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Right temporal frontotemporal dementia

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https://www.readbyqxmd.com/read/28646671/frontotemporal-lobar-degeneration-and-social-behaviour-dissociation-between-the-knowledge-of-its-consequences-and-its-conceptual-meaning
#1
Roland Zahn, Sophie Green, Helen Beaumont, Alistair Burns, Jorge Moll, Diana Caine, Alexander Gerhard, Paul Hoffman, Benjamin Shaw, Jordan Grafman, Matthew A Lambon Ralph
Inappropriate social behaviour is an early symptom of frontotemporal lobar degeneration (FTLD) in both behavioural variant frontotemporal dementia (bvFTD) and semantic dementia (SD) subtypes. Knowledge of social behaviour is essential for appropriate social conduct. The superior anterior temporal lobe (ATL) has been identified as one key neural component for the conceptual knowledge of social behaviour, but it is unknown whether this is dissociable from knowledge of the consequences of social behaviour. Here, we used a newly-developed test of knowledge about long-term and short-term consequences of social behaviour to investigate its impairment in patients with FTLD relative to a previously-developed test of social conceptual knowledge...
June 3, 2017: Cortex; a Journal Devoted to the Study of the Nervous System and Behavior
https://www.readbyqxmd.com/read/28632523/frontotemporal-dementia-presenting-as-gambling-disorder-when-a-psychiatric-condition-is-the-clue-to-a-neurodegenerative-disease
#2
Giacomo Tondo, Fabiola De Marchi, Emanuela Terazzi, Marta Sacchetti, Roberto Cantello
Behavioral variant frontotemporal dementia, the most common form of frontotemporal dementia, is characterized by executive dysfunction and changes in personality and behavior, sometimes with associated psychiatric disorders. We report a man who suddenly developed a gambling disorder when he was 55 years old. A year later he developed personality changes of agitation, euphoria, and disinhibition, along with binge eating and dysthymia. He did not improve on paroxetine 40 mg/day. Two years after the onset of his symptoms, he came to our clinic for evaluation...
June 2017: Cognitive and Behavioral Neurology: Official Journal of the Society for Behavioral and Cognitive Neurology
https://www.readbyqxmd.com/read/28616383/multiparametric-mri-to-distinguish-early-onset-alzheimer-s-disease-and-behavioural-variant-of-frontotemporal-dementia
#3
Elisa Canu, Federica Agosta, Gorana Mandic-Stojmenovic, Tanja Stojković, Elka Stefanova, Alberto Inuggi, Francesca Imperiale, Massimiliano Copetti, Vladimir S Kostic, Massimo Filippi
This prospective study explored whether an approach combining structural [cortical thickness and white matter (WM) microstructure] and resting state functional MRI can aid differentiation between 62 early onset Alzheimer's disease (EOAD) and 27 behavioural variant of frontotemporal dementia (bvFTD) patients. Random forest and receiver operator characteristic curve analyses assessed the ability of MRI in classifying the two clinical syndromes. All patients showed a distributed pattern of brain alterations relative to controls...
2017: NeuroImage: Clinical
https://www.readbyqxmd.com/read/28473626/patterns-of-atrophy-in-pathologically-confirmed-dementias-a-voxelwise-analysis
#4
Lorna Harper, Femke Bouwman, Emma J Burton, Frederik Barkhof, Philip Scheltens, John T O'Brien, Nick C Fox, Gerard R Ridgway, Jonathan M Schott
OBJECTIVE: Imaging is recommended to support the clinical diagnoses of dementias, yet imaging research studies rarely have pathological confirmation of disease. This study aims to characterise patterns of brain volume loss in six primary pathologies compared with controls and to each other. METHODS: One hundred and eighty-six patients with a clinical diagnosis of dementia and histopathological confirmation of underlying pathology, and 73 healthy controls were included in this study...
