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"Primary progressive aphasia"

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https://www.readbyqxmd.com/read/29679378/prominent-microglial-activation-in-cortical-white-matter-is-selectively-associated-with-cortical-atrophy-in-primary-progressive-aphasia
#1
Daniel T Ohm, Garam Kim, Tamar Gefen, Alfred Rademaker, Sandra Weintraub, Eileen Bigio, M-Marsel Mesulam, Emily Rogalski, Changiz Geula
AIMS: Primary progressive aphasia (PPA) is a clinical syndrome characterized by selective language impairments associated with focal cortical atrophy favouring the language dominant hemisphere. PPA is associated with Alzheimer's disease (AD), frontotemporal lobar degeneration (FTLD), and significant accumulation of activated microglia. Activated microglia can initiate an inflammatory cascade that may contribute to neurodegeneration, but their quantitative distribution in cortical white matter and their relationship with cortical atrophy are unknown...
April 21, 2018: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/29665116/atrophy-and-microglial-distribution-in-primary-progressive-aphasia-with-tdp-43
#2
Garam Kim, Kabriya Bolbolan, Tamar Gefen, Sandra Weintraub, Eileen H Bigio, Emily Rogalski, Marek-Marsel Mesulam, Changiz Geula
OBJECTIVE: To quantitatively determine the density and distribution of activated microglia across cortical regions and hemispheres in the brains of primary progressive aphasia (PPA) participants with pathologic diagnoses of FTLD-TDP, and to examine the relationships between microglial densities, patterns of focal atrophy, TDP-43 inclusions, and clinical phenotype. METHODS: Activated microglia and TDP-43 inclusions were visualized in whole-hemisphere brain sections using immunohistochemical methods from 5 participants with PPA-TDP...
April 17, 2018: Annals of Neurology
https://www.readbyqxmd.com/read/29656245/individual-differences-in-socioemotional-sensitivity-are-an-index-of-salience-network-function
#3
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/29584904/combined-pathologies-in-ftld-tdp-types-a-and-c
#4
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
https://www.readbyqxmd.com/read/29569990/molecular-neuroimaging-in-primary-progressive-aphasia-with-predominant-agraphia
#5
Rene L Utianski, Joseph R Duffy, Rodolfo Savica, Jennifer L Whitwell, Mary M Machulda, Keith A Josephs
A 62-year-old male presented with progressive isolated writing and spelling difficulties. Neurological, neuropsychological, speech, and language evaluations identified only minimal additional abnormalities. The presenting characteristics did not meet criteria for any particular variant of primary progressive aphasia; his clinical presentation is best described as primary progressive aphasia, with a predominant, almost pure agraphia. Brain MRI showed asymmetric, bilateral parenchymal volume loss, with left hippocampal atrophy...
March 23, 2018: Neurocase
https://www.readbyqxmd.com/read/29561315/a-degenerative-form-of-mixed-transcortical-aphasia
#6
Leila Saadatpour, Usama Tariq, Alicia Parker, Leilani Doty, Kenneth M Heilman
Mixed transcortical aphasia (MTA) is characterized by decreased spontaneous speech, impaired naming, and poor comprehension, but with intact repetition. MTA has been reported to be the sequela of left hemisphere watershed infarction that isolates Wernicke's perisylvian arc. We report a 55-year-old right-handed woman who began having word-finding difficulty and then gradually developed impaired spontaneous speech, comprehension, and naming, but with intact repetition. Magnetic resonance imaging showed atrophy in the left frontal, parietal, and temporal lobes...
March 2018: Cognitive and Behavioral Neurology: Official Journal of the Society for Behavioral and Cognitive Neurology
https://www.readbyqxmd.com/read/29559782/repetitive-transcranial-magnetic-stimulation-for-the-treatment-of-cognitive-impairment-in-frontotemporal-dementia-an-open-label-pilot-study
#7
Jakub Antczak, Katarzyna Kowalska, Aleksandra Klimkowicz-Mrowiec, Barbara Wach, Katarzyna Kasprzyk, Marta Banach, Karolina Rzeźnicka-Brzegowy, Jadwiga Kubica, Agnieszka Słowik
Background: Frontotemporal dementia (FTD) is one of the most frequent dementia types in patients under 65 years of age. Currently, no therapy can effectively improve the cognitive deficits associated with FTD. Repetitive transcranial magnetic stimulation (rTMS) is a noninvasive method of inducing brain plasticity with therapeutic potential in neurodegenerative diseases. The purpose of this study was to evaluate the effect of rTMS on cognitive, behavioral, and emotional function in FTD...
2018: Neuropsychiatric Disease and Treatment
https://www.readbyqxmd.com/read/29544341/semantic-typicality-effects-in-primary-progressive-aphasia
#8
Ellyn A Riley, Elena Barbieri, Sandra Weintraub, M Marsel Mesulam, Cynthia K Thompson
Prototypical items within a semantic category are processed faster than atypical items within the same category. This typicality effect reflects normal representation and processing of semantic categories and when absent may be reflective of lexical-semantic deficits. We examined typicality effects in individuals with semantic and nonsemantic variants of primary progressive aphasia (PPA; semantic-PPA-S, agrammatic-PPA-G), a neurodegenerative disorder characterized by specific decline in language function, and age-matched controls...
January 1, 2018: American Journal of Alzheimer's Disease and Other Dementias
https://www.readbyqxmd.com/read/29520325/olfactory-and-imaging-features-in-atypical-alzheimer-s-disease
#9
Zhang Huihong, Wang Pan, Zhang Chunfeng, Wang Yan, Zhang Hui, Cai Li, Zhou Yuying
Objectives: Cognition and speech disorders are the most common symptoms of dementia in neurodegenerative disease. Here, we present a detailed clinical evaluation of a case of logopenic variant of primary progressive aphasia (lv-PPA), an atypical form of Alzheimer disease (AD), including cognitive testing over time, brain imaging, electrophysiology, and tests of olfactory function. Case report: We present the case of a 58-year-old man suffering from progressive language difficulties who was finally diagnosed with lv-PPA...
2018: Translational Neuroscience
https://www.readbyqxmd.com/read/29516757/determining-stability-in-connected-speech-in-primary-progressive-aphasia-and-alzheimer-s-disease
#10
Ashleigh Beales, Anne Whitworth, Jade Cartwright, Peter K Panegyres, Robert T Kane
PURPOSE: Using connected speech to assess progressive language disorders is confounded by uncertainty around whether connected speech is stable over successive sampling, and therefore representative of an individual's performance, and whether some contexts and/or language behaviours show greater stability than others. METHOD: A repeated measure, within groups, research design was used to investigate stability of a range of behaviours in the connected speech of six individuals with primary progressive aphasia and three individuals with Alzheimer's disease...
March 8, 2018: International Journal of Speech-language Pathology
https://www.readbyqxmd.com/read/29514947/clinical-value-of-neurofilament-and-phospho-tau-tau-ratio-in-the-frontotemporal-dementia-spectrum
#11
Lieke H H Meeter, Everard G Vijverberg, Marta Del Campo, Annemieke J M Rozemuller, Laura Donker Kaat, Frank Jan de Jong, Wiesje M van der Flier, Charlotte E Teunissen, John C van Swieten, Yolande A L Pijnenburg
OBJECTIVE: To examine the clinical value of neurofilament light chain (NfL) and the phospho-tau/total tau ratio (p/t-tau) across the entire frontotemporal dementia (FTD) spectrum in a large, well-defined cohort. METHODS: CSF NfL and p/t-tau levels were studied in 361 patients with FTD: 179 behavioral variant FTD, 17 FTD with motor neuron disease (FTD-MND), 36 semantic variant primary progressive aphasia (PPA), 19 nonfluent variant PPA, 4 logopenic variant PPA (lvPPA), 42 corticobasal syndrome, and 64 progressive supranuclear palsy...
April 3, 2018: Neurology
https://www.readbyqxmd.com/read/29514157/cognitive-reserve-hypothesis-in-frontotemporal-dementia-evidence-from-a-brain-spect-study-in-a-series-of-greek-frontotemporal-dementia-patients
#12
Pantelis Maiovis, Panagiotis Ioannidis, Georgios Gerasimou, Anna Gotzamani-Psarrakou, Dimitrios Karacostas
BACKGROUND AND OBJECTIVE: Cognitive reserve (CR) mediates the clinical expression of brain pathology in Alzheimer's disease, while there are much less relevant data in frontotemporal dementia (FTD). In the present study we examined whether CR, measured using the Cognitive Reserve Index (CRI), correlated with regional cerebral blood flow (rCBF) in Greek FTD patients. METHODS: Eighty FTD patients, i.e., 47 with behavioral variant FTD (bvFTD) and 33 with primary progressive aphasia (PPA), were enrolled into this study...
