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.

Evidence-based guideline: Neuromuscular ultrasound for the diagnosis of ulnar neuropathy at the elbow.

INTRODUCTION/AIMS: 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?" METHODS: 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. RESULTS: 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. RECOMMENDATION: 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).

American Academy of Neurology Telehealth Position Statement.

Telehealth services complement in-person neurologic care. The American Academy of Neurology supports patient access to telehealth services regardless of location, coverage for telehealth services by all subscriber benefits and insurance, equitable provider reimbursement, simplified state licensing requirements easing access to virtual care, and expanding telehealth research and quality initiatives. The roles and responsibilities of providers should be clearly delineated in telehealth service models.

Use of a Smartphone to Gather Parkinson's Disease Neurological Vital Signs during the COVID-19 Pandemic.

INTRODUCTION: To overcome travel restrictions during the COVID-19 pandemic, consumer-based technology was rapidly deployed to the smartphones of individuals with Parkinson's disease (PD) participating in a 12-month exercise trial. The aim of the project was to determine the feasibility of utilizing a combined synchronous and asynchronous self-administered smartphone application to characterize PD symptoms. METHODS: A synchronous video virtual visit was completed for the administration of virtual Movement Disorder Society-Unified Parkinson's Disease Rating Scale III (vMDS-UPDRS III). Participants asynchronously completed a mobile application consisting of a measure of upper extremity bradykinesia (Finger Tapping Test) and information processing. RESULTS: Twenty-three individuals completed the assessments. The mean vMDS-UPDRS III was 23.65 ± 8.56 points. On average, the number of taps was significantly greater for the less affected limb, 97.96 ± 17.77 taps, compared to the more affected, 89.33 ± 18.66 taps (p = 0.025) with a significantly greater number of freezing episodes for the more affected limb (p < 0.05). Correlation analyses indicated the number of errors and the number of freezing episodes were significantly related to clinical ratings of vMDS-UPDRS III bradykinesia (Rho = 0.44, p < 0.01; R = 0.43, p < 0.01, resp.) and finger tapping performance (Rho = 0.31, p = 0.03; Rho = 0.32, p = 0.03, resp.). Discussion. The objective characterization of bradykinesia, akinesia, and nonmotor function and their relationship with clinical disease metrics indicate smartphone technology provides a remote method of characterizing important aspects of PD performance. While theoretical and position papers have been published on the potential of telemedicine to aid in the management of PD, this report translates the theory into a viable reality.

Enhancing diagnostic accuracy using a side-to-side cross-sectional area ratio for the diagnosis of unilateral ulnar mononeuropathy at the elbow.

INTRODUCTION: Neuromuscular ultrasonography (NMUS) is a valuable adjunct to electrodiagnostic testing for the diagnosis of entrapment neuropathy. The aim of this study was to determine whether diagnostic accuracy of NMUS could be enhanced in patients with unilateral ulnar mononeuropathy at the elbow (UNE) by utilizing side-to-side ulnar nerve cross-sectional area (CSA) ratios. METHODS: Retrospective case-control analysis of unilateral UNE cases identified cutoff values for elbow segment ulnar nerve maximum CSA (MCSA) of the symptomatic/asymptomatic limb (M ratio), as well as side-to-side ratios comparing MCSA with ipsilateral CSA at the Guyon canal (E/G), middle forearm (E/F), and middle humerus (E/H). Diagnostic accuracy values were calculated. RESULTS: The optimal M-ratio cut-off was 1.22 (sensitivity, 92.9%; specificity, 97.8%; accuracy, 95.4%). Optimal cutoffs for inter-E/G, -E/F, and -E/H ratios were 1.07 (sensitivity, 98%; specificity, 78%; accuracy, 87.7%), 1.11 (sensitivity, 95%; specificity, 80%; accuracy, 87.2%), and 1.18 (sensitivity, 95%; specificity, 93%; accuracy, 94%), respectively. DISCUSSION: The M ratio and inter-E/H ratio exhibited high diagnostic accuracy for unilateral UNE. Prospective studies are needed to compare the accuracy of the new measures with a single MCSA measurement.

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.

Reducing Wrong-level Spinal Surgeries Through Root Cause Analyses: A 10-year Longitudinal Analysis of a Single Tertiary Institution's Iterative Policy Improvements.

MINI: This study is a comprehensive narrative of all wrong-level spine surgeries and prevention strategies employed at our institution between 2008 and 2019, and aims to provide a roadmap for developing a rigorous prevention protocol. We systematically track root cause analyses and policy changes to determine which prevention strategies are most effective.

