Neuromuscular Medicine Research Articles

Book Review for Neuromuscular and Electrodiagnostic Medicine Board Review.

Book Review for Neuromuscular and Electrodiagnostic Medicine Board Review.

Phrenic neuropathy etiologies and recovery trajectories in outpatient rehabilitation and neuromuscular medicine clinics: A retrospective analysis.

Phrenic neuropathy (PhN) impairs diaphragm muscle function, causing a spectrum of breathing disability. PhN etiologies and their natural history are ill-defined. This knowledge gap hinders informed prognosis and management decisions. This study aims to help fill this knowledge gap on PhN etiologies, outcomes, and recovery patterns, especially in the context of nonsurgical clinical practice. This was a retrospective study from two interdisciplinary clinics, physiatry and neurology based. Patients were included if PhN was identified, and other causes of hemi-diaphragm muscle dysfunction excluded. Patients were followed serially at the discretion of the neuromuscular-trained neurologist or physiatrist. Recovery was assessed using pulmonary function tests (PFTs), diaphragm muscle ultrasound (US) thickening ratio, and patient-reported outcomes in patients presenting within 2 years of PhN onset. We identified 151 patients with PhN. The most common etiologies were idiopathic (27%), associated with cardiothoracic procedure (24%), and intensive care unit (17%). Of these patients, 117 (77%) were evaluated within 2 years of PhN onset. Of patients included in outcome analyses, 64% saw improvement on serial US, 50% on serial PFTs and 79% reported symptomatic improvement at an average of 15, 16, and 17 months, respectively. A clear majority of PhN patients show improvement in diaphragm muscle function, but on average, improvements took 15-17 months depending on the assessment type. These insights are vital for developing tailored treatments and can guide physicians in prognosis and decision-making, especially if more invasive interventions are being considered.

Newborn Screening for Spinal Muscular Atrophy: Variations in Practice and Early Management of Infants with Spinal Muscular Atrophy in the United States.

In the United States (U.S.), newborn screening (NBS) for spinal muscular atrophy (SMA) is implemented by individual states. There is likely variation in the practice patterns of state NBS programs and among the providers caring for newborns with SMA. This is a prospective, descriptive, observational study that seeks to quantify and describe practice patterns and heterogeneities in state NBS programs and provider practices in the U.S. We surveyed U.S. state NBS programs and care providers of newborns with SMA. Thirty states and 41 practitioners responded. NBS program practices vary by state. Most (74%) state programs provide results to both primary care and specialist providers and also defer confirmatory SMA testing to those providers. Two states had relatively high rates of false-positive or inclusive results. The total birth prevalence of SMA was 1:13,862. Most providers were in tertiary care centers (90%) and were child neurologists (81%) and/or had fellowship training in Neuromuscular Medicine or Electromyography (76%). All providers see new referrals in less than a week, but many do not initiate treatment until >3 weeks of age (39%), with most commonly reported delays related to insurance processes. Most (81%) prefer onasemnogene abeparvovec-xioi (OA) as the treatment of choice, mainly due to perceived efficacy and the route/frequency of administration. NBS practice patterns in the U.S. vary by state but overall yielded the predicted birth prevalence of positive results. Providers evaluate these newborns urgently, but many do not initiate therapy until after 3 weeks of age. Treatment delays are mainly related to insurance processes.

Real-time artificial intelligence-based texture analysis of muscle ultrasound data for neuromuscular disorder assessment

ObjectiveMany artificial intelligence approaches to muscle ultrasound image analysis have not been implemented on usable devices in clinical neuromuscular medicine practice, owing to high computational demands and lack of standardised testing protocols. This study evaluated the feasibility of using real-time texture analysis to differentiate between various pathological conditions. MethodsWe analysed 17,021 cross-sectional ultrasound images of the biceps brachii of 75 participants, including 25 each with neurogenic disorders, myogenic disorders, and healthy controls. The size and location of the regions of interest were randomly selected to minimise bias. A random forest classifier utilising texture features such as Dissimilarity and Homogeneity was developed and deployed on a mobile PC, enabling real-time analysis. ResultsThe classifier distinguished patients with an accuracy of 81 %. Echogenicity and Contrast from the Co-Occurrence Matrix were significant predictive features. Validation on 15 patients achieved accuracies of 78 %/93 % per image/patient over 15-second videos, respectively. The use of a mobile PC facilitated real-time estimation of the underlying pathology during ultrasound examination, without influencing procedures. ConclusionsReal-time automatic texture analysis is feasible as an adjunct for the diagnosis of neuromuscular disorders. SignificanceArtificial intelligence using texture analysis with a light computational load supports the semi-quantitative evaluation of neuromuscular ultrasound.

