Search

Neuromuscular ultrasound (NMUS) is a valuable tool in establishing a diagnosis of carpal tunnel syndrome (CTS), and can be particularly helpful in patients with clinical CTS but normal nerve conduction studies (NCS). This case involves the uncommon presentation of enlarged median nerves on NMUS with normal NCS in a breast cancer patient who developed chemotherapy induced peripheral neuropathy (CIPN) and CTS following taxane treatment. This case demonstrates that CTS should not be excluded based on electrodiagnostic studies alone, and comorbid CTS should be considered in patients receiving neurotoxic chemotherapy, even in the setting of normal NCS.

Contains fulltext : 244190.pdf (Publisher’s version ) (Closed access) 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.

Item does not contain fulltext 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. 01 januari 2023

Contains fulltext : 291244.pdf (Publisher’s version ) (Open Access) Neuralgic amyotrophy (NA), also referred to as idiopathic brachial plexitis and Parsonage-Turner syndrome, is a peripheral nerve disorder characterized by acute severe shoulder pain followed by progressive upper limb weakness and muscle atrophy. While NA is incompletely understood and often difficult to diagnose, early recognition may prevent unnecessary tests and interventions and, in some situations, allow for prompt treatment, which can potentially minimize adverse long-term sequalae. High-resolution ultrasound (HRUS) has become a valuable tool in the diagnosis and evaluation of NA. Pathologic HRUS findings can be grouped into four categories: nerve swelling, swelling with incomplete constriction, swelling with complete constriction, and fascicular entwinement, which may represent a continuum of pathologic processes. Certain ultrasound findings may help predict the likelihood of spontaneous recovery with conservative management versus the need for surgical intervention. We recommend relying heavily on history and physical examination to determine which nerves are clinically affected and should therefore be assessed by HRUS. The nerves most frequently affected by NA are the suprascapular, long thoracic, median and anterior interosseous nerve (AIN) branch, radial and posterior interosseous nerve (PIN) branch, axillary, spinal accessory, and musculocutaneous. When distal upper limb nerves are affected (AIN, PIN, superficial radial nerve), the lesion is almost always located in their respective fascicles within the parent nerve, proximal to its branching point. The purpose of this review is to describe a reproducible, standardized, ultrasonographic approach for evaluating suspected NA, and to share reliable techniques and clinical considerations when imaging commonly affected nerves. 01 januari 2023

The purpose of this literature review is to develop an evidence-based guideline for the use of neuromuscular ultrasound in the diagnosis of ulnar neuropathy at the elbow (UNE). The proposed research question was: "In patients with suspected UNE, does ulnar nerve enlargement as measured with ultrasound accurately identify those patients with UNE?"A systematic review and meta-analysis was performed, and studies were classified according to American Academy of Neurology criteria for rating articles for diagnostic accuracy.Based on Class I evidence in four studies, it is probable that neuromuscular ultrasound measurement of the ulnar nerve at the elbow, either of diameter or cross-sectional area (CSA), is accurate for the diagnosis of UNE.For patients with symptoms and signs suggestive of ulnar neuropathy, clinicians should offer ultrasonographic measurement of ulnar nerve cross-sectional area or diameter to confirm the diagnosis and localize the site of compression (Level B).

Nerve ultrasound is useful in the diagnosis and follow-up of peripheral nerve disorders in children. The aim of this study was to explore and analyze the current literature on nerve cross-sectional area (CSA) in healthy children, with the goal of presenting reference values and discussing their implications.We performed a systematic review and meta-analysis of studies that reported ultrasound measurements of the upper or lower limb nerves in healthy children through a search of Web of Science, PubMed, Embase and Scopus.Sixteen studies with measurements of 10 nerves covering a total of 5149 nerves measured in 823 healthy children (445 boys and 378 girls) were included. Mean nerve CSA increased with age in the median nerve at the middle and lower third of the upper arm, mid-forearm, and distal wrist crease, the ulnar nerve at the middle third of the upper arm and elbow, the radial nerve at the spiral groove, and the tibial nerve at the popliteal fossa. Growth charts for nerve CSA for different age groups were developed.This meta-analysis provides robust reference values for nerve CSA at different sites in children, and this can inform clinical practice and assist in identifying nerve enlargement. Moreover, it identifies the strength and quality of the current published data. We recommend future studies divide their samples into smaller age subgroups and standardize the anatomic site of measurement This article is protected by copyright. All rights reserved.

