Repeatability of innervation zone identification in the external anal sphincter muscle

Knowledge of the distribution of the innervation zones (IZs) of the external anal sphincter (EAS) may be useful for preventing anal sphincter incompetence during vaginal delivery. A method proposed for the automatic estimation of the distribution of IZs of EAS from high-density surface electromyography (EMG) was evaluated for repeatability in continent volunteers. Methods: In 13 healthy female subjects (age: 35 11 years) surface EMG signals were acquired using an anal probe with three circumferential electrode arrays (of 16 contacts each) at different depths within the anal canal (15mm distance between the centers of adjacent arrays), during four independent experimental sessions. Three maximal voluntary contractions (MVCs) of 10 sec were performed for each session for a total of 12 contractions per subject. Repeatability of the estimation of the distribution of IZ was tested by evaluating the coefficient of multiple correlations (CMC) between the IZ distributions estimated from the signals recorded from each subject. Results: A high repeatability (CMC > 0.8) was found comparing IZ distributions estimated from signals recorded by each array within the same session. A slightly lower value was obtained considering signals recorded during different sessions (CMC > 0.7), but a higher value (CMC > 0.8) was obtained after aligning the estimated IZ distributions. The realignment compensates for the operator's error in repositioning the probe in the same position during different sessions. Conclusion: This result justifies clinical studies using high-density surface EMG in routine examinations, providing information about IZs of EAS and assessing the possibilities of preventing neuronal trauma during vaginal delivery

Wearable Inertial Devices in Duchenne Muscular Dystrophy: A Scoping Review

In clinical practice and research, innovative digital technologies have been proposed for the characterization of neuromuscular and movement disorders through objective measures. Among these, wearable devices prove to be a suitable solution for tele-monitoring, tele-rehabilitation, and daily activities monitoring. Inertial Measurement Units (IMUs) are low-cost, compact, and easy-to-use wearable devices that evaluate kinematics during different movements. Kinematic variables could support the clinical evaluation of the progression of some neuromuscular diseases and could be used as outcome measures. The current review describes the use of IMUs for the biomechanical assessment of meaningful outcome measures in individuals affected by Duchenne muscular dystrophy (DMD). The PRISMA methodology was used and the search was conducted in different databases (Scopus, Web of Science, PubMed). A total of 23 articles were examined and classified according to year of publication, ambulatory/non-ambulatory subjects, and IMU positioning on human body. The analysis points out the recent regulatory identification of Stride Velocity 95th Centile as a new endpoint in therapeutic DMD trials when measured continuously from a wearable device, while only a few studies proposed the use of IMUs in non-ambulatory patients. Clinical recognition of reliable and accurate outcome measures for the upper body is still a challeng

Repeatability of innervation zone identification in the external anal sphincter muscle

Knowledge of the distribution of the innervation zones (IZs) of the external anal sphincter (EAS) may be useful for preventing anal sphincter incompetence during vaginal delivery. A method proposed for the automatic estimation of the distribution of IZs of EAS from high-density surface electromyography (EMG) was evaluated for repeatability in continent volunteers. Methods: In 13 healthy female subjects (age: 35 11 years) surface EMG signals were acquired using an anal probe with three circumferential electrode arrays (of 16 contacts each) at different depths within the anal canal (15mm distance between the centers of adjacent arrays), during four independent experimental sessions. Three maximal voluntary contractions (MVCs) of 10 sec were performed for each session for a total of 12 contractions per subject. Repeatability of the estimation of the distribution of IZ was tested by evaluating the coefficient of multiple correlations (CMC) between the IZ distributions estimated from the signals recorded from each subject. Results: A high repeatability (CMC > 0.8) was found comparing IZ distributions estimated from signals recorded by each array within the same session. A slightly lower value was obtained considering signals recorded during different sessions (CMC > 0.7), but a higher value (CMC > 0.8) was obtained after aligning the estimated IZ distributions. The realignment compensates for the operator’s error in repositioning the probe in the same position during different sessions. Conclusion: This result justifies clinical studies using high-density surface EMG in routine examinations, providing information about IZs of EAS and assessing the possibilities of preventing neuronal trauma during vaginal delivery

A muscle synergy-based method to estimate muscle activation patterns of children with cerebral palsy using data collected from typically developing children

