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Frontiers in Bioengineering and... 2023The central nervous system simplifies motor control by sending motor commands activating groups of muscles, known as synergies. Physiological locomotion can be described... (Review)
Review
The central nervous system simplifies motor control by sending motor commands activating groups of muscles, known as synergies. Physiological locomotion can be described as a coordinated recruitment of four to five muscle synergies. The first studies on muscle synergies in patients affected by neurological diseases were on stroke survivors. They showed that synergies can be used as biomarkers for motor impairment as they vary in patients with respect to healthy people. Likewise, muscle synergy analysis has been applied to developmental diseases (DD). The need for a comprehensive view of the present findings is crucial for comparing results achieved so far and promote future directions in the field. In the present review, we screened three scientific databases and selected thirty-six papers investigating muscle synergies extracted from locomotion in children affected by DD. Thirty-one articles investigate how cerebral palsy (CP) influences motor control, the currently exploited method in studying motor control in CP and finally the effects of treatments in these patients in terms of synergies and biomechanics; two articles investigate how muscle synergies vary in Duchenne muscular dystrophy (DMD), and three other articles assess other developmental pathologies, such as chronic and acute neuropathic pain. For CP, most of the studies demonstrate that the number of synergies is lower and that the synergy composition varies in the affected children with respect to normal controls. Still, the predictability of treatment's effects and the etiology of muscle synergy variation are open questions, as it has been reported that treatments minimally modify synergies, even if they improve biomechanics. The application of different algorithms in extracting synergies might bring about more subtle differences. Considering DMD, no correlation was found between non-neural muscle weakness and muscle modules' variation, while in chronic pain a decreased number of synergies was observed as a possible consequence of plastic adaptations. Even if the potential of the synergistic approach for clinical and rehabilitation practices is recognized, there is not full consensus on protocols nor widely accepted guidelines for the systematic clinical adoption of the method in DD. We critically commented on the current findings, on the methodological issues and the relative open points, and on the clinical impact of muscle synergies in neurodevelopmental diseases to fill the gap for applying the method in clinical practice.
PubMed: 37180039
DOI: 10.3389/fbioe.2023.1145937 -
Therapeutic Advances in Respiratory... 2023Upper limb (UL) muscle dysfunction is a common extrapulmonary manifestation of chronic obstructive pulmonary disease (COPD). UL muscle dysfunction is associated with... (Meta-Analysis)
Meta-Analysis Review
The effects of upper limb exercise training on upper limb muscle strength in people with chronic obstructive pulmonary disease: a systematic review and meta-analysis of randomized controlled trials.
BACKGROUND
Upper limb (UL) muscle dysfunction is a common extrapulmonary manifestation of chronic obstructive pulmonary disease (COPD). UL muscle dysfunction is associated with muscle weakness, dyspnea, and exercise intolerance. Although upper limb exercise training (ULET) is typically incorporated in pulmonary rehabilitation programs, its effects on UL muscle strength remains unclear.
OBJECTIVES
The purpose of this systematic review was to investigate the effectiveness of ULET, in UL muscle strength of people with COPD.
DESIGN
This is systematic review and meta-analysis study.
DATA SOURCES AND METHODS
Preferred Reporting Items for Systematic Review and Meta-Analysis (PRISMA) Protocols 2020 guidelines were used for this study. PubMed, Cochrane, CINAHL Plus and SPORTDiscus and clinicaltrials.gov registry were searched from inception to July 2022. Included studies were randomized controlled trials, assessing the effectiveness in muscle strength of ULET, compared with other types of upper or lower limb exercise or no exercise. The quality and risk of bias were assessed using the Physiotherapy Evidence Database (PEDro) scale and certainty of evidence with the Grading of Recommendations, Assessment, Development, and Evaluations approach. Treatment effects of ULET were calculated using standardized mean differences and 95% confidence intervals.
RESULTS
Twenty-four studies, with a total sample of 882 patients, were included. Most studies were of moderate quality and high risk of bias. Very low to low certainty evidence indicates a significant difference in UL muscle strength in favor of resistance ULET, compared with lower limb exercise alone or no exercise. No significant differences were found in different types of ULET comparisons.