March 7, 2018: Neuro-degenerative Diseases
https://www.readbyqxmd.com/read/29508660/a-case-of-semantic-variant-primary-progressive-aphasia-with-pick-s-pathology
#13
Alyssa Caplan, Gabe Marx, Jonathan Elofson, Claire Lis, Lea Grinberg, Bruce Miller, Howard Rosen
Neurodegenerative diseases are caused by aggregation of specific proteins that catalyze a cascade of changes that ultimately lead to neurodegeneration. This concept guides current diagnostic approaches, as well as clinical trials, that focus on detecting or removing amyloid or tau from the brain. The semantic variant of primary progressive aphasia (svPPA), a clinical syndrome associated with frontotemporal lobar degeneration (FTLD) pathology, is usually associated with the molecular pathology TDP-C, but there are cases with TDP-B and Pick's disease...
March 6, 2018: Neurocase
https://www.readbyqxmd.com/read/29504965/amyloid-pet-positivity-in-different-primary-progressive-aphasia-phenotypes
#14
Emilie Beaufils, Johnny Vercouillie, Emilie Vierron, Jean-Philippe Cottier, Vincent Camus, Karl Mondon, Denis Guilloteau, Caroline Hommet, Maria Joao Ribeiro
PURPOSE: Primary progressive aphasia (PPA) is a neurological syndrome in which language functions become progressively impaired with relative sparing of memory and other instrumental functions. The pathologic causes of PPA are heterogeneous, but studies suggest that logopenic PPA (LPA) is underpinned by Alzheimer disease (AD) pathology in a high proportion of cases. The purposes of this descriptive and retrospective study were to characterize F-florbetapir PET imaging in a group of patients with a clinical syndrome of PPA, to determine the value of clinical characterization based on language phenotype in predicting the underlying pathology of PPA with F-florbetapir, and to quantify amyloid load in PPA subjects classified as "positive" F-florbetapir scans...
April 2018: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/29503630/a-longitudinal-study-of-a-chinese-man-presenting-with-non-fluent-agrammatic-variant-of-primary-progressive-aphasia
#15
Xiaoyan Liu, Fangping He, Zhongqin Chen, Ping Liu, Guoping Peng
Primary progressive aphasia (PPA) is a neurodegenerative disease characterized by declining language ability. However, the difficulty in defining the central clinical features in its earliest stage and establishing the dynamics of its progression has led to controversy. We report a 71-year-old man with Han language suffering from non-fluent/agrammatic variant of PPA but presenting as typical Alzheimer's disease (AD) and confused with logopenic variant of PPA in its early stage, longitudinally describing his clinical characteristics, neuroanatomical basis, and genetic associations, and exploring the underlying pathology...
2018: Frontiers in Neurology
https://www.readbyqxmd.com/read/29497748/describing-phonological-paraphasias-in-three-variants-of-primary-progressive-aphasia
#16
Sarah Grace Hudspeth Dalton, Christine Shultz, Maya L Henry, Argye E Hillis, Jessica D Richardson
Purpose: The purpose of this study was to describe the linguistic environment of phonological paraphasias in 3 variants of primary progressive aphasia (semantic, logopenic, and nonfluent) and to describe the profiles of paraphasia production for each of these variants. Method: Discourse samples of 26 individuals diagnosed with primary progressive aphasia were investigated for phonological paraphasias using the criteria established for the Philadelphia Naming Test (Moss Rehabilitation Research Institute, 2013)...
March 1, 2018: American Journal of Speech-language Pathology
https://www.readbyqxmd.com/read/29451323/-18f-av-1451-tau-pet-and-primary-progressive-aphasia
#17
Keith A Josephs, Peter R Martin, Hugo Botha, Christopher G Schwarz, Joseph R Duffy, Heather M Clark, Mary M Machulda, Jonathan Graff-Radford, Stephen D Weigand, Matthew L Senjem, Rene L Utianski, Daniel A Drubach, Bradley F Boeve, David T Jones, David S Knopman, Ronald C Petersen, Clifford R Jack, Val J Lowe, Jennifer L Whitwell
OBJECTIVES: To assess [ 18 F]AV-1451 tau-PET uptake patterns across the primary progressive aphasia (PPA) variants (logopenic, semantic and agrammatic), examine regional uptake patterns of [ 18 F]AV-1451 independent of clinical diagnosis, and compare the diagnostic utility of [ 18 F]AV-1451, [ 18 F]-fluorodeoxygluclose (FDG)-PET and MRI to differentiate the PPA variants. METHODS: We performed statistical parametric mapping of [ 18 F]AV-1451 across 40 PPA patients (logopenic-PPA=14, semantic-PPA=13 and agrammatic-PPA=13) compared to 80 cognitively normal, PiB-negative controls, age and gender matched 2:1...
