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Behavioral variant frontotemporal dementia

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https://www.readbyqxmd.com/read/29769783/trichotillomania-ranging-from-ritual-to-illness-and-as-a-rare-clinical-manifestation-of-frontotemporal-dementia-review-of-literature-and-case-report
#1
Thomas Gregor Issac, Ashay Vivek Telang, Sadanandavalli Retnaswami Chandra
Frontotemporal dementia (FTD) is the most common form of dementia in the younger age group and often exists with comorbid obsessions and compulsions in up to 80% of the patients. Trichotillomania or compulsive "hair-pulling" disorder is a rare manifestation of FTD and is a poorly evaluated symptom in this condition. The release of "grooming functions" due to frontal disinhibition is often attributed to the evolutionary perspective; however, recent findings also implicate the role of neurotransmitter dysfunction...
March 2018: International Journal of Trichology
https://www.readbyqxmd.com/read/29760288/-neuropathologic-subtypes-of-frontotemporal-lobar-degeneration
#2
Mari Tada, Akiyoshi Kakita
Frontotemporal lobar degeneration (FTLD) is a heterogeneous disease entity encompassing a wide variety of histopathological features and genetic backgrounds. The last two decades have seen the discovery of causative genes and the identification of relevant proteins. The current histopathological classification is based on the major types of protein deposition in the brain, and most FTLD cases can be placed into one of three pathological subgroups: FTLD-tau, FTLD-TDP, and FTLD-FUS. Further sub-classification within each subgroup is based on the morphology of neuronal and glial inclusions and lesion distribution...
May 2018: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/29751289/perfusion-alterations-converge-with-patterns-of-pathological-spread-in-transactive-response-dna-binding-protein-43-proteinopathies
#3
Pilar M Ferraro, Charles Jester, Christopher A Olm, Katerina Placek, Federica Agosta, Lauren Elman, Leo McCluskey, David J Irwin, John A Detre, Massimo Filippi, Murray Grossman, Corey T McMillan
Amyotrophic lateral sclerosis (ALS) and the behavioral variant of frontotemporal dementia (bvFTD) commonly share the presence of transactive response DNA-binding protein 43 (TDP-43) inclusions. Structural magnetic resonance imaging studies demonstrated evidence for TDP-43 pathology spread, but while structural imaging usually reveals overt neuronal loss, perfusion imaging may detect more subtle neural activity alterations. We evaluated perfusion as an early marker for incipient pathology-associated brain alterations in TDP-43 proteinopathies...
April 17, 2018: Neurobiology of Aging
https://www.readbyqxmd.com/read/29748771/inhibition-impairment-in-frontotemporal-dementia-amyotrophic-lateral-sclerosis-and-alzheimer-s-disease-clinical-assessment-and-metabolic-correlates
#4
Jordi A Matías-Guiu, María Nieves Cabrera-Martín, María Valles-Salgado, Teresa Rognoni, Lucía Galán, Teresa Moreno-Ramos, José Luis Carreras, Jorge Matías-Guiu
The ability to reject an automatic tendency, i.e. inhibition, has been linked to the prefrontal cortex, but its neural underpinnings are still controversial. Neurodegenerative diseases represent an interesting model to explore this issue, given its frequent impairment in these disorders. We investigated the inhibitory impairment and its neural basis using four different tests, which evaluate the presence of inhibitory dysfunction (Stroop test, Hayling test, and two graphical perseveration tests), and assessed their correlation with brain metabolism using 18 F-fluorodeoxyglucose positron emission tomography in a group of 76 participants with behavioral variant frontotemporal dementia (bvFTD), Alzheimer's disease (AD), amyotrophic lateral sclerosis (ALS) and healthy controls (HC)...
