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Primary progressive dementia

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https://www.readbyqxmd.com/read/29348375/-suspected-non-alzheimer-s-disease-pathophysiology-snap-and-its-pathological-backgrounds-in-the-diagnosis-of-preclinical-and-clinical-alzheimer-s-disease
#1
Masahito Yamada
Suspected non-Alzheimer's disease pathophysiology (SNAP) is a biomarker-based condition that is found in individuals with normal levels of amyloid-β protein (Aβ) markers (A-) and abnormal levels of markers of neurodegeneration or neuronal injury (N+). SNAP is found in 20-26% of cognitively normal (CN) individuals aged 65 years or older and 17-35% of individuals with mild cognitive impairment (MCI). Similarly, 7-39% of patients with clinically probable Alzheimer's disease (AD) dementia are negative for Aβ...
January 2018: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/29346380/rapidly-progressive-dementia-an-eight-year-2008-2016-retrospective-study
#2
Patil Anuja, Vishnu Venugopalan, Naheed Darakhshan, Pandit Awadh, Vinny Wilson, Goyal Manoj, Modi Manish, Lal Vivek
BACKGROUND AND PURPOSE: Rapidly progressive dementia (RPD) is an emergency in cognitive neurology, defined as cognitive impairment affecting the daily living activities developed over less than 1 year. This study investigated the profile of patients with rapidly progressive dementia at first presentation. METHODS: Retrospective case analysis was done in 187 patients with rapidly progressive dementia who presented to the Postgraduate Institute of Medical Education and Research, Chandigarh, India from January 2008 to August 2016...
2018: PloS One
https://www.readbyqxmd.com/read/29334498/evidence-based-interpretation-of-amyloid-%C3%AE-pet-results-a-clinician-s-tool
#3
David Bergeron, Rik Ossenkoppele, Robert Jr Laforce
BACKGROUND: Amyloid-β positron emission tomography (PET) allows for in vivo detection of fibrillar amyloid plaques, a pathologic hallmark of Alzheimer's disease (AD). However, amyloid-β PET interpretation is limited by the imperfect correlation between PET and autopsy, and the fact that it is positive in about 20% to 30% of cognitively normal individuals and non-AD dementias, especially when older or carrying the ε4 allele of apolipoprotein E (ApoE4). When facing a positive amyloid PET, clinicians have to evaluate the probability of a pathologic false positive as well as the probability of amyloid positivity being age-related, comorbid to a primary non-AD dementia (clinicopathologic false positive)...
January 12, 2018: Alzheimer Disease and Associated Disorders
https://www.readbyqxmd.com/read/29302060/c9orf72-ggggcc-repeat-associated-non-aug-translation-is-upregulated-by-stress-through-eif2%C3%AE-phosphorylation
#4
Weiwei Cheng, Shaopeng Wang, Alexander A Mestre, Chenglai Fu, Andres Makarem, Fengfan Xian, Lindsey R Hayes, Rodrigo Lopez-Gonzalez, Kevin Drenner, Jie Jiang, Don W Cleveland, Shuying Sun
Hexanucleotide repeat expansion in C9ORF72 is the most frequent cause of both amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Here we demonstrate that the repeat-associated non-AUG (RAN) translation of (GGGGCC) n -containing RNAs into poly-dipeptides can initiate in vivo without a 5'-cap. The primary RNA substrate for RAN translation of C9ORF72 sense repeats is shown to be the spliced first intron, following its excision from the initial pre-mRNA and transport to the cytoplasm. Cap-independent RAN translation is shown to be upregulated by various stress stimuli through phosphorylation of the α subunit of eukaryotic initiation factor-2 (eIF2α), the core event of an integrated stress response (ISR)...
January 4, 2018: Nature Communications
https://www.readbyqxmd.com/read/29287728/-towards-a-national-strategy-on-the-diagnosis-of-neurocognitive-disorders-a-shared-approach-among-the-french-national-college-of-general-practitioners-and-specialists-of-neurocognitive-disorders
#5
Pierre Krolak-Salmon, Laurent Letrilliart, Mathieu Ceccaldi, Sandrine Andrieu, Olivier Guérin, Bruno Dubois, Bruno Brochet, Pierre Vandel, Claude Jeandel, Armelle Leperre-Desplanques, Michel Clanet, Pierre-Louis Druais
Neurocognitive disorders leading to progressive cognitive, functional and behavioural impairment are often undiagnosed or diagnosed lately. But tailored care and therapeutics help in implementing secondary and tertiary prevention dynamics aiming at preserving quality of life and delaying, anticipating or preventing behavioural crisis and severe stages of dementia. Moreover, the diagnosis of numerous diseases induces specific care and therapeutics, as well access to research and clinical trials. For the first time, the representatives of the National College of General Practitioners, the French Federation of Memory Centres, the French Federation of Gerontology and Geriatrics, the French Federation of Neurology, the French Society of Psychogeriatrics and the national plan on neurodegenerative diseases propose a graduated and tailored diagnosis strategy involving primary care and specialists of neurocognitive disorders...
