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Behavioral variant frontotemporal dementia

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https://www.readbyqxmd.com/read/28103593/executive-dysfunction-and-behavioral-symptoms-are-associated-with-deficits-in-instrumental-activities-of-daily-living-in-frontotemporal-dementia
#1
Negar Moheb, Mario F Mendez, Sarah A Kremen, Edmond Teng
BACKGROUND: Deficits in instrumental activities of daily living (ADLs) may be more prominent in behavioral variant frontotemporal dementia (bvFTD) than in nonfluent/agrammatic variant primary progressive aphasia (nfvPPA) or semantic variant primary progressive aphasia (svPPA). It is uncertain whether frontotemporal dementia (FTD) subgroups exhibit different patterns and/or predictors of functional impairment. METHODS: We examined data from participants diagnosed with bvFTD (n = 607), svPPA (n = 132), and nfvPPA (n = 155) who were included in the National Alzheimer's Coordinating Center (NACC) Uniform Data Set (UDS) and assessed with the Functional Activities Questionnaire (FAQ)...
January 20, 2017: Dementia and Geriatric Cognitive Disorders
https://www.readbyqxmd.com/read/28097206/-18-f-av-1451-pet-in-behavioral-variant-frontotemporal-dementia-due-to-mapt-mutation
#2
W Richard Bevan Jones, Thomas E Cope, Luca Passamonti, Tim D Fryer, Young T Hong, Franklin Aigbirhio, Jillian J Kril, Shelley L Forrest, Kieren Allinson, Jonathan P Coles, P Simon Jones, Maria G Spillantini, John R Hodges, John T O'Brien, James B Rowe
The validation of tau radioligands could improve the diagnosis of frontotemporal lobar degeneration and the assessment of disease-modifying therapies. Here, we demonstrate that binding of the tau radioligand [(18)F]AV-1451 was significantly abnormal in both magnitude and distribution in a patient with familial frontotemporal dementia due to a MAPT 10 + 16C>T gene mutation, recapitulating the pattern of neuropathology seen in her father. Given the genetic diagnosis and the non-Alzheimer's pathology, these findings suggest that [(18)F]AV-1451 might be a useful biomarker in primary tauopathies...
December 2016: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/28072380/network-functional-connectivity-and-whole-brain-functional-connectomics-to-investigate-cognitive-decline-in-neurodegenerative-conditions
#3
REVIEW
O Dipasquale, Mara Cercignani
Non-invasive mapping of brain functional connectivity (FC) has played a fundamental role in neuroscience, and numerous scientists have been fascinated by its ability to reveal the brain's intricate morphology and functional properties. In recent years, two different techniques have been developed that are able to explore FC in pathophysiological conditions and to provide simple and non-invasive biomarkers for the detection of disease onset, severity and progression. These techniques are independent component analysis, which allows a network-based functional exploration of the brain, and graph theory, which provides a quantitative characterization of the whole-brain FC...
October 2016: Functional Neurology
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
#4
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...
December 3, 2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28038340/spatio-temporal-and-kinematic-gait-analysis-in-patients-with-frontotemporal-dementia-and-alzheimer-s-disease-through-3d-motion-capture
#5
Rosaria Rucco, Valeria Agosti, Francesca Jacini, Pierpaolo Sorrentino, Pasquale Varriale, Manuela De Stefano, Graziella Milan, Patrizia Montella, Giuseppe Sorrentino
Alzheimer's disease (AD) and behavioral variant of Frontotemporal Dementia (bvFTD) are characterized respectively by atrophy in the medial temporal lobe with memory loss and prefrontal and anterior temporal degeneration with dysexecutive syndrome. In this study, we hypothesized that specific gait patterns are induced by either frontal or temporal degeneration. To test this hypothesis, we studied the gait pattern in bvFTD (23) and AD (22) patients in single and dual task ("motor" and "cognitive") conditions...
