14,571 results match your criteria Huntington Disease


Language Disorders in Huntington Disease: A Systematic Literature Review.

Cogn Behav Neurol 2018 Dec;31(4):179-192

Speech-Language Pathology Program, Department of Rehabilitation, Faculty of Medicine, Laval University, Quebec City, Quebec, Canada.

Objective: A systematic review examining the presence and nature of language disorders associated with Huntington disease (HD).

Background: HD is characterized by gradual motor dysfunction, psychiatric problems, and cognitive decline. Communication abilities in HD may be affected not only by dysarthria but also by language impairment. Read More

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http://dx.doi.org/10.1097/WNN.0000000000000171DOI Listing
December 2018

Pulmonary vein thrombosis secondary to tuberculosis in a non-HIV infected patient.

Respir Med Case Rep 2019 5;26:91-93. Epub 2018 Dec 5.

Department of Internal Medicine, Marshall University School of Medicine and Huntington VAMC, Huntington, WV, USA.

Our patient is a 67-year-old male with a past medical history significant for hypertension and hyperlipidemia came to a hospital with hemoptysis. He was also having cough and shortness of breath for the last 1 month. He said that his hemoptysis was about 1 cup per day mixed with yellowish sputum. Read More

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http://dx.doi.org/10.1016/j.rmcr.2018.11.020DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6288975PMC
December 2018

Pathologic Findings in Bronchopulmonary Leukemic Infiltrates in Patients With Chronic Lymphocytic Leukemia.

Clin Lymphoma Myeloma Leuk 2018 Nov 12. Epub 2018 Nov 12.

Division of Pulmonary, Critical Care and Sleep Medicine, Northwell Health - Zucker School of Medicine at Hofstra/Northwell, New Hyde Park, NY.

Introduction: Chronic lymphocytic leukemia (CLL) is the most common lymphoproliferative disorder worldwide. Although thoracic complications are frequent in CLL, only limited data exists regarding these complications. Pleural, parenchymal, and airway disease may occur owing to CLL itself, treatment-related adverse events, typical or opportunistic infections, or from preexisting comorbidities. Read More

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http://dx.doi.org/10.1016/j.clml.2018.11.005DOI Listing
November 2018

Effect of communicating personalized rheumatoid arthritis risk on concern for developing RA: A randomized controlled trial.

Patient Educ Couns 2018 Dec 10. Epub 2018 Dec 10.

Department of Medicine, Division of Rheumatology, Immunology and Allergy, Brigham and Women's Hospital, 60 Fenwood Road, 02115, Boston, MA, USA; Harvard Medical School, 25 Shattuck Street, 02115, Boston, MA, USA. Electronic address:

Objective: To investigate the effect of providing comprehensive personalized risk information on concern for chronic disease development.

Methods: Unaffected first-degree relatives (FDRs) of rheumatoid arthritis (RA) patients (n = 238) were randomly allocated to: 1) disclosure of RA risk personalized to demographics, genetics, biomarkers, and behaviors using a web-based tool (PRE-RA arm, n = 78); 2) PRE-RA with interpretation by a health educator (PRE-RA Plus arm, n = 80); and 3) standard RA education (Comparison arm, n = 80). Concern for developing RA was assessed at baseline and immediately, 6 weeks, 6 months, and 12 months post-intervention. Read More

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http://dx.doi.org/10.1016/j.pec.2018.12.011DOI Listing
December 2018

Which patients initiate cognitive processing therapy and prolonged exposure in department of veterans affairs PTSD clinics?

J Anxiety Disord 2018 Nov 20;62:53-60. Epub 2018 Nov 20.

Center for Chronic Disease Outcomes Research, Minneapolis VA Health Care System, One Veterans Drive, Minneapolis, MN 55417, USA; Department of Medicine, University of Minnesota, 420 Delaware St SE, Minneapolis, MN 55455, USA; Department of Psychiatry, University of Minnesota, 2450 Riverside Ave, Minneapolis, MN 55454, USA; Department of Psychology, University of Minnesota, 75 E River Rd, Minneapolis, MN 55455, USA. Electronic address:

The United States Department of Veterans Affairs (VA) provides Cognitive Processing Therapy (CPT) and Prolonged Exposure therapy (PE) for PTSD at all of its facilities, but little is known about systematic differences between patients who do and do not initiate these treatments. VA administrative data were analyzed for 6,251 veterans receiving psychotherapy over one year in posttraumatic stress disorder (PTSD) specialty clinics at nine VA medical centers. CPT and PE were initiated by 2,173 (35%) patients. Read More

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http://dx.doi.org/10.1016/j.janxdis.2018.11.003DOI Listing
November 2018
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Drp1/Fis1-mediated mitochondrial fragmentation leads to lysosomal dysfunction in cardiac models of Huntington's disease.

J Mol Cell Cardiol 2018 Dec 11. Epub 2018 Dec 11.

Department of Chemical and Systems Biology, Stanford University School of Medicine, Stanford, CA, United States. Electronic address:

Huntington's disease (HD) is a fatal hereditary neurodegenerative disorder, best known for its clinical triad of progressive motor impairment, cognitive deficits and psychiatric disturbances, is caused by CAG-repeat expansion in exon 1 of Huntingtin (HTT). However, in addition to the neurological disease, mutant HTT (mHTT), which is ubiquitously expressed in all tissues, impairs other organ systems. Not surprisingly, cardiovascular dysautonomia as well as the deterioration of circadian rhythms are among the earliest detectable pathophysiological changes in individuals with HD. Read More

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http://dx.doi.org/10.1016/j.yjmcc.2018.12.004DOI Listing
December 2018
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DNA damage signatures in peripheral blood cells (PBMC) as biomarkers in prodromal Huntington's disease.

