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"Primary progressive aphasia"

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https://www.readbyqxmd.com/read/29909180/parkinsonism-is-associated-with-altered-primary-motor-cortex-plasticity-in-frontotemporal-dementia-primary-progressive-aphasia-variant
#1
Flavio Di Stasio, Suppa Antonio, Fabbrini Andrea, Marsili Luca, Asci Francesco, Conte Antonella, Trebbastoni Alessandro, De Lena Carlo, Berardelli Alfredo
In frontotemporal dementia (FTD), the behavioral variant (bv-FTD) and nonfluent variant of primary progressive aphasia (nfv-PPA) reflect a prominent neurodegenerative involvement of the frontal lobe networks, which may include the premotor and motor areas and thus cause heterogeneous clinical symptoms including parkinsonism. With the technique of transcranial magnetic stimulation, we investigated long-term potentiation- and long-term depression-like plasticity in the primary motor cortex of bv-FTD and nfv-PPA patients, with and without parkinsonism, by using the theta-burst stimulation (TBS) protocol...
May 29, 2018: Neurobiology of Aging
https://www.readbyqxmd.com/read/29876259/thalamic-atrophy-in-frontotemporal-dementia-not-just-a-c9orf72-problem
#2
Martina Bocchetta, Elizabeth Gordon, M Jorge Cardoso, Marc Modat, Sebastien Ourselin, Jason D Warren, Jonathan D Rohrer
Background: Frontotemporal dementia (FTD) is a heterogeneous neurodegenerative disorder associated with frontal and temporal atrophy. Subcortical involvement has been described as well, with early thalamic atrophy most commonly associated with the C9orf72 expansion. However thalamic involvement has not been comprehensively investigated across the FTD spectrum. Methods: We investigated thalamic volumes in a sample of 341 FTD patients (age: mean(standard deviation) 64...
2018: NeuroImage: Clinical
https://www.readbyqxmd.com/read/29851876/primary-progressive-aphasia-and-stroke-aphasia
#3
Murray Grossman, David J Irwin
PURPOSE OF REVIEW: This article summarizes the clinical and anatomic features of the three named variants of primary progressive aphasia (PPA): semantic variant PPA, nonfluent/agrammatic variant PPA, and logopenic variant PPA. Three stroke aphasia syndromes that resemble the PPA variants (Broca aphasia, Wernicke aphasia, and conduction aphasia) are also presented. RECENT FINDINGS: Semantic variant PPA and Wernicke aphasia are characterized by fluent speech with naming and comprehension difficulty; these syndromes are associated with disease in different portions of the left temporal lobe...
June 2018: Continuum: Lifelong Learning in Neurology
https://www.readbyqxmd.com/read/29851873/bedside-approach-to-the-mental-status-assessment
#4
David F Tang-Wai, Morris Freedman
PURPOSE OF REVIEW: This article presents a clinically useful approach to obtaining the history and performing the mental status examination of patients with cognitive, language, or behavioral problems. RECENT FINDINGS: Laboratory and imaging biomarkers are being developed for accurate diagnosis of neurobehavioral disorders, yet few are currently available for clinical use. Moreover, not all centers have access to these potential tools. Practicing clinicians are therefore left primarily with their skills of history taking and examination...
June 2018: Continuum: Lifelong Learning in Neurology
https://www.readbyqxmd.com/read/29845004/abnormal-language-related-oscillatory-responses-in-primary-progressive-aphasia
#5
A Kielar, T Deschamps, R Jokel, J A Meltzer
Patients with Primary Progressive Aphasia (PPA) may react to linguistic stimuli differently than healthy controls, reflecