View clinical trials related to Motor Neuron Disease.
Filter by:Introduction: About 50-60% of Amyotrophic Lateral Sclerosis (ALS) is characterized by hypermetabolism, defined as 10% or more excess resting energy expenditure (REE) compared to theoretical values. Harris and Benedict's (HB) formula is the equation mainly used to predict REE, but others are also applied in current practice. The present study aimed to assess REE in ALS patients compared to control populations and to compare six formulas commonly used to predict REE. Nutritional assessments were performed in ALS patients and in two control populations without hypermetabolism: healthy elderly people (control 1) and patients with non-restrictive-eating disorders (control 2). Weight, height and body composition (by bioimpedance analysis) were assessed. EE was measured (mREE) by indirect calorimetry and calculated (cREE) using HB 1919 and 1984, World Schofield, De Lorenzo, Johnstone and Mifflin formulas. Mann-Whitney and Chi2 tests were used to compare the equations.
Introduction: Resting energy expenditure (REE) formulas for healthy people (HP) are used to calculate REE (cREE) in amyotrophic lateral sclerosis (ALS) patients. In ALS an increase of measured REE (mREE) in indirect calometry (IC) compared to cREE is found in 50-60%. The aims were (i) to assess accuracy of cREE assessed using eleven formulas as compared to mREE and (ii) to create if needed a specific cREE formula for ALS patients. Method: ALS patients followed in the ALS expert center of Limoges between 1996 and 2014 and with a nutritional evaluation were included. mREE assessed with IC and cREE calculated with eleven predictive formulas (Harris Benedict (HB) 1919, HB 1984, WSchofield, De Lorenzo, Johnstone, Mifflin, WHO/FAO, Owen, Fleisch, Wang and Rosenbaum) were collected at time of diagnosis. Fat free mass (FFM) and fat mass (FM) were measured with impedancemetry. A Bland and Altman analysis was carried out. The percentage of accurate prediction ± 10%of mREE, and intraclass correlation coefficients (ICC) were calculated. Using a derivation sample, a new REE formula was created using multiple linear regression according to sex, age, FFM and FM. Accuracy of this formula was assessed in a validation sample.
In the last years research has pointed out potential mechanisms of pathogenesis in ALS including lack of degradation of abnormally accumulated proteins inside motor neurons, and an unbalanced function of the immune system leading to the prevalence of a neurotoxic function over neuroprotection. These two mechanisms contribute to ALS progression hence representing important therapeutic targets to modify disease expression. With a phase II clinical trial the investigators aim to study the biological response in ALS treated with Rapamycin, to obtain predictive information for a larger study. Eight Italian Centres will enroll 63 patients; treatment will be double blinded to patients and physicians, and will last 18 weeks.Follow up will be carried out for 36 months (total duration: 54 weeks).
This study will test the use of a new handheld device (called the N-Tidal C), that measures a person's tidal breath carbon dioxide, in diagnosing the cause of someone's breathlessness. It will also evaluate whether this device can detect when a person's breathing problem is getting worse.
This study evaluated the influence of a tailored aerobic exercise protocol on the functional outcome in ALS patients. In addition, the investigators compare some CPET variables collected during exercise testing in both groups.
ALS, also known as "Lou Gehrig's" disease, is a neurodegenerative disease which is fatal. Treatment for ALS is limited and currently consists of primary symptom relief or support. In addition, time from diagnosis to death averages 3-5 years. New Biotic, LLC has submitted an Orphan Drug Designation Application for an investigational probiotic and have indicated the need for more study of this orphaned drug in ALS patients.
Amyotrophic Lateral Sclerosis (ALS) is a motor neuron disease, which is a group of neurological disorders that selectively affect motor neurons, the cells that control voluntary muscles of the body. The disorder causes muscle weakness and atrophy throughout the body due to the degeneration of the upper and lower motor neurons. Current drugs approved for ALS treatment only modestly slow disease progression. Transcranial direct current stimulation (tDCS) is a non-invasive technique, which has been demonstrated to modulate cerebral excitability in several neurodegenerative disorders and modulate intracortical connectivity measures. In this randomized, double-blind, sham-controlled study, the investigators will evaluate whether a two-weeks' treatment with bilateral motor cortex anodal tDCS and spinal cathodal tDCS can improve symptoms in patients with amyotrophic lateral sclerosis and modulate intracortical connectivity, at short and long term.
This study will evaluate the safety and efficacy of repeated administration of NurOwn® (MSC-NTF cells) therapy, which is based on transplantation of autologous bone marrow derived mesenchymal stromal cells (MSC), which are enriched from the patient's own bone marrow, propagated ex vivo and induced to secrete Neurotrophic factors (NTFs). The autologous NurOwn® (MSC-NTF cells) are back-transplanted into the patient intrathecally by standard lumbar puncture where neurons and glial cells are expected to take up the neurotrophic factors secreted by the transplanted cells
This study will evaluate the pharmacokinetics and safety of GDC-0134 in healthy female volunteers of non-childbearing potential. The first part of the study will compare the bioavailability of a prototype capsule of GDC-0134 relative to an existing GDC-0134 reference capsule (Periods 1 and 2). The second part of the study will assess the effect of GDC-0134-in-applesauce preparation under fasting conditions, the effect of low and high fat foods as well as the effect of elevated stomach pH via pre-treatment with rabeprazole, a proton pump inhibitor (PPI), under fasted and high-fat meal conditions (Periods 3 and 4).
The specific objective of this study is to validate the practice of remote pulmonary function testing (rPFT) conducted in the home through the use of connected mobile health devices and the Penn State Hershey ALS Telemanagement program.