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Background Peroneal neuropathy at the fibular head (PNFH) is a mononeuropathy that typically presents with drop foot and sensory abnormalities over the skin area innervated by the peroneal nerve. Objective The aim of the present study was to evaluate neuropathic pain in patients with PNFH. Methods Patients with clinical and electrodiagnostic features consistent with PNFH associated with weight loss, leg postures, or prolonged sleep were included in the present retrospective cohort study. Nerve conduction studies were performed in the bilateral lower extremities of all patients. The Leeds assessment of neuropathic symptoms and signs scale (LANSS) was applied to all patients. Results Thirty-two PNFH patients (78% males) were included in the study. The LANSS score in the majority of patients was lower than 12. There was 1 patient with a LANSS score of 12. The electrodiagnostic features of 16 patients were compatible with axonal degeneration. The mean LANSS scores of PNFH patients with and without axonal degeneration were 4.3 ± 3.7 and 5.2 ± 2.9, respectively (p = 0.255). Conclusion The present study showed that neuropathic pain is a rare symptom in patients with PNFH associated with weight loss, leg postures, or prolonged sleep. Resumo Antecedentes A neuropatia fibular na cabeça da fíbula (PNFH) é uma mononeuropatia que normalmente se apresenta com pé caído e anormalidades sensoriais sobre a área da pele inervada pelo nervo fibular. Objetivo O objetivo do presente estudo foi avaliar a dor neuropática em pacientes com PNFH. Métodos Pacientes com características clínicas e eletrodiagnósticas consistentes com PNFH associada a perda de peso, postura das pernas ou sono prolongado foram incluídos neste estudo de coorte retrospectivo. Estudos de condução nervosa foram realizados nas extremidades inferiores bilaterais de todos os pacientes. A escala de avaliação de sintomas e sinais neuropáticos de Leeds (LANSS) foi aplicada a todos os pacientes. Resultados Trinta e dois pacientes com PNFH (78%) foram incluídos no estudo. A pontuação LANSS em outros pacientes foi menor que 12. Houve 1 paciente com pontuação LANSS de 12. As características eletrodiagnósticas de 16 pacientes foram compatíveis com degeneração axonal. Os escores médios do LANSS de pacientes com PNFH com e sem degeneração axonal foram 4,3 ± 3,7 e 5,2 ± 2,9, respectivamente (p = 0,255). Conclusão O presente estudo mostrou que a dor neuropática é um sintoma raro em pacientes com PNFH associada à perda de peso, postura das pernas ou sono prolongado.

The diagnostic values of measuring median nerve (MN) stiffness and vascularity with shear wave elastography (SWE) and high-definition (HD) color were investigated in carpal tunnel syndrome (CTS).Seventy patients (123 wrists) with CTS and thirty-five healthy volunteers (70 wrists) were enrolled. Based on nerve conduction studies (NCS), the patients were subdivided into NCS-negative, mild-to-moderate, and severe CTS groups. MN and abductor pollicis brevis (APB) SWE and MN HD color were performed on a longitudinal plane.The mild-to-moderate and severe CTS groups showed increased MN stiffness at the wrist and MN stiffness ratio (wrist-to forearm) compared with the control (p 0.001). The NCS-negative CTS group showed increased MN stiffness at the wrist (p = 0.022) and MN stiffness ratio (p = 0.032) compared with the control. The severe CTS group showed increased MN stiffness at the wrist compared with the mild-to-moderate CTS group (p = 0.034). The cutoff-values in diagnosing NCS-confirmed CTS were 50.12 kPa for MN stiffness at the wrist, 1.91 for MN stiffness ratio, and grade 1 for HD color.SWE and HD color are good supportive tools in diagnosing and assessing severity in CTS.SWE and HD color demonstrated that MN in CTS was associated with increased stiffness and hypervascularity.

Summary Objective Several techniques have been practiced in the field of voice therapy to balance glottal pressures and improve glottal adduction. As such, Semioccluded Vocal Tract Exercises using narrow diameter straws have shown promise as a technique to increase glottal contact as reflected by Closed Quotients (CQ) measured from electroglottograph (EGG). This study explored the effect of nasal resistance (NR) technique, a manually induced NR approach, on glottal adduction in phononormal subjects as a possible new therapeutic technique to assist in glottal closure. Subjects Thirty-four vocally healthy adult females qualified for this study. All subjects were reportedly and perceptually normal. Methods Subjects were instructed to perform tasks including (a) steady modal phonation, (b) straw phonation, (c) humming, and (d) NR technique in a random order. EGG measures included calculation of the CQ and jitter during the mid-section of each stimuli for a minimum of 10 EGG cycles. One-way repeated measures ANOVAs and Bonferroni post-hoc comparisons were calculated to determine differences between each phonatory task for the outcome measures of CQ and jitter. Results Analyses revealed that the mean CQ was significantly different in all the tasks performed with humming (33.99%) at the lowest and NR technique at highest (43.88%). Though the CQ of straw phonation (40.53%) was higher than modal steady phonation (36.65%), jitter was found to be significantly lower for NR technique (0.23%). Jitter during straw phonation (0.43%) did not differ significantly from modal phonation (0.44%). Conclusion These results provide preliminary evidence that NR technique can increase CQ with lower levels of jitter compared to straw phonation using a narrow diameter of 2 mm. Future studies should expand enrollment to men, larger age ranges, and patients with hypofunctional symptoms (eg, Parkinson disease) to evaluate the potential benefits of NR as a therapeutic technique.

Supplemental digital content is available in the text., A growing number of studies have documented a wide variety of neurological manifestations associated with the novel SARS-CoV-2 (COVID-19). Of the available literature, cranial neuropathies and central nervous system disorders, such as encephalopathy and ischemic strokes, remain the predominant discussion. Limited investigations exist examining peripheral neuropathies of those with COVID-19. This case series discusses eight patients who tested positive for COVID-19 and presented with localized weakness after a prolonged course of mechanical ventilation (>21 days). We retrospectively reviewed all patients’ charts who received electrodiagnostic evaluation between March and November 2020 in the outpatient clinic or in the acute care hospital at the JFK Medical Center/JFK Johnson Rehabilitation Institute and Saint Peter's University Hospital of New Jersey. A total of eight COVID-19–positive patients were identified to have a clinical presentation of localized weakness after a prolonged course of mechanical ventilation. All patients were subsequently found to have a focal peripheral neuropathy of varying severity that was confirmed by electrodiagnostic testing. Patient demographics, clinical, and electrodiagnostic findings were documented. The findings of local weakness and focal peripheral neuropathies after diagnosis of COVID-19 raise significant questions regarding underlying pathophysiology and overall prognosis associated with COVID-19.

