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.

Short-term educational value of online neuromuscular ultrasound courses.

INTRODUCTION/AIMS: We have previously reported that online neuromuscular ultrasound courses are feasible and were found to be useful by most survey respondents. However, our previous report lacked objective assessment of the educational value of the courses. Therefore, we aimed in this study to evaluate the learning outcomes of online neuromuscular ultrasound courses. METHODS: Each of the basic and advanced courses featured one pre- and two post-course online knowledge tests. The percentage of corrected answers and the participants' scores in the three tests were calculated and compared. RESULTS: A total of 153 out of 277 course participants answered the course test. The mean percentage of correct answers were significantly higher in the second and first post-course tests compared to the pre-course test (Basic course test: 80.2 ± 14.8%, 75.5 ± 15.9%, 64.3 ± 19.1%, respectively; Advanced course test: 80.9 ± 20.1, 78.9 ± 15.2%, 69.5 ± 20.2%, respectively). The mean scores of the participants in the basic course test significantly improved in the first and second post-course tests (from 66.6% to 77.5% and from 67.2% to 80.2%, respectively) whereas those of the participants in the advanced course test significantly improved in the first post-course test only (from 76.3% to 85.4%). DISCUSSION: This report demonstrates the capability of online neuromuscular ultrasound courses, particularly the basic-level courses, to enhance knowledge. This information can further help integrate virtual neuromuscular ultrasound teaching as a standard complementary educational format together with supervised in-person or remote hands-on training.

Hemodynamic Changes Associated With Transcervical Laryngeal Injection of Botulinum Toxin.

PURPOSE: Laryngeal dystonia is a chronic neurologic disorder characterized by intention-induced spasms of the vocal folds driven by aberrant central motor processing. The use of in-office transcervical botulinum toxin injection for the treatment of laryngeal disorders, such as laryngeal dystonia, has been deemed safe and efficacious. There is, however, no available data outlining the hemodynamic changes experienced by patients undergoing this frequently performed procedure. METHODS: One hundred and one patients diagnosed with laryngeal dystonia were enrolled in this prospective study. These patients underwent transcervical laryngeal botulinum toxin injection to address their dysphonia. Vital signs where acquired prior to, and at the time of injection. Alterations in these parameters were then evaluated for statistical significance. RESULTS: Statistically significant increases in mean heart rate (5.8 ± 10.8 bpm, P < 0.0001), systolic blood pressure and diastolic blood pressure (7.0 ± 9.5 mm Hg, P < 0.0001; 8.7 ± 14.7 mm Hg, P < 0.0001) were discovered. No statistically significant difference in oxygen saturation was noted and no patients in the study faced major adverse outcomes. CONCLUSIONS: Though these findings may not have related to clinically significant complication, our study demonstrates the importance of understanding potential stressors in a procedure routinely performed by laryngologists. This may result in more careful patient selection, alterations in procedure, and improved safety by acting in a timely fashion if alarming changes in hemodynamic parameters are noted.

Layers of Loss: A Scoping Review and Taxonomy of HD Caregivers' Spiritual Suffering, Grief/Loss and Coping Strategies.

CONTEXT: Huntington's disease (HD), an incurable, multi-generational, autosomal dominant disorder, creating unique challenges and a myriad of spiritually-related stressors in those affected and their familial caregivers. Spiritual suffering, experiences of grief/loss, and coping strategies have not been systematically studied in HD caregivers. OBJECTIVES: To comprehensively define spiritual suffering, grief/loss, and coping strategies used by HD caregivers. METHODS: A PRISMA-ScR scoping literature review was conducted. Data from included research articles were organized thematically using induction and open coding. A grounded, deductive approach was used to delineate a demarcated taxonomy of themes, which encompasses all three over-arching domains. Four reviewers, employing a modified Delphi approach, ascertained which themes were demonstrated by research participants in each study. RESULTS: 36 of 583 articles met the review criteria; none were published in the palliative care literature. Investigations primarily focused on intrapersonal (self-image) distress and existential angst; only rarely looking deeper into divine/transpersonal suffering, disrupted religious relationships, or meaning distress. HD caregivers experience profound grief/loss, expressed as disenfranchised grief that is associated with the ambiguous loss of their loved one, loss of family structure, social connectedness, and personal losses. Half of the studies reported maladaptive HD caregiver coping strategies-characterized by dysfunctional escape schemes; in contrast, transcendent/creative strategies were often unexplored. CONCLUSION: HD caregivers experience prolonged grief and other forms of spiritual suffering as they progressively lose their loved ones and disruption to their own lives. With an improved assessment tool, teams with spiritual and palliative care experts will better be able to support HD family caregivers.

