy Muscle Nerve (IF 3.4) Pub Date : 2024-01-10 Adrian Alexander Marth, Clarissa Hosse, Jin Yamamura, Simone Went, Raid Al Shinibr, Pimrapat Gebert, Johannes Kolck, Oliver Keresztyen, Martin Krusche, Sarah Keller, Elif Can
The performance of magnetic resonance imaging (MRI) for diagnosing suspected idiopathic inflammatory myopathy (IIM) remains controversial. Furthermore, the role of contrast-enhanced magnetic resonance imaging (CE-MRI) sequences is unclear. The aim of this study was to evaluate the sensitivity and specificity of a non-enhanced magnetic resonance imaging (NE-MRI) protocol compared to a CE-MRI protocol
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Diagnosis and treatment of hereditary transthyretin amyloidosis with polyneuropathy in the United States: Recommendations from a panel of experts Muscle Nerve (IF 3.4) Pub Date : 2024-01-04 Chafic Karam, Michelle L. Mauermann, Alejandra Gonzalez-Duarte, Michelle C. Kaku, Senda Ajroud-Driss, Thomas H. Brannagan, Michael Polydefkis
Hereditary transthyretin (ATTRv; v for variant) amyloidosis is a rare, multisystem, progressive, and fatal disease in which polyneuropathy is a cardinal manifestation. Due to a lack of United States (US)-specific guidance on ATTRv amyloidosis with polyneuropathy, a panel of US-based expert clinicians convened to address identification, monitoring, and treatment of this disease. ATTRv amyloidosis with
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Quantitative muscle ultrasound in adult spinal muscular atrophy. A pilot study Muscle Nerve (IF 3.4) Pub Date : 2023-12-29 Luciana Pelosi, Miriam Rodrigues, Cathy Zhong, Shilpan Patel, Richard Roxburgh
Muscle ultrasound has been investigated in children with spinal muscular atrophy (SMA) and proposed as a potential biomarker of disease severity. We studied the ultrasound properties in adults with SMA to see whether they also have potential as markers of disease severity in older patients.
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Training factors that influence electrodiagnostic medicine knowledge Muscle Nerve (IF 3.4) Pub Date : 2023-12-29 Lawrence R. Robinson
Self-assessment examinations (SAEs) help trainees assess their progress in education. SAEs also provide feedback to training programs as to how factors in training influence examination performance. This study's goal was to examine the relationship between the number of months of training in electrodiagnostic (EDx) medicine, the number of EDx studies during training, and scores on the American Association
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Pregnancy planning may impact maternal and neonatal outcomes in people with myasthenia gravis Muscle Nerve (IF 3.4) Pub Date : 2023-12-29 Saja Anabusi, Aaron Izenberg, Carolina Barnett, Anne Berndl
Myasthenia Gravis (MG) is an acquired autoimmune condition commonly diagnosed in young people of reproductive age resulting in neuromuscular junction dysfunction. The course of MG during pregnancy and its impact on maternal and neonatal outcomes is vary in the literature. Pregnancy planning is a known strategy and modifiable risk factor in obstetric practice to decrease maternal and neonatal morbidity
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Home-based multi-sensory and proximal strengthening program to improve balance in Charcot–Marie–Tooth disease Type 1A: A proof of concept study Muscle Nerve (IF 3.4) Pub Date : 2023-12-29 Magdalena M. Dudziec, Laurence E. Lee, Charlotte Massey, David Tropman, Mariola Skorupinska, Matilde Laurá, Mary M. Reilly, Gita M. Ramdharry
People with Charcot–Marie–Tooth Disease (CMT) frequently report problems with balance, which lead to an increased risk of falls. Evidence is emerging of training interventions to improve balance for people with CMT, but to date all have relied on clinic-based treatment and equipment. This proof-of-concept study explored whether a multi-modal program of proprioceptive rehabilitation and strength training
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Gastrointestinal and genitourinary symptoms in facioscapulohumeral muscular dystrophy: Prevalence and impact Muscle Nerve (IF 3.4) Pub Date : 2023-12-29 Michael R. Cole, Christopher S. Cooper, Eyad M. Hanna, M. Bridget Zimmerman, June Kinoshita, Katherine D. Mathews
Anecdotally, patients with facioscapulohumeral muscular dystrophy (FSHD) describe gastrointestinal (GI) and genitourinary (GU) symptoms. We explored the prevalence of GI and GU symptoms and their impact on quality of life (QOL) in people with FSHD compared to healthy household controls.
