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Behavioral variant frontotemporal dementia

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https://www.readbyqxmd.com/read/29131982/multimodal-mri-quantification-of-the-common-neurostructural-bases-within-the-ftd-als-continuum
#1
Chiara Crespi, Alessandra Dodich, Stefano F Cappa, Nicola Canessa, Sandro Iannaccone, Massimo Corbo, Christian Lunetta, Andrea Falini, Chiara Cerami
The continuum hypothesis linking the behavioral variant of frontotemporal dementia (bvFTD) and amyotrophic lateral sclerosis (ALS) is supported by clinical, pathological, genetic, and neuroimaging evidence. In the present multimodal magnetic resonance study, we characterized the site and extent of shared neurostructural changes in gray and white matter in 20 bvFTD and 19 ALS patients without dementia. We found an overlap of macrostructural and microstructural damage in both patient groups compared with healthy controls, involving the right orbital and the bilateral anterior cingulate cortices, the corticospinal tract and corpus callosum...
September 28, 2017: Neurobiology of Aging
https://www.readbyqxmd.com/read/29107846/relevance-of-raised-cerebrospinal-fluid-monocyte-levels-in-patients-with-frontotemporal-dementia
#2
Matthias Pawlowski, Gero Lueg, Catharina C Gross, Andreas Johnen, Julia Krämer, Matthias Weckesser, Heinz Wiendl, Sven G Meuth, Thomas Duning
Frontotemporal dementia (FTD) is a heterogeneous neurodegenerative disorder. The contribution of the immune system to its pathogenesis remains incompletely understood. In this study, we performed comprehensive immune cell profiling in the cerebrospinal fluid (CSF) and peripheral blood of patients with FTD. Thirty-two patients with behavioral variant frontotemporal dementia and 25 patients with primary progressive aphasia were included and compared to 14 healthy elderly controls. All patients underwent neuropsychological examination, magnetic resonance imaging, voxel-based diffusion tensor imaging, and peripheral blood and CSF immune cell profiling by multiparameter flow cytometry...
October 13, 2017: Neurobiology of Aging
https://www.readbyqxmd.com/read/29099546/the-diagnostic-challenge-of-the-late-onset-frontal-lobe-syndrome-clinical-predictors-for-primary-psychiatric-disorders-versus-behavioral-variant-frontotemporal-dementia
#3
Everard G B Vijverberg, Flora Gossink, Welmoed Krudop, Sietske Sikkes, Cora Kerssens, Niels Prins, Max Stek, Philip Scheltens, Yolande Pijnenburg, Annemiek Dols
OBJECTIVE: Primary psychiatric disorders (PsD) can present with symptomatology identical to that of behavioral variant frontotemporal dementia (bvFTD). To date, clinical guidelines do not provide a solution for this diagnostic challenge. The aim of our study was to prospectively determine which demographic, clinical, neuropsychological, neuroimaging, and cerebrospinal fluid biomarkers are important in distinguishing PsD from bvFTD. METHODS: Patients with late-onset behavioral disturbances (aged 45-75 years, 73% male) were included based on their scores on the Frontal Behavioral Inventory and the Stereotypy Rating Inventory and followed for 2 years from April 2011 to June 2015...
October 31, 2017: Journal of Clinical Psychiatry
https://www.readbyqxmd.com/read/29089874/mistakes-too-few-to-mention-impaired-self-conscious-emotional-processing-of-errors-in-the-behavioral-variant-of-frontotemporal-dementia
#4
Carole S Scherling, Jessica Zakrzewski, Samir Datta, Robert W Levenson, Arthur P Shimamura, Virginia E Sturm, Bruce L Miller, Howard J Rosen
Anosognosia, or lack of awareness of one's deficits, is a core feature of the behavioral variant of frontotemporal dementia (bvFTD). We hypothesized that this deficit has its origins in failed emotional processing of errors. We studied autonomic and facial emotional reactivity to errors in patients with bvFTD (n = 17), Alzheimer's disease (AD, n = 20), and healthy controls (HC, n = 35) during performance of a timed two-alternative-choice button press task. Performance-related behavioral responses to errors were quantified using rates of error correction and post-error slowing of reaction times...
