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Huntington Disease Dementia

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A comparison between the neurocognitive profile of Huntington Disease-Like 2 and Huntington Disease: Exploring the presence of double dissociations.

Aline Ferreira-Correia, David G Anderson, Kate Cockcroft, Amanda Krause,

Huntington Disease Like-2 (HDL2) is a rare autosomal dominant genetic disease caused by a mutation in the JPH3 gene. HDL2 is the Huntington Disease (HD) phenocopy that has the greatest clinical resemblance to HD. Both are characterized by movement, psychiatric and cognitive dysfunction, which progress to dementia. The present study ... Read more >>

Appl Neuropsychol Adult (Applied neuropsychology. Adult)
[2020, :1-11]

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Antisense oligonucleotides for neurodegeneration.

Blair R Leavitt, Sarah J Tabrizi,

Science (Science (New York, N.Y.))
[2020, 367(6485):1428-1429]

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Systematic review protocol: Quantitative susceptibility mapping of brain iron accumulation in neurodegenerative diseases.

Parsa Ravanfar, Samantha Loi, Tamsyn Van Rheenen, Ashley Bush, Patricia Desmond, Vanessa Cropley, Bradford Moffat, Dennis Velakoulis, Christos Pantelis,

Iron has been found to play an important role in neurodegeneration. Quantitative susceptibility mapping (QSM) is a relatively new, and the most accurate, MRI technique available for assessment of iron deposition in the brain. There is a rapidly growing number of studies using QSM to investigate brain iron distribution in ... Read more >>

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Association Between Neurological Disorders and Death by Suicide in Denmark.

Annette Erlangsen, Egon Stenager, Yeates Conwell, Per Kragh Andersen, Keith Hawton, Michael Eriksen Benros, Merete Nordentoft, Elsebeth Stenager,

Importance:Neurological disorders have been linked to suicide, but the risk across a broad spectrum of neurological disorders remains to be assessed. Objectives:To examine whether people with neurological disorders die by suicide more often than other people and to assess for temporal associations. Design, Setting, and Participants:Nationwide, retrospective cohort study on ... Read more >>

JAMA (JAMA)
[2020, 323(5):444-454]

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Predictors of Home Care Costs Among Persons With Dementia, Amyotrophic Lateral Sclerosis, and Multiple Sclerosis in Ontario.

Clare Cheng, John P Hirdes, George Heckman, Jeff Poss,

Home care is an important service for persons with neurological conditions, but little is known about factors affecting health care costs in this setting. Using administrative data collected with the Resident Assessment Instrument for Home Care (RAI-HC), this study identified factors associated with home care costs for recipients of home ... Read more >>

Health Serv Insights (Health Services Insights)
[2020, 13:1178632920903731]

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Peroxisomal Dysfunction in Neurological Diseases and Brain Aging.

Ndidi-Ese Uzor, Louise D McCullough, Andrey S Tsvetkov,

Peroxisomes exist in most cells, where they participate in lipid metabolism, as well as scavenging the reactive oxygen species (ROS) that are produced as by-products of their metabolic functions. In certain tissues such as the liver and kidneys, peroxisomes have more specific roles, such as bile acid synthesis in the ... Read more >>

Front Cell Neurosci (Frontiers in Cellular Neuroscience)
[2020, 14:44]

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Current Advances in Allosteric Modulation of Muscarinic Receptors.

Jan Jakubik, Esam E El-Fakahany,

Allosteric modulators are ligands that bind to a site on the receptor that is spatially separated from the orthosteric binding site for the endogenous neurotransmitter. Allosteric modulators modulate the binding affinity, potency, and efficacy of orthosteric ligands. Muscarinic acetylcholine receptors are prototypical allosterically-modulated G-protein-coupled receptors. They are a potential therapeutic ... Read more >>

Biomolecules (Biomolecules)
[2020, 10(2):]

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Intranasal administration of dantrolene increased brain concentration and duration.

Jintao Wang, Yun Shi, Shuchun Yu, Yan Wang, Qingcheng Meng, Ge Liang, Maryellen F Eckenhoff, Huafeng Wei,

Dantrolene has been demonstrated to be neuroprotective for multiple neurodegenerative diseases. However, dantrolene's limited penetration into the CNS hampers its effectiveness as a neuroprotective agent. Here, we studied whether the intranasal administration of dantrolene provided better penetration into the brain than the commonly used oral approach. C57BL/6 mice, aged 2-4 ... Read more >>

PLoS ONE (PloS one)
[2020, 15(3):e0229156]

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Therapeutic importance of hydrogen sulfide in age-associated neurodegenerative diseases.

Rubaiya Tabassum, Na Young Jeong, Junyang Jung,

Hydrogen sulfide (H2S) is a gasotransmitter that acts as an antioxidant and exhibits a wide variety of cytoprotective and physiological functions in age-associated diseases. One of the major causes of age-related diseases is oxidative stress. In recent years, the importance of H2S has become clear, although its antioxidant function has ... Read more >>

Neural Regen Res (Neural regeneration research)
[2020, 15(4):653-662]

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Measuring the quality of care in nursing home residents with early-onset neurodegenerative diseases: a scoping review.

Joyce C F Heffels, Irma H J Everink, Mayke Oosterloo, Raymund A C Roos, Jos M G A Schols,

BACKGROUND:Nursing home residents with early-onset neurodegenerative diseases are often younger in comparison with other residents, and need different, often more complex care. Accordingly, the measurements currently used for measuring quality of care in nursing homes may not be suitable for use in this target group. Little is known about the ... Read more >>

BMC Palliat Care (BMC palliative care)
[2020, 19(1):25]

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Osmolytes: A Possible Therapeutic Molecule for Ameliorating the Neurodegeneration Caused by Protein Misfolding and Aggregation.

Neetu Kushwah, Vishal Jain, Dhananjay Yadav,

Most of the neurological disorders in the brain are caused by the abnormal buildup of misfolded or aggregated proteins. Osmolytes are low molecular weight organic molecules usually built up in tissues at a quite high amount during stress or any pathological condition. These molecules help in providing stability to the ... Read more >>

Biomolecules (Biomolecules)
[2020, 10(1):]

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Prostaglandins as the Agents That Modulate the Course of Brain Disorders.

Hamidreza Famitafreshi, Morteza Karimian,

Neurologic and neuropsychiatric diseases are associated with great morbidity and mortality. Prostaglandins (PGs) are formed by sequential oxygenation of arachidonic acid in physiologic and pathologic conditions. For the production of PGs cyclooxygenase is a necessary enzyme that has two isoforms, that are named COX-1 and COX-2. COX-1 produces type 1 ... Read more >>

Degener Neurol Neuromuscul Dis (Degenerative neurological and neuromuscular disease)
[2020, 10:1-13]

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Real-state of autophagy signaling pathway in neurodegenerative disease; focus on multiple sclerosis.

Mehdi Hassanpour, Fateme Hajihassani, Amirataollah Hiradfar, Nasser Aghamohammadzadeh, Reza Rahbarghazi, Nasser Safaie, Mohammad Nouri, Yunes Panahi,

The occurrence of neurodegenerative disease is increasingly raised. From physiopathological aspect, the emergence of auto-reactive antibodies against the nervous system antigens contributes to de-myelination in Multiple sclerosis (MS). These features cause the nervous system dysfunction. The follow-up of molecular alterations could give us a real-state vision about intracellular status during ... Read more >>

J Inflamm (Lond) (Journal of inflammation (London, England))
[2020, 17:6]

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On the wrong DNA track: Molecular mechanisms of repeat-mediated genome instability.

Alexandra N Khristich, Sergei M Mirkin,

Expansions of simple tandem repeats are responsible for almost 50 human diseases, the majority of which are severe, degenerative, and not currently treatable or preventable. In this review, we first describe the molecular mechanisms of repeat-induced toxicity, which is the connecting link between repeat expansions and pathology. We then survey ... Read more >>

J. Biol. Chem. (The Journal of biological chemistry)
[2020, 295(13):4134-4170]

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Genetic targeting of astrocytes to combat neurodegenerative disease.