CONCLUSION
The results of this review showed that resistance ULET could improve UL muscle strength in people with COPD. Most studies, however, were of moderate quality and high risk of bias. Further studies with larger sample sizes, better methodological quality, and standardized training protocols are needed to confirm these findings.
Topics: Humans; Quality of Life; Randomized Controlled Trials as Topic; Pulmonary Disease, Chronic Obstructive; Exercise; Physical Therapy Modalities; Upper Extremity; Muscle Strength
PubMed: 37165688
DOI: 10.1177/17534666231170813 -
Journal of Clinical Neuroscience :... Jun 2023Myasthenia gravis (MG) can have a variety of respiratory presentations, ranging from mild symptoms through to respiratory failure. The evaluation of respiratory function... (Review)
Review
BACKGROUND
Myasthenia gravis (MG) can have a variety of respiratory presentations, ranging from mild symptoms through to respiratory failure. The evaluation of respiratory function in MG can be limited by accessibility to testing facilities, availability of medical equipment, and facial weakness. The single count breath test (SCBT) may be a useful adjunct in the evaluation of respiratory function in MG.
METHOD
A systematic review of the databases PubMed, EMBASE, and the Cochrane Library was conducted from inception to October 2022 in accordance with PRISMA guidelines and was registered on PROSPERO.
RESULTS
There were 6 studies that fulfilled the inclusion criteria. The described method of evaluating SCBT involves inhaling deeply, then counting at two counts per second, in English or Spanish, sitting upright, with normal vocal register, until another breath needs to be taken. The identified studies support that the SCBT has a moderate correlation with forced vital capacity. These results also support that SCBT can assist the identification of MG exacerbation, including via assessment over the telephone. The included studies support a threshold count of ≥ 25 as consistent with normal respiratory muscle function. Although further analysis is needed, the included studies describe the SCBT as a quick bedside tool that is inexpensive and well tolerated.
CONCLUSIONS
The results of this review support the clinical utility of the SCBT in assessing respiratory function in MG and describe the most current and effective methods of administration.
Topics: Humans; Myasthenia Gravis; Breath Tests
PubMed: 37094510
DOI: 10.1016/j.jocn.2023.04.011 -
Frontiers in Neurology 2023Constraint-induced movement therapy (CIMT) targeting the lower limb function uses various methods. The influence of CIMT methods on lower limb outcomes after stroke has...
BACKGROUND
Constraint-induced movement therapy (CIMT) targeting the lower limb function uses various methods. The influence of CIMT methods on lower limb outcomes after stroke has rarely been examined.
OBJECTIVES
This study aimed to examine CIMT effects on lower limb outcomes and explore the influence of CIMT methods on treatment effects after stroke, with other potential factors considered as covariates.
METHODS
PubMed, Web of Science, Cochrane Library, Academic Search Premier EBSCOHost, and PEDro databases were searched until September 2022. We included randomized control trials with CIMT targeting the lower limb function and dosage-matched active control. The Cochrane risk-of-bias tool was used to evaluate the methodological quality of each study. Hedges' g was used to quantify the effect size of CIMT on outcomes compared to the active control. Meta-analyses were conducted across all studies. A mixed-variable meta-regression analysis was used to investigate the influence of CIMT methods on treatment effects after stroke, with other potential factors considered as covariates.
RESULTS
Twelve eligible randomized controlled trials with CIMT were included in the meta-analysis, where 10 trials were with a low risk of bias. A total of 341 participants with stroke were involved. For the treatment effects on the lower limb function, CIMT showed a moderate short-term effect size [Hedges' g = 0.567; > 0.05; 95% confidence interval (CI): 0.203-0.931], but a small and insignificant long-term effect size (Hedges' g = 0.470; > 0.05; 95%CI: -0.173 to 1.112), compared with conventional treatment. The CIMT method of using a weight strapped around the non-paretic leg and the ICF outcome category of the movement function were identified as significant factors contributing to the heterogeneity of short-term effect sizes across studies (β = -0.854 and 1.064, respectively, = 98%, > 0.05). Additionally, using a weight strapped around the non-paretic leg had a significant contribution to the heterogeneity of long-term effect sizes across studies as well (β = -1.000, = 77%, > 0.05).