2017: Frontiers in Behavioral Neuroscience
https://www.readbyqxmd.com/read/29080331/optn-p-met468arg-and-atxn2-intermediate-length-polyq-extension-in-families-with-c9orf72-mediated-amyotrophic-lateral-sclerosis-and-frontotemporal-dementia
#5
Sali M K Farhan, Tania F Gendron, Leonard Petrucelli, Robert A Hegele, Michael J Strong
We have ascertained two families affected with familial amyotrophic lateral sclerosis (ALS) in which they both carry a hexanucleotide repeat expansion in the C9orf72 gene, specifically in individuals who also presented with frontotemporal dementia (FTD) or behavioral variant FTD (bvFTD). While some reports attribute this phenotypic heterogeneity to the C9orf72 expansion alone, we screened for additional genetic variation in known ALS-FTD genes that may also contribute to or modify the phenotypes. We performed genetic testing consisting of C9orf72 hexanucleotide expansion, ATXN2 polyglutamine (polyQ) expansion, and targeted next generation sequencing using the ONDRISeq, a gene panel consisting of 80 genes known to be associated with neurodegenerative diseases such as ALS, FTD, Alzheimer's disease, Parkinson's disease, and vascular cognitive impairment...
October 28, 2017: American Journal of Medical Genetics. Part B, Neuropsychiatric Genetics
https://www.readbyqxmd.com/read/29075567/prosocial-deficits-in-behavioral-variant-frontotemporal-dementia-relate-to-reward-network-atrophy
#6
Virginia E Sturm, David C Perry, Kristie Wood, Alice Y Hua, Oscar Alcantar, Samir Datta, Katherine P Rankin, Howard J Rosen, Bruce L Miller, Joel H Kramer
INTRODUCTION: Empathy and shared feelings of reward motivate individuals to share resources with others when material gain is not at stake. Behavioral variant frontotemporal dementia (bvFTD) is a neurodegenerative disease that affects emotion- and reward-relevant neural systems. Although there is diminished empathy and altered reward processing in bvFTD, how the disease impacts prosocial behavior is less well understood. METHODS: A total of 74 participants (20 bvFTD, 15 Alzheimer's disease [AD], and 39 healthy controls) participated in this study...
October 2017: Brain and Behavior
https://www.readbyqxmd.com/read/29057638/erratum-to-prognosis-of-patients-with-behavioral-variant-frontotemporal-dementia-who-have-focal-versus-diffuse-frontal-atrophy
#7
Jin San Lee, Na Yeon Jung, Young Kyoung Jang, Hee Jin Kim, Sang Won Seo, Juyoun Lee, Yeo Jin Kim, Jae Hong Lee, Byeong C Kim, Kyung Won Park, Soo Jin Yoon, Jee H Jeong, Sang Yun Kim, Seung Hyun Kim, Eun Joo Kim, Key Chung Park, David S Knopman, Duk L Na
This corrects the article on p. 234 in vol. 13, PMID: 28748674.
October 2017: Journal of Clinical Neurology
https://www.readbyqxmd.com/read/29051562/motifs-in-the-tau-protein-that-control-binding-to-microtubules-and-aggregation-determine-pathological-effects
#8
Aurélien Lathuilière, Pamela Valdés, Stéphanie Papin, Matthias Cacquevel, Catherine Maclachlan, Graham W Knott, Andreas Muhs, Paolo Paganetti, Bernard L Schneider
Tau pathology is associated with cognitive decline in Alzheimer's disease, and missense tau mutations cause frontotemporal dementia. Hyperphosphorylation and misfolding of tau are considered critical steps leading to tauopathies. Here, we determine how motifs controlling conformational changes in the microtubule-binding domain determine tau pathology in vivo. Human tau was overexpressed in the adult mouse forebrain to compare variants carrying residues that modulate tau propensity to acquire a β-sheet conformation...
