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Neurologia I Neurochirurgia Polska Jun 2024Lambert-Eaton myasthenic syndrome (LEMS) is an ultrarare neuromuscular disease with a triad of symptoms: muscle paresis, dysautonomy, and areflexia. Amifampridine is the...
INTRODUCTION
Lambert-Eaton myasthenic syndrome (LEMS) is an ultrarare neuromuscular disease with a triad of symptoms: muscle paresis, dysautonomy, and areflexia. Amifampridine is the symptomatic treatment of LEMS.
AIM OF STUDY
To assess the effectiveness and safety of treatment in the real world.
MATERIAL AND METHODS
14 patients with non-neoplastic LEMS treated with amifampridine were enrolled in the study (female 42.9%, mean age 48.8 ± 11.4 years). The patients were assessed using the Quantitative Myasthenia Gravis (QMG) scale, QMG limb domain (LD) score, spirometry, Hand Grip Strength (GRIP) test, and repetitive nerve stimulation study (RNS) at baseline and at the end of follow-up. Diagnostic delay since first symptoms was from seven months up to 22 years. Treatment delay ranged from one to 26 years. The patients were treated and reevaluated after 21.1 ± 12.0 weeks (range 13-48).
RESULTS
All of the patients improved in QMG score. Mean improvement was 5.1 ± 2.0 (range 1-8) points (p < 0.001) and this showed no correlation with the duration of the disease before treatment (p = 0.477). 85.7% of patients (N = 12) improved ≥ 3 points (clinically meaningful) in QMG. 78.6% of the patients improved in QMG LD (mean 2.2 ± 1.6 points (p < 0.001)). Also, forced vital capacity (FVC) improved after treatment (p = 0.031). Mean improvement in GRIP test was 7.0 ± 7.1 kg in the right hand and 5.2 ± 7.5 kg in the left hand (p < 0.001). In RNS before treatment, facilitation ( > 100%) was observed in 78.6% (N = 11) of patients, and was higher before treatment (p < 0.001). Compound muscle action potential (CMAP) amplitude was higher after treatment (p < 0.001). Mean increase of CMAP amplitude was 2.1 ± 1.6 times. In 64.3% (N = 9) of patients lowering of corticosteroid dose was achieved.
CONCLUSIONS
Amifampridine is an effective treatment in non-neoplastic LEMS patients, regardless of disease duration. The treatment is well-tolerated and allows to reduce dose of corticosteroids in the majority of patients.
PubMed: 38935421
DOI: 10.5603/pjnns.99335 -
Indian Journal of Public Health Oct 2023The activity of daily living (ADL) related to stair climbing is one of the most compromised ADLs among the elders. It requires good muscle strength, balance, and range...
Effect of Combined Intervention of Yoga and Physiotherapy as Compared to Only Physiotherapy on Lower Limb Muscle Strength, Balance and Range of Motion among Elders with Compromised Stair Climbing in Puducherry - A Parallel Arm Non-Randomized Control Trial.
The activity of daily living (ADL) related to stair climbing is one of the most compromised ADLs among the elders. It requires good muscle strength, balance, and range of motion (ROM) in the lower limb. We aimed to investigate the effects of Yoga complemented with physiotherapy (Intervention group) compared to only physiotherapy (control group) on lower limb muscle strength, balance, and ROMs among elders with ADL limitation in stair climbing. Sixty-five community-dwelling elders with compromised stair climbing were enrolled. Elders who consented to yoga along with physiotherapy were enrolled in the intervention arm. Data were collected and analyzed following 12 weeks of intervention. Endline assessment showed that the intervention group had more improvement in all test scores and ROMs as compared to the control group, although there were improvements in both arms as compared to baseline. Considering the beneficial effects of both interventions, yoga can be added to gain additional advantages.Trial Registration:Indian Clinical Trials Registry number CTRI/2021/08/035825.
