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Primary progressive dementia

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https://www.readbyqxmd.com/read/29149795/development-of-a-psycho-educational-support-program-for-individuals-with-primary-progressive-aphasia-and-their-care-partners
#1
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
#2
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
#3
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
#4
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
#5
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
https://www.readbyqxmd.com/read/29107846/relevance-of-raised-cerebrospinal-fluid-monocyte-levels-in-patients-with-frontotemporal-dementia
#6
Matthias Pawlowski, Gero Lueg, Catharina C Gross, Andreas Johnen, Julia Krämer, Matthias Weckesser, Heinz Wiendl, Sven G Meuth, Thomas Duning
Frontotemporal dementia (FTD) is a heterogeneous neurodegenerative disorder. The contribution of the immune system to its pathogenesis remains incompletely understood. In this study, we performed comprehensive immune cell profiling in the cerebrospinal fluid (CSF) and peripheral blood of patients with FTD. Thirty-two patients with behavioral variant frontotemporal dementia and 25 patients with primary progressive aphasia were included and compared to 14 healthy elderly controls. All patients underwent neuropsychological examination, magnetic resonance imaging, voxel-based diffusion tensor imaging, and peripheral blood and CSF immune cell profiling by multiparameter flow cytometry...
October 13, 2017: Neurobiology of Aging
https://www.readbyqxmd.com/read/29107401/-map-of-alzheimer-s-disease-and-other-dementias-in-spain-mapea-project
#7
Pablo Martínez-Lage, Manuel Martín-Carrasco, Enrique Arrieta, Jesús Rodrigo, Francesc Formiga
INTRODUCTION: In the current context of increased life expectancy and progressive aging of the population a very significant increase in the number of people with cognitive impairment and dementia is expected. Consequently, Spain will face an enormous social and health problem in the next decades. The Mapa de la enfermedad de Alzheimer y otras demencias en España project aims to analyse plans, prevention and early diagnosis activities, process of care and resources available across the 17 Spanish regions for the management of cognitive impairment and dementia in order to identify improvement areas, as well as to provide a list of recommendations...
October 26, 2017: Revista Española de Geriatría y Gerontología
https://www.readbyqxmd.com/read/29105977/tau-pet-imaging-predicts-cognition-in-atypical-variants-of-alzheimer-s-disease
#8
Jeffrey S Phillips, Sandhitsu R Das, Corey T McMillan, David J Irwin, Emily E Roll, Fulvio Da Re, Ilya M Nasrallah, David A Wolk, Murray Grossman
Accumulation of paired helical filament tau contributes to neurodegeneration in Alzheimer's disease (AD). (18) F-flortaucipir is a positron emission tomography (PET) radioligand sensitive to tau in AD, but its clinical utility will depend in part on its ability to predict cognitive symptoms in diverse dementia phenotypes associated with selective, regional uptake. We examined associations between (18) F-flortaucipir and cognition in 14 mildly-impaired patients (12 with cerebrospinal fluid analytes consistent with AD pathology) who had amnestic (n = 5) and non-amnestic AD syndromes, including posterior cortical atrophy (PCA, n = 5) and logopenic-variant primary progressive aphasia (lvPPA, n = 4)...
November 6, 2017: Human Brain Mapping
https://www.readbyqxmd.com/read/29097599/brains-with-sporadic-creutzfeldt-jakob-disease-and-copathology-showed-a-prolonged-end-stage-of-disease
#9
Aitzol Miguelez-Rodriguez, Jorge Santos-Juanes, Ikerne Vicente-Etxenausia, Katty Perez de Heredia-Goñi, Beatriz Garcia, Luis M Quiros, Laura Lorente-Gea, Isabel Guerra-Merino, Jose J Aguirre, Ivan Fernandez-Vega
AIMS: To investigate the expression of major proteins related to primary neurodegenerative diseases and their prognostic significance in brains with Creutzfeldt-Jakob disease (CJD). MATERIALS AND METHODS: Thirty consecutive cases of confirmed CJD during the period 2010-2015 at Basque Brain bank were retrospectively reviewed. Moreover, major neurodegenerative-associated proteins (phosphorylated Tau, 4R tau, 3R tau, alpha-synuclein, TDP43, amyloid beta) were tested...
