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https://www.readbyqxmd.com/read/29028540/endo-lysosomal-dysfunction-a-converging-mechanism-in-neurodegenerative-diseases
#1
REVIEW
Chao Wang, Maria A Telpoukhovskaia, Ben A Bahr, Xu Chen, Li Gan
Endo-lysosomal pathways are essential in maintaining protein homeostasis in the cell. Numerous genes in the endo-lysosomal pathways have been found to associate with neurodegenerative diseases, including Alzheimer's disease (AD), Parkinson's disease (PD), and frontotemporal dementia (FTD). Mutations of these genes lead to dysfunction in multiple steps of the endo-lysosomal network: autophagy, endocytic trafficking and lysosomal degradation, resulting in accumulation of pathogenic proteins. Although the exact pathogenic mechanism varies for different disease-associated genes, dysfunction of the endo-lysosomal pathways represents a converging mechanism shared by these diseases...
October 10, 2017: Current Opinion in Neurobiology
https://www.readbyqxmd.com/read/29021741/the-neuroprotective-role-of-protein-quality-control-in-halting-the-development-of-alpha-synuclein-pathology
#2
REVIEW
Destiny-Love Manecka, Benoît Vanderperre, Edward A Fon, Thomas M Durcan
Synucleinopathies are a family of neurodegenerative disorders that comprises Parkinson's disease, dementia with Lewy bodies, and multiple system atrophy. Each of these disorders is characterized by devastating motor, cognitive, and autonomic consequences. Current treatments for synucleinopathies are not curative and are limited to improvement of quality of life for affected individuals. Although the underlying causes of these diseases are unknown, a shared pathological hallmark is the presence of proteinaceous inclusions containing the α-synuclein (α-syn) protein in brain tissue...
2017: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/29016447/systemic-inflammation-in-lewy-body-diseases-a-systematic-review
#3
Eleanor King, Alan Thomas
Few studies have investigated the role of inflammation in Lewy body dementia (LBD) and variable results have been found. We systematically reviewed the literature for evidence of systemic inflammatory changes in dementia with Lewy bodies and Parkinson disease dementia. Owing to the low number of studies we also included Parkinson disease. Key terms were used to search the relevant databases. Titles and abstracts were screened and potentially relevant articles were reviewed in full. References of included studies and relevant reviews were searched...
October 9, 2017: Alzheimer Disease and Associated Disorders
https://www.readbyqxmd.com/read/28993533/clinical-reasoning-a-55-year-old-man-with-rapidly-progressive-dementia-and-parkinsonism
#4
Miguel Tábuas-Pereira, Isabel Santana, Gustavo Cordeiro Santo
No abstract text is available yet for this article.
October 10, 2017: Neurology
https://www.readbyqxmd.com/read/28991700/cardiac-sympathetic-denervation-and-dementia-in-de-novo-parkinson-s-disease-a-7-year-follow-up-study
#5
Mun Hee Choi, Jung Han Yoon, Suk Woo Yong
BACKGROUND: Postganglionic cardiac sympathetic denervation is evident in patients with early-stage Parkinson's disease (PD). Cardiac iodine-123-meta-iodobenzylguanidine (MIBG) uptake is correlated with the non-motor symptoms of PD, suggesting that low cardiac MIBG uptake may reflect wider alpha-synuclein pathology. In addition, low cardiac MIBG could be related to orthostatic hypotension in PD, which may affect cognition. However, the prognostic validity of baseline MIBG scintigraphy in terms of the risk of subsequent dementia remains unclear...
October 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28991698/pitx3-genotype-and-risk-of-dementia-in-parkinson-s-disease-a-population-based-study
#6
David Bäckström, Magdalena Eriksson Domellöf, Gabriel Granåsen, Jan Linder, Sofia Mayans, Eva Elgh, Susanna Jakobson Mo, Lars Forsgren
Dementia is a devastating manifestation of Parkinson's disease (PD). This study investigates whether a common polymorphism in the PITX3 gene (rs2281983), which is of importance for the function of dopaminergic neurons, affects the risk of developing dementia in PD and whether it affects dopamine transporter (DAT) uptake. We PITX3 genotyped 133 patients with new-onset, idiopathic PD, participating in a population-based study in Sweden. Patients were followed prospectively during 6-11years with extensive investigations, including neuropsychology and DAT-imaging with (123)I FP-CIT...
October 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28991666/educational-attainment-and-motor-burden-in-advanced-parkinson-s-disease-the-emerging-role-of-education-in-motor-reserve
#7
Josefine Blume, Eva Rothenfusser, Jürgen Schlaier, Ulrich Bogdahn, Max Lange
OBJECTIVE: To explore the relationship of motor burden and educational attainment in patients with advanced stage PD. MATERIALS AND METHODS: We included 102 consecutive patients who underwent a complete evaluation for DBS surgery, including detailed neuropsychological testing and UPDRSIII in a standardized Levodopa challenge. Years of education (YoE) were calculated as the highest grade attained in secondary school plus years for post-secondary training. RESULTS: The OFF medication UPDRS-III score was associated with YoE (p=0...
