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Behavioral variant frontotemporal dementia

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
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
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
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
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
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
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
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
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
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
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
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
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
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
Sadanandavalli Retnaswami Chandra, Thomas Gregor Issac, Suresh Kumar Korada, Karru Venkata Ravi Teja, Mariamma Philip
INTRODUCTION: About 20-50% of relatively young onset dementia belongs to frontotemporal type. Most of these patients are diagnosed as psychiatric illness as their memory and instrumental activities of daily living remain unaltered till late and most of these patients do not qualify for dementia by the Diagnostic and Statistical Manual of Mental Disorders-IV criteria. In this study, we analyzed the behavioral symptoms in our patients with radiologically and neuropsychologically proven as probable behavioral variant of frontotemporal dementia (FTD)...
July 2016: Indian Journal of Psychological Medicine
Maxime Bertoux, Siddharth Ramanan, Andrea Slachevsky, Stephanie Wong, Fernando Henriquez, Gada Musa, Carolina Delgado, Emma Flanagan, Michel Bottlaender, Marie Sarazin, Michael Hornberger, Bruno Dubois
BACKGROUND: Memory impairment in behavioral variant frontotemporal dementia (bvFTD) is traditionally considered to be mild and attributed to prefrontal cortex dysfunction. Recent studies, however, indicated that some patients can present with a memory impairment of the hippocampal type, showing storage and consolidation deficits in addition to the more executive/prefrontal related encoding and strategic difficulties. OBJECTIVE: This study aimed to study the relationship between executive functions (EF) and memory processes in bvFTD via a data-driven approach...
October 4, 2016: Journal of Alzheimer's Disease: JAD
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
Pawel Tacik, Michael A DeTure, Yari Carlomagno, Wen-Lang Lin, Melissa E Murray, Matthew C Baker, Keith A Josephs, Bradley F Boeve, Zbigniew K Wszolek, Neill R Graff-Radford, Joseph E Parisi, Leonard Petrucelli, Rosa Rademakers, Richard S Isaacson, Kenneth M Heilman, Ronald C Petersen, Dennis W Dickson, Naomi Kouri
Mutations in microtubule-associated protein tau gene (MAPT) cause frontotemporal dementia with parkinsonism linked to chromosome 17 (FTDP-17). Here, we describe a patient with FTDP-17 and a novel missense mutation in exon 13 of MAPT, p.E372G. We compare clinicopathologic features of this patient to two previously unreported patients with another exon 13 mutation, p.G389R. The patient with the p.E372G mutation was a 40-year-old man with behavioral variant frontotemporal dementia (bvFTD), who subsequently developed agrammatic speech and parkinsonism...
October 5, 2016: Brain Pathology
Jan Jastorff, Francois-Laurent De Winter, Jan Van den Stock, Rik Vandenberghe, Martin A Giese, Mathieu Vandenbulcke
Several brain regions are involved in the processing of emotional stimuli, however, the contribution of specific regions to emotion perception is still under debate. To investigate this issue, we combined behavioral testing, structural and resting state imaging in patients diagnosed with behavioral variant frontotemporal dementia (bvFTD) and age matched controls, with task-based functional imaging in young, healthy volunteers. As expected, bvFTD patients were impaired in emotion detection as well as emotion categorization tasks, testing dynamic emotional body expressions as stimuli...
August 11, 2016: Human Brain Mapping
Anat Perry, Sandy J Lwi, Alice Verstaen, Callum Dewar, Robert W Levenson, Robert T Knight
Interpersonal distance is central to communication and complex social behaviors but the neural correlates of interpersonal distance preferences are not defined. Previous studies suggest that damage to the orbitofrontal cortex (OFC) is associated with impaired interpersonal behavior. To examine whether the OFC is critical for maintaining appropriate interpersonal distance, we tested two groups of patients with OFC damage: Patients with OFC lesions and patients with behavioral variant frontotemporal dementia...
August 10, 2016: Social Cognitive and Affective Neuroscience
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