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Behavioral variant frontotemporal dementia

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https://www.readbyqxmd.com/read/28541543/validation-of-the%C3%A2-d%C3%A3-pistage-cognitif-de-qu%C3%A3-bec-a-new-cognitive-screening-tool-for-atypical-dementias
#1
Robert Laforce, Leila Sellami, David Bergeron, Audrey Paradis, Louis Verret, Marie-Pierre Fortin, Michèle Houde, Martin Roy, Stéphane Poulin, Joël Macoir, Carol Hudon, Rémi W Bouchard
Objective: This study aimed to validate and provide normative data for the Dépistage Cognitif de Québec (DCQ; www.dcqtest.org), a new cognitive screening tool for atypical dementias. Method: The DCQ was developed by expert behavioral neurologists and clinical neuropsychologists based on updated criteria for Alzheimer's disease, primary progressive aphasia, and behavioral variant frontotemporal dementia. It targets five relevant domains: Memory, Visuospatial, Executive, Language, and Behavior...
May 25, 2017: Archives of Clinical Neuropsychology: the Official Journal of the National Academy of Neuropsychologists
https://www.readbyqxmd.com/read/28500752/which-ante-mortem-clinical-features-predict-progressive-supranuclear-palsy-pathology
#2
Gesine Respondek, Carolin Kurz, Thomas Arzberger, Yaroslau Compta, Elisabet Englund, Leslie W Ferguson, Ellen Gelpi, Armin Giese, David J Irwin, Wassilios G Meissner, Christer Nilsson, Alexander Pantelyat, Alex Rajput, John C van Swieten, Claire Troakes, Keith A Josephs, Anthony E Lang, Brit Mollenhauer, Ulrich Müller, Jennifer L Whitwell, Angelo Antonini, Kailash P Bhatia, Yvette Bordelon, Jean-Christophe Corvol, Carlo Colosimo, Richard Dodel, Murray Grossman, Jan Kassubek, Florian Krismer, Johannes Levin, Stefan Lorenzl, Huw Morris, Peter Nestor, Wolfgang H Oertel, Gil D Rabinovici, James B Rowe, Thilo van Eimeren, Gregor K Wenning, Adam Boxer, Lawrence I Golbe, Irene Litvan, Maria Stamelou, Günter U Höglinger
BACKGROUND: Progressive supranuclear palsy (PSP) is a neuropathologically defined disease presenting with a broad spectrum of clinical phenotypes. OBJECTIVE: To identify clinical features and investigations that predict or exclude PSP pathology during life, aiming at an optimization of the clinical diagnostic criteria for PSP. METHODS: We performed a systematic review of the literature published since 1996 to identify clinical features and investigations that may predict or exclude PSP pathology...
May 13, 2017: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/28487499/updated-meta-analysis-of-the-role-of-apoe-%C3%AE%C2%B52-%C3%AE%C2%B53-%C3%AE%C2%B54-alleles-in-frontotemporal-lobar-degeneration
#3
Wen-Hua Su, Zhi-Hong Shi, Shu-Ling Liu, Xiao-Dan Wang, Shuai Liu, Yong Ji
We performed an updated meta-analysis to assess the role of the ε2/ε3/ε4 alleles of Apolipoprotein E gene (APOE) in frontotemporal lobar degeneration (FTLD). The relevant articles were retrieved from PubMed, CENTRAL, EMBASE and Web of Science databases, and 51 eligible case-control studies with 5123 cases and 20566 controls were selected after screening according to inclusion and exclusion criteria. Our analysis demonstrated that APOE ε4 was associated with increased FTLD risk in all genetic models (ε4 vs...
April 21, 2017: Oncotarget
https://www.readbyqxmd.com/read/28482638/von-economo-neuron-density-and-thalamus-volumes-in-behavioral-deficits-in-frontotemporal-dementia-cases-with-and-without-a-c9orf72-repeat-expansion
#4
Yue Yang, Glenda M Halliday, John R Hodges, Rachel H Tan
BACKGROUND: The early and selective loss of von Economo neurons in the anterior cingulate cortex has been linked to behavioral deficits in frontotemporal dementia (FTD). Importantly, whether these neurons are also targeted in patients with the C9ORF72 repeat expansion has yet to be established. This is of particular interest given the recent evidence highlighting the thalamus rather than anterior cingulate cortex as a region of significant degeneration in patients with the C9ORF72 repeat expansion...