May 4, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28373956/a-neural-network-underlying-intentional-emotional-facial-expression-in-neurodegenerative-disease
#5
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/28304293/coordinate-based-meta-analysis-of-the-default-mode-and-salience-network-for-target-identification-in-non-invasive-brain-stimulation-of-alzheimer-s-disease-and-behavioral-variant-frontotemporal-dementia-networks
#6
Michela Pievani, Lorenzo Pini, Clarissa Ferrari, Francesca B Pizzini, Ilaria Boscolo Galazzo, Chiara Cobelli, Maria Cotelli, Rosa Manenti, Giovanni B Frisoni
BACKGROUND: The accurate choice of the site of non-invasive brain stimulation (NIBS) is an important factor in trial design. OBJECTIVE: Based on the observation that Alzheimer's disease (AD) and behavioral frontotemporal dementia (bvFTD) affect specific large-scale networks, i.e., the default mode network (DMN) and the salience network (SN), respectively, we aimed to identify population-average coordinates of these networks that could be used as potential targets in NIBS trials aiming to modulate these circuits...
2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28288787/evolution-of-autobiographical-memory-impairments-in-alzheimer-s-disease-and-frontotemporal-dementia-a-longitudinal-neuroimaging-study
#7
Muireann Irish, Ramon Landin-Romero, Annu Mothakunnel, Siddharth Ramanan, Sharpley Hsieh, John R Hodges, Olivier Piguet
Compromised autobiographical memory (ABM) retrieval is well established in dementia, attributable to degeneration of a core memory brain network. It remains unclear, however, how the progressive spread of atrophy with advancing disease severity impacts ABM retrieval across life epochs. To this end, we conducted a longitudinal study of recent and remote ABM in Alzheimer's disease (AD, n =11), and a frontotemporal lobar degeneration group (FTD, n =13) comprising 7 behavioral variant FTD and 6 semantic dementia patients, in comparison with 23 healthy older Controls...
March 10, 2017: Neuropsychologia
https://www.readbyqxmd.com/read/28235777/observing-conversational-laughter-in-frontotemporal-dementia
#8
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...
May 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28229125/the-clinical-neuroanatomical-and-neuropathologic-phenotype-of-tbk1-associated-frontotemporal-dementia-a-longitudinal-case-report
#9
Carolin A M Koriath, Martina Bocchetta, Emilie Brotherhood, Ione O C Woollacott, Penny Norsworthy, Javier Simón-Sánchez, Cornelis Blauwendraat, Katrina M Dick, Elizabeth Gordon, Sophie R Harding, Nick C Fox, Sebastian Crutch, Jason D Warren, Tamas Revesz, Tammaryn Lashley, Simon Mead, Jonathan D Rohrer
INTRODUCTION: Mutations in the TANK-binding kinase 1 (TBK1) gene have recently been shown to cause frontotemporal dementia (FTD). However, the phenotype of TBK1-associated FTD is currently unclear. METHODS: We performed a single case longitudinal study of a patient who was subsequently found to have a novel A705fs mutation in the TBK1 gene. He was assessed annually over a 7-year period with a series of clinical, cognitive, and magnetic resonance imaging assessments...
2017: Alzheimer's & Dementia: Diagnosis, Assessment & Disease Monitoring
https://www.readbyqxmd.com/read/28219620/brain-structural-correlates-of-executive-and-social-cognition-profiles-in-behavioral-variant-frontotemporal-dementia-and-elderly-bipolar-disorder
#10
Sandra Baez, Clara Pinasco, María Roca, Jesica Ferrari, Blas Couto, Indira García-Cordero, Agustín Ibañez, Francy Cruz, Pablo Reyes, Diana Matallana, Facundo Manes, Marcelo Cetcovich, Teresa Torralva
An early stage of behavioral variant frontotemporal dementia (bvFTD) often displays a mix of behavioral disturbances and personality changes hindering a differential diagnosis from elderly bipolar disorder (BD), making this process a big challenge. However, no studies have compared these pathologies from neuropsychological and neuroanatomical perspectives. The aim of the present study was to compare the executive functions (EF) and social cognition profiles as well as the structural neuroimaging of bvFTD and elderly patients with BD...
February 17, 2017: Neuropsychologia
https://www.readbyqxmd.com/read/28209520/deconstructing-empathy-neuroanatomical-dissociations-between-affect-sharing-and-prosocial-motivation-using-a-patient-lesion-model
#11
Suzanne M Shdo, Kamalini G Ranasinghe, Kelly A Gola, Clinton J Mielke, Paul V Sukhanov, Bruce L Miller, Katherine P Rankin
Affect sharing and prosocial motivation are integral parts of empathy that are conceptually and mechanistically distinct. We used a neurodegenerative disease (NDG) lesion model to examine the neural correlates of these two aspects of real-world empathic responding. The study enrolled 275 participants, including 44 healthy older controls and 231 patients diagnosed with one of five neurodegenerative diseases (75 Alzheimer's disease, 58 behavioral variant frontotemporal dementia (bvFTD), 42 semantic variant primary progressive aphasia (svPPA), 28 progressive supranuclear palsy, and 28 non-fluent variant (nfvPPA)...
