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Amyotrophic Lateral Sclerosis

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1. Amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis Amyotrophic lateral sclerosis - Symptoms, diagnosis and treatment | BMJ Best Practice You'll need a subscription to access all of BMJ Best Practice Search  Amyotrophic lateral sclerosis Last reviewed: February 2019 Last updated: February 2019 Summary Progressive disease characterised by degeneration of the motor neurons with cortical, brainstem, and ventral cord locations. Usually presents as a combination of upper motor neuron and lower motor neuron symptoms (...) care is to provide supportive and palliative intervention, aiming to improve the quality of life for patients. Riluzole is used for modifying the disease course and should be offered to patients at the time of diagnosis. Continued discussions regarding advance directives, as well as methods of respiratory and nutritional support are critical; palliative care options should also be presented and discussed prior to the need for their initiation. Definition Amyotrophic lateral sclerosis (ALS

2019 BMJ Best Practice

2. Treatment of fatigue in amyotrophic lateral sclerosis/motor neuron disease. (PubMed)

Treatment of fatigue in amyotrophic lateral sclerosis/motor neuron disease. Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease (MND), is terminal, progressive neurological condition for which there are no curative treatments. Among people with ALS/MND, fatigue is a common and debilitating symptom, which is characterised by reversible motor weakness and whole-body tiredness that is only partially relieved by rest. The effectiveness of pharmacological or non-pharmacological

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2018 Cochrane

4. Mechanical ventilation for amyotrophic lateral sclerosis/motor neuron disease. (PubMed)

Mechanical ventilation for amyotrophic lateral sclerosis/motor neuron disease. Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease, is a fatal neurodegenerative disease. Neuromuscular respiratory failure is the most common cause of death, which usually occurs within two to five years of the disease onset. Supporting respiratory function with mechanical ventilation may improve survival and quality of life. This is the second update of a review first published in 2009

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2017 Cochrane

5. Gamma aminobutyric acid (GABA) modulators for amyotrophic lateral sclerosis/motor neuron disease. (PubMed)

Gamma aminobutyric acid (GABA) modulators for amyotrophic lateral sclerosis/motor neuron disease. Imbalance of gamma aminobutyric acid (GABA) and related modulators has been implicated as an important factor in the pathogenesis of amyotrophic lateral sclerosis (ALS), which is also known as motor neuron disease (MND). In this context, the role and mechanism of action of gabapentin and baclofen have been extensively investigated, although with conflicting results. This is the first systematic

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2017 Cochrane

6. Symptomatic treatments for amyotrophic lateral sclerosis/motor neuron disease. (PubMed)

Symptomatic treatments for amyotrophic lateral sclerosis/motor neuron disease. Motor neuron disease (MND), which is also known as amyotrophic lateral sclerosis (ALS), causes a wide range of symptoms but the evidence base for the effectiveness of the symptomatic treatment therapies is limited.To summarise the evidence from Cochrane Systematic Reviews of all symptomatic treatments for MND.We searched the Cochrane Database of Systematic Reviews (CDSR) on 15 November 2016 for systematic reviews

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2017 Cochrane

7. Amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis Amyotrophic lateral sclerosis - Symptoms, diagnosis and treatment | BMJ Best Practice You'll need a subscription to access all of BMJ Best Practice Search  Amyotrophic lateral sclerosis Last reviewed: February 2019 Last updated: February 2019 Summary Progressive disease characterised by degeneration of the motor neurons with cortical, brainstem, and ventral cord locations. Usually presents as a combination of upper motor neuron and lower motor neuron symptoms (...) care is to provide supportive and palliative intervention, aiming to improve the quality of life for patients. Riluzole is used for modifying the disease course and should be offered to patients at the time of diagnosis. Continued discussions regarding advance directives, as well as methods of respiratory and nutritional support are critical; palliative care options should also be presented and discussed prior to the need for their initiation. Definition Amyotrophic lateral sclerosis (ALS

2017 BMJ Best Practice

8. Cell-based therapies for amyotrophic lateral sclerosis/motor neuron disease. (PubMed)

Cell-based therapies for amyotrophic lateral sclerosis/motor neuron disease. Amyotrophic lateral sclerosis (ALS), which is also known as motor neuron disease (MND) is a fatal disease associated with rapidly progressive disability, for which no definitive treatment as yet exists. Current treatment regimens largely focus on relieving symptoms to improve the quality of life of those affected. Based on data from preclinical studies, cell-based therapy is a promising treatment for ALS/MND.To assess

