Active clinical trials for "Frontotemporal Dementia"

This study is a US based qualitative PRO research study to document the health insurance literacy as well as the patient experience in Niemann-Pick as it relates to accessing desired care, services and medications for patients. The outcome of this research will be used to inform various other workstreams as NNPDF works to assist families. The core research objectives are to understand the following from Niemann-Pick patients and their families in the US

It is commonly admitted that social cognition impairment, like deficit in facial emotion recognition or misinterpretation of others' intentions (Theory of Mind), are associated with social behavior disorders. This kind of disorders are observed in Fronto-Temporal Dementia (FTD), Alzheimer's Dementia (AD) and Parkinson's Disease (PD), with severe deficits in FTD and lighter deficits in AD and PD. One explanation might be that patients apply inappropriate visual exploration strategies to decode emotions and intentions of others. This study aims to test this hypothesis and further to analyse whether different patterns emerge from these pathologies.

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.

The purpose of this research is to better understand how dementia affects activity in different parts of the brain.

The primary objective of the study is to evaluate the feasibility of eliciting continuous narrative speech in different neurodegenerative and psychiatric indications, using remote, self-administered speech tasks, as measured by the average length of speech elicitation for each speech task during the first week of self-assessment. Secondary objectives include (1) evaluating the reliability of speech tasks in the remote self-administered setting, as measured by the intra- and inter-subject variance; (2) accessing the adherence of speech tasks in this setting, as measured by the subject average fraction of days during the first week, where at least one task response is submitted; (3) evaluating the feasibility of using speech tasks in the setting of a telemedicine videoconference, as measured by the average length of speech elicited in each group; (4) evaluate whether a set of acoustic and linguistic patterns can detect each indication, compare to either a control group or all other indications, as measured by the area under the receiver operating characteristic curve (AUC), sensitivity, specificity and Cohen's kappa of the relevant binary classifier; (5) evaluating how the performance of such algorithms can be impacted by speaker and environment covariates, as measured by the Kendall rank correlation coefficient of the AUC of each classifier and each of age group, gender and speech-to-reverberation modulation energy ratio.

Behavioural disorders are very common right from the initial stage of dementia and contribute to loss of autonomy. Behavioural dysexecutive disorders have a particular status due to their prevalence and their diagnostic importance, as they often constitute the initial symptoms of Frontotemporal Dementia (FTD), Semantic Dementia (SD) and Huntington's disease (HD) and they are classically more frequent in vascular dementia (VaD) than in Alzheimer's disease (AD). The presence of these disorders at the stage of Mild Cognitive Impairment (MCI) has only been partially evaluated and would increase the risk of progression to dementia. These classical data are based on non-standardized assessments and non-validated diagnostic criteria. The Groupe de Reflexion pour l'Evaluation des Fonctions EXécutives (GREFEX) has developed a standardized assessment tool for behavioural dysexecutive disorders, the Behavioural Dysexecutive Syndrome Inventory (BDSI) and has validated diagnostic criteria for this syndrome.

This study will measure the function of a protein called P-glycoprotein (P-gp), which is found at the blood-brain barrier, a membrane that normally prevents toxic material from entering the brain. Impaired P-gp function may allow toxins to enter the brain and cause some people to develop certain brain diseases. Healthy subjects and people with Alzheimer s disease, Parkinson s disease or frontotemporal dementia who are 35 years of age or older and in overall good health may be eligible for this study. Participants undergo the following procedures during three outpatient visits to the NIH Clinical Center: Medical history, psychological evaluation, physical examination and blood and urine tests, including tests for illegal and addictive drugs. PET scan: This test uses small amounts of a radioactive chemical called a tracer that labels active areas of the brain so the activity can be seen with a special camera. Before starting the scan, a catheter (plastic tube) is placed in a vein in the arm to inject the tracer. The subject lies on the scanner bed, with a special mask fitted to the head and attached to the bed to help keep the head still during the scan so the images obtained are clear. A brief initial scan is done to calibrate the scanner. Then, a radioactive tracer called [(15)O]H(2)O is injected into the catheter and the PET camera takes pictures of blood flow to the brain for about 60 seconds. Next, another tracer, [(11)C]dLop, is injected into the catheter and pictures are taken for about 2 hours to determine how much of this tracer is allowed to enter the brain. Magnetic resonance imaging (MRI): This procedure is done within 1 year (before or after) the PET scan. MRI uses a magnetic field and radio waves to produce images of the brain. For this procedure, the patient lies on a table that can slide in and out of the scanner (a tube-like device), wearing earplugs to muffle loud knocking and thumping sounds that occur during the scan.

The goal of this study is to examine olfactory function in preclinical subjects or individuals with neurological diseases such as Probable Alzheimer's Disease (PRAD), Frontotemporal Dementias (FTD), Dementia with Lewy Bodies (DLB), Traumatic Brain Injury (TBI), and Amyotrophic Lateral Sclerosis (ALS).

This is an open-label, single subject, expanded access protocol (EAP) of the LAM-002A investigational product administered orally at 125 mg BID for 52 weeks.

This study will use positron emission tomography (PET) imaging to measure a receptor in the brain that is involved in inflammation. Certain neurological disorders, possibly including frontotemporal dementia (FTD), are associated with increased inflammation in the brain. This study may help elucidate the relationship between FTD and inflammation. Patients with FTD and healthy volunteers who are 35 years of age or older may be eligible for this study. Candidates are screened with a medical history, physical examination, electrocardiogram, and blood and urine tests. Participants undergo the following procedures: Whole body PET scan: PET uses small amounts of a radioactive chemical called a tracer that labels active areas of the brain so the activity can be seen with a special camera. The tracer used in this study is [11C]PBR28. Before starting the scan, a catheter (plastic tube) is placed in a vein in the arm to inject the tracer. Pictures are taken for 1 hour. This short scan is done to determine if [11C]PBR28 binds to the subject s receptors, since a number of people do not have binding. Subjects who have binding continue with brain PET and MRI scans, described below. Brain PET imaging: Before starting the scan, a catheter is placed in a vein in the arm to inject the tracer,<TAB> and another catheter is placed in an artery in the wrist to obtain blood samples during the scan. The subject lies on the scanner bed. A special mask is fitted to the head and attached to the bed to help keep the person s head still during the scan so the images will be clear. An 8-minute transmission scan is done just before the tracer is injected to provide measures of the brain that are helpful in calculating information from subsequent scans. After the tracer is injected, pictures are taken for about 2.5 hours, while the subject lies still on the scanner bed. Blood and urine tests are done the day of and the day following each PET scan. Magnetic resonance imaging (MRI): An MRI scan is done within 1 year (before or after) of the PET scan. This procedure uses a magnetic field and radio waves to produce images of the brain. The subject lies on a table that is moved into the scanner (a tube-like device), wearing earplugs to muffle the noise of the machine during the scanning process. The test takes about 1 hour....