Signal-averaged sympathetic transduction of blood pressure (BP) is inversely related to resting muscle sympathetic nerve activity (MSNA) burst frequency in healthy cohorts. Whether this represents a physiological compensatory adaptation or a methodological limitation, remains unclear. The current analysis aimed to determine the contribution of methodological limitations by evaluating the dependency of MSNA transduction at different levels of absolute BP. Thirty-six healthy participants (27 ± 7 yr, 9 females) underwent resting measures of beat-to-beat heart rate, BP, and muscle sympathetic nerve activity (MSNA). Tertiles of mean arterial pressure (MAP) were computed for each participant to identify cardiac cycles occurring below, around, and above the MAP operating pressure (OP). Changes in hemodynamic variables were computed across 15 cardiac cycles within each MAP tertile to quantify sympathetic transduction. MAP increased irrespective of sympathetic activity when initiated below the OP, but with MSNA bursts provoking larger rises (3.0 ± 0.9 vs. 2.1 ± 0.7 mmHg

The aim of the study was to check the risk factors for subjects with motor conduction velocity (MCV) reduction of the ulnar nerve across the elbow without symptoms/signs of ulnar neuropathy at the elbow (UNE) using a database of a previous multicenter case–control study on UNE patients. From the previous database, we extracted all asymptomatic UNE (A-UNE) and matched for age and sex with a control and UNE groups with a ratio of 1:2. Anthropometric factors were measured and all participants filled in a questionnaire on demographic, lifestyle factors, and medical history. One-sample proportion test and univariate and multivariate logistic regression analyses were performed. We enrolled 64 A-UNE, 124 UNE, and 124 controls (mean age 53 years). There were more males with A-UNE than females (74.2%). The predominantly or exclusively concerned side of A-UNE was the right. Logistic regression showed that A-UNE was associated with diabetes (OR = 2.99, 95% CI = 1.21–7.39) and width of cubital groove (CGW) (OR = 0.89, 95% CI = 0.81–0.97). Risk factors for A-UNE are different from UNE. The prevalence of right side in A-UNE was not due to particular elbow postures. Diabetes is a risk factor, probably because MCV reduction of the ulnar nerve across the elbow was an early manifestation of asymptomatic polyneuropathy in diabetes. A-UNE is associated with narrow CGW as already demonstrated in UNE, even if the OR was higher in UNE than in A-UNE. Only future longitudinal studies will be able to check whether the A-UNE subjects develop symptoms and signs of true mononeuropathy with time.

Compression of ulnar nerve at the elbow is the second most common peripheral neuropathy of the upper extremity.Due to the lack of the gold diagnostic standard for ulnar nerve involvement at elbow level (UNE) and the lack of sufficient study in this field, we decided to evaluate patients with symptoms of this disease who have normal conventional electrodiagnostic study (EDX) in first evaluation.In this cross-sectional study, 18 persons were selected from patients who were referred to the clinic of Physical Medicine and Rehabilitation. If conventional EDX was normal, compound nerve action potential (CNAP) test (peak latency and amplitude) was carried out. Patients with normal conventional EDX but abnormal ulnar CNAP included to our study. After 3 months, if they had not been treated for ulnar neuropathy, they were reexamined by conventional EDX plus ulnar CNAP measurement.In total, 18 patients (11 females, 7 males) aged 28-58 years old (mean = 40.11) were analyzed in this study. After 3 months, 14 patients (77.8%) demonstrated parameter changes consistent with UNE in conventional EDX.Based on the results of this study, ulnar CNAP has diagnostic value in patients with symptoms of UNE who have normal routine EDX. Therefore, ulnar CNAP should be taken into account for early diagnosis of ulnar neuropathy when routine electrodiagnostic tests are normal.

Purpose The purpose of this study is to identify the extent to which various measurements of contacting parameters differ between children and adults during habitual range and overlap vocal frequency/intensity, using voice map–based assessment of noninvasive electroglottography (EGG). Method EGG voice maps were analyzed from 26 adults (22–45 years) and 22 children (4–8 years) during connected speech and vowel /a/ over the habitual range and the overlap vocal frequency/intensity from the voice range profile task on the vowel /a/. Mean and standard deviations of contact quotient by integration, normalized contacting speed, quotient of speed by integration, and cycle-rate sample entropy were obtained. Group differences were evaluated using the linear mixed model analysis for the habitual range connected speech and the vowel, whereas analysis of covariance was conducted for the overlap vocal frequency/intensity from the voice range profile task. Presence of a “knee” on the EGG wave shape was determined by visual inspection of the presence of convexity along the decontacting slope of the EGG pulse and the presence of the second derivative zero-crossing. Results The contact quotient by integration, normalized contacting speed, quotient of speed by integration, and cycle-rate sample entropy were significantly different in children compared to (a) adult males for habitual range and (b) adult males and adult females for the overlap vocal frequency/intensity. None of the children had a “knee” on the decontacting slope of the EGG slope. Conclusion EGG parameters of contact quotient by integration, normalized contacting speed, quotient of speed by integration, cycle-rate sample entropy, and absence of a “knee” on the decontacting slope characterize the wave shape differences between children and adults, whereas the normalized contacting speed, quotient of speed by integration, cycle-rate sample entropy, and presence of a “knee” on the downward pulse slope characterize the wave shape differences between adult males and adult females. Supplemental Material https://doi.org/10.23641/asha.15057345

Identifying the severity of carpal tunnel syndrome (CTS) is essential to providing appropriate therapeutic interventions. We developed and validated machine-learning (ML) models for classifying CTS severity. Here, 1037 CTS hands with 11 variables each were retrospectively analyzed. CTS was confirmed using electrodiagnosis, and its severity was classified into three grades: mild, moderate, and severe. The dataset was randomly split into a training (70%) and test (30%) set. A total of 507 mild, 276 moderate, and 254 severe CTS hands were included. Extreme gradient boosting (XGB) showed the highest external validation accuracy in the multi-class classification at 76.6% (95% confidence interval [CI] 71.2–81.5). XGB also had an optimal model training accuracy of 76.1%. Random forest (RF) and k-nearest neighbors had the second-highest external validation accuracy of 75.6% (95% CI 70.0–80.5). For the RF and XGB models, the numeric rating scale of pain was the most important variable, and body mass index was the second most important. The one-versus-rest classification yielded improved external validation accuracies for each severity grade compared with the multi-class classification (mild, 83.6%; moderate, 78.8%; severe, 90.9%). The CTS severity classification based on the ML model was validated and is readily applicable to aiding clinical evaluations.

INTRODUCTION/AIMS: Isolated injuries to the lateral cutaneous nerve of the calf (LCNC) branch of the common peroneal nerve can cause obscure chronic posterolateral knee and upper calf pain and sensory symptoms. Routine examination and electrodiagnostic testing do not detect them because the LCNC has no motor distribution and it is not interrogated by the typical peroneal nerve conduction study. There are only about 10 prior cases, thus scant physician awareness, so most LCNC injuries remain misdiagnosed or undiagnosed, hindering care. METHODS: We extracted pertinent records from seven patients with unexplained posterolateral knee/calf pain, six labeled as complex regional pain syndrome, to investigate for mononeuropathies. Patients were asked to outline their skin area with abnormal responses to pin self-examination independently. Three underwent an LCNC-specific electrodiagnostic study, and two had skin-biopsy epidermal innervation measured. Cadaver dissection of the posterior knee nerves helped identify potential entrapment sites. RESULTS: Initiating events included knee surgery (three), bracing (one), extensive kneeling (one), and other knee trauma. All pin-outlines included the published LCNC neurotome. One oftwo LCNC-specific electrodiagnostic studies revealed unilaterally absent potentials. Longitudinal, controlled skin biopsies documented profound LCNC-neurotome denervation then re-innervation contemporaneous with symptom recovery. Cadaver dissection identified the LCNC traversing through the dense fascia of the proximolateral gastrocnemius muscle insertion. DISCUSSION: Isolated LCNC mononeuropathy can cause unexplained posterolateral knee/calf pain syndromes. This series characterizes presentations and supports patient pin-mappings as a sensitive, globally available, low-cost diagnostic aid. Improved recognition may facilitate more rapid, accurate diagnosis and, thus, optimize management and improve outcomes.

