Degenerative neurological and neuromuscular disease最新文献

{"title":"Interventions to Improve Quality of Life in Multiple Sclerosis: New Opportunities and Key Talking Points.","authors":"Erin Faraclas","doi":"10.2147/DNND.S395733","DOIUrl":"10.2147/DNND.S395733","url":null,"abstract":"<p><strong>Background: </strong>Today, living well with multiple sclerosis (MS) is often measured by a person's overall quality of life rather than being limited to the more traditional metrics of reduced frequency of relapses or progression of disability. This change in focus, to a more holistic view of health, such as overall quality of life, has shifted the views of what both providers and people with multiple sclerosis view as essential for living well with MS.</p><p><strong>Purpose: </strong>This narrative review aims to examine the relevant literature on existing and emerging non-pharmacological interventions shown to improve the quality of life for people with multiple sclerosis across all health domains.</p><p><strong>Methods: </strong>A literature search was conducted on MEDLINE, CINAHL, and Scopus electronic databases using the following search terms: quality of life, health-related quality of life, life quality, life satisfaction, non-pharmacological intervention, non-drug, and intervention. After screening the abstracts, 24 were selected for this review.</p><p><strong>Results: </strong>Common non-pharmacological interventions were used for fatigue and sleep, mental and emotional health, cognition, physical health, and chronic pain. Several non-pharmacological interventions included in this review positively improved the overall quality of life for people with multiple sclerosis. These interventions included exercise, cognitive behavior therapy, and cognitive rehabilitation.</p><p><strong>Conclusion: </strong>Non-pharmacological interventions such as exercise and cognitive behavioral therapy improve the quality of life for people with MS. These interventions should be prescribed more during routine medical care. Translating this research into standard clinical practice should be one area of focus. In addition, higher quality studies, such as randomized control trials, need to be conducted on emerging nonpharmacological interventions to assess effectiveness.</p>","PeriodicalId":93972,"journal":{"name":"Degenerative neurological and neuromuscular disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-09-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/09/ce/dnnd-13-55.PMC10517677.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41175848","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Effectiveness of Respiratory Muscle Training on Respiratory Muscle Strength, Pulmonary Function, and Respiratory Complications in Stroke Survivors: A Systematic Review of Randomized Controlled Trials","authors":"Sisay Deme, Dheeraj Lamba, Abayneh Alamer, Haimanot Melese, Sileshi Ayhualem, Dechassa Imeru, Tsegereda Abebe","doi":"10.2147/DNND.S348736","DOIUrl":"https://doi.org/10.2147/DNND.S348736","url":null,"abstract":"Background Stroke is the most common leading cause of mortality and related morbidities worldwide. After stroke, the motor function of extremities and spinal muscles is significantly impairment, but not only this, it also has attributable factors leading to respiratory dysfunction. Nevertheless, to the extent of the authors’ knowledge, there is a dearth of conclusive studies which examined the effectiveness of RMT on muscle strength, pulmonary function, and respiratory complications of individuals after stroke. Objective The purpose of this systematic review was to evaluate the effectiveness of respiratory muscle training on respiratory muscle strength, pulmonary function, and respiratory complications in patients after stroke. Methods An electronic database search of HINARI, PEDro, PubMed, Cochrane Library and Google scholar was used to identify randomized controlled trials that evaluated the effectiveness of respiratory muscle training in patients with stroke. Articles published from 2010 to 2019 were included. The quality of the articles was assessed using PEDro scale. Articles with abstract only, PEDro scores less than 5, published in non-English language, not freely available articles, and quasi experimental studies were excluded from this study. Results The literature search yielded a total of 7 articles (6 randomized controlled trials with 1 pilot randomized controlled trial) which met inclusion criteria despite their heterogeneity. The methodological quality of all studies ranged from 6 to 8 in Pedro score. Most of the articles reported a significant increase in respiratory muscle strength, respiratory muscle function, and reduced risk of complications with a p value <0.05. Conclusion Respiratory muscle training could potentially improve muscle strength and pulmonary functions of subjects after stroke. Thus, it may reduce stroke-related respiratory complications in subjects after stroke. However, further study is warranted with high quality RCTs and pooled synthesis of