Ann Neurol 2018 Dec 14. Epub 2018 Dec 14.

Department of Biology, University of Naples "Federico II", Naples, Italy.

Easily accessible biomarkers in Huntington disease (HD) are actively searched. We investigated telomere length (TL) and DNA double-strand breaks (histone variant pγ-H2AX) as predictive disease biomarkers in peripheral blood mononuclear cells (PBMC) from 25 pre-manifest, 58 HD patients, with similar CAG expansion in the huntingtin gene (HTT) gene and 44 healthy controls (HC). PBMC from PRE-HD and HD groups showed shorter telomeres (p<0. Read More

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http://dx.doi.org/10.1002/ana.25393DOI Listing
December 2018
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27 years of prenatal diagnosis for Huntington disease in the United Kingdom.

Genet Med 2018 Dec 14. Epub 2018 Dec 14.

School of Medicine, Medical Sciences and Nutrition, University of Aberdeen, Aberdeen, UK.

Purpose: There is little long-term, population-based data on uptake of prenatal diagnosis for Huntington disease (HD), a late-onset autosomal dominant neurodegenerative disorder, and the effect of the availability of preimplantation genetic diagnosis (PGD) on families' decisions about conventional prenatal diagnosis is not known. We report trends in prenatal diagnosis and preimplantation diagnosis for HD in the United Kingdom since services commenced.

Methods: Long-term UK-wide prospective case record-based service evaluation in 23 UK Regional Genetic Centres 1988-2015, and four UK PGD centers 2002-2015. Read More

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http://dx.doi.org/10.1038/s41436-018-0367-zDOI Listing
December 2018
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A Rare Instance of Simultaneous Infection with Disseminated Nocardia and Pulmonary Aspergillus in a Patient Receiving Treatment with Ibrutinib for Chronic Lymphocytic Leukemia.

Cureus 2018 Oct 8;10(10):e3427. Epub 2018 Oct 8.

Infectious Disease, Joan C. Edwards School of Medicine - Marshall University, Huntington, USA.

Disseminated Nocardia infections are a rare occurrence that typically occur in immunocompromised hosts. Chronic lymphocytic leukemia (CLL) is a hematologic malignancy which makes patients susceptible to infections through various mechanisms. The treatments for CLL also target immunologic pathways which can contribute to infections. Read More

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http://dx.doi.org/10.7759/cureus.3427DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6284869PMC
October 2018

De-Novo Ligand Design against Mutated Huntington Gene by Ligand-Based Pharmacophore Modeling Approach.

Curr Comput Aided Drug Des 2018 Dec 6. Epub 2018 Dec 6.

Department of Mechanical Engineering (SMME), National University of Science and Technology, Islamabad . Pakistan.

Background: Huntington's disease is characterized by three side effects, including motor disturbances, psychiatric elements, and intellectual weakness. The onset for HD has nonlinear converse associations with the number of repeat sequences of the polyglutamine mutations, so that younger patients have a tendency for longer repeats length. This HD variation is because of a development of a polyglutamine (CAG) repeats in the exon 1 of the Huntingtin protein. Read More

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http://dx.doi.org/10.2174/1573409915666181207104437DOI Listing
December 2018

High-mobility group box 1 links sensing of reactive oxygen species by huntingtin to its nuclear entry.

J Biol Chem 2018 Dec 11. Epub 2018 Dec 11.

Biochemsitry and Biomedical Sciences, McMaster University, Canada.

Huntington's disease (HD) is a neurodegenerative, age-onset disorder caused by a CAG DNA expansion in exon 1 of the HTT gene, resulting in a polyglutamine expansion in the huntingtin protein. Nuclear accumulation of mutant huntingtin is a hallmark of HD, resulting in elevated mutant huntingtin levels in cell nuclei. Huntingtin is normally retained at the endoplasmic reticulum via its N17 amphipathic alpha-helix domain but is released by oxidation of methionine eight during reactive oxygen species (ROS) stress. Read More

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http://dx.doi.org/10.1074/jbc.RA117.001440DOI Listing
December 2018
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Age- and sex-related changes in cortical and striatal nitric oxide synthase in the Q175 mouse model of Huntington's disease.

Nitric Oxide 2018 Dec 5. Epub 2018 Dec 5.

Department of Neuroscience, The Chicago Medical School at Rosalind Franklin University of Medicine and Science, North Chicago, IL, USA.

In Huntington's disease (HD), corticostriatal and striatopallidal projection neurons preferentially degenerate as a result of mutant huntingtin expression. Pathological deficits in nitric oxide (NO) signaling have also been reported in corticostriatal circuits in HD, however, the impact of age and sex on nitrergic transmission is not well characterized. Thus, we utilized NADPH-diaphorase (NADPH-d) histochemistry and qPCR assays to assess neuronal NO synthase (nNOS) activity/expression in aged male and female Q175 heterozygous mice. Read More

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http://dx.doi.org/10.1016/j.niox.2018.12.002DOI Listing
December 2018
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Clinical and genetic characteristics of late-onset Huntington's disease.

Parkinsonism Relat Disord 2018 Nov 29. Epub 2018 Nov 29.

Department of Clinical Genetics, Maastricht University Medical Center, Maastricht, the Netherlands; GROW Research Institute for Oncology and Developmental Biology, Maastricht University, Maastricht, the Netherlands.

