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"Neuromuscular diseases Physical therapy."
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Modern neuromuscular techniques
Neuromuscular techniques focus on the assessment and treatment of myofascial trigger points using thumb/finger applications. This book explains the significance of local soft tissue dysfunction.
Book
A scoping review of the contralateral effects of unilateral peripheral stimulation on neuromuscular function
It is known that resistance exercise using one limb can affect motor function of both the exercised limb and the unexercised contralateral limb, a phenomenon termed cross-education. It has been suggested that cross-education has clinical implications, e.g. in rehabilitation for orthopaedic conditions or post-stroke paresis. Much of the research on the contralateral effect of unilateral intervention on motor output is based on voluntary exercise. This scoping review aimed to map the characteristics of current literature on the cross-education caused by three most frequently utilised peripheral neuromuscular stimulation modalities in this context: electrical stimulation, mechanical vibration and percutaneous needling, that may direct future research and translate to clinical practice. A systematic search of relevant databases (Ebsco, ProQuest, PubMed, Scopus, Web of Science) through to the end of 2020 was conducted following the PRISMA Extension for Scoping Review. Empirical studies on human participants that applied a unilateral peripheral neuromuscular stimulation and assessed neuromuscular function of the stimulated and/or the unstimulated side were selected. By reading the full text, the demographic characteristics, context, design, methods and major findings of the studies were synthesised. The results found that 83 studies were eligible for the review, with the majority (53) utilised electrical stimulation whilst those applied vibration (18) or needling (12) were emerging. Although the contralateral effects appeared to be robust, only 31 studies claimed to be in the context of cross-education, and 25 investigated on clinical patients. The underlying mechanism for the contralateral effects induced by unilateral peripheral stimulation remains unclear. The findings suggest a need to enhance the awareness of cross-education caused by peripheral stimulation, to help improve the translation of theoretical concepts to clinical practice, and aid in developing well-designed clinical trials to determine the efficacy of cross-education therapies.
Journal Article
Antisense Oligonucleotide-Based Therapy for Neuromuscular Disease
Neuromuscular disorders such as Duchenne Muscular Dystrophy and Spinal Muscular Atrophy are neurodegenerative genetic diseases characterized primarily by muscle weakness and wasting. Until recently there were no effective therapies for these conditions, but antisense oligonucleotides, a new class of synthetic single stranded molecules of nucleic acids, have demonstrated promising experimental results and are at different stages of regulatory approval. The antisense oligonucleotides can modulate the protein expression via targeting hnRNAs or mRNAs and inducing interference with splicing, mRNA degradation, or arrest of translation, finally, resulting in rescue or reduction of the target protein expression. Different classes of antisense oligonucleotides are being tested in several clinical trials, and limitations of their clinical efficacy and toxicity have been reported for some of these compounds, while more encouraging results have supported the development of others. New generation antisense oligonucleotides are also being tested in preclinical models together with specific delivery systems that could allow some of the limitations of current antisense oligonucleotides to be overcome, to improve the cell penetration, to achieve more robust target engagement, and hopefully also be associated with acceptable toxicity. This review article describes the chemical properties and molecular mechanisms of action of the antisense oligonucleotides and the therapeutic implications these compounds have in neuromuscular diseases. Current strategies and carrier systems available for the oligonucleotides delivery will be also described to provide an overview on the past, present and future of these appealing molecules.
