INTRODUCTION/AIMS:Pacinian corpuscles are end organs of the sensory nervous system. The size and superficial location of Pacinian corpuscles allows them to be visualized with high-resolution ultrasound. This pilot study sought to assess the reliability of Pacinian corpuscle counting in the hand using ultrasound. METHODS:Two healthy participants underwent ultrasound evaluation by three ultrasonographers using a scanning protocol developed for this study. The ultrasound protocol used anatomic landmarks to identify and trace digital nerves in the palms and the adjacent Pacinian corpuscles. The ultrasonographers used morphologic features to identify and count Pacinian corpuscles at two sites in each hand for each participant using a 10-22 MHz linear-array transducer. The procedure was repeated at 2 time points. Inter-rater and intrarater reliabilities were determined using intraclass correlation coefficients (ICCs). RESULTS:Pacinian corpuscles were identified in the second-third and fourth-fifth intermetacarpal spaces. The ultrasound appearance of Pacinian corpuscles is of a hypoechoic, rounded structure without a hyperechoic rim. A clustered appearance and septate internal structure are distinct features of Pacinian corpuscles, but these features are not always present. The mean number at each site was between 10.71 and 11.71. Inter-rater and intrarater reliability both resulted in ICC values over 0.8, indicating "good" inter-rater and intrarater reliabilities. DISCUSSION:Pacinian corpuscles can be reliably counted in the hand using high-resolution ultrasound. The reliability data from this pilot study may facilitate further ultrasound studies of Pacinian corpuscles, which may be decreased in number or undergo other changes in polyneuropathies.

Consensus was sought from 17 nominated global expert leaders to provide guidance on the combined use of neuromuscular ultrasound (NMUS) and electrodiagnostic tests (EDX) in the investigation of peripheral neuropathy (PN) in clinical practice. Consensus was based on an initial systematic review of the literature by the experts themselves, followed by three anonymised surveys, using the Delphi method. No consensus was defined as < 60 % rating frequency. The panel agreed that NMUS should be used if EDX cannot differentiate an axonal from a demyelinating process or an acquired from a hereditary process, and in the investigation of vasculitic neuropathy; NMUS of the brachial plexus should be used in Guillain-Barre' syndrome if EDX is negative; when NMUS is used, at least two nerves of the upper limbs should be scanned; NMUS should be used for screening or repeat testing for carpal tunnel syndrome in children with mucopolysaccharidoses. Areas of disagreement exposed gaps in current knowledge and informed the direction of future research, which should aim to identify situations in which NMUS can stand alone as a diagnostic test, particularly for screening or repeat testing and especially in vulnerable individuals and paediatric populations.

See article on pages 148–156 in this issue .

The classification of peripheral neuropathies has traditionally been based on etiology, electrodiagnostic findings, or histopathologic features. With the advent of modern imaging, they now can also be characterized based on their varied distribution of imaging findings. We describe the major morphologic patterns of these changes, which include homogeneous enlargement; homogeneous thinning; focal, multifocal, and segmental enlargement; and focal thinning and beading (multifocal thinning). Representative disorders in each of these categories are discussed, along with examples of the more complex imaging manifestations of neuralgic amyotrophy, nerve transection, and hereditary amyloidosis. An appreciation of the diverse morphologic manifestations of neuropathy can help neuromuscular clinicians conduct appropriate imaging studies with ultrasound and, when needed, order suitable investigations with magnetic resonance neurography.

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.

ContextHuntington'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.ObjectivesTo comprehensively define spiritual suffering, grief/loss, and coping strategies used by HD caregivers.MethodsA 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.Results36 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.ConclusionHD 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.

Background: The American Board of Plastic Surgery has been collecting practice data on carpal tunnel syndrome treatment since 2004 as part of its Continuous Certification Program. These data allow plastic surgeons to compare their surgical experience to national trends and analyze those trends in relation to current evidence-based medicine. Methods: Data on carpal tunnel syndrome treatment from 2004 to 2014 were compared to those from 2015 to 2020. National practice trends observed in these data were evaluated relative to current literature regarding evidence-based practices. Results: A total of 11,090 carpal tunnel syndrome cases were included from 2004 to 2020. Electrodiagnostic and imaging studies were performed on most patients despite adding little sensitivity and specificity when physical examination tests are performed and not being considered cost-effective. An open “mini” approach has remained the most common surgical technique in carpal tunnel release for the last 15 years, with growing usage (53 percent versus 59 percent, p < 0.001). Splinting has decreased significantly over the last 15 years, from usage in 39 percent of patients to 28 percent ( p < 0.001). Formal postoperative hand therapy has declined from 27 percent of patients to 22 percent ( p < 0.001). Despite their low efficacy, 63 percent of patients received one or more perioperative doses of antibiotics. Conclusions: Analysis of the Continuous Certification Program tracer data from the American Board of Plastic Surgery provides an excellent overview of current practice and its development over the 15 years since its inception. This analysis provides insight into how effectively plastic surgeons have remained aligned with developments in best practices in treating carpal tunnel syndrome.

Introduction/AimsWe 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. MethodsEach 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. ResultsA 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%). DiscussionThis 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.

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. (C) 2022 International Federation of Clinical Neurophysiology. Published by Elsevier B.V. All rights reserved.

ABSTRACT 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

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.

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.

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.

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.

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.

BACKGROUND:The huntingtin gene (HTT) pathogenic cytosine-adenine-guanine (CAG) repeat expansion responsible for Huntington disease (HD) is phased with single nucleotide polymorphisms (SNPs), providing targets for allele-selective treatments.OBJECTIVE:This prospective observational study defined the frequency at which rs362307 (SNP1) or rs362331 (SNP2) was found on the same allele with pathogenic CAG expansions.METHODS:Across 7 US sites, 202 individuals with HD provided blood samples that were processed centrally to determine the number and size of CAG repeats, presence and heterozygosity of SNPs, and whether SNPs were present on the mutant HTT allele using long-read sequencing and phasing.RESULTS:Heterozygosity of SNP1 and/or SNP2 was identified in 146 (72%) individuals. The 2 polymorphisms were associated only with the mHTT allele in 61% (95% high density interval: 55%, 67%) of individuals.CONCLUSIONS:These results are consistent with previous reports and demonstrate the feasibility of genotyping, phasing, and targeting of HTT SNPs for personalized treatment of HD.