View clinical trials related to Huntington Disease.
Filter by:The purpose of this study is to evaluate the safety, tolerability, and pharmacokinetics of SD-809 extended release (ER) in participants switching from tetrabenazine to SD-809 ER. In addition, the safety and tolerability of long-term treatment with SD-809 ER will be assessed in "Switch" participants as well as "Rollover" participants completing a randomized, double blind, placebo-controlled study of SD-809 ER.
The hypotheses of the project are 1. Diffusion MRI using compressed sensing could have reduced motion sensitivity and improved susceptibility related artifact because of accelerated acquisition. 2. The macromolecule deposition in the brain of patients with Huntington Disease (HD) can lead to changes detectible by diffusion MRI. To validate the hypothesis that the new accelerated diffusion MRI technique could produce a new biomarker for HD, patients with Huntington Disease will be recruited. The diffusion index will be calculated using accelerated acquisition. The diagnostic performance will be evaluated for data reconstructed with and without acceleration. The correlation with the disease severity will be assessed.
The purpose of this project is to study the efficacy of an anaplerotic treatment on brain energy profile evolution at an early stage of the Huntington disease.
Huntington's disease (HD) is an incurable and fatal disorder characterised by progressive degeneration of the basal ganglia and the cerebral cortex. Contrary to earlier thinking, HD is associated with abnormalities in peripheral tissues which might even contribute to brain pathology including muscle wasting, mitochondrial abnormalities, and impaired muscle energy metabolism. Mitochondrial impairment and muscle atrophy in human HD patients and murine models of HD are associated with altered expression of PGC-1a, a transcriptional cofactor that seems to regulate many, if not all of the adaptations of muscle fibres to chronic endurance training, and induces improved exercise performance and increased peak oxygen uptake. We aim at investigating whether endurance exercise has the capability of stabilizing and / or reversing PGC-1a dependent alterations of muscle function and structure in HD patients, and whether muscle training ameliorates musculoskeletal and cardiovascular function, as well as motor and cognitive symptoms in HD patients.
Huntington's Disease (HD) is an autosomal dominant disease manifested in a triad of cognitive, psychiatric, and motor signs and symptoms. HD is caused by a triplet repeat (CAG)expansion in the gene Huntingtin (HTT). This disease has classically been conceptualized as a neurodegenerative disease. However, recent evidence suggests that abnormal brain development may play an important role in the etiology of HD. Huntingtin (HTT)is expressed during development and through life. In animal studies, the HTT gene has been shown to be vital for brain development. This suggests that a mutant form of HTT (gene-expanded or CAG repeats of 40 and above) would affect normal brain development. In addition, studies in adults who are gene-expanded for HD, but have not yet manifested the illness, (pre-HD subjects) have significant changes in the structure of their brain, even up to 20 years before onset of clinical diagnosis. How far back these changes are evident is unknown. One possibility is that these brain changes are present throughout life, due to changes in brain development,though initially associated with only subtle functional abnormalities. In an effort to better understand the developmental aspects of this brain disease, the current study proposes to evaluate brain structure and function in children, adolescents, and young adults (ages 6-30) who are at risk for developing HD - those who have a parent or grandparent with HD. Brain structure will be evaluating using Magnetic Resonance Imaging (MRI) with quantitative measures of the entire brain, cerebral cortex, as well as white matter integrity via Diffusion Tensor Imaging. Brain function will be assessed by cognitive tests, behavioral assessment, and physical and neurologic evaluation. Subjects that are gene-expanded (GE) will be compared to subjects who are gene non-expanded (GNE). Changes in brain structure and/or function in the GE group compared to the GNE group would lend support to the notion that this disease has an important developmental component.
In this project investigators will evaluate the benefits of contemporary dance training using a comprehensive test battery combining standard neuropsychological batteries, psychological questionnaires on emotion, empathy and quality of life, structural magnet-resonance tomography (MRI), as well as psychophysical tests on movement recognition and agency, the sense of being in control of one's own movement. For 10 years now two experienced dancer‐choreographers lead dance workshops for people with Huntington's Disease (HD) and their family and caregivers in Paris. This project will evaluate objectively the effects these workshops have, by assessing a new group of 18 patients and their partners and caregivers before and after 8 month of weekly dance training. People with HD are troubled by involuntary movements, of which they are however not accurately aware, but moreover they become impaired at recognising instrumental actions in others. It is well known that observing somebody else's action and executing the same action rest on a common neural network. This might mean that improving one's own action execution can improve the observation and understanding of others' actions in turn. Here, investigators will investigate both the impact the movement impairments caused by HD might have on patients themselves as well as on their partners and caregivers, as a consequence of the fact that own and other action representations are shared. After 8 months of contemporary dance training, both groups will be tested again, in order to establish if both action execution (self) and perception (in others) have improved. Other recent psychophysics and brain imaging experiments have demonstrated how the sense of agency is composed from external cues (for example sound) of the consequences of movements, and from internal sensorimotor information that result from the action plan. Importantly, in HD the latter input might be impaired, but this has never been systematically tested. Making use of a psychophysics paradigm disentangling the two cues to agency investigators first monitor the sense of their own movement in HD, and further assess the changes in agency and in the role of these cues to agency after eight months of contemporary dance practice. Finally investigators will monitor the structural brain changes accompanying this progress, comparing the brain before and after regular dance practice and correlating action recognition psychophysics measures of agency with these changes. In sum, this project has a double impact. Firstly it will scientifically evaluate the impact of dance on the normal but especially the brain affected by a neurodegenerative disease that causes movement impairments, and establish its effect on behaviour and wellbeing. Secondly it will evaluate in patient partners and caregivers how they represent the patients' as well as their own movements and how this changes with dance practice.
Tetrabenazine has been shown to improve gating of abnormal visual stimuli and improve postural stability in Huntington disease (HD) patients as measured by computerized dynamic posturography testing. This study aims to elucidate whether partial dopaminergic depletion via low dose tetrabenazine has a similar effect on masking out of abnormal visual stimuli on the Stroop interference test.
This study is Single arm, Single Centre trial to check the Safety and Efficacy of Bone Marrow Derived Autologous mononuclear cell {MNC} (100 Million per dose).trial to be conducted for 36 months in patients with diabetes Mellitus in India,Primary outcome measure are Improvement in cognitive and Psychiatric Symptoms and Improvement in Jerky,random, and Uncontrollable Movements called Chorea.
This study will evaluate the Safety, Tolerability and Brain Function of 2 doses of PF-0254920 in Subjects with Early Huntington's Disease.
The purpose of this study is to determine whether SD-809 tablets are effective in the treatment of chorea associated with Huntington's Disease.