Electrical Stimulation-Based Twitch Exercise Suppresses Progression of Immobilization-Induced Muscle Fibrosis via Downregulation of PGC-1?/VEGF Pathway.

This study aimed to determine whether electrical stimulation-based twitch exercise is effective in inhibiting the progression of immobilization-induced muscle fibrosis. 19 Wistar rats were randomly divided into a control group (n=6), an immobilization group (n=6; with immobilization only), and a Belt group (n=7; with immobilization and twitch exercise through the belt electrode device, beginning 2 weeks after immobilization). The bilateral soleus muscles were harvested after the experimental period. The right soleus muscles were used for histological analysis, and the left soleus muscles were used for biochemical and molecular biological analysis. As a result, in the picrosirius red images, the perimysium and endomysium were thicker in both the immobilization and Belt groups compared to the control group. However, the perimysium and endomysium thickening were suppressed in the Belt group. The hydroxyproline content and alpha-SMA, TGF-beta1, and HIF-1alpha mRNA expressions were significantly higher in the immobilization and belt groups than in the control group. These expressions were significantly lower in the Belt group than in the immobilization group. The capillary-to-myofiber ratio and the mRNA expressions of VEGF and PGC-1alpha were significantly lower in the immobilization and belt groups than in the control group, these were significantly higher in the Belt group than in the immobilization group. From these results, Electrical stimulation-based twitch exercise using the belt electrode device may prevent the progression of immobilization-induced muscle fibrosis caused by downregulating PGC-1alpha/VEGF pathway, we surmised that this intervention strategy might be effective against the progression of muscle contracture. Keywords: Immobilization, Skeletal muscle, Fibrosis, Electrical stimulation-based twitch exercise, PGC-1alpha/VEGF pathway.

[Peripheral Nerve and Muscle Disorders Associated with Sjögren's Syndrome].

Sjögren's syndrome is often accompanied by various neurological complications, among which peripheral neuropathy is the most common. A variety of clinical phenotypes of peripheral neuropathy, including axonal polyneuropathy and sensory ataxic neuropathy are reported in the literature. We present an overview of the pathophysiology and differential diagnosis of each phenotype. Immunotherapy using corticosteroids and high-dose intravenous immunoglobulin therapy tends to elicit varied therapeutic responses depending on the peripheral neuropathy phenotype. We also discuss myositis, a possible complication of Sjögren's syndrome.

Type selective ablation of postnatal slow and fast fatigue-resistant motor neurons in mice induces late onset kinetic and postural tremor following fiber-type transition and myopathy.

Animals on Earth need to hold postures and execute a series of movements under gravity and atmospheric pressure. VAChT-Cre is a transgenic Cre driver mouse line that expresses Cre recombinase selectively in motor neurons of S-type (slow-twitch fatigue-resistant) and FR-type (fast-twitch fatigue-resistant). Sequential motor unit recruitment is a fundamental principle for fine and smooth locomotion; smaller-diameter motor neurons (S-type, FR-type) first contract low-intensity oxidative type I and type IIa muscle fibers, and thereafter larger-diameter motor neurons (FInt-type, FF-type) are recruited to contract high-intensity glycolytic type IIx and type IIb muscle fibers. To selectively eliminate S- and FR-type motor neurons, VAChT-Cre mice were crossbred with NSE-DTA mice in which the cytotoxic diphtheria toxin A fragment (DTA) was expressed in Cre-expressing neurons. The VAChT-Cre;NSE-DTA mice were born normally but progressively manifested various characteristics, including body weight loss, kyphosis, kinetic and postural tremor, and muscular atrophy. The progressive kinetic and postural tremor was remarkable from around 20 weeks of age and aggravated. Muscular atrophy was apparent in slow muscles, but not in fast muscles. The increase in motor unit number estimation was detected by electromyography, reflecting compensatory re-innervation by remaining FInt- and FF-type motor neurons to the orphaned slow muscle fibers. The muscle fibers gradually manifested fast/slow hybrid phenotypes, and the remaining FInt-and FF-type motor neurons gradually disappeared. These results suggest selective ablation of S- and FR-type motor neurons induces progressive muscle fiber-type transition, exhaustion of remaining FInt- and FF-type motor neurons, and late-onset kinetic and postural tremor in mice.