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The role of artificial intelligence in electrodiagnostic and neuromuscular medicine: Current state and future directions Muscle Nerve (IF 3.4) Pub Date : 2023-12-27 Mohamed A. Taha, John A. Morren
The rapid advancements in artificial intelligence (AI), including machine learning (ML), and deep learning (DL) have ushered in a new era of technological breakthroughs in healthcare. These technologies are revolutionizing the way we utilize medical data, enabling improved disease classification, more precise diagnoses, better treatment selection, therapeutic monitoring, and highly accurate prognostication
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Another step forward for neuromuscular ultrasound Muscle Nerve (IF 3.4) Pub Date : 2023-12-20 James B. Caress, Ethan R. Wiesler
CONFLICT OF INTEREST STATEMENT Drs. Caress and Wiesler report no conflicts of interest with the material presented in the editorial.
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Latency and distance Muscle Nerve (IF 3.4) Pub Date : 2023-12-21 Francis O. Walker, Frederick Kremkau
See article on pages 148–156 in this issue.
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Diaphragmatic ultrasonography as a predictor of respiratory muscle fatigue in myasthenia gravis Muscle Nerve (IF 3.4) Pub Date : 2023-12-21 Jing Ma, Huiqiu Zhang, Xiaomin Pang, Jing Zhang, Rongjuan Zhao, Juan Wang, Xueli Chang, Junhong Guo, Wei Zhang
Easy fatigability, the clinical hallmark of generalized myasthenia gravis (GMG), cannot be detected in a dynamic way. The aim of this study was to assess respiratory function dynamically through diaphragmatic ultrasonography (DUS) in GMG patients.
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Neuromuscular implants: Interfacing with skeletal muscle for improved clinical translation of prosthetic limbs Muscle Nerve (IF 3.4) Pub Date : 2023-12-21 Kiara N. Quinn, Yucheng Tian, Ryan Budde, Pedro P. Irazoqui, Sami Tuffaha, Nitish V. Thakor
After an amputation, advanced prosthetic limbs can be used to interface with the nervous system and restore motor function. Despite numerous breakthroughs in the field, many of the recent research advancements have not been widely integrated into clinical practice. This review highlights recent innovations in neuromuscular implants—specifically those that interface with skeletal muscle—which could
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Caffeine and cortical excitability, as measured with paired-pulse transcranial magnetic stimulation Muscle Nerve (IF 3.4) Pub Date : 2023-12-21 Kristine Strunge, Hugh Bostock, James Howells, Bülent Cengiz, Gintaute Samusyte, Martin Koltzenburg, Hatice Tankisi
The transcranial magnetic stimulation tests of short-interval intracortical inhibition (SICI) by both conventional amplitude measurements (A-SICI) and threshold-tracking (T-SICI) are important methods to investigate intracortical inhibitory circuits, and T-SICI has been proposed to aid the diagnosis of amyotrophic lateral sclerosis. Beverages containing caffeine are widely consumed, and caffeine has
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Advancing electrical impedance myography one small step at a time Muscle Nerve (IF 3.4) Pub Date : 2023-12-21 Seward B. Rutkove
See article on pages 288–294 in this issue.
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Initial validation of the Mass. General Neuropathy Exam Tool (MAGNET) for evaluation of distal small-fiber neuropathy Muscle Nerve (IF 3.4) Pub Date : 2023-12-19 Gary R. Zirpoli, Khosro Farhad, Madeleine C. Klein, Sean Downs, Max M. Klein, Anne Louise Oaklander
Diagnosis of small-fiber neuropathy (SFN) is hampered by its subjective symptoms and signs. Confirmatory testing is insufficiently available and expensive, so predictive examinations have value. However, few support the 2020 SFN consensus-case-definition requirements or were validated for non-diabetes neuropathies. Thus we developed the Massachusetts General Hospital Neuropathy Exam Tool (MAGNET) and