Role of artificial intelligence in neuromuscular and electrodiagnostic medicine.

Role of artificial intelligence in neuromuscular and electrodiagnostic medicine.

The Management of Intraoperative Spinal Cord Injury - A Scoping Review.

Scoping Review. To review the literature and summarize information on checklists and algorithms for responding to intraoperative neuromonitoring (IONM) alerts and management of intraoperative spinal cord injuries (ISCIs). MEDLINE® was searched from inception through January 26, 2022 as were sources of grey literature. We attempted to obtain guidelines and/or consensus statements from the following sources: American Association of Neuromuscular & Electrodiagnostic Medicine (AANEM), American Academy of Neurology (AAN), American Clinical Neurophysiology Society, NASS (North American Spine Society), and other spine surgery organizations. Of 16 studies reporting on management strategies for ISCIs, two were publications of consensus meetings which were conducted according to the Delphi method and eight were retrospective cohort studies. The remaining six studies were narrative reviews that proposed intraoperative checklists and management strategies for IONM alerts. Of note, 56% of included studies focused only on patients undergoing spinal deformity surgery. Intraoperative considerations and measures taken in the event of an ISCI are divided and reported in three categories of i) Anesthesiologic, ii) Neurophysiological/Technical, and iii) Surgical management strategies. There is a paucity of literature on comparative effectiveness and harms of management strategies in response to an IONM alert and possible ISCI. There is a pressing need to develop a standardized checklist and care pathway to avoid and minimize the risk of postoperative neurologic sequelae.

Response to Letters to the Editor by Gregory J, Rice K, McCarthy K, et al. 2023 Regarding: "Guidelines for Qualifications of Neurodiagnostic Personnel: A Joint Position Statement of the American Clinical Neurophysiology Society, the American Association of Neuromuscular & Electrodiagnostic Medicine, the American Society of Neurophysiological Monitoring, and ASET-The Neurodiagnostic Society".

Response to Letters to the Editor by Gregory J, Rice K, McCarthy K, et al. 2023 Regarding: "Guidelines for Qualifications of Neurodiagnostic Personnel: A Joint Position Statement of the American Clinical Neurophysiology Society, the American Association of Neuromuscular & Electrodiagnostic Medicine, the American Society of Neurophysiological Monitoring, and ASET-The Neurodiagnostic Society".

Letter to the Editor Regarding: "Guidelines for Qualifications of Neurodiagnostic Personnel: A Joint Position Statement of the American Clinical Neurophysiology Society, the American Association of Neuromuscular & Electrodiagnostic Medicine, the American Society of Neurophysiological Monitoring, and ASET-The Neurodiagnostic Society".

Letter to the Editor Regarding: "Guidelines for Qualifications of Neurodiagnostic Personnel: A Joint Position Statement of the American Clinical Neurophysiology Society, the American Association of Neuromuscular & Electrodiagnostic Medicine, the American Society of Neurophysiological Monitoring, and ASET-The Neurodiagnostic Society".

Training factors that influence electrodiagnostic medicine knowledge.

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 of Neuromuscular and Electrodiagnostic Medicine SAE. This was a retrospective study of the 2023 AANEM-SAE results. In addition to the examination score, participants were asked approximately how many EDx studies they performed in training and how many months of training they had completed. Analysis included correlation of the examination scores with months of training as well as number of EDx studies. In addition, a multivariate linear regression model was developed. A total of 756 participants completed the proctored examination in May 2023. Examination score was moderately and positively correlated with the number of months of training (Pearson r = .5; p < .001) as well as the number of EDx studies during training (Pearson r = .55; p < .001). Scores steadily improved with additional months of training, but leveled off after 300-400 EDx studies. Regression analysis indicated that higher numbers of EDx studies were correlated with a higher examination score even after accounting for the number of months of study. We believe that a greater number of months of training is associated with better performance on the AANEM-SAE and that greatest improvement in examination performance occurs during the first 300-400 EDx studies.

The role of artificial intelligence in electrodiagnostic and neuromuscular medicine: Current state and future directions.