November 2, 2017: Journal of Clinical Pathology
https://www.readbyqxmd.com/read/29097166/24-month-intervention-with-a-specific-multinutrient-in-people-with-prodromal-alzheimer-s-disease-lipididiet-a-randomised-double-blind-controlled-trial
#10
Hilkka Soininen, Alina Solomon, Pieter Jelle Visser, Suzanne B Hendrix, Kaj Blennow, Miia Kivipelto, Tobias Hartmann
BACKGROUND: Nutrition is an important modifiable risk factor in Alzheimer's disease. Previous trials of the multinutrient Fortasyn Connect showed benefits in mild Alzheimer's disease dementia. LipiDiDiet investigated the effects of Fortasyn Connect on cognition and related measures in prodromal Alzheimer's disease. Here, we report the 24-month results of the trial. METHODS: LipiDiDiet was a 24-month randomised, controlled, double-blind, parallel-group, multicentre trial (11 sites in Finland, Germany, the Netherlands, and Sweden), with optional 12-month double-blind extensions...
October 30, 2017: Lancet Neurology
https://www.readbyqxmd.com/read/29053874/tau-pathology-and-neurodegeneration-contribute-to-cognitive-impairment-in-alzheimer-s-disease
#11
Alexandre Bejanin, Daniel R Schonhaut, Renaud La Joie, Joel H Kramer, Suzanne L Baker, Natasha Sosa, Nagehan Ayakta, Averill Cantwell, Mustafa Janabi, Mariella Lauriola, James P O'Neil, Maria L Gorno-Tempini, Zachary A Miller, Howard J Rosen, Bruce L Miller, William J Jagust, Gil D Rabinovici
Neuropathological and in vivo studies have revealed a tight relationship between tau pathology and cognitive impairment across the Alzheimer's disease spectrum. However, tau pathology is also intimately associated with neurodegeneration and amyloid pathology. The aim of the present study was therefore to assess whether grey matter atrophy and amyloid pathology contribute to the relationship between tau pathology, as measured with 18F-AV-1451-PET imaging, and cognitive deficits in Alzheimer's disease. We included 40 amyloid-positive patients meeting criteria for mild cognitive impairment due to Alzheimer's disease (n = 5) or probable Alzheimer's disease dementia (n = 35)...
October 7, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/29053860/clinicopathological-correlations-in-behavioural-variant-frontotemporal-dementia
#12
David C Perry, Jesse A Brown, Katherine L Possin, Samir Datta, Andrew Trujillo, Anneliese Radke, Anna Karydas, John Kornak, Ana C Sias, Gil D Rabinovici, Maria Luisa Gorno-Tempini, Adam L Boxer, Mary De May, Katherine P Rankin, Virginia E Sturm, Suzee E Lee, Brandy R Matthews, Aimee W Kao, Keith A Vossel, Maria Carmela Tartaglia, Zachary A Miller, Sang Won Seo, Manu Sidhu, Stephanie E Gaus, Alissa L Nana, Jose Norberto S Vargas, Ji-Hye L Hwang, Rik Ossenkoppele, Alainna B Brown, Eric J Huang, Giovanni Coppola, Howard J Rosen, Daniel Geschwind, John Q Trojanowski, Lea T Grinberg, Joel H Kramer, Bruce L Miller, William W Seeley
Accurately predicting the underlying neuropathological diagnosis in patients with behavioural variant frontotemporal dementia (bvFTD) poses a daunting challenge for clinicians but will be critical for the success of disease-modifying therapies. We sought to improve pathological prediction by exploring clinicopathological correlations in a large bvFTD cohort. Among 438 patients in whom bvFTD was either the top or an alternative possible clinical diagnosis, 117 had available autopsy data, including 98 with a primary pathological diagnosis of frontotemporal lobar degeneration (FTLD), 15 with Alzheimer's disease, and four with amyotrophic lateral sclerosis who lacked neurodegenerative disease-related pathology outside of the motor system...