December 26, 2017: La Presse Médicale
https://www.readbyqxmd.com/read/29251794/11%C3%AE-hsd1-deficiency-or-inhibition-enhances-hepatic-myofibroblast-activation-in-murine-liver-fibrosis
#6
X Zou, P Ramachandran, T J Kendall, A Pellicoro, E Dora, R L Aucott, K Manwani, T Y Man, K E Chapman, N C Henderson, S J Forbes, S P Webster, J P Iredale, B R Walker, Z Michailidou
A hallmark of chronic liver injury is fibrosis, with accumulation of extracellular matrix orchestrated by activated hepatic stellate cells (HSCs). Glucocorticoids (GC) limit HSC activation in vitro and tissue GC levels are amplified by 11beta-hydroxysteroid dehydrogenase-1 (11βHSD1). Although 11βHSD1 inhibitors have been developed for type 2 diabetes mellitus and improve diet-induced fatty liver in various mouse models, effects on the progression and/or resolution of liver injury and consequent fibrosis have not been characterised...
December 18, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/29241655/citalopram-restores-short-term-memory-deficit-and-non-cognitive-behaviors-in-app-ps1-mice-while-halting-the-advance-of-alzheimer-s-disease-like-pathology
#7
Qin Zhang, Chen Yang, Tianyao Liu, Liang Liu, Fen Li, Yulong Cai, Keyi Lv, Xin Li, Junwei Gao, Dayu Sun, Haiwei Xu, Qingwu Yang, Xiaotang Fan
Alzheimer's disease (AD) is the most common cause of dementia. In addition to cognitive impairments, deficits in non-cognitive behaviors are also common neurological sequelae in AD. Here, we show that complex behavioral deficits in 6-month-old APPswe/PSEN1dE9 (APP/PS1) mice include impairments in object recognition, deficient social interaction, increased depression and buried marbles. Citalopram, one of the selective serotonin reuptake inhibitors (SSRIs), ameliorated the amyloid deposition in AD patients and transgenic animal models...
December 11, 2017: Neuropharmacology
https://www.readbyqxmd.com/read/29229845/brain-urea-increase-is-an-early-huntington-s-disease-pathogenic-event-observed-in-a-prodromal-transgenic-sheep-model-and-hd-cases
#8
Renee R Handley, Suzanne J Reid, Rudiger Brauning, Paul Maclean, Emily R Mears, Imche Fourie, Stefano Patassini, Garth J S Cooper, Skye R Rudiger, Clive J McLaughlan, Paul J Verma, James F Gusella, Marcy E MacDonald, Henry J Waldvogel, C Simon Bawden, Richard L M Faull, Russell G Snell
The neurodegenerative disorder Huntington's disease (HD) is typically characterized by extensive loss of striatal neurons and the midlife onset of debilitating and progressive chorea, dementia, and psychological disturbance. HD is caused by a CAG repeat expansion in the Huntingtin (HTT) gene, translating to an elongated glutamine tract in the huntingtin protein. The pathogenic mechanism resulting in cell dysfunction and death beyond the causative mutation is not well defined. To further delineate the early molecular events in HD, we performed RNA-sequencing (RNA-seq) on striatal tissue from a cohort of 5-y-old OVT73-line sheep expressing a human CAG-expansion HTT cDNA transgene...
December 26, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/29223183/conversion-between-addenbrooke-s-cognitive-examination-iii-and-mini-mental-state-examination
#9
Jordi A Matías-Guiu, Vanesa Pytel, Ana Cortés-Martínez, María Valles-Salgado, Teresa Rognoni, Teresa Moreno-Ramos, Jorge Matías-Guiu
BACKGROUND: We aim to provide a conversion between Addenbrooke's Cognitive Examination III (ACE-III) and Mini-Mental State Examination (MMSE) scores, to predict the MMSE result based on ACE-III, thus avoiding the need for both tests, and improving their comparability. METHODS: Equipercentile equating method was used to elaborate a conversion table using a group of 400 participants comprising healthy controls and Alzheimer's disease (AD) patients. Then, reliability was assessed in a group of 100 healthy controls and patients with AD, 52 with primary progressive aphasia and 22 with behavioral variant frontotemporal dementia...