May 10, 2018: Brain Imaging and Behavior
https://www.readbyqxmd.com/read/29748150/targeted-exome-sequencing-reveals-homozygous-trem2-r47c-mutation-presenting-with-behavioral-variant-frontotemporal-dementia-without-bone-involvement
#5
Adeline Sl Ng, Yi Jayne Tan, Zhao Yi, Moses Tandiono, Elaine Chew, Jacqueline Dominguez, Mabel Macas, Ebonne Ng, Shahul Hameed, Simon Ting, Eng King Tan, Jia Nee Foo, Nagaendran Kandiah
To identify genes associated with frontotemporal dementia (FTD) in South-East Asia, targeted exome sequencing and C9orf72 genotyping was performed in 198 subjects (52 patients with FTD and 146 healthy controls) who were screened for mutations in 12 FTD-associated genes. We detected a homozygous TREM2 R47C mutation in a patient with behavioral variant FTD without bone cysts or bone-associated phenotype. Two novel nonsense GRN mutations in 3 FTD patients from the Philippines were detected, but no known pathogenic mutations in other FTD-associated genes were found...
April 16, 2018: Neurobiology of Aging
https://www.readbyqxmd.com/read/29742972/validity-and-clinical-utility-of-different-clock-drawing-test-scoring-systems-in-multiple-forms-of-dementia
#6
Diana Duro, Miguel Tábuas-Pereira, Sandra Freitas, Beatriz Santiago, Maria Amália Botelho, Isabel Santana
The Clock Drawing Test (CDT) has a known potential for the detection of cognitive impairment in populations with dementia, especially Alzheimer disease (AD). Our aim was to compare the clinical utility of 3 CDT scoring systems (Rouleau, Cahn, and Babins) in several pathologies with cognitive compromise from a tertiary center memory clinic. We selected patients with a clinical diagnosis of mild stage AD, behavioral variant frontotemporal dementia (FTD), vascular dementia (VaD), dementia with Lewy bodies (DLB), and Parkinson disease with dementia (PDD)...
January 1, 2018: Journal of Geriatric Psychiatry and Neurology
https://www.readbyqxmd.com/read/29734264/the-accuracy-of-ineco-frontal-screening-in-the-diagnosis-of-executive-dysfunction-in-frontotemporal-dementia-and-alzheimer-disease
#7
Valéria S Bahia, Mário A Cecchini, Luciana Cassimiro, Rene Viana, Thais B Lima-Silva, Leonardo Cruz de Souza, Viviane Amaral Carvalho, Henrique C Guimarães, Paulo Caramelli, Márcio L F Balthazar, Benito Damasceno, Sônia M D Brucki, Ricardo Nitrini, Mônica S Yassuda
INTRODUCTION: Executive dysfunction is a common symptom in neurodegenerative disorders and is in need of easy-to-apply screening tools that might identify it. The aims of the present study were to examine some of the psychometric characteristics of the Brazilian version of the INECO frontal screening (IFS), and to investigate its accuracy to diagnose executive dysfunction in dementia and its accuracy to differentiate Alzheimer disease (AD) from the behavioral variant of frontotemporal dementia (bvFTD)...
May 4, 2018: Alzheimer Disease and Associated Disorders
https://www.readbyqxmd.com/read/29708208/apathy-and-functional-disability-in-behavioral-variant-frontotemporal-dementia
#8
Mônica S Yassuda, Thais B Lima da Silva, Claire M O'Connor, Shailaja Mekala, Suvarna Alladi, Valeria S Bahia, Viviane Almaral-Carvalho, Henrique C Guimaraes, Paulo Caramelli, Marcio L F Balthazar, Benito Damasceno, Sonia M D Brucki, Ricardo Nitrini, John R Hodges, Olivier Piguet, Eneida Mioshi
Background: Behavioral variant frontotemporal dementia (bvFTD) has profound consequences on patients and their families. In this multicenter study, we investigated the contribution of cognitive and neuropsychiatric factors to everyday function at different levels of overall functional impairment. Methods: In a retrospective cross-sectional study, 109 patients with bvFTD from 4 specialist frontotemporal dementia centers (Australia, England, India, and Brazil) were included...