Rachel Kery, Allen P F Chen, Gregory W Kirschen,

Astrocytes, glial cells that interact extensively with neurons and other support cells throughout the central nervous system, have recently come under the spotlight for their potential contribution to, or potential regenerative role in a host of neurodegenerative disorders. It is becoming increasingly clear that astrocytes, in concert with microglial cells, ... Read more >>

Neural Regen Res (Neural regeneration research)
[2020, 15(2):199-211]

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Safety and efficacy of Jujadokseo-hwan for memory deficit (amnesia) in mild neurocognitive disorder: A protocol for randomized, double-blind, placebo-controlled, parallel-group, multicenter clinical trial.

Jin-Hyung Jeong, Ji-Yoon Lee, Ju-Yeon Kim, Young-Kyung Seo, Wee-Chang Kang, Hyung-Won Kang, So-Jung Park, Hye-Kyoung Jang, Yang-Chun Park, In Chul Jung,

BACKGROUND:Mild neurocognitive disorder (mNCD) is one of the degenerative diseases that results in memory deficit, and can progress to dementia. The effectiveness of drug therapy for mNCD is still debatable, but treatment of this disease has important implications for postponing or preventing dementia. Jujadokseo-hwan (JDH) is a traditional herbal medicine ... Read more >>

Medicine (Baltimore) (Medicine)
[2020, 99(8):e19231]

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Hypoxia-Induced Degenerative Protein Modifications Associated with Aging and Age-Associated Disorders.

Sunil S Adav, Siu Kwan Sze,

Aging is an inevitable time-dependent decline of various physiological functions that finally leads to death. Progressive protein damage and aggregation have been proposed as the root cause of imbalance in regulatory processes and risk factors for aging and neurodegenerative diseases. Oxygen is a modulator of aging. The oxygen-deprived conditions (hypoxia) ... Read more >>

Aging Dis (Aging and Disease)
[2020, 11(2):341-364]

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A Tablet App Supporting Self-Management for People With Dementia: Explorative Study of Adoption and Use Patterns.

Laila Øksnebjerg, Bob Woods, Kathrine Ruth, Annette Lauridsen, Susanne Kristiansen, Helle Dalsgaard Holst, Gunhild Waldemar,

BACKGROUND:Assistive technology (AT) is rapidly emerging within dementia care and support. One area of AT application is support of people with dementia in compensating for cognitive symptoms and thereby promoting their self-management. There is, however, little evidence for the applicability, usability, and effectiveness of AT for people with dementia, and ... Read more >>

JMIR Mhealth Uhealth (JMIR mHealth and uHealth)
[2020, 8(1):e14694]

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Human health risk assessment of elevated and variable iron and manganese intake with arsenic-safe groundwater in Jashore, Bangladesh.

Gopal Chandra Ghosh, Md. Jahed Hassan Khan, Tapos Kumar Chakraborty, Samina Zaman, A. H. M. Enamul Kabir, Hiroaki Tanaka,

Groundwater through hand-operated tubewell (a type of water well) tapping is the main source of drinking water in Bangladesh. This study investigated iron and manganese concentration in groundwater across Jashore district–one of the worst arsenic contaminated area in Bangladesh. One working tubewell that had been tested previously for arsenic and ... Read more >>

Sci Rep (Scientific reports)
[2020, 10:]

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Tianzhi granule improves cognition and BPSD of vascular dementia: a randomized controlled trial.