December 21, 2016: Gait & Posture
https://www.readbyqxmd.com/read/28017247/clinical-characteristics-of-parkinsonism-in-frontotemporal-dementia-according-to-subtypes
#6
Hee Kyung Park, Kee Hyung Park, Bora Yoon, Jae-Hong Lee, Seong Hye Choi, Jee H Joung, Soo Jin Yoon, Byeong C Kim, Seung Hyun Kim, Eun-Joo Kim, Duk L Na, Kyung Won Park
BACKGROUND: We investigated the prevalence of parkinsonism in frontotemporal dementia (FTD) subtypes and the cognitive and behavioral differences between FTD with and without parkinsonism in a well-structured, prospective cohort. METHODS: One hundred and ninety-one FTD patients were enrolled and all patients underwent comprehensive neurological evaluations, neuropsychological tests, and the Unified Parkinson's Disease Rating Scale. RESULTS: The prevalence of parkinsonism was 38...
January 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/27999880/-pathomechanisms-and-clinical-aspects-of-frontotemporal-lobar-degeneration
#7
REVIEW
K Bürger, T Arzberger, J Stephan, J Levin, D Edbauer
BACKGROUND: Frontotemporal lobar degeneration (FTLD) includes a spectrum of heterogeneous clinical and neuropathological diseases. In a strict sense this includes the behavioral variant of frontotemporal dementia (bvFTD) and primary progressive aphasia (PPA) and both variants can be associated with amyotrophic lateral sclerosis (FTD-ALS). In a broader sense FTLD also includes progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS). In recent years the strong genetic component of FTLD has become increasingly clear...
December 20, 2016: Der Nervenarzt
https://www.readbyqxmd.com/read/27976608/network-functional-connectivity-and-whole-brain-functional-connectomics-to-investigate-cognitive-decline-in-neurodegenerative-conditions
#8
O Dipasquale, Mara Cercignani
Non-invasive mapping of brain functional connectivity (FC) has played a fundamental role in neuroscience, and numerous scientists have been fascinated by its ability to reveal the brain's intricate morphology and functional properties. In recent years, two different techniques have been developed that are able to explore FC in pathophysiological conditions and to provide simple and non-invasive biomarkers for the detection of disease onset, severity and progression. These techniques are independent component analysis, which allows a network-based functional exploration of the brain, and graph theory, which provides a quantitative characterization of the whole-brain FC...
December 15, 2016: Functional Neurology
https://www.readbyqxmd.com/read/27940352/gray-matter-network-differences-between-behavioral-variant-frontotemporal-dementia-and-alzheimer-s-disease
#9
E G B Vijverberg, B M Tijms, J Dopp, Y J Hong, C E Teunissen, F Barkhof, P Scheltens, Y A L Pijnenburg
We set out to study whether single-subject gray matter (GM) networks show disturbances that are specific for Alzheimer's disease (AD; n = 90) or behavioral variant frontotemporal dementia (bvFTD; n = 59), and whether such disturbances would be related to cognitive deficits measured with mini-mental state examination and a neuropsychological battery, using subjective cognitive decline subjects as reference. AD and bvFTD patients had a lower degree, connectivity density, clustering, path length, betweenness centrality, and small world values compared with subjective cognitive decline...
February 2017: Neurobiology of Aging
https://www.readbyqxmd.com/read/27905268/mapt-mutation-associated-with-frontotemporal-dementia-and-parkinsonism-ftdp-17
#10
Robert Haussmann, Marek Wysocki, Moritz D Brandt, Andreas Hermann, Markus Donix
We present a 56-year-old patient suffering from frontotemporal dementia with parkinsonism linked to chromosome 17 (FTDP-17). The history included a three-generation pedigree and the patient was found to be a mutation carrier. The diagnosis was hindered by late appearance of the hypokinetic movement disorder. For clinicians, it is important to consider rare neurodegenerative disease variants in early-onset familial dementia syndromes with behavioral, cognitive, and motor symptoms.
December 1, 2016: International Psychogeriatrics
https://www.readbyqxmd.com/read/27859539/clinicopathologic-heterogeneity-in-ftdp-17-due-to-mapt-p-p301l-mutation-including-a-patient-with-globular-glial-tauopathy
#11
Pawel Tacik, Monica Sanchez-Contreras, Michael DeTure, Melissa E Murray, Rosa Rademakers, Owen A Ross, Zbigniew K Wszolek, Joseph E Parisi, David S Knopman, Ronald C Petersen, Dennis 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)...