Journal Article
The ICM research agenda on intensive care unit-acquired weakness
We present areas of uncertainty concerning intensive care unit-acquired weakness (ICUAW) and identify areas for future research. Age, pre-ICU functional and cognitive state, concurrent illness, frailty, and health trajectories impact outcomes and should be assessed to stratify patients. In the ICU, early assessment of limb and diaphragm muscle strength and function using nonvolitional tests may be useful, but comparison with established methods of global and specific muscle strength and physical function and determination of their reliability and normal values would be important to advance these techniques. Serial measurements of limb and respiratory muscle strength, and systematic screening for dysphagia, would be helpful to clarify if and how weakness of these muscle groups is independently associated with outcome. ICUAW, delirium, and sedatives and analgesics may interact with each other, amplifying the effects of each individual factor. Reduced mobility in patients with hypoactive delirium needs investigations into dysfunction of central and peripheral nervous system motor pathways. Interventional nutritional studies should include muscle mass, strength, and physical function as outcomes, and prioritize elucidation of mechanisms. At follow-up, ICU survivors may suffer from prolonged muscle weakness and wasting and other physical impairments, as well as fatigue without demonstrable weakness on examination. Further studies should evaluate the prevalence and severity of fatigue in ICU survivors and define its association with psychiatric disorders, pain, cognitive impairment, and axonal loss. Finally, methodological issues, including accounting for baseline status, handling of missing data, and inclusion of patient-centered outcome measures should be addressed in future studies.
Journal Article
Electrical stimulation for neuromuscular testing and training: state-of-the art and unresolved issues
Issue Title: Special Issue : Selected Contributions of the XVIII Congress of the International Society of Electrophysiology and Kinesiology (ISEK 2010), Aalborg, Denmark on 16-19 June 2010 (pp 2391-2507)
Journal Article
Systematic review on wearable lower-limb exoskeletons for gait training in neuromuscular impairments
Gait disorders can reduce the quality of life for people with neuromuscular impairments. Therefore, walking recovery is one of the main priorities for counteracting sedentary lifestyle, reducing secondary health conditions and restoring legged mobility. At present, wearable powered lower-limb exoskeletons are emerging as a revolutionary technology for robotic gait rehabilitation. This systematic review provides a comprehensive overview on wearable lower-limb exoskeletons for people with neuromuscular impairments, addressing the following three questions: (1) what is the current technological status of wearable lower-limb exoskeletons for gait rehabilitation?, (2) what is the methodology used in the clinical validations of wearable lower-limb exoskeletons?, and (3) what are the benefits and current evidence on clinical efficacy of wearable lower-limb exoskeletons? We analyzed 87 clinical studies focusing on both device technology (e.g., actuators, sensors, structure) and clinical aspects (e.g., training protocol, outcome measures, patient impairments), and make available the database with all the compiled information. The results of the literature survey reveal that wearable exoskeletons have potential for a number of applications including early rehabilitation, promoting physical exercise, and carrying out daily living activities both at home and the community. Likewise, wearable exoskeletons may improve mobility and independence in non-ambulatory people, and may reduce secondary health conditions related to sedentariness, with all the advantages that this entails. However, the use of this technology is still limited by heavy and bulky devices, which require supervision and the use of walking aids. In addition, evidence supporting their benefits is still limited to short-intervention trials with few participants and diversity among their clinical protocols. Wearable lower-limb exoskeletons for gait rehabilitation are still in their early stages of development and randomized control trials are needed to demonstrate their clinical efficacy.
Journal Article
Mitochondrial Dysfunctions: A Red Thread across Neurodegenerative Diseases
Mitochondria play a central role in a plethora of processes related to the maintenance of cellular homeostasis and genomic integrity. They contribute to preserving the optimal functioning of cells and protecting them from potential DNA damage which could result in mutations and disease. However, perturbations of the system due to senescence or environmental factors induce alterations of the physiological balance and lead to the impairment of mitochondrial functions. After the description of the crucial roles of mitochondria for cell survival and activity, the core of this review focuses on the “mitochondrial switch” which occurs at the onset of neuronal degeneration. We dissect the pathways related to mitochondrial dysfunctions which are shared among the most frequent or disabling neurodegenerative diseases such as Alzheimer’s, Parkinson’s, and Huntington’s, Amyotrophic Lateral Sclerosis, and Spinal Muscular Atrophy. Can mitochondrial dysfunctions (affecting their morphology and activities) represent the early event eliciting the shift towards pathological neurobiological processes? Can mitochondria represent a common target against neurodegeneration? We also review here the drugs that target mitochondria in neurodegenerative diseases.