Jing Shi, Mingqing Wei, Jingnian Ni, Feng Sun, Li Sun, Junfu Wang, Tao Yu, Kai Wang, Peiyuan Lv, Yunfu Wang, Yulian Zhang, Xuguang Gao, Xuanzhao Gao, Benyan Luo, Shanping Mao, Baorong Zhang, Xiangyang Ren, Fengchun Yu, Wenli Hu, Ping Yin, Nanjin Wu, Xianfeng Liu, Qi Bi, Yongyan Wang, Jinzhou Tian, ,

BACKGROUND AND PURPOSE:Tianzhi granule (TZ) is usually used for patients with vascular dementia (VaD) in China. The aim was to assess the effect of TZ by a randomized clinical trial (RCT). METHODS:A 24-week RCT was conducted in 16 centres. Participants were grouped into TZ, donepezil or placebo. The co-primary outcomes ... Read more >>

J Transl Med (Journal of translational medicine)
[2020, 18(1):76]

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The Overlapping Genetics of Amyotrophic Lateral Sclerosis and Frontotemporal Dementia.

Yevgeniya A Abramzon, Pietro Fratta, Bryan J Traynor, Ruth Chia,

Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are two diseases that form a broad neurodegenerative continuum. Considerable effort has been made to unravel the genetics of these disorders, and, based on this work, it is now clear that ALS and FTD have a significant genetic overlap. TARDBP, SQSTM1, VCP, ... Read more >>

Front Neurosci (Frontiers in neuroscience)
[2020, 14:42]

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Selective Neuronal Death in Neurodegenerative Diseases: The Ongoing Mystery.

Srinivasa Subramaniam,

A major unresolved problem in neurodegenerative disease is why and how a specific set of neurons in the brain are highly vulnerable to neuronal death. Multiple pathways and mechanisms have been proposed to play a role in Alzheimer disease (AD), Parkinson disease (PD), amyotrophic lateral sclerosis (ALS), and Huntington disease ... Read more >>

Yale J Biol Med (The Yale journal of biology and medicine)
[2019, 92(4):695-705]

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Risks and benefits of unapproved disease-modifying treatments for neurodegenerative disease.

Aden C Feustel, Amanda MacPherson, Dean A Fergusson, Karl Kieburtz, Jonathan Kimmelman,

OBJECTIVE:To determine whether patients randomized to unapproved, disease-modifying interventions in neurodegenerative disease trials have better outcomes than patients randomized to placebo by performing a systematic review and meta-analysis of risk and benefit experienced by patients in randomized placebo-controlled trials testing investigational treatments for Alzheimer disease, Parkinson disease, Huntington disease, or ... Read more >>

Neurology (Neurology)
[2020, 94(1):e1-e14]

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Cannabinoids and the expanded endocannabinoid system in neurological disorders.

Luigia Cristino, Tiziana Bisogno, Vincenzo Di Marzo,

Anecdotal evidence that cannabis preparations have medical benefits together with the discovery of the psychotropic plant cannabinoid Δ9-tetrahydrocannabinol (THC) initiated efforts to develop cannabinoid-based therapeutics. These efforts have been marked by disappointment, especially in relation to the unwanted central effects that result from activation of cannabinoid receptor 1 (CB1), which ... Read more >>

Nat Rev Neurol (Nature reviews. Neurology)
[2020, 16(1):9-29]

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Unveiling synapse pathology in spinal bulbar muscular atrophy by genome-wide transcriptome analysis of purified motor neurons derived from disease specific iPSCs.

Kazunari Onodera, Daisuke Shimojo, Yasuharu Ishihara, Masato Yano, Fuyuki Miya, Haruhiko Banno, Naoko Kuzumaki, Takuji Ito, Rina Okada, Bruno de Araújo Herculano, Manabu Ohyama, Mari Yoshida, Tatsuhiko Tsunoda, Masahisa Katsuno, Manabu Doyu, Gen Sobue, Hideyuki Okano, Yohei Okada,

Spinal bulbar muscular atrophy (SBMA) is an adult-onset, slowly progressive motor neuron disease caused by abnormal CAG repeat expansion in the androgen receptor (AR) gene. Although ligand (testosterone)-dependent mutant AR aggregation has been shown to play important roles in motor neuronal degeneration by the analyses of transgenic mice models and ... Read more >>

Mol Brain (Molecular brain)
[2020, 13(1):18]

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