April 2018: Neurology. Clinical Practice
https://www.readbyqxmd.com/read/29706989/caregiver-burden-in-semantic-dementia-with-right-and-left-sided-predominant-cerebral-atrophy-and-in-behavioral-variant-frontotemporal-dementia
#9
Asuka Koyama, Mamoru Hashimoto, Ryuji Fukuhara, Naoko Ichimi, Akihiro Takasaki, Masateru Matsushita, Tomohisa Ishikawa, Hibiki Tanaka, Yusuke Miyagawa, Manabu Ikeda
Background: Caregiver burden is a serious concern for family caregivers of dementia patients, but its nature is unclear in patients with semantic dementia (SD). This study aimed to clarify caregiver burden for right- (R > L) and left-sided (L > R) predominant SD versus behavioral-variant frontotemporal dementia (bvFTD) patients. Methods: Using the Japanese version of the Zarit Burden Interview (ZBI) and the Neuropsychiatric Inventory, we examined caregiver burden and behavioral and psychological symptoms of dementia (BPSD) in 43 first-visit outpatient/family caregiver dyads (bvFTD, 20 dyads; SD [L > R], 13 dyads; SD [R > L], 10 dyads)...
January 2018: Dementia and Geriatric Cognitive Disorders Extra
https://www.readbyqxmd.com/read/29704671/apathy-in-alzheimer-s-disease-and-frontotemporal-dementia-distinct-clinical-profiles-and-neural-correlates
#10
Fiona Kumfor, Alice Zhen, John R Hodges, Olivier Piguet, Muireann Irish
OBJECTIVE: Apathy is the most prevalent and disabling non-cognitive symptom of dementia and affects 90% of patients across the disease course. Despite its pervasiveness, how apathy manifests across dementia syndromes and the neurobiological mechanisms driving these symptoms are poorly understood. Here, we applied the multidimensional ABC model of apathy, which recognizes Affective, Behavioural and Cognitive apathy, in Alzheimer's disease (AD) and behavioural-variant frontotemporal dementia (bvFTD)...
March 29, 2018: Cortex; a Journal Devoted to the Study of the Nervous System and Behavior
https://www.readbyqxmd.com/read/29686739/screening-for-emotional-expression-in-frontotemporal-dementia-a-pilot-study
#11
Andrew R Carr, Mark M Ashla, Elvira E Jimenez, Mario F Mendez
Objective: Although emotional blunting is a core feature of behavioral variant frontotemporal dementia (bvFTD), there are no practical clinical measures of emotional expression for the early diagnosis of bvFTD. Method: Three age-matched groups (bvFTD, Alzheimer's disease (AD), and healthy controls (HC)) of eight participants each were presented with real-life vignettes varying in emotional intensity (high versus low) with either negative or positive outcomes. This study evaluated verbal (self-reports of distress) and visual (presence or absence of facial affect) measures of emotional expression during the vignettes...
2018: Behavioural Neurology
https://www.readbyqxmd.com/read/29682237/recognizing-late-onset-frontotemporal-dementia-with-the-daphne-scale-a-case-report
#12
Leonardo Tafarello Martins, Ivan Abdalla Teixeira, Jerson Laks, Valeska Marinho
Frontotemporal dementias are classically described as early onset dementias with personality and behavioral changes, however, late onset forms can also be found. Considering the paucity of information about late onset behavioral variant frontotemporal dementia and its challenging diagnosis, we present a case report of an 85-year-old woman with behavioral changes and slow progression to dementia who was first diagnosed as having bipolar disorder and then Alzheimer's disease. The Daphne scale provided a structured means to improve clinical diagnosis, also supported by characteristic features on MRI and SPECT, while CSF biomarkers ruled out atypical Alzheimer's disease...