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. Different ML and DL models have been used to distinguish between electromyography signals in normal individuals and those with amyotrophic lateral sclerosis and myopathy, with accuracy ranging from 67% to 99.5%. DL models have also been successfully applied in neuromuscular ultrasound, with the use of segmentation techniques achieving diagnostic accuracy of at least 90% for nerve entrapment disorders, and 87% for inflammatory myopathies. Other successful AI applications include prediction of treatment response, and prognostication including prediction of intensive care unit admissions for patients with myasthenia gravis. Despite these remarkable strides, significant knowledge, attitude, and practice gaps persist, including within the field of electrodiagnostic and neuromuscular medicine. In this narrative review, we highlight the fundamental principles of AI and draw parallels with the intricacies of human brain networks. Specifically, we explore the immense potential that AI holds for applications in electrodiagnostic studies, neuromuscular ultrasound, and other aspects of neuromuscular medicine. While there are exciting possibilities for the future, it is essential to acknowledge and understand the limitations of AI and take proactive steps to mitigate these challenges. This collective endeavor holds immense potential for the advancement of healthcare through the strategic and responsible integration of AI technologies.

Neuromuscular implants: Interfacing with skeletal muscle for improved clinical translation of prosthetic limbs.

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 improve the clinical translation of prosthetic technologies. Skeletal muscle provides a physiologic gateway to harness and amplify signals from the nervous system. Recent surgical advancements in muscle reinnervation surgeries leverage the "bio-amplification" capabilities of muscle, enabling more intuitive control over a greater number of degrees of freedom in prosthetic limbs than previously achieved. We anticipate that state-of-the-art implantable neuromuscular interfaces that integrate well with skeletal muscle and novel surgical interventions will provide a long-term solution for controlling advanced prostheses. Flexible electrodes are expected to play a crucial role in reducing foreign body responses and improving the longevity of the interface. Additionally, innovations in device miniaturization and ongoing exploration of shape memory polymers could simplify surgical procedures for implanting such interfaces. Once implanted, wireless strategies for powering and transferring data from the interface can eliminate bulky external wires, reduce infection risk, and enhance day-to-day usability. By outlining the current limitations of neuromuscular interfaces along with potential future directions, this review aims to guide continued research efforts and future collaborations between engineers and specialists in the field of neuromuscular and musculoskeletal medicine.

Response to a Letter to the Editor by Gregory et al. Regarding: “Guidelines for Qualifications of Neurodiagnostic Personnel: A Joint Position Statement of the American Clinical Neurophysiology Society, the American Association of Neuromuscular & Electrodiagnostic Medicine, the American Society of Neurophysiological Monitoring, and ASET – The Neurodiagnostic Society”

Response to a Letter to the Editor by Gregory et al. Regarding: “Guidelines for Qualifications of Neurodiagnostic Personnel: A Joint Position Statement of the American Clinical Neurophysiology Society, the American Association of Neuromuscular & Electrodiagnostic Medicine, the American Society of Neurophysiological Monitoring, and ASET – The Neurodiagnostic Society”

The Neuromuscular Portal and Match: Working Toward a More Fair, Equitable, and Transparent Process.

Prior to 2021, the neuromuscular medicine fellowship application process suffered from non-standardized timelines and substantial variability. To rectify this, the American Association of Neuromuscular & Electrodiagnostic Medicine (AANEM) established a standardized application timeline and an online application portal in 2020-2021, followed by the introduction of a partial match process. In 2021-2022, AANEM launched a traditional, binding, two-way match system for fellowship positions allocation based on the Gale-Shapley stable matching algorithm. Surveys assessing perceptions of fairness in the application portal and match process were dispatched to applicants and program directors following the 2021 and 2022 recruitment cycles. In the 2020-21 cycle, 90% of program directors and 95% of applicants affirmed the standardized timeline benefited applicants. However, 57% of applicants deemed the process as unfair. All programs and most applicants (58%) favored a transition to a two-way match. The implementation of the two-way match in 2021-22 attracted participation from 97% of programs, with 80% of applicants and 95% of programs viewing the process as fair to applicants. A significant majority of both applicants (86%) and programs (94%) supported maintaining the standardized timeline and two-way match. We advocate for the universal adoption of the AANEM Match for neuromuscular fellowship recruitment and a standardized fellowship application timeline across all neurologic specialties to promote transparency, fairness, and equity for applicants.

The incidence of ulnar nerve subluxation in patients with ulnar neuropathy at the elbow and healthy control: A clinical, electrodiagnostic, and ultrasonographic study

Abstract Background: Ulnar neuropathy at the elbow is a common presenting disorder in neuromuscular medicine. Patients suffer from paresthesia in the 4/5 finger, pain at the elbow, and weakness and atrophy in advanced cases. Objective: This study aimed to find the incidence of ulnar nerve subluxation in patients with ulnar neuropathy at the elbow. Materials and Methods: This is a case-control study conducted in Al-Sadiq teaching hospital at the period of January 2018–August 2022. It included 84 patients diagnosed with ulnar neuropathy at the elbow by history, clinical examination and electrodiagnostic testing. All patients and control undergo ultrasonographic examination by high-resolution ultrasound looking for ulnar nerve cross-sectional area at 3 points: (1) 7 cm proximal to medial epicondyle, (2) at the medial epicondyle, and (3) at the cubital tunnel 2-3 cm distal to medial epicondyle. Also, dynamic ultrasonographic examination were done looking for subluxation of ulnar nerve behind medial epicondyle during elbow flexion. If ulnar nerve move on the top of medial epicondyle it is considered partial and when it dislocates anteriorly beyond the tip of medial epicondyle it is considered complete subluxation.

