View clinical trials related to Huntington Disease.
Filter by:Huntington's disease (HD) is an inherited neurodegenerative disease for which there are no existing disease-modifying treatments. Repair-HD is an EU FP7 consortium that aims to establish all the preclinical requirements for transplantation of stem cell-derived neurons in HD in order to replace those lost to the disease process. These requirements include the generation of new clinical assessments for detailed monitoring of patients with HD who have undergone cell replacement therapy. This protocol describes the beta testing of a new clinical assessment battery: Core Assessment Protocol for Intrastriatal Transplantation in HD version 2 (CAPIT-HD beta / CAPIT-HD2). CAPIT-HD beta represents a substantial revision of a previous CAPIT-HD battery published over 20 years ago, which is in need of updating in order to accommodate knowledge from clinical transplant studies over this time and to take advantage of technological advances in patient assessment. HD is a complex disorder in which there is relentless deterioration of motor, cognitive and behavioural functions, usually from mid-life onwards. The original CAPIT battery aimed to capture elements of change in all three domains, but was based predominantly on subjective semi-quantitative assessment tools that have poor inter-rater reliability. Moreover, a number of deficits, such as impairments in social cognition, were not recognised when the original CAPIT-HD battery was constructed, so we have developed novel assessments of these deficits, some of which are included in CAPIT-HD beta. The beta testing will take place in established HD clinical centres in Cardiff, Manchester, Paris, and Munster by teams of researchers who are experienced in leading clinic research in HD. Patients with early to moderate HD will be assessed at baseline, and at one and twelve months later, to assess the reliability and sensitivity of the CAPIT-HD beta battery. Arrangements for data storage and analysis are in place.
The role of disorders of socio-emotional processes in cerebral diseases such as Alzheimer's disease, frontal temporal dementia, Parkinson's disease, Huntington's disease, traumatic brain injury, stroke, focal lesions, has been recognized recently. Social cognition refers to a large group of emotional and cognitive abilities regulating inter-individuals relationships and it includes mainly theory of mind, emotional information processing and empathy. However, assessment of socio-emotional processes is still largely based on experimental tests that are not validated for clinical purpose. In addition their long duration of administration is not adapted to clinical examination. Finally these tests have not been standardized and normalized in French-speaking population.
The aim of the study is to establish if caffeine consumption is associated with the evolution of the disease in premanifest HD.
The purpose of this study is to demonstrate engagement of pridopidine with S1R and D2R (optional) in the living human brain. No formal statistical analysis will be conducted
The purpose of this study is to investigate whether speed-dependent measures of gait can be identified in patients with neurological conditions that affect gait, particularly in subjects with parkinsonian disorders.
Cognitive impairments, especially deficits of executive function, have been well documented as a core and early feature in Huntington's disease (HD). Cognitive impairments can be considerably burdensome and devastating for people and families affected by HD. Computerised cognitive training interventions that focus on improving executive function present a potentially exciting non-pharmacological treatment option. Novel work conducted in mouse models of HD, has demonstrated that cognitive training, administered from an early stage in the disease, can improve motor performance at an older age, even in the absence of further training in the intervening time. This represents proof of principle in an animal model of HD that cognitive training can improve HD disease symptoms. Improvements associated with executive function training have also been reported in a clinical setting in a variety of neurodegenerative diseases. For example, cognitive training, can improve executive function as people age, and training specifically focused on tasks of executive function has been shown to improve both cognitive and motor outcomes in neurodegenerative diseases such as Parkinson's disease (PD) and Alzheimer's disease (AD). Therefore, this study is a feasibility study which aims to establish proof of principle for using computerised cognitive training in people with HD. The investigators propose to determine the feasibility, acceptability and gather preliminary evidence of the effectiveness of a cognitive training intervention programme, targeted for people with HD. The investigators will also aim to investigate the most appropriate outcome measures to use in this study and gather feedback on the cognitive training intervention. The investigators will also establish proof of concept via the study of brain structure and function, using MRI scanning techniques. The computerised cognitive training software and the associated outcome measures will be investigated, taking into account the views of people and families who are affected by HD. A randomised feasibility study of computerised cognitive training for people with HD will then be conducted. Participants who are randomised to the cognitive training intervention group will be asked to complete a cognitive training intervention utilising "HAPPYneuron" software. Participants in the intervention group will be asked to complete the cognitive training programme for a minimum of 30 minutes, 3 times a week for the 12 week study duration. Participants in the control group will not receive any cognitive training and will be asked to continue as normal, however they will have home visits to control for the confounding effect of social interaction. Additional monitoring and prompting for the intervention group, will be conducted via email, text or telephone reminders (as preferred by the participant) and home visits. The motor and cognitive function of participants will be assessed at the beginning and end of the study, using a range of motor and cognitive assessments. Additional cognitive measurements will be recorded as part of the HAPPYneuron programme throughout the cognitive training intervention, such as accuracy and response time measures of particular computer games. MRI scans (optional) will be conducted at the beginning and end of the study to identify any structural changes in the brain that may be associated with the cognitive training intervention. As part of the feasibility and acceptability assessment, participants, family members and carers will be invited to complete a semi-structured interview at the end of the study, if consent is obtained, focusing on using this type of software as a home based therapeutic intervention.
The aim of this study is to measure longitudinally the availability of the PDE10A enzyme in HDGECs using the radioligand [18F]MNI-659. The study will be a follow-up, examining HDGECs from the CHDIKI1201/PET-HD-PDE10A (NCT02061722) study from 18 to 28 months after the initial PET measurement.
There is a paucity of investigation into effective interventions to enhance cognitive function and/or mitigate cognitive decline in individuals with Huntington disease (HD). This study targeted working memory (WM), which is the ability to actively hold information in the mind in order to perform complex mental tasks, given reports of WM dysfunction in patients with HD. The investigators examined the feasibility of conducting a 5-week WM training program (Cogmed). Patient adherence and treatment tolerance were assessed. In addition, preliminary evidence for the efficacy of this training program on targeted cognitive abilities was examined. Nine patients with pre-manifest or early stage HD underwent training. Patients were assessed before the intervention and one week after completion.
To assess the safety and tolerability of five doses of PBF-999 (160 mg and 320mg) after repeated (8 days) single daily oral dose administration in young male and female healthy subjects.
The FuRST 2.0 scale is being developed as a Patient Reported Outcome (PRO) with information coming directly from the Huntington's Disease Gene Expansion Carrier (HDGEC) and companion through self-report. The purpose of this study is to identify real or potential comprehension or usage problems with questionnaire items or response options. Through a process of structured cognitive de-briefing with HDGEC participants and companions, independently, followed by qualitative analysis, the final phrasing of the individual items and response options for the scale will be generated. Depending on the results of the first round of cognitive pre-testing, additional rounds of cognitive pre-testing may be required.