Journal Article
Current Genetic Survey and Potential Gene-Targeting Therapeutics for Neuromuscular Diseases
Neuromuscular diseases (NMDs) belong to a class of functional impairments that cause dysfunctions of the motor neuron-muscle functional axis components. Inherited monogenic neuromuscular disorders encompass both muscular dystrophies and motor neuron diseases. Understanding of their causative genetic defects and pathological genetic mechanisms has led to the unprecedented clinical translation of genetic therapies. Challenged by a broad range of gene defect types, researchers have developed different approaches to tackle mutations by hijacking the cellular gene expression machinery to minimize the mutational damage and produce the functional target proteins. Such manipulations may be directed to any point of the gene expression axis, such as classical gene augmentation, modulating premature termination codon ribosomal bypass, splicing modification of pre-mRNA, etc. With the soar of the CRISPR-based gene editing systems, researchers now gravitate toward genome surgery in tackling NMDs by directly correcting the mutational defects at the genome level and expanding the scope of targetable NMDs. In this article, we will review the current development of gene therapy and focus on NMDs that are available in published reports, including Duchenne Muscular Dystrophy (DMD), Becker muscular dystrophy (BMD), X-linked myotubular myopathy (XLMTM), Spinal Muscular Atrophy (SMA), and Limb-girdle muscular dystrophy Type 2C (LGMD2C).
Journal Article
It’s never too late - balance and endurance training improves functional performance, quality of life, and alleviates neuropathic symptoms in cancer survivors suffering from chemotherapy-induced peripheral neuropathy: results of a randomized controlled trial
Background
Chemotherapy-induced peripheral neuropathy (CIPN) can affect functional performance and quality of life considerably. Since balance training has proven to enhance physical function, it might be a promising strategy to manage CIPN-induced functional impairments.
Methods
Fifty cancer survivors with persisting CIPN after finishing their treatment were randomly allocated to an intervention (IG) or active control group (CG). The IG did endurance plus balance training, the CG only endurance training (twice weekly over 12 weeks). Pre- and post-assessments included functional performance, cardiorespiratory fitness, vibration sense, and self-reported CIPN symptoms (EORTC QLQ-CIPN20).
Results
Intention-to-treat analyses (
n
= 41) did not reveal a significant group difference (CG minus IG) for sway path in semi-tandem stance after intervention (primary endpoint), adjusted for baseline. However, our per-protocol analysis of 37 patients with training compliance ≥70% revealed: the IG reduced their sway path during semi-tandem stance (− 76 mm, 95% CI -141 – -17; CG: -6 mm, 95% CI -52 – 50), improved the duration standing on one leg on instable surface (11 s, 95% CI 8–17; CG: 0 s, 95%CI 0–5) and reported decreased motor symptoms (−8points, 95% CI -18 – 0; CG: -2points 95% CI -6 – 2). Both groups reported reduced overall- (IG: -10points, 95% CI -17 – -4; CG: -6points, 95% CI -11 – -1) and sensory symptoms (IG: -7points, 95% CI -15 – 0; CG: -7points, 95% CI -15 – 0), while only the CG exhibited objectively better vibration sense (knuckle: 0.8points, 95% CI 0.3–1.3; IG: 0.0points, 95% CI -1.1 – 0.9; patella: 1.0points, 95% CI 0.4–1.6: IG: -0.8points, 95% CI -0.2 – 0.0). Furthermore, maximum power output during cardiopulmonary exercise test increased in both groups (IG and CG: 0.1 W/kg, 95% CI 0.0–0.2), but only the CG improved their jump height (2 cm, 95% CI 0.5–3.5; IG: 1 cm, 95% CI -0.4 – 3.2).
Conclusion
We suppose that endurance training induced a reduction in sensory symptoms in both groups, while balance training additionally improved patients’ functional status. This additional functional effect might reflect the IG’s superiority in the CIPN20 motor score. Both exercises provide a clear and relevant benefit for patients with CIPN.
Trial registration
German Clinical Trials Register (DRKS) number:
DRKS00005419
, prospectively registered on November 19, 2013.
Journal Article