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Right temporal frontotemporal dementia

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https://www.readbyqxmd.com/read/27889911/multiregional-analysis-of-global-5-methylcytosine-and-5-hydroxymethylcytosine-throughout-the-progression-of-alzheimer-s-disease
#1
Elizabeth M Ellison, Erin L Abner, Mark A Lovell
Epigenetic modifications to cytosine are known to alter transcriptional states and deregulate gene expression in cancer, embryonic development, and most recently in neurodegeneration. To test the hypothesis that global levels of cytosine modification are altered throughout the progression of Alzheimer's disease, 5-methylcytosine (5-mC) and 5-hydroxmethylcytosine (5-hmC) were quantified using gas chromatography/mass spectrometry (GC/MS) and stable labeled internal standards of cytosine, 5-mC, and 5-hmC. Cytosine modifications were quantified in DNA extracted from tissue specimens of four brain regions (cerebellum, inferior parietal lobe, superior and middle temporal gyrus, and hippocampus/parahippocampal gyrus) of cognitively normal control (NC) subjects and subjects with mild cognitive impairment (MCI), preclinical Alzheimer's disease (PCAD), late onset Alzheimer's disease (LOAD), frontotemporal lobar degeneration (FTLD) and dementia with Lewy bodies (DLB)...
November 27, 2016: Journal of Neurochemistry
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/27723823/grey-and-white-matter-clinico-anatomical-correlates-of-disinhibition-in-neurodegenerative-disease
#3
Alexander Frizell Santillo, Karl Lundblad, Markus Nilsson, Maria Landqvist Waldö, Danielle van Westen, Jimmy Lätt, Erik Blennow Nordström, Susanna Vestberg, Olof Lindberg, Christer Nilsson
Disinhibition is an important symptom in neurodegenerative diseases. However, the clinico-anatomical underpinnings remain controversial. We explored the anatomical correlates of disinhibition in neurodegenerative disease using the perspective of grey and white matter imaging. Disinhibition was assessed with a neuropsychological test and a caregiver information-based clinical rating scale in 21 patients with prefrontal syndromes due to behavioural variant frontotemporal dementia (n = 12) or progressive supranuclear palsy (n = 9), and healthy controls (n = 25)...
2016: PloS One
https://www.readbyqxmd.com/read/27707194/psychotic-like-speech-in-frontotemporal-dementia
#4
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/27647788/person-based-versus-generalized-impulsivity-disinhibition-in-frontotemporal-dementia-and-alzheimer-disease
#5
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/27639684/shared-neural-correlates-of-limb-apraxia-in-early-stages-of-alzheimer-s-dementia-and-behavioural-variant-frontotemporal-dementia
#6
Andreas Johnen, Lisa Brandstetter, Christian Kärgel, Heinz Wiendl, Hubertus Lohmann, Thomas Duning
Limb apraxia denotes a cognitive impairment of gesture production. Lesion studies in patients with stroke point towards distinct neural processing streams for limb imitation and object-pantomime within left parietal and temporal cortex, respectively. Despite its frequent occurrence as an early symptom in both, Alzheimer's dementia (AD) and behavioural variant frontotemporal dementia (bvFTD), neural correlates of limb apraxia within these patient groups remain unexplored. Using voxel-based morphometry and multiple regression models, associations between limb apraxia and gray matter (GM) volume were investigated in 36 dementia patients (18 AD, 18 bvFTD) in early disease stages...
August 26, 2016: Cortex; a Journal Devoted to the Study of the Nervous System and Behavior
https://www.readbyqxmd.com/read/27567867/first-symptoms-and-neurocognitive-correlates-of-behavioral-variant-frontotemporal-dementia
#7
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
https://www.readbyqxmd.com/read/27497488/healthy-brain-connectivity-predicts-atrophy-progression-in-non-fluent-variant-of-primary-progressive-aphasia
#8
Maria Luisa Mandelli, Eduard Vilaplana, Jesse A Brown, H Isabel Hubbard, Richard J Binney, Suneth Attygalle, Miguel A Santos-Santos, Zachary A Miller, Mikhail Pakvasa, Maya L Henry, Howard J Rosen, Roland G Henry, Gil D Rabinovici, Bruce L Miller, William W Seeley, Maria Luisa Gorno-Tempini
Neurodegeneration has been hypothesized to follow predetermined large-scale networks through the trans-synaptic spread of toxic proteins from a syndrome-specific epicentre. To date, no longitudinal neuroimaging study has tested this hypothesis in vivo in frontotemporal dementia spectrum disorders. The aim of this study was to demonstrate that longitudinal progression of atrophy in non-fluent/agrammatic variant primary progressive aphasia spreads over time from a syndrome-specific epicentre to additional regions, based on their connectivity to the epicentre in healthy control subjects...
August 6, 2016: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/27489094/prosopagnosia-as-unusual-presentation-of-semantic-dementia-a-case-study
#9
Viviana Lo Buono, Francesco Corallo, Angela Marra, Rocco Salvatore Calabrò, Placido Bramanti, Silvia Marino
OBJECT: Semantic dementia is one of the main clinical variants of frontotemporal dementia and it is characterized by severe loss of conceptual knowledge with relative preservation of other cortical abilities. METHODS: We described a case of a patient affected by right temporal variant of frontotemporal lobar degeneration, characterized by prosopagnosia as an unusual disease onset. RESULTS: The neuropsychological evaluation showed a progressive deficit in face recognition and a slow decline in language in the absence of behavioral alterations...
August 4, 2016: Acta Clinica Belgica
https://www.readbyqxmd.com/read/27341359/significance-of-the-feuerstein-approach-in-neurocognitive-rehabilitation
#10
Jo Lebeer
BACKGROUND: The theory of Structural Cognitive Modifiability and Mediated Learning Experience of Reuven Feuerstein states that individuals with brain impairment, because of congenital or acquired origin, may substantially and structurally improve their cognitive functioning, by a systematic intervention based on a specific, criteria-based type of interaction ("mediated learning"). Three application systems are based on it: a dynamic-interactive assessment of learning capacity and processes of learning, the LPAD (Learning Propensity Assessment Device); a cognitive intervention program called "Instrumental Enrichment Program", which trains cognitive, metacognitive and executive functions; and a program, which is oriented at working in context, Shaping Modifying Environments...
June 18, 2016: NeuroRehabilitation
https://www.readbyqxmd.com/read/27222795/structural-and-functional-brain-abnormalities-place-phenocopy-frontotemporal-dementia-ftd-in-the-ftd-spectrum
#11
Rebecca M E Steketee, Rozanna Meijboom, Esther E Bron, Robert Jan Osse, Inge de Koning, Lize C Jiskoot, Stefan Klein, Frank Jan de Jong, Aad van der Lugt, John C van Swieten, Marion Smits
PURPOSE: 'Phenocopy' frontotemporal dementia (phFTD) patients may clinically mimic the behavioral variant of FTD (bvFTD), but do not show functional decline or abnormalities upon visual inspection of routine neuroimaging. We aimed to identify abnormalities in gray matter (GM) volume and perfusion in phFTD and to assess whether phFTD belongs to the FTD spectrum. We compared phFTD patients with both healthy controls and bvFTD patients. MATERIALS & METHODS: Seven phFTD and 11 bvFTD patients, and 20 age-matched controls underwent structural T1-weighted magnetic resonance imaging (MRI) and 3D pseudo-continuous arterial spin labeling (pCASL) at 3T...
2016: NeuroImage: Clinical
https://www.readbyqxmd.com/read/27194245/grey-matter-density-predicts-the-improvement-of-naming-abilities-after-tdcs-intervention-in-agrammatic-variant-of-primary-progressive-aphasia
#12
Maria Cotelli, Rosa Manenti, Donata Paternicò, Maura Cosseddu, Michela Brambilla, Michela Petesi, Enrico Premi, Roberto Gasparotti, Orazio Zanetti, Alessandro Padovani, Barbara Borroni
Agrammatic variant primary progressive aphasia is a neurodegenerative disorder specifically characterized by language deficits. A recent study has demonstrated a beneficial effect of transcranial direct current stimulation (tDCS) in combination with language training on naming accuracy in these patients. The aim of the study was to evaluate whether the improvement of naming accuracy after tDCS during language training was related to regional grey matter (GM) density. Eighteen avPPA patients underwent a brain magnetic resonance imaging before receiving a treatment that consisted of tDCS over the left dorsolateral prefrontal cortex during individualized language training (10 daily therapy sessions, 5 days per week from Monday to Friday)...
