View clinical trials related to Neurodegenerative Disease.
Filter by:This is a phase II feasibility, safety, tolerability and preliminary efficacy study of an e-Health application versus in-person nutritional counseling to maintain or increase weight in patients with neurodegenerative diseases including amyotrophic lateral sclerosis (ALS), Parkinson's Disease (PD) and Huntington's disease (HD). Primary Objectives include the feasibility, safety, tolerability and efficacy of an e-Health application to maintain or increase body weight compared to in-person nutritional counseling. Secondary Objectives are to measure the number of calories required to maintain or increase body weight in neurodegenerative diseases at all stages of the disease. Tertiary Objectives are to test the effects of an e-Health application compared to in-person nutritional counseling on disease progression using the ALSFRS-R, UHDRS or UDysRS, on survival, and on quality of life using the PROMIS SF v1.1 scale.
The purpose of this study is to evaluate the safety of HLA-haplo matched Allogenic Bone Marrow Derived stem cells("HYNR-CS-Allo inj"), through intrathecal delivery for the treatment in patients with amyotrophic lateral sclerosis(ALS). This study is an open label, dose up and down study using the 3+3 design to assess the safety of HLA-haplo matched Allogenic Bone Marrow Derived stem cells("HYNR-CS-Allo inj")
New technologies are giving people with motor disabilities alternative communication and control channels. The investigators are interested in using the Cyberlink Control System as a hands free means to access a computer for people with Amyotrophic Lateral Sclerosis (ALS). The goal of this project is to determine whether this device is a practical and realistic means for ALS patients to communicate with only the use of facial muscle, brainwave, and eye movements. The benefit of this study may be of substantial value to many people with severe motor impairment. Additionally, it is hoped that some of the study subjects may benefit by incorporating hands-free computer use into their daily lives. This study is intended to evaluate the effectiveness of the cyberlink as a tool for daily communication compared to the standard manual letter board.
Amyotrophic lateral sclerosis (ALS) is a progressive neuromuscular condition characterized by weakness, muscle wasting, fasciculations and increased reflexes. Depending on the site of onset, individuals with ALS progressively lose control of their skeletal muscles; bulbar or the extremities. As symptoms worsen and spread, muscle atrophy becomes apparent and upper motor neuron symptoms such as spasticity complicate gait (in lower limb involvement) and manual dexterity (in upper limb involvement). The patients progress to a state of profound disability and have great difficulty in communicating; some may even be entirely "locked in" to their bodies. The capacity for simple communication could greatly improve their quality of life. New technologies are giving people with disabilities alternate communication and control options. One such instrument is the EEG-based Brain-Computer Interface (BCI) which can provide both communication and control functions to those who have lost muscle control. By recording electroencephalographic (EEG) signals or brain waves from the scalp and then decoding them, the Wadsworth BCI allows people to make selections on a computer screen [i] In this study we will be investigating the feasibility of using EEG-based Brain-Computer Interface technology as a communication solution for individuals with ALS. The specific question addressed will be: Can individuals with ALS use the BCI for communication when they present with extreme loss of neuromuscular control and severe communication impairments? The goal of the project is to determine whether this device is a practical and realistic means for individuals with ALS to communicate. The study is intended to evaluate both the complexity of the system and the degree to which each participant will be able to communicate. Trials will consist of asking the subject to follow a series of simple instructions and complete certain tasks while using the BCI. This study design requires that the individual live in the Philadelphia region. Please contact the Wadsworth Center of the New York State Department of Health and State University of New York at Albany directly if you reside outside of this area.
Amyotrophic lateral sclerosis is a uniformly progressive and fatal neurodegenerative disorder for which there is no known cure. In a novel attempt to widen the search for potential therapeutic agents, a NINDS- led cooperative group performed an in-vitro screening program of 1040 FDA approved drugs in over 28 assays relevant to various neurodegenerative disorders. Several cephalosporins showed hits in ALS relevant assays. Efficacy was noted in models suggesting increased expression of the astrocytic glutamate transporter, EAAT2, as well as models of superoxide dismutase mediated toxicity. Ceftriaxone is a third generation cephalosporin with good CNS penetration, a long half-life, and was effective in both types of ALS assays. Ceftriaxone has calcium binding activity, antioxidant properties, and rescues motor neurons in culture from chronic glutamate toxicity. Since completion of the original NINDS screen, Ceftriaxone has been shown to increase by three fold EAAT2 activity in rodent brains, due to ceftriaxone's ability to increase EAAT2 promotor activation This program is for the use of ceftriaxone in ALS for compassionate care. Currently ceftriaxone is approved by the U.S. Food and Drug Administration (FDA) for treating bacterial infections but not for treating ALS. However, there is an ongoing phase I study -by NEALS Consortium and the National Institute of Health- with three cohorts -a placebo group and two groups receiving either 2 or 4 grams of ceftriaxone daily-. Unfortunately there are only a limited number of patients being enrolled and the next phase of the project will not be undertaken until next year. At this point there are ALS patients unable to participate in this Phase I trial and unlikely to be alive when the next phase of study begins. Some of these patients want to receive the drug and are willing to pay for the drug and nursing care. We are therefore requesting a compassionate use protocol for these patients who request the medication and are willing to pay for the drug and nursing care to administer it. Dr. Terry Heiman-Patterson will supervise the administration and safety monitoring including labs for renal and hepatic function as well as IV site inspection.