Limited evidence exists on real-world adherence to nusinersen for the treatment of spinal muscular atrophy (SMA). Data are presented from a multi-site retrospective chart review of 86 adults with SMA initiating nusinersen at nine US centers between January 2017 and February 2019. Seventy-nine (92%) adults remained on nusinersen during the study; 454 (92%) of 493 total nusinersen doses were received on time. Fifty-eight (67%) adults received all nusinersen doses on time. The majority of patients with at least one nonadherent dose resumed nusinersen on time. Most patients followed the dosing schedule across the loading and maintenance dose periods.

Introduction/aims There are currently three medications approved for spinal muscular atrophy (SMA), but the use of these medications in combination has not been well described. Methods This is a retrospective report of four cases of SMA treated with dual onasemnogene and risdiplam therapy at our institution. Results Following onasemnogene therapy, all four patients experienced a perceived plateau of therapeutic benefit, at which time daily risdiplam was started. Transient fatigue and weakness was seen in two patients following risdiplam initiation, but this resolved within 1 mo. One patient was hospitalized with severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) and post-viral pneumonia, weeks following risdiplam initiation. No other adverse effects related to onasemnogene and risdiplam combination therapy were identified and all patients experienced objective and subjective improvement. Discussion Combination therapy with onasemnogene and risdiplam in patients with SMA appears to be well-tolerated. Further large prospective trials are needed to determine whether dual therapy is more efficacious than monotherapy, and to identify rare adverse events that may occur with the use of combination therapy.

There is currently a shortage of neurologists in the United States. Multiple efforts are underway to increase the strength of the neurology workforce. One potential approach is early exposure to neurology research and clinical care to pique interest and promote the specialty as a career choice. This study details the rewarding experience of working with undergraduate students, both in clinical research and clinical care. The logistics, benefits to students, and positive aspects for neurologists are outlined. Examples provided by undergraduate students who have participated in neurology research and clinical care are presented. The ultimate goals of this work are to encourage and inspire academic neurologists to involve undergraduate students in research and clinical care, to facilitate this process by outlining the steps needed to make this pairing successful, and to ultimately promote a pathway to build the neurology pipeline.

INTRODUCTION Ultrasound is emerging as a useful tool for the evaluation of immune-mediated neuropathies because it can provide high-resolution anatomic information to complement electrodiagnostic data. Nerve enlargements are commonly found in chronic inflammatory demyelinating polyneuropathy (CIDP), and their presence likely useful in diagnosis, particularly if multifocal. METHODS In this study, the authors undertook a retrospective chart review to identify ultrasound findings in patients with CIDP previously studied in a single busy neurodiagnostic laboratory. RESULTS Of the 50 cases identified from 2000 to 2017, individuals with a confirmed diagnosis of CIDP (21 cases) were more likely to have multiple sites of enlargement, as well as more pronounced nerve enlargement, than patients who were subsequently found to have an alternate cause of neuropathy (22 cases). The presence of any moderately enlarged nerve segment predicted definite CIDP with sensitivity of 81% and specificity 77%. CONCLUSION This study demonstrates that ultrasound can be of diagnostic utility in patients with suspected CIDP, even when conducted in a nonstandardized real-world setting.