November 8, 2016: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/27858712/self-consciousness-deficits-in-alzheimer-s-disease-and-frontotemporal-dementia
#12
Eva Ma Arroyo-Anlló, Adèle Turpin Bouston, Marie-Noëlle Fargeau, Begoña Orgaz Baz, Roger Gil
Self-consciousness (SC) is multifaceted and considered to be the consciousness of one's own mental states. The main aim of this paper is to compare SC in patients with Alzheimer's disease (AD) and behavioral variant frontotemporal dementia (bvFTD). Three groups (control and patient groups) of 23 subjects each were assessed using an SC questionnaire. Both types of dementia clearly induce an alteration of SC. The bvFTD group showed a greater impairment in SC than the AD and control groups. The SC score was strongly associated with frontal functions...
2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/27847684/longitudinal-change-in-everyday-function-and-behavioral-symptoms-in-frontotemporal-dementia
#13
Claire M O'Connor, Lindy Clemson, Michael Hornberger, Cristian E Leyton, John R Hodges, Olivier Piguet, Eneida Mioshi
BACKGROUND: The relationship between behavioral changes and functional decline in frontotemporal dementia (FTD) is not well understood. METHODS: Thirty-nine patients (21 behavioral variant FTD [bvFTD], 18 semantic variant primary progressive aphasia [svPPA]) were followed up longitudinally (2-4 years follow-up). Functional (Disability Assessment for Dementia) and behavioral (Cambridge Behavioural Inventory Revised) assessments were included for between-group (pairwise comparisons, mixed model analysis) and within-group analyses (bivariate correlations)...
October 2016: Neurology. Clinical Practice
https://www.readbyqxmd.com/read/27831627/-pathological-gambling-and-epilepsy-in-patients-with-frontotemporal-dementia-two-case-reports
#14
Sheila Castro-Suarez, Peggy Martinez, María Meza
Frontotemporal dementia is a neurodegenerative disorder of which the behavioral variant is most common. This condition is currently considered the most common cause of dementia in people younger than 60 years. Here, we present two unrelated cases in which the typical symptoms were cognitive and behavioral progressive deterioration and psychiatric disorders such as disinhibition, impulsive acts, apathy, lack of empathy, stereotypies, and changes in eating habits. The first case exhibited pathological gambling as the initial symptom and resided in a psychiatric facility for a year...
July 2016: Revista Peruana de Medicina Experimental y Salud Pública
https://www.readbyqxmd.com/read/27814992/a-cluster-of-progranulin-c157kfsx97-mutations-in-southern-italy-clinical-characterization-and-genetic-correlations
#15
Cinzia Coppola, Dario Saracino, Gianfranco Puoti, Giacomo Lus, Clemente Dato, Isabelle Le Ber, Jeremie Pariente, Paola Caroppo, Elena Piccoli, Fabrizio Tagliavini, Giuseppe Di Iorio, Giacomina Rossi
Frontotemporal lobar degeneration (FTLD) is a group of neurodegenerative diseases displaying high clinical, pathologic, and genetic heterogeneity. Several autosomal dominant progranulin (GRN) mutations have been reported, accounting for 5%-10% of FTLD cases worldwide. In this study, we described the clinical characteristics of 7 Italian patients, 5 with a diagnosis of frontotemporal dementia behavioral variant and 2 of corticobasal syndrome (CBS), carrying the GRN deletion g.101349_101355delCTGCTGT, resulting in the C157KfsX97 null mutation, and hypothesized the existence of a founder effect by means of haplotype sharing analysis...
January 2017: Neurobiology of Aging
https://www.readbyqxmd.com/read/27772665/-reconciliating-neurology-and-psychiatry-the-prototypical-case-of-frontotemporal-dementia
#16
J Lagarde, M Sarazin
Frontotemporal degeneration (FTD) in its behavioral variant (bvFTD) is probably one of the conditions that best illustrates the links between psychiatry and neurology. It is indeed admitted that between a third and half of patients with this condition, especially in early-onset forms, receive an initial diagnosis of psychiatric disorder (depression, schizophrenia, bipolar disorder) and are then referred to a psychiatric ward. BvFTD can thus be considered a neurological disorder with a psychiatric presentation...