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2016 Cochrane

9. Cortico-efferent tract involvement in primary lateral sclerosis and amyotrophic lateral sclerosis: A two-centre tract of interest-based DTI analysis (PubMed)

Cortico-efferent tract involvement in primary lateral sclerosis and amyotrophic lateral sclerosis: A two-centre tract of interest-based DTI analysis After the demonstration of a corticoefferent propagation pattern in amyotrophic lateral sclerosis (ALS) by neuropathological studies, this concept has been used for in vivo staging of individual patients by diffusion tensor imaging (DTI) techniques, both in `classical` ALS and in restricted phenotypes such as primary lateral sclerosis (PLS

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2018 NeuroImage : Clinical

10. Identical patterns of cortico-efferent tract involvement in primary lateral sclerosis and amyotrophic lateral sclerosis: A tract of interest-based MRI study (PubMed)

Identical patterns of cortico-efferent tract involvement in primary lateral sclerosis and amyotrophic lateral sclerosis: A tract of interest-based MRI study There is an ongoing debate whether primary lateral sclerosis (PLS) should be regarded as an independent disease entity separate from amyotrophic lateral sclerosis (ALS) or as a slowly progressive variant of ALS.The study was designed to investigate specific white matter alterations in diffusion tensor imaging (DTI) data from PLS patients

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2018 NeuroImage : Clinical

11. Bulbar impairment score and survival of stable amyotrophic lateral sclerosis patients after noninvasive ventilation initiation (PubMed)

Bulbar impairment score and survival of stable amyotrophic lateral sclerosis patients after noninvasive ventilation initiation There is general agreement that noninvasive ventilation (NIV) prolongs survival in amyotrophic lateral sclerosis (ALS) and that the main cause of NIV failure is the severity of bulbar dysfunction. However, there is no evidence that bulbar impairment is a contraindication for NIV. The aim of this study was to determine the effect of bulbar impairment on survival in ALS

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2018 ERJ open research

12. Exploring the Use of Educational Material About Shoulder Dysfunction: A Quality Improvement Project in People With Amyotrophic Lateral Sclerosis (PubMed)

Exploring the Use of Educational Material About Shoulder Dysfunction: A Quality Improvement Project in People With Amyotrophic Lateral Sclerosis Shoulder pain is a common secondary complication of amyotrophic lateral sclerosis (ALS) that can contribute to functional decline and decreased participation in daily activities. The purpose of this study was to assess the effectiveness of an educational brochure aimed at improving knowledge regarding shoulder pain and dysfunction in people with ALS

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2018 American journal of physical medicine & rehabilitation

13. RNA-Targeted Therapies and Amyotrophic Lateral Sclerosis (PubMed)

RNA-Targeted Therapies and Amyotrophic Lateral Sclerosis Amyotrophic lateral sclerosis (ALS) is a fatal motor disease in adults. Its pathophysiology remains mysterious, but tremendous advances have been made with the discovery of the most frequent mutations of its more common familial form linked to the C9ORF72 gene. Although most cases are still considered sporadic, these genetic mutations have revealed the role of RNA production, processing and transport in ALS, and may be important players

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2018 Biomedicines

14. Edaravone (Radicava) - for the treatment of amyotrophic lateral sclerosis

Edaravone (Radicava) - for the treatment of amyotrophic lateral sclerosis Search Page - Drug and Health Product Register Language selection Search and menus Search Search website Search Topics menu You are here: Summary Basis of Decision - - Health Canada Expand all Summary Basis of Decision (SBD) for Contact: Summary Basis of Decision (SBD) documents provide information related to the original authorization of a product. The for is located below. Recent Activity for SBDs written for approved

2019 Health Canada - Drug and Health Product Register

15. In silico analysis of PFN1 related to amyotrophic lateral sclerosis. (PubMed)

In silico analysis of PFN1 related to amyotrophic lateral sclerosis. Profilin 1 (PFN1) protein plays key roles in neuronal growth and differentiation, membrane trafficking, and regulation of the actin cytoskeleton. Four natural variants of PFN1 were described as related to ALS, the most common adult-onset motor neuron disorder. However, the pathological mechanism of PFN1 in ALS is not yet completely understood. The goal of this work is to thoroughly analyze the effects of the ALS-related