INTRODUCTION/AIM There is currently insufficient clinical and epidemiological data concerning small fiber neuropathy (SFN). This research analyzes data from medical records to determine epidemiology, demographics, clinical characteristics and etiology of SFN. METHODS This is a retrospective, observational study of sequential patients diagnosed with definite SFN (typical clinical features, normal nerve conduction studies, abnormal epidermal nerve fiber density) from the end of November 2016 to the middle of July 2019 at the Cantonal Hospital Lucerne, central Switzerland. RESULTS A total of 84 patients (64.3% female) with a mean age of 54.7���y were analyzed. Symptoms had been present in patients for an average of 4.8���y when entering the study. A length dependent clinical pattern was seen in 79.8%. All patients had sensory discomfort. Etiology could not be determined in 35.7% of patients, who were diagnosed with idiopathic SFN; 34.5% of patients had an apparently autoimmune SFN, followed by14.3% of patients with metabolic causes. The estimated incidence was at least 4.4 cases/100.000 inhabitants/y. The minimum prevalence was 131.5 cases/100.000 inhabitants. DISCUSSION This study indicates significant incidence and prevalence rates of SFN in Switzerland. SFN can vary greatly in its symptoms and severity. Extensive work-up resulted in two thirds of the patients being assigned an etiological association. The largest group of patients could not be etiologically defined, underlining the importance of further research on etiologic identification. We expect increased awareness of the developing field of SFN.

Elsberg syndrome is a rare cause of lumbosacral radiculitis with concomitant thoracic and lumbosacral myelitis that can be seen after an acute or reactivated viral infection. After the initial coronavirus surge in New York City, a 68-year-old man developed progressive lower extremity weakness and a defined sensory level at the lower abdomen. He had highly elevated SARS-CoV-2 IgG antibodies despite an absence of preceding COVID-19 symptoms. Serial electrodiagnostic testing revealed absent lower extremity late responses, with otherwise normal distal sensorimotor conductions. Electromyography revealed active neurogenic changes and reduced motor unit recruitment in the L3-L4 myotomes. Treatment with methylprednisolone and intravenous immunoglobulin was followed by minimal clinical improvement but re-emergence of the lower extremity late responses on electrodiagnostic testing. We report here, to the best of our knowledge, the first case of suspected COVID-19-associated Elsberg syndrome, which expands the spectrum of neuromuscular manifestations associated with SARS-CoV-2 infection and sheds light on ways to approach diagnostic and treatment options for these patients.

The objectivity of physical and electrodiagnostic tests is limited in detecting carpal tunnel syndrome and its recurrence. Predicting the median nerve blood supply using superb microvascular imaging will allow exact diagnosis and a good follow-up system. The aims of this study include using superb microvascular imaging to correlate with electromyographic diagnosis, and to determine the impact of surgery on improvement in superb microvascular imaging.Between July of 2019 and January of 2020, 32 wrists of 21 patients were evaluated prospectively. After preoperative electrodiagnostic studies and vascular index measurement with superb microvascular imaging, open carpal tunnel release was performed by a single surgeon, and 3 months later standardized superb microvascular imaging was performed. Preoperative vascular indexes were compared with the mild, moderate, and severe electrodiagnostic study results. Preoperative and postoperative vascular index results were compared.The average of the preoperative and postoperative imaging groups was 2.77 and 1.48, respectively, and there was a statistically significant difference between the two groups (p0.05). Although no significant difference was found between preoperative and postoperative vascular index values in patients presenting with mild carpal tunnel syndrome (p0.05), there was a significant decrease in vascular index values in patients presenting with moderate and severe carpal tunnel syndrome after surgical decompression.Superb microvascular imaging is emerging as a groundbreaking, new, and reliable technique. Evaluation of the median nerve blood supply is a reliable method that would be helpful for early diagnosis, planning treatment, determining the severity of carpal tunnel syndrome, and postoperative follow-up.Diagnostic, IV.

Objective To evaluate the European Federation of Neurological Societies (EFNS)/Peripheral Nerve Society (PNS) diagnostic criteria for chronic inflammatory demyelinating polyneuropathy (CIDP) in a cohort of patients diagnosed and treated for CIDP in a tertiary university hospital. Methods In a monocentric retrospective study of 203 CIDP patients, diagnosed according to expert opinion, we evaluated the EFNS/PNS diagnostic criteria. Clinical course and nerve conduction studies (NCS) over 1 year from first referral were studied. Secondarily, we compared the clinical and paraclinical characteristics, including nerve ultrasound, of patients who failed with those who fulfilled the criteria in order to identify clinically relevant differences. Results At 1 year, 182 (89.7%) patients fulfilled the criteria (156/76.9% definite, 22/10.8% probable, and 4/2% possible). Twenty‐one (10.3%) patients did not because the electrodiagnostic criteria remained negative. These still showed signs of demyelination but did not reach the cut‐off values. They also presented typical, albeit less pronounced, multifocal nerve enlargement in ultrasonography. Mean disability at presentation and 1 year after was significantly lower. Most importantly, a relevant proportion of these patients also responded to therapy (6/21 = 28.6% vs. 82/182 = 45.3% of those fulfilling the criteria). Interpretation CIDP diagnosis could be established for 89.7% of patients over the course of 1 year using EFNS/PNS criteria. The remaining patients (10.3%) presented with milder disability, less accentuated demyelination, but otherwise similar characteristics and still considerable probability of treatment response. Failure to fulfill diagnostic criteria should not automatically preclude treatment. Nerve ultrasound should be considered as a complementary diagnostic tool to detect signs of inflammation in CIDP.

Background Critical illness polyneuropathy and myopathy (CIPNM) is a frequent neurological manifestation in patients with acute respiratory distress syndrome (ARDS) from coronavirus disease 2019 (COVID‐19) infection. CIPNM diagnosis is usually limited to clinical evaluation. We compared patients with ARDS from COVID‐19 and other aetiologies, in whom a neurophysiological evaluation for the detection of CIPNM was performed. The aim was to determine if there were any differences between these two groups in frequency of CINPM and outcome at discharge from the intensive care unit (ICU). Materials and Methods This was a single‐centre retrospective study performed on mechanically ventilated patients consecutively admitted (January 2016‐June 2020) to the ICU of Careggi Hospital, Florence, Italy, with ARDS of different aetiologies. Neurophysiological evaluation was performed on patients with stable ventilation parameters, but marked widespread hyposthenia (Medical Research Council score

Introduction Peripheral nerve hyperexcitability syndrome (PNHS) is characterized by muscle fasciculations and spasms. Nerve hyperexcitability and after-discharges can be observed in electrophysiological studies. Autoimmune mechanisms play a major role in the pathophysiology of primary PNHS. Methods We retrospectively conducted a case-control study recruiting patients with clinical and electrophysiological features of PNHS. Control patients were diagnosed with other neuronal or muscular diseases. Contactin-associated protein2 (CASPR2) and leucine-rich glioma-inactivated1 (LGI1) antibodies were examined. Results A total of 19 primary PNHS patients and 39 control patients were analyzed. The most common symptoms for the case group were fasciculations (11/19) and muscle spasms (13/19). Case group patients were likely to demonstrate electrodiagnostic findings of nerve hyperexcitability (17/19) and after-discharges in the tibial nerve (19/19). We found high prevalence of CASPR2 (9/19) and LGI1 (6/19) antibodies in the case group. Discussion Primary PNHS patients were likely to show after-discharges in the tibial nerve. The pathogenesis of PNHS is autoimmune CASPR2 and LGI1 antibodies are possible pathogenic antibodies for primary PNHS.