Topics: Humans; Yoga; Muscle Strength; Male; Female; Aged; Postural Balance; Lower Extremity; Range of Motion, Articular; Physical Therapy Modalities; Stair Climbing; India; Activities of Daily Living; Middle Aged; Combined Modality Therapy
PubMed: 38934820
DOI: 10.4103/ijph.ijph_1718_22 -
Kidney & Blood Pressure Research Jun 2024Background Kidney transplantation constitutes the most effective therapeutic option for patients suffering from end-stage renal disease but remains burdened by a high... (Review)
Review
Background Kidney transplantation constitutes the most effective therapeutic option for patients suffering from end-stage renal disease but remains burdened by a high incidence of cardiovascular disease. To date, exercise is an important preventive strategy that has been underestimated; in kidney transplant patients exercise programs leads to an improvement in cardiorespiratory performance, muscle strength, arterial stiffness and patients' quality of life perception. Summary The nephrology and transplant community have moved from generic suggestions to specific indications regarding Frequency, Intensity, Time, Type, Volume, and Progression of physical exercise both in the pre- and post-transplant phase. The latest guidelines from the World Health Organization for patients with chronic conditions propose a combination of aerobic, muscle strengthening and multicomponent exercises (e.g. balance) to improve health. Based on recent evidence, a combined exercise program (aerobic and strength exercise) is largely proposed to kidney transplant recipients. Aerobic exercise should be performed at an intensity >60% of theoretical maximum heart rate or maximum oxygen uptake possibly every day, strength training should be performed at a >60% the estimate single Maximum Repetition, at least 2 times per week. Key Messages Physical exercise should be personalized in relation to the patient's baseline performance; increases must be progressive and gradual. Regular physical activity should also be recommended to patients awaiting for a transplant. Eventually, an organizational models based on a network of Nephrology Units, Transplant Centers, Sports Medicine Centers and fitness center or outdoor gym are essential elements for overcoming the logistical barriers for prescribing and carrying out regular physical activity.
PubMed: 38934158
DOI: 10.1159/000539996 -
Heliyon Jun 2024The botulinum toxin is an extremely potent substance that impacts the nervous system. There has been a rise in cases of medical poisoning associated with it,...
BACKGROUND
The botulinum toxin is an extremely potent substance that impacts the nervous system. There has been a rise in cases of medical poisoning associated with it, particularly in the field of plastic and aesthetic procedures, in recent years.
CASE DESCRIPTION
A 51-year-old woman underwent a facial wrinkle reduction procedure with an unauthorized injection of 100 U of botulinum toxin at an unlicensed medical facility six days prior to hospitalization. Over time, her toxicity symptoms intensified, impacting her respiratory muscles, and she did not receive antitoxin treatment. She was concurrently diagnosed with a COVID-19 infection during this period. Nonetheless, she experienced a full recovery 86 days after the injection.
CONCLUSION
Currently, there is no effective antidote for botulism. Nevertheless, the timely administration of antitoxin can contribute to reducing the duration of the illness, alleviating symptoms, and preventing its recurrence. It is essential to recognize that individual responses may vary, and in this instance, the absence of antitoxin treatment did not significantly prolong the course of the disease. Accurate diagnosis of medical poisoning can be based on injection history and clinical symptoms. Early indications like fatigue and dry mouth warrant particular attention, emphasizing the importance of immediate medical intervention. To address emergencies, the Center for Disease Control (CDC) should maintain an accessible supply of antitoxin. Patients with severe poisoning should be hospitalized until their respiratory muscle strength is fully restored.
PubMed: 38933984
DOI: 10.1016/j.heliyon.2024.e32237 -
Frontiers in Endocrinology 2024Tryptophan's (Trp) metabolites are undervalued markers of human health. Their serum concentrations are modified by physical exercise and other factors, among which...
INTRODUCTION
Tryptophan's (Trp) metabolites are undervalued markers of human health. Their serum concentrations are modified by physical exercise and other factors, among which fasting has a well-documented role. Although this mechanism is hardly explored, thus, the study aimed to determine the effect of the 8-day fasting period and the impact of such a procedure on a single bout of an endurance exercise on the concentration of kynurenine pathway (KP) metabolites.
METHODS
10 participants fasted for 8 days, and 10 as a control group participated in the study. The exercise was performed at baseline after an overnight fast and repeated post 8 days.
RESULTS
The 8 days of fasting increased the resting 3-hydroxy-L-kynurenine (3HK), picolinic acid (PA), kynurenic acid (KYNA), and xanthurenic acid (XA) serum concentration. Also elevated phenylalanine (Phe) and tyrosine (Tyr) levels were recorded, suggesting expanded proteolysis of muscle proteins. In turn, physical activity caused a decrease in the concentration of 3-hydroxyanthranilic acid (3HAA) and PA after fasting. The obtained results were not recorded in controls.
CONCLUSION
The results of this study show that the health-promoting effects of fasting are associated with changes in the KYN pathway. The increase in the concentration of PA and XA metabolites following fasting is capable of penetrating the blood-brain barrier, and KYNA, which initiates several beneficial changes, supports this assumption.
Topics: Humans; Male; Fasting; Kynurenine; Exercise; Adult; Young Adult; Rest; Healthy Volunteers; Kynurenic Acid; Tryptophan; Biomarkers; Picolinic Acids
PubMed: 38933822
DOI: 10.3389/fendo.2024.1403491 -
Sensors (Basel, Switzerland) Jun 2024Muscles play an indispensable role in human life. Surface electromyography (sEMG), as a non-invasive method, is crucial for monitoring muscle status. It is characterized...