Age-dependent loss of Crls1 causes myopathy and skeletal muscle regeneration failure.

Skeletal muscle aging results in the gradual suppression of myogenesis, leading to muscle mass loss. However, the specific role of cardiolipin in myogenesis has not been determined. This study investigated the crucial role of mitochondrial cardiolipin and cardiolipin synthase 1 (Crls1) in age-related muscle deterioration and myogenesis. Our findings demonstrated that cardiolipin and Crls1 are downregulated in aged skeletal muscle. Moreover, the knockdown of Crls1 in myoblasts reduced mitochondrial mass, activity, and OXPHOS complex IV expression and disrupted the structure of the mitochondrial cristae. AAV9-shCrls1-mediated downregulation of Crls1 impaired muscle regeneration in a mouse model of cardiotoxin (CTX)-induced muscle damage, whereas AAV9-mCrls1-mediated Crls1 overexpression improved regeneration. Overall, our results highlight that the age-dependent decrease in CRLS1 expression contributes to muscle loss by diminishing mitochondrial quality in skeletal muscle myoblasts. Hence, modulating CRLS1 expression is a promising therapeutic strategy for mitigating muscle deterioration associated with aging, suggesting potential avenues for developing interventions to improve overall muscle health and quality of life in elderly individuals.

STAC3-related myopathy: A Report of a Cohort of Seven Saudi Arabian Patients.

AIM: The study aims to review all the genetically confirmed STAC3-related myopathy being followed in a single center in the Eastern Province of Saudi Arabia. METHODOLOGY: A retrospective review of all genetically confirmed STAC3-related myopathy followed in our clinic has been conducted. RESULTS: 7 patients with STAC3-related myopathy have been found in our cohort, with all the patients presenting with infantile hypotonia, myopathic facies, and muscle weakness in the first year of life. Feeding difficulties and failure to thrive were found in all patients except one who died during the neonatal period. Respiratory muscle involvement was also found in 5 out of 6 formally tested patients while cleft palate was found in 5 patients. CONCLUSION: STAC3-related myopathy is a relatively rare, malignant hyperthermia (MH)--causing muscle disease described in specific, highly consanguineous populations. Making the diagnosis in myopathic patients with cleft palate preoperatively can prevent MH-induced, anesthesia-related perioperative complications.

Spontaneous tendon or ligament ruptures in patients undergoing dialysis: First pediatric case report and literature review.

Spontaneous tendon or ligament ruptures are quite rare and mostly associated with chronic systemic diseases such as diabetes mellitus, systemic lupus erythematosus, rheumatoid arthritis, and chronic kidney disease (CKD). In this study, we present the first documented case of a spontaneous rupture of the medial patellofemoral ligament (MPFL) in a pediatric patient. The patient was undergoing long-term peritoneal dialysis (PD) and had a history of severe secondary hyperparathyroidism. Additionally, we discussed spontaneous tendon and ligament ruptures associated with CKD or dialysis through a comprehensive literature review. This case report highlights the importance of recognizing that spontaneous tendon or ligament injuries are not exclusive to adults; children with CKD can also be affected. Several factors including poor parathyroid hormone (PTH) and metabolic acidosis control, prolonged CKD duration and presence of malnutrition play role in the pathogenesis. Early diagnosis is crucial as it allows for timely surgical intervention and leads to a favorable functional recovery.

Atrial myopathy.

This paper delves into the progressive concept of atrial myopathy, shedding light on its development and its impact on atrial characteristics. It extensively explores the intricate connections between atrial myopathy, atrial fibrillation (AF), and strokes. Researchers have sought additional contributors to AF-related strokes due to the absence of a clear timing correlation between paroxysmal AF episodes and strokes in patients with cardiac implantable electronic devices. Through various animal models and human investigations, a close interrelation among aging, inflammation, oxidative stress, and stretching mechanisms has been identified. These mechanisms contribute to fibrosis, alterations in electrical properties, autonomic remodeling, and a heightened pro-thrombotic state. These interconnected factors establish a detrimental cycle, exacerbating atrial myopathy and elevating the risk of sustained AF and strokes. By emphasizing the significance of atrial myopathy and the risk of strokes that are distinct from AF, the paper also discusses methods for identifying patients with atrial myopathy. Moreover, it proposes an approach to incorporate the concept of atrial myopathy into clinical practice to guide anticoagulation decisions in individuals with AF.