October 6, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/29050205/catalpol-protects-synaptic-proteins-from-beta-amyloid-induced-neuron-injury-and-improves-cognitive-functions-in-aged-rats
#13
Zhiming Xia, Fengfei Wang, Shuang Zhou, Rui Zhang, Fushun Wang, Jason H Huang, Erxi Wu, Yongfang Zhang, Yaer Hu
Synapse loss is one of the common factors contributing to cognitive disorders, such as Alzheimer's disease (AD), which is manifested by the impairment of basic cognitive functions including memory processing, perception, problem solving, and language. The current therapies for patients with cognitive disorders are mainly palliative; thus, regimens preventing and/or delaying dementia progression are urgently needed. In this study, we evaluated the effects of catalpol, isolated from traditional Chinese medicine Rehmannia glutinosa, on synaptic plasticity in aged rat models...
September 19, 2017: Oncotarget
https://www.readbyqxmd.com/read/29046362/in-vivo-staging-of-regional-amyloid-deposition
#14
Michel J Grothe, Henryk Barthel, Jorge Sepulcre, Martin Dyrba, Osama Sabri, Stefan J Teipel
OBJECTIVES: To estimate a regional progression pattern of amyloid deposition from cross-sectional amyloid-sensitive PET data and evaluate its potential for in vivo staging of an individual's amyloid pathology. METHODS: Multiregional analysis of florbetapir ((18)F-AV45)-PET data was used to determine individual amyloid distribution profiles in a sample of 667 participants from the Alzheimer's Disease Neuroimaging Initiative cohort, including cognitively normal older individuals (CN) as well as patients with mild cognitive impairment and Alzheimer disease (AD) dementia...
October 18, 2017: Neurology
https://www.readbyqxmd.com/read/29043848/lexical-access-in-a-bilingual-speaker-with-dementia-changes-over-time
#15
Marianne Lind, Hanne Gram Simonsen, Ingeborg Sophie Bjønness Ribu, Bente Ailin Svendsen, Jan Svennevig, Kees de Bot
In this article, we explore the naming skills of a bilingual English-Norwegian speaker diagnosed with Primary Progressive Aphasia, in each of his languages across three different speech contexts: confrontation naming, semi-spontaneous narrative (picture description), and conversation, and at two points in time: 12 and 30 months post diagnosis, respectively. The results are discussed in light of two main theories of lexical retrieval in healthy, elderly speakers: the Transmission Deficit Hypothesis and the Inhibitory Deficit Theory...
October 18, 2017: Clinical Linguistics & Phonetics
https://www.readbyqxmd.com/read/29038245/mitochondrial-calcium-dysregulation-contributes-to-dendrite-degeneration-mediated-by-pd-lbd-associated-lrrk2-mutants
#16
Manish Verma, Jason Callio, P Anthony Otero, Israel Sekler, Zachary P Wills, Charleen T Chu
Mutations in leucine-rich repeat kinase 2 (LRRK2) contribute to development of late-onset familial Parkinson's disease (PD), with clinical features of motor and cognitive dysfunction indistinguishable from sporadic PD. Calcium dysregulation plays an important role in PD pathogenesis, but the mechanisms of neurodegeneration remain unclear. Recent reports indicate enhanced excitatory neurotransmission in cortical neurons expressing mutant LRRK2, which occurs prior to the well-characterized phenotype of dendritic shortening...
October 16, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28991698/pitx3-genotype-and-risk-of-dementia-in-parkinson-s-disease-a-population-based-study
#17
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/28990425/mendelian-forms-of-disease-and-age-at-onset-affect-survival-in-frontotemporal-dementia
#18
Maura Cosseddu, Alberto Benussi, Stefano Gazzina, Rosanna Turrone, Silvana Archetti, Elisa Bonomi, Giorgio Biasiotto, Isabella Zanella, Raffaele Ferrari, Maria S Cotelli, Antonella Alberici, Alessandro Padovani, Barbara Borroni
OBJECTIVE: Frontotemporal dementia (FTD) is a common cause of young onset dementia. Very few reports on disease duration are currently available and predictors of survival are still undefined. The aim of the present study was to assess the natural history of FTD and to define predictors of survival. METHODS: Four hundred amd eleven FTD patients, including 294 with behavioural variant FTD, 77 with agrammatic variant primary progressive aphasia (PPA) and 40 with semantic variant PPA, were consecutively enrolled and demographic and clinical variables carefully recorded...
October 8, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28987182/tauopathies
#19
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/28982489/palliative-care-in-neurology
#20
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
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