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Behavioral variant frontotemporal dementia

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https://www.readbyqxmd.com/read/27905268/mapt-mutation-associated-with-frontotemporal-dementia-and-parkinsonism-ftdp-17
#1
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
#2
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
#3
Eva Ma Arroyo-Anlló, Adèle Turpin Boustonb, 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...
November 14, 2016: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/27847684/longitudinal-change-in-everyday-function-and-behavioral-symptoms-in-frontotemporal-dementia
#4
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
#5
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
#6
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...
October 11, 2016: Neurobiology of Aging
https://www.readbyqxmd.com/read/27772665/-reconciliating-neurology-and-psychiatry-the-prototypical-case-of-frontotemporal-dementia
#7
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
#8
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
#9
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...
October 18, 2016: 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
#10
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...
October 14, 2016: 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
#11
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
https://www.readbyqxmd.com/read/27707194/psychotic-like-speech-in-frontotemporal-dementia
#12
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.
October 6, 2016: Journal of Neuropsychiatry and Clinical Neurosciences
https://www.readbyqxmd.com/read/27706428/kraepelin-s-description-of-chronic-mania-a-clinical-picture-that-meets-the-behavioral-variant-frontotemporal-dementia-phenotype
#13
Leandro Boson Gambogi, Henrique Cerqueira Guimarães, Maurício Viotti Daker, Leonardo Cruz de Souza, Paulo Caramelli
Chronic mania is an under-investigated condition and few reports have associated this disorder with an organic background. The present work examines Kraepelin's reliable description of chronic mania from a current behavioral neurology viewpoint. Kraepelin had described a cluster of symptoms that are now recognized as core manifestations of the behavioral variant frontotemporal dementia (bvFTD) clinical phenotype. We also carried out additional reviews of original manuscripts from Kraepelin's peers, in order to find any case reports that might fulfill the current diagnostic proposal for bvFTD...
September 2016: Arquivos de Neuro-psiquiatria
https://www.readbyqxmd.com/read/27698154/cognition-and-neuropsychiatry-in-behavioral-variant-frontotemporal-dementia-by-disease-stage
#14
Maxime Bertoux, Marie Sarazin, Florence Pasquier, Michel Bottlaender, Leonardo Cruz de Souza, Eneida Mioshi, Michael Hornberger, Kamalini G Ranasinghe, Katherine P Rankin, Iryna V Lobach, Joel H Kramer, Virginia E Sturm, Brianne M Bettcher, Katherine Possin, S Christine You, Amanda K Lamarre, Tal Shany-Ur, Melanie L Stephens, David C Perry, Suzee E Lee, Zachary A Miller, Maria L Gorno-Tempini, Howard J Rosen, Adam Boxer, William W Seeley, Gil D Rabinovici, Keith A Vossel, Bruce L Miller
No abstract text is available yet for this article.
October 4, 2016: Neurology
https://www.readbyqxmd.com/read/27662284/a-longitudinal-study-on-resting-state-functional-connectivity-in-behavioral-variant-frontotemporal-dementia-and-alzheimer-s-disease
#15
Anne Hafkemeijer, Christiane Möller, Elise G P Dopper, Lize C Jiskoot, Annette A van den Berg-Huysmans, John C van Swieten, Wiesje M van der Flier, Hugo Vrenken, Yolande A L Pijnenburg, Frederik Barkhof, Philip Scheltens, Jeroen van der Grond, Serge A R B Rombouts
BACKGROUND/OBJECTIVE: Alzheimer's disease (AD) and behavioral variant frontotemporal dementia (bvFTD) are the most common types of early-onset dementia. We applied longitudinal resting state functional magnetic resonance imaging (fMRI) to delineate functional brain connections relevant for disease progression and diagnostic accuracy. METHODS: We used two-center resting state fMRI data of 20 AD patients (65.1±8.0 years), 12 bvFTD patients (64.7±5.4 years), and 22 control subjects (63...
September 23, 2016: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/27660450/late-life-bipolar-disorder-evolving-into-frontotemporal-dementia-mimic
#16
Annemiek Dols, Welmoed Krudop, Christiane Möller, Kenneth Shulman, Martha Sajatovic, Yolande Al Pijnenburg
OBJECTIVES: Although bipolar disorder has been understood classically as a cyclic disease with full recovery between mood episodes, in the last decade, evidence has accumulated supporting progressive features. The clinical picture of advanced or end-stage bipolar disorder is heterogeneous with possible deficits in cognition and behavior, as illustrated by our case series. CASES: From our neuropsychiatric outpatient clinic, we describe four cases with bipolar disorder gradually developing a clinical syndrome, including apathy, disinhibition, loss of empathy, stereotypical behavior, and compulsiveness, fulfilling the criteria for possible behavioral variant frontotemporal dementia...
2016: Neuropsychiatric Disease and Treatment
https://www.readbyqxmd.com/read/27647788/person-based-versus-generalized-impulsivity-disinhibition-in-frontotemporal-dementia-and-alzheimer-disease
#17
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/27618645/dissociation-in-rating-negative-facial-emotions-between-behavioral-variant-frontotemporal-dementia-and-major-depressive-disorder
#18
Isabelle Chiu, Olivier Piguet, Janine Diehl-Schmid, Lina Riedl, Johannes Beck, Thomas Leyhe, Edith Holsboer-Trachsler, Manfred Berres, Andreas U Monsch, Marc Sollberger
OBJECTIVE: Features of behavioral variant frontotemporal dementia (bvFTD) such as executive dysfunction, apathy, and impaired empathic abilities are also observed in major depressive disorder (MDD). This may contribute to the reason why early stage bvFTD is often misdiagnosed as MDD. New assessment tools are thus needed to improve early diagnosis of bvFTD. Although emotion processing is affected in bvFTD and MDD, growing evidence indicates that the pattern of emotion processing deficits varies between the two disorders...
November 2016: American Journal of Geriatric Psychiatry
https://www.readbyqxmd.com/read/27581216/serum-neurofilament-light-chain-protein-is-a-measure-of-disease-intensity-in-frontotemporal-dementia
#19
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
https://www.readbyqxmd.com/read/27572272/rhapsody-internet-based-support-for-caregivers-of-people-with-young-onset-dementia-program-design-and-methods-of-a-pilot-study
#20
Alexander Kurz, Christian Bakker, Markus Böhm, Janine Diehl-Schmid, Bruno Dubois, Catarina Ferreira, Heather Gage, Caroline Graff, Thierry Hergueta, Sabine Jansen, Bridget Jones, Alexander Komar, Alexandre de Mendonça, Anna Metcalfe, Katrina Milecka, Joany Millenaar, Anneli Orrung Wallin, Jan Oyebode, Helga Schneider-Schelte, Susanna Saxl, Marjolein de Vugt
BACKGROUND: Young Onset Dementia (YOD), defined by first symptoms of cognitive or behavioral decline occurring before the age of 65 years, is relatively rare compared to dementia of later onset, but it is associated with diagnostic difficulty and heavy burden on affected individuals and their informal carers. Existing health and social care structures rarely meet the needs of YOD patients. Internet-based interventions are a novel format of delivering health-related education, counseling, and support to this vulnerable yet underserved group...
August 30, 2016: International Psychogeriatrics
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