May 5, 2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28474365/tackling-variability-a-multicenter-study-to-provide-a-gold-standard-network-approach-for-frontotemporal-dementia
#5
Lucas Sedeño, Olivier Piguet, Sofía Abrevaya, Horacio Desmaras, Indira García-Cordero, Sandra Baez, Laura Alethia de la Fuente, Pablo Reyes, Sicong Tu, Sebastian Moguilner, Nicolas Lori, Ramon Landin-Romero, Diana Matallana, Andrea Slachevsky, Teresa Torralva, Dante Chialvo, Fiona Kumfor, Adolfo M García, Facundo Manes, John R Hodges, Agustin Ibanez
Biomarkers represent a critical research area in neurodegeneration disease as they can contribute to studying potential disease-modifying agents, fostering timely therapeutic interventions, and alleviating associated financial costs. Functional connectivity (FC) analysis represents a promising approach to identify early biomarkers in specific diseases. Yet, virtually no study has tested whether potential FC biomarkers prove to be reliable and reproducible across different centers. As such, their implementation remains uncertain due to multiple sources of variability across studies: the numerous international centers capable conducting FC research vary in their scanning equipment and their samples' socio-cultural background, and, more troublingly still, no gold-standard method exists to analyze FC...
May 5, 2017: Human Brain Mapping
https://www.readbyqxmd.com/read/28464702/pavlov-s-orienting-response-in-frontotemporal-dementia
#6
Aditi Joshi, Elvira Jimenez, Mario F Mendez
Behavior variant frontotemporal dementia (bvFTD) is a neurodegenerative disorder with initial disturbances in socioemotional behavior in the absence of a sensitive diagnostic test. This study evaluated Pavlov's "orienting response" (OR) or "what is it?" reflex as a measure of their ability to refocus attention on socioemotional stimuli and as a potentially distinguishing measure for bvFTD. Ten patients with bvFTD were compared with 18 normal controls (NC) on ORs (defined as initial heart rate [HR] deceleration) to different pictures based on social and emotional (valence) differences from the International Affective Picture Stimuli...
May 3, 2017: Journal of Neuropsychiatry and Clinical Neurosciences
https://www.readbyqxmd.com/read/28458550/psychosis-in-behavioral-variant-frontotemporal-dementia
#7
Flora T Gossink, Everard Gb Vijverberg, Welmoed Krudop, Philip Scheltens, Max L Stek, Yolande Al Pijnenburg, Annemiek Dols
BACKGROUND: Dementia is generally characterized by cognitive impairment that can be accompanied by psychotic symptoms; for example, visual hallucinations are a core feature of dementia with Lewy bodies, and delusions are often seen in Alzheimer's disease. However, for behavioral variant of frontotemporal dementia (bvFTD), studies on the broad spectrum of psychotic symptoms are still lacking. The aim of this study was to systematically and prospectively subtype the wide spectrum of psychotic symptoms in probable and definite bvFTD...
2017: Neuropsychiatric Disease and Treatment
https://www.readbyqxmd.com/read/28458182/the-attribution-of-animacy-and-agency-in-frontotemporal-dementia-versus-alzheimer-s-disease
#8
Sylvia S Fong, Pongsatorn Paholpak, Madelaine Daianu, Mariel B Deutsch, Brandalyn C Riedel, Andrew R Carr, Elvira E Jimenez, Michelle M Mather, Paul M Thompson, Mario F Mendez
Impaired attribution of animacy (state of living or being sentient) and of agency (capability of intrinsically-driven action) may underlie social behavior disturbances in behavioral variant frontotemporal dementia (bvFTD). We presented the Heider and Simmel film of moving geometric shapes to 11 bvFTD patients, 11 Alzheimer's disease (AD) patients, and 12 healthy controls (HCs) and rated their recorded verbal responses for animacy attribution and agency attribution. All participants had skin conductance (SC) continuously recorded while viewing the film, and all dementia participants underwent magnetic resonance imaging (MRI) for regions of interest...
