Neuromuscular ultrasound standardized scanning techniques and protocols: Expert panel recommendations.

Neuromuscular ultrasound has become an integral part of the diagnostic workup of neuromuscular disorders at many centers. Despite its growing utility, uniform standard scanning techniques do not currently exist. Scanning approaches for similar diseases vary in the literature creating heterogeneity in the studies as reported in several meta-analysis. Moreover, neuromuscular ultrasound experts including the group in this study have different views with regards to technical aspects, scanning protocols, and the parameters that should be assessed. Establishing standardized neuromuscular scanning protocols is essential for the development of the subspeciality to ensure uniform clinical and research practices. Therefore, we aimed to recommend consensus-based standardized scanning techniques and protocols for common neuromuscular disorders using the Delphi approach. A panel of 17 experts participated in the study, which consisted of three consecutive electronic surveys. The first survey included voting on six scanning protocols addressing the general scanning technique and five common categories of suspected neuromuscular disorders. The subsequent surveys focused on refining the protocols and voting on new steps, rephrased statements, or areas of non-agreement. A high degree of consensus was achieved on the general neuromuscular ultrasound scanning technique and the scanning protocols for focal mononeuropathies, brachial plexopathies, polyneuropathies, amyotophic lateral sclerosis, and muscle diseases. In this study, a group of neuromuscular ultrasound experts developed six consensus-based neuromuscular ultrasound scanning protocols that may serve as references for clinicians and researchers. The standardized protocols could also aid in achieving high-quality uniform neuromuscular ultrasound practices.

The neuromuscular fellowship portal and match.

For many years, Neuromuscular Medicine programs lacked a standardized means of handling fellowship applications and offering positions. Programs interviewed applicants and made offers as early as the first half of Post Graduate Year 3 (PGY3), a suboptimal timeline for applicants who may have had little prior exposure to neuromuscular or electrodiagnostic medicine. In 2021, the American Association of Neuromuscular & Electrodiagnostic Medicine (AANEM) developed the Neuromuscular Fellowship Portal to standardize a later timeline and establish a process for fellowship applications and offers. In its first year, the Neuromuscular Fellowship Portal used a unique one-way match, in which the portal released serial offers to applicants based on rank order lists submitted by programs. Fifty-two Neuromuscular Medicine programs and seven electromyography (EMG)-focused Clinical Neurophysiology programs participated. Sixty-eight positions were filled, a similar number to previous years. A survey of fellowship directors and applicants following this process showed overwhelming support for the standardized timeline and application portal, but all program directors and most applicants favored moving to a traditional match. To maintain the existing application timeline and minimize costs for all parties, the AANEM Neuromuscular Fellowship Portal will host a two-way match, based on existing commercial match algorithms, in 2022. A match will afford a fair and efficient process for all involved. Both Neuromuscular Medicine and EMG-focused Clinical Neurophysiology programs will be encouraged to participate. The process undertaken by the AANEM can stand as an example for other neurologic subspecialties who are interested in standardizing their application timeline.

Research reporting in cubital tunnel syndrome studies: an analysis of the literature.

PURPOSE: There is a strong need for a set of consensus outcomes to be utilized for future studies on cubital tunnel syndrome. The goal was to assess the outcome measures utilized in the cubital tunnel syndrome literature as a way of measuring popularity/acceptability and then to perform a literature review for the most commonly used outcomes. METHODS: A literature search was performed using the pubmed.gov database and Medical Subject Headings (MeSH). For each article, the following data were abstracted: study type, motor outcome(s), sensory outcome(s), composite outcome(s), patient-reported outcome (PRO) metric(s), pain outcome(s), psychological outcome(s), electrodiagnostic outcome(s), and any other outcomes that were used. RESULTS: A composite outcome was reported in 52/85 (61%) studies, with the modified Bishop score (27/85; 32%) most common. A motor outcome was reported in 44/85 (52%) studies, with dynamometry (38/85; 45%) most common. The majority of studies (55%) did not report a sensory outcome. The majority of studies (52%) did not report a PRO. A specific pain outcome was reported in the minority (23/85; 27%), with the visual analogue scale (VAS) (22/85; 26%) most common. Pre- and postoperative electrodiagnostic results were presented in 22/85 studies (26%). DISCUSSION: Understanding current clinical practice and historical outcomes reporting provides a foundation for discussion regarding the development of a core outcome set for cubital tunnel syndrome. We hope that the data provided in the current study will stoke a discussion that will culminate in a consensus statement for research reporting in cubital tunnel syndrome studies.

