View clinical trials related to Motor Neuron Disease.
Filter by:The purpose of this study is to study the transporters of serotonin and dopamine in ALS patients in relation with the clinical phenotype, i.e., patients without stiffness, patients with pyramidal stiffness, patients with mixed (pyramidal and extra pyramidal) stiffness. For such a goal the investigators will use SPECT to compare the binding of two specific tracers in ALS patients and in matched healthy controls.
The purpose of the study is to test whether noninvasive positive pressure ventilation (NIPPV) without expiratory positive airway pressure (EPAP) (inspiratory positive airway pressure (IPAP)-only) will result in an increase in patient usage of NIPPV compared with standard NIPPV. Secondarily, the investigators will assess measures of dyspnea, quality of life, patient satisfaction, and side effects.
Background: - Primary lateral sclerosis (PLS) is a disorder in which nerve cells in the brain that control movement degenerate. The cause of PLS is not known, but some research has suggested that environmental factors that produce oxidative stress trigger PLS in people who carry certain genes. Oxidative stress is caused when the body makes chemicals called "free radicals" faster than its natural systems can break them down. Oxidative stress can be triggered by exposures to chemicals related to the bodily effects of lead, smoking, alcohol consumption, physical activity, and psychological stress. Chemicals produced by the body during oxidative stress can be measured in the blood and urine. Researchers are interested in studying the physical, neurological, and chemical effects of PLS to better understand the effects of oxidative stress on the disorder. Objectives: - To study the relation of oxidative stress to the diagnosis and progression of motor neuron disease. Eligibility: - Individuals 20 years of age or older who have been diagnosed with PLS, and have had symptoms of PLS for at least 5 but not more than 8 years and been previously enrolled in 01-N-0145 Screening: Neurologic Disorders with Muscle Stiffness Design: - Participants will have an initial study visit and three follow-up visits. Each visit will require approximately 3 days of testing at the National Institutes of Health Clinical Center. - As part of this study, participants will have the following tests and procedures: - Neurological examination to test muscle strength, sensation, coordination, and reflexes, as well as clarity of speech - Tests of memory, attention, concentration, and thinking - Surveys on oxidative stress, including questions on life, mood, jobs held, and habit - Electromyography to record the electrical activity of muscles - Transcranial magnetic stimulation to measure electrical activity translated from their brain to the muscles - Blood, urine, and skin biopsy samples for testing and sample collection - After the initial visit, participants will have three more visits, once each in the following 3 years.
The purpose of this study is to determine the safety of injecting mesenchymal stem cells through intraspinal delivery for the treatment of ALS.
The investigators are developing a tool to help people who are severely paralyzed. This tool is called a brain-computer interface (BCI). BCIs can connect to computers or other electronic devices. This study allows a person with ALS to communicate, control their wheelchair tilt and perform other tasks using a BCI, thus increasing their independence.
People with Amyotrophic Lateral Sclerosis (ALS) will use a P300 based brain computer interface (BCI) keyboard to type in assistive technology devices. The results of this study will be compared with a previous study of a P300 BCI keyboard used by healthy volunteers.
Primary objectives: To assess the safety and tolerability of ascending doses of NP001 compared to placebo in subjects with ALS. Secondary objective: To explore the effects of NP001 on biomarkers potentially relevant to ALS.
The primary objective of this study is to demonstrate a pharmacodynamic effect of CK 2017357 on measures of skeletal muscle function or fatigability in patients with ALS.
The objective of this study will be to evaluate the safety, tolerability and effect on SOD1 levels by pyrimethamine in patients with familial amyotrophic lateral sclerosis.
A Phase I, single center, prospective, non-randomized, open label, safety/efficacy study of the infusion of autologous bone marrow-derived stem cells, in 6 patients with Amyotrophic Lateral Sclerosis according to established criteria (1), (2) with a moderate to severe diagnosis of ALS according to the World Federation of Neurology El Escorial criteria. The primary purpose of this study is to evaluate safety of the infusion procedure, as assessed by absence of complications at the site of infusion or the appearance of new neurologic deficit not attributed to the natural progression of the disease. Secondary outcomes will include a)neurological evidence of trends toward a slowing down of the decline of the forced vital capacity (FVC) (3) and of the functional rating scale (ALS-FRS) scores, as assessed at 3-month intervals, b)evidence of a decline of the maximum voluntary isometric contraction-arm (MVIC-arm) and MVIC-grip Z (4) scores and c)patient evaluation that the treatment was effective and consider the possibility of a new cell product stem cell infusion. Subjects who fulfill inclusion/exclusion criteria and sign informed consent will undergo an aspiration of bone marrow from the iliac crest for preparation of the cellular product. The day of infusion, the investigational product will be injected into the patient's intrathecal space. After cell infusion patients will be followed at WK 2, MN 1, MN 2, MN 6 and a long-term followup at MN 12 in the clinic and/or office. Electromyographic (EMG) studies, Forced vital capacity (FVC), functional rating scale (FRS) and maximum voluntary isometric contraction-arm (MVIC-arm) and MVIC-grip Z scores will have been used to assess the status of the disease before (historical record acceptable if done within three months of Screening Visit) and during the 12-month study period after cell infusion.