October 20, 2016: L'Encéphale
https://www.readbyqxmd.com/read/27752510/diagnostic-utility-of-asl-mri-and-fdg-pet-in-the-behavioral-variant-of-ftd-and-ad
#17
Duygu Tosun, Norbert Schuff, Gil D Rabinovici, Nagehan Ayakta, Bruce L Miller, William Jagust, Joel Kramer, Michael M Weiner, Howard J Rosen
OBJECTIVE: To compare the values of arterial spin-labeled (ASL) MRI and fluorodeoxyglucose (FDG) PET in the diagnosis of behavioral variant of frontotemporal dementia (bvFTD) and Alzheimer's disease (AD). METHODS: Partial least squares logistic regression was used to identify voxels with diagnostic value in cerebral blood flow (CBF) and cerebral metabolic rate of glucose (CMRgl) maps from patients with bvFTD (n = 32) and AD (n = 28), who were compared with each other and with cognitively normal controls (CN, n = 15)...
October 2016: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/27751195/longitudinal-executive-function-and-episodic-memory-profiles-in-behavioral-variant-frontotemporal-dementia-and-alzheimer-s-disease
#18
Siddharth Ramanan, Maxime Bertoux, Emma Flanagan, Muireann Irish, Olivier Piguet, John R Hodges, Michael Hornberger
OBJECTIVES: With comparable baseline performance on executive functions (EF) and memory between Alzheimer's disease (AD) and behavioral-variant frontotemporal dementia (bvFTD), it is currently unclear if both diseases can be distinguished longitudinally on these measures reliably. METHODS: A total of 111 participants (33 AD, 31 bvFTD, and 47 controls) were followed-up annually over a 4-year period and tested on measures of EF, memory, and orientation. Linear mixed-effect models were constructed using disease severity as a nuisance variable to examine profiles of neuropsychological performance decline...
January 2017: Journal of the International Neuropsychological Society: JINS
https://www.readbyqxmd.com/read/27737532/an-autopsy-case-of-dementia-with-lewy-bodies-clinically-diagnosed-to-have-a-behavioral-variant-of-frontotemporal-dementia
#19
Hirotaka Sekiguchi, Masatsugu Moriwaki, Shuji Iritani, Chikako Habuchi, Youta Torii, Kentaro Umeda, Hiroshige Fujishiro, Mari Yoshida, Kiyoshi Fujita
We herein report the case of a 75-year-old male who had shown many psychiatric symptoms, but whose autopsy disclosed the presence of dementia with Lewy bodies (DLB). When he was 70 years old, the patient had presented with stereotyped behavior, dietary changes, and a decline in social interpersonal conduct in clinical settings, and it was thought that these symptoms were consistent with a behavioral variant of frontotemporal dementia (bvFTD), and he lacked the core features of DLB. Nevertheless, this case was pathologically defined as the limbic type of DLB after he died at the age of 75 years...
January 2017: Clinical Neuropathology
https://www.readbyqxmd.com/read/27714552/mapping-temporo-parietal-and-temporo-occipital-cortico-cortical-connections-of-the-human-middle-longitudinal-fascicle-in-subject-specific-probabilistic-and-stereotaxic-talairach-spaces
#20
Nikos Makris, A Zhu, G M Papadimitriou, P Mouradian, I Ng, E Scaccianoce, G Baselli, F Baglio, M E Shenton, Y Rathi, B Dickerson, E Yeterian, M Kubicki
Originally, the middle longitudinal fascicle (MdLF) was defined as a long association fiber tract connecting the superior temporal gyrus and temporal pole with the angular gyrus. More recently its description has been expanded to include all long postrolandic cortico-cortical association connections of the superior temporal gyrus and dorsal temporal pole with the parietal and occipital lobes. Despite its location and size, which makes MdLF one of the most prominent cerebral association fiber tracts, its discovery in humans is recent...
October 6, 2016: Brain Imaging and Behavior
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