Neuromuscular ultrasound competency assessment: Consensus-based survey.

Neuromuscular ultrasound is a rapidly evolving specialty with direct application for patient care. Competency assessment is an essential standard needed to ensure quality for practitioners, particularly for those newly acquiring skills with the technique. Our aim was to survey experts' opinions regarding physician competency assessment of neuromuscular ultrasound and to identify minimal competency of knowledge and skills. The opinions of 18 experts were obtained through the Delphi method using two consecutive electronic surveys. A high degree of consensus was achieved on items regarding framework and the conduct of neuromuscular ultrasound assessment and the knowledge and skills that a candidate needs to attain minimal competency in neuromuscular ultrasound. In this study, a group of neuromuscular ultrasound experts developed a general framework for neuromuscular ultrasound competency assessment and recommended testable areas of knowledge and skills suitable for establishing minimal competency.

Assessment of variability in motor grading and patient-reported outcome reporting: a multi-specialty, multi-national survey.

BACKGROUND: The goal of this survey-based study was to evaluate the current practice patterns of clinicians who assess patients with peripheral nerve pathologies and to assess variance in motor grading on the Medical Research Council (MRC) scale using example case vignettes. METHODS: An electronic survey was distributed to clinicians who regularly assess patients with peripheral nerve pathology. Survey sections included (1) demographic data, (2) vignettes where respondents were asked to assess on the MRC scale, and (3) assessment of practice patterns regarding the use of patient-reported outcome measures. Inter-rater reliability statistics were calculated for the application of the MRC scale on example vignettes. RESULTS: There were 109 respondents. There was significant dispersion in motor grading seen on the example vignettes. For the raw responses grading the example vignettes on the MRC scale, Krippendorff's alpha was 0.788 (95% CI 0.604, 0.991); Gwet's AC2 was 0.808 (95% CI 0.683, 0.932); Fleiss' kappa was 0.416 (95% CI 0.413, 0.419). Most respondents reported not utilizing any patient-reported outcome measures across peripheral nerve pathologies. DISCUSSION: Our data show that there is significant disagreement among providers when applying the MRC scale. It is important for us to reassess our current tools for patient evaluation in order to improve upon both clinical evaluation and outcomes reporting. Consensus guidelines for outcomes reporting are needed, and domains outside of manual muscle testing should be included.

Guidelines for neuromuscular ultrasound training.

Neuromuscular ultrasound has become an essential tool in the diagnostic evaluation of various neuromuscular disorders, and, as such, there is growing interest in neuromuscular ultrasound training. Effective training is critical in mastering this modality. Our aim was to develop consensus-based guidelines for neuromuscular ultrasound training courses. A total of 18 experts participated. Expert opinion was sought through the Delphi method using 4 consecutive electronic surveys. A high degree of consensus was achieved with regard to the general structure of neuromuscular ultrasound training; the categorization of training into basic, intermediate, and advanced levels; the learning objectives; and the curriculum for each level. In this study, a group of neuromuscular ultrasound experts established consensus-based guidelines for neuromuscular ultrasound training. These guidelines can be used in the development of the specialty and the standardization of neuromuscular ultrasound training courses and workshops.

Physician Perceptions of Performance Feedback and Impact on Personal Well-Being: A Qualitative Exploration of Patient Satisfaction Feedback in Neurology.

BACKGROUND: To understand neurologists' experiences and perspectives on patient satisfaction feedback and its impact on personal well-being and behavior. METHODS: From May to June 2021, the researchers conducted 19 semistructured interviews with neurologists from a large academic medical center. Clinical Performance Feedback Intervention Theory informed a combined inductive and deductive thematic analysis of the qualitative data, which focused on perceptions of current feedback practices, its impact on physician behavior, and recommendations for improvement. RESULTS: Participants tended to be female (n = 12/19, 63.2%), aged 30-39 (n = 8/19, 42.1%), white (n = 9/19, 47.4%), and were 10+ years into clinical practice (n = 18/19, 94.7%). Physicians were receptive to feedback overall, but perceptions varied by feedback type. Physicians preferred informal feedback (delivered unprompted directly by patients), given its tendency toward actionability. They disliked formal feedback (derived from anonymous surveys) due to low actionability, bias and validity issues, lack of contextual considerations, delivery through public reports, and links to financial incentives. Nearly all physicians reported formal feedback programs had the potential to negatively affect well-being and were not beneficial to their practice; a few reported adjusting their clinical practice to improve patient satisfaction performance. Five recommendations to improve patient satisfaction feedback programs emerged: Align on feedback intent, acknowledge survey limitations during program administration, increase actionability of feedback through specificity and control, support direct patient-physician feedback and problem resolution, and support empathetic integration of feedback. CONCLUSION: Understanding physician perceptions of current approaches to patient satisfaction feedback offers the opportunity to shape subsequent collection and distribution methods to improve physician performance and optimize professional fulfillment.