Background: The frequency of late-onset Huntington's disease (>59 years) is assumed to be low and the clinical course milder. However, previous literature on late-onset disease is scarce and inconclusive.

Objective: Our aim is to study clinical characteristics of late-onset compared to common-onset HD patients in a large cohort of HD patients from the Registry database. Read More

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http://dx.doi.org/10.1016/j.parkreldis.2018.11.009DOI Listing
November 2018
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Lipidomic Analysis of α-Synuclein Neurotoxicity Identifies Stearoyl CoA Desaturase as a Target for Parkinson Treatment.

Mol Cell 2018 Dec 1. Epub 2018 Dec 1.

Ann Romney Center for Neurologic Diseases, Department of Neurology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA 02115, USA. Electronic address:

In Parkinson's disease (PD), α-synuclein (αS) pathologically impacts the brain, a highly lipid-rich organ. We investigated how alterations in αS or lipid/fatty acid homeostasis affect each other. Lipidomic profiling of human αS-expressing yeast revealed increases in oleic acid (OA, 18:1), diglycerides, and triglycerides. Read More

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http://dx.doi.org/10.1016/j.molcel.2018.11.028DOI Listing
December 2018

Longer schooling but not better off? A quasi-experimental study of the effect of compulsory schooling on biomarkers in France.

Soc Sci Med 2018 Nov 23;220:379-386. Epub 2018 Nov 23.

Department of Social and Behavioral Sciences, Harvard T H Chan School of Public Health, 677 Huntington Ave, Boston, MA, 02115, USA; CUNY Graduate School of Public Health and Health Policy, 205 East 42nd Street, New York, NY, 10017, USA.

Less schooling is associated with increased biological risks for chronic disease, but whether increasing years of schooling through policy interventions reduces these risks remains unclear. We examine the effect of a major education reform introduced in 1959 that raised the minimum school leaving age from 14 to 16 years in France, offering a unique natural experiment. We assess the causal impact of increased schooling duration on 16 biomarkers of cardiovascular, metabolic, organ and immune function in a large cohort of men and women born around 1953. Read More

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http://dx.doi.org/10.1016/j.socscimed.2018.11.033DOI Listing
November 2018
7 Reads

Insights into GABAergic system alteration in Huntington's disease.

Open Biol 2018 Dec 5;8(12). Epub 2018 Dec 5.

PhD Program for Translational Medicine, China Medical University and Academia Sinica, Taiwan, Republic of China

Huntington's disease (HD) is an autosomal dominant progressive neurodegenerative disease that is characterized by a triad of motor, psychiatric and cognitive impairments. There is still no effective therapy to delay or halt the disease progress. The striatum and cortex are two particularly affected brain regions that exhibit dense reciprocal excitatory glutamate and inhibitory gamma-amino butyric acid (GABA) connections. Read More

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http://dx.doi.org/10.1098/rsob.180165DOI Listing
December 2018
3 Reads

Adipocyte OGT governs diet-induced hyperphagia and obesity.

Nat Commun 2018 11 30;9(1):5103. Epub 2018 Nov 30.

Program in Integrative Cell Signaling and Neurobiology of Metabolism and Department of Comparative Medicine, Yale University School of Medicine, New Haven, CT, 06520, USA.

Palatable foods (fat and sweet) induce hyperphagia, and facilitate the development of obesity. Whether and how overnutrition increases appetite through the adipose-to-brain axis is unclear. O-linked beta-D-N-acetylglucosamine (O-GlcNAc) transferase (OGT) couples nutrient cues to O-GlcNAcylation of intracellular proteins at serine/threonine residues. Read More

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http://dx.doi.org/10.1038/s41467-018-07461-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6269424PMC
November 2018
1 Read
10.742 Impact Factor

Application of Artificial Intelligence-based Technology in Cancer Management: A Commentary on the Deployment of Artificial Neural Networks.

Anticancer Res 2018 Dec;38(12):6607-6613

School of Engineering, University of Newcastle upon Tyne, Newcastle upon Tyne, U.K.

Artificial intelligence was recognised many years ago as a potential and powerful tool to predict disease outcome in many clinical situations. The conventional approaches using statistical methods have provided much information, but are subject to limitations imposed by the complexity of medical data. The structures of the important variants of the machine learning system artificial neural networks (ANN) are discussed and emphasis is given to the powerful analytical support that could be provided by ANN for the prediction of cancer progression and prognosis. Read More

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http://ar.iiarjournals.org/lookup/doi/10.21873/anticanres.13
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http://dx.doi.org/10.21873/anticanres.13027DOI Listing
December 2018
2 Reads

Treatment of psychiatric disturbances in common hyperkinetic movement disorders.

Expert Rev Neurother 2018 Dec 1. Epub 2018 Dec 1.

b Department of Human Neurosciences , Sapienza University of Rome , Rome.

Introduction: This paper reviews studies that have assessed the treatment of psychiatric disturbances in dystonia, tic disorders, Tourette syndrome, Huntington's disease and essential tremor. Areas covered: We searched for papers in English in Pubmed using the following keywords: blepharospasm, cervical dystonia, arm dystonia, laryngeal dystonia, spasmodic dysphonia, tic disorders, Tourette syndrome, Huntington's chorea, essential tremor, depression, anxiety, obsessive compulsive disorders, attention deficit hyperactivity disorders, psychosis, apathy. Expert commentary: Although psychiatric disturbances are frequent in hyperkinetic movement disorders, few controlled studies have assessed the treatment of psychiatric disturbances in such disorders. Read More

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https://www.tandfonline.com/doi/full/10.1080/14737175.2019.1
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http://dx.doi.org/10.1080/14737175.2019.1555475DOI Listing
December 2018
4 Reads

Assessment Scales for Patients with Advanced Huntington's Disease: Comparison of the UHDRS and UHDRS-FAP.