Despite significant progress in the identification of mechanisms involved in motor neuron degeneration in Amyotrophic Lateral Sclerosis (ALS) and other motor system diseases, the actual pathogenesis and cause of these diseases remains unknown. Effective treatment of these diseases are dependent on the elucidation of their causes. The availability of diseased and control human tissues will be a critical resource for this research progress. . Samples of serum, spinal fluid, and urine from patients with motor system diseases can be used to study biochemical and genetic differences compared to tissues of neurologic disease controls and normal controls. Furthermore, the availability of autopsied CNS, PNS, as well as other tissues from patients with ALS or suspected ALS are useful for current and future research studies into the disease. Therefore, we propose to institute a Tissue Bank containing blood, urine, and cerebrospinal fluid donated from not only ALS and other motor neuron disease patients, but also those with other neurologic diseases and normals whose tissue can be used as controls. In addition there will be an autopsy band for post-mortem specimens of ALS and other motor neuron disease patients. Each specimen, whether from a living patient or autopsy will be de-identified and accompanied by a standard set of clinical information collected from the medical records in order that each specimen is characterized with the relevant clinical information to maximize the usefulness of the specimens. Once established, this tissue bank will provide a resource in which a large number of samples will be readily available and expedite research by circumventing the delays in collecting specimens prospectively. These specimens will be used for research in the ALS Center of Hope at Drexel University College of Medicine and shared with any outside investigator with a valid IRB approved protocol.
The purpose of a CSF repository is to collect samples of spinal fluid from controls and patients with neurologic disorders including but not exclusively ALS, Dementia, CRPS, neuropathies, and other neuromuscular diseases. This CSF repository will allow the use of CSF in biochemical studies of various neurologic diseases. It would also provide a supply of the necessary normal and disease control patients. CSF would be obtained from patients who are undergoing spinal taps for other reasons including diagnosis, treatment, or participation in clinical trials. We are proposing to collect an additional < 3 ml of CSF from a lumbar puncture that is already being performed for diagnostic or therapeutic reasons, in order to store it in our laboratory for use in future research studies. No lumbar punctures will be initiated specifically for this protocol.
This study will examine the origin and development of certain neurological diseases involving abnormal metabolism. A significant number of patients with progressive neurological disorders have not been diagnosed despite extensive workups. Lack of a specific diagnosis may amplify the distress of both the patient and family and decrease the chance of obtaining effective therapy. This study will try to advance the diagnosis and management of such patients. Patients with a metabolic neurological disease of unknown cause or one which presents an unusual or difficult management problem may be eligible for this study. This study does not include patients with known or suspected leukodystrophy. Participants will undergo various procedures, including physical and neurologic examinations, blood and urine tests, and magnetic resonance imaging (MRI) to determine the extent and severity of disease. MRI scanning uses a strong magnetic field and radio waves to show structural and chemical changes in the brain. During the procedure, the patient lies on a table in a narrow cylinder containing a magnetic field. He or she can speak with a staff member via an intercom system at all times during the procedure. Patients will also have a lumbar puncture (spinal tap) to examine the cerebrospinal fluid (CSF), which bathes the brain and spinal cord. To obtain the fluid, a local anesthetic is administered and a needle is inserted in the space between the bones in the lower back where the CSF circulates below the spinal cord. A small amount of fluid is collected through the needle. Although spinal fluid will not be examined regularly, this test may be requested during some clinic visits. X-rays, nuclear medicine scans and consultations may be obtained as needed. Other tests may include electroencephalograms (brain wave recordings), psychological tests, and speech and language and rehabilitation evaluations. A skin biopsy may be done to grow cells in culture for metabolic and genetic testing and to analyze the skin under a microscope. For the biopsy, an area of skin is numbed with an anesthetic and a small circular area is removed, using a sharp cookie cutter-type instrument. First degree relatives (parents, children or siblings) of patients with a metabolic disorder of unknown cause will be asked to provide a blood sample for DNA studies to try to identify genetic basis of the disorder. The study is expected to continue for 3 years, with yearly monitoring of patients for changes in neurological, ophthalmological and general medical status.
This study is designed to determine whether dextromethorphan, a drug commonly found in cough medicine, is beneficial and safe for the treatment of Parkinson's disease and other diseases that might share biochemical abnormalities with Parkinson's disease. Patients with Parkinson's disease are missing the chemical neurotransmitter dopamine. This occurs as a result of destructive changes in an area of the brain responsible for making dopamine, the basal ganglia. Rhythmical muscular tremors, rigidity of movement, shuffling footsteps, droopy posture, and a mask-like expression on the face characterize Parkinson's disease. Researchers believe that dextromethorphan may be able to safely modify psychomotor function of patients with Parkinson's Disease.