September 2016: Brain Topography
https://www.readbyqxmd.com/read/27163810/neuroimaging-correlates-of-frontotemporal-dementia-associated-with-sqstm1-mutations
#13
Elkin Luis, Alexandra Ortiz, Luis Eudave, Sara Ortega-Cubero, Barbara Borroni, Julie van der Zee, Stefano Gazzina, Paola Caroppo, Elisa Rubino, Federico D'Agata, Isabelle Le Ber, Isabel Santana, Gil Cunha, Maria R Almeida, Claire Boutoleau-Bretonnière, Didier Hannequin, David Wallon, Innocenzo Rainero, Daniela Galimberti, Christine Van Broeckhoven, Maria A Pastor, Pau Pastor
BACKGROUND: Frontotemporal lobar degeneration (FTLD) is a progressive dementia characterized by focal atrophy of frontal and/or temporal lobes caused by mutations in the gene coding for sequestosome 1 (SQSTM1), among other genes. Rare SQSTM1 gene mutations have been associated with Paget's disease of bone, amyotrophic lateral sclerosis, and, more recently, frontotemporal lobar degeneration (FTLD). OBJECTIVE: The aim of the study was to determine whether a characteristic pattern of grey and white matter loss is associated with SQSTM1 dysfunction...
May 7, 2016: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/27003218/hippocampal-sclerosis-of-aging-a-common-alzheimer-s-disease-mimic-risk-genotypes-are-associated-with-brain-atrophy-outside-the-temporal-lobe
#14
Kwangsik Nho, Andrew J Saykin, Peter T Nelson
Hippocampal sclerosis of aging (HS-Aging) is a common brain disease in older adults with a clinical course that is similar to Alzheimer's disease. Four single-nucleotide polymorphisms (SNPs) have previously shown association with HS-Aging. The present study investigated structural brain changes associated with these SNPs using surface-based analysis. Participants from the Alzheimer's Disease Neuroimaging Initiative cohort (ADNI; n = 1,239), with both MRI scans and genotype data, were used to assess the association between brain atrophy and previously identified HS-Aging risk SNPs in the following genes: GRN, TMEM106B, ABCC9, and KCNMB2 (minor allele frequency for each is >30%)...
2016: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/26928581/mapping-brain-morphological-and-functional-conversion-patterns-in-predementia-late-onset-bvftd
#15
Silvia Morbelli, Michela Ferrara, Francesco Fiz, Barbara Dessi, Dario Arnaldi, Agnese Picco, Irene Bossert, Ambra Buschiazzo, Jennifer Accardo, Lorena Picori, Nicola Girtler, Paola Mandich, Marco Pagani, Gianmario Sambuceti, Flavio Nobili
PURPOSE: The diagnosis of behavioural variant frontotemporal dementia (bvFTD) is challenging during the predementia stage when symptoms are subtle and confounding. Morphological and functional neuroimaging can be particularly helpful during this stage but few data are available. METHODS: We retrospectively selected 25 patients with late-onset probable bvFTD. Brain structural MRI and FDG PET were performed during the predementia stage (mean MMSE score 27.1 ± 2...
July 2016: European Journal of Nuclear Medicine and Molecular Imaging
https://www.readbyqxmd.com/read/26859219/fusion-fdg-pet-mri-evidence-to-support-diagnosis-of-frontotemporal-dementia-related-primary-progressive-aphasia
#16
Aaron VanDreel, Syed A Aftab, Prateek Sanan, Christopher Grant, Yang Lu
A 61-year-old right-handed man presented for cognitive neurological evaluation with word-finding difficulty, impaired word retrieval, impaired repetition of phrases, and stammering. Brain MRI and FDG-PET/CT were performed as initial imaging workup. Further FDG PET/MRI brain images were obtained through software fusion and revealed regional cortical atrophy with corresponding hypometabolic activity involving the posterior aspects of the left middle and inferior temporal gyri. These characteristic imaging findings are supportive of patient's diagnosis of frontotemporal dementia-related primary progressive aphasia...