Expert consensus on the combined investigation of carpal tunnel syndrome with electrodiagnostic tests and neuromuscular ultrasound.

Expert consensus was sought to guide clinicians on the use of electrodiagnostic tests (EDX) and neuromuscular ultrasound (NMUS) in the investigation of suspected carpal tunnel syndrome (CTS). Consensus was achieved using the Delphi method via three consecutive anonymised surveys of 15 experts and was defined as rating agreement ≥ 80%. The panel agreed that combining EDX and NMUS is more informative than using each modality alone. NMUS adds value in patients with clinically suspected CTS with non-localizing or normal EDX, atypical EDX, failed CTS surgery, polyneuropathy, and CTS suspected to be secondary to structural pathology. The median nerve cross-sectional area should be measured at the site of maximal nerve enlargement, and the nerve should be scanned from mid-forearm to the palm. The group also identified those situations where the wrist-to-forearm area ratio and longitudinal scans of the median nerve should also be obtained. EDX should always be performed to quantify CTS severity and in individuals over age 70. This document is an initial step to guide clinicians on the combined investigation of CTS using EDX and NMUS, to be updated regularly with the emergence of new research.

Virtual neuromuscular ultrasound courses during COVID-19 pandemic: Leveraging technology to enhance learning opportunities.

INTRODUCTION/AIMS: Hands-on supervised training is essential for learning diagnostic ultrasound. Unfortunately, the coronavirus disease 2019 (COVID-19) pandemic led to suspension of in-person training courses. As a result, many hands-on training courses were converted into virtual courses during the pandemic. Several reports regarding virtual ultrasound courses exist, but none has addressed virtual neuromuscular ultrasound courses, their design, or participants' views of this form of training. Therefore, the aims of this study were: (1) to determine the feasibility of conducting virtual neuromuscular ultrasound courses during the COVID-19 pandemic; and (2) to report the positive and negative aspects of the courses through the analyses of the responses of post-course surveys. METHODS: Two virtual neuromuscular ultrasound courses, basic and intermediate level, were conducted by the Egyptian Neuromuscular Ultrasound society during August 2020. Post-course, the attendees were directed to an electronic survey that consisted of eight questions. Ninety-three responses (23.8%) were obtained from the survey of the basic course and 156 responses (44.4%) were obtained from the survey of the intermediate course. RESULTS: Ninety-eight percent of the respondents to basic course surveys, and 100% of the respondents to the intermediate course survey found the courses useful or very useful. DISCUSSION: This report demonstrates the utility of virtual neuromuscular ultrasound courses for those participants willing to respond to a survey and describes a proposed design for such courses. Although hands-on supervised ultrasound training is ideal, virtual courses can be useful alternatives to in-person training when in-person interaction is restricted.

An MDS Evidence-Based Review on Treatments for Huntington's Disease.

BACKGROUND: Huntington's disease (HD) is a rare neurodegenerative disorder with protean clinical manifestations. Its management is challenging, consisting mainly of off-label treatments. OBJECTIVES: The International Parkinson and Movement Disorder Society commissioned a task force to review and evaluate the evidence of available therapies for HD gene expansion carriers. METHODS: We followed the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach. Eligible randomized controlled trials were identified via an electronic search of the CENTRAL, MEDLINE, and EMBASE databases. All eligible trials that evaluated one or more of 33 predetermined clinical questions were included. Risk of bias was evaluated using the Cochrane Risk of Bias tool. A framework was adapted to allow for efficacy and safety conclusions to be drawn from the balance between the GRADE level of evidence and the importance of the benefit/harm of the intervention. RESULTS: Twenty-two eligible studies involving 17 interventions were included, providing data to address 8 clinical questions. These data supported a likely effect of deutetrabenazine on motor impairment, chorea, and dystonia and of tetrabenazine on chorea. The data did not support a disease-modifying effect for premanifest and manifest HD. There was no eligible evidence to support the use of specific treatments for depression, psychosis, irritability, apathy, or suicidality. Similarly, no evidence was eligible to support the use of physiotherapy, occupational therapy, exercise, dietary, or surgical treatments. CONCLUSIONS: Data for therapeutic interventions in HD are limited and support only the use of VMAT2 inhibitors for specific motor symptoms. © 2021 International Parkinson and Movement Disorder Society.