CONCLUSION
Constraint-induced movement therapy is superior to conventional treatment for improvement of lower limb function in the short-term but not in the long-term. The CIMT method of using a weight strapped around a non-paretic leg contributed negatively to the treatment effect, and therefore might not be recommended.
SYSTEMATIC REVIEW REGISTRATION
https://www.crd.york.ac.uk/PROSPERO, identifier: CRD42021268681.
PubMed: 37006479
DOI: 10.3389/fneur.2023.1090808 -
Journal of Clinical Neuroscience :... May 2023Although global vaccination against COVID-19 infection has its excellence, potential side effects are yet of concern. Several lines of evidence have proposed ADEM... (Review)
Review
BACKGROUND
Although global vaccination against COVID-19 infection has its excellence, potential side effects are yet of concern. Several lines of evidence have proposed ADEM occurrence after SARS-CoV-2 infection. Moreover, a large number of case reports and case series have also suggested the casual association between ADEM and COVID-19 vaccination. To better understand the development of ADEM following COVID-19 vaccination and its potential association, we aimed to systematically review ADEM cases reported after COVID-19 vaccination.
METHODS
We conducted a comprehensive systematic search using three databases including PubMed, Scopus, and Web of Science. Studies that reported ADEM after COVID-19 vaccination were eligible to include in our study. Observational studies, case reports, and case series which reported cases of ADEM with sufficient detail to confirm clinical diagnosis following COVID-19 vaccination were eligible to enter our study.
RESULTS
Twenty studies were included in our systematic review after the abstract and full-text screening with a total of 54 cases. Among included patients, 45 (85.1 %) developed ADEM after the first dose of the COVID-19 vaccine, and seven (12.9 %) cases experienced ADEM after the second dose. The median time interval between vaccination and neurological symptoms was 14 days which ranged from 12 h to 63 days. Twelve (22.2 %) patients experienced symptoms of muscle weakness, ten (18.5 %) presented unconsciousness, nine (16.6 %) patients had urinary complaints, nine (16.6 %) had visual impairments, and five (9.2 %) experienced a seizure. After treatments, four (13.8 %) patients died. Forty-six patients had clinical improvement (85.1 %), also improvement in brain MRI was observed among 44 (81.4 %) patients.
CONCLUSION
In conclusion, it is not clear that ADEM could be a potential complication of COVID-19 vaccination based on the current evidence and further studies are needed. However, this rare condition should not trigger stopping the mass vaccination programs since the only way to eradicate the current pandemic of COVID-19 is to extend the number of immunized people.
Topics: Humans; COVID-19; COVID-19 Vaccines; Encephalomyelitis, Acute Disseminated; Observational Studies as Topic; SARS-CoV-2; Vaccination
PubMed: 36963124
DOI: 10.1016/j.jocn.2023.03.008 -
Research Square Mar 2023Neurological manifestations have been widely reported in adults with COVID-19, yet the extent of involvement among the pediatric population is currently poorly...
BACKGROUND
Neurological manifestations have been widely reported in adults with COVID-19, yet the extent of involvement among the pediatric population is currently poorly characterized. The objective of our systematic review is to evaluate the association of SARS-CoV-2 infection with neurological symptoms and neuroimaging manifestations in the pediatric population.
METHODS
A literature search of Cochrane Library; EBSCO CINAHL; Global Index Medicus; OVID AMED, Embase, Medline, PsychINFO; and Scopus was conducted in accordance with the Peer Review of Electronic Search Strategies form (October 1, 2019 to March 15, 2022). Studies were included if they reported (1) COVID-19-associated neurological symptoms and neuroimaging manifestations in individuals aged < 18 years with a confirmed, first SARS-CoV-2 infection and were (2) peer-reviewed. Full-text reviews of 222 retrieved articles were performed, along with subsequent reference searches.