Interleukin-6 (IL6) expression increases in atrophying muscles and lung tissue during compromised function. Considering ALS patients undergo these same pathological changes, IL6 levels may be relevant for prognostication and treatment. The amount of soluble IL6 receptor, dictated by the IL6R358Ala variant, and local tissue environment in which IL6 signaling occurs is known to influence the ultimate effects of IL6 in multiple diseases. In this longitudinal study, we show that serum IL6 levels negatively correlate both with the patient's functional status as measured by the overall ALSFRS-R and subscores, and with respiratory function as measured by the percent predicted FVC (ppFVC). The correlations are only present in the two-thirds of patients who carry the IL6R358Ala variant that mediates pro-inflammatory transsignaling in the cases of ALSFRS-R limb and respiratory subscores and ppFVC. These results suggest that some observed associations between IL6 and ALS are driven by the subset of patients carrying the IL6R358Ala variant and thus that any IL6-targeted therapeutic approaches may be more advantageous when aimed at this group. Specifically, with relation to respiratory decline, these patients may benefit from closer respiratory follow-up and early initiation of noninvasive ventilation.

ObjectiveTo describe rapid implementation of telehealth during the COVID-19 pandemic and assess for disparities in video visit implementation in the Appalachian region of the United States.MethodsA retrospective cohort of consecutive patients seen in the first 4 weeks of telehealth implementation was identified from the Neurology Ambulatory Practice at a large academic medical center. Telehealth visits defaulted to video, and when unable, phone-only visits were scheduled. Patients were divided into 2 groups based on the telehealth visit type: video or phone only. Clinical variables were collected from the electronic medical record including age, sex, race, insurance status, indication for visit, and rural-urban status. Barriers to scheduling video visits were collected at the time of scheduling. Patient satisfaction was obtained by structured postvisit telephone call.ResultsOf 1,011 telehealth patient visits, 44% were video and 56% phone only. Patients who completed a video visit were younger (39.7 vs 48.4 years,p< 0.001), more likely to be female (63% vs 55%,p< 0.007), be White or Caucasian (p= 0.024), and not have Medicare or Medicaid insurance (p< 0.001). The most common barrier to scheduling video visits was technology limitations (46%). Although patients from rural and urban communities were equally likely to be scheduled for video visits, patients from rural communities were more likely to consider future telehealth visits (55% vs 42%,p= 0.05).ConclusionRapid implementation of ambulatory telemedicine defaulting to video visits successfully expanded video telehealth. Emerging disparities were revealed, as older, male, Black patients with Medicare or Medicaid insurance were less likely to complete video visits.

PURPOSE Electrodiagnostic testing is often critical for the diagnosis of neuromuscular disease, but it can be challenging in the pediatric population because of poor tolerance. This study evaluates an ultrasound-first approach as a means of reducing the number of electrodiagnostic tests (nerves stimulated and muscles sampled) in the pediatric population. METHODS A retrospective analysis of 21 pediatric patients who underwent ultrasound before electrodiagnostic testing as part of a quality-improvement project was conducted and compared with an age-matched control group of 84 patients. RESULTS The ultrasound-first group underwent significantly fewer muscle samplings (P = 0.0002) and overall number of electrodiagnostic tests (P = 0.0010) than the control group. The ultrasound-first group also underwent fewer nerve stimulations, which approached statistical significance (P = 0.0600). CONCLUSIONS Using ultrasound first in the electrodiagnostic laboratory in pediatric patients could reduce the number of electrodiagnostic tests needed to arrive at a diagnosis.

A 10-year-old boy presented with muscle stiffness of all extremities. His symptoms typically began during the second or third period of his hockey games and were intermittent, resolving within minutes of cessation of activity. Otherwise, he was asymptomatic. He was able to play baseball and soccer without similar symptoms, and he and his family denied clear triggers such as cold, heat, or meals with high carbohydrates or potassium. He denied vision problems, facial weakness, dysphagia, dysarthria, dyspnea, orthopnea, cramping, and fasciculations. He also denied sensory changes and bladder or bowel dysfunction. His family history was notable for myotonia congenita in his father, who was genetically heterozygous in 2007 for the pathogenic c.1437_1450del variant in the CLCN1 gene.1 His father reported similar symptoms including stiffness exacerbated by physical exertion. His parents were specifically concerned that the patient had inherited the mutation from his father. On neurologic examination, the patient had eyelid myotonia and trace percussion myotonia in the right thenar eminence. Strength was 5/5 throughout, and deep tendon reflexes were 2+ and symmetrical in all 4 extremities. Muscle bulk and tone were normal, sensory examination was normal, and coordination was intact. The patient's father had grip and eyelid myotonia and muscle hypertrophy.