February 16, 2018: Annals of Neurology
https://www.readbyqxmd.com/read/29444966/cerebral-microbleeds-and-csf-alzheimer-biomarkers-in-primary-progressive-aphasias
#18
Aline Mendes, Anne Bertrand, Foudil Lamari, Olivier Colliot, Alexandre Routier, Olivier Godefroy, Frédérique Etcharry-Bouyx, Olivier Moreaud, Florence Pasquier, Philippe Couratier, Karim Bennys, Martine Vercelletto, Olivier Martinaud, Bernard Laurent, Jérémie Pariente, Michèle Puel, Stéphane Epelbaum, Serge Belliard, Takoua Kaaouana, Ludovic Fillon, Marie Chupin, Bruno Dubois, Marc Teichmann
OBJECTIVE: To reveal the prevalence and localization of cerebral microbleeds (CMBs) in the 3 main variants of primary progressive aphasia (PPA) (logopenic, semantic, and nonfluent/agrammatic), to identify the relationship with underlying Alzheimer pathology, and to explore whether CMBs contribute to language breakdown. METHODS: We used a cross-sectional design in a multicenter cohort of 82 patients with PPA and 19 similarly aged healthy controls. MRI allowed for rating CMBs (2-dimensional gradient recalled echo T2*, susceptibility weighted imaging sequences) and white matter hyperintensities...
February 14, 2018: Neurology
https://www.readbyqxmd.com/read/29438114/validity-and-reliability-of-the-frontotemporal-dementia-rating-scale-ftd-frs-for-the-progression-and-staging-of-dementia-in-brazilian-patients
#19
Thaís B Lima-Silva, Valéria S Bahia, Mário A Cecchini, Luciana Cassimiro, Henrique C Guimarães, Leandro B Gambogi, Paulo Caramelli, Márcio Balthazar, Benito Damasceno, Sônia M D Brucki, Leonardo C de Souza, Ricardo Nitrini, Eneida Mioshi, Mônica S Yassuda
INTRODUCTION: Few studies on instruments for staging frontotemporal dementia (FTD) have been conducted. OBJECTIVE: The objective of this study was to analyze the factor structure, internal consistency, reliability, and convergent validity of the Brazilian version of the Frontotemporal Dementia Rating Scale (FTD-FRS). METHODS: A total of 97 individuals aged 40 years and above with >2 years' education took part in the study, 31 patients diagnosed with behavioral variant FTD (bvFTD), 8 patients with primary progressive aphasia, 28 with Alzheimer disease, 8 with mild cognitive impairment, and a control group of 22 healthy subjects...
February 13, 2018: Alzheimer Disease and Associated Disorders
https://www.readbyqxmd.com/read/29429985/posterior-associative-and-cingulate-cortex-involvement-of-brain-single-photon-emission-computed-tomography-spect-imaging-in-semantic-dementia-with-probable-alzheimer-disease-pathology-a-case-report
#20
Yumi Takano, Keiko Kunitoki, Yasuko Tatewaki, Tatsushi Mutoh, Tomoko Totsune, Hideo Shimomura, Manabu Nakagawa, Hiroyuki Arai, Yasuyuki Taki
BACKGROUND Semantic dementia (SD) is a type of primary progressive aphasia with prominent language dysfunction, mostly within the spectrum of frontotemporal lobar degeneration (FTLD). Although there is an overlap in clinical manifestations of SD attributable to FTLD and neuropathologically proven Alzheimer disease (AD), clinical diagnostic clues are not readily available. We present a characteristic finding based on a single-photon emission computed tomography (SPECT)-based regional cerebral blood flow study and its statistical imaging analysis for a rare case of SD with AD-like pathology...
February 12, 2018: American Journal of Case Reports
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