View clinical trials related to Amyotrophic Lateral Sclerosis.
Filter by:The aim of this study is to investigate the usability of the 1 Minute Sit to Stand Test (1 MSTS) in evaluating physical capacity and effort-related desaturation in individuals with Amyotrophic Lateral Sclerosis (ALS). In this context, patients diagnosed with ALS who meet the inclusion criteria will be included in the study. For the physical capacity assessment of patients with ALS at different ambulatory levels included in this study, a 6 minutes walking test (6 MWT) and a 1 MSTS will be applied. Before and at the end of the test, the severity of dyspnea and leg fatigue of the patients will be determined by the Modified Borg Scale; effort-induced desaturation and heart rate by fingertip pulse oximetry; peripheral muscle strength (for the quadriceps femoris muscle) will be evaluated with a hand held dynamometer. When the studies are examined, it has been determined that the 6 MWT is relatively difficult to tolerate in individuals with ALS, it may be difficult to provide the necessary space and equipment for the test, and a shorter, easy-to-apply, and practical evaluation method that can be used instead of this test in the clinic is needed. As a result of this study, the usability of the 1 MSTS in individuals with ambulatory ALS in evaluating physical capacity and desaturation due to effort will be revealed.
Amyotrophic lateral sclerosis (ALS) is a severe neurodegenerative condition, mainly characterized by progressive weakness and wasting of the limbs, the respiratory and bulbar muscles. Respiratory insufficiency leads to a fatal outcome after a mean diseases duration of only three to five years. The disease is characterized by pathological accumulations of a protein called TDP-43, which can be found large cortical and sub-cortical areas of post-mortem ALS brains. No causal treatment for this condition is known to date, and there is a large unmet need to develop new strategies in order to halt or slow down its progression. The aim of this study is to test the safety and tolerability of Tideglusib, a treatment that is already in clinical trials for other neuromuscular conditions, in patients with ALS. It is assumed that this drug may have a significant therapeutic benefit in this population due to his mode of action: In the ALS mouse model, Tideglusib decreases significantly the amount of accumulated TDP-43 proteins within the cells.
The aim of the study is to evaluate the interest of the determination of pNFH and NFL neurofilaments in serum for the diagnosis of ALS in patients with a diagnostic standoff after evaluation in an expert ALS center. The hypothesis is that one of these biomarkers, or their combined analysis, will make it possible to confirm or invalidate the diagnosis of ALS.
This study will establish a comprehensive exon database of ALS patients, lay the foundation for screening the genes related to the occurrence and development of the disease, support the theory of ALS disease progression from peripheral to central, and reveal the correlation between the functional level of peripheral nerve and the prognosis of the disease at the gene level for the first time, and provide the basis for the mechanism research at the molecular level.
Our working hypothesis is that the injection of autologous bone marrow mononuclear cells (BMNC) has a positive effect on the natural loss of motor units and on the increase in the size of the motor unit that occurs in patients with ALS during the evolution of the disease
Thirty cases of amyotrophic lateral sclerosis patients were recruited from the neurology department of Ruijin Hospital, the pain department and the encephalopathy center of Luwan Branch of Ruijin Hospital. After the informed consent was signed, they were divided into a trial group and a control group. Each group contains 15 cases. The patients in the control group was treated with edaravone dissolved in saline during hospitalization, while the patients in the trial group was treated with edaravone, scopolamine, atropine and dexmedetomidine. Both groups of subjects were treated for 7 days within 3 weeks, followed by a buffer period of 3 weeks for observation, which was one treatment course. The total treatment protocol contains 3 treatment courses (or 18 weeks). Patients with amyotrophic lateral sclerosis were evaluated before treatment and 6, 12, 18, 24, 36, 48 weeks after treatment. The observations include whether the functional scores of patients with amyotrophic lateral sclerosis, Norris amyotrophic lateral sclerosis score, amyotrophic lateral sclerosis self-score, forced expiratory volume in one second, partial pressure of oxygen and maximum displacement of the hyoid were superior to those before treatment, and whether the partial pressure of carbon dioxide was inferior to those before treatment. Study hypothesis: Cholinergic receptor blocking therapy for amyotrophic lateral sclerosis is safe and effective in improving motor function and delaying disease progression in patients with amyotrophic lateral sclerosis.
Dyspnea; subjective experience of respiratory discomfort; which produces negative emotional experience, is the most common symptom of patients afflicted with chronic respiratory failure and its treatments are limited. Amyotrophic Lateral Sclerosis (ALS) related - dyspnea, due to diaphragmatic dysfunction, is similar to dyspnea during mechanical inspiratory load (activation of the supplementary motor area, SMA). The perception of pain and dyspnea is processed in similar brain areas (insula, dorsal anterior cingulate cortex, amygdala and medial thalamus) and in ALS; relieving dyspnea by noninvasive ventilation (NIV) is associated with decreased pain thresholds. Otherwise, it is reported systemic elevations of pro-inflammatory cytokines in chronic pain patients, correlating with intensity of pain, and during respiratory load in healthy volunteers. The objectives are to evaluate the cytokines and endorphins rates variations after initiation of NIV in ALS patients, and to correlate cytokines and endorphins rates with the intensity of the affective component and the intensity of the sensory component of dyspnea. The investigators will perform a prospective, experimental study, including 30 ALS patients. Dyspnea, ventilatory and cardiac settings, electromyographic recording of the scalene muscle and biological assays (ACTH, endorphin, Neuropeptide P, BDNF, IL1, IL6, IL8, IL10, TNF), will be measured during spontaneous breathing and during NIV at different times after initiation. The investigators expect a reduction of immunological and neurobiological markers after relieving dyspnea by NIV. This work could lead to the development of new treatments for dyspnea.
Non-invasive ventilation (NIV) in patients diagnosed with amyotrophic lateral sclerosis (ALS) is nowadays common practice to provide comfort at the end stage of the disease. As complaints vary there is the need of a non-invasive device to measure respiratory volume to objectify complaints. The ExSpiron© is a device for non-invasive monitoring of respiratory volume. The validation of this monitor in patients with ALS is the aim of this study. The hypothesis is that the ExSpiron© delivers a valid assessment of respiratory volume in patients with ALS
The research is aimed to asses the validity of the communication system improving the quality of life and the degree of sanctification of clinical patients and their caregiver.
Amyotrophic Lateral Sclerosis (ALS) is an adult-onset, devastating, neurodegenerative disease characterized by the loss of cortical, brain stem, and spinal motor neurons. Visual evoked potentials studies in patients with ALS suggest visual pathway involvement. Optic coherence tomography (OCT) is a non-invasive cross-sectional imaging modality measuring the optical reflections in biological tissues. The main objective of this observational cohort study is to explore the correlation between changes on OCT retinal parameters and and clinical disability as measured by the ALS Functional Rating Scale (ALS-FRS-r) in patients with ALS at baseline, 3 and 6 months. A secondary objective is to explore the correlation between changes in retinal OCT parameters and pulmonary function tests (FVC and FEV1) in this cohort of patients with ALS. A parallel cohort of healthy age and sex matched subjects will participate as controls to obtain reference values of their retinal layers' thickness at baseline, 3 and 6 months.