results.","PeriodicalId":93972,"journal":{"name":"Degenerative neurological and neuromuscular disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49304588","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Ponesimod in the Treatment of Relapsing Forms of Multiple Sclerosis: An Update on the Emerging Clinical Data.","authors":"Serena Ruggieri, Maria Esmeralda Quartuccio, Luca Prosperini","doi":"10.2147/DNND.S313825","DOIUrl":"10.2147/DNND.S313825","url":null,"abstract":"<p><p>Sphingosine 1-phosphate (S1P) receptors are bioactive lipid metabolites that bind five different types of receptors expressed ubiquitously in human body and mediate a broad range of biological functions. Targeting S1P receptors is nowadays a well-established pharmacological strategy to treat multiple sclerosis (MS). However, the adverse events associated with the ancestor (fingolimod), especially in terms of heart conduction and slow reversibility of its pharmacodynamics effect on lymphocytes, have stimulated a search for a S1P modulator with greater selectivity for S1P₁ (the most important immune mechanism to prevent MS-related neuroinflammation). Ponesimod is a second-generation, orally active, directly bioavailable, highly selective, and rapidly reversible modulator of the S1P₁ receptor. Gradual 14-day up-titration of ponesimod mitigates its first-dose effects on heart rate and facilitates its use over fingolimod, as it does not require first-dose cardiac monitoring. Ponesimod is rapidly eliminated within 1 week of discontinuation, thereby representing a more manageable approach in case of vaccination, pregnancy, or adverse events. However, the fast reversibility of ponesimod may also raise concerns about the possibility of a rapid reactivation of disease activity following its discontinuation. Ponesimod was recently approved for the treatment of relapsing MS forms on the basis of a Phase III, double-blind, double-dummy, randomized clinical trial (OPTIMUM) that demonstrated the superiority of ponesimod over teriflunomide on disease activity markers, without unexpected safety concerns. This review summarizes the pharmacodynamic and pharmacokinetic characteristics of ponesimod, and the main Phase II and III studies that led to its approval. Comparisons of ponesimod with other S1P receptor modulators currently available for MS (fingolimod, ozanimod, siponimod) are also provided.</p>","PeriodicalId":93972,"journal":{"name":"Degenerative neurological and neuromuscular disease","volume":null,"pages":null},"PeriodicalIF":2.1,"publicationDate":"2022-03-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8958267/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48099650","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Invasive Physiotherapy as a Treatment of Spasticity: A Systematic Review","authors":"Alberto Javier-Ormazábal, M. González-Platas, Elena González-Sierra, Marta González-Sierra","doi":"10.2147/DNND.S350192","DOIUrl":"https://doi.org/10.2147/DNND.S350192","url":null,"abstract":"Introduction Nowadays, a set of novel physiotherapy techniques have emerged, in which the physical agent used to try to reduce spasticity is applied percutaneously, specifically, through the patient’s skin. The aim of this work is to encompass all the invasive techniques used in spasticity in a single article, updating the existing bibliography. Methodology A systematic review was carried out between December 2020 and April 2021 in the Web of Science, Scopus and PubMed databases, selecting the clinical trials that used acupuncture, electroacupuncture or dry needling as a treatment for spasticity. Sixteen clinical trials were included, summarizing all the study characteristics and the outcome measures, at last the evidence was described for their results. Results Most of the studies find a difference of significant decrease in spasticity between the subjects of the experimental groups. Only four studies found no significant changes in spasticity. All the studies are carried out together with the conventional physiotherapy treatment in spasticity. Conclusion Treatment with invasive physiotherapy, combined with conventional physiotherapy, seems to have positive effects in reducing spasticity, although more studies are needed to improve the heterogeneity of the interventions and to assess their long-term effectiveness.","PeriodicalId":93972,"journal":{"name":"Degenerative neurological and neuromuscular disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48946128","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Potential Roles of Glucagon-Like Peptide-1 and Its Analogues in Dementia Targeting Impaired Insulin Secretion and Neurodegeneration","authors":"Sidharth Mehan, S. Bhalla, Ehraz Mehmood Siddiqui, Nidhi Sharma, Ambika Shandilya, Andleeb Khan","doi":"10.2147/DNND.S247153","DOIUrl":"https://doi.org/10.2147/DNND.S247153","url":null,"abstract":"Abstract Dementia is a chronic, irreversible condition marked by memory loss, cognitive decline, and mental instability. It is clinically related to various progressive