January 2018: Dementia & Neuropsychologia
https://www.readbyqxmd.com/read/29662677/auto-antibodies-against-p-q-and-n-type-voltage-dependent-calcium-channels-mimicking-frontotemporal-dementia
#13
Kyan Younes, Lauren A Lepow, Cynthia Estrada, Paul E Schulz
The behavioral variant of frontotemporal dementia is usually a sporadic and progressive neurodegenerative disorder. Here, we report the subacute onset of a frontotemporal dementia phenotype with a treatable etiology. The patient has a history of rheumatoid arthritis, episcleritis, and thyroid eye disease on immunosuppressive therapy. He experienced a rapid personality change, including inappropriate behavior, which suggested frontotemporal dementia. Results of imaging and neuropsychological testing also suggested frontotemporal dementia...
2018: SAGE Open Medical Case Reports
https://www.readbyqxmd.com/read/29656245/individual-differences-in-socioemotional-sensitivity-are-an-index-of-salience-network-function
#14
Gianina Toller, Jesse Brown, Marc Sollberger, Suzanne M Shdo, Laura Bouvet, Paul Sukhanov, William W Seeley, Bruce L Miller, Katherine P Rankin
Connectivity in intrinsically connected networks (ICNs) may predict individual differences in cognition and behavior. The drastic alterations in socioemotional awareness of patients with behavioral variant frontotemporal dementia (bvFTD) are presumed to arise from changes in one such ICN, the salience network (SN). We examined how individual differences in SN connectivity are reflected in overt social behavior in healthy individuals and patients, both to provide neuroscientific insight into this key brain-behavior relationship, and to provide a practical tool to diagnose patients with early bvFTD...
February 27, 2018: Cortex; a Journal Devoted to the Study of the Nervous System and Behavior
https://www.readbyqxmd.com/read/29621927/skin-conduction-levels-differentiate-frontotemporal-dementia-from-alzheimer-s-disease
#15
Mario F Mendez, Sylvia S Fong, Mark M Ashla, Elvira E Jimenez, Andrew R Carr
Patients with behavioral variant frontotemporal dementia (bvFTD) and Alzheimer's disease (AD) differ in basic emotional tone. Skin conduction levels (SCLs), a measure of sympathetic tone, may be a sensitive test for discriminating these two dementias early in their course. Previous research has shown differences in resting SCLs between patients with bvFTD and AD, but no study has evaluated the discriminability of SCLs during different environmental conditions. The authors compared bvFTD patients (N=8), AD patients (N=10), and healthy control subjects (N=9) on SCL measures pertaining to real-life vignettes or scenarios differing in valence and emotional intensity...
April 6, 2018: Journal of Neuropsychiatry and Clinical Neurosciences
https://www.readbyqxmd.com/read/29614652/single-subject-classification-of-alzheimer-s-disease-and-behavioral-variant-frontotemporal-dementia-using-anatomical-diffusion-tensor-and-resting-state-functional-magnetic-resonance-imaging
#16
Mark J R J Bouts, Christiane Möller, Anne Hafkemeijer, John C van Swieten, Elise Dopper, Wiesje M van der Flier, Hugo Vrenken, Alle Meije Wink, Yolande A L Pijnenburg, Philip Scheltens, Frederik Barkhof, Tijn M Schouten, Frank de Vos, Rogier A Feis, Jeroen van der Grond, Mark de Rooij, Serge A R B Rombouts
BACKGROUND/OBJECTIVE: Overlapping clinical symptoms often complicate differential diagnosis between patients with Alzheimer's disease (AD) and behavioral variant frontotemporal dementia (bvFTD). Magnetic resonance imaging (MRI) reveals disease specific structural and functional differences that aid in differentiating AD from bvFTD patients. However, the benefit of combining structural and functional connectivity measures to-on a subject-basis-differentiate these dementia-types is not yet known...
January 24, 2018: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/29614645/structural-anatomical-investigation-of-long-term-memory-deficit-in-behavioral-frontotemporal-dementia
#17
Maxime Bertoux, Emma C Flanagan, Matthew Hobbs, Amparo Ruiz-Tagle, Carolina Delgado, Marcelo Miranda, Agustín Ibáñez, Andrea Slachevsky, Michael Hornberger
Although a growing body of work has shown that behavioral variant frontotemporal dementia (bvFTD) could present with severe amnesia in approximately half of cases, memory assessment is currently the clinical standard to distinguish bvFTD from Alzheimer's disease (AD). Thus, the concept of "relatively preserved episodic memory" in bvFTD remains the basis of its clinical distinction from AD and a criterion for bvFTD's diagnosis. This view is supported by the idea that bvFTD is not characterized by genuine amnesia and hippocampal degeneration, by contrast to AD...
