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Huntington's Disease - Europe PMC

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Last Updated: 11 May 2022

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Beyond the clinical context: the process of losing oneself living with Huntington's disease.

Background People with Huntington's disease have an elevated incidence of physical and cognitive impairment. Our aim is to explore how people with HD live outside of the clinical environment to better support these patients. Health and Wellbeing Starting with acceptance or denial that one is at risk of the disease, raising concern of the condition due to motor, behavioral, and cognitive changes, as well as loss of autonomy with physical dependence on another individual and loss of identity with family and family life With HD is a process that begins with acceptance or denial. Conclusion Although the regular life of patients before disease onset was defined by physical and mental/cognitive independence, with HD, they become increasingly embedded in their bodies, and their problems are related to a lack of effective curable therapy.

Source link: https://europepmc.org/article/MED/35526033


Natural History and Burden of Huntington's Disease in the UK: A Population-Based Cohort Study.

Huntington's disease is a rare neurodegenerative disease that causes progressive mental, cognitive, and motor dysfunction. This study sought to determine the epidemiology and clinical burden in people with HD relative to general population controls. Methods This cohort used general practitioner medical records to determine the prevalence and incidence of HD between Jan 2000 and Dec 2018. A cohort of incident HD cases was matched 1:3 to controls from the general population, in whom common medical diagnoses, drugs, and healthcare interventions were compared among patients at the time of first reported diagnosis and at a time close to death. Following HD diagnosis, incidence rates of common diagnoses and mortality were compared to matched controls in the time following HD diagnosis. Prevalence of psychiatric diagnoses and symptomatic medications was higher in HD patients than in controls.

Source link: https://europepmc.org/article/MED/35514071


No optical coherence tomography changes in premanifest Huntington's disease mutation carriers far from disease onset.

Background Spectral-domain optical coherence tomography imaging can reveal retinal changes as a biomarker for neurodegenerative disorders such as Huntington's disease. In a pre-manifest HD cohort and healthy controls, we investigate macular retinal layer thicknesses. Methods Pre-HD mutation carriers were tested and assessed by a preset macular OCT scan as part of a national registry. In the exploratory correlation studies with paraclinical ratings and compared to HC, the IR thickness of the ganglion cell layer, retinal nerve fiber layer, GCL + inner plexiform layer, and total retina were included. Established HD biomarker synthesis correlations were robust. The GCL, GCIPL, and total retina thicknesses of the GCL, GCIPL, and total retina did not differ between pre-HD and HC, and HC. The IR thickness of the RNFL was noticeably higher in pre-HD participants. Since our pre-HD family was more than 16 years before disease development, OCT may not be able to detect early signs of disease.

Source link: https://europepmc.org/article/MED/35511084


The Huntington's Disease Health Index: Initial Evaluation of a Disease-Specific Patient Reported Outcome Measure.

Background: When properly developed, disease-specific patient reported outcome measures have the ability to assess important changes in how a patient feels and behaves in the context of a therapeutic trial. The Huntington's Disease Health Index is a multifaceted disease-specific patient outcome survey designed specifically to meet recently released FDA recommendations for approving PROMs for product manufacturing and labeling claims. Lastly, we established the ability of the HD-HI to distinguish between groups of HD viewers with higher or lower total functional capacity scores, prodromal versus manifest HD, and normal ambulation versus mobility impairment. The total HD-HI score and each subscale score were statistically differentiated between HD participants with elevated versus low disease burden in groups. Conclusions based on the following: The HD-HI's preliminary analysis confirms the HID's reliability and reliability as a PROM for assessing how people with HD feel and function.

Source link: https://europepmc.org/article/MED/35527560


Disordered Decision Making: A Cognitive Framework for Apathy and Impulsivity in Huntington's Disease.

HD is a neurodegenerative disease with a sporadic appearance and psychiatry. HD, as well as mobility and cognitive signs, neurobehavioral abnormalities, especially apathy and impulsivity, are common signs of HD, which occur early in the disease course, often exacerbating with disease progression and drastically lowering quality of life. These findings fill the void between clinical pathology and clinical phenotype, providing new therapeutic approaches, novel behavioral and physiological biomarkers of HD, and a deeper understanding of human behaviour. We apply the neurobiological framework of cost-benefit decision making in this research to HD's problems of apathy and impulsivity. We develop a mechanistic model that clarifies the occurrence of these behavioral disorders, as well as suggestions for future treatment techniques and critical research goals through this decision-making lens. International Parkinson Movement Disorder Society, Inc. , is a nonprofit organization that promotes the International Parkinson Movement Disorder Society. Wiley Periodicals LLC's International Parkinson Movement Disorder Society is a member of the International Parkinson Movement Disorder Society.

Source link: https://europepmc.org/article/MED/35491758


Preliminary examination of early neuroconnectivity features in the R6/1 mouse model of Huntington's disease by ultra-high field diffusion MRI.