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.

Ever since their origin more than one half-century ago, microneurographic recordings of sympathetic nerve activity have significantly advanced our understanding of the generation and regulation of central sympathetic outflow in human health and disease. For example, it is now appreciated that a myriad of disease states exhibit chronic sympathetic overactivity, a significant predictor of cardiovascular morbidity and mortality. Although microneurographic recordings allow for the direct quantification of sympathetic outflow, they alone do not provide information with respect to the ensuing sympathetically mediated vasoconstriction and blood pressure (BP) response. Therefore, the study of vascular and/or BP responses to sympathetic outflow (i.e., sympathetic transduction) has now emerged as an area of growing interest within the field of neural cardiovascular control in human health and disease. To date, studies have primarily examined sympathetic transduction under two distinct paradigms: when reflexively evoking sympatho-excitation through the induction of a laboratory stressor (i.e., sympathetic transduction during stress) and/or following spontaneous bursts of sympathetic outflow occurring under resting conditions (i.e., sympathetic transduction at rest). The purpose of this brief review is to highlight how our physiological understanding of sympathetic transduction has been advanced by these studies and to evaluate the primary analytical techniques developed to study sympathetic transduction in humans. We also discuss the framework by which the assessment of sympathetic transduction during stress reflects a fundamentally different process relative to sympathetic transduction at rest and why findings from investigations using these different techniques should be interpreted as such and not necessarily be considered one and the same.

This update begins with myasthenia gravis and the roles of anti-agrin and cortactin antibodies. Regarding diagnosis, a report on repeated ice pack testing is highlighted as are several reports on the close correlation of electrodiagnostic testing with clinical features and the response to treatment. The incidence of head drop and associated clinical and ventilatory features are gleaned from a retrospective study. We also discuss a study that assessed the predominantly symmetric and conjugate ocular findings in MuSK-myasthenia gravis. Other topics that are covered include quality of life and preoperative risk. We then summarize the positive treatment trials of subcutaneous immunoglobulin and eculizumab. Turning to Lambert-Eaton Myasthenic Syndrome, we report on an epidemiologic study performed on the veteran affairs population, the results of the DAPPER study of 3, 4 diaminopyridine, and look to the future for other treatment options involving calcium gating modifiers.

Objectives Our primary goal was to evaluate the effect of stimulus duration on electrogustometry (EGM) thresholds. Additionally, we sought to evaluate any sex-related influences and compare the above results to those of taste strips. Design Electrogustometry thresholds of various stimulus durations (0.5, 1.0, 1.5 and 2.0 seconds) were measured in 212 non-smokers (age range: 10-80 years, divided into eight age-groups) without self-reported gustatory impairment. Furthermore, taste strips chemogustometry measurements in 132 participants were performed. Setting Tertiary referral medical centre. Participants 212 non-smokers, divided into eight age-groups participated in the study. Main outcome measures Electrogustometry thresholds and taste strips, duration of EGM stimuli. Results Electrogustometry thresholds increased progressively with age and with stimulus duration from 0.5 to 2 seconds. This pattern was consistent in all six anatomic areas, irrespective of sex. In contrast, when using chemogustometry, no age- or sex-related differences were observed. Conclusions Electrogustometry-threshold values increase progressively with age and with stimulus duration. Therefore, we recommend documenting stimulus duration in the future EGM recordings as it may significantly affect EGM amplitude threshold values.

OBJECTIVE: To test a novel technology for assessment of the volume conduction properties (VCPs) of the tongue. These properties are electrophysiological data that might reflect alterations in patients with tongue involvement. METHODS: Seven healthy individuals were self-measured. The depressor was placed on the surface of the anterior tongue. Directional differences of VCPs were determined with standard descriptive statistics. RESULTS: Conductivity in longitudinal direction was larger than in transverse direction at 16 (p

The use of spontaneous bursts of muscle sympathetic nerve activity (MSNA) to assess arterial baroreflex control of sympathetic nerve activity has seen increased utility in studies of both health and disease. However, methods used for analyzing spontaneous MSNA baroreflex sensitivity are highly variable across published studies. Therefore, we sought to comprehensively examine methods of producing linear regression slopes to quantify spontaneous MSNA baroreflex sensitivity in a large cohort of subjects ( n = 150) to support a standardized procedure for analysis that would allow for consistent and comparable results across laboratories. The primary results demonstrated that 1) consistency of linear regression slopes was considerably improved when the correlation coefficient was above −0.70, which is more stringent compared with commonly reported criterion of −0.50, 2) longer recording durations increased the percentage of linear regressions producing correlation coefficients above −0.70 (1 min = 15%, 2 min = 28%, 5 min = 53%, 10 min = 67%, P < 0.001) and reaching statistical significance (1 min = 40%, 2 min = 69%, 5 min = 78%, 10 min = 89%, P < 0.001), 3) correlation coefficients were improved with 3-mmHg versus 1-mmHg and 2-mmHg diastolic blood pressure (BP) bin size, and 4) linear regression slopes were reduced when the acquired BP signal was not properly aligned with the cardiac cycle triggering the burst of MSNA. In summary, these results support the use of baseline recording durations of 10 min, a correlation coefficient above −0.70 for reliable linear regressions, 3-mmHg bin size, and importance of properly time-aligning MSNA and diastolic BP. Together, these findings provide best practices for determining spontaneous MSNA baroreflex sensitivity under resting conditions for improved rigor and reproducibility of results.

Objective To collect preliminary data on the effects of mexiletine on cortical and axonal hyperexcitability in sporadic amyotrophic lateral sclerosis (ALS) in a phase 2 double-blind randomized controlled trial. Methods Twenty ALS subjects were randomized to placebo and mexiletine 300 mg or 600 mg daily for 4 weeks and assessed by transcranial magnetic stimulation and axonal excitability studies. The primary endpoint was change in resting motor threshold (RMT). Results RMT was unchanged with 4 weeks of mexiletine (combined active therapies) as compared to placebo, which showed a significant increase (p=0.039). Reductions of motor evoked potential (MEP) amplitude (p=0.013) and accommodation half-time (p=0.002), secondary outcome measures of cortical and axonal excitability, respectively, were also evident at 4 weeks on mexiletine. Conclusions The relative stabilization of RMT in the treated subjects was unexpected and could be attributed to unaccounted sources of error or chance. However, a possible alternative cause is neuromodulation preventing an increase. The change in MEP amplitude and accommodation half-time supports the reduction of cortical and axonal hyperexcitability with mexiletine. This article is protected by copyright. All rights reserved.