Mov Disord Clin Pract 2018 Sep-Oct;5(5):527-533. Epub 2018 Aug 24.

Department of Neurology Leiden University Medical Center Leiden The Netherlands.

Background: The standard clinical assessment tool in Huntington's disease is the Unified Huntington's Disease Rating Scale (UHDRS). In patients with advanced Huntington's disease ceiling and floor effects of the UHDRS hamper the detection of changes. Therefore, the UHDRS-For Advanced Patients (UHDRS-FAP) has been designed for patients with late-stage Huntington's disease. Read More

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http://dx.doi.org/10.1002/mdc3.12646DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6207107PMC
August 2018
1 Read

Altered brain iron content and deposition rate in Huntington's disease as indicated by quantitative susceptibility MRI.

J Neurosci Res 2018 Nov 29. Epub 2018 Nov 29.

F.M. Kirby Research Center for Functional Brain Imaging, Kennedy Krieger Institute, Baltimore, Maryland.

Altered brain iron content in the striatum of premanifest and manifest Huntington's disease (HD) has been reported. However, its natural history remains unclear. This study aims to investigate altered brain iron content in premanifest and early HD, and the iron deposition rate in these patients through a longitudinal one-year follow-up test, with quantitative magnetic susceptibility as an iron imaging marker. Read More

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http://dx.doi.org/10.1002/jnr.24358DOI Listing
November 2018
1 Read

Gait, balance, and falls in Huntington disease.

Handb Clin Neurol 2018 ;159:251-260

Sydney School of Public Health, University of Sydney, Sydney, NSW, Australia. Electronic address:

Huntington disease (HD) is an autosomal-dominant, progressive, neurodegenerative disorder, characterized by involuntary movements and other motor impairments, cognitive/behavioral symptoms, and psychiatric disorders. Gait and balance impairments and falls greatly impact on the quality of life among people with HD, and being fall-prone is one of the strongest predictors of nursing-home placement. Gait impairment in HD is characterized by bradykinesia, reduced velocity, and increased variability in spatiotemporal features. Read More

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http://dx.doi.org/10.1016/B978-0-444-63916-5.00016-1DOI Listing
January 2018
1 Read

Effects of Stimulus-Related Variables on Mental States Recognition in Huntington'S Disease.

Int J Neurosci 2018 Nov 27:1-21. Epub 2018 Nov 27.

b Huntington and Rare Diseases Unit at IRCCS Casa Sollievo della Sofferenza Hospital (Rome CSS-Mendel) , San Giovanni Rotondo , Italy.

Background: 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.

Aims: At the scope of assessing how the difficulties in mental state recognition might be an HD early sign before motor symptoms appear, our study was aimed to investigate how the recognition of others' mental states in HD subjects is moderated by different stimulus related features (gender, difficulty (low, medium, high) and valence (positive, negative, neutral) of the mental states that are to be recognized).

Methods: Subjects with pre-manifest (n = 20) and manifest (n = 40) HD performed the revised "Reading the Mind in the Eyes Test" and were compared with age-matched healthy controls (HC, 40 subjects per cohort). Read More

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http://dx.doi.org/10.1080/00207454.2018.1552691DOI Listing
November 2018
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DNA Repair Signaling of Huntingtin: The Next Link Between Late-Onset Neurodegenerative Disease and Oxidative DNA Damage.

DNA Cell Biol 2018 Nov 27. Epub 2018 Nov 27.

Department of Biochemistry and Biomedical Sciences, McMaster University , Hamilton, Canada .

A new hypothesis for the mechanism of Huntington's disease (HD) is driven by a small molecule lead that may connect age-associated reactive oxygen stress, oxidative DNA damage, and mitochondrial dysfunction. These pathways have also recently been defined in genome-wide association studies of cytosine-adenine-guanine-expansion polyglutamine neurodegenerative diseases, including HD and the spinocerebellar ataxias. We discuss how N6-furfuryladenine (N6FFA) nucleotide salvage and role as a kinase neosubstrate may have important mechanistic implications for both HD and familial Parkinson's disease. Read More

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http://dx.doi.org/10.1089/dna.2018.4476DOI Listing
November 2018
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Imaging Transplantation in Movement Disorders.

Int Rev Neurobiol 2018 3;143:213-263. Epub 2018 Nov 3.

Neurodegeneration Imaging Group, Maurice Wohl Clinical Neuroscience Institute, Institute of Psychiatry, Psychology and Neuroscience (IoPPN), King's College London, London, United Kingdom. Electronic address:

Cell replacement therapy with graft transplantation has been tested as a disease-modifying treatment in neurodegenerative diseases characterized by the damage of a predominant cell type, such as substantia nigra dopaminergic neurons in Parkinson's disease (PD) or striatal medium spiny projection neurons in Huntington's disease (HD). The results of these trials are mixed with success in preclinical and pilot open-label trials, which were not consistently reproduced in randomized controlled trials. Positron emission tomography (PET) and single photon emission computed tomography (SPECT) molecular imaging and functional magnetic resonance imaging allow the graft survival, and its relationship with the host tissues to be studied in vivo. Read More

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http://dx.doi.org/10.1016/bs.irn.2018.10.002DOI Listing
November 2018
1 Read

Molecular Pathogenesis in Huntington's Disease.