April 8, 2017: Cortex; a Journal Devoted to the Study of the Nervous System and Behavior
https://www.readbyqxmd.com/read/28453474/neuropsychological-profile-in-the-c9orf72-associated-behavioral-variant-frontotemporal-dementia
#9
Noora-Maria Suhonen, Ramona M Haanpää, Ville Korhonen, Jari Jokelainen, Anni Pitkäniemi, Anna-Leena Heikkinen, Johanna Krüger, Päivi Hartikainen, Seppo Helisalmi, Mikko Hiltunen, Tuomo Hänninen, Anne M Remes
While the C9ORF72 expansion is a major cause of behavioral variant frontotemporal dementia (bvFTD), little is known of the resultant cognitive profile. Our aim was to characterize the neuropsychological profile of the C9ORF72 associated bvFTD. We contrasted structured neuropsychological assessments of the C9ORF72 expansion carrier bvFTD patients (n = 26) with non-carrier bvFTD patients (n = 47) and those with Alzheimer's disease (AD) (n = 47). As compared to the non-carrier bvFTD patients, the C9ORF72 expansion carriers performed at a higher level in an immediate verbal memory test while showing poorer phonemic verbal fluency...
2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28413716/data-driven-regions-of-interest-for-longitudinal-change-in-three-variants-of-frontotemporal-lobar-degeneration
#10
COMPARATIVE STUDY
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/28410663/frontotemporal-dementia
#11
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/28395276/lower-visual-avoidance-in-dementia-patients-is-associated-with-greater-psychological-distress-in-caregivers
#12
Marcela C Otero, Robert W Levenson
Caring for a spouse with dementia can lead to increased health problems in caregivers. The present study examined whether patient deficits in visual avoidance, a common form of emotion regulation, are related to greater psychological distress in caregivers. Participants were 43 Alzheimer disease (AD) patients, 43 behavioral variant frontotemporal dementia (bvFTD) patients, and their spousal caregivers. Patient visual avoidance (e.g., gaze aversion) was measured using behavioral coding of head, body, and eye position while viewing a disgusting film...
April 11, 2017: Dementia and Geriatric Cognitive Disorders
https://www.readbyqxmd.com/read/28387671/predicting-development-of-amyotrophic-lateral-sclerosis-in-frontotemporal-dementia
#13
Tim Van Langenhove, Olivier Piguet, James R Burrell, Cristian Leyton, David Foxe, Melissa Abela, Lauren Bartley, Woojin S Kim, Eve Jary, Yue Huang, Carol Dobson-Stone, John B Kwok, Glenda M Halliday, John R Hodges
BACKGROUND: A proportion of patients with frontotemporal dementia (FTD) also develop amyotrophic lateral sclerosis (ALS). OBJECTIVE: We aimed to establish the risk of developing ALS in patients presenting with FTD and to identify the relevant clinical variables associated with progression from FTD to FTD-ALS. METHODS: Of 218 consecutive patients with FTD, 10.1% had a dual FTD-ALS diagnosis at presentation. The remaining 152 FTD patients with follow-up of at least 12 months were included in the present study...
2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28373956/a-neural-network-underlying-intentional-emotional-facial-expression-in-neurodegenerative-disease
#14
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/28348957/predicting-behavioral-variant-frontotemporal-dementia-with-pattern-classification-in-multi-center-structural-mri-data
#15
Sebastian Meyer, Karsten Mueller, Katharina Stuke, Sandrine Bisenius, Janine Diehl-Schmid, Frank Jessen, Jan Kassubek, Johannes Kornhuber, Albert C Ludolph, Johannes Prudlo, Anja Schneider, Katharina Schuemberg, Igor Yakushev, Markus Otto, Matthias L Schroeter
PURPOSE: Frontotemporal lobar degeneration (FTLD) is a common cause of early onset dementia. Behavioral variant frontotemporal dementia (bvFTD), its most common subtype, is characterized by deep alterations in behavior and personality. In 2011, new diagnostic criteria were suggested that incorporate imaging criteria into diagnostic algorithms. The study aimed at validating the potential of imaging criteria to individually predict diagnosis with machine learning algorithms. MATERIALS & METHODS: Brain atrophy was measured with structural magnetic resonance imaging (MRI) at 3 Tesla in a multi-centric cohort of 52 bvFTD patients and 52 healthy control subjects from the German FTLD Consortium's Study...