Contains fulltext : 248859.pdf (Publisher’s version ) (Closed access) 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.

Objectives Diagnosis and treatment monitoring in CIDP is primarily based on clinical parameters. High-frequency ultrasound of peripheral nerves can reflect CIDP pathophysiology and changes with treatment. This project investigated potential diagnostic and prognostic biomarkers utilizing neuromuscular ultrasound. Methods We conducted a standardized clinical and ultrasonographic assessment of 50 CIDP patients (25 at WFBMC, 25 at Austin), comparing to 25 healthy controls and 25 axonal neuropathy subjects. Our protocol included whole-length assessment of both median and ulnar nerves, with unilateral assessment of other nerves. Results 25 of 25 CIDP patients studied at WFBMC had an abnormality on ultrasound (as determined by focal nerve enlargement determined by increased cross-sectional area), with 23 of 25 subjects having >=4 enlarged segments. 23 of 25 Austin CIDP patients had at least one enlarged segment, and 20 of 25 had >=4 enlarged segments. 46 of 48 had at least one abnormality in either median or ulnar nerve. Mild nerve enlargements were infrequently seen in healthy and disease controls. However, CIDP patients had clear difference in extent and pattern of enlargements, particularly with proximal upper limb enlargement. Specific markers differentiating CIDP patients will be presented. We analyzed our data in line with previously published diagnostic scores. We will discuss these findings for typical vs. atypical CIDP subtypes, and clinical correlations. Conclusions This cross-sectional study of neuromuscular ultrasound in patients with CIDP suggests assessing bilateral whole-length median and ulnar nerves may be adequate for diagnosis, and differentiating potentially treatment responsive immune-mediated neuropathies from axonal neuropathies and healthy controls.

BACKGROUND AND PURPOSE Mycobacterium leprae complex affects peripheral nerves, causing nerve enlargement. This proof-of-concept pilot study was conducted to determine if a small hand-held ultrasound device can accurately identify nerve involvement in individuals with leprosy. METHODS Peripheral nerve ultrasound was conducted of the bilateral median (wrist, forearm, elbow, and mid-humerus), ulnar (wrist, forearm, elbow, 4 cm proximal to the elbow, and mid-humerus), C5 root, and greater auricular nerves with a standard ultrasound device (15 Mhz) and a hand-held ultrasound device (5 MHz). Nerve cross-sectional areas were compared using the two devices. RESULTS Eight individuals with leprosy were examined. Strong correlation was found between the standard and hand-held ultrasound devices (r = .76, P < .001). A certain amount of variability between ultrasound devices may occur for multiple reasons. CONCLUSION A hand-held ultrasound device can readily identify nerve enlargement in individuals with leprosy. This type of device may assist in the diagnosis of leprosy in areas with limited healthcare resources because of the portability and low-cost nature of such devices.

Spinal muscular atrophy (SMA) is an inherited lower motor neuron disease. SMA occurs secondary to alterations in the survival motor neuron 1 gene (SMN1), which is the main driver of SMN protein production. The severity of the disease is determined by the number of copies of the SMN2 gene, which is a homolog to SMN1 but not as efficient in protein production. Three medications have recently been approved for the treatment of SMA. Nusinersen is an intrathecal antisense oligonucleotide that alters SMN2 pre-mRNA, onasemnogene abeparvovec-xioi is an intravenous SMN1 gene replacement therapy, and risdiplam is an oral small molecule splicing modifier of SMN2. No head-to-head studies have been conducted comparing these medications, so selection of one of these medications for an individual with SMA can be challenging. In this article we outline the efficacy, safety, and other pertinent factors to consider when selecting a therapy for an individual with SMA. The age of the individual and comorbidities, such as liver or kidney disease, help guide treatment choices. All three of these medications are efficacious, and early initiation is critical for obtaining the best outcomes.