In addition, it is also possible to determine if early imaging microstructural characteristics can be related to histological biomarkers. In addition, qualitative research examining histological complexity in brain areas vulnerable to neurodegeneration could reveal more detail about inflammation events, compensatory growth of neuronectivity, and brain repair and regeneration mechanisms. Although there are some differences in volumetric changes among preclinical and clinical models, the introduction of new diffusion MRI techniques such as diffusion tensor imaging, neurite orientation dispersion, and density imaging have all been useful in determining early parameters attributed to changes in brain white and gray matter's cellular diffusion exchange. Thus, the combination of diffusion MRI imaging techniques and more complicated neuropathological analysis could lead to the discovery of new imaging biomarkers and the early detection and neuromonitoring of patients affected by HD.

Source link: https://europepmc.org/article/MED/34558512


Epidemiology of Huntington's Disease in the United States Medicare and Medicaid Populations.

Using 2016-2017 claims data from the Medicaid Analytic eXtract files for 17 states, this research used a retrospective cross-sectional approach to examine the epidemiology of HD in the U. S. Medicare and Medicaid beneficiary populations. In 2017, Medicare beneficiaries 65 years with a diagnosis of HD in 2017 and Medicaid recipients 65 years with a diagnosis of HD in 2014 were identified. The study's findings included the 2017 prevalence proportion and incidence rate of HD in the Medicare population, as well as the 2014 prevalence percentage of HD in the Medicaid population. This report indicates that the prevalence and incidence of HD in the United States may be higher than previously expected. This has a major effect on increasing the prevalence of HD among physicians and payers, as well as ensuring the availability of and access to services for HD patients and care partners in the Medicare and Medicaid populations.

Source link: https://europepmc.org/article/MED/35483335


Huntington's Disease Progression and Caregiver Burden.

Huntington's disease is a neurodegenerative, progressive disorder caused by a HTT gene mutation. When caregivers were assessed in Caregiver Burden Inventory, the patient's demographic, sociodemographic, and disease burden was calculated. No evidence for any discrepancy between clinical progress measured in UHDRS, nor biological growth measured in DB and caregiver burden progression measured in CBI were found, according to the Longitudinal study. The relationship between caregivers' burden and biological and medical change has no suggestion that caregivers' burden are overloaded with care tasks or adaptation to the situation.

Source link: https://europepmc.org/article/MED/35483333


Neuroprotective effect of CTK 01512-2 recombinant toxin at the spinal cord in a model of Huntington's disease.

In a mouse model for Huntington s disease, we investigated the consequences of intrathecal delivery of a novel toxin in a rat model. Since the spinal cord is supposed to be involved in HD motor-symptoms, we wondered if spinal cord neurons could be a therapeutic target. Ph1 is a neurotoxin from the venom of the Phoneutria nigrivent spider's venom, and it is available as CTK01512-2, a recombinant peptide. Here, we investigated whether CTK01512-2 has a neuroprotector role in a mouse model of HD. Since the spinal cord appears to be involved in HD mice's motor-symptoms, we hypothesized that spinal cord neurons could be a therapeutic target. CTK01512-2 was also able to reverse BACHD muscle atrophy. Since HD has similar symptoms to several neurodegenerative disorders, the findings published herein may also be applicable to other disorders.

Source link: https://europepmc.org/article/MED/35478205


Inosine attenuates 3-nitropropionic acid-induced Huntington's disease-like symptoms in rats via the activation of the A2AR/BDNF/TrKB/ERK/CREB signaling pathway.

Huntington's disease is an autosomal dominant neurodegenerative disease with variable symptoms, psychophysiologic signs, dementia, and early death. While investigating potential root causes, the current research sought to demonstrate the neuroprotective role of inosine in 3-nitropropionic acid-induced neurotoxicity in rats. Rats were randomly divided into five groups, and group 1 was awarded i. p. During group 4, and 5, SCH58261, a selective adenosine 2A receptor antagonist with extracellular signal-regulated kinase inhibitor, received 3-NP for 14 days, while groups 3, 4, and 5, a selective adenosine 2A receptor antagonist in group 4, and 5, a special kinase inhibitor, whereas group 5, a selective adenosine 2A receptor antagonist, received a gene inhibitor, PD98059, pharmac pharmacy, 5 days, pharmacy In the same manner, histopathological results showed reduced striatal injury score, the expression of the glial fibrillary acidic protein, and neuronal loss after inosine therapy. In conclusion, inosine attenuated 3-NP-like signs in rats, at least in part, due to the activation of the A2AR/BDNF/ERK/CREB signaling pathway.

Source link: https://europepmc.org/article/MED/35472453

* Please keep in mind that all text is summarized by machine, we do not bear any responsibility, and you should always check original source before taking any actions

* Please keep in mind that all text is summarized by machine, we do not bear any responsibility, and you should always check original source before taking any actions