Guidelines for Qualifications of Neurodiagnostic Personnel: A Joint Position Statement of the American Clinical Neurophysiology Society, the American Association of Neuromuscular & Electrodiagnostic Medicine, the American Society of Neurophysiological Monitoring, and ASET The Neurodiagnostic Society.

The Guidelines for Qualifications of Neurodiagnostic Personnel (QNP) document has been created through the collaboration of the American Clinical Neurophysiology Society (ACNS), the American Society of Neurophysiological Monitoring (ASNM), the American Association of Neuromuscular & Electrodiagnostic Medicine (AANEM), and ASET The Neurodiagnostic Society (ASET). The quality of patient care is optimized when neurophysiological procedures are performed and interpreted by appropriately trained and qualified practitioners at every level. These societies recognize that neurodiagnostics is a large field with practitioners who have entered the field through a variety of training paths. This document suggests job titles, associated job responsibilities, and the recommended levels of education, certification, experience, and ongoing education appropriate for each job. This is important because of the growth and development of standardized training programs, board certifications, and continuing education in recent years. This document matches training, education, and credentials to the various tasks required for performing and interpreting neurodiagnostic procedures. This document does not intend to restrict the practice of those already working in neurodiagnostics. It represents recommendations of these societies with the understanding that federal, state, and local regulations, as well as individual hospital bylaws, supersede these recommendations. Because neurodiagnostics is a growing and dynamic field, the authors fully intend this document to change over time.

Needs of persons living with ALS at home and their family caregivers: A scoping review.

Most persons with amyotrophic lateral sclerosis (ALS) live at home with support of family caregivers, with escalating complexity of care over the trajectory of the disease requiring resources and support to mitigate negative physical, social, and emotional outcomes. This scoping review identifies the home health/home care needs of persons with ALS and their caregivers as a basis for creating a home health medical standard. We used the PRISMA Extension for Scoping Reviews (PRISMA-ScR) to examine studies describing home care needs published between 2011 and 2021. Our search yielded 481 articles, of which 44 were included with a total of 3592 (9-273) participants. Most studies used a cross-sectional design and 20 (45%) were rated as high quality. We grouped the needs identified as emotional/psychological, assistive devices and technology, information and education, and human resources and professional services. Most studies demonstrated persistent unmet needs and that available interventions were helpful while needs generally were not met proactively, despite the predictable trajectory. This review describes biopsychosocial and equipment interventions over the trajectory of ALS with implications for anticipatory planning by clinicians, as well as policy for coverage of necessary services and supports. Interdisciplinary expert teams could develop consensus around needs across the trajectory and recommended services and supports. To make knowledge more accessible, encourage availability of services, and clarify the need for coverage of services, we aim to develop an expert consensus-based ALS home health medical standard guidance document in collaboration with the American Association of Neuromuscular and Electrodiagnostic Medicine.

A Phase 3, Open-Label, Multicenter Study To Evaluate Eculizumab In Adolescents With Refractory Generalized Myasthenia Gravis (S5.009)

A Phase 3, Open-Label, Multicenter Study To Evaluate Eculizumab In Adolescents With Refractory Generalized Myasthenia Gravis (S5.009)

Subgroup outcomes from RAISE: A randomized, Phase 3 trial of zilucoplan in generalized myasthenia gravis (P1-5.006)

Subgroup outcomes from RAISE: A randomized, Phase 3 trial of zilucoplan in generalized myasthenia gravis (P1-5.006)

Overview of the Safety Profile from Efgartigimod Clinical Trials in Participants with Diverse IgG-Mediated Autoimmune Diseases (P1-5.001)

Overview of the Safety Profile from Efgartigimod Clinical Trials in Participants with Diverse IgG-Mediated Autoimmune Diseases (P1-5.001)

Myasthenia Gravis Activities of Daily Living (MG-ADL) Response to Eculizumab Treatment in Patients from the Generalized Myasthenia Gravis Registry (P1-5.020)

Myasthenia Gravis Activities of Daily Living (MG-ADL) Response to Eculizumab Treatment in Patients from the Generalized Myasthenia Gravis Registry (P1-5.020)