BACKGROUND Unilateral diaphragmatic paralysis (UDP) has major clinical and etiological implications and, therefore, is important to diagnose. Lung function tests and invasive transdiaphragmatic pressure (Pdi) measurements are widely used to this end but, contrary to phrenic nerve conduction study (NCS), they require volitional maneuvers and/or may be poorly tolerated by patients. The purpose of this study was to compare the diagnostic accuracy of Pdi and phrenic NCS for UDP. METHODS We retrospectively reviewed 28 patients with suspected UDP. The diagnosis established during a multidisciplinary meeting was the reference standard. RESULTS Phrenic NCS correlated well with Pdi (r = 0.82, P

BACKGROUND: Electrogastrography (EGG) is a noninvasive technique for the assessment of gastric myoelectrical activity using electrodes placed on the abdominal surface. Changes in gastric myoelectrical activity may be associated with diseases such as gastroparesis, functional dyspepsia, nausea, and recurrent vomiting. In Brazil, no studies to date have assessed gastric myoelectrical activity using multichannel EGG in healthy individuals. OBJECTIVE: To establish normal values of transcutaneous multichannel EGG in healthy Brazilian individuals. METHODS: This was a prospective study including 20 healthy individuals who underwent EGG. Recording was performed during two periods: a preprandial recording was performed for 30 minutes, and a postprandial recording was performed for 30 minutes after a soft-solid meal of 400 kcal (20 grams of proteins, 60 grams of carbohydrates, and 9 grams of fat). RESULTS: We assessed dominant frequency (DF) parameters, %DF distribution, the instability coefficient, and the power ratio (PR). A total of 20 individuals (11 women and 9 men) with a mean age of 39.5±7.4 years were included. Mean DF (95%CI) ranged from 2.4 to 3.1 cpm in the resting phase and 2.6 to 3.2 cpm in the postprandial period. The %DF in normogastria range was >70% in all healthy individuals. We identified that only one individual did not present a positive response to the test meal, and the other 19 individuals showed a PR greater than 1. The instability coefficient did not change significantly with meal intake. CONCLUSION: Multichannel EGG may be applied in future studies to evaluate gastric motility disorders in the Brazilian population.

A literature review was performed on the use of electrodiagnostic (EDX) tests including nerve conduction study, electromyography, exteroceptive reflex, blink reflex, and late response in the evaluation of patients with stiff person syndrome (SPS). A web survey was conducted to report the extent of EDX testing usage in the evaluation of SPS among laboratories accredited by the American Academy of Neuromuscular and Electrodiagnostic Medicine. Coactivation of selected agonist and antagonist muscles was performed in 5 healthy subjects to determine its specificity for SPS. Observation of continuous motor unit activity on electromyography and elicitation of exteroceptive reflexes by electric stimulation are informative in assisting a diagnosis of SPS, but further studies focusing on their sensitivities in diagnosing SPS and specificities in differentiating SPS from other movement disorders are needed. The value of EDX testing in SPS lies in ruling out other neuromuscular disorders.

Limited literature is available on stimulus induced after discharges (SIAD) in patients with peripheral nerve hyperexcitability (PNH). The aim of the study was to examine the diagnostic utility of SIAD in the diagnosis and monitoring of primary PNH disorders. In this retrospective study, we studied 26 patients who were admitted with a diagnosis of primary PNH to the department of Neurology from January 2013 to April 2019. Their clinical profile, immunological characteristics were extracted from the database and nerve conduction studies were relooked for the presence of SIAD. 76% of patients in the primary PNH cohort had SIAD with 90% of them being voltage-gated potassium channel complex antibody positive; predominantly against contactin-associated protein-2 antigen and rest being paraneoplastic. There was also resolution of SIAD following treatment indicating reversible hyperexcitability. SIAD is a sensitive marker for Primary PNH syndrome with monitoring and diagnostic implications.

Background The clinical characteristics of electrophysiological subtypes and prognostic factors of Mexican adults diagnosed with Guillain-Barre Syndrome (GBS) have not been described. Materials and methods A single center, ambispective, cohort study was performed (2015–2019). GBS was defined following the Asbury and Cornblath criteria. Electrodiagnosis was made according to Hadden criteria. Clinical, biochemical and electrodiagnostic parameters were described, compared and analyzed using a multivariate model. Only patients who completed a 3-month follow-up were included. Results 137 GBS patients (92 males; mean age 46.6 ± 16.6). 132 (96.3%) underwent an electrodiagnostic assessment. 68 (51.5%) were classified as axonal GBS, with further classified into two groups: acute motor axonal neuropathy (AMAN) 45.4%, and acute motor and sensory axonal neuropathy (AMSAN) 8,6%. The following characteristics were lower in the AMAN group: Medical Research Counsel sumscore (MRC) 30.1 ± 16.3 vs 36.4 ± 14.4, unilateral facial palsy 10% vs 25.9% and albuminocytologic dissociation 41.3% vs. 71.7%. Multivariate analysis found AMAN as an independent predictor of an unfavorable outcome OR: 3.34 (p = 0.03) Conclusions AMAN subtype is the most frequent presentation of GBS in Mexican adult patients and an independent predictor of inability to walk independently at 3 months after discharge.

ObjectiveTo improve myasthenia gravis (MG) autoantibody testing.MethodsMG serologic tests with confirmatory or refuting clinical–electrodiagnostic (EDX) testing and cancer evaluations were reviewed over 4 years (2012–2015). All patients had acetylcholine receptor–binding (AChR-Bi), modulating (AChR-Mo), and striational (STR) autoantibody testing, and negatives reflexed to muscle-specific kinase (MuSK). Thymoma and cancer occurrences were correlated with STR and reflexed glutamic acid decarboxylase 65 (GAD65), ganglionic acetylcholine receptor (α3), collapsin response mediating protein-5, and voltage-gated potassium channel complex autoantibodies.ResultsOf 433 samples tested, 133 (31%) met clinical–EDX criteria for MG. Best sensitivity (90%) occurred at AChR-Bi >0.02 nmol/L, leaving 14 negative (6 ocular MG, 7 generalized MG, 1 MuSK MG) with specificity 90% (31 false-positives). Using AChR-Mo antibodies (>20% loss), specificity was better (92%, 24 false-positives), but sensitivity dropped (85%). Specificity improved (95%) by testing AChR-Mo when AChR-Bi are positive, resulting in 45% reduction of false-positives (31–17), maintaining AChR-Bi 90% sensitivity. Cutoff values recommended by area under the curve analysis did not outperform this approach. AChR-Bi and AChR-Mo values were significantly higher in true-positives. CT evaluations in 121 MG samples revealed 16 thymomas. Historical or subsequent cancers occurred in 22. STR and reflexed autoantibodies were not more common in MG with thymoma or other cancers. Full-body CT (n = 34) was performed in those with STR and reflex autoantibody positivity, but without additional cancers found.ConclusionAccuracy of MG serologic testing is improved by reflexing AChR-Bi–positive cases to AChR-Mo. STR and other reflexed cancer evaluation autoantibodies did not provide value beyond standard CT chest imaging at the time of MG diagnosis. Diagnostic certainty is informed by AChR-Bi and AChR-Mo with higher values increasing specificity.

Background Changes in radiation therapy practice and cancer incidence bring into question prior evidence suggesting that radiation therapy predominantly injures the brachial plexus upper trunk, while tumor invasion typically injures the lower trunk. Methods We reviewed electrodiagnostic brachial plexopathy reports in cancer survivors for predominant trunk involvement, injury mechanism (tumor invasion vs radiation), and primary cancer location. Results Fifty-six cases of cancer-associated brachial plexopathy were identified. There was no relationship between injury mechanism and brachial plexus injury level. However, primary cancer location superior/inferior to the clavicle increased the odds of predominantly upper/lower trunk involvement by a factor of 60.0 (95% confidence interval: 7.9, 1401, respectively). Conclusions Cancers superior/inferior to the clavicle increase the likelihood of predominantly upper/lower trunk plexopathy, respectively, regardless plexus injury mechanism. These findings contrast with older work, possibly due to more precise radiation therapy techniques and increased incidence of radiosensitive head and neck cancers.