Muscles play an indispensable role in human life. Surface electromyography (sEMG), as a non-invasive method, is crucial for monitoring muscle status. It is characterized by its real-time, portable nature and is extensively utilized in sports and rehabilitation sciences. This study proposed a wireless acquisition system based on multi-channel sEMG for objective monitoring of grip force. The system consists of an sEMG acquisition module containing four-channel discrete terminals and a host computer receiver module, using Bluetooth wireless transmission. The system is portable, wearable, low-cost, and easy to operate. Leveraging the system, an experiment for grip force prediction was designed, employing the bald eagle search (BES) algorithm to enhance the Random Forest (RF) algorithm. This approach established a grip force prediction model based on dual-channel sEMG signals. As tested, the performance of acquisition terminal proceeded as follows: the gain was up to 1125 times, and the common mode rejection ratio (CMRR) remained high in the sEMG signal band range (96.94 dB (100 Hz), 84.12 dB (500 Hz)), while the performance of the grip force prediction algorithm had an R of 0.9215, an MAE of 1.0637, and an MSE of 1.7479. The proposed system demonstrates excellent performance in real-time signal acquisition and grip force prediction, proving to be an effective muscle status monitoring tool for rehabilitation, training, disease condition surveillance and scientific fitness applications.
Topics: Electromyography; Humans; Hand Strength; Algorithms; Male; Signal Processing, Computer-Assisted; Adult; Wearable Electronic Devices; Muscle, Skeletal; Monitoring, Physiologic; Wireless Technology
PubMed: 38931601
DOI: 10.3390/s24123818 -
Pharmaceuticals (Basel, Switzerland) May 2024Pearl oysters have been extensively utilized in pearl production; however, most pearl oyster shells are discarded as industrial waste. In a previous study, we...
Pearl oysters have been extensively utilized in pearl production; however, most pearl oyster shells are discarded as industrial waste. In a previous study, we demonstrated that the intraperitoneal administration of pearl oyster shell-derived nacre extract (NE) prevented d-galactose-induced brain and skin aging. In this study, we examined the anti-aging effects of orally administered NE in senescence-accelerated mice (SAMP8). Feeding SAMP8 mice NE prevented the development of aging-related characteristics, such as coarse and dull hair, which are commonly observed in aged mice. Additionally, the NE mitigated muscle aging in SAMP8 mice, such as a decline in grip strength. Histological analysis of skeletal muscle revealed that the NE suppressed the expression of aging markers, cyclin-dependent kinase inhibitor 2A (p16) and cyclin-dependent kinase inhibitor 1 (p21), and increased the expression of sirtuin1 and peroxisome proliferator-activated receptor gamma coactivator 1 (PGC1)- α, which are involved in muscle synthesis. These findings suggest that the oral administration of NE suppresses skeletal muscle aging. Moreover, NE administration suppressed skin aging, including a decline in water content. Interestingly, oral administration of NE significantly extended the lifespan of SAMP8 mice, suggesting that its effectiveness as an anti-aging agent of various tissues including skeletal muscle, skin, and adipose tissue.
PubMed: 38931380
DOI: 10.3390/ph17060713 -
Nutrients Jun 2024Individuals with chronic kidney disease (CKD) often experience reduced muscle strength and diminished health-related quality of life (HRQoL), and engaging in regular...
BACKGROUND
Individuals with chronic kidney disease (CKD) often experience reduced muscle strength and diminished health-related quality of life (HRQoL), and engaging in regular exercise may improve them. The aim of this study was to assess the effect of intradialytic exercise using non-immersive virtual reality (VR) on body composition of patients with CKD on hemodialysis (HD).
METHODS
This was a substudy in a clinical trial of intradialytic exercise intervention using a non-immersive VR game in which the patient interacted by moving the lower limbs. Body composition was determined by BCM Fresenius multifrequency stereoscopic bioimpedance. Body mass index (BMI), fat tissue index (FTI), lean tissue index (LTI), extracellular/intracellular water (EIW), and phase angle (PA) were recorded in 52 patients, 24 in the control group (CG) and 28 in the exercise group (EG).
RESULTS
Statistically significant differences were observed between both groups. The LTI increased in the EG while it decreased in the CG. The FTI and the EIW decreased in the EG compared to the increase observed in the CG.
CONCLUSIONS
Intradialytic exercise using non-immersive VR was associated with an increase in LTI and a decrease in FTI of CKD patients on HD.
