View clinical trials related to Aphasia, Primary Progressive.
Filter by:Investigations into the components of cognition damaged in frontotemporal dementia (FTD) demonstrate that patients with FTD show deficits in facial and verbal expression recognition, lack insight into what others think or might do (theory of mind skills), and in decision making tasks requiring processing of positive versus negative feedback. These cognitive functions are thought to be critical for appropriate social behavioural regulation (Blair, 2003). Recent studies in animal models and humans suggest that the neuropeptide oxytocin is an important mediator of social behavior and that oxytocin may facilitate emotion recognition, theory of mind processing, and prosocial behaviors (Donaldson and Young, 2008). Together, these findings suggest that upregulation of oxytocin dependent mechanisms of social and emotional cognition may be a valuable treatment approach in patients with FTD. The aim of this study is to determine how administration of intranasal oxytocin to patients with frontotemporal dementia affects behavior and processing of specific types of social and emotional information.The investigators' hypothesis is that oxytocin administration will improve emotional and social cognitive deficits in patients with FTD, resulting in improved decision making and behaviour.
Background: - Niemann-Pick disease type C (NPC) is a genetic disorder that results in progressive loss of nervous system function by affecting the membranes of nerve cells. There is no known cure for NPC. - N-acetyl cysteine (NAC) is a drug that has been approved by the Food and Drug Administration to use either orally or IV for the treatment of acetaminophen (Tylenol) poisoning or as an aerosol to reduce the stickiness of mucous in patients with cystic fibrosis. In the body, NAC is converted to an amino acid called cysteine, which cells can convert to a chemical called glutathione. Glutathione is important in helping cells deal with oxidative stress. Based on a number of experiments in cells, mice and patients with NPC, we believe that oxidative stress is increased in NPC. If we can increase glutathione levels, we may be able to decrease the oxidative stress. Objectives: - To test the safety and effectiveness of N-acetyl cysteine to treat Niemann-Pick disease (type C). Eligibility: - Individuals at least 1 year of age who have been diagnosed with NPC. Design: - Patients entering this study will be seen at the National Institutes of Health Clinical Center four times during the 20 weeks of the study. These admissions will occur at the start of the study and at weeks 8, 12, and 20. The first NIH visit will last 2 days, and the other visits will last 1 day. - Patients will participate in a two-stage study: a period of 8 weeks receiving NAC and a second period of 8 weeks when receiving a placebo (a pill without NAC). Every patient participating in this study will receive NAC during one of the two time periods. - The two treatment periods will be separated by a wash-out period, 4 weeks when patients will receive neither NAC nor placebo. Patients will also have a 4-week wash-out period at the beginning of the study. Most physician-prescribed medications, such as seizure medications, will be allowed. - During each visit, examinations, procedures, and tests will be done, including blood and urine samples.
Progressive aphasia is characterized by a steady and progressive loss of language skills in the presence of relatively preserved memory, attention, and thinking. The aim of this study is to slow the progression of language decline in progressive aphasia via language therapy. The first goal of this study is to improve naming abilities of individuals with progressive aphasia. This will be accomplished by carrying out an intensive treatment program for anomia. The second goal is to evaluate whether this intense language treatment re-activates affected areas and/or connections within the language network, using functional Magnetic Resonance Imaging (to measure neural activity in specific brain regions) and Diffusion Tensor Imaging tractography (to measure the connectivity between specific brain regions). This is the first study on progressive aphasia addressing both treatment and imaging in the same patients.
This is a prospective, open label, non-therapeutic, diagnostic imaging study. The purpose of this study is to utilize Pittsburgh Compound B positron emission imaging (PiB PET) to ascertain the relationship between change in amyloid burden over time, and concurrent change in clinical status.
The proposed study is designed to evaluate the performance of the COGNISION™ System as a tool to assist physicians in diagnosing Alzheimer's Disease (AD) in real-world clinical settings. The design of this study is guided by two overriding factors: 1) to optimize the performance of the event related potentials (ERP) classifiers, the subjects making up the training sets must be well characterized as to their clinical diagnosis, and 2) all ERP tests must be performed and reproduced in real-world clinical settings.
We are collecting blood samples, clinical and family information from ALS (amyotrophic lateral sclerosis) patients and their families to identify causes of ALS and ALS/dementia.
Alzheimer's disease (AD) is characterized by neuritic plaques, neurofibrillary tangles, and neuronal cell loss. Amyloid plaques are believed to play an integral role in AD. Elevated levels of Aβ in the brain are correlated with cognitive decline. There are no approved ways to measure amyloid load in humans. Several compounds are under investigation. All of these compounds use radioactive chemical tags for positron emission tomography (PET) imaging. The most promising compound is 11C-PIB, or Pittsburgh Compound-B. This compound can be injected and a PET scan performed. This allows doctors to see the amyloid plaques in the brain, and to use this information to look at other types of dementia to see if there are differences and/or similarities in the plaques. We will recruit a total of 30 subjects, 10 from each of the following three diagnostic categories: frontotemporal dementia (FTD), Alzheimer's disease, and normal volunteers. All subjects will be given an [18F]fluorodeoxyglucose or FDG-PET scan (if they haven't had one in the past) and a PIB-PET scan. The overall objective of this project is to study the biodistribution of 11C-PIB using PET imaging in normal elderly volunteers and relevant patient groups.
This is a clinical trial of bone marrow transplantation for patients with the diagnosis of a genetic disease of blood cells that do not have an HLA-matched sibling donor. Genetic diseases of blood cell include: Red blood cell defects e.g. hemoglobinopathies (sickle cell disease and thalassemia), Blackfan-Diamond anemia and congenital or chronic hemolytic anemias; White blood cells defects/immune deficiencies e.g. chronic granulomatous disease, Wiskott-Aldrich syndrome,Osteopetrosis, Kostmann's syndrome (congenital neutropenia), Hereditary Lymphohistiocytosis (HLH); Platelets defects e.g.Congenital amegakaryocytic thrombocytopenia; Metabolic/storage disorders e.g. leukodystrophies,mucopolysaccharidoses as Hurler disease;Stem cell defects e.g.reticular agenesis, among many other rare similar conditions. The study treatment plan uses a new transplant treatment regimen that aims to try to decrease the acute toxicities and complications associated with the standard treatment plans and to improve outcome The blood stem cells will be derived from either unrelated donor or unrelated umbilical cord blood.
Pick's disease, also known as Pick disease,or FTD is a rare fronto-temporal neurodegenerative disease. This study will investigate the use of far infrared radiation for the control, management and treatment of Pick's disease.
The primary objective of this clinical trial is to evaluate the ability to achieve and sustain donor engraftment in patients with lysosomal and peroxisomal inborn errors of metabolism undergoing hematopoietic stem cell transplantation (HCT).