December 10, 2017: International Psychogeriatrics
https://www.readbyqxmd.com/read/29213499/an-interdisciplinary-approach-aiding-the-diagnosis-of-primary-progressive-aphasia-a-case-report
#10
Nadia Shigaeff, Mayra Zanetti, Sibelle de Almeida Tierno, Ana Beatriz Galhardi Di Tommaso, Thais Cristina Marques, Fábio Gazelato de Mello Franco
Frontotemporal dementia (FTD) is one of the most common causes of early-onset dementia with primary progressive aphasia (PPA) being the second-most-frequent form of this degenerative disease. Despite the similarity with progressive dementia (especially in early stages of Alzheimer´s disease), three types of PPA can be differentiated: semantic, agrammatic and logopenic (subtype discussed in this study). To date, no medications have been shown to improve or stabilize cognitive deficits in patients with PPA. We report the case of a 62-year-old woman with difficulty naming objects and planning...
January 2017: Dementia & Neuropsychologia
https://www.readbyqxmd.com/read/29204655/blood-pressure-trajectories-in-the-20-years-before-death
#11
João Delgado, Kirsty Bowman, Alessandro Ble, Jane Masoli, Yang Han, William Henley, Scott Welsh, George A Kuchel, Luigi Ferrucci, David Melzer
Importance: There is mixed evidence that blood pressure (BP) stabilizes or decreases in later life. It is also unclear whether BP trajectories reflect advancing age, proximity to end of life, or selective survival of persons free from hypertension. Objective: To estimate individual patient BP for each of the 20 years before death and identify potential mechanisms that may explain trajectories. Design, Study, and Participants: We analyzed population-based Clinical Practice Research Datalink primary care and linked hospitalization electronic medical records from the United Kingdom, using retrospective cohort approaches with generalized linear mixed-effects modeling...
December 4, 2017: JAMA Internal Medicine
https://www.readbyqxmd.com/read/29201014/impaired-interoceptive-accuracy-in-semantic-variant-primary-progressive-aphasia
#12
Charles R Marshall, Chris J D Hardy, Lucy L Russell, Camilla N Clark, Katrina M Dick, Emilie V Brotherhood, Rebecca L Bond, Catherine J Mummery, Jonathan M Schott, Jonathan D Rohrer, James M Kilner, Jason D Warren
Background: Interoception (the perception of internal bodily sensations) is strongly linked to emotional experience and sensitivity to the emotions of others in healthy subjects. Interoceptive impairment may contribute to the profound socioemotional symptoms that characterize frontotemporal dementia (FTD) syndromes, but remains poorly defined. Methods: Patients representing all major FTD syndromes and healthy age-matched controls performed a heartbeat counting task as a measure of interoceptive accuracy...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/29191183/an-economic-model-of-advance-care-planning-in-australia-a-cost-effective-way-to-respect-patient-choice
#13
Kim-Huong Nguyen, Marcus Sellars, Meera Agar, Sue Kurrle, Adele Kelly, Tracy Comans
BACKGROUND: Advance care planning (ACP) is a process of planning for future health and personal care. A person's values and preferences are made known so that they can guide decision making at a future time when that person cannot make or communicate his or her decisions. This is particularly relevant for people with dementia because their ability to make decisions progressively deteriorates over time. This study aims to evaluate the cost-effectiveness of delivering a nationwide ACP program within the Australian primary care setting...
December 1, 2017: BMC Health Services Research
https://www.readbyqxmd.com/read/29183926/protocol-for-a-feasibility-randomised-controlled-trial-of-screening-and-enhanced-risk-management-for-vascular-event-related-decline-in-memory-served-memory
#14
Phyo Kyaw Myint, Yoon K Loke, William Davison, Katharina Mattishent, George Christopher Fox, Robert Fleetcroft, David Turner, Lee Shepstone, John F Potter
INTRODUCTION: Stroke is a leading cause of death and disability. The development of dementia after stroke is common. Vascular risk factors (VRF) which contribute to stroke risk can also contribute to cognitive decline, especially in vascular dementia (VaD). There is no established treatment for VaD, therefore strategies for prevention could have major health resource implications. This study was designed to assess whether patients with early cognitive decline after stroke/transient ischaemic attack (TIA) can be easily identified and whether target-driven VRF management can prevent progression to dementia...
November 28, 2017: BMJ Open
https://www.readbyqxmd.com/read/29167065/a-systematic-review-of-practice-guidelines-and-recommendations-for-discontinuation-of-cholinesterase-inhibitors-in-dementia
#15
REVIEW
Brenna N Renn, Ali Abbas Asghar-Ali, Stephen Thielke, Angela Catic, Sharyl R Martini, Brian G Mitchell, Mark E Kunik
Cholinesterase inhibitors (ChEIs) are the primary pharmacological treatment for symptom management of Alzheimer disease (AD), but they carry known risks during long-term use, and do not guarantee clinical effects over time. The balance of risks and benefits may warrant discontinuation at different points during the disease course. Indeed, although there is limited scientific study of deprescribing ChEIs, clinicians routinely face practical decisions about whether to continue or stop medications. This review examined published practice recommendations for discontinuation of ChEIs in AD...