Retrospective review. We aim to create a comprehensive narrative of all wrong-level spinal surgeries (WLSS) and subsequent prevention strategies employed at our institution and provide a roadmap for developing a rigorous prevention protocol. There is currently no published evidence-based protocol to prevent WLSS. Previous studies are limited to multi-institution surgeon surveys and opinion pieces; the impact of serial interventions to eliminate WLSS is lacking. No studies have longitudinally analyzed a single institution's serial root cause analyses (RCA) of individual WLSS cases and the stepwise impact of targeted interventions to reduce WLSS occurrence. We reviewed all wrong-site spine surgeries and prevention strategies employed at our institution between 2008 and 2019, and corresponding WLSS-related RCAs were collected from institutional records. We conducted a longitudinal analysis of these reports and tracked policy implementations that resulted along with the incidence of WLSS following each policy. Fifteen WLSS were identified with 13 corresponding RCAs of 21,179 spine surgeries between 2008 and 2019. Three policy categories emerged: imaging, operating room (OR) culture, and vertebral body marking. The salient changes from each category were: requiring two immovable vertebral markers (2013); requiring intraoperative radiographs with markers and retractors positioned (2014); open-ended questioning during spinal level verification by residents and fellows (2015); and requiring an impartial radiologist to have verbal contact with the operating surgeon intraoperatively to collaboratively discuss localization (2018). Each change resulted in WLSS incidence decline (five in 2014, three in 2015, 0 in 2019). Stepwise process improvement based on WLSS case review is necessary, as no one change in standard operating procedure effectively eliminated WLSS. Improvements in communication between OR staff, surgeon, and radiologist, as well as intraoperative imaging and marking optimization all contributed to improvements in WLSS rates. By focusing on lessons learned from RCAs using this methodology, institutions can iteratively improve rates of WLSS. Level of Evidence: 4.

Teleneurology as a Solution for Outpatient Care During the COVID-19 Pandemic.

Background: The coronavirus disease of 2019 (COVID-19) pandemic and the need for social distancing have dramatically changed health care delivery. There is an urgent need to continue to deliver outpatient care for chronic neurological disease and teleneurology has the potential to fulfill this gap. Introduction: This study reports the implementation and utilization of teleneurology across all neurological subspecilities during the COVID-19 pandemic. Materials and Methods: This is a retrospective observational study that identified all in-person and teleneurology outpatient nonprocedural visits from January 5 to April 4, 2020, across neurological specialties at a single academic center. Visit volumes were assessed weekly and practice patterns were compared before and after March 15, 2020, as this was the date of a major statewide stay-at-home order in Ohio. Results: Before March 15 the mean in-person visit per week was 5129.4 and decreased to 866.7 after that date. The mean teleneurology visits per week increased from 209.1 to 2619.3 for the same time period. The overall teleneurology visit volume in the 3 weeks after March 15 increased by 533%. Discussion: In a relatively short time frame of 3 weeks, a single academic center was able to dramatically increase teleneurology visits to provide outpatient neurological care. Conclusions: This study demonostrates that teleneruology can be a solution for outpatient neurological care in the context of COVID-19. The increased utilization of teleneurology during this crisis has the potential to expand teleneurology and improve access to neurological care in the future outside the pandemic setting.

Ultrasound in the diagnosis and management of fibular mononeuropathy.

INTRODUCTION: Ultrasound (US) evaluation of peripheral nerves is a noninvasive, cost-effective approach to diagnosing focal mononeuropathies and guiding surgical management. We used the intranerve ratio to evaluate for possible cut-off values in diagnosis of fibular mononeuropathies (FNs). METHODS: A retrospective analysis of FN confirmed by electrodiagnosis (EDx) was performed to identify intranerve ratio values between affected and unaffected limbs at the fibular head and popliteal fossa. RESULTS: The optimal fibular head/popliteal fossa intranerve ratio to discriminate between limbs with and without disease was 1.25 (sensitivity, 51%; specificity, 71%). There was no statistically significant difference between affected vs unaffected limbs (ratio, 1.13; P = .15) nor in subgroup analyses. However, 25% of patients had structural lesions amenable to surgery. DISCUSSION: The utility of US in diagnosis of FN is limited using intranerve ratio data, but US has a distinct advantage over EDx for identifying treatable structural lesions.

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.

Association of statin pretreatment with collateral circulation and final infarct volume in acute ischemic stroke patients: A meta-analysis.