Expert consensus on the combined investigation of ulnar neuropathy at the elbow using electrodiagnostic tests and nerve ultrasound.

The addition of ultrasound (US) to electrodiagnostic (EDX) tests can significantly enhance the accuracy of testing for ulnar neuropathy at the elbow (UNE). We aimed to obtain expert consensus to guide clinicians on the combined use of EDX and US in UNE investigation. Consensus was achieved using the Delphi method. Two consecutive anonymised questionnaires were submitted to 15 experts, who were asked to choose their level of agreement with each statement. Consensus was pre-defined as ≥ 80% rating agreement. The experts concluded that all investigations of UNE should include both nerve conduction studies and US. There was consensus that US should include cross-sectional area measurement and assessment of nerve mobility at the elbow, and that the entire ulnar nerve should be imaged. This study defined expert opinion on the 'core' techniques that should be used routinely in the UNE investigation using EDX and US. Areas with lack of consensus highlighted some controversial issues in the current use of these diagnostic modalities and the need for future research. This document is an initial step to guide clinicians on the combined investigation of UNE using EDX and US, to be regularly updated as new research emerges.

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.

Managing uncommon and unexpected findings during neuromuscular ultrasound.

One barrier to widespread adoption of neuromuscular ultrasound by clinical neurophysiologists is concern over how to identify and manage non-neuromuscular findings. This review addresses this concern by describing the sonographic appearance of a variety of commonly observed pathologies and anatomic variants in dermal, subcutaneous, bony, glandular, lymphatic, vascular, and other superficial tissues. Additionally, it outlines techniques to ensure proper clinical and ultrasound evaluation of unexpected or uncommon findings. Finally, it highlights strategies to manage unexpected findings, including how to best communicate findings to patients and referring clinicians to avoid unnecessary testing and ensure appropriate follow-up. Ultrasound extends the ability of the neuromuscular sonographer-clinician to contribute to patient care.

Neuromuscular ultrasound for taxane peripheral neuropathy in breast cancer.

BACKGROUND: Our study aim was to evaluate neuromuscular ultrasound (NMUS) for the assessment of taxane chemotherapy-induced peripheral neuropathy (CIPN), the dose-limiting toxicity of this agent. METHODS: This cross-sectional study of breast cancer patients with taxane CIPN measured nerve cross-sectional area (CSA) by NMUS and compared with healthy historical controls. Correlations were determined between CSA and symptom scale, nerve conduction studies, and intraepidermal nerve fiber density (IENFD). RESULTS: A total of 20 participants reported moderate CIPN symptoms at a median of 3.8 months following the last taxane dose. Sural nerve CSA was 1.2 mm2 smaller than healthy controls (P ≤ .01). Older age and time since taxane were associated with smaller sural nerve CSA. For each 1 mm2 decrease in sural nerve CSA, distal IENFD decreased by 2.1 nerve/mm (R2 0.30; P = .04). CONCLUSIONS: These data support a sensory predominant taxane neuropathy or neuronopathy and warrant future research on longitudinal NMUS assessment of CIPN.

Ulnar Nerve Enlargement at the Medial Epicondyle Negatively Correlates With Nerve Conduction Velocity in Cubital Tunnel Syndrome.