September 2016: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/26827655/white-matter-hyperintensities-characterize-monogenic-frontotemporal-dementia-with-granulin-mutations
#17
Donata Paternicò, Enrico Premi, Stefano Gazzina, Maura Cosseddu, Antonella Alberici, Silvana Archetti, Maria S Cotelli, Anna Micheli, Marinella Turla, Roberto Gasparotti, Alessandro Padovani, Barbara Borroni
No study but one has suggested the presence of white matter hyperintensities (WMHs) in frontotemporal dementia (FTD), limited to 4 cases carrying pathogenic Granulin (GRN) gene mutations. We investigated the presence of WMHs in a cohort of 14 FTD patients with pathogenic GRN mutations (GRN+), 28 patients without GRN mutations (GRN-) and 18 healthy controls (HC). We further considered 11 asymptomatic GRN+ subjects and 11 young age-matched healthy controls (yHC). The WMH burden was automatically computed and a voxelwise-based analysis was carried out to explore the differences in WMH brain spatial distribution...
February 2016: Neurobiology of Aging
https://www.readbyqxmd.com/read/26811253/on-the-right-side-a-longitudinal-study-of-left-versus-right-lateralized-semantic-dementia
#18
COMPARATIVE STUDY
Fiona Kumfor, Ramon Landin-Romero, Emma Devenney, Rosalind Hutchings, Roberto Grasso, John R Hodges, Olivier Piguet
The typical presentation of semantic dementia is associated with marked, left predominant anterior temporal lobe atrophy and with changes in language. About 30% of individuals, however, present with predominant right anterior temporal lobe atrophy, usually accompanied by behavioural changes and prosopagnosia. Here, we aimed to establish whether these initially distinct clinical presentations evolve into a similar syndrome at the neural and behavioural level. Thirty-one patients who presented with predominant anterior temporal lobe atrophy were included...
March 2016: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/26646114/neuropsychological-and-neuroanatomical-correlates-of-the-social-norms-questionnaire-in-frontotemporal-dementia-versus-alzheimer-s-disease
#19
Hemali Panchal, Pongsatorn Paholpak, Grace Lee, Andrew Carr, Joseph P Barsuglia, Michelle Mather, Elvira Jimenez, Mario F Mendez
Traditional neuropsychological batteries may not distinguish early behavioral variant frontotemporal dementia (bvFTD) from Alzheimer's disease (AD) without the inclusion of a social behavioral measure. We compared 33 participants, 15 bvFTD, and 18 matched patients with early-onset AD (eAD), on the Social Norms Questionnaire (SNQ), neuropsychological tests and 3-dimensional T1-weighted magnetic resonance imaging (MRI). The analyses included correlations of SNQ results (total score, overendorsement or "overadhere" errors, and violations or "break" errors) with neuropsychological results and tensor-based morphometry regions of interest...
June 2016: American Journal of Alzheimer's Disease and Other Dementias
https://www.readbyqxmd.com/read/26606247/white-matter-changes-associated-with-resting-sympathetic-tone-in-frontotemporal-dementia-vs-alzheimer-s-disease
#20
Mario F Mendez, Aditi Joshi, Madelaine Daianu, Elvira Jimenez, Paul Thompson
BACKGROUND: Resting sympathetic tone, a measure of physiological arousal, is decreased in patients with apathy and inertia, such as those with behavioral variant frontotemporal dementia (bvFTD) and other frontally-predominant disorders. OBJECTIVE: To identify the neuroanatomical correlates of skin conductance levels (SCLs), an index of resting sympathetic tone and apathy, among patients with bvFTD, where SCLs is decreased, compared to those with Alzheimer's disease (AD), where it is not...
2015: PloS One
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