Biochemistry (Mosc) 2018 Sep;83(9):1030-1039

Institute of Cytology, Russian Academy of Sciences, St. Petersburg, 194064, Russia.

Huntington's disease (HD) is a severe autosomal dominant neurodegenerative disorder characterized by a combination of motor, cognitive, and psychiatric symptoms, atrophy of the basal ganglia and the cerebral cortex, and inevitably progressive course resulting in death 5-20 years after manifestation of its symptoms. HD is caused by expansion of CAG repeats in the HTT gene, which leads to pathological elongation of the polyglutamine tract within the respective protein - huntingtin. In this review, we present a modern view on molecular biology of HD as a representative of the group of polyglutamine diseases, with an emphasis on conformational changes of mutant huntingtin, disturbances in its cellular processing, and proteolytic stress in degenerating neurons. Read More

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http://dx.doi.org/10.1134/S0006297918090043DOI Listing
September 2018
2 Reads

Body composition and bone mineral density in Huntington's disease.

Nutrition 2018 Aug 21;59:145-149. Epub 2018 Aug 21.

Section of Clinical Nutrition and Nutrigenomic, Department of Biomedicine and prevention, University of Rome Tor Vergata, Rome, Italy; Casa di Cura Madonna dello Scoglio, Cotronei, Italy. Electronic address:

Objective: 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.

Methods: Twenty-one age- and sex-matched patients with HD and 29 healthy controls (CT) were enrolled. Read More

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http://dx.doi.org/10.1016/j.nut.2018.08.005DOI Listing
August 2018
6 Reads

Adherence to the 2015 Dietary Guidelines for Americans and mortality risk in a Mediterranean cohort: The SUN project.

Prev Med 2018 Nov 20;118:317-324. Epub 2018 Nov 20.

University of Navarra, Medical School, Department of Preventive Medicine and Public Health, Irunlarrea 1, 31008 Pamplona, Spain; Navarra Institute for Health Research (IdisNa), Calle de Irunlarrea, 3, 31008 Pamplona, Spain; CIBER Physiopathology of Obesity and Nutrition (CIBERobn), Carlos III Institute of Health, Calle de Melchor Fernández Almagro, 3, 28029 Madrid, Spain. Electronic address:

The 2015-2020 Dietary Guidelines for Americans (DGA) was issued in early 2016. It remains untested if adherence to these guidelines could reduce mortality risk. Using a modified version of the 2015 Dietary Guidelines for American Index (2015 DGAI), we investigated if adherence to the new DGA is associated with mortality in a Spanish (the Seguimiento Universidad de Navarra, SUN) cohort. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00917435183036
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http://dx.doi.org/10.1016/j.ypmed.2018.11.015DOI Listing
November 2018
3 Reads

The Clinical Picture of Psychosis in Manifest Huntington's Disease: A Comprehensive Analysis of the Enroll-HD Database.

Front Neurol 2018 6;9:930. Epub 2018 Nov 6.

Department of Psychiatry and Behavioral Sciences, McGovern Medical School, The University of Texas Health Science Center at Houston (UTHealth), Houston, TX, United States.

Psychotic symptoms have been under-investigated in Huntington's disease (HD) and research is needed in order to elucidate the characteristics linked to the unique phenotype of HD patients presenting with psychosis. To evaluate the frequency and factors associated with psychosis in HD. Cross-sectional study including manifest individuals with HD from the Enroll-HD database. Read More

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http://dx.doi.org/10.3389/fneur.2018.00930DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6232301PMC
November 2018
7 Reads

Thermoregulatory disorders in Huntington disease.

Handb Clin Neurol 2018 ;157:761-775

Translational Neuroendocrine Research Unit, Department of Experimental Medical Sciences, Lund University, Lund, Sweden.

Huntington disease (HD) is a paradigmatic autosomal-dominant adult-onset neurodegenerative disease. Since the identification of an abnormal expansion of a trinucleotide repeat tract in the huntingtin gene as the underlying genetic defect, a broad range of transgenic animal models of the disease has become available and these have helped to unravel the relevant molecular pathways in unprecedented detail. Of note, some of the most informative of these models develop thermoregulatory defects such as hypothermia, problems with adaptive thermogenesis, and an altered circadian temperature rhythm. Read More

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http://dx.doi.org/10.1016/B978-0-444-64074-1.00047-1DOI Listing
January 2018
2 Reads

Best interest versus advance decisions to refuse treatment in advance care planning for neurodegenerative illness.

Authors:
Chris Lennard

Br J Nurs 2018 Nov;27(21):1261-1267

Registered mental health nurse, Pirton Grange Specialist Care Centre, Pirton, Worcestershire.

This article describes the role of nurses assisting people with degenerative illness in advance care planning (ACP) for a time when they may lose decision-making capacity. It looks at the concept of advance decisions to refuse treatment (ADRT), as defined in the Mental Capacity Act 2005 , exploring the legal, ethical and philosophical ramifications of carrying out, or overriding, formerly expressed wishes of someone who has subsequently lost decision-making capacity. It uses an illustrative composite case study of an individual with Huntington's disease whose prognosis includes future deterioration in swallowing, together with consideration of whether to have or refuse a percutaneous endoscopic gastrostomy. Read More

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http://dx.doi.org/10.12968/bjon.2018.27.21.1261DOI Listing
November 2018
1 Read

Characteristics of Globus Pallidus Internus Local Field Potentials in Hyperkinetic Disease.

Front Neurol 2018 5;9:934. Epub 2018 Nov 5.

Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.