Concentration of lipids, cholesterol, and fatty acid profile in chicken breast meat affected by wooden breast myopathy frozen for up to 12 mo.

The aim of this study was to examine the effects of frozen storage for 12 mo on the concentrations of lipids and cholesterol and fatty acid profile of wooden chicken breast meat. A total of 120 samples of chicken breasts were selected, according to the degree of "wooden breast" myopathy ["severe," "moderate," and "normal" (absence of myopathy)], from male chickens slaughtered at 42 d of age, from Cobb 500 strain. Part of the samples (n = 20/grade of severity) were evaluated on the day of collection and the remainder were packaged, frozen and stored at -18°C for up to 12 mo. At the beginning (collection day) and at the end of the proposed freezing period (12 mo), analyses of lipid, cholesterol, and fatty acid profile were carried out. Percentage of saturated (SFA), monounsaturated (MUFA), and polyunsaturated (PUFA) fatty acids were evaluated. Meats affected by wooden breast myopathy had lower levels of PUFA that exert beneficial effects on health, such as DHA, EPA and ARA, and this profile is impaired by prolonged storage (12 mo), which results in important nutritional losses for the consumer.

Hoffmann's syndrome in subclinical hypothyroidism.

Hypothyroidism is an endocrine disorder which occurs due to a deficiency of thyroid hormones. Hoffmann's syndrome is a rare complication of hypothyroidism - presenting as hypothyroid myopathy. We describe the case of a 20-year-old lactating female, known to have hypothyroidism (diagnosed during her pregnancy and having discontinued treatment following delivery), presenting with complaints of pain, swelling of bilateral calf muscles with cramps in bilateral lower limbs. Symptoms of muscle pseudohypertrophy with muscle stiffness are relatively rare in subclinical hypothyroidism and it is important to identify and diagnose this rare condition, and initiate appropriate treatment.

Myopathies of endocrine origin: A review for physicians.

Myopathies are a common manifestation of endocrine disorders. Endocrine myopathies are often overlooked while considering differential diagnoses in patients with musculoskeletal symptoms. The hindrance to mobility and the musculoskeletal discomfort owing to these myopathies are important causes of disability and depreciated quality of life in these patients. Endocrine myopathies occur due to the effects of endogenous or iatrogenic hormonal imbalance on skeletal muscle protein and glucose metabolism, disrupting the excitation-contraction coupling. Abnormalities of the pituitary, thyroid, parathyroid, adrenal, and gonadal hormones have all been associated with myopathies and musculoskeletal symptoms. Endocrine myopathies can either be the complication of a secondary endocrine disorder or a presenting symptom of a missed underlying disorder. Therefore, an underlying endocrine abnormality must always be excluded in all patients with musculoskeletal symptoms. This review presents a compilation of various endocrine myopathies, their etiopathogenesis, clinical presentation, diagnostic modalities, and treatment protocols.

Impact of Wooden Breast myopathy on in vitro protein digestibility, metabolomic profile, and cell cytotoxicity of cooked chicken breast meat.

This study investigated the impacts of Wooden Breast (WB) abnormality on in vitro protein digestibility and cytotoxicity of cooked chicken breast meat. Chicken breasts without (non-WB, n = 6) or with severe WB condition (WB, n = 6) were cooked and subjected to static in vitro protein digestion. The results showed no significant differences in free-NH2, degree of hydrolysis and distribution of peptide molecular weight between non-WB and WB samples at late intestinal digestion (P5), suggesting no adverse effects of WB on protein digestibility. Based on peptidomic analysis, P5 fraction of WB showed greater content of peptides with oxidative modification than that of non-WB. Untargeted metabolomics did not find any metabolites with potential toxicity either in non-WB and WB. Hydrolyzed non-WB and WB (1.56-100 µg/mL) did not affect viability of Caco-2 and Vero cells but addition of WB samples reduced Caco-2 cell viability compared with non-WB.