Accurate assessment of neuromuscular disorders is critical to facilitate timely treatment and achieve the best outcomes. Historically, electrodiagnostic studies have filled this role, but recently, neuromuscular ultrasound is being used in the electrodiagnostic laboratory. This review discusses the uses of neuromuscular ultrasound in the electrodiagnostic laboratory that have strong evidence, emphasizing those that could be adopted in a typical electrodiagnostic laboratory with a reasonable level of equipment and training. The evidence currently supports using neuromuscular ultrasound to diagnose carpal tunnel syndrome and ulnar neuropathies at the elbow and as a supplementary test when electrodiagnostic studies are suspected to be falsely negative or in axonal nonlocalizing lesions. Neuromuscular ultrasound can identify the causes of focal mononeuropathies, which can change treatment in specific cases. It is sensitive at identifying fasciculations and providing complementary evidence of autoimmune demyelinating polyneuropathies. It is particularly helpful in assessing nerves after trauma. Neuromuscular ultrasound is likely to prove even more useful in the electrodiagnostic laboratory as the technology continues to advance.

Neuromuscular ultrasound (NMUS) is a diagnostic technique that is now commonly used to complement electrodiagnostic studies when evaluating for suspected peripheral nerve pathology. The use of NMUS has been described in focal mononeuropathies, polyneuropathies, and motor neuron disease. This article provides a general overview of NMUS for the evaluation of peripheral neuropathies based on clinical practice guidelines, primary literature, and expert opinion. The characteristic changes detected by NMUS are described in focal mononeuropathies (including entrapment and other causes), polyneuropathies (including demyelinating and axonal processes), and motor neuron disease.

ObjectiveTo evaluate the cost-effectiveness of neuromuscular ultrasound (NMUS) for the evaluation of focal neuropathies.MethodsA 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.ResultsThe 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.ConclusionsFrom 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.ClinicalTrials.gov identifier:NCT01394822.

ObjectiveThis study was conducted to determine whether longer lifespans in African Americans with amyotrophic lateral sclerosis (ALS), compared to white non-Hispanics, are secondary to higher rates of tracheostomy and invasive ventilation (TIV) in African Americans.MethodsA retrospective case-control study was conducted with 49 African Americans with ALS matched by age, gender, and site of onset to 137 white persons with ALS.ResultsAfrican Americans had longer survival than whites when the outcome was death (p = 0.016), but this was no longer significant when the outcome was death or TIV (p = 0.100). African Americans also had a lower rate of noninvasive ventilation use compared to whites (27 [55%] vs 96 [70%], p = 0.015) and a higher rate of TIV (8 [16%] vs 7 [5%], p = 0.016), but after controlling for baseline severity, only the noninvasive ventilation difference (p = 0.036), and not the TIV difference (p = 0.115), remained significant.ConclusionAfrican Americans with ALS live longer than white persons with ALS, and this may be secondary to higher rates of TIV use among African Americans.

BACKGROUND AND PURPOSE There is a lack of consensus in the literature as to which body habitus parameter most influences nerve cross-sectional area (CSA). This study was specifically designed to determine if height or weight is more influential. METHODS Fifteen young healthy participants, 8 extremely tall and 7 heavy, with no peripheral nerve symptoms were recruited. The tall cohort consisted of males who were 74 inches or taller and females who were 68 inches or taller. The heavy cohort consisted of males who were 274 lbs or heavier and females who were 200 lbs or heavier. Measurements were obtained bilaterally at 7 sites using neuromuscular ultrasound: median nerve at the wrist and mid-forearm, ulnar at the elbow, radial at the spiral groove, fibular at the knee, tibial at the ankle, and sural at the ankle. The nerve CSA at each site was measured by tracing of the nerve using the "freehand" trace function of the ultrasound device. RESULTS Weight tightly correlated with nerve CSA (R2 = .69, P < .001), while height did not significantly predict CSA (R2 = .10, P = .244). Nerve CSA for tall participants did not systematically deviate from an historical control group. Conversely, with exception of the tibial and sural nerves at the ankle, all nerve CSAs for heavy participants were higher than in the control group. CONCLUSION Weight was found to be the body habitus parameter that most influences nerve CSA. This will inform clinicians when using nerve imaging to evaluate participants at either extreme of weight.