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.

Allograft Nerve Repair Reduces Postoperative Neuropathic Pain Following Nerve Biopsy.

BACKGROUND: New or worsened neuropathic pain is common following nerve biopsy and significantly impacts quality of life. OBJECTIVE: To examine the impact of allograft nerve repair on the likelihood of postoperative worsened neuropathic pain following nerve biopsy. METHODS: A retrospective cohort study was performed comparing standard nerve biopsy to nerve biopsy with allograft repair. Consecutive patients (N = 51) who underwent whole nerve biopsy between August 1, 2017, and August 1, 2019, by a single surgeon were evaluated for inclusion. The primary outcome was significant worsening of visual analog scale (VAS) score in the nerve distribution 6-mo postbiopsy. Secondary outcomes included significant worsening of VAS in the nerve distribution 3-wk postbiopsy and significant change in Zung Self-Rating Depression Scale 6-mo postbiopsy. RESULTS: In a multivariate model, allograft nerve repair significantly reduced the likelihood of increased neuropathic pain at 6-mo postbiopsy (odds ratio 0.02, P = .03). Worsened neuropathic pain occurred in 28% of the standard nerve biopsy cohort compared to 4% of the allograft nerve repair cohort. In a multivariate model, an increase in neuropathic pain was strongly associated with an increased likelihood of self-reported depression (odds ratio 57.4, P = .01). CONCLUSION: Allograft nerve repair significantly reduces the likelihood of postbiopsy worsened neuropathic pain compared to standard techniques. Neuropathic pain significantly impacts quality of life after nerve biopsy, and this is the first technique to demonstrate a significant reduction in neuropathic pain while maintaining the ability to harvest an adequate nerve specimen.

Imaging of Damaged Nerves.

Nerve imaging is an important component in the assessment of patients presenting with suspected peripheral nerve pathology. Although magnetic resonance neurography and ultrasound are the most commonly utilized techniques, several promising new modalities are on the horizon. Nerve imaging is useful in localizing the nerve injury, determining the severity, providing prognostic information, helping establish the diagnosis, and helping guide surgical decision making. The focus of this article is imaging of damaged nerves, focusing on nerve injuries and entrapment neuropathies.

An update on the management of adult traumatic nerve injuries-replacing old paradigms: A review.

Acute nerve injuries are routinely encountered in multisystem trauma patients. Advances in surgical treatment of nerve injuries now mean that good outcomes can be achieved. Despite this, old mantras associated with management of nerve injuries, including "wait a year to see if recovery occurs" and "there's nothing we can do", persist. Practicing by these mantras places these patients at a disadvantage. Changes begin to occur in the nerve, neuromuscular junction, and muscle from the moment a nerve injury occurs. These changes can become irreversible approximately 18 to 24 months following denervation. Thus, it is a race to reestablish a functional nerve-muscle connection before these irreversible changes. Good outcomes rely on appropriate acute management and avoiding delays in care. Primary nerve surgery options include direct primary repair, nerve graft repair, and nerve transfer. Acute management of nerve injuries proceeds according to the rule of 3's and requires early cooperation between trauma surgeons who recognize the nerve injury and consultant nerve surgeons. Care of patients with acute traumatic nerve injuries should not be delayed. Awareness of current management paradigms among trauma surgeons will help facilitate optimal upfront management. With the ever-expanding surgical options for management of these injuries and the associated improvement of outcomes, early multidisciplinary approaches to these injuries have never been more important. Old mantras must be replaced with new paradigms to continue to see improvements in outcomes for these patients. The importance of this review is to raise awareness among trauma surgeons of new paradigms for management of traumatic nerve injuries.

Increased EEG Theta Spectral Power in Sleep in Myotonic Dystrophy Type 1.