February 14, 2017: Neuropsychologia
https://www.readbyqxmd.com/read/28100023/frontotemporal-lobar-degeneration-pathogenesis-pathology-and-pathways-to-phenotype
#12
REVIEW
David M A Mann, Julie S Snowden
Frontotemporal Lobar Degeneration (FTLD) is a clinically, pathologically and genetically heterogeneous group of disorders that affect principally the frontal and temporal lobes of the brain. There are three major associated clinical syndromes, behavioral variant frontotemporal dementia (bvFTD), semantic dementia (SD) and progressive non-fluent aphasia (PNFA); three principal histologies, involving tau, TDP-43 and FUS proteins; and mutations in three major genes, MAPT, GRN and C9orf72, along with several other less common gene mutations...
January 18, 2017: Brain Pathology
https://www.readbyqxmd.com/read/28059782/differential-hemispheric-predilection-of%C3%A2-microstructural-white-matter-and%C3%A2-functional-connectivity-abnormalities-between-respectively-semantic-and%C3%A2-behavioral-variant-frontotemporal-dementia
#13
Rozanna Meijboom, Rebecca M E Steketee, Leontine S Ham, Aad van der Lugt, John C van Swieten, Marion Smits
Semantic dementia (SD) and behavioral variant frontotemporal dementia (bvFTD), subtypes of frontotemporal dementia, are characterized by distinct clinical symptoms and neuroimaging features, with predominant left temporal grey matter (GM) atrophy in SD and bilateral or right frontal GM atrophy in bvFTD. Such differential hemispheric predilection may also be reflected by other neuroimaging features, such as brain connectivity. This study investigated white matter (WM) microstructure and functional connectivity differences between SD and bvFTD, focusing on the hemispheric predilection of these differences...
2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/27889911/multiregional-analysis-of-global-5-methylcytosine-and-5-hydroxymethylcytosine-throughout-the-progression-of-alzheimer-s-disease
#14
Elizabeth M Ellison, Erin L Abner, Mark A Lovell
Epigenetic modifications to cytosine are known to alter transcriptional states and deregulate gene expression in cancer, embryonic development, and most recently in neurodegeneration. To test the hypothesis that global levels of cytosine modification are altered throughout the progression of Alzheimer's disease, 5-methylcytosine (5-mC) and 5-hydroxymethylcytosine (5-hmC) were quantified using gas chromatography/mass spectrometry (GC/MS) and stable labeled internal standards of cytosine, 5-mC, and 5-hmC. Cytosine modifications were quantified in DNA extracted from tissue specimens of four brain regions (cerebellum, inferior parietal lobe, superior and middle temporal gyrus, and hippocampus/parahippocampal gyrus) of cognitively normal control (NC) subjects and subjects with mild cognitive impairment (MCI), preclinical Alzheimer's disease (PCAD), late onset Alzheimer's disease, frontotemporal lobar degeneration (FTLD) and dementia with Lewy bodies (DLB)...
February 2017: Journal of Neurochemistry
https://www.readbyqxmd.com/read/27859539/clinicopathologic-heterogeneity-in-frontotemporal-dementia-and-parkinsonism-linked-to-chromosome-17-ftdp-17-due-to-microtubule-associated-protein-tau-mapt-p-p301l-mutation-including-a-patient-with-globular-glial-tauopathy
#15
P Tacik, M Sanchez-Contreras, M DeTure, M E Murray, R Rademakers, O A Ross, Z K Wszolek, J E Parisi, D S Knopman, R C Petersen, D W Dickson
AIM: The p.P301L mutation in microtubule-associated protein tau (MAPT) is a common cause of frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17). We compare clinicopathologic features of five unrelated and three related (brother, sister and cousin) patients with FTDP-17 due to p.P301L mutation. METHODS: Genealogical, clinical, neuropathologic and genetic data were reviewed from eight individuals. RESULTS: The series consisted of five men and three women with an average age of death of 58 years (52-65 years) and average disease duration of 9 years (3-14 years)...