Immunosuppressants are commonly used in the treatment of neuromuscular diseases. The use of these medications requires health maintenance screening and monitoring for multiple potential sides effects, which may be best conducted when the prescribing physician and primary care physician work in tandem. Clinical practice guidelines, recommendations from professional organizations, prescription drug labeling, and expert opinion were reviewed and combined to provide recommendations for health maintenance for eight different immunosuppressants. Recommendations are provided for vaccinations, infection prophylaxis, laboratory monitoring, bone density screening, cancer screening, and other considerations. While studies in individuals with neuromuscular diseases are sparse, knowledge gained from the use of immunosuppressants in other conditions is applied in this review to provide recommendations to be used during the treatment of neuromuscular diseases in adults. Muscle Nerve 59:397-403, 2019.

INTRODUCTION This study explores the reliability and responsiveness of neuromuscular ultrasound in amyotrophic lateral sclerosis (ALS). METHODS Investigations were conducted with 10 healthy controls, 10 patients with ALS (single point in time), and 10 different patients with ALS (followed over 6 months; 4 completed follow-up). Ultrasound was used to measure the thickness of the geniohyoid, bilateral biceps/brachialis, bilateral tibialis anterior, and bilateral hemidiaphragms (at inspiration and expiration). Interrater and intrarater reliability and change in muscle thickness over 6 months were measured. RESULTS Interrater correlation coefficients ranged between 0.80 and 0.99 in healthy controls and between 0.78 and 0.97 in patients with ALS. Intrarater correlation coefficients ranged between 0.83 and 0.98 in healthy controls. The mean percentage decline in muscle thickness over 6 months was 20.25%. DISCUSSION Muscle ultrasound appears to be a reliable technique for measuring important muscles in patients with ALS. Larger studies with age-matched controls should be conducted to assess further the responsiveness of this biomarker in ALS. Muscle Nerve 59:181-186, 2019.

Contains fulltext : 238533.pdf (Publisher’s version ) (Open Access) 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.

Item does not contain fulltext 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.

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.

Interleukin-6 (IL6) expression increases in atrophying muscles and lung tissue during compromised function. Considering ALS patients undergo these same pathological changes, IL6 levels may be relevant for prognostication and treatment. The amount of soluble IL6 receptor, dictated by the IL6R

BACKGROUND The most common neuromuscular ultrasound abnormality in carpal tunnel syndrome is enlargement of the median nerve near the distal wrist crease, but sometimes the median nerve enlarges in the palm, just distal to the transverse carpal ligament. This study was conducted to devise a protocol for measuring the median nerve in the palm and to generate reference values for nerve cross-sectional area at this site. METHODS A systematic protocol was developed, 30 healthy individuals underwent ultrasound of the median nerve in the palm bilaterally, and cross-sectional area was recorded. RESULTS The mean cross-sectional area of the median nerve at the palm was between 11.4 mm2 (95% confidence interval [CI] [10.4,12.4]) and 11.6 mm2 (95%CI [10.6,12.6]). CONCLUSIONS The median nerve can be measured just distal to the hook of the hamate in the palm, and the mean cross-sectional area at this site is slightly larger than it is at the distal wrist crease.

We systematically identified and reviewed 29 studies of peripheral nerve ultrasound or magnetic resonance imaging (MRN) in amyotrophic lateral sclerosis (ALS). The majority of the ultrasound studies reported smaller nerves and nerve roots in ALS compared to healthy controls, but there was a large overlap of the cross-sectional nerve area between ALS and controls. Most of the MRN studies confirmed nerve abnormalities demonstrating slight T2 hyperintensities and, sometimes, mild enlargement of more proximal nerve segments (plexus, roots) in ALS. The size of the proximal nerve segments, i.e. nerve roots, is thus somewhat incongruent between nerve ultrasound and MRN in ALS. Peripheral nerve ultrasound has the potential to differentiate between ALS and multifocal motor neuropathy (MMN) in that patients with MMN have significantly larger nerves. Conversely, there is an overlap of MRN abnormalities in ALS and MMN, restricting the techniques' utility in the clinical setting. A subgroup of patients with ALS seems to reveal a sonographic nerve pattern suggesting peripheral nerve inflammation. In the future, combined imaging with nerve ultrasound and MRN assessing parameters such as blood flow or textural markers may aid in the understanding of the deep nerve microstructure down to the fascicle level, and thus, in the classification of the nerve state as more degenerative or more inflammatory in ALS. This systematic review provides evidence that nerve imaging abnormalities are common in ALS.