RESULTS
A total of 843 nonduplicate records were retrieved. Of the 19 identified studies, there were ten retrospective observational studies, seven case series, one case report, and one prospective cohort study. A total of 6,985 individuals were included, where 12.8% of hospitalized patients experienced neurocognitive impairments: MIS-C (24.2%), neuroinflammation (10.1%), and encephalopathy (8.1%) were the most common disorders; headaches (16.8%) and seizures (3.8%) were the most common symptoms. Based on pediatric-specific cohorts, children experienced more drowsiness (7.3% vs. 1.3%) and muscle weakness (7.3% vs. 6.3%) as opposed to adolescents. Agitation or irritability was observed more in children (7.3%) than infants (1.3%).
CONCLUSION
Our findings revealed a high prevalence of immune-mediated patterns of disease among COVID-19 positive pediatric patients with neurocognitive abnormalities.
PubMed: 36945594
DOI: 10.21203/rs.3.rs-2653722/v1 -
International Journal of Molecular... Feb 2023Among the most common muscular dystrophies in adults is Myotonic Dystrophy type 1 (DM1), an autosomal dominant disorder characterized by myotonia, muscle wasting and... (Review)
Review
Among the most common muscular dystrophies in adults is Myotonic Dystrophy type 1 (DM1), an autosomal dominant disorder characterized by myotonia, muscle wasting and weakness, and multisystemic dysfunctions. This disorder is caused by an abnormal expansion of the CTG triplet at the gene that, when transcribed to expanded mRNA, can lead to RNA toxic gain of function, alternative splicing impairments, and dysfunction of different signaling pathways, many regulated by protein phosphorylation. In order to deeply characterize the protein phosphorylation alterations in DM1, a systematic review was conducted through PubMed and Web of Science databases. From a total of 962 articles screened, 41 were included for qualitative analysis, where we retrieved information about total and phosphorylated levels of protein kinases, protein phosphatases, and phosphoproteins in DM1 human samples and animal and cell models. Twenty-nine kinases, 3 phosphatases, and 17 phosphoproteins were reported altered in DM1. Signaling pathways that regulate cell functions such as glucose metabolism, cell cycle, myogenesis, and apoptosis were impaired, as seen by significant alterations to pathways such as AKT/mTOR, MEK/ERK, PKC/CUGBP1, AMPK, and others in DM1 samples. This explains the complexity of DM1 and its different manifestations and symptoms, such as increased insulin resistance and cancer risk. Further studies can be done to complement and explore in detail specific pathways and how their regulation is altered in DM1, to find what key phosphorylation alterations are responsible for these manifestations, and ultimately to find therapeutic targets for future treatments.
Topics: Animals; Adult; Humans; Myotonic Dystrophy; Phosphorylation; Alternative Splicing; RNA, Messenger; Muscular Atrophy; Muscle, Skeletal
PubMed: 36834509
DOI: 10.3390/ijms24043091 -
The Journal of Steroid Biochemistry and... May 2023Studies in vitro have demonstrated a key molecular role for 1,25-dihydroxyvitamin D (1,25D) in skeletal muscle function, with vitamin D-deficiency (low serum...
Studies in vitro have demonstrated a key molecular role for 1,25-dihydroxyvitamin D (1,25D) in skeletal muscle function, with vitamin D-deficiency (low serum 25-hydroxyvitamin D, 25D) being associated with muscle pain and weakness. Despite this, an understanding of the overall role of vitamin D in muscle health (particularly the impact of vitamin D-related genetic variants) has yet to be fully resolved, relative to more well-studied targets such as the skeleton. Thus, we aimed to review existing studies that have investigated relationships between skeletal muscle function and single nucleotide polymorphisms (SNPs) within vitamin D-related genes. A systematic review of papers published between January 2000 and June 2022 on PubMed, EMBASE and Web of Science pertaining to association between functionally relevant vitamin D receptor genetic variants and variants within genes of the vitamin D pathway and skeletal muscle function/outcomes was performed. 21 articles were included in the review for final analysis, of which 20 only studied genetic variation of the VDR gene. Of the included articles, 81 % solely included participants aged ≥ 50 years and of the 9 studies that did not only include White individuals, only 2 included Black participants. Within the vitamin D system, the VDR gene is the primary gene of which associations between polymorphisms and muscle function have been investigated. VDR polymorphisms have been significantly associated with muscle phenotypes in two or more studies. Of note A1012G was significantly associated with higher handgrip strength, but the results for other SNPs were notably variable between studies. While the lack of definitive evidence and study heterogeneity makes it difficult to draw conclusions, the findings of this review highlight a need for improvements with regards to the use of more diverse study populations, i.e., inclusion of Black individuals and other people of colour, and expanding research scope beyond the VDR gene.