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2019 PLoS ONE

16. Repeated Intrathecal Mesenchymal Stem Cells for Amyotrophic Lateral Sclerosis

Repeated Intrathecal Mesenchymal Stem Cells for Amyotrophic Lateral Sclerosis To assess the safety and efficacy of 2 repeated intrathecal injections of autologous bone marrow-derived mesenchymal stem cells (BM-MSCs) in amyotrophic lateral sclerosis (ALS).In a phase 2 randomized controlled trial (NCT01363401), 64 participants with ALS were randomly assigned treatments (1:1) of riluzole alone (control group, n = 31) or combined with 2 BM-MSC injections (MSC group, n = 33). Safety was assessed (...) based on the occurrence of adverse events. The primary efficacy outcome was changes in Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R) score from baseline to 4 and 6 months postinjection. Post hoc analysis includes investigation of cerebrospinal fluid biomarkers and long-term survival analysis.Safety rating showed no groupwise difference with absence of serious treatment-related adverse events. Mean changes in ALSFRS-R scores from baseline to 4 and 6 months postinjection

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2018 EvidenceUpdates

17. Safety and efficacy of rasagiline as an add-on therapy to riluzole in patients with amyotrophic lateral sclerosis: a randomised, double-blind, parallel-group, placebo-controlled, phase 2 trial

Safety and efficacy of rasagiline as an add-on therapy to riluzole in patients with amyotrophic lateral sclerosis: a randomised, double-blind, parallel-group, placebo-controlled, phase 2 trial Rasagiline, a monoamine oxidase B inhibitor with neuroprotective potential in Parkinson's disease, has shown a disease-modifying effect in the SOD1-Gly93Ala low-expressing mouse model of amyotrophic lateral sclerosis, both alone and in combination with riluzole. We sought to test whether or not rasagiline (...) 1 mg/day can prolong survival in patients with amyotrophic lateral sclerosis also receiving riluzole.Patients with possible, probable, or definite amyotrophic lateral sclerosis were enrolled to our randomised, placebo-controlled, parallel-group, double-blind, phase 2 trial from 15 German network for motor neuron diseases (MND-NET) centres (university hospitals or clinics). Eligible patients were aged at least 18 years, had onset of progressive weakness within the 36 months before the study, had

2018 EvidenceUpdates

18. Evaluating a Gene-Environment Interaction in Amyotrophic Lateral Sclerosis: Methylmercury Exposure and Mutated SOD1 (PubMed)

Evaluating a Gene-Environment Interaction in Amyotrophic Lateral Sclerosis: Methylmercury Exposure and Mutated SOD1 Gene-environment (GxE) interactions likely contribute to numerous diseases, but are often difficult to model in the laboratory. Such interactions have been widely hypothesized for amyotrophic lateral sclerosis (ALS); recent controlled laboratory studies are discussed here and hypotheses related to possible mechanisms of action are offered. Using methylmercury exposure and mutated

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2017 Current environmental health reports

19. Is the evidence strong enough for acupuncture ameliorates clinical symptoms in patients with amyotrophic lateral sclerosis: a protocol for a systematic review and meta-analysis

Is the evidence strong enough for acupuncture ameliorates clinical symptoms in patients with amyotrophic lateral sclerosis: a protocol for a systematic review and meta-analysis Print | PDF PROSPERO This information has been provided by the named contact for this review. CRD has accepted this information in good faith and registered the review in PROSPERO. The registrant confirms that the information supplied for this submission is accurate and complete. CRD bears no responsibility or liability

2019 PROSPERO

20. Serum lipid levels in patients with amyotrophic lateral sclerosis: a meta-analysis

Serum lipid levels in patients with amyotrophic lateral sclerosis: a meta-analysis Print | PDF PROSPERO This information has been provided by the named contact for this review. CRD has accepted this information in good faith and registered the review in PROSPERO. The registrant confirms that the information supplied for this submission is accurate and complete. CRD bears no responsibility or liability for the content of this registration record, any associated files or external websites. Email

2019 PROSPERO

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