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Behavioral variant frontotemporal dementia

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https://www.readbyqxmd.com/read/28453474/neuropsychological-profile-in-the-c9orf72-associated-behavioral-variant-frontotemporal-dementia
#1
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...
April 28, 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
#2
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
#3
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
#4
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
#5
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...
April 3, 2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28373956/a-neural-network-underlying-intentional-emotional-facial-expression-in-neurodegenerative-disease
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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/28304289/episodic-memory-dysfunction-in%C3%A2-behavioral-variant-frontotemporal-dementia-a-clinical-and-fdg-pet-study
#13
Marta Fernández-Matarrubia, Jordi A Matías-Guiu, María Nieves Cabrera-Martín, Teresa Moreno-Ramos, María Valles-Salgado, José Luis Carreras, Jorge Matías-Guiu
BACKGROUND: Episodic memory disturbance is still considered as an exclusion criterion for behavioral variant frontotemporal dementia (bvFTD), but growing evidence suggests that memory can be impaired. OBJECTIVE: Our main purposes were to assess episodic memory in a group of bvFTD patients comparatively with Alzheimer's disease (AD) patients, and analyze the relationship between episodic memory and brain metabolism measured using positron emission tomography imaging with 18F-fluorodeoxyglucose (FDG-PET)...
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
#14
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/28288787/evolution-of-autobiographical-memory-impairments-in-alzheimer-s-disease-and-frontotemporal-dementia-a-longitudinal-neuroimaging-study
#15
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/28268100/intrafamilial-variable-phenotype-including-corticobasal-syndrome-in-a-family-with-p-p301l-mutation-in-the-mapt-gene-first-report-in-south-america
#16
Emilia M Gatto, Ricardo F Allegri, Gustavo Da Prat, Patricio Chrem Mendez, David S Hanna, Michael O Dorschner, Ezequiel I Surace, Cyrus P Zabetian, Ignacio F Mata
Frontotemporal lobar degeneration is a neuropathological disorder that causes a variety of clinical syndromes including frontotemporal dementia (FTD), progressive supranuclear palsy, and corticobasal syndrome (CBS). FTD associated with parkinsonism occurs frequently as a result of mutations in the C9orf72 gene and also in the genes coding for the protein associated with microtubule tau (MAPT) and progranulin (GRN) on chromosome 17 (FTDP-17). Herein, we report an Argentinean family, of Basque ancestry, with an extensive family history of behavioral variant of FTD...
May 2017: Neurobiology of Aging
https://www.readbyqxmd.com/read/28265458/diagnosing-the-frontal-variant-of-alzheimer-s-disease-a-clinician-s-yellow-brick-road
#17
REVIEW
Russell P Sawyer, Federico Rodriguez-Porcel, Matthew Hagen, Rhonna Shatz, Alberto J Espay
BACKGROUND: Disruption of the frontal lobes and its associated networks are a common consequence of neurodegenerative disorders. Given the wide range of cognitive, behavioral and motor processes in which the frontal lobes are involved, there can be a great variety of manifestations depending on the pathology distribution. The most common are the behavioral variant of frontotemporal dementia (bvFTD) and the frontal variant of Alzheimer's disease (fvAD), which are particularly challenging to disentangle...
2017: Journal of Clinical Movement Disorders
https://www.readbyqxmd.com/read/28264768/clinical-and-genetic-analyses-of-familial-and-sporadic-frontotemporal-dementia-patients-in-southern-italy
#18
Rosa Capozzo, Celeste Sassi, Monia B Hammer, Simona Arcuti, Chiara Zecca, Maria R Barulli, Rosanna Tortelli, J Raphael Gibbs, Cynthia Crews, Davide Seripa, Francesco Carnicella, Claudia Dell'Aquila, Marco Rossi, Filippo Tamma, Francesco Valluzzi, Bruno Brancasi, Francesco Panza, Andrew B Singleton, Giancarlo Logroscino
INTRODUCTION: We investigated the clinical differences between familial and sporadic frontotemporal dementia (FTD), screening for mutations in known FTD genes. METHODS: We diagnosed 22 affected individuals belonging to eight families and 43 sporadic cases with FTD in Apulia, Southern Italy, in 2 years. Mutations in common causative FTD genes (GRN, MAPT, VCP, and TARDBP) and C9ORF72 expansions were screened. RESULTS: Behavioral variant of FTD was the most common clinical subtype (50% and 69% in familial and sporadic cases, respectively)...
March 3, 2017: Alzheimer's & Dementia: the Journal of the Alzheimer's Association
https://www.readbyqxmd.com/read/28243755/differential-diagnosis-between-patients-with-probable-alzheimer-s-disease-parkinson-s-disease-dementia-or-dementia-with-lewy-bodies-and-frontotemporal-dementia-behavioral-variant-using-quantitative-electroencephalographic-features
#19
Heinrich Garn, Carmina Coronel, Markus Waser, Georg Caravias, Gerhard Ransmayr
The objective of this work was to develop and evaluate a classifier for differentiating probable Alzheimer's disease (AD) from Parkinson's disease dementia (PDD) or dementia with Lewy bodies (DLB) and from frontotemporal dementia, behavioral variant (bvFTD) based on quantitative electroencephalography (QEEG). We compared 25 QEEG features in 61 dementia patients (20 patients with probable AD, 20 patients with PDD or probable DLB (DLBPD), and 21 patients with bvFTD). Support vector machine classifiers were trained to distinguish among the three groups...
February 27, 2017: Journal of Neural Transmission
https://www.readbyqxmd.com/read/28240379/different-apathy-clinical-profile-and-neural-correlates-in-behavioral-variant-frontotemporal-dementia-and-alzheimer-s-disease
#20
Marta Fernández-Matarrubia, Jordi A Matías-Guiu, María Nieves Cabrera-Martín, Teresa Moreno-Ramos, María Valles-Salgado, José Luis Carreras, Jorge Matías-Guiu
OBJECTIVES: Apathy is one of the most common and disabling syndromes of dementia. Clinical apathy expression and neuroanatomical basis of apathy seem to differ between behavioral variant frontotemporal dementia (bvFTD) and Alzheimer's disease (AD), although evidence is scarce and poorly understood. Our main purposes were to compare the clinical apathy profile from patients with bvFTD and AD and analyze the relationship between apathy and brain metabolism measured using positron emission tomography imaging with (18) F fluorodeoxyglucose (FDG-PET)...
February 27, 2017: International Journal of Geriatric Psychiatry
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