BACKGROUND The treatment of spinal muscular atrophy (SMA) with nusinersen requires intrathecal medication administration, which can be challenging in individuals with complicated spines. This retrospective case series reviews the nusinersen treatment experience at one academic medical center with children and adults with SMA and complicated spines. METHODS Twenty medical records of individuals receiving nusinersen were reviewed and administration methods summarized and assessed. RESULTS Ten children and 10 adults were treated, and 55% had complicated spines. In total, 163 treatments were given, 91 in those with complicated spines. In the complicated spines, 74% of treatments were done by means of fluoroscopic lumbar puncture, 22% by means of intrathecal Ommaya reservoir, 3% by means of palpation, and

Reliable assessment of brachial plexus disorders can be challenging due to the complexity of the anatomy and variation of potential pathology. Electrodiagnostic testing can be both uncomfortable for the patient and inconclusive. Ultrasound can serve as a complement to clinical assessment, electrodiagnostic testing, and other imaging modalities. This study describes a systematic approach for performing neuromuscular ultrasound for suspected pathology in the brachial plexus. The literature regarding techniques for brachial plexus ultrasound was reviewed. A team composed of specialists in neurology, physiatry, anesthesiology, orthopedic surgery, and vascular surgery used this as the basis for describing standardized techniques for performing brachial plexus ultrasound. Four standard views, along with other supplemental views, are described for the evaluation of the brachial plexus. An illustrative case is presented. Ultrasound is a high-resolution point of care diagnostic tool that allows assessment of structural pathology affecting the brachial plexus. Muscle Nerve 58: 618-624, 2018.

Introduction Magnet therapy has been proposed as a treatment for neurologic conditions. In this this trial we assessed the feasibility and efficacy of a magnet inserted into a wristband for carpal tunnel syndrome (CTS). Methods Twenty-two patients with mild to moderate CTS were randomized to wear a high-dose or low-dose "sham" magnetic wristband for 6 weeks. The primary outcome was the Symptom Severity Scale (SSS) of the Boston Carpal Tunnel Questionnaire. Secondary measures were nerve conduction studies (NCS), median nerve ultrasound, and compliance. Results Compliance for both groups was >90%. Improvements in the mean SSS, NCS, and median nerve ultrasound did not reach statistical significance. Discussion Magnet therapy via wristband is well-tolerated. Further investigations in larger populations are needed to determine efficacy. Muscle Nerve 58: 310-313, 2018.

INTRODUCTION Ultrasound can potentially identify nerves and guide recording and stimulating electrode placement for nerve conduction studies (NCS). This prospective study was performed to determine whether ultrasound guidance of sural NCS results in higher action potential amplitude, fewer stimuli required, lower stimulus strength required, and less pain experienced. METHODS Fourteen healthy individuals underwent bilateral sural NCS, both with and without ultrasound guidance. Studies were separated by at least 48 h, and the order of testing was randomly assigned. RESULTS Ultrasound guidance resulted in significantly fewer stimuli and lower stimuli strength required to obtain supramaximal responses (P

Introduction: Neuromuscular ultrasound (NMUS) is an accepted, but underutilized, diagnostic tool in the evaluation of neuromuscular disorders. The lack of uniform high-quality standards in published research and in clinical practice has been a limitation to its growth. Methods: In this article we address the shortcomings of NMUS research and establish basic standards to guide further work in the field. Results: A review of the basic types of NMUS research studies is presented, along with the elements that each should include. Basic standards for this imaging technique are also presented. An explanation of common data elements (CDEs) is provided with discussion of how NMUS may benefit from their creation. Conclusions: High-quality NMUS research is needed to advance the field. Assuring that publications meet basic standards is essential. In addition, the creation of CDEs specific for NMUS is proposed. Muscle Nerve 56: 375–378, 2017

