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Primary progressive dementia

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https://www.readbyqxmd.com/read/28413716/data-driven-regions-of-interest-for-longitudinal-change-in-three-variants-of-frontotemporal-lobar-degeneration
#1
Richard J Binney, Aleksandr Pankov, Gabriel Marx, Xuanzie He, Faye McKenna, Adam M Staffaroni, John Kornak, Suneth Attygalle, Adam L Boxer, Norbert Schuff, Maria-Luisa Gorno-Tempini, Michael W Weiner, Joel H Kramer, Bruce L Miller, Howard J Rosen
INTRODUCTION: Longitudinal imaging of neurodegenerative disorders is a potentially powerful biomarker for use in clinical trials. In Alzheimer's disease, studies have demonstrated that empirically derived regions of interest (ROIs) can provide more reliable measurement of disease progression compared with anatomically defined ROIs. METHODS: We set out to derive ROIs with optimal effect size for quantifying longitudinal change in a hypothetical clinical trial by comparing atrophy rates in 44 patients with behavioral variant of frontotemporal dementia (bvFTD), 30 with the semantic variant primary progressive aphasia (svPPA), and 26 with the nonfluent variant PPA (nfvPPA) to atrophy in 97 cognitively healthy controls...
April 2017: Brain and Behavior
https://www.readbyqxmd.com/read/28413412/subjective-cognitive-impairment-is-a-predominantly-benign-condition-in-memory-clinic-patients-followed-for-6-years-the-gothenburg-oslo-mci-study
#2
Erik Hessen, Marie Eckerström, Arto Nordlund, Ina Selseth Almdahl, Jacob Stålhammar, Maria Bjerke, Carl Eckerström, Mattias Göthlin, Tormod Fladby, Ivar Reinvang, Anders Wallin
BACKGROUND/AIMS: In the quest for prevention or treatment, there is a need to find early markers for preclinical dementia. This study observed memory clinic patients with subjective cognitive impairment (SCI) and normal cognitive function at baseline. The primary aim was to address SCI as a potential risk factor for cognitive decline. The secondary aim was to address a potential relation between (1) baseline cerebrospinal fluid biomarkers and (2) a decline in memory performance over the first 2 years of follow-up, with a possible cognitive decline after 6 years...
January 2017: Dementia and Geriatric Cognitive Disorders Extra
https://www.readbyqxmd.com/read/28412599/group-intervention-for-individuals-with-primary-progressive-aphasia-and-their-spouses-who-comes-first
#3
Regina Jokel, Jed Meltzer, J D R, L D M, J J C, E A N, C D T
Primary progressive aphasia (PPA) is a neurodegenerative dementia in which language impairment is the first and most dominant symptom. There is a considerable dearth of interventions for PPA although language rehabilitation has made headway in managing the disorder. Thus far, no comprehensive services have been proposed for PPA clients and/or their spouses. This paper describes the first structured group intervention program designated exclusively for people with PPA and their caregivers. This pilot project originates from a clinical service and presents supporting evidence for initiation of a larger study to establish an evidence-based intervention for PPA...
April 9, 2017: Journal of Communication Disorders
https://www.readbyqxmd.com/read/28410663/frontotemporal-dementia
#4
REVIEW
Nicholas T Olney, Salvatore Spina, Bruce L Miller
Frontotemporal dementia (FTD) is a heterogeneous disorder with distinct clinical phenotypes associated with multiple neuropathologic entities. Presently, the term FTD encompasses clinical disorders that include changes in behavior, language, executive control, and often motor symptoms. The core FTD spectrum disorders include behavioral variant FTD, nonfluent/agrammatic variant primary progressive aphasia, and semantic variant PPA. Related FTD disorders include frontotemporal dementia with motor neuron disease, progressive supranuclear palsy syndrome, and corticobasal syndrome...
May 2017: Neurologic Clinics
https://www.readbyqxmd.com/read/28398367/-implantable-cardioverter-defibrillator-deactivation-at-the-end-of-life-ethical-clinical-and-communication-issues
#5
Massimo Romanò, Maria Antonella Piga, Roberta Bertona, Roberto Negro, Chiara Ruggeri, Federica Zorzoli, Rosvaldo Villani
The number of cardioverter-defibrillator implants is increasing worldwide, with the main indication being primary prevention of sudden cardiac death. During the follow-up, patients may die from progression of their underlying heart disease or from nonarrhythmic causes, such as malignancies, dementia and lung disease, without receiving appropriate shocks until the last few days or weeks of their life. These events occur roughly in 30% of patients, mainly in the last 24 hours before death. In this case, inappropriate and even appropriate shock deliveries can no longer prolong life and may simply lead to pain and reduced quality of life...
February 2017: Giornale Italiano di Cardiologia
https://www.readbyqxmd.com/read/28387447/withdrawn-interventions-for-fatigue-and-weight-loss-in-adults-with-advanced-progressive-illness
#6
REVIEW
Cathy Payne, Philip J Wiffen, Suzanne Martin
BACKGROUND: Fatigue and unintentional weight loss are two of the commonest symptoms experienced by people with advanced progressive illness. Appropriate interventions may bring considerable improvements in function and quality of life to seriously ill people and their families, reducing physical, psychological and spiritual distress. OBJECTIVES: To conduct an overview of the evidence available on the efficacy of interventions used in the management of fatigue and/or unintentional weight loss in adults with advanced progressive illness by reviewing the evidence contained within Cochrane reviews...
April 7, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28379955/preferences-for-care-towards-the-end-of-life-when-decision-making-capacity-may-be-impaired-a-large-scale-cross-sectional-survey-of-public-attitudes-in-great-britain-and-the-united-states
#7
Gemma Clarke, Elizabeth Fistein, Anthony Holland, Matthew Barclay, Pia Theimann, Stephen Barclay
BACKGROUND: There is continuing public debate about treatment preferences at the end of life, and the acceptability and legal status of treatments that sustain or end life. However, most surveys use binary yes/no measures, and little is known about preferences in neurological disease when decision-making capacity is lost, as most studies focus on cancer. This study investigates changes in public preferences for care towards the end of life, with a focus on measures to sustain or end life...
2017: PloS One
https://www.readbyqxmd.com/read/28373956/a-neural-network-underlying-intentional-emotional-facial-expression-in-neurodegenerative-disease
#8
Kelly A Gola, Tal Shany-Ur, Peter Pressman, Isa Sulman, Eduardo Galeana, Hillary Paulsen, Lauren Nguyen, Teresa Wu, Babu Adhimoolam, Pardis Poorzand, Bruce L Miller, Katherine P Rankin
Intentional facial expression of emotion is critical to healthy social interactions. Patients with neurodegenerative disease, particularly those with right temporal or prefrontal atrophy, show dramatic socioemotional impairment. This was an exploratory study examining the neural and behavioral correlates of intentional facial expression of emotion in neurodegenerative disease patients and healthy controls. One hundred and thirty three participants (45 Alzheimer's disease, 16 behavioral variant frontotemporal dementia, 8 non-fluent primary progressive aphasia, 10 progressive supranuclear palsy, 11 right-temporal frontotemporal dementia, 9 semantic variant primary progressive aphasia patients and 34 healthy controls) were video recorded while imitating static images of emotional faces and producing emotional expressions based on verbal command; the accuracy of their expression was rated by blinded raters...
2017: NeuroImage: Clinical
https://www.readbyqxmd.com/read/28363508/risk-factors-associated-with-the-onset-and-progression-of-alzheimer-s-disease-a-systematic-review-of-the-evidence
#9
Mona Hersi, Brittany Irvine, Pallavi Gupta, James Gomes, Nicholas Birkett, Daniel Krewski
A systematic review was conducted to identify risk factors associated with the onset and progression of Alzheimer's disease (AD). Moderate and high quality systematic reviews were eligible for inclusion. Primary studies reporting on non-genetic risk factors associated with neuropathologically or clinically confirmed AD were considered. Eighty one systematic reviews reporting on AD onset and 12 reporting on progression satisfied the eligibility criteria. Four hundred and thirty-two relevant primary studies reporting on onset were identified; however, only those published between 2010 and 2012 (n=65) were included in the qualitative synthesis...
March 28, 2017: Neurotoxicology
https://www.readbyqxmd.com/read/28333613/collaborative-management-of-neurocognitive-disorders-in-primary-care-explorations-of-an-attempt-at-culture-change
#10
Lewis Mehl-Madrona, Barbara Mainguy
INTRODUCTION: Minor neurocognitive disorder (MiND; previously mild cognitive impairment) is a transitional zone between normal cognitive function and early stages of major neurocognitive disorder (previously called dementia). Of people with MiND, 5% to 10% progress to major neurocognitive disorder. Simple interventions such as memory activities, balance exercises, and anti-inflammatory diets have been shown to improve cognitive ability. Also, education and support in group settings have proved beneficial for patients with MiND...
2017: Permanente Journal
https://www.readbyqxmd.com/read/28330458/the-effects-of-an-extensive-exercise-programme-on-the-progression-of-mild-cognitive-impairment-mci-study-protocol-for-a-randomised-controlled-trial
#11
Kate E Devenney, Brian Lawlor, Marcel G M Olde Rikkert, Stefan Schneider
BACKGROUND: Exercise interventions to prevent dementia and delay cognitive decline have gained considerable attention in recent years. Human and animal studies have demonstrated that regular physical activity targets brain function by increasing cognitive reserve. There is also evidence of structural changes caused by exercise in preventing or delaying the genesis of neurodegeneration. Although initial studies indicate enhanced cognitive performance in patients with mild cognitive impairment (MCI) following an exercise intervention, little is known about the effect of an extensive, controlled and regular exercise regimen on the neuropathology of patients with MCI...
March 22, 2017: BMC Geriatrics
https://www.readbyqxmd.com/read/28323683/genetic-alzheimer-disease-and-sporadic-dementia-with-lewy-bodies-a-comorbidity-presenting-as-primary-progressive-aphasia
#12
Tereza Picková, Radoslav Matěj, Ondrej Bezdicek, Jiří Keller, Julie van der Zee, Christine Van Broeckhoven, Zsolt Cséfalvay, Robert Rusina
We report a 44-year-old woman, with a family history of early-onset dementia, presenting with primary progressive aphasia. This clinically variable syndrome has multiple underlying pathologies, and correlations between clinical manifestations and postmortem neuropathologic findings are controversial. Our patient suffered worsening language impairment with major word-finding difficulties but preserved comprehension. She also developed episodic memory impairment. Her condition progressed to dementia with behavioral changes...
March 2017: Cognitive and Behavioral Neurology: Official Journal of the Society for Behavioral and Cognitive Neurology
https://www.readbyqxmd.com/read/28320296/biological-basis-for-amyloidogenesis-in-alzheimer-s-disease
#13
REVIEW
T V Andreeva, W J Lukiw, E I Rogaev
Certain cellular proteins normally soluble in the living organism under certain conditions form aggregates with a specific cross-β sheet structure called amyloid. These intra- or extracellular insoluble aggregates (fibers or plaques) are hallmarks of many neurodegenerative pathologies including Alzheimer's disease (AD), Huntington's disease, Parkinson's disease, prion disease, and other progressive neurological diseases that develop in the aging human central nervous system. Amyloid diseases (amyloidoses) are widespread in the elderly human population, a rapidly expanding demographic in many global populations...
February 2017: Biochemistry. Biokhimii︠a︡
https://www.readbyqxmd.com/read/28304311/frontotemporal-dementia-due-to-the-novel-grn-arg161glyfsx36-mutation
#14
Stefano Gazzina, Silvana Archetti, Antonella Alberici, Elisa Bonomi, Maura Cosseddu, Diego Di Lorenzo, Alessandro Padovani, Barbara Borroni
Progranulin is a multifunctional growth factor mainly expressed in neurons and microglia. Loss-of-function mutations in the Granulin (GRN) gene are causative of frontotemporal dementia with TAR DNA-binding protein-43 inclusions. We reported the case of a 51-year-old male patient affected by sporadic agrammatic variant of primary progressive aphasia, in whom we identified a novel heterozygous deletion in the exon 6 (g.10338_39delAG, p.Arg161GlyfsX36). Plasma progranulin levels were significantly reduced and in silico analysis predicted a premature termination codon...
March 18, 2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28304308/violation-of-laws-in-frontotemporal-dementia-a-multicenter-study-in-japan
#15
Shunichiro Shinagawa, Kazue Shigenobu, Kenji Tagai, Ryuji Fukuhara, Naoto Kamimura, Takaaki Mori, Kenji Yoshiyama, Hiroaki Kazui, Kazuhiko Nakayama, Manabu Ikeda
Although violations of laws, such as shoplifting, are considered to be common in frontotemporal dementia (FTD) patients, there have been few studies on this subject and the frequencies and types of such violations have not been clarified. The objective of this study was to conduct a retrospective investigation of FTD patients in the psychiatry departments of multiple institutions to determine the types and frequencies of any law violations and compare them with those of AD patients. All patients were examined between January 2011 and December 2015 at the specialized dementia outpatient clinics of 10 facilities (5 psychiatry departments of university hospitals, 5 psychiatric hospitals)...
March 18, 2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28289749/motor-speech-phenotypes-of-frontotemporal-dementia-primary-progressive-aphasia-and-progressive-apraxia-of-speech
#16
Matthew L Poole, Amy Brodtmann, David Darby, Adam P Vogel
Purpose: Our purpose was to create a comprehensive review of speech impairment in frontotemporal dementia (FTD), primary progressive aphasia (PPA), and progressive apraxia of speech in order to identify the most effective measures for diagnosis and monitoring, and to elucidate associations between speech and neuroimaging. Method: Speech and neuroimaging data described in studies of FTD and PPA were systematically reviewed. A meta-analysis was conducted for speech measures that were used consistently in multiple studies...
March 13, 2017: Journal of Speech, Language, and Hearing Research: JSLHR
https://www.readbyqxmd.com/read/28279992/association-of-comorbidity-and-health-service-usage-among-patients-with-dementia-in-the-uk-a-population-based-study
#17
Jorge Browne, Duncan A Edwards, Kirsty M Rhodes, D James Brimicombe, Rupert A Payne
BACKGROUND: The majority of people with dementia have other long-term diseases, the presence of which may affect the progression and management of dementia. This study aimed to identify subgroups with higher healthcare needs, by analysing how primary care consultations, number of prescriptions and hospital admissions by people with dementia varies with having additional long-term diseases (comorbidity). METHODS: A retrospective cohort study based on health data from the Clinical Practice Research Datalink (CPRD) was conducted...
March 9, 2017: BMJ Open
https://www.readbyqxmd.com/read/28274963/mri-based-comparative-study-of-different-mild-cognitive-impairment-subtypes-protocol-for-an-observational-case-control-study
#18
Yang Yu, Weina Zhao, Siou Li, Changhao Yin
INTRODUCTION: Amnestic mild cognitive impairment (aMCI) and vascular mild cognitive impairment (VaMCI) comprise the 2 main types of mild cognitive impairment (MCI). The first condition generally progresses to Alzheimer's disease, whereas the second is likely to develop into vascular dementia (VD). The brain structure and function of patients with MCI differ from those of normal elderly individuals. However, whether brain structures or functions differ between these 2 MCI subtypes has not been studied...
March 8, 2017: BMJ Open
https://www.readbyqxmd.com/read/28235777/observing-conversational-laughter-in-frontotemporal-dementia
#19
Peter S Pressman, Michaela Simpson, Kelly Gola, Suzanne M Shdo, Edoardo G Spinelli, Bruce L Miller, Maria Luisa Gorno-Tempini, Katherine Rankin, Robert W Levenson
BACKGROUND: We performed an observational study of laughter during seminaturalistic conversations between patients with dementia and familial caregivers. Patients were diagnosed with (1) behavioural variant frontotemporal dementia (bvFTD), (2) right temporal variant frontotemporal dementia (rtFTD), (3) semantic variant of primary progressive aphasia (svPPA), (4) non-fluent variant primary progressive aphasia (nfvPPA) or (5) early onset Alzheimer's disease (eoAD). We hypothesised that those with bvFTD would laugh less in response to their own speech than other dementia groups or controls, while those with rtFTD would laugh less regardless of who was speaking...
February 24, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28225048/thiamine-deficiency-oxidative-metabolic-pathways-and-ethanol-induced-neurotoxicity-how-poor-nutrition-contributes-to-the-alcoholic-syndrome-as-marchiafava-bignami-disease
#20
REVIEW
L M P Fernandes, F R Bezerra, M C Monteiro, M L Silva, F R de Oliveira, R R Lima, E A Fontes-Júnior, C S F Maia
Ethanol is an important risk factor for the occurrence of several brain disorders that depend on the amount, period and frequency of its consumption. Chronic use of ethanol often leads to the development of neurodegenerative syndromes, which cause morphological and functional impairments such as foetal alcohol syndrome in newborns exposed to ethanol during pregnancy, Wernicke-Korsakoff Syndrome and, more rarely, Marchiafava-Bignami disease (MBD). MBD is characterized by primary degeneration of the corpus callosum, without inflammation and is associated with oxidative stress and hypovitaminosis, as well as altered mental status, to mention dementia, seizures, depression and so on...
February 22, 2017: European Journal of Clinical Nutrition
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