Dystonia and Huntington's disease (HD) are both hyperkinetic movement disorders but exhibit distinct clinical characteristics. Aberrant output from the globus pallidus internus (GPi) is involved in the pathophysiology of both HD and dystonia, and deep brain stimulation (DBS) of the GPi shows good clinical efficacy in both disorders. The electrode externalized period provides an opportunity to record local field potentials (LFPs) from the GPi to examine if activity patterns differ between hyperkinetic disorders and are associated with specific clinical characteristics. Read More

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http://dx.doi.org/10.3389/fneur.2018.00934DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6230660PMC
November 2018
3 Reads

Increased oxidative stress and CaMKII activity contribute to electro-mechanical defects in cardiomyocytes from a murine model of Huntington's disease.

FEBS J 2018 Nov 19. Epub 2018 Nov 19.

Department of Morphology, Federal University of Minas Gerais, Belo Horizonte, MG, Brazil.

Huntington's disease (HD) is a neurodegenerative genetic disorder. Although described as a brain pathology, there is evidence suggesting that defects in other systems can contribute to disease progression. In line with this, cardiovascular defects are a major cause of death in HD. Read More

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http://dx.doi.org/10.1111/febs.14706DOI Listing
November 2018
1 Read

Inhibition of Drp1 hyperactivation reduces neuropathology and behavioral deficits in zQ175 knock-in mouse model of Huntington's disease.

Biochem Biophys Res Commun 2018 Dec 16;507(1-4):319-323. Epub 2018 Nov 16.

Department of Physiology & Biophysics, Case Western Reserve University School of Medicine, Cleveland, OH, 44106, USA. Electronic address:

Mitochondrial dysfunction manifests in the pathogenesis of Huntington's disease (HD), a fatal and inherited neurodegenerative disease. Dynamin-related protein 1 (Drp1) is the primary component of mitochondrial fission and becomes hyperactivated in various models of HD. We previously reported that inhibition of Drp1 hyperactivation by P110, a rationally designed peptide inhibitor of Drp1-Fis1 interaction, is protective in the HD R6/2 mouse model, which expresses a fragment of mutant Huntingtin (mHtt). Read More

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http://dx.doi.org/10.1016/j.bbrc.2018.11.031DOI Listing
December 2018
3 Reads
2.300 Impact Factor

Cardiovascular effects of marijuana.

Trends Cardiovasc Med 2018 Nov 10. Epub 2018 Nov 10.

Huntington Medical Research Institute, Pasadena, CA 91105, United States. Electronic address:

More than four decades ago, the United States Surgeon General issued a warning regarding the medical problems of marijuana smoking, including cardiac toxicity. Since then, many reports have described atrial fibrillation, ventricular tachycardia, acute coronary syndromes, and cardiac arrest temporally related to marijuana use. The subjects were quite young, with no significant cardiovascular risk factors, with the only obvious trigger being marijuana use. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S10501738183014
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http://dx.doi.org/10.1016/j.tcm.2018.11.004DOI Listing
November 2018
3 Reads

Perioperative Cardiac Considerations in the Surgical Patient.

Clin Podiatr Med Surg 2019 Jan 30;36(1):103-113. Epub 2018 Oct 30.

Department of Adult Cardiovascular Medicine, Joan C. Edwards School of Medicine, Marshall University, 1249 15th Street, Suite 4000, Huntington, WV 25701, USA. Electronic address:

The ability to identify and guide evaluation of the patient with cardiac disease represents a necessary skill for success in surgery of the foot and ankle. Common risk factors, such as diabetes and peripheral arterial disease, are encountered in podiatric practice. Recognition of patients at risk for cardiac disease and a predilection for sustaining a major adverse cardiac event perioperatively advocates for preoperative cardiology consultation. Read More

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http://dx.doi.org/10.1016/j.cpm.2018.09.001DOI Listing
January 2019

A cross-brain regions study of ANK1 DNA methylation in different neurodegenerative diseases.

Neurobiol Aging 2018 Oct 13;74:70-76. Epub 2018 Oct 13.

University of Exeter Medical School, University of Bristol, Exeter, UK. Electronic address:

Recent epigenome-wide association studies in Alzheimer's disease have highlighted consistent robust neuropathology-associated DNA hypermethylation of the ankyrin 1 (ANK1) gene in the cortex. The extent to which altered ANK1 DNA methylation is also associated with other neurodegenerative diseases is not currently known. In the present study, we used bisulfite pyrosequencing to quantify DNA methylation across 8 CpG sites within a 118 bp region of the ANK1 gene across multiple brain regions in Alzheimer's disease, Vascular dementia, Dementia with Lewy bodies, Huntington's disease, and Parkinson's disease. Read More

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http://dx.doi.org/10.1016/j.neurobiolaging.2018.09.024DOI Listing
October 2018
1 Read

How can we evaluate the cost-effectiveness of health system strengthening? A typology and illustrations.

Soc Sci Med 2018 Nov 3;220:141-149. Epub 2018 Nov 3.

Imperial College Business School, Imperial College London, South Kensington Campus, London, SW7 2AZ, United Kingdom. Electronic address:

Health interventions often depend on a complex system of human and capital infrastructure that is shared with other interventions, in the form of service delivery platforms, such as healthcare facilities, hospitals, or community services. Most forms of health system strengthening seek to improve the efficiency or effectiveness of such delivery platforms. This paper presents a typology of ways in which health system strengthening can improve the economic efficiency of health services. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S02779536183062
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http://dx.doi.org/10.1016/j.socscimed.2018.10.030DOI Listing
November 2018
4 Reads

Identification of a novel caspase cleavage site in huntingtin that regulates mutant huntingtin clearance.

