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Deutetrabenazine Treatment Huntington Chorea PubMed articles on BioPortfolio. Our PubMed references draw on over 21 million records from the medical literature. Here you can see the latest Deutetrabenazine Treatment Huntington Chorea articles that have been published worldwide.
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Chorea, a hallmark symptom of Huntington's disease (HD), is characterized by jerky involuntary movements affecting the whole body that can interfere with daily functioning and impact health-related quality of life (HRQOL). To characterize chorea's impact on everyday functioning and HRQOL and identify patterns of perception and experiences of chorea among patients, caregivers, and providers. Data from focus groups of individuals with manifest HD (n = 8 early-stage HD; n = 16 late-stage HD), individua...
Huntington's disease is a rare, neurodegenerative disease caused by an expanded CAG repeat mutation in the huntingtin gene. Compared with adult-onset Huntington's disease, juvenile Huntington's disease (onset ≤20 years) is even rarer and has not been studied extensively. We aimed to further characterise juvenile Huntington's disease by examining the effect of CAG repeat size on disease presentation, progression, and survival.
Huntington's disease can present at almost any age but traditionally, those with an onset ≤20 years are described as having juvenile onset Huntington's disease (JOHD). They are more likely to have bradykinesia and dystonia earlier in the course of the disease. The Total Motor Score of the Unified Huntington's Disease Rating Scale (UHDRS-TMS) is often used as the principal outcome measure in clinical trials.
In this edition of the Huntington's Disease Clinical Trials Corner we expand on the GENERATION-HD1 and PACE-HD trials, and we list all currently registered and ongoing clinical trials in Huntington's disease.
For young people in families with Huntington's disease (HD) the challenge of having an affected family member (AFM) compounds challenges related to being at risk of HD themselves.
Imaging biomarkers for neurodegenerative disorders are primarily developed with the goal to aid diagnosis, to monitor disease progression, and to assess the efficacy of disease-modifying therapies in support to clinical outcomes that may either show limited sensitivity or need extended time for their evaluation. This article will review the most recent concepts and findings in the field of neuroimaging applied to Huntington's disease and Huntington-like syndromes. Emphasis will be given to the discussion of...
Physical exercise improves neurological conditions, but adherence is hard to establish. Dance might be a promising alternative; however, since patients with Huntington's disease (HD) suffer from rhythmic movement execution deficits, any metric dance practice must be avoided.
Paraneoplastic neurologic syndromes are a group of immune-mediated, cancer-associated disorders affecting the nervous system. While these syndromes are not understood fully, they are reportedly caused by an immune response against common antigens expressed by the cancer and nervous system. We describe the course of a patient who suffered paraneoplastic chorea before being diagnosed with breast cancer. A 70-year-old female presented with complaints of "shaking" movements of her head. History, physical exam f...
DriveSafe DriveAware (DSDA) has been validated as an off-road screening tool for predicting on-road driving performance in clinical populations, but its utility in people with Huntington's disease (HD) is unknown.
Huntington's disease (HD) is a rare neurodegenerative disease associated with disability and loss of patient independence. The caregivers of HD patients are at high risk for burnout. We aimed to identify variables that impact caregiver burden, as measured by the Modified Caregiver Strain Index (MCSI) and the Huntington's disease Quality of Life Battery for Carers Short Form (HD-SF). Total functional capacity and being sole caregiver were significantly associated with higher caregiver burden via MCSI. There ...
Understanding the body composition (BC) of patients with Huntington's disease (HD) could help to delay disease progression and improve treatment efficacy. The aim of this study was to assess BC parameters, including bone mineral density (BMD), and to find new biomarkers that can be early indicators for weight loss in patients with HD.
Huntington disease (HD) is a rare progressive neurodegenerative disorder of genetic origin, with no definitive treatment. Unintentional weight loss (UWL) is a clinical feature of symptomatic HD-subjects. To prevent UWL, a customized HD-diet was designed and its impact on plasma miRNA HD-footprint and neurological parameters was examined.
Huntington's disease (HD) presents with motor, cognitive, and behavioral symptoms that impair functional capacity and the ability to maintain employment. The relative contribution of cognitive decline to work disability remains controversial.
The aim of this study was to review our "real-world" experience with the vesicular monoamine transporter 2 (VMAT2) inhibitors tetrabenazine (TBZ), deutetrabenazine (DTBZ), and valbenazine (VBZ) for treatment of hyperkinetic movement disorders. Access and adherence to VMAT2 inhibitors may be limited by insurance and regulatory issues, inexperience with their use by the prescribing physician, lack of efficacy, or side effects.
Previous studies suggest that Huntingtin, the protein mutated in Huntington's disease (HD), is required for actin based changes in cell morphology, and undergoes stimulus induced targeting to plasma membranes where it interacts with phospholipids involved in cell signaling. The small GTPase Rac1 is a downstream target of growth factor stimulation and PI 3-kinase activity and is critical for actin dependent membrane remodeling.
Altered γ-aminobutyric acid signaling is believed to disrupt the excitation/inhibition balance in the striatum, which may account for the motor symptoms of Huntington's disease. Na-K-2Cl cotransporter-1 is a key molecule that controls γ-aminobutyric acid-ergic signaling. However, the role of Na-K-2Cl cotransporter-1 and efficacy of γ-aminobutyric acid-ergic transmission remain unknown in Huntington's disease.
An increased risk of suicide in Huntington's disease (HD) patients is well documented, with rates significantly higher than those of the general population as well as other neurodegenerative diseases. However, despite its prevalence, the magnitude of this phenomenon as well as its predictors and etiology are still poorly understood.
Cognitive abnormalities in Huntington's Disease (HD) can involve the specific impairment of the social perspective taking as well as difficulties in recognizing others' mental state many years before the onset of motor symptoms.
We aimed to assess whether differences in energy metabolism in fibroblast cell lines derived from patients with Huntington disease were associated with age at onset independent of the cytosine-adenine-guanine (CAG) repeat number in the mutant allele.
Huntington's disease is a chronic progressive neurodegenerative condition for which there is no disease-modifying treatment. The known genetic cause of Huntington's disease makes it possible to identify individuals destined to develop the disease and instigate treatments before the onset of symptoms. Multiple trials are already underway that target the cause of HD, yet clinical measures are often insensitive to change over typical clinical trial duration. Robust biomarkers of drug target engagement, disease...
Neuropsychiatric symptoms, such as avolition, apathy, and anhedonia, precede the onset of debilitating motor symptoms in Huntington's disease (HD), and their development may give insight into early disease progression and treatment. However, the neuronal and circuit mechanisms of premanifest HD pathophysiology are not well-understood. Here, using a transgenic rat model expressing the full-length human mutant HD gene, we find early and profound deficits in reward motivation in the absence of gross motor abno...
Although medication management is a necessary daily activity for individuals with Huntington's disease (HD), medication management abilities and their relation to cognitive functioning have not been evaluated.