STUDY OBJECTIVES: Myotonic dystrophy type 1 (DM1) is a multisystemic disorder that involves the central nervous system (CNS). Individuals with DM1 commonly present with sleep dysregulation, including excessive daytime sleepiness and sleep-disordered breathing. We aim to characterize electroencephalogram (EEG) power spectra from nocturnal polysomnography (PSG) in patients with DM1 compared to matched controls to better understand the potential CNS sleep dysfunction in DM1. METHODS: A retrospective, case-control (1:2) chart review of patients with DM1 (n = 18) and matched controls (n = 36) referred for clinical PSG at the Stanford Sleep Center was performed. Controls were matched based on age, sex, apnea-hypopnea index (AHI), body mass index (BMI), and Epworth Sleepiness Scale (ESS). Sleep stage and respiratory metrics for the two groups were compared. Power spectral analysis of the EEG C3-M2 signal was performed using the fast Fourier transformation. RESULTS: Patients with DM1 had significantly increased theta percent power in stage N2 sleep compared to matched controls. Theta/beta and theta/alpha percent power spectral ratios were found to be significantly increased in stage N2, N3, all sleep stages combined, and all wake periods combined in patients with DM1 compared to controls. A significantly lower nadir O2 saturation was also found in patients with DM1 versus controls. CONCLUSIONS: Compared to matched controls, patients with DM1 had increased EEG theta spectral power. Increased theta/beta and theta/alpha power spectral ratios in nocturnal PSG may reflect DM1 pathology in the CNS.

Phrenic nerve conduction studies as a biomarker of respiratory insufficiency in amyotrophic lateral sclerosis.

Our objective was to examine the value of phrenic nerve conduction studies (PNCS) in quantifying diaphragm dysfunction in ALS, as no ideal test of respiratory insufficiency exists in ALS. We prospectively recorded bilateral PNCS, forced vital capacity (FVC), maximum inspiratory pressure (MIP), sniff nasal inspiratory pressure (SNIP), respiratory rate, ALSFRS-R, and respiratory symptoms in 100 ALS patients attending our clinic over a nine-month period. Survival data were collected for two years. Results showed that PNCS were reproducible and well tolerated. When the Pamp was abnormal (<0.3 mV), the relative risk of a respiratory rate >18 was 7.2 (95% CI 2.2-37.2, p <0.01) compared with a Pamp ≥0.3 mV. Similarly, the relative risk of orthopnea was 3.5 (95% CI 1.6-8.7, p <0.01) and dyspnea 2.4 (95% CI 1.4-4.0, p <0.01). FVC had the strongest correlation with Pamp (R(2) = 0.48 (p <0.001)). Fourteen of 15 patients with a FVC <50% had a Pamp <0.3 mV. However, eight with a Pamp <0.3 had a FVC >80%. The median survival was 1.07 years when the Pamp was <0.3 mV and >2 years when the Pamp was >0.3 mV (p <0.001). In conclusion, the phrenic Pamp correlated closely with multiple symptoms, signs, and laboratory measures of respiratory insufficiency and may prove to be a useful biomarker of respiratory dysfunction in ALS.

Congenital muscular dystrophy and generalized epilepsy caused by GMPPB mutations.

The alpha-dystroglycanopathies are genetically heterogeneous muscular dystrophies that result from hypoglycosylation of alpha-dystroglycan (α-DG). Alpha-dystroglycan is an essential link between the extracellular matrix and the muscle fiber sarcolemma, and proper glycosylation is critical for its ability to bind to ligands in the extracellular matrix. We sought to identify the genetic basis of alpha-dystroglycanopathy in a family wherein the affected individuals presented with congenital muscular dystrophy, brain abnormalities and generalized epilepsy. We performed whole exome sequencing and identified compound heterozygous GMPPB mutations in the affected children. GMPPB is an enzyme in the glycosylation pathway, and GMPPB mutations were recently linked to eight cases of alpha-dystroglycanopathy with a range of symptoms. We identified a novel mutation in GMPPB (p.I219T) as well as a previously published mutation (p.R287Q). Thus, our work further confirms a role for GMPPB defects in alpha-dystroglycanopathy, and suggests that glycosylation may play a role in the neuronal membrane channels or networks involved in the physiology of generalized epilepsy syndromes. This article is part of a Special Issue entitled RNA Metabolism 2013.