BACKGROUND Compound muscle action potential (CMAP) scan and MScanFit have been used to understand the consequences of denervation and reinnervation. This study aimed to monitor these parameters during Wallerian degeneration (WD) after acute nerve transections (ANT). METHODS Beginning after urgent surgery, CMAP scans were recorded at 1-2 day intervals in 12 patients with ANT of the ulnar or median nerves, by stimulating the distal stump (DS). Stimulus intensities (SI), steps, returners, and MScanFit were calculated. Studies were grouped according to the examination time after ANT. Results were compared with those of 27 controls. RESULTS CMAP amplitudes and MScanFit progressively declined, revealing a positive correlation with one another. SIs were higher in WD groups than controls. Steps appeared or disappeared in follow-up scans. The late WD group had higher returner% than the early WD and control groups. CONCLUSIONS MScanFit can monitor neuromuscular dysfunction during WD. SIs revealed excitability changes in DS.

Introduction In contrast to needle electromyography (EMG), surface EMG recordings are painless. It is of interest to develop methods to analyze surface EMG for diagnostic purposes. Methods Surface EMG interference pattern (SIP) recordings from the abductor pollicis brevis muscle of healthy subjects and subjects with amyotrophic lateral sclerosis (ALS) were analyzed by measuring root-mean-square (RMS) voltage, mean rectified voltage, form factor (FF), and the clustering index (CI). The FF vs SIP area plot was used for analysis. Results The SIP FF was increased and abnormal in ALS subjects, especially when SIP area was less than 200 mVms. Power regression showed a faster FF decline with SIP area in ALS patients than in healthy subjects. The CI and FF showed a strong correlation. Discussion FF is easy to calculate and demonstrates abnormalities in ALS patients.

Automatic sleep staging methods usually extract hand-crafted features or network trained features from signals recorded by polysomnography (PSG), and then estimate the stages by various classifiers. In this study, we propose a classification approach based on a hierarchical neural network to process multi-channel PSG signals for improving the performance of automatic five-class sleep staging. The proposed hierarchical network contains two stages: comprehensive feature learning stage and sequence learning stage. The first stage is used to obtain the feature matrix by fusing the hand-crafted features and network trained features. A multi-flow recurrent neural network (RNN) as the second stage is utilized to fully learn temporal information between sleep epochs and fine-tune the parameters in the first stage. The proposed model was evaluated by 147 full night recordings in a public sleep database, the Montreal Archive of Sleep Studies (MASS). The proposed approach can achieve the overall accuracy of 0.878, and the F1-score is 0.818. The results show that the approach can achieve better performance compared to the state-of-the-art methods. Ablation experiment and model analysis proved the effectiveness of different components of the proposed model. The proposed approach allows automatic sleep stage classification by multi-channel PSG signals with different criteria standards, signal characteristics, and epoch divisions, and it has the potential to exploit sleep information comprehensively.

Background Various techniques are described for proximal motor nerve conduction studies (NCSs). We investigated alternative reference electrode (E2) locations for accessory and axillary NCSs. Methods Multi-channel recordings were made from trapezius or deltoid referred to different sites, and from those sites referred to a remote electrode. Responses were compared using grouped statistics, and correlation analysis. Results For accessory NCSs, all belly:E2 montages showed comparable responses but axillary NCSs were more variable. Low amplitude contamination was seen at the sternum and contralateral acromion but greater distortion using other potential E2 sites. In both accessory and axillary studies, the ipsilateral acromion showed moderate activity, which correlated with the belly:remote response. Conclusions Variation in E2 electrode sites may significantly distort the measured compound muscle action potential (CMAP). For accessory and axillary NCS, a sternal reference has favorable characteristics. Other sites, such as ipsilateral acromion or deltoid insertion, may not yield a representative CMAP.

ObjectiveTo investigate the clinicopathologic features of eosinophilic granulomatosis with polyangiitis (EGPA)–associated neuropathy with a focus on the presence or absence of anti-neutrophil cytoplasmic antibodies (ANCAs).MethodsWe examined the clinical features and pathologic findings of sural nerve biopsy specimens from 82 patients with EGPA-associated neuropathy. Of these patients, 32.9% were myeloperoxidase (MPO)-ANCA positive, and 67.1% were MPO-ANCA negative. PR3-ANCA was negative in all of 78 examined patients.ResultsUpper limb symptoms were more frequently reported as initial neuropathic manifestations in the MPO-ANCA–positive group than in the MPO-ANCA–negative group (44.4% vs 14.6%, p < 0.01). The serum levels of C-reactive protein were significantly higher in the MPO-ANCA–positive group than in the MPO-ANCA–negative group (p < 0.05). Sural nerve biopsy specimens showed findings suggestive of vasculitis (i.e., destruction of vascular structures) in epineurial vessels; these results were seen more frequently in the MPO-ANCA–positive group than in the MPO-ANCA–negative group (p < 0.0001). Conversely, the numbers of eosinophils in the lumen of the epineurial vessels (p < 0.01) and epineurial vessels occluded by intraluminal eosinophils (p < 0.05) were higher in the MPO-ANCA–negative group than in the MPO-ANCA–positive group. Furthermore, the incidence of eosinophil infiltration in the endoneurium was higher in the MPO-ANCA–negative group than in the MPO-ANCA–positive group (p < 0.01).ConclusionsThis study suggests that the pathogenesis of EGPA comprises at least 2 distinct mechanisms: ANCA-associated vasculitis resulting in ischemic effects and inflammation, which is prominent in MPO-ANCA–positive patients, and eosinophil-associated vascular occlusion leading to ischemia and eosinophil-associated tissue damage, which is conspicuous in MPO-ANCA–negative patients.

Objective The prognostic value of nerve excitability tests (NET) and electroneurography (ENoG) for recurrent peripheral facial palsy is poorly understood. This study aimed to evaluate the association between NET/ENoG results for the current palsy and recovery. Methods We extracted data on patients who were referred to our hospital (2005–2017). Adult patients with recurrent peripheral facial palsy who underwent NET and ENoG within 3 weeks of onset were retrospectively analyzed (n = 26). Favorable recovery was defined as the achievement of either House–Brackmann grade I/II or the same level of facial movement as before the current palsy. We evaluated the predictive NET/ENoG results by making comparison between the favorable recovery group and the unfavorable recovery group, which were subdivided based on the length of time after the previous palsy. Results In terms of patients with a >4-year recurrent interval, 8 out of 12 patients achieved favorable recovery. Compared to the favorable recovery group, the unfavorable recovery group had significantly higher NET results (9.03 mA vs. −1.08 mA, p = 0.017). Also, the unfavorable recovery group had significantly higher NET results in patients with a >2-year recurrent interval (9.03 mA vs. 1.06 mA, p = 0.036). However, other test results (NET in ≤4-year recurrent interval/all 26 patients, and ENoG in >4-year recurrent interval/≤4-year recurrent interval/all 26 patients) did not differ significantly between patients with favorable and unfavorable recovery. Conclusion NET might be a useful prediction method in patients with at least a few years interval between the previous and the current palsy.