Background: In cubital tunnel syndrome (CuTS), chronic compression often occurs at the origin of the flexor carpi ulnaris at the medial epicondyle. Motor nerve conduction velocity (NCV) across the elbow is assessed preoperatively to corroborate the clinical impression of CuTS. The purpose of this study was to correlate preoperative NCV to the direct measurements of ulnar nerve size about the elbow at the time of surgery in patients with clinical and/or electrodiagnostic evidence of CuTS. Methods: Data from 51 consecutive patients who underwent cubital tunnel release over a 2-year period were reviewed. Intraoperative measurements of the decompressed nerve were taken at 3 locations: at 4 cm proximal to the medial epicondyle, at the medial epicondyle, and at the distal aspect of Osborne fascia at the flexor aponeurotic origin. Correlation analysis was performed comparing nerve size measurements to slowing of ulnar motor nerve conduction velocities (NCV) below the normal threshold of 49 m/s across the elbow. Results: Enlargement of the ulnar nerve at the medial epicondyle and nerve compression at the flexor aponeurotic origin was a consistent finding. The mean calculated cross-sectional area of the ulnar nerve was 0.21 cm2 above the medial epicondyle, 0.30 cm2 at the medial epicondyle, and 0.20 cm2 at the flexor aponeurotic origin (P < .001). There was an inverse correlation between change in nerve diameter and NCV slowing (r = -0.529, P < .001). Conclusions: For patients with significantly reduced preoperative NCV and clinical findings of advanced ulnar neuropathy, surgeons can expect nerve enlargement, all of which may affect their surgical decision-making.

Clinical and Ultrasonographic Features of Distal Ulnar Neuropathy: A Review.

Focal ulnar neuropathy at the wrist is a rare but problematic disorder often associated with the unique anatomy of this nerve as it courses through Guyon's canal, a superficial fibro-osseous tunnel in the proximal ulnar palm. The electrophysiologic features of this disorder have been well-characterized, but the sonographic anatomy of the nerve across the wrist and palm has yet to be systematically described in normal and abnormal states. In this review, we describe the basic anatomy and the sonographic appearance of the nerve in the wrist and palm in normals and individuals with pathology. The value of using US in conjunction with electrodiagnostic testing is emphasized as the two tests together provide critical information regarding etiology, predisposing factors, and functional significance. Furthermore, ultrasound is useful as a patient educational tool to promote behavioral changes that assist in nerve recovery when pathology is related to repetitive stress.

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.

Trochlear nerve palsy associated with Moyamoya disease.

A 48-year-old woman with intracranial hemorrhage at the right hemisphere and the right midbrain was diagnosed as Moyamoya disease (MMD). While restoring consciousness, she complained of diplopia. The left hypertropia with a compensatory right head tilt was noted. The follow up brain image showed the evidence of past hemorrhage at the level of the right inferior colliculus. In this case, the hemorrhage directly damaged the right trochlear nucleus or intra-axial trochlear nerve before decussation and caused left (contralateral) hypertropia. To our knowledge, no report has been described of the trochlear nerve palsy in hemorrhagic Moyamoya disease. Here, we present a case of the patient with trochlear nerve palsy after hemorrhagic MMD and summarize the characteristics of trochlear nerve palsy according to its lesion sites.

Cost-effectiveness of neuromuscular ultrasound in focal neuropathies.

OBJECTIVE: To evaluate the cost-effectiveness of neuromuscular ultrasound (NMUS) for the evaluation of focal neuropathies. METHODS: A prior prospective, randomized, double-blind controlled trial demonstrated that NMUS, when added to electrodiagnostic testing, resulted in improved clinical outcomes after 6 months of follow-up. From this study, we abstracted quality-adjusted life-years (QALYs) from the 36-item Short Form Health Survey and entered this health-utility estimate into a mixed trial and model-based cost-effectiveness analysis from the societal perspective. Costs of intervention (NMUS) were estimated from Medicare payment rates for Current Procedural Terminology codes. Health care use was otherwise estimated to be equal, but sensitivity analyses further examined this and other key assumptions. Incremental cost-effectiveness ratio (ICER) was used as the primary outcome with a willingness-to-pay threshold of $50,000 per QALY. RESULTS: The predicted mean health outcome associated with use of NMUS was 0.079 QALY, and the mean cost was $37, resulting in an ICER of $463 per QALY. Results and conclusions remained robust across all sensitivity analyses, including variations in time horizon, initial distribution of health states, costs, and effectiveness. CONCLUSIONS: From a societal perspective, the addition of NMUS to electrodiagnostic testing when evaluating a focal neuropathy is cost-effective. A study of longer follow-up incorporating total health care use would further quantify the value of NMUS. CLINICALTRIALSGOV IDENTIFIER: NCT01394822.