October 19, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28991661/behavioral-and-affective-features-of-amyotrophic-lateral-sclerosis-patients
#9
Yasuyuki Ohta, Kota Sato, Mami Takemoto, Yoshiaki Takahashi, Ryuta Morihara, Yumiko Nakano, Keiichiro Tsunoda, Emi Nomura, Nozomi Hishikawa, Toru Yamashita, Koji Abe
Evaluating the cognitive and behavioral features in amyotrophic lateral sclerosis (ALS) patients is important for therapy and care. Fifty-seven ALS, 5 ALS with the behavioral variant of frontotemporal dementia (FTD) (ALS-FTD), 12 FTD patients, and 35 control subjects were evaluated by 10 different tests for cognitive and behavioral (mini-mental state examination (MMSE), Hasegawa dementia rating scale - revised (HDS-R), frontal assessment battery (FAB), Montreal cognitive assessment (MoCA), ALS-frontotemporal dementia-Questionnaire (ALS-FTD-Q), and anosognosia scale), affective (depression, apathy, and behavioral and psychological symptoms of dementia (BPSD)), and activities of daily living (ADL) assessments...
October 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28984605/the-pitfall-of-behavioral-variant-frontotemporal-dementia-mimics-despite%C3%A2-multidisciplinary-application-of%C3%A2-the%C3%A2-ftdc-criteria
#10
Welmoed A Krudop, Annemieke Dols, Cora J Kerssens, Piet Eikelenboom, Niels D Prins, Christiane Möller, Sigfried Schouws, Didi Rhebergen, Eric van Exel, Wiesje M van der Flier, Sietske Sikkes, Philip Scheltens, Max L Stek, Yolande A L Pijnenburg
BACKGROUND: The behavioral variant of frontotemporal dementia (bvFTD) has a broad differential diagnosis including other neurological and psychiatric disorders. Psychiatric misdiagnoses occur in up to 50% of bvFTD patients. Numbers on misdiagnosis of bvFTD in psychiatric disorders are lacking. OBJECTIVE: The aim of our study was to investigate the frequency and characteristics of bvFTD misdiagnoses in psychiatric disorders and other neurologic disorders. METHODS: Thirty-five patients with a (possible or probable) bvFTD diagnosis made by specialized memory clinic neurologists were included...
2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28975068/longitudinal-white-matter-change-in-frontotemporal-dementia-subtypes-and-sporadic-late-onset-alzheimer-s-disease
#11
Fanny M Elahi, Gabe Marx, Yann Cobigo, Adam M Staffaroni, John Kornak, Duygu Tosun, Adam L Boxer, Joel H Kramer, Bruce L Miller, Howard J Rosen
BACKGROUND: Degradation of white matter microstructure has been demonstrated in frontotemporal lobar degeneration (FTLD) and Alzheimer's disease (AD). In preparation for clinical trials, ongoing studies are investigating the utility of longitudinal brain imaging for quantification of disease progression. To date only one study has examined sample size calculations based on longitudinal changes in white matter integrity in FTLD. OBJECTIVE: To quantify longitudinal changes in white matter microstructural integrity in the three canonical subtypes of frontotemporal dementia (FTD) and AD using diffusion tensor imaging (DTI)...