April 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/27723823/grey-and-white-matter-clinico-anatomical-correlates-of-disinhibition-in-neurodegenerative-disease
#16
Alexander Frizell Santillo, Karl Lundblad, Markus Nilsson, Maria Landqvist Waldö, Danielle van Westen, Jimmy Lätt, Erik Blennow Nordström, Susanna Vestberg, Olof Lindberg, Christer Nilsson
Disinhibition is an important symptom in neurodegenerative diseases. However, the clinico-anatomical underpinnings remain controversial. We explored the anatomical correlates of disinhibition in neurodegenerative disease using the perspective of grey and white matter imaging. Disinhibition was assessed with a neuropsychological test and a caregiver information-based clinical rating scale in 21 patients with prefrontal syndromes due to behavioural variant frontotemporal dementia (n = 12) or progressive supranuclear palsy (n = 9), and healthy controls (n = 25)...
2016: PloS One
https://www.readbyqxmd.com/read/27707194/psychotic-like-speech-in-frontotemporal-dementia
#17
Mario F Mendez, Andrew R Carr, Pongsatorn Paholpak
Behavioral variant frontotemporal dementia (bvFTD) may result in psychotic-like speech without other psychotic features. The authors identified a bvFTD subgroup with pressure of speech, tangentiality, derailment, clanging/rhyming, and punning associated with the right anterior temporal atrophy and sparing of the left frontal lobe.
2017: Journal of Neuropsychiatry and Clinical Neurosciences
https://www.readbyqxmd.com/read/27647788/person-based-versus-generalized-impulsivity-disinhibition-in-frontotemporal-dementia-and-alzheimer-disease
#18
Pongsatorn Paholpak, Andrew R Carr, Joseph P Barsuglia, Robin J Barrows, Elvira Jimenez, Grace J Lee, Mario F Mendez
BACKGROUND: While much disinhibition in dementia results from generalized impulsivity, in behavioral variant frontotemporal dementia (bvFTD) disinhibition may also result from impaired social cognition. OBJECTIVE: To deconstruct disinhibition and its neural correlates in bvFTD vs. early-onset Alzheimer's disease (eAD). METHODS: Caregivers of 16 bvFTD and 21 matched-eAD patients completed the Frontal Systems Behavior Scale disinhibition items...
September 19, 2016: Journal of Geriatric Psychiatry and Neurology
https://www.readbyqxmd.com/read/27639684/shared-neural-correlates-of-limb-apraxia-in-early-stages-of-alzheimer-s-dementia-and-behavioural-variant-frontotemporal-dementia
#19
Andreas Johnen, Lisa Brandstetter, Christian Kärgel, Heinz Wiendl, Hubertus Lohmann, Thomas Duning
Limb apraxia denotes a cognitive impairment of gesture production. Lesion studies in patients with stroke point towards distinct neural processing streams for limb imitation and object-pantomime within left parietal and temporal cortex, respectively. Despite its frequent occurrence as an early symptom in both, Alzheimer's dementia (AD) and behavioural variant frontotemporal dementia (bvFTD), neural correlates of limb apraxia within these patient groups remain unexplored. Using voxel-based morphometry and multiple regression models, associations between limb apraxia and gray matter (GM) volume were investigated in 36 dementia patients (18 AD, 18 bvFTD) in early disease stages...
August 26, 2016: Cortex; a Journal Devoted to the Study of the Nervous System and Behavior
https://www.readbyqxmd.com/read/27567867/first-symptoms-and-neurocognitive-correlates-of-behavioral-variant-frontotemporal-dementia
#20
Hernando Santamaría-García, Pablo Reyes, Adolfo García, Sandra Baéz, Angela Martinez, José Manuel Santacruz, Andrea Slachevsky, Mariano Sigman, Diana Matallana, Agustín Ibañez
BACKGROUND: Previous works highlight the neurocognitive differences between apathetic and disinhibited clinical presentations of the behavioral variant frontotemporal dementia (bvFTD). However, little is known regarding how the early presentation (i.e., first symptom) is associated to the neurocognitive correlates of the disease's clinical presentation at future stages of disease. OBJECTIVES: We analyzed the neurocognitive correlates of patients with bvFTD who debuted with apathy or disinhibition as first symptom of disease...
October 4, 2016: Journal of Alzheimer's Disease: JAD
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