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Semantic dementia

M Mortamais, J A Ash, J Harrison, J Kaye, J Kramer, C Randolph, C Pose, B Albala, M Ropacki, C W Ritchie, K Ritchie
Significant progress has been made in characterizing the biological changes occurring in preclinical Alzheimer's disease (AD). Cognitive dysfunction has been viewed, however, as a late-stage phenomenon, despite increasing evidence that changes may be detected in the decades preceding dementia. In the absence of comprehensive evidence-based guidelines for preclinical cognitive assessment, longitudinal cohort and neuroimaging studies have been reviewed to determine the temporal order and brain biomarker correlates of specific cognitive functions...
October 1, 2016: Alzheimer's & Dementia: the Journal of the Alzheimer's Association
Aida Suárez-González, Sharon A Savage, Diana Caine
Patients with semantic dementia (SD) can rapidly and successfully re-learn word labels during cognitive intervention. This new learning, however, usually remains rigid and context-dependent. Conceptual enrichment (COEN) training is a therapy approach aimed to produce more flexible and generalisable learning in SD. In this study we compare generalisation and maintenance of learning after COEN with performance achieved using a classical naming therapy (NT). The study recruited a 62-year-old woman with SD. An AB1ACAB2 experimental design was implemented, with naming performance assessed at baseline, post- intervention, 3 and 6 weeks after the end of each treatment phase...
September 28, 2016: Neuropsychological Rehabilitation
Chiara Cerami, Alessandra Dodich, Lucia Greco, Sandro Iannaccone, Giuseppe Magnani, Alessandra Marcone, Elisabetta Pelagallo, Roberto Santangelo, Stefano F Cappa, Daniela Perani
BACKGROUND AND OBJECTIVE: Primary progressive aphasia (PPA) is a clinical syndrome due to different neurodegenerative conditions in which an accurate early diagnosis needs to be supported by a reliable diagnostic tool at the individual level. In this study, we investigated in PPA the FDG-PET brain metabolic patterns at the single-subject level, in order to assess the case-to-case variability and its relationship with clinical-neuropsychological findings. MATERIAL AND METHODS: 55 patients (i...
September 20, 2016: Journal of Alzheimer's Disease: JAD
Raffaella I Rumiati, Francesco Foroni, Giulio Pergola, Paola Rossi, Maria Caterina Silveri
The study of category specific deficits in brain-damaged patients has been instrumental in explaining how knowledge about different types of objects is organized in the brain. Much of this research focused on testing putative semantic sensory/functional subsystems that could explain the observed dissociations in performance between living things (e.g., animals and fruits/vegetables) and non-living things (e.g., tools). As neuropsychological patterns that did not fit the original living/non-living distinction were observed, an alternative organization of semantic memory in domains constrained by evolutionary pressure was hypothesized...
September 17, 2016: Brain and Cognition
Joanna J Gan, Andrew Lin, Mersal S Samimi, Mario F Mendez
BACKGROUND: Semantic dementia (SD) is a neurodegenerative disorder characterized by loss of semantic knowledge. SD may be associated with somatic symptom disorder due to excessive preoccupation with unidentified somatic sensations. OBJECTIVE: To evaluate the frequency of somatic symptom disorder among patients with SD in comparison to comparably demented patients with Alzheimer׳s disease. METHODS: A retrospective cohort study was conducted using clinical data from a referral-based behavioral neurology program...
August 4, 2016: Psychosomatics
Pin-Hsuan Lin, Hsiu-Hui Chen, Nai-Ching Chen, Wen-Neng Chang, Chi-Wei Huang, Ya-Ting Chang, Shih-Wei Hsu, Che-Wei Hsu, Chiung-Chih Chang
PURPOSE: Patients with dementia who have dissociations in verbal and non-verbal sound processing may offer insights into the anatomic basis for highly related auditory modes. METHODS: To determine the neuronal networks on non-verbal perception, 16 patients with Alzheimer's dementia (AD), 15 with behavior variant fronto-temporal dementia (bv-FTD), 14 with semantic dementia (SD) were evaluated and compared with 15 age-matched controls. Neuropsychological and auditory perceptive tasks were included to test the ability to compare pitch changes, scale-violated melody and for naming and associating with environmental sound...
2016: Frontiers in Aging Neuroscience
Karin Fehsel, Tamara Schikowski, Michaela Jänner, Anke Hüls, Mohammed Voussoughi, Thomas Schulte, Andrea Vierkötter, Tom Teichert, Christian Herder, Dorothea Sugiri, Ursula Krämer, Christian Luckhaus
Genetic and environmental risk factors contribute to the pathogenesis of Alzheimer's dementia. Besides known genetic risk factors like the apolipoprotein (APO) Eε4 allele, single nuclear polymorphisms (SNPs) of the estrogen receptors (ESRs) are candidate genetic risk factors, while air pollution represents an environmental risk factor for dementia. Effects of these risk factors and their interaction were investigated in the SALIA cohort of 834 non-demented elderly women. Cognitive function was assessed by the CERAD-plus test battery...