2017: NeuroImage: Clinical
https://www.readbyqxmd.com/read/28954876/brain-network-connectivity-differs-in-early-onset-neurodegenerative-dementia
#12
Massimo Filippi, Silvia Basaia, Elisa Canu, Francesca Imperiale, Alessandro Meani, Francesca Caso, Giuseppe Magnani, Monica Falautano, Giancarlo Comi, Andrea Falini, Federica Agosta
OBJECTIVE: To investigate functional brain network architecture in early-onset Alzheimer disease (EOAD) and behavioral variant frontotemporal dementia (bvFTD). METHODS: Thirty-eight patients with bvFTD, 37 patients with EOAD, and 32 age-matched healthy controls underwent 3D T1-weighted and resting-state fMRI. Graph analysis and connectomics assessed global and local functional topologic network properties, regional functional connectivity, and intrahemispheric and interhemispheric between-lobe connectivity...
October 24, 2017: Neurology
https://www.readbyqxmd.com/read/28906375/correlations-between-clinical-characteristics-and-neuroimaging-in-chinese-patients-with-subtypes-of-frontotemporal-lobe-degeneration
#13
Zhihong Shi, Shuai Liu, Ying Wang, Shuling Liu, Tong Han, Li Cai, Yuying Zhou, Shuo Gao, Yong Ji
The aim of the study was to obtain an overview of the clinical and neuroimaging features of Chinese patients with subtypes of frontotemporal lobe degeneration (FTLD).We evaluated the demographic features, clinical presentation, and lobe atrophy depicted by magnetic resonance imaging (MRI) in 133 patients with FTLD. Two positron emission tomography (PET) scans were performed at baseline: [C]Pittsburgh compound B PET to assess amyloid-β plaque load and [F]fluorodeoxyglucose (FDG) PET to assess glucose metabolism...
September 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28894929/recalling-feature-bindings-differentiates-alzheimer-s-disease-from-frontotemporal-dementia
#14
Mario Amore Cecchini, Mônica Sanches Yassuda, Valéria Santoro Bahia, Leonardo Cruz de Souza, Henrique Cerqueira Guimarães, Paulo Caramelli, Maria Teresa Carthery-Goulart, Flávia Patrocínio, Maria Paula Foss, Vitor Tumas, Thaís Bento Lima-Silva, Sônia Maria Dozzi Brucki, Ricardo Nitrini, Sergio Della Sala, Mario A Parra
It has been challenging to identify clinical cognitive markers that can differentiate patients with Alzheimer's disease (AD) from those with behavioral variant frontotemporal dementia (bvFTD). The short-term memory binding (STMB) test assesses the ability to integrate colors and shapes into unified representations and to hold them temporarily during online performance. The objective of this study is to investigate whether free recall deficits during short-term memory binding (STMB) test can differentiate patients with AD from those with bvFTD and controls...
October 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28838910/assessment-of-iron-deposition-in-the-brain-in-frontotemporal-dementia-and-its-correlation-with-behavioral-traits
#15
R Sheelakumari, C Kesavadas, T Varghese, R M Sreedharan, B Thomas, J Verghese, P S Mathuranath
BACKGROUND AND PURPOSE: Brain iron deposition has been implicated as a major culprit in the pathophysiology of neurodegeneration. However, the quantitative assessment of iron in behavioral variant frontotemporal dementia and primary progressive aphasia brains has not been performed, to our knowledge. The aim of our study was to investigate the characteristic iron levels in the frontotemporal dementia subtypes using susceptibility-weighted imaging and report its association with behavioral profiles...
October 2017: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/28803444/atypical-parkinsonian-syndromes-a-general-neurologist-s-perspective
#16
REVIEW
Angela B Deutschländer, Owen A Ross, Dennis W Dickson, Zbigniew K Wszolek
The differential diagnosis of atypical parkinsonian syndromes is challenging. These severe and often rapidly progressive neurodegenerative disorders are clinically heterogeneous and show significant phenotypic overlap. Here we review clinical, imaging, neuropathologic and genetic features of multiple system atrophy (MSA), progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), and frontotemporal lobar degeneration (FTLD). The terms CBD and FTLD refer to pathologically confirmed cases of corticobasal syndrome (CBS) and frontotemporal dementia (FTD)...