neurological diseases, including Parkinson’s disease, Alzheimer’s disease, and Huntington’s. The primary cause of neurological disorders is insulin desensitization, demyelination, oxidative stress, and neuroinflammation accompanied by various aberrant proteins such as amyloid-β deposits, Lewy bodies accumulation, tau formation leading to neurofibrillary tangles. Impaired insulin signaling is directly associated with amyloid-β and α-synuclein deposition, as well as specific signaling cascades involved in neurodegenerative diseases. Insulin dysfunction may initiate various intracellular signaling cascades, including phosphoinositide 3-kinase (PI3K), c-Jun N-terminal kinases (JNK), and mitogen-activated protein kinase (MAPK). Neuronal death, inflammation, neuronal excitation, mitochondrial malfunction, and protein deposition are all influenced by insulin. Recent research has focused on GLP-1 receptor agonists as a potential therapeutic target. They increase glucose-dependent insulin secretion and are beneficial in neurodegenerative diseases by reducing oxidative stress and cytokine production. They reduce the deposition of abnormal proteins by crossing the blood-brain barrier. The purpose of this article is to discuss the role of insulin dysfunction in the pathogenesis of neurological diseases, specifically dementia. Additionally, we reviewed the therapeutic target (GLP-1) and its receptor activators as a possible treatment of dementia.","PeriodicalId":93972,"journal":{"name":"Degenerative neurological and neuromuscular disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48999730","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Progressive Multifocal Leukoencephalopathy: Current Insights.","authors":"Marge Kartau, Jussi Ot Sipilä, Eeva Auvinen, Maarit Palomäki, Auli Verkkoniemi-Ahola","doi":"10.2147/DNND.S203405","DOIUrl":"10.2147/DNND.S203405","url":null,"abstract":"<p><p>Cases of PML should be evaluated according to predisposing factors, as these subgroups differ by incidence rate, clinical course, and prognosis. The three most significant groups at risk of PML are patients with hematological malignancies mostly previously treated with immunotherapies but also untreated, patients with HIV infection, and patients using monoclonal antibody (mAb) treatments. Epidemiological data is scarce and partly conflicting, but the distribution of the subgroups appears to have changed. While there is no specific anti-JCPyV treatment, restoration of the immune function is the most effective approach to PML treatment. Research is warranted to determine whether immune checkpoint inhibitors could benefit certain PML subgroups. There are no systematic national or international records of PML diagnoses or a risk stratification algorithm, except for MS patients receiving natalizumab (NTZ). These are needed to improve PML risk assessment and to tailor better prevention strategies.</p>","PeriodicalId":93972,"journal":{"name":"Degenerative neurological and neuromuscular disease","volume":null,"pages":null},"PeriodicalIF":2.1,"publicationDate":"2019-12-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6896915/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47391281","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Orphan Drugs In Development For The Treatment Of Friedreich’s Ataxia: Focus On Omaveloxolone","authors":"Shaila D. Ghanekar, W. W. Miller, C. Meyer, Kevin J Fenelon, Alvin Lacdao, T. Zesiewicz","doi":"10.2147/DNND.S180027","DOIUrl":"https://doi.org/10.2147/DNND.S180027","url":null,"abstract":"Abstract Friedreich’s Ataxia (FRDA) is a devastating and progressive ataxia, marked by mitochondrial dysfunction and oxidative stress. Nrf2 activators such as omaveloxolone (Omav) modulate antioxidative mechanisms, and thus may be viable therapeutic agents in FRDA.","PeriodicalId":93972,"journal":{"name":"Degenerative neurological and neuromuscular disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2019-10-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2147/DNND.S180027","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49316134","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinical and research applications of neuromuscular ultrasound in amyotrophic lateral sclerosis.","authors":"Stephanie L Barnes, Neil G Simon","doi":"10.2147/DNND.S215318","DOIUrl":"10.2147/DNND.S215318","url":null,"abstract":"<p><p>Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disorder characterized by dysfunction at multiple levels of the neuraxis. It remains a clinical diagnosis without a definitive diagnostic investigation. Electrodiagnostic testing provides supportive information and, along with imaging and biochemical markers, can help exclude mimicking conditions. Neuromuscular ultrasound has a valuable role in the diagnosis and monitoring of ALS and provides complementary information to clinical assessment and electrodiagnostic testing as well as insights into the underlying pathophysiology of this disease. This review highlights the evidence for ultrasound in the evaluation of bulbar, limb and respiratory musculature and peripheral nerves in ALS. Further research in this evolving area is required.