FASEB J 2018 Nov 13:fj201701510RRR. Epub 2018 Nov 13.

Department of Medical Genetics, Centre for Molecular Medicine and Therapeutics, The University of British Columbia, Vancouver, British Columbia, Canada.

Huntington disease (HD) is a progressive neurodegenerative disease that initially affects the striatum and leads to changes in behavior and loss of motor coordination. It is caused by an expansion in the polyglutamine repeat at the N-terminus of huntingtin (HTT) that leads to aggregation of mutant HTT. The loss of wild-type function, in combination with the toxic gain of function mutation, initiates various cell death pathways. Read More

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https://www.fasebj.org/doi/10.1096/fj.201701510RRR
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http://dx.doi.org/10.1096/fj.201701510RRRDOI Listing
November 2018
8 Reads

Recipient BCL2 inhibition and NK cell ablation form part of a reduced intensity conditioning regime that improves allo-bone marrow transplantation outcomes.

Cell Death Differ 2018 Nov 12. Epub 2018 Nov 12.

The Walter and Eliza Hall Institute of Medical Research, 1G Royal Parade, Parkville, Melbourne, VIC, 3052, Australia.

Allogeneic hematopoietic stem cell transplantation (alloSCT) is used to treat over 15,000 patients with acute myeloid leukemia (AML) per year. Donor graft-versus-leukemia (GVL) effect can prevent AML relapse; however, alloSCT is limited by significant toxicity related to conditioning intensity, immunosuppression, opportunistic infections, and graft-versus-host disease (GVHD). Reducing the intensity of conditioning regimens prior to alloSCT has improved their tolerability, but does not alter the pattern of GVHD and has been associated with increased rates of graft rejection and relapse. Read More

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https://www.nature.com/articles/s41418-018-0228-y.pdf
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http://www.nature.com/articles/s41418-018-0228-y
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http://dx.doi.org/10.1038/s41418-018-0228-yDOI Listing
November 2018
6 Reads

Huntington disease: A quarter century of progress since the gene discovery.

J Neurol Sci 2018 Sep 27;396:52-68. Epub 2018 Sep 27.

Parkinson's Disease Center and Movement Disorders Clinic, Department of Neurology, Baylor College of Medicine, Houston, TX, USA.

Huntington disease (HD) is an autosomal dominant neurodegenerative disorder characterized by motor, behavioral, and cognitive manifestations. It is caused by an expansion of a trinucleotide repeat in the huntingtin gene (HTT) on chromosome 4. Although disease onset is currently clinically defined by motor signs, the presence of non-motor symptoms prior to motor diagnosis is increasingly recognized. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S0022510X183038
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http://dx.doi.org/10.1016/j.jns.2018.09.022DOI Listing
September 2018
4 Reads

Alternative routes of administration of the neuroprotective pituitary adenylate cyclase activating polypeptide.

Curr Pharm Des 2018 Nov 11. Epub 2018 Nov 11.

Department of Anatomy, MTA-PTE PACAP Research Team, Centre for Neuroscience, University of Pecs Medical School. Hungary.

Pituitary adenylate cyclase activating polypeptide (PACAP) is a neuropeptide with diverse actions, including strong neurotrophic and neuroprotective effects. The aim of our present review is to provide a summary of the different approaches how in vivo neuroprotective effects can be achieved, emphasizing the potential translational values for future therapeutic applications. In the central nervous system, PACAP has been shown to have in vivo protective effects in models of cerebral ischemia, Parkinson's and Alzheimer's disease, Huntington chorea, traumatic brain and spinal cord injury and different retinal pathologies. Read More

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http://www.eurekaselect.com/167238/article
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http://dx.doi.org/10.2174/1381612824666181112110934DOI Listing
November 2018
14 Reads

Dissociation between urate and blood pressure in mice and in people with early Parkinson's disease.

EBioMedicine 2018 Nov 8;37:259-268. Epub 2018 Nov 8.

Department of Neurology, Massachusetts General Hospital, 55 Fruit Street, Boston, MA 02114, USA.

Background: Epidemiological, laboratory and clinical studies have established an association between elevated urate and high blood pressure (BP). However, the inference of causality remains controversial. A naturally occurring antioxidant, urate may also be neuroprotective, and urate-elevating treatment with its precursor inosine is currently under clinical development as a potential disease-modifying strategy for Parkinson's disease (PD). Read More

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http://dx.doi.org/10.1016/j.ebiom.2018.10.039DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6284456PMC
November 2018
5 Reads

Functional Magnetic Resonance Imaging in Huntington's Disease.

Int Rev Neurobiol 2018 29;142:381-408. Epub 2018 Oct 29.

Huntington's Disease Research Centre, UCL Institute of Neurology, London, United Kingdom. Electronic address:

Huntington's disease is an inherited neurodegenerative condition characterized by motor dysfunction, cognitive impairment and neuropsychiatric disturbance. The effects of the underlying pathology on brain morphology are relatively well understood. Numerous structural Magnetic Resonance Imaging (MRI) studies have demonstrated macrostructural change with widespread striatal and cortical atrophy and microstructural white matter loss in premanifest and manifest HD gene carriers. Read More

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http://dx.doi.org/10.1016/bs.irn.2018.09.013DOI Listing
October 2018
9 Reads

Structural Magnetic Resonance Imaging in Huntington's Disease.

Int Rev Neurobiol 2018 8;142:335-380. Epub 2018 Oct 8.