August 12, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28795386/dementia-caregiver-burden-a-research-update-and-critical-analysis
#17
REVIEW
Sheung-Tak Cheng
PURPOSE OF REVIEW: This article provides an updated review of the determinants of caregiver burden and depression, with a focus on care demands and especially the differential effects of various neuropsychiatric symptoms or symptom clusters. Moreover, studies on caregivers for frontotemporal and Lewy body dementias were referred to in order to identify differences and similarities with the mainstream literature based largely on Alzheimer caregivers. RECENT FINDINGS: As a group, neuropsychiatric symptoms are most predictive of caregiver burden and depression regardless of dementia diagnosis, but the effects appear to be driven primarily by disruptive behaviors (e...
August 10, 2017: Current Psychiatry Reports
https://www.readbyqxmd.com/read/28749089/cognitive-deficits-in-patients-with-neuropsychiatric-symptoms-a-comparative-study-between-behavioral-variant-frontotemporal-dementia-and-primary-psychiatric-disorders
#18
Everard G B Vijverberg, Sigfried Schouws, Paul David Meesters, Esmée Verwijk, Hannie Comijs, Ted Koene, Charlotte Schreuder, Aartjan Beekman, Philip Scheltens, Max Stek, Yolande Pijnenburg, Annemieke Dols
OBJECTIVE: To compare neuropsychological profiles in behavioral variant frontotemporal dementia (bvFTD) with its most common primary psychiatric differential diagnoses, major depressive disorder (MDD), bipolar disorder (BD), and schizophrenia, in older patients with active symptoms. METHODS: We included patients from different cohorts with MDD (DSM-IV-TR: 296.20-296.23, 296.30-296.33; n = 42; mean ± SD age, 72.0 ± 8.0 years; female = 57.1%) included from 2002 to 2007, noneuthymic BD (DSM-IV-TR: 296...
July 25, 2017: Journal of Clinical Psychiatry
https://www.readbyqxmd.com/read/28748674/prognosis-of-patients-with-behavioral-variant-frontotemporal-dementia-who-have-focal-versus-diffuse-frontal-atrophy
#19
Jin San Lee, Na Yeon Jung, Young Kyoung Jang, Hee Jin Kim, Sang Won Seo, Juyoun Lee, Yeo Jin Kim, Jae Hong Lee, Byeong C Kim, Kyung Won Park, Soo Jin Yoon, Jee H Jeong, Sang Yun Kim, Seung Hyun Kim, Eun Joo Kim, Key Chung Park, David S Knopman, Duk L Na
BACKGROUND AND PURPOSE: Only a few studies have investigated the relationship between different subtypes and disease progression or prognosis in patients with behavioral variant frontotemporal dementia (bvFTD). Since a localized injury often produces more focal signs than a diffuse injury, we hypothesized that the clinical characteristics differ between patients with bvFTD who show diffuse frontal lobe atrophy (D-type) on axial magnetic resonance imaging (MRI) scans versus those with focal or circumscribed frontal lobe atrophy (F-type)...
July 2017: Journal of Clinical Neurology
https://www.readbyqxmd.com/read/28733335/motor-speech-signature-of-behavioral-variant-frontotemporal-dementia-refining-the-phenotype
#20
Adam P Vogel, Matthew L Poole, Hugh Pemberton, Marja W J Caverlé, Frederique M C Boonstra, Essie Low, David Darby, Amy Brodtmann
OBJECTIVE: To provide a comprehensive description of motor speech function in behavioral variant frontotemporal dementia (bvFTD). METHODS: Forty-eight individuals (24 bvFTD and 24 age- and sex-matched healthy controls) provided speech samples. These varied in complexity and thus cognitive demand. Their language was assessed using the Progressive Aphasia Language Scale and verbal fluency tasks. Speech was analyzed perceptually to describe the nature of deficits and acoustically to quantify differences between patients with bvFTD and healthy controls...
August 22, 2017: Neurology
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