</p>","PeriodicalId":93972,"journal":{"name":"Degenerative neurological and neuromuscular disease","volume":null,"pages":null},"PeriodicalIF":2.1,"publicationDate":"2019-07-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6642653/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49435329","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Effect of the micro-immunotherapy medicine 2LPARK® on rat primary dopaminergic neurons after 6-OHDA injury: oxidative stress and survival evaluation in an in vitro model of Parkinson’s disease","authors":"N. L. Lilli, Delphine Révy, S. Robelet, B. Lejeune","doi":"10.2147/DNND.S202966","DOIUrl":"https://doi.org/10.2147/DNND.S202966","url":null,"abstract":"Background Parkinson’s disease (PD) is a neurodegenerative disease characterized by motor impairments and resulting from progressive degenerative loss of midbrain dopaminergic (DAergic) neurons in the substantia nigra. Although the main cause of the loss of DAergic neurons is still unknown, various etiopathogenic mechanisms are distinguished, including release and accumulation of endogenous excitotoxic mediators along with the production of oxidative free radicals. Several neurotrophic and growth factors are known to increase DAergic neuronal survival and enhance antioxidant mechanisms. In this context, the micro-immunotherapy (MI) approach consists to regulate the immune system in order to protect DAergic neurons and control oxidative stress. Purpose The aim of the present study was to investigate the effect of the MI medicine (MIM), 2LPARK® (Labo’Life), on oxidative stress and on the number of neurons positive for tyrosine hydroxylase (TH), in an in vitro model of PD. Methods Rat primary mesencephalic DAergic neurons cultures were pre-treated for 1 hr with the MIM (10 μM and 10 mM), placebo (10 μM and 10 mM) or brain-derived neurotrophic factor (BDNF; 3.3 μM) and then intoxicated with 6-hydroxydopamine (6-OHDA; 20 μM) for 48 hrs. After incubation, cells were incubated 30 mins at 37°C with CellROX green reagent and number of labeled cells were quantified. Then, cells were fixed and incubated with anti-TH antibody and the number of TH+ neurons was evaluated. Results We showed that, contrary to placebo, MIM was able to reduce oxidative stress and protect DAergic neurons from 6-OHDA-induced cell death. Conclusion Our results demonstrate the in vitro efficacy of MIM on two essential mechanisms of PD and propose the MI approach as a new ally in the regulation of neuroinflammation and in the treatment of this degenerative disease.","PeriodicalId":93972,"journal":{"name":"Degenerative neurological and neuromuscular disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2019-07-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2147/DNND.S202966","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49479214","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Efficacy, safety, and quality-of-life of treatments for acute relapses of multiple sclerosis: results from a literature review of randomized controlled trials","authors":"J. Costello, A. Njue, M. Lyall, A. Heyes, Nancy Mahler, M. Philbin, T. Nazareth","doi":"10.2147/DNND.S208815","DOIUrl":"https://doi.org/10.2147/DNND.S208815","url":null,"abstract":"Background Intravenous methylprednisolone (IVMP), repository corticotropin injection (RCI), plasmapheresis (PMP), and intravenous immunoglobulin (IVIG) are used in the treatment of acute multiple sclerosis (MS) relapse. A systematic literature review (SLR) of randomized controlled trials (RCTs) was conducted to examine the highest quality evidence available for these therapies. Methods English-language articles were searched in MEDLINE, Embase, and Cochrane Library through May 2016 per Preferred Reporting Items for Systematic Reviews and Meta-Analyses standards. MS conferences, SLRs, and bibliographies of included studies were also searched. Eligible studies included adults treated with ≥1 aforementioned therapy. Results Twenty-three RCTs were identified: 22 on efficacy, 11 on safety, and 3 on QOL (ie 18 IVMP, 2 RCI, 2 PMP, and 2 IVIG). IVMP and RCI improved relapse-related disability; however, IVIG and PMP showed inconsistent efficacy. QOL data were only ascertained for IVMP. Conclusions RCTs indicate IVMP and RCI are efficacious and well tolerated treatments for MS relapse. Overall, many RCTs were dated, with sample sizes of fewer than 30 patients and no definitions for relapse nor clinically significant change. Contemporary evidence generation for all relapse treatments of interest, across efficacy, safety, and QOL outcomes, is still needed.","PeriodicalId":93972,"journal":{"name":"Degenerative neurological and neuromuscular disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2019-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2147/DNND.S208815","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48642648","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}