Neurodegeneration Imaging Group (NIG), Institute of Psychiatry, Psychology and Neuroscience (IoPPN), King's College London, London, United Kingdom. Electronic address:

Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder, caused by expansion of the CAG repeat in the huntingtin gene. HD is characterized clinically by progressive motor, cognitive and neuropsychiatric symptoms. There are currently no disease modifying treatments available for HD, and there is a great need for biomarkers to monitor disease progression and identify new targets for therapeutic intervention. Read More

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http://dx.doi.org/10.1016/bs.irn.2018.09.006DOI Listing
October 2018
5 Reads

Molecular Imaging in Huntington's Disease.

Int Rev Neurobiol 2018 29;142:289-333. Epub 2018 Aug 29.

Neurodegeneration Imaging Group (NIG), Institute of Psychiatry, Psychology and Neuroscience (IoPPN), King's College London, London, United Kingdom. Electronic address:

Huntington's disease (HD) is a rare monogenic neurodegenerative disorder caused by a trinucleotide CAG repeat expansion in the huntingtin gene resulting in the formation of intranuclear inclusions of mutated huntingtin. The accumulation of mutated huntingtin leads to loss of GABAergic medium spiny neurons (MSNs); subsequently resulting in the development of chorea, cognitive dysfunction and psychiatric symptoms. Premanifest HD gene expansion carriers, provide a unique cohort to examine very early molecular changes, occurring before the development of overt symptoms, to elucidate disease pathophysiology and identify reliable biomarkers of HD progression. Read More

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http://dx.doi.org/10.1016/bs.irn.2018.08.007DOI Listing
August 2018
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Coenzyme Q10 supplementation in acute ischemic stroke: Is it beneficial in short-term administration?

Nutr Neurosci 2018 Nov 7:1-6. Epub 2018 Nov 7.

a Skull Base Research Center , Loghman Hakim Hospital, Shahid Beheshti University of Medical Sciences , Tehran , Iran.

Backgrounds And Aims: Clinical studies demonstrated that the efficacy of Coenzyme Q10 (CoQ10) as an adjuvant therapeutic agent in several neurological diseases such as Parkinson disease (PD), Huntington disease (HD), and migraine. The purpose of this study is to investigate oxidative stress effects, antioxidant enzymes activity, neuroinflammatory markers levels, and neurological outcome in acute ischemic stroke (AIS) patients following administration of CoQ10 (300 mg/day).

Methods: Patients with AIS (n = 60) were randomly assigned to a placebo group (wheat starch, n = 30) or CoQ10-supplemented group (300 mg/day, n = 30). Read More

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https://www.tandfonline.com/doi/full/10.1080/1028415X.2018.1
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http://dx.doi.org/10.1080/1028415X.2018.1541269DOI Listing
November 2018
5 Reads

Disease Progression in Huntington Disease: An Analysis of Multiple Longitudinal Outcomes.

J Huntingtons Dis 2018 ;7(4):337-344

Department of Neurology, Columbia University Medical Center, New York, NY, USA.

Background: Critical to discovering targeted therapies for Huntington disease (HD) are validated methods that more precisely predict when clinical outcomes occur for different patient profiles.

Objective: To more precisely predict the probability of when motor diagnosis (diagnostic confidence level 4) on the Unified Huntington's Disease Rating Scale (UHDRS), cognitive impairment (two or more neuropsychological scores on the UHDRS were 1.5 standard deviations below normative means) and Stage II Total Functional Capacity (TFC) first occur by accounting for dependencies between these outcomes. Read More

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http://www.medra.org/servlet/aliasResolver?alias=iospress&am
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http://dx.doi.org/10.3233/JHD-180297DOI Listing
January 2018
4 Reads

Heterozygous huntingtin promotes cadmium neurotoxicity and neurodegeneration in striatal cells via altered metal transport and protein kinase C delta dependent oxidative stress and apoptosis signaling mechanisms.

Neurotoxicology 2018 Nov 3;70:48-61. Epub 2018 Nov 3.

Neuroscience Department, Oberlin College, Oberlin, OH, USA.

Huntington's disease (HD) is functionally linked to environmental factors including cigarette use and dyshomeostasis in the levels of metals. Interestingly, one of the most abundant heavy metals in cigarettes is cadmium (Cd), which also accumulates in the striatum and causes neurotoxicity upon exposure. Thus, we hypothesized that heterozygous huntingtin (HTT), responsible for the majority of cases of HD in patients, in combination with Cd exposure would cause neurotoxicity and neurodegeneration via increased intracellular accumulation of Cd and activation of oxidative stress signaling mechanisms in a mouse striatal cell line model of HD. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S0161813X183045
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http://dx.doi.org/10.1016/j.neuro.2018.10.012DOI Listing
November 2018
5 Reads

Leukocyte telomere shortening in Huntington's disease.

J Neurol Sci 2018 Oct 28;396:25-29. Epub 2018 Oct 28.

CNR Institute of Molecular Biology and Pathology, c/o Department of Biology and Biotechnology, P.le Aldo Moro 5, 00185 Rome, Italy; Department of Biology and Biotechnology, La Sapienza University, P.le Aldo Moro 5, 00185 Rome, Italy. Electronic address:

Huntington's disease (HD) is an autosomal dominant neurodegenerative disease caused by an expanded CAG repeat. Though symptom onset commonly occurs at midlife and inversely correlates with the CAG repeat expansion, age at clinical onset and progression rate are variable. In the present study we investigated the relationship between leukocyte telomere length (LTL) and HD development. Read More

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http://dx.doi.org/10.1016/j.jns.2018.10.024DOI Listing
October 2018
11 Reads