View clinical trials related to Motor Neuron Disease.
Filter by:Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease affecting both upper and lower motor neurons. Electroneuromyography is an important tool for the diagnosis. Previous studies have shown that different components of the blink reflex, such as the latencies of homo- and contralateral R2 responses can be affected. Studies have found that a contralateral R1 component can appear in neurological diseases with affection of the central nervous system especially upper motor neuron, such as HTLV1 infection. Thus, you aim to determine if a contralateral R1 component could be present in ALS.
The goal of this observational study is to is to ascertain the spiritual needs of palliative patients in a standardized manner using the Spiritual Needs Questionnaire and to promptly address those needs by (specialized) spiritual care. To determine whether the effort of implementing the Spiritual Needs Questionnaire on a sustainable basis on the one hand brings the expected benefit to the patients and on the other hand can be provided by the pastoral care personnel, at Muenster University Hospital (specialized) spiritual care interventions will be documented in detail and retrospectively and prospectively collected data will be compared.
The New York Stem Cell Foundation (NYSCF) Research Institute is performing this research to accelerate diverse disease research using cells from the body (such as skin or blood cells) to make stem cells and other types of cells, conduct research on the samples, perform genetic testing, and store the samples for future use. Through this research, researchers hope to identify future treatments or even cures for the major diseases of our time.
This study assesses the performance of serum neurofilament light chain (sNfL) in amyotrophic lateral sclerosis (ALS) in a wide range of disease courses, in terms of ALS progression, disease duration, and tracheostomy invasive ventilation (TIV). The aim of the research project is to investigate the correlation between NfL serum concentration and the natural course of the disease, the ALS progression rate, and specific phenotypes of ALS. Furthermore, the performance of NfL as a therapeutic biomarker will be studied. A systematic analysis of the NfL serum concentration in a cohort of 3,000 ALS patients using the Single Molecule Analysis method (SIMOA) will be performed. This analysis is carried out as a multi-center study.
Using a MRI gait motor imagery paradigm in ALS patients in order to study how ALS affects the function of the central neural networks involved in gait function, we showed a reorganization of the motor networks that represents a compensatory response to the dysfunction of the networks involved in gait function. Our main hypothesis is that by providing coherent proprioceptive input to the sensorimotor integration areas, gait training with an exoskeleton may boost compensatory network reorganization and help to maintain function. We hypothesize that this can be achieved through a locomotion training strategy that reproduces normal gait motor patterns and appropriate sensory feedback. Gait training with an exoskeleton can meet these needs. The Atalante exoskeleton offers unique potential thanks to its cutting-edge technological features, hands-free functions and availability in numerous centers across Europe. Evaluation of its safety and efficacy in ALS is of the utmost interest in order to generalize this new approach in ALS.
This study is a multicenter, 12-month open label extension study, following Phase 1 Study MON-2021-001, with a single dose of monepantel (MPL) once daily (QD) for the treatment of individuals with MND.
Veterans are at higher risk than non-Veterans of falling ill with amyotrophic lateral sclerosis (ALS). The investigators have shown that synchronized stimulation over the brain and cervical spinal cord can temporarily strengthen weakened nerve circuits between the brain and hand muscles in people with ALS. The current proposal will take the next step of individualizing this intervention, then applying it repetitively in an attempt to achieve direct clinical benefit on hand strength and function. Following an initial 2-3 month period of optimizing the intervention for each individual, the investigators will compare the effects of two-week programs of paired brain-spinal stimulation with or without hand exercises.
This is a single-center, single-arm, open-label study aiming to assess the safety and feasibility of the MyoRegulator® device when used to treat individuals with amyotrophic lateral sclerosis (ALS). This study is the first use of the MyoRegulator® device to treat individuals with ALS. The main objective of this study is to confirm that individuals with ALS can tolerate the study treatment regimen without any evidence of serious adverse events related to the use of the device. The MyoRegulator® device is a non-significant risk (NSR) investigational non-invasive neuromodulation device that uses multi-site direct current (multi-site DCS) stimulation. It has been used in two completed clinical trials evaluating its efficacy to treat post-stroke muscle spasticity and is currently being evaluated in a third trial in this post-stroke population.
The goal of this study is to improve our understanding of speech production, and to translate this into medical devices called intracortical brain-computer interfaces (iBCIs) that will enable people who have lost the ability to speak fluently to communicate via a computer just by trying to speak.
Genetic diagnosis of Amyotrophic Lateral Sclerosis (ALS) could identify the origin of the disease, potentially allowing the patient to pursue targeted/gene therapy. However, many familial forms of ALS are genetically undiagnosed, either because no variant has been detected in the genes of interest, or because the detected variant(s) have uncertain significance. Currently, molecular diagnosis takes place in two stages: 1) Search for the GGGGCC expansion in the C9ORF72 gene by RP-PCR; 2) Analysis of the coding regions by high-throughput sequencing of a panel of 30 genes involved in ALS. Many of these variants of uncertain significance affect splicing. Their impact can be predicted using in silico tools, but only an analysis of the patient's RNA can confirm their pathogenic nature. Currently, the analysis of transcripts is only done a posteriori, when a variant predicted to impact splicing is detected on the patient's DNA. RT-PCR followed by Sanger sequencing then verifies the impact of the splice variants. This method confirmed the impact of certain splice variants in patients. However, this method is time-consuming and requires custom development, and is mutation/gene/patient-dependent. In contrast, high-throughput RNA sequencing (RNA-Seq) simultaneously analyzes the splicing of numerous genes, with a global approach, applicable to all patients. This approach avoids the custom design of primers, which can be biased by the interpretation of splicing predictions, while RNA-Seq systematically captures and sequences all the transcripts. Finally, RNA-Seq provides additional information compared to DNA sequencing such as the detection of exon skipping, intron inclusion, and the creation of fusion transcripts. In the GTEx project (GTEx Consortium, 2013), expression levels of human genome transcripts were quantified by RNA-Seq. Using these results, the study investigators measured expression of transcripts of known ALS genes in whole blood. Applying a threshold value of 0.5 transcripts per million reads (TPM), 25 of the 30 ALS genes currently analyzed by NGS in routine diagnostics at Nîmes University Hospital could be eligible for a complete analysis by RNA-Seq. None of the French laboratories carrying out genetic analyzes of ALS has yet developed RNA-Seq as a routine diagnostic tool. The study laboratory receives more than 600 requests for genetic diagnosis of ALS patients per year. The aim of this study is therefore to develop a global method for analyzing RNA transcripts of ALS genes to categorize the mutations to improve the diagnostic management of patients.