Femoral nerve palsy as a complication due to COVID-19 coagulopathy and iliopsoas muscle hematoma - case report.

BACKGROUND: COVID-19 (Coronavirus disease 2019) pandemic is the main medical problem around the world from the end of 2019. We found until now many symptoms of this disease, but one of the most problematic was thrombosis. Wide recommendation on COVID-19 treatment was pharmacological thromboprophylaxis. In some papers we found that clinicians face the problem of bleeding in those patients. Is still unknown that coronavirus could led to the coagulopathy. CASE PRESENTATION: We described case report of patient who with COVID-19 disease present femoral nerve palsy caused by the iliopsoas hematoma. There were no deviations in coaguology parameters, patient got standard thromboprophylaxis, besides above probably COVID-19 was risk factor of hematoma formation. Non-operative treatment was applied, thrombophylaxis was discontinued. In the follow up in the radiological exam we saw reduction of the haematoma and patient report decrease of symptoms. CONCLUSIONS: We should assess individually patient with COVID-19 according to thrombosis risk factors. Probably we should be more careful in ordering thrombophylaxis medications in COVID-19 patients.

The incidence and associated risk factors affecting myopathies in broiler chickens in Lithuania.

The objectives of this research were to highlight the main factors, which have relevant significance for etiology of myopathies and to assess the incidence of myopathies in a representative population of broilers raised in Lithuania. Eighteen flocks were evaluated to assess the incidence of musculus pectoralis major myopathies (PMM) (total 54,000 broilers) and dorsal cranial myopathy (DCM) (total 124,200 broilers). Thirteen flocks (total 19,500 broilers) were evaluated to find out deep pectoral myopathy (DPM) occurrence in Lithuania. Investigated parameters of each flock were: average broiler live body weight (BW) at slaughter, average slaughter age, treatment and seasons. A correlation analysis was used to measure the strength of the linear relationship between the investigated traits and incidence of these myopathies. Overall, the incidence of PMM in Lithuania was 18.19%. DCM and DPM were 5.16% and 0.27%, respectively. The percentage of PMM in flocks was strongly associated with average broiler live BW at slaughter (r=0.898, p<0.001) and age at slaughter (r=0.693, p<0.001). The percentage of PMM in flocks was negatively related with treatment of broilers (rs=-0.535, p<0.05). The percentage of DCM was positively associated with average broiler live BW at slaughter (r=0.537, p<0.05) and with seasons (rs=0.658, p<0.01). However, our study results revealed, that the analyzed parameters are not so important in DPM etiology. Furthermore, predisposing factors of PMM, DCM and DPM are different. These findings suggest that not only broiler's heavy weight and age at slaughter could have influence for etiology of myopathies.

Muscle Properties, Gross Motor Performance, and Quality of Life in Children With Sickle Cell Disease.

PURPOSE: To explore muscle properties, gross motor performance, and quality of life (QoL) in children with sickle cell disease (SCD) compared with controls and to assess relationships among these outcomes. METHODS: A cross-sectional study of 24 children assessed muscle properties including: knee extension strength by dynamometry; vastus lateralis (VL) and rectus femoris (RF) muscle thickness by ultrasonography; and VL and RF neuromuscular activation (rate of muscle activation [RoA]) by electromyography (EMG). Gross motor performance and QoL were assessed by standardized tests and questionnaires. RESULTS: Children with SCD had impaired knee extension strength, VL EMG RoA, gross motor performance, and QoL compared with children without SCD. Relationships among muscle properties, gross motor performance, and QoL were identified. CONCLUSIONS: These findings indicate that comprehensive muscle properties, gross motor performance, and QoL assessments should be considered to support and develop individualized physical therapy plans for children with SCD.

Physical, chemical, and microbiological evaluation of sausages produced with chicken meat affected by deep pectoral myopathy.