Topics: Humans; Body Composition; Renal Dialysis; Male; Renal Insufficiency, Chronic; Female; Middle Aged; Virtual Reality; Aged; Exercise Therapy; Body Mass Index; Quality of Life; Exercise; Adult; Electric Impedance
PubMed: 38931320
DOI: 10.3390/nu16121968 -
Nutrients Jun 2024Sarcopenia is an age-related condition characterized by progressive loss of muscle mass, strength, and function. The occurrence of sarcopenia has a huge impact on...
BACKGROUND
Sarcopenia is an age-related condition characterized by progressive loss of muscle mass, strength, and function. The occurrence of sarcopenia has a huge impact on physical, psychological, and social health. Therefore, the prevention and treatment of sarcopenia is becoming an important public health issue.
METHOD
35 six-week-old male C57BL/6 mice were randomly divided into five groups, one of which served as a control group, while the rest of the groups were constructed as a model of sarcopenia by intraperitoneal injection of D-galactose. The intervention with lactoferrin, creatine, and their mixtures, respectively, was carried out through gavage for 8 weeks. Muscle function was assessed based on their endurance, hanging time, and grip strength. The muscle tissues were weighed to assess the changes in mass, and the muscle RNA was extracted for myogenic factor expression and transcriptome sequencing to speculate on the potential mechanism of action by GO and KEGG enrichment analysis.
RESULT
The muscle mass (lean mass, GAS index), and muscle function (endurance, hanging time, and grip strength) decreased, and the size and structure of myofiber was smaller in the model group compared to the control group. The intervention with lactoferrin and creatine, either alone or combination, improved muscle mass and function, restored muscle tissue, and increased the expression of myogenic regulators. The combined group demonstrated the most significant improvement in these indexes. The RNA-seq results revealed enrichment in the longevity-regulated pathway, MAPK pathway, focal adhesion, and ECM-receptor interaction pathway in the intervention group. The intervention group may influence muscle function by affecting the proliferation, differentiation, senescence of skeletal muscle cell, and contraction of muscle fiber. The combined group also enriched the mTOR-S6K/4E-BPs signaling pathway, PI3K-Akt signaling pathway, and energy metabolism-related pathways, including Apelin signaling, insulin resistance pathway, and adipocytokine signaling pathway, which affect energy metabolism in muscle.
CONCLUSIONS
Lactoferrin and creatine, either alone or in combination, were found to inhibit the progression of sarcopenia by influencing the number and cross-sectional area of muscle fibers and muscle protein synthesis. The combined intervention appears to exert a more significant effect on energy metabolism.
Topics: Animals; Lactoferrin; Male; Sarcopenia; Mice, Inbred C57BL; Creatine; Disease Models, Animal; Muscle, Skeletal; Mice; Muscle Strength; Signal Transduction
PubMed: 38931310
DOI: 10.3390/nu16121958 -
Nutrients Jun 2024Handgrip strength (HGS) is an indicator of muscular strength, used in the diagnosis of sarcopenia, undernutrition, and physical frailty as well as recovery. Typically,... (Review)
Review
BACKGROUND
Handgrip strength (HGS) is an indicator of muscular strength, used in the diagnosis of sarcopenia, undernutrition, and physical frailty as well as recovery. Typically, the maximum HGS value is used; however, recent evidence suggests the exploration of new indicators provided based on the force-time curve to achieve a more comprehensive assessment of muscle function. Therefore, the objective was to identify indicators of the HGS profile beyond maximum HGS, based on force-time curves, and to systematize knowledge about their applications to various types of samples, health issues, and physical performance.
METHODS
A systematic review was performed including studies whose participants' HGS was assessed with a digital or adapted dynamometer. The outcome measures were HGS profile indicators calculated from the force-time curve.
RESULTS
a total of 15 studies were included, and the following indicators were identified: grip fatigue, fatigability index, fatigue rate, fatigue resistance, time to 80% maximal voluntary contraction, plateau coefficient of variability, time to maximum value, T-90%, release rate, power factor, grip work, average integrated area, endurance, cycle duration, time between cycles, maximum and minimum force-velocity, rate of grip force, final force, inflection point, integrated area, submaximal control, and response time.
CONCLUSIONS
Various indicators based on the force-time curve can be assessed through digital or adapted dynamometers. Future research should analyze these indicators to understand their implications for muscle function assessment, to standardize evaluation procedures, to identify clinically relevant measures, and to clarify their implications in clinical practice.
Topics: Humans; Hand Strength; Muscle Strength Dynamometer; Female; Male; Muscle Fatigue; Aged; Muscle, Skeletal; Muscle Strength; Middle Aged; Sarcopenia; Time Factors; Adult
PubMed: 38931305
DOI: 10.3390/nu16121951