October 10, 2017: American Journal of Geriatric Psychiatry
https://www.readbyqxmd.com/read/29149795/development-of-a-psycho-educational-support-program-for-individuals-with-primary-progressive-aphasia-and-their-care-partners
#16
Darby J Morhardt, Mary C O'Hara, Kristine Zachrich, Christina Wieneke, Emily J Rogalski
Primary progressive aphasia is a language-based dementia that initially spares other cognitive domains; however, aphasia interferes with many life roles such as work and interpersonal relationships. Psycho-educational programs, such as support groups have been shown to be effective for persons with Alzheimer's dementia; however, little is known regarding their effectiveness for persons with primary progressive aphasia. This paper describes the development of a program that offers support, education and activities for persons with primary progressive aphasia and their care-partners and its feasibility...
January 1, 2017: Dementia
https://www.readbyqxmd.com/read/29142232/tdp-43-misexpression-causes-defects-in-dendritic-growth
#17
Josiah J Herzog, Mugdha Deshpande, Leah Shapiro, Avital A Rodal, Suzanne Paradis
Amyotrophic Lateral Sclerosis (ALS) and Frontotemporal Dementia (FTD) share overlapping genetic causes and disease symptoms, and are linked neuropathologically by the RNA binding protein TDP-43 (TAR DNA binding protein-43 kDa). TDP-43 regulates RNA metabolism, trafficking, and localization of thousands of target genes. However, the cellular and molecular mechanisms by which dysfunction of TDP-43 contributes to disease pathogenesis and progression remain unclear. Severe changes in the structure of neuronal dendritic arbors disrupt proper circuit connectivity, which in turn could contribute to neurodegenerative disease...
November 15, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29122458/adult-onset-leukoencephalopathy-with-axonal-spheroids-and-pigmented-glia-alsp-integrating-the-literature-on-hereditary-diffuse-leukoencephalopathy-with-spheroids-hdls-and-pigmentary-orthochromatic-leukodystrophy-pold
#18
REVIEW
Scott J Adams, Andrew Kirk, Roland N Auer
Adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP) is a progressive degenerative white matter disorder. ALSP was previously recognized as two distinct entities, hereditary diffuse leukoencephalopathy with spheroids (HDLS) and pigmentary orthochromatic leukodystrophy (POLD). However, recent identification of mutations in the tyrosine kinase domain of the colony stimulating factor 1 receptor (CSF1R) gene, which regulates mononuclear cell lineages including microglia, have provided genetic and mechanistic evidence that POLD and HDLS should be regarded as a single clinicopathologic entity...
November 6, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/29117794/cognitive-telerehabilitation-in-mild-cognitive-impairment-alzheimer-s-disease-and-frontotemporal-dementia-a-systematic-review
#19
Maria Cotelli, Rosa Manenti, Michela Brambilla, Elena Gobbi, Clarissa Ferrari, Giuliano Binetti, Stefano F Cappa
Introduction Given the limited effectiveness of pharmacological treatments, non-pharmacological interventions in neurodegenerative diseases have gained increasing attention in recent years and telerehabilitation has been proposed as a cognitive rehabilitation strategy. The purpose of this systematic review is to examine the evidence for the efficacy of cognitive telerehabilitation interventions compared with face-to-face rehabilitation in patients with mild cognitive impairment, Alzheimer's disease and frontotemporal dementia...
January 1, 2017: Journal of Telemedicine and Telecare
https://www.readbyqxmd.com/read/29109235/glial-draper-rescues-a%C3%AE-toxicity-in-a-drosophila-model-of-alzheimer-s-disease
#20
Arpita Ray, Sean D Speese, Mary A Logan
Pathological hallmarks of Alzheimer's disease (AD) include amyloid-beta (Aβ) plaques, neurofibrillary tangles, and reactive gliosis. Glial cells offer protection against AD by engulfing extracellular Aβ peptides, but the repertoire of molecules required for glial recognition and destruction of Aβ are still unclear. Here, we show that the highly conserved glial engulfment receptor Draper/MEGF10 provides neuroprotection in an AD model of Drosophila (both sexes). Neuronal expression of human Aβ42(arc) in adult flies results in robust Aβ accumulation, neurodegeneration, locomotor dysfunction, and reduced lifespan...
November 6, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
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