INTRODUCTION Brody myopathy (BM) is a recessive condition caused by mutations in the ATP2A1 gene and usually induces impaired muscle relaxation during and after exercise. Diagnosis relies on needle electromyography showing electrical silence, muscle biopsy with decreased sarcoplasmic reticulum calcium adenosine triphosphatase activity, and genetic analysis. Electrodiagnostic functional analyses are useful in the diagnosis of channelopathies, and thus may be impaired in BM. METHODS We performed exercise tests and repetitive nerve stimulation (RNS; 10 supramaximal stimuli at 3 Hz) in 10 patients with BM. RESULTS All participants showed incremental responses on RNS. Compound muscle action potential amplitude was increased and duration was decreased, especially in the ulnar nerve (+30.2 ± 7.1% and - 30.3 ± 2.8%, respectively; both P

Background Increment of compound muscle action potential amplitude is a diagnostic hallmark of Lambert‐Eaton myasthenic syndrome (LEMS). Making a diagnosis can be challenging, therefore, a proper cutoff for abnormal increment is highly relevant for improved recognition of this rare disease. Methods We determined the sensitivity and specificity of 60% and 100% cutoff values in all consecutive patients who underwent increment testing in our hospital from 1999 to 2016. Results We included 156 patients, 63 with LEMS and 93 without LEMS. Sensitivity of a 60% cutoff for increment testing was 77.8% (95% confidence interval 65.5%–87.3%) and 58.7% (45.6%–71.0%) for 100%. Specificity was 98.9% (94.2%–100%) and 100% (96.1%–100%) using a threshold of 60% and 100%, respectively. Conclusions Lowering the cutoff value for abnormal increment to 60% greatly increases sensitivity to diagnose LEMS without an overt loss in specificity.

Malignant lymphocytic infiltration of the central nervous system (CNS) and peripheral nervous system (PNS) is diagnostically challenging and informs treatment and prognosis.We describe the clinical course of a 49-year-old man with CNS and PNS relapse of mantle cell lymphoma and the diagnostic modalities that enabled the diagnosis of neurolymphomatosis.This clinical phenotype reinforces previously reported presentations of neurolymphomatosis and the ability of multimodal diagnostics, when combined with clinical suspicion phenotype, to enable diagnosis of malignant lymphocytic infiltration of the CNS and PNS.

ObjectiveCompare high-resolution ultrasound (HRUS) and electrodiagnostic examination (EDX) in the diagnostic workup of patients with scapulae alatae.Methods27 patients with scapulae alatae and 41 healthy subjects (HS) and underwent a standardized clinical examination (CEX), EDX and HRUS. We measured the thickness of the serratus anterior (SER), rhomboid major and trapezius muscles and the diameter of the long thoracic (LTN), dorsal scapular and spinal accessory nerves (SAN).ResultsTwenty patients showed medial winging and six patients showed lateral winging on CEX. One patient had both lateral and medial winging. In patients with medial winging, the SER muscle was thinner and the LTN diameter was larger on the symptomatic side compared with the asymptomatic side and with the dominant side in HS. In this group, both EDX and HRUS detected abnormalities of SER muscle/ LTN with sensitivity of 65%, and with specificity of 100% and 57%, respectively.EDX and HRUS detected abnormalities of the trapezius muscle/ SAN with sensitivity of 60% and 40%, and specificity of 91%, and 86 % a, respectively. There was no significant difference between the two methods.ConclusionHRUS can contribute to the diagnostic workup of scapulae alatae by demonstrating atrophy of muscles and enlargement in nerve diameter.SignificanceHRUS supplements EDX in the diagnostic workup of scapulae alatae.KeywordsScapulae alataeScapular wingingLong thoracic nerveSerratus anterior musclebrachial plexusspinal accessory nerve

Background & aimsInvasive gastric electrical mapping has revealed spatial abnormalities of the slow wave in subjects with gastroparesis and functional gastrointestinal disorders. Cutaneous high-resolution electrogastrography (HR-EGG) is a non-invasive method that can detect spatial features of the gastric slow wave. We performed HR-EGG in subjects with active foregut symptoms to evaluate associations between gastric myoelectric abnormalities, symptoms (based on a validated questionnaire), and gastric emptying.MethodsWe performed a case-control study of 32 subjects, including 7 healthy individuals (controls), 7 subjects with functional dyspepsia and normal gastric emptying, and 18 subjects with gastroparesis, from a tertiary care program. All subjects were assessed by computed tomography imaging of the abdomen and HR-EGG and completed the PAGI-SYM questionnaire on foregut symptoms, which includes the gastroparesis cardinal symptom index. We performed volume reconstruction of the torso and stomach from computed tomography images to guide accurate placement of the HR-EGG array.ResultsSpatial slow-wave abnormalities were detected in 44% of subjects with foregut symptoms. Moreover, subjects with a higher percentage of slow waves with aberrant propagation direction had a higher total gastroparesis cardinal symptom index score (r= 0.56; P < .001) and more severe abdominal pain (r= 0.46; P= .009). We found no correlation between symptoms and traditional EGG parameters.ConclusionsIn case-control study, we found that the genesis of symptoms of functional dyspepsia and gastroparesis is likely multifactorial, including possible contribution from gastric myoelectric dysfunction. Abnormal spatial parameters, detected by cutaneous HR-EGG, correlated with severity of upper gastrointestinal symptoms, regardless of gastric emptying. This noninvasive, repeatable approach might be used to identify patients for whom gastric myoelectric dysfunction contributes to functional dyspepsia and gastroparesis.

Muscle sympathetic nerve activity (MSNA) can be acquired from humans using the technique of microneurography. The resulting integrated neurogram displays pulse-synchronous bursts of sympathetic activity, which undergoes processing for standard MSNA metrics including burst frequency, height, area, incidence, total activity, and latency. The procedure for detecting bursts of MSNA and calculating burst metrics is tedious and differs widely among laboratories worldwide. We sought to develop an open-source, cross-platform web application that provides a standardized approach for burst identification and a tool to increase research reproducibility for those measuring MSNA. We compared the performance of this web application against a manual scoring approach under conditions of rest, chemoreflex activation (n = 9, 20-min isocapnic hypoxia), and metaboreflex activation (n = 13, 2-min isometric handgrip exercise and 4-min postexercise circulatory occlusion). The intraclass correlation coefficient (ICC) indicated good to strong agreement between scoring approaches for burst frequency (ICC = 0.92-0.99), incidence (ICC = 0.94-0.99), height (ICC = 0.76-0.88), total activity (ICC = 0.85-0.99), and latency (ICC = 0.97-0.99). Agreement with burst area was poor to moderate (ICC = 0.04-0.67) but changes in burst area were similar with chemoreflex and metaboreflex activation. Scoring using the web application was highly efficient and provided data visualization tools that expedited data processing and the analysis of MSNA. We recommend the open-source web application be adopted by the community for the analysis of MSNA.NEW & NOTEWORTHY The basic analysis of muscle sympathetic nerve activity (MSNA) requires the identification of pulse-synchronous bursts from the integrated neurogram before standard MSNA metrics can be quantified. This process is a time-consuming task requiring an experienced microneurographer to visually identify and manually label bursts. We developed an open-source, cross-platform application permitting a standardized approach for sympathetic burst identification and present the performance of this application against a manual scorer under basal conditions and during sympathoexcitatory stresses.