The purpose of the present study was to characterize the chemical composition, lipid oxidation, and physical characteristics of fresh sausages produced with meat from the pectoralis major muscle affected by deep pectoral myopathy. For the characterization of myopathy, samples were collected from broiler chickens slaughtered between 42- and 46-days old in a slaughterhouse in the state of São Paulo. The experiment consisted of samples from pectoralis major muscle of birds affected or not by DPM (normal-absence of myopathy and DPM category 3-progressive degeneration of the pectoralis minor with a greenish appearance). After classification of the samples, a part of raw products with the pectoralis minor removed, was submitted to microbiological and physical analyses for the characterization of DPM. Afterward, only the pectoralis major muscle was used to manufacture fresh sausages and 2 groups were established. The evaluated parameters were: color, pH, water-holding capacity (WHC), cooking loss (CL), shear force (SF), water activity (Aw), and chemical composition. Meat pH was higher (P < 0.05) in meats with DPM, however, WHC values were lower (71.92%), which made CL value increase (25.31%). In addition, the coloring increased and the technological characteristics of raw breasts were less favorable with the presence of DPM. All fresh sausages' color (except a* higher control group), pH, and Aw values were higher in DPM group. No effect on SF values was observed between fresh sausages. In chemical composition, only protein content increased in fresh sausages with DPM. Microbiological analyses did not indicate bacterial contamination of raw samples and fresh sausages. Data obtained in the present study suggest fresh sausages produced with breasts affected by DPM are a viable alternative and may confer more favorable qualitative characteristics to sausages than the raw marketed meat.

Critical illness-associated weakness and related motor disorders.

Weakness of limb and respiratory muscles that occurs in the course of critical illness has become an increasingly common and serious complication of adult and pediatric intensive care unit patients and a cause of prolonged ventilatory support, morbidity, and prolonged hospitalization. Two motor disorders that occur singly or together, namely critical illness polyneuropathy and critical illness myopathy, cause weakness of limb and of breathing muscles, making it difficult to be weaned from ventilatory support, commencing rehabilitation, and extending the length of stay in the intensive care unit, with higher rates of morbidity and mortality. Recovery can take weeks or months and in severe cases, and may be incomplete or absent. Recent findings suggest an improved prognosis of critical illness myopathy compared to polyneuropathy. Prevention and treatment are therefore very important. Its management requires an integrated team approach commencing with neurologic consultation, creatine kinase (CK) measurement, detailed electrodiagnostic, respiratory and neuroimaging studies, and potentially muscle biopsy to elucidate the etiopathogenesis of the weakness in the peripheral and/or central nervous system, for which there may be a variety of causes. These tenets of care are being applied to new cases and survivors of the coronavirus-2 disease pandemic of 2019. This chapter provides an update to the understanding and approach to critical illness motor disorders.

Mitochondrial dysfunction and calcium dyshomeostasis in the pectoralis major muscle of broiler chickens with wooden breast myopathy.

The incidence of wooden breast (WB) meat of commercial broiler chicken has been increasing in recent years. Histological examination found that the occurrence of WB myopathy was accompanied by the pectoralis major (PM) muscle damage. So far, the potential mechanisms are not fully understood. This study aimed to explore the underlying mechanism of the damage of WB-affected PM muscle caused by changes in mitochondrial function, mitochondrial redox status and Ca2+ homeostasis. A total of 80 market-age Arbor Acres male broiler chickens were sampled and categorized into control (CON) and WB groups based on the evaluation of myopathic lesions. PM muscle samples were collected (n = 8 in each group) for histopathological evaluation and biochemical analyses. Ultrastructural examination and histopathological changes suggested the occurrence of PM muscle damage in broiler chickens with WB myopathy. The WB group showed an increased level of reactive oxygen species and enhanced antioxidant capacities in mitochondria of PM muscle. These changes were related to impaired mitochondria morphology and mitochondrial dysfunction. In addition, abnormal expressions of Ca2+ channels led to substantial Ca2+ loss in SR and cytoplasmic Ca2+ overload, as well as Ca2+ accumulation in mitochondria, resulting in Ca2+ dyshomeostasis in PM muscle of broiler chickens with WB myopathy. Combined, these findings indicate that WB myopathy is related to mitochondrial dysfunction, mitochondrial redox status imbalance and Ca2+ dyshomeostasis, leading to WB-affected PM muscle damage.