October 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28990131/diagnosis-and-management-of-cognitive-and-behavioral-changes-in-dementia-with-lewy-bodies
#8
REVIEW
Babak Tousi
Proper diagnosis of dementia with Lewy bodies (DLB) in clinical practice remains suboptimal as many cases are misdiagnosed, usually as Alzheimer disease (AD) or Parkinson's disease (PD) and, in rare cases, psychosis. Therefore, it is important for patients with dementia to be thoroughly evaluated by a specialist who is familiar with current diagnostic tests and treatment options. New diagnostic criteria from the Dementia with Lewy Bodies Consortium have been developed to increase diagnostic sensitivity for DLB (Diagnosis and management of dementia with Lewy bodies: fourth consensus report of the DLB Consortium; McKeith et al...
October 9, 2017: Current Treatment Options in Neurology
https://www.readbyqxmd.com/read/28987182/tauopathies
#9
Gabor G Kovacs
Tauopathies are neurodegenerative disorders characterized by the deposition of abnormal tau protein in the brain. The spectrum of tau pathologies expands beyond the traditionally discussed disease forms like Pick disease, progressive supranuclear palsy, corticobasal degeneration, and argyrophilic grain disease. Emerging entities and pathologies include globular glial tauopathies, primary age-related tauopathy, which includes neurofibrillary tangle dementia, chronic traumatic encephalopathy (CTE), and aging-related tau astrogliopathy...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28987181/alpha-synucleinopathies
#10
Irina Alafuzoff, Päivi Hartikainen
A neurodegenerative disorder displaying an altered α-synuclein (αS) in the brain tissue is called α-synucleinopathy (αS-pathy) and incorporates clinical entities such as Parkinson disease (PD), PD with dementia, dementia with Lewy bodies, and multiple-system atrophy. Neuroradiologic techniques visualizing αS pathology in the brain or assays of αS in the cerebrospinal fluid or blood are probably available and will be implemented in the near future but currently the definite diagnosis of αS-pathy relies on a postmortem examination of the brain...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28984591/curcumin-inhibits-tau-aggregation-and-disintegrates-preformed-tau-filaments-in-vitro
#11
Jitendra Subhash Rane, Prasenjit Bhaumik, Dulal Panda
The pathological aggregation of tau is a common feature of most of the neuronal disorders including frontotemporal dementia, Parkinson's disease, and Alzheimer's disease. The inhibition of tau aggregation is considered to be one of the important strategies for treating these neurodegenerative diseases. Curcumin, a natural polyphenolic molecule, has been reported to have neuroprotective ability. In this work, curcumin was found to bind to adult tau and fetal tau with a dissociation constant of 3.3±0.4 and 8±1 μM, respectively...
2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28982489/palliative-care-in-neurology
#12
REVIEW
Maisha T Robinson, Robert G Holloway
Palliative medicine is a specialty that focuses on improving the quality of life for patients with serious or advanced medical conditions, and it is appropriate at any stage of disease, including at the time of diagnosis. Neurologic conditions tend to have high symptom burdens, variable disease courses, and poor prognoses that affect not only patients but also their families and caregivers. Patients with a variety of neurologic conditions such as Parkinson disease, dementia, amyotrophic lateral sclerosis, brain tumors, stroke, and acute neurologic illnesses have substantial unmet needs that can be addressed through a combination of primary and specialty palliative care...
October 2017: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/28975502/l-serine-mediated-neuroprotection-includes-the-upregulation-of-the-er-stress-chaperone-protein-disulfide-isomerase-pdi
#13
R A Dunlop, J T Powell, J S Metcalf, G J Guillemin, P A Cox
The unfolded protein response (UPR) is a highly evolutionarily conserved response to endoplasmic reticulum (ER) stress, which functions to return cells to homeostasis or send them into apoptosis, depending on the degree of cellular damage. β-N-methylamino-L-alanine (L-BMAA) has been shown to induce ER stress in a variety of models and has been linked to several types of neurodegenerative disease including Guamanian amyotrophic lateral sclerosis/Parkinsonism dementia complex (ALS/PDC). L-Serine, an amino acid critical for cellular metabolism and neurological signaling, has been shown to be protective against L-BMAA-induced neurotoxicity in both animal and cell culture models...
October 3, 2017: Neurotoxicity Research
https://www.readbyqxmd.com/read/28973176/association-between-poor-cognitive-functioning-and-risk-of-incident-parkinsonism-the-rotterdam-study
#14
Sirwan K L Darweesh, Frank J Wolters, Ronald B Postuma, Bruno H Stricker, Albert Hofman, Peter J Koudstaal, M Kamran Ikram, M Arfan Ikram
Importance: Cognitive dysfunction is a common feature among patients with parkinsonism, including Parkinson disease (PD). However, there is a scarcity of data on cognitive functioning before parkinsonism diagnosis, a stage at which patients may still respond to putative disease-modifying interventions. Objective: To assess whether poor cognitive functioning is associated with an increased risk of parkinsonism. Design, Setting, and Participants: Between January 8, 2002, and December 14, 2008, baseline cognitive function was assessed in 7386 participants of the Rotterdam Study who were free of parkinsonism and dementia...