Background The acute presentation of carpal tunnel syndrome (CTS) is rare. When symptoms start acutely with no obvious causes, ultrasound (US) imaging may provide clues to the etiology. Objective This study describes the clinical, electromyographic, and US findings in 25 patients presenting with acute CTS (ACTS). Methods In this analysis, of the patients referred for electrodiagnostic confirmation of CTS over the past decade, 25 had an acute onset of symptoms. All patients underwent EMG/NCV and US of the median nerve at the carpal tunnel and forearm. Results Of the 25 cases with ACTS, 5 (20%) had bilateral involvement leading to the total hands studied to 30. In 14 (56%) patients, an inciting event was identified as a possible cause of ACTS. In 11 (44%) patients without an antecedent event, 7 (64%) had a persistent median artery (PMA) detected by US. Electrodiagnostic studies showed prolonged distal motor latency with normal motor conduction velocity proximal to the carpal tunnel in 24 (80%) of 30 hands, 6 (20%) hands showed absent compound muscle action potential s over the abductor pollicis brevis (APB), and 22 (73%) hands had absent sensory potentials. Denervation changes were seen in the APB in 13 (43%) hands, and motor unit potentials were absent in 6 (20%) hands. Sixteen (64%) patients underwent a carpal tunnel release for severe symptoms. Conclusion CTS may present acutely without a clinically identifiable cause. US complements electrodiagnostic studies and is particularly useful when electrodiagnostic tests are non-diagnostic (due to absent compound muscle and sensory action potentials). US may also provide clues to the underlying cause.

Objective To establish length of the affected nerve segment (LANS) in ulnar neuropathy at the elbow (UNE). Methods In a group of our previously reported UNE patients we identified 2-cm segments with reduced motor nerve conduction velocity (MNCV) on electrodiagnostic (EDx) studies and increased nerve cross-sectional areas (CSA) on ultrasonographic (US) studies. LANS was obtained by summation of these abnormal 2-cm segments separately for each approach. We also studied effect of selected independent parameters on LANS. Results Altogether we studied 189 patients (194 arms). Mean (SD) LANS determined in 171 arms with reduced ulnar MNCV was 4.15 (1.89) cm, and was similar (p = 0. 21) to LANS obtained in 147 arms with increased CSA 4.46 (2.29) cm. Longer LANS were found in right arms, clinically severe UNE, axonal UNE and UNE due to entrapment. The most commonly affected 6 cm segment included 89% of abnormal 2-cm segments, with 50% of included 2-cm segments being normal. By contrast, the whole 10 cm segment included all abnormal 2-cm segments, with 66% of included segments being normal. Conclusions In UNE both EDx and US studies revealed average LANS of around 4 cm. LANS was longer in more severe UNE. Significance LANS needs to be taken into account in discussion of the mechanisms of UNE and approach to EDx diagnosis of UNE, particularly length of the segment used in nerve conduction studies across the elbow.

BACKGROUND: It has been observed that patients with carpal tunnel syndrome (CTS) who also experience emotional distress, depression, or anxiety report more severe symptoms. As patients’ own perspectives about their health increasingly are guiding treatment decisions, it seems important to study the simultaneous association of psychological distress and neuropathology with hand disability in patients who have CTS, as this may help prioritize and sequence management steps. QUESTIONS/PURPOSES: What are the relationships among validated scores for (1) depression, (2) anxiety, (3) pain catastrophizing, and (4) nerve electrodiagnostic severity with measures of hand disability in patients with confirmed CTS? METHODS: Between 2017 and 2019, we evaluated 116 patients for CTS in a referral urban hospital in Mashhad, Iran. Of those, we considered 85% (99) as potentially eligible by considering the following Electromyography-Nerve Conduction Study (EMG-NCS) diagnostic criteria: sensory latency ≥ 3.5 Ms, median-ulnar latency difference ≥ 0.5 Ms, motor latency ≥ 4.2 Ms, and abnormal EMG findings in the opponens pollicis muscle (neurogenic motor unit action potentials, positive sharp waves, or fibrillation). A further 13% (15 of 116) were excluded because of nonidiopathic CTS and prior surgery, and another 12% (14 of 116) were lost because of incomplete datasets, leaving 60% (70 of 116) for final inclusion in this cross-sectional study. In all, 89% of patients were women with total mean age of 47 years. We measured depression and anxiety using the Hospital Anxiety and Depression Scale (HADS) questionnaire (scored from 0 to 21, with a minimum clinically important difference [MCID] of 1.7 points), and we evaluated patients’ state of mind regarding pain using the Pain Catastrophizing Scale (PCS) (scored from 0 to 52). Higher scores on these questionnaires represent more distress and pain catastrophizing. Hand disability was assessed with Quick Disabilities of the Arm, Shoulder, and Hand (QuickDASH) questionnaire outcomes (scored from 0 [no disability] to 100 [most severe disability]; MCID of 15 points), Likert pain score (from 0 to 10), and grip/pinch dynamometry results. Correlational analyses were conducted once among HADS and PCS scores and again among EMG-NCS indices with pain and disability variables to answer our first, third, and fourth questions, respectively. Regression analysis was performed to assess the percentage of variance in QuickDASH and pain severity, which could be explained by psychological and electrodiagnostic factors. We did not include grip and pinch in our multivariable model (regression analysis) as dependent variables because they did not correlate with any of psychological or EMG-NCS variables (all p values > 0.1). Significance was set at p < 0.05. RESULTS: Correlational analysis showed that the scores of all three psychological questionnaires correlated with the QuickDASH score (r = 0.50, 0.42, and 0.53 for HADS-A, HADS-D, and PCS, respectively; p < 0.001 for all three), while EMG-NCS parameters had no correlation with QuickDASH and pain scores. We also found that 37% of the variance in QuickDASH score can be explained by HADS and PCS scores (r(2) = 0.37; p < 0.001). CONCLUSION: Evaluation and treatment of psychological distress before deciding on elective surgery for CTS is important because patient-reported disability—often used as a factor in surgical decision-making—is substantially correlated with emotional distress. Future prospective, controlled studies on this topic are recommended; ideally, these should evaluate psychological interventions specifically to ascertain whether they improve patients’ ratings of hand disability. LEVEL OF EVIDENCE: Level III, prognostic study.

INTRODUCTION/AIMS Neurophysiological patterns in patients with foodborne botulism are rarely described after the acute phase. We report data from a large Italian outbreak of botulism, with patients evaluated at different timepoints after poisoning. METHODS Eighteen male patients (mean age 47 ± 8.4 y) underwent 22 clinical and neurophysiological evaluations (4 patients were re-evaluated). The resting compound muscle action potential (CMAP) amplitude, postexercise CMAP amplitude, CMAP change after high-frequency (50 Hz) repetitive nerve stimulation (HFRNS), and motor unit action potentials (MUAPs) were assessed in the acute (4-8 days after poisoning; 5 patients), early post-acute (32-39 days after poisoning; 5 patients), and late post-acute (66-80 days after poisoning; 12 patients) phases. RESULTS In the acute, early post-acute, and late post-acute phases, respectively, reduced CMAP amplitudes were found in 100%, 20%, and 17% of patients; abnormal postexercise CMAP facilitation was observed in 100%, 40%, and 0% of patients; and pathological incremental responses to HFRNS were found in 80%, 50%, and 8% of patients. Baseline CMAP amplitudes, postexercise CMAP facilitation, and CMAP increases in response to HFRNS differed significantly between the acute and post-acute phases. Small MUAPs were found in 100% of patients in the acute and early post-acute phases and in 50% of patients in the late post-acute phase. DISCUSSION The neurophysiological findings of foodborne botulism vary considerably according to the evaluation time point. In the post-acute phase, different neurophysiological techniques must be applied to support a diagnosis of botulism.