2017: NeuroImage: Clinical
https://www.readbyqxmd.com/read/28337476/cerebrospinal-fluid-biomarker-examination-as-a-tool-to-discriminate-behavioral-variant-frontotemporal-dementia-from-primary-psychiatric%C3%A2-disorders
#16
Everard G B Vijverberg, Annemiek Dols, Welmoed A Krudop, Marta Del Campo Milan, Cora J Kerssens, Flora Gossink, Niels D Prins, Max L Stek, Philip Scheltens, Charlotte E Teunissen, Yolande A L Pijnenburg
INTRODUCTION: To prospectively determine the diagnostic value of cerebrospinal fluid (CSF) levels total-tau (tau) to amyloid-β1-42 ratio (Aβ1-42) ratio (tau/Aβ1-42 ratio), phosphorylated-tau (p-tau) to tau ratio (p-tau/tau ratio), neurofilament light chain (NfL) and YKL40 in the late-onset frontal lobe syndrome, in particular for the differential diagnosis of behavioral variant frontotemporal dementia (bvFTD) versus primary psychiatric disorders (PSY). METHOD: We included patients with a multidisciplinary 2-year-follow-up diagnosis of probable/definite bvFTD (n = 22) or PSY (n = 25), who underwent a detailed neuropsychiatric clinical examination, neuropsychological test battery, and magnetic resonance imaging at baseline...
2017: Alzheimer's & Dementia: Diagnosis, Assessment & Disease Monitoring
https://www.readbyqxmd.com/read/28337409/network-degeneration-and-dysfunction-in-presymptomatic-c9orf72-expansion-carriers
#17
Suzee E Lee, Ana C Sias, Maria Luisa Mandelli, Jesse A Brown, Alainna B Brown, Anna M Khazenzon, Anna A Vidovszky, Theodore P Zanto, Anna M Karydas, Mochtar Pribadi, Deepika Dokuru, Giovanni Coppola, Dan H Geschwind, Rosa Rademakers, Maria Luisa Gorno-Tempini, Howard J Rosen, Bruce L Miller, William W Seeley
Hexanucleotide repeat expansions in C9ORF72 are the most common known genetic cause of familial and sporadic frontotemporal dementia and amyotrophic lateral sclerosis. Previous work has shown that patients with behavioral variant frontotemporal dementia due to C9ORF72 show salience and sensorimotor network disruptions comparable to those seen in sporadic behavioral variant frontotemporal dementia, but it remains unknown how early in the lifespan these and other changes in brain structure and function arise...
2017: NeuroImage: Clinical
https://www.readbyqxmd.com/read/28322905/bilingualism-delays-the-onset-of-behavioral-but-not-aphasic-forms-of-frontotemporal-dementia
#18
Suvarna Alladi, Thomas H Bak, Mekala Shailaja, Divyaraj Gollahalli, Amulya Rajan, Bapiraju Surampudi, Michael Hornberger, Vasanta Duggirala, Jaydip Ray Chaudhuri, Subhash Kaul
Bilingualism has been found to delay onset of dementia and this has been attributed to an advantage in executive control in bilinguals. However, the relationship between bilingualism and cognition is complex, with costs as well as benefits to language functions. To further explore the cognitive consequences of bilingualism, the study used Frontotemporal dementia (FTD) syndromes, to examine whether bilingualism modifies the age at onset of behavioral and language variants of Frontotemporal dementia (FTD) differently...
March 18, 2017: Neuropsychologia
https://www.readbyqxmd.com/read/28304308/violation-of-laws-in-frontotemporal-dementia-a-multicenter-study-in-japan
#19
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)...
2017: Journal of Alzheimer's Disease: JAD
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
#20
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
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