BACKGROUND AND AIMS: Statin pretreatment (SP) is associated with improved outcomes in acute ischemic stroke (AIS) patients. Collateral circulation status and final infarct volume (FIV) are independent predictors of functional outcome in AIS. METHODS: We sought to evaluate the association of SP with collateral circulation and FIV in AIS patients. We used a random-effects model for all the analyses, and pooled standardized mean differences (SMDs) and odds ratios (OR) on the FIV and collateral status according to SP history, respectively. RESULTS: We identified 9 eligible studies (1186 AIS patients). History of SP was associated with lower FIV (SMD = 0.25, 95%CI: 0.07-0.42, p = 0.005) compared to negative history of SP. A trend towards good collateral scores was observed in the SP group (OR = 1.45; 95% CI, 0.92-2.29, p = 0.11). Subgroup analysis demonstrated reduced FIV among atherosclerotic stroke patients with history of SP (SMD = 0.49; 95% CI, 0.19-0.80, p = 0.001). CONCLUSIONS: SP appears to be associated with decreased FIV, especially in atherosclerotic AIS.

Modeling of Lung Function Recovery in Neuralgic Amyotrophy With Diaphragm Impairment.

BACKGROUND: Neuralgic amyotrophy is an inflammatory peripheral nerve disorder in which phrenic nerve involvement can lead to diaphragm paralysis. The prevalence, magnitude, and time course of diaphragm recovery are uncertain. METHODS: This study modeled the course of recovery of lung function in 16 subjects with diaphragm impairment from neuralgic amyotrophy. The first and last available vital capacity, sitting-to-supine decline in vital capacity, and maximal inspiratory pressures were compared. RESULTS: An asymptotic regression model analysis in 11 subjects with at least partial recovery provided estimates of the vital capacity at onset (47%, 95% CI 25-68%), the final vital capacity (81%, 95% CI 62-101%), and the half-time to recovery (22 months, 95% CI 15-43 months). In those subjects, there was a significant improvement between the first and last measured FVC (median 44-66%, P = .004) and maximal inspiratory pressure (mean 34-51%, P = .004). Five subjects (31%) with complete recovery had a final sitting-to-supine drop of vital capacity of 16% and a maximal predicted inspiratory pressure of 63%. CONCLUSIONS: Sixty-nine percent of subjects with diaphragm impairment from neuralgic amyotrophy experience recovery of lung function and diaphragm strength, but recovery is slow and may be incomplete.

Intraneural nodular fasciitis of the radial nerve with rapidly progressive motor symptoms.

BACKGROUND: Nodular fasciitis is a benign mesenchymal tumor arising from fascia that typically presents as a rapidly growing, subcutaneous mass. Intraneural cases are very rare and can present with neurological symptoms, requiring surgical resection. CASE DESCRIPTION: A 31-year-old woman presented to us with painful paresthesias in her elbow and progressive motor deficits, for which she underwent surgery. CONCLUSION: The authors report the first case of intraneural nodular fasciitis occurring in the radial nerve and highlight the possibility of rapidly progressive motor deficit in patients presenting with this rare clinical entity.

Reversible cerebral vasoconstriction syndrome associated with interferon beta-1a use for multiple sclerosis.

BACKGROUND: Reversible cerebral vasoconstriction syndrome (RCVS) has been associated with multiple medications, cocaine, pregnancy, migraine, and other conditions. OBJECTIVES: RCVS associated with interferon beta use has never before been described. METHODS: We describe the case of a 20-year-old female who developed acute onset severe headache and was found to have subarachnoid hemorrhage 2 months after initiating Rebif (Interferon beta-1a) for multiple sclerosis (MS). Cerebral angiography showed multiple areas of distal stenosis and dilatation with radiographic resolution 1 month later. RESULTS/CONCLUSIONS: This is the first case report of RCVS in an MS patient treated with Rebif.

Evidence-based guideline: neuromuscular ultrasound for the diagnosis of carpal tunnel syndrome.

INTRODUCTION: The purpose of this study was to develop an evidence-based guideline for the use of neuromuscular ultrasound in the diagnosis of carpal tunnel syndrome (CTS). METHODS: Two questions were asked: (1) What is the accuracy of median nerve cross-sectional area enlargement as measured with ultrasound for the diagnosis of CTS? (2) What added value, if any, does neuromuscular ultrasound provide over electrodiagnostic studies alone for the diagnosis of CTS? A systematic review was performed, and studies were classified according to American Academy of Neurology criteria for rating articles of diagnostic accuracy (question 1) and for screening articles (question 2). RESULTS: Neuromuscular ultrasound measurement of median nerve cross-sectional area at the wrist is accurate and may be offered as a diagnostic test for CTS (Level A). Neuromuscular ultrasound probably adds value to electrodiagnostic studies when diagnosing CTS and should be considered in screening for structural abnormalities at the wrist in those with CTS (Level B).

A 5-item scale to predict stroke outcome after cortical middle cerebral artery territory infarction: validation from results of the Diffusion and Perfusion Imaging Evaluation for Understanding Stroke Evolution (DEFUSE) Study.

BACKGROUND AND PURPOSE: Various clinical, laboratory, and radiographic parameters have been identified as predictors of outcome for ischemic stroke. The purpose of this study was to combine these parameters into a validated scale for outcome prognostication in patients with a middle cerebral artery territory infarction. METHODS: We retrospectively reviewed 129 patients over a 2-year period and considered demographic, clinical, laboratory, and radiographic parameters as potential predictors of outcome. Inclusion criteria were unilateral hemispheric infarcts within the middle cerebral artery territory >15 mm in diameter. Our primary outcome measure was a favorable recovery defined as a modified Rankin Score was ≤2 at 30 days. A multivariable model was used to determine independent predictors of outcome and weighted to create a 5-item scale to predict stroke recovery. External validation of this model was done using data from the Diffusion and Perfusion Imaging Evaluation for Understanding Stroke Evolution (DEFUSE) study. RESULTS: The 5 independent predictors of outcome were as follows: age (OR, 1.09; 95% CI, 1.03 to 1.14; P=0.001), National Institutes of Health Stroke Scale score (OR, 1.17; 95% CI, 1.06 to 1.30; P=0.003), infarct volume (OR, 1.01; 95% CI, 1.00 to 1.02; P=0.03), admission white blood cell count (8.5×10(3)/mm(3); OR, 1.16; 95% CI, 1.03 to 1.27; P=0.04), and presence of hyperglycemia (OR, 4.2; 95% CI, 1.1 to 16.4; P=0.04). Combining these variables into a point scale significantly improved prediction over the individual variables accounted alone as evidenced by the area underneath the receiver operating curve (OR, 0.91; 95% CI, 0.87 to 0.96; P=0.0001). When applied to the DEFUSE study population for validation, the model achieved a sensitivity of 83% and specificity of 86%. CONCLUSIONS: With validation from a prospective study of similar patients, this model serves as a useful clinical and research tool to predict stroke recovery after cortical middle cerebral artery territory infarction.

Novel GNE mutations in hereditary inclusion body myopathy patients of non-Middle Eastern descent.

Autosomal recessive hereditary inclusion body myopathy (HIBM or IBM2) is a progressive adult onset muscle wasting disorder characterized by sparing of the quadriceps. IBM2 is also known as distal myopathy with rimmed vacuoles or nonaka myopathy. IBM2 is associated with mutations in the UDP-GlcNAc 2-Epimerase/ManNAc Kinase gene (GNE). GNE is the rate-limiting enzyme of N-Acetylneuraminate (Neu5Ac, Sialic acid) biosynthesis. The GNE coding region of 64 symptomatic patients were sequenced. Twenty-eight patients were found to bear GNE mutations. Ten novel mutations were identified among nine patients, including four nonsense (p.R8X, p.W204X, p.Q436X, and p.S615X) and five missense (p.R71W, p.I142T, p.I298T, p.L556S, and p.E2G) variations spanning both the epimerase and kinase domains of GNE. Additionally, a synonymous variation (p.Y591Y, codon tac > tat) was seen in a patient bearing compound heterozygous nonsynonymous mutations (p.S615X and p.Y675H). Six of the nine are Caucasian, one patient is Taiwanese, one patient is Asian Indian, and one patient is of European descent. These findings further expand the clinical and genetic spectrum of IBM2.

Racing against the clock: recognizing, differentiating, diagnosing, and referring the amyotrophic lateral sclerosis patient.

Recognition of the early symptoms and signs in amyotrophic lateral sclerosis, exclusion of alternative diagnoses, and referral to a tertiary center can have a significant positive impact on the lives of patients and their caregivers. This article provides the most current amyotrophic lateral sclerosis criteria, as well as helpful clinical clues to the diagnosis. An approach to laboratory testing, electrodiagnostic testing, and imaging to exclude diseases that mimic ALS also are discussed, as are atypical presentations that can confound timely diagnosis.