Preparing children with cerebral palsy prior to gait analysis may be a challenging and time-intensive task, especially when large number of sensors are involved. Collecting minimum number of electromyograms (EMG) and yet providing adequate information for clinical assessment might improve clinical workflow. The main goal of this study was to develop a method to estimate activation patterns of lower limb muscles from EMG measured from a small set of muscles in children with cerebral palsy. We developed and implemented a muscle synergy extrapolation method able to estimate the full set of lower limbs muscle activation patterns from only three experimentally measured EMG. Specifically, we extracted a set of hybrid muscle synergies from muscle activation patterns of children with cerebral palsy and their healthy counterparts. Next, those muscle synergies were used to estimate activation patterns of muscles, which were not initially measured in children with cerebral palsy. Two best combinations with three (medial gastrocnemius, semi membranous, and vastus lateralis) and four (lateral gastrocnemius, semi membranous, sartorius, and vastus medialis) experimental EMG were able to estimate the full set of 10 muscle activation patterns with mean (+/- standard deviation) variance accounted for of 79.93 (+/- 9.64)% and 79.15 (+/- 6.40)%, respectively, using only three muscle synergies. In conclusion, muscle activation patterns of unmeasured muscles in children with cerebral palsy can be estimated from EMG measured from three to four muscles using our muscle synergy extrapolation method. In the future, the proposed muscle synergy-based method could be employed in gait clinics to minimise the required preparation time

Psychopharmacological Treatments for Mental Disorders in Patients with Neuromuscular Diseases: A Scoping Review

Mental disorders are observed in neuromuscular diseases, especially now that patients are living longer. Psychiatric symptoms may be severe and psychopharmacological treatments may be required. However, very little is known about pharmacotherapy in these conditions. We aimed to summarize the current knowledge on the use of psychopharmacological treatments for mental disorders in patients living with a neuromuscular disease. A scoping review was performed using the methodology of the Joanna Briggs Institute. Four databases were searched from January 2000 to July 2021. Articles were screened based on titles and abstracts. Full-text papers published in peer-reviewed journals in English were selected. Twenty-six articles met eligibility criteria, all being case reports/series focusing on the psychopharmacological control of psychiatric symptoms for the following conditions: myasthenia gravis (n = 11), Duchenne (n = 5) and Becker (n = 3) muscular dystrophy, mitochondrial disorders (n = 3), glycogen storage disease (n = 1), myotonic dystrophy (n = 1), hyperkalemic periodic paralysis (n = 1), and congenital myasthenic syndrome (n = 1). None of the articles provided details on the decision-making process to choose a specific drug/regimen or on follow-up strategies to monitor safety and efficacy. Larger studies showing real-world data would be required to guide consensus-based recommendations, thus improving current standards of care and, ultimately, the quality of life of patients and their families

Assessing cognitive function in neuromuscular diseases: A pilot study in a sample of children and adolescents

Central nervous system (CNS) involvement has been variously studied in pediatric neuromuscular disorders (NMDs). The primary goal of this study was to assess cognitive functioning in NMDs, and secondary aims were to investigate possible associations of cognitive impairment with motor impairment, neurodevelopmental delay, and genotype. This was a cross-sectional study of 43 pediatric patients, affected by six NMDs. Myotonic dystrophy type 1 (DM1) and glycogen storage disease type 2 (GSD2) patients had a delay on the Bayley-III scales. On Wechsler scales, DMD and DM1 patients showed lower FSIQ scores, with an intellectual disability (ID) in 27% and 50%, respectively. FSIQ was normal in Becker muscular dystrophy (BMD), GSD2, and hereditary motor sensory neuropathy (HMSN) patients, while higher individual scores were found in the spinal muscular atrophy (SMA) group. In the DM1 cohort, lower FSIQ correlated with worse motor performance (ρ = 0.84, p < 0.05), and delayed speech acquisition was associated with ID (p = 0.048), with worse cognitive impairment in the congenital than in the infantile form (p = 0.04). This study provides further evidence of CNS in some NMDs and reinforces the need to include cognitive assessment in protocols of care of selected pediatric NMDs

Creating the cultures of the future: cultural strategy, policy and institutions in Gramsci. Part three: Is there a theory of cultural policy in Gramsci’s prison notebooks?

In this article, I argue that Gramsci’s prison notes on questions of cultural strategy, policy and institutions, which have so far been largely overlooked by scholars, provide further analytical insights to those offered by his more general concepts. Together they enrich the theoretical underpinnings for critical frameworks of analysis as well as for radical practices of cultural strategy, cultural policy-making and cultural organisation. On the basis of a detailed analysis of these notes, I then answer the question of whether they amount to a theory of cultural policy