March 24, 2018: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/29614640/diagnostic-value-of-diffusion-tensor-imaging-and-positron-emission-tomography-in-early-stages-of-frontotemporal-dementia
#18
Julia Krämer, Gero Lueg, Patrick Schiffler, Alexis Vrachimis, Matthias Weckesser, Christian Wenning, Matthias Pawlowski, Andreas Johnen, Anja Teuber, Heike Wersching, Sven G Meuth, Thomas Duning
BACKGROUND: Due to suboptimal sensitivity and specificity of structural and molecular neuroimaging tools, the diagnosis of behavioral variant frontotemporal dementia (bvFTD) remains challenging. OBJECTIVE: Investigation of the sensitivity of diffusion tensor imaging (DTI) and fluorodeoxyglucose positron emission tomography (FDG-PET) to detect cerebral alterations in early stages of bvFTD despite inconspicuous conventional MRI. METHODS: Thirty patients with early stages of bvFTD underwent a detailed neuropsychological examination, cerebral 3T MRI with DTI analysis, and FDG-PET...
March 28, 2018: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/29606954/automatic-mri-quantifying-methods-in-behavioral-variant-frontotemporal-dementia-diagnosis
#19
Antti Cajanus, Anette Hall, Juha Koikkalainen, Eino Solje, Antti Tolonen, Timo Urhemaa, Yawu Liu, Ramona M Haanpää, Päivi Hartikainen, Seppo Helisalmi, Ville Korhonen, Daniel Rueckert, Steen Hasselbalch, Gunhild Waldemar, Patrizia Mecocci, Ritva Vanninen, Mark van Gils, Hilkka Soininen, Jyrki Lötjönen, Anne M Remes
Aims: We assessed the value of automated MRI quantification methods in the differential diagnosis of behavioral-variant frontotemporal dementia (bvFTD) from Alzheimer disease (AD), Lewy body dementia (LBD), and subjective memory complaints (SMC). We also examined the role of the C9ORF72 -related genetic status in the differentiation sensitivity. Methods: The MRI scans of 50 patients with bvFTD (17 C9ORF72 expansion carriers) were analyzed using 6 quantification methods as follows: voxel-based morphometry (VBM), tensor-based morphometry, volumetry (VOL), manifold learning, grading, and white-matter hyperintensities...
January 2018: Dementia and Geriatric Cognitive Disorders Extra
https://www.readbyqxmd.com/read/29584904/combined-pathologies-in-ftld-tdp-types-a-and-c
#20
Tamar Gefen, Saman S Ahmadian, Qinwen Mao, Garam Kim, Mustafa Seckin, Borna Bonakdarpour, Eliana Marisa Ramos, Giovanni Coppola, Rosa Rademakers, Emily Rogalski, Alfred Rademaker, Sandra Weintraub, M-Marsel Mesulam, Changiz Geula, Eileen H Bigio
This study investigated the presence of combined pathologies in a large cohort of autopsies that show a primary pathologic diagnosis of phosphorylated 43-kDa TAR DNA-binding protein (FTLD-TDP), the majority of which portrayed clinical phenotypes consistent with primary progressive aphasia or behavioral variant frontotemporal dementia (bvFTD). Thirty-eight cases with FTLD-TDP (30 type-A and 8 type-C) were identified to determine characteristic differences between cases with and without combined pathologies. Findings indicated that combined pathologies co-occur with FTLD-TDP type-A at a high frequency (50%)-greater than when compared to FTLD-TDP type-C cases (12...
March 23, 2018: Journal of Neuropathology and Experimental Neurology
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