Topics: Receptors, Calcitriol; Hand Strength; Vitamin D; Muscle, Skeletal; Vitamins; Polymorphism, Single Nucleotide
PubMed: 36822332
DOI: 10.1016/j.jsbmb.2023.106266 -
Journal of Clinical Medicine Feb 2023Recurrent laryngeal nerve (RLN) injury is a well and long-known complication of thyroid and parathyroid surgery that significantly affects the quality of life of... (Review)
Review
Recurrent laryngeal nerve (RLN) injury is a well and long-known complication of thyroid and parathyroid surgery that significantly affects the quality of life of patients. Despite the advances in surgical techniques and technology, it still occurs in clinical practice either as temporary paresis or as permanent paralysis of the corresponding vocal cord. The purpose of the current systematic review is to examine the value of intraoperative repair of the RLN in voice restoration. A systematic review of the existing literature was conducted using PubMed, Scopus, Cochrane Library, and Google Scholar databases according to the PRISMA guidelines. The systematic review resulted in 18 studies, which met the inclusion criteria. An improvement in phonatory function and voice quality was observed in all these studies after immediate RLN reconstruction (not always statistically significant). This improvement appears to be comparable to or even higher than that achieved with other methods of repair, and in some cases, the improvement approaches levels found in normal subjects. Intraoperative RLN reconstruction is not widely used in clinical practice, but the evidence so far makes it a viable and safe alternative to traditional techniques with better long-term results, as it prevents the occurrence of atrophy of the vocal cord and should be considered in the operating room if possible.
PubMed: 36769864
DOI: 10.3390/jcm12031212 -
International Journal of Molecular... Jan 2023PURA-related neurodevelopmental disorders (PURA-NDDs) are a rare genetic disease caused by pathogenic autosomal dominant variants in the PURA gene or a deletion... (Review)
Review
PURA-related neurodevelopmental disorders (PURA-NDDs) are a rare genetic disease caused by pathogenic autosomal dominant variants in the PURA gene or a deletion encompassing the PURA gene. PURA-NDD is clinically characterized by neurodevelopmental delay, learning disability, neonatal hypotonia, feeding difficulties, abnormal movements, and epilepsy. It is generally considered to be central nervous system disorders, with generalized weakness, associated hypotonia, cognitive and development deficits in early development, and seizures in late stages. Although it is classified predominantly as a central nervous syndrome disorder, some phenotypic features, such as myopathic facies, respiratory insufficiency of muscle origin, and myopathic features on muscle biopsy and electrodiagnostic evaluation, point to a peripheral (neuromuscular) source of weakness. Patients with PURA-NDD have been increasingly identified in exome-sequenced cohorts of patients with neuromuscular- and congenital myasthenic syndrome-like phenotypes. Recently, fluctuating weakness noted in a PURA-NDD patient, accompanied by repetitive nerve stimulation abnormalities, suggested the disease to be a channelopathy and, more specifically, a neuromuscular junction disorder. Treatment with pyridostigmine or salbutamol led to clinical improvement of neuromuscular function in two reported cases. The goal of this systematic retrospective review is to highlight the motor symptoms of PURA-NDD, to further describe the neuromuscular phenotype, and to emphasize the role of potential treatment opportunities of the neuromuscular phenotype in the setting of the potential role of PURA protein in the neuromuscular junction and the muscles.
Topics: Humans; Neuromuscular Junction; Myasthenic Syndromes, Congenital; Neurodevelopmental Disorders; Learning Disabilities; Epilepsy; Muscle Hypotonia; Nervous System Malformations; DNA-Binding Proteins; Transcription Factors
PubMed: 36768582
DOI: 10.3390/ijms24032260