September 25, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28970540/microstructural-network-alterations-of-olfactory-dysfunction-in-newly-diagnosed-parkinson-s-disease
#15
Ming-Ching Wen, Zheyu Xu, Zhonghao Lu, Ling Ling Chan, Eng King Tan, Louis C S Tan
Olfactory dysfunction is a robust and early sign for Parkinson's disease (PD). Previous studies have revealed its association with dementia and related neural changes in PD. Yet, how olfactory dysfunction affects white matter (WM) microstructure in newly diagnosed and untreated PD remains unclear. Here we comprehensively examined WM features using unbiased whole-brain analyses. 88 newly diagnosed PD patients without dementia (70 with hyposmia and 18 without hyposmia) and 33 healthy controls underwent clinical assessment and diffusion tensor imaging (DTI) scanning...
October 2, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28969462/low-sensitivity-of-the-mini-mental-state-examination-for-cognitive-assessment-of-brazilian-patients-with-parkinson-disease
#16
Raphael Breder, Marco Antonio Araujo Leite, Jony Arrais Pinto, Igor Pinto Cavalcante, Bruno Lima Pessoa, Marco Antônio Orsini Neves
INTRODUCTION: Recent publications have highlighted the low sensitivity of the Mini-Mental State Examination (MMSE) for the cognitive assessment of patients with Parkinson disease (PD). The Montreal Cognitive Assessment (MoCA), otherwise, has shown greater sensitivity when compared to the MMSE. Based on this, we have searched for the cognitive impairment measurable by the MoCA and the functional performance on activities of daily living in a sample of Brazilian patients with PD and normal MMSE...
January 1, 2017: Journal of Geriatric Psychiatry and Neurology
https://www.readbyqxmd.com/read/28968236/decreased-n-acetyl-aspartate-myo-inositol-ratio-in-the-posterior-cingulate-cortex-shown-by-magnetic-resonance-spectroscopy-may-be-one-of-the-risk-markers-of-preclinical-alzheimer-s-disease-a-7-year-follow-up-study
#17
Masaaki Waragai, Masaru Moriya, Takeshi Nojo
Although molecular positron emission tomography imaging of amyloid and tau proteins can facilitate the detection of preclinical Alzheimer's disease (AD) pathology, it is not useful in clinical practice. More practical surrogate markers for preclinical AD would provide valuable tools. Thus, we sought to validate the utility of conventional magnetic resonance spectroscopy (MRS) as a screening method for preclinical AD. A total of 289 older participants who were cognitively normal at baseline were clinically followed up for analysis of MRS metabolites, including N-acetyl aspartate (NAA) and myo-inositol (MI) in the posterior cingulate cortex (PCC) for 7 years...
September 29, 2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28966932/frequency-of-gba-variants-in-autopsy-proven-multiple-system-atrophy
#18
Miriam Sklerov, Un Jung Kang, Christopher Liong, Lorraine Clark, Karen Marder, Michael Pauciulo, William C Nichols, Wendy K Chung, Lawrence S Honig, Etty Cortes, Jean Paul Vonsattel, Roy N Alcalay
BACKGROUND: Multiple system atrophy (MSA) is marked by abnormal inclusions of alpha-synuclein in oligodendrogliocytes. Etiology remains unknown. Variants in the glucocerebrosidase gene have been associated with other synucleinopathies, dementia with Lewy bodies and Parkinson disease. It is unclear whether glucocerebrosidase variants are associated with MSA. OBJECTIVES: To analyze the frequency of glucocerebrosidase gene variants among autopsy-proven cases of MSA at a brain bank in New York City...
July 2017: Movement Disorders Clinical Practice
https://www.readbyqxmd.com/read/28963338/getting-in-charge-of-%C3%AE-synuclein-fibrillation
#19
Meytal Landau
The synuclein family has long been associated with Parkinson's disease and dementia. Although the self-assembly of α-synuclein (αS) into oligomers and amyloid fibrils is well established, the aggregation propensity of other members of the family and their role in disease is still under debate. Moriarty et al. now suggest that the pH switching that occurs between different cellular environments could control β-synuclein (βS) aggregation via altering its charge distribution, thus opening new possible roles for βS in Parkinson's and other neurodegenerative diseases...
September 29, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28963060/dna-versus-rna-oxidation-in-parkinson-s-disease-which-is-more-important
#20
K Gmitterová, J Gawinecka, U Heinemann, P Valkovič, I Zerr
BACKROUND: 8-hydroxy-2 deoxyguanosine (8-OHdG) and the 8-hydroxyguanosine (8-OHG) are the most widely used biomarkers of nucleoside oxidation affecting DNA and RNA and are considered reliable markers of oxidative stress. Increased levels of these markers are found in the various biological fluids of patients with neurodegenerative disorders. OBJECTIVE: The primary aim of our study was to assess the differences of investigated markers between patient groups and subsequently study the influence of clinical factors that might modify the levels of investigated markers during the disease progression...
September 27, 2017: Neuroscience Letters
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