September 14, 2016: Journal of Neural Transmission
Jonathan D Rohrer, Ione O C Woollacott, Katrina M Dick, Emilie Brotherhood, Elizabeth Gordon, Alexander Fellows, Jamie Toombs, Ronald Druyeh, M Jorge Cardoso, Sebastien Ourselin, Jennifer M Nicholas, Niklas Norgren, Simon Mead, Ulf Andreasson, Kaj Blennow, Jonathan M Schott, Nick C Fox, Jason D Warren, Henrik Zetterberg
OBJECTIVE: To investigate serum neurofilament light chain (NfL) concentrations in frontotemporal dementia (FTD) and to see whether they are associated with the severity of disease. METHODS: Serum samples were collected from 74 participants (34 with behavioral variant FTD [bvFTD], 3 with FTD and motor neuron disease and 37 with primary progressive aphasia [PPA]) and 28 healthy controls. Twenty-four of the FTD participants carried a pathogenic mutation in C9orf72 (9), microtubule-associated protein tau (MAPT; 11), or progranulin (GRN; 4)...
September 27, 2016: Neurology
Maxime Bertoux, Siddharth Ramanan, Andrea Slachevsky, Stephanie Wong, Fernando Henriquez, Gada Musa, Carolina Delgado, Emma Flanagan, Michel Bottlaender, Marie Sarazin, Michael Hornberger, Bruno Dubois
BACKGROUND: Memory impairment in behavioral variant frontotemporal dementia (bvFTD) is traditionally considered to be mild and attributed to prefrontal cortex dysfunction. Recent studies, however, indicated that some patients can present with a memory impairment of the hippocampal type, showing storage and consolidation deficits in addition to the more executive/prefrontal related encoding and strategic difficulties. OBJECTIVE: This study aimed to study the relationship between executive functions (EF) and memory processes in bvFTD via a data-driven approach...
October 4, 2016: Journal of Alzheimer's Disease: JAD
Marianne Chapleau, Joséphine Aldebert, Maxime Montembeault, Simona M Brambati
BACKGROUND/OBJECTIVES: Alzheimer's disease (AD) and semantic dementia (SD) have distinct episodic memory profiles despite the hippocampal atrophy that characterizes both diseases. The aim of this study was to delineate the pattern of gray matter (GM) atrophy associated with AD and SD as well as any differences in these patterns by pooling together the results of previous voxel-based morphometry (VBM) studies.Methods/Overview: We conducted a meta-analysis of VBM studies that investigated GM atrophy in AD patients versus controls (CTRLs) and in SD patients versus CTRLs using the activation likelihood estimation (ALE) approach...
October 4, 2016: Journal of Alzheimer's Disease: JAD
R A Armstrong
Factors associated with survival were studied in 84 neuropathologically documented cases of the pre-senile dementia frontotemporal dementia lobar degeneration (FTLD) with transactive response (TAR) DNA-binding protein of 43 kDa (TDP-43) proteinopathy (FTLD-TDP). Kaplan-Meier survival analysis estimated mean survival as 7.9 years (range: 1-19 years, SD = 4.64). Familial and sporadic cases exhibited similar survival, including progranulin (GRN) gene mutation cases. No significant differences in survival were associated with sex, disease onset, Braak disease stage, or disease subtype, but higher survival was associated with lower post-mortem brain weight...
2016: Folia Neuropathologica
Viviana Lo Buono, Francesco Corallo, Angela Marra, Rocco Salvatore Calabrò, Placido Bramanti, Silvia Marino
OBJECT: Semantic dementia is one of the main clinical variants of frontotemporal dementia and it is characterized by severe loss of conceptual knowledge with relative preservation of other cortical abilities. METHODS: We described a case of a patient affected by right temporal variant of frontotemporal lobar degeneration, characterized by prosopagnosia as an unusual disease onset. RESULTS: The neuropsychological evaluation showed a progressive deficit in face recognition and a slow decline in language in the absence of behavioral alterations...
August 4, 2016: Acta Clinica Belgica
Robert J Dawe, Lei Yu, Sue E Leurgans, Julie A Schneider, Aron S Buchman, Konstantinos Arfanakis, David A Bennett, Patricia A Boyle
This study tested the hypothesis that indices of brain tissue integrity derived from postmortem magnetic resonance imaging (MRI) are associated with late life decline in cognitive function and dementia, over and above contributions from common age-related neuropathologies. Cerebral hemispheres were obtained from 425 deceased older adults who had undergone 2 or more annual cognitive assessments, which included clinical diagnosis of dementia. Specimens underwent MRI to produce maps of transverse relaxation rate, R2...