BACKGROUND AND PURPOSE Autonomic neuropathy is a serious and common complication of diabetes mellitus. The vagus nerve is the longest autonomic nerve, and may be affected in diabetes as a part of generalized neuropathy. Our objective was to assess for possible sonographic changes of the vagus nerve in diabetic patients. METHODS The vagus nerve was bilaterally scanned in 20 healthy volunteers and 54 patients with diabetes in the axial plane at the lateral neck. RESULTS The mean cross-sectional area of the vagus nerve was significantly smaller in patients with diabetes compared with controls. Receiver operating characteristic curve analyses revealed an area under the curve of .96, and an optimum cutoff point of 3 mm2 with a sensitivity of 85.2% and specificity of 100%. CONCLUSIONS This study demonstrates a degree of vagus nerve atrophy in patients with diabetes. This finding may have relevance in the diagnosis and treatment of diabetic neuropathy if further validated.

Introduction An ultrahigh-frequency (70 MHZ) ultrasound device has recently been approved for human use. This study seeks to determine whether this device facilitates counting of fascicles within the median nerve at the wrist. Methods Twenty healthy volunteers underwent imaging of the median nerve at the wrist bilaterally. The number of fascicles in each nerve was counted by two independent raters. Results The mean fascicle number was 22.68. Correlation was strong between the two raters (r = 0.68, P < 0.001). Age, sex, body mass index, and nerve area did not predict fascicle number. Those with bifid median nerves and persistent median arteries had lower fascicle density than those without anatomic anomalies (1.79 vs. 2.29; P = 0.01). Discussion Fascicles within the median nerve at the wrist can be readily imaged. Ultrahigh-frequency ultrasound technology may be informative in a variety of disorders affecting the peripheral nervous system. Muscle Nerve 56: 819–822, 2017

Introduction Clinicians who treat nerve and muscle disorders may be asked to evaluate patients who have unilateral or bilateral scapular winging. Traditionally, this evaluation has relied upon a thorough history, physical examination, and electrodiagnostic testing to localize the cause of winging and detect the underlying neuromuscular pathology. Neuromuscular ultrasound has emerged as a non-invasive technique that can be used for structural evaluation of nerve and muscle abnormalities. Methods Previous studies of imaging in scapular winging and experiences from our diagnostic laboratory are reviewed. Results Four standard and 4 ancillary ultrasound views are described for evaluation of scapular winging. Conclusion Ultrasound is a non-invasive, painless, and radiation-free technology that can be used to evaluate scapular winging. Muscle Nerve 56: 7-14, 2017.

Neuromuscular ultrasound is a distinct specialty concerned with the assessment of primary neuromuscular disorders using high-resolution ultrasonography. Ultrasound allows the assessment of the structural and anatomical aspect of the peripheral nervous system and thus complements the electrodiagnostic tests which assess the functional aspect. Given its many advantages, neuromuscular ultrasound is an attractive diagnostic, interventional, and follow-up tool in children with neuromuscular disorders. The applications of neuromuscular ultrasound in children are not fully explored but the future is promising. In this chapter, we present the principles of neuromuscular ultrasound, the general scanning techniques of peripheral nerves and skeletal muscles, the scanning protocols in nerve and muscle disorders, and the value of neuromuscular ultrasound in different pediatric neuromuscular diseases in addition to highlights on advanced ultrasound techniques, ultrasound-guided procedures, and future directions. The detailed hands-on scanning techniques of all peripheral nerves and muscles are out of scope of this chapter and are best learned through hands-on courses and workshops.

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 mm(2) smaller than healthy controls (P ≤ .01). Older age and time since taxane were associated with smaller sural nerve CSA. For each 1 mm(2) decrease in sural nerve CSA, distal IENFD decreased by 2.1 nerve/mm (R(2) 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.

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.