Degenerative neurological and neuromuscular disease最新文献

{"title":"Development and Validation of a Risk Prediction Model to Estimate the Risk of Stroke Among Hypertensive Patients in University of Gondar Comprehensive Specialized Hospital, Gondar, 2012 to 2022.","authors":"Yazachew Moges Chekol, Mehari Woldemariam Merid, Getayeneh Antehunegn Tesema, Tigabu Kidie Tesfie, Tsion Mulat Tebeje, Negalegn Byadgie Gelaw, Nebiyu Bekele Gebi, Wullo Sisay Seretew","doi":"10.2147/DNND.S435806","DOIUrl":"10.2147/DNND.S435806","url":null,"abstract":"<p><strong>Background: </strong>A risk prediction model to predict the risk of stroke has been developed for hypertensive patients. However, the discriminating power is poor, and the predictors are not easily accessible in low-income countries. Therefore, developing a validated risk prediction model to estimate the risk of stroke could help physicians to choose optimal treatment and precisely estimate the risk of stroke.</p><p><strong>Objective: </strong>This study aims to develop and validate a risk prediction model to estimate the risk of stroke among hypertensive patients at the University of Gondar Comprehensive Specialized Hospital.</p><p><strong>Methods: </strong>A retrospective follow-up study was conducted among 743 hypertensive patients between September 01/2012 and January 31/2022. The participants were selected using a simple random sampling technique. Model performance was evaluated using discrimination, calibration, and Brier scores. Internal validity and clinical utility were evaluated using bootstrapping and a decision curve analysis.</p><p><strong>Results: </strong>Incidence of stroke was 31.4 per 1000 person-years (95% CI: 26.0, 37.7). Combinations of six predictors were selected for model development (sex, residence, baseline diastolic blood pressure, comorbidity, diabetes, and uncontrolled hypertension). In multivariable logistic regression, the discriminatory power of the model was 0.973 (95% CI: 0.959, 0.987). Calibration plot illustrated an overlap between the probabilities of the predicted and actual observed risks after 10,000 times bootstrap re-sampling, with a sensitivity of 92.79%, specificity 93.51%, and accuracy of 93.41%. The decision curve analysis demonstrated that the net benefit of the model was better than other intervention strategies, starting from the initial point.</p><p><strong>Conclusion: </strong>An internally validated, accurate prediction model was developed and visualized in a nomogram. The model is then changed to an offline mobile web-based application to facilitate clinical applicability. The authors recommend that other researchers eternally validate the model.</p>","PeriodicalId":93972,"journal":{"name":"Degenerative neurological and neuromuscular disease","volume":"13 ","pages":"89-110"},"PeriodicalIF":0.0,"publicationDate":"2023-12-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10728309/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138806821","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":"Practical Guidance on the Use of Cladribine Tablets in the Management or Relapsing Multiple Sclerosis: Expert Opinion from Qatar.","authors":"Dirk Deleu, Beatriz Garcia Canibano, Osama Elalamy, Mohamed Sayed Abdelmoneim, Amir Boshra","doi":"10.2147/DNND.S433459","DOIUrl":"https://doi.org/10.2147/DNND.S433459","url":null,"abstract":"<p><p>The increasing availability of high-efficacy disease-modifying therapies (DMT) for the management of relapsing multiple sclerosis (RMS) has increased the potential for individualised patient management but has added complexity to the design of treatment regimens. The long-term application of immune reconstitution therapy (IRT) is supported by an increasing database of real world studies that have added important information on the long-term safety and efficacy of this approach. Cladribine tablets (CladT) is an IRT given as two annual short courses of treatment, following which a majority of patients then demonstrate no significant MS disease activity over a period of years. Whether, and how, to treat patients beyond the first two years of treatment remains a matter for debate, as clinical evidence accumulates. We, a group of neurologists who manage people with RMS in Qatar, provide our expert consensus recommendations on the application and long-term management of CladT therapy based on our experience with treatment in the last 5 years. These include pragmatic recommendations for people with MS disease activity in years 3 and 4 (ie up to four years following first dose of CladT), and for people with or without MS disease activity in subsequent years. We believe our recommendations will help to ensure the optimal application of CladT-based IRT, with the potential benefit for the patient of achieving prolonged periods free of both MS disease symptoms and the burden of regular applications of immunosuppressive DMT.</p>","PeriodicalId":93972,"journal":{"name":"Degenerative neurological and neuromuscular disease","volume":"13 ","pages":"81-88"},"PeriodicalIF":0.0,"publicationDate":"2023-12-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10725680/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138806901","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":"Neuroprotective Effects of Leptin on the APP/PS1 Alzheimer's Disease Mouse Model: Role of Microglial and Neuroinflammation.","authors":"Jing Ma,&nbsp;Yi-Hui Hou,&nbsp;Zhe-Yan Liao,&nbsp;Zheng Ma,&nbsp;Xiao-Xuan Zhang,&nbsp;Jian-Li Wang,&nbsp;Yun-Bo Zhu,&nbsp;Hai-Lei Shan,&nbsp;Ping-Yue Wang,&nbsp;Cheng-Bo Li,&nbsp;Ying-Lei Lv,&nbsp;Yi-Lan Wei,&nbsp;Jie-Zhi Dou","doi":"10.2147/DNND.S427781","DOIUrl":"10.2147/DNND.S427781","url":null,"abstract":"<p><strong>Background: </strong>Microglia are closely linked to Alzheimer's disease (AD) many years ago; however, the pathological mechanisms of AD remain unclear. The purpose of this study was to determine whether leptin affected microglia in the hippocampus of young and aged male APP/PS1 mice.</p><p><strong>Objective: </strong>In a transgenic model of AD, we investigated the association between intraperitoneal injection of leptin and microglia.</p><p><strong>Methods: </strong>We intraperitoneal injection of leptin (1mg/kg) every day for one week and analyzed inflammatory markers in microglia in the hippocampus of adult (6 months) and aged (12 months) APP/PS1 mice.</p><p><strong>Results: </strong>In all leptin treatment group, the brain Aβ levels were decrease. We found increased levels of IL-1β, IL-6 and microglial activation in the hippocampus of adult mice. Using aged mice as an experimental model for chronic neuroinflammation and leptin resistance, the number of Iba-1+ microglia and the levels of IL-1β/IL-6 in the hippocampus were greatly increased as compared to the adult. But between the leptin treatment and un-treatment, there were no difference.</p><p><strong>Conclusion: </strong>Leptin signaling would regulate the activation of microglia and the release of inflammatory factors, but it is not the only underlying mechanism in the neuroprotective effects of AD pathogenesis.</p>","PeriodicalId":93972,"journal":{"name":"Degenerative neurological and neuromuscular disease","volume":"13 ","pages":"69-79"},"PeriodicalIF":0.0,"publicationDate":"2023-10-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10613410/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"71416318","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":"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":"13 ","pages":"55-68"},"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":"12 1","pages":"75 - 84"},"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":"12 1","pages":"61-73"},"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":"12 1","pages":"23 - 29"},"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":"12 1","pages":"31 - 59"},"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":"9 1","pages":"109-121"},"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":"9 1","pages":"103 - 107"},"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}