September 2016: Neurobiology of Aging
Gamze Balci Camsari, Melissa E Murray, Neill R Graff-Radford
Many dementia subtypes have more shared signs and symptoms than defining ones. We review 8 cases with 4 overlapping syndromes and demonstrate how to distinguish the cases. These include focal cortical presentations of Alzheimer's disease (AD; posterior cortical atrophy and corticobasal syndrome [CBS]), fluent aphasia (semantic dementia and logopenic aphasia), late-onset slowly progressive dementia (hippocampal sclerosis and limbic predominant AD) and rapidly progressive dementia (Creutzfeldt-Jakob disease and limbic encephalitis)...
August 2016: Neurologic Clinics
Kamalini G Ranasinghe, Katherine P Rankin, Peter S Pressman, David C Perry, Iryna V Lobach, William W Seeley, Giovanni Coppola, Anna M Karydas, Lea T Grinberg, Tal Shany-Ur, Suzee E Lee, Gil D Rabinovici, Howard J Rosen, Maria Luisa Gorno-Tempini, Adam L Boxer, Zachary A Miller, Winston Chiong, Mary DeMay, Joel H Kramer, Katherine L Possin, Virginia E Sturm, Brianne M Bettcher, Michael Neylan, Diana D Zackey, Lauren A Nguyen, Robin Ketelle, Nikolas Block, Teresa Q Wu, Alison Dallich, Natanya Russek, Alyssa Caplan, Daniel H Geschwind, Keith A Vossel, Bruce L Miller
IMPORTANCE: Clearer delineation of the phenotypic heterogeneity within behavioral variant frontotemporal dementia (bvFTD) will help uncover underlying biological mechanisms and improve clinicians' ability to predict disease course and to design targeted management strategies. OBJECTIVE: To identify subtypes of bvFTD syndrome based on distinctive patterns of atrophy defined by selective vulnerability of specific functional networks targeted in bvFTD using statistical classification approaches...
September 1, 2016: JAMA Neurology
Amarnath Jena, Sangeeta Taneja, Aashish Gambhir, Pushpendra N Renjen
No abstract text is available yet for this article.
July 2016: Neurology India
Josselin Baumard, Mathieu Lesourd, Christophe Jarry, Catherine Merck, Frédérique Etcharry-Bouyx, Valérie Chauviré, Serge Belliard, Olivier Moreaud, Bernard Croisile, François Osiurak, Didier Le Gall
In the field of apraxia, it has been suggested that the ability to use tools and objects in daily life depends not only on semantic knowledge about tool function and context of use but also on technical reasoning about mechanical properties of tools and objects. The aim of the present work was to assess tool use abilities regarding these hypotheses in patients with neurodegenerative diseases and reduced autonomy. Performance of patients with Alzheimer's disease (AD) (n = 31), semantic dementia (SD) (n = 16) and corticobasal syndrome (CBS) (n = 7) was compared to that of healthy control participants (n = 31) in familiar tool use tasks, functional/contextual associations and mechanical problem solving (MPS)...
September 2016: Cortex; a Journal Devoted to the Study of the Nervous System and Behavior
Jeremy J Tree, David Playfoot
The role of the semantic system in recognizing objects is a matter of debate. Connectionist theories argue that it is impossible for a participant to determine that an object is familiar to them without recourse to a semantic hub; localist theories state that accessing a stored representation of the visual features of the object is sufficient for recognition. We examine this issue through the longitudinal study of two cases of semantic dementia, a neurodegenerative disorder characterized by a progressive degradation of the semantic system...
October 2015: Cognitive Neuropsychology
Jennifer M Harris, Matthew Jones, Claire Gall, Anna M T Richardson, David Neary, Daniel du Plessis, Piyali Pal, David M A Mann, Julie S Snowden, Jennifer C Thompson
BACKGROUND/OBJECTIVES: We aimed to evaluate the co-occurrence of language and behavioural impairment in patients with frontotemporal lobar degeneration (FTLD) spectrum pathology. METHODS: Eighty-one dementia patients with pathological confirmation of FTLD were identified. Anonymized clinical records from patients' first assessment were rated for language and behavioural features from frontotemporal dementia consensus criteria, primary progressive aphasia (PPA) criteria and 1998 FTLD criteria...
May 2016: Dementia and Geriatric Cognitive Disorders Extra
Tim Van Langenhove, Cristian E Leyton, Olivier Piguet, John R Hodges
BACKGROUND: Differentiating between primary progressive aphasia (PPA) variants based on the profile of language deficits can be difficult in a proportion of patients. Further, little is presently know about the pattern of longitudinal changes in behavior in PPA variants. OBJECTIVE: To determine the presence of behavioral changes in the main variants of PPA: semantic (sv-PPA), nonfluent/agrammatic (nfv-PPA), and logopenic (lv-PPA), and establish the course of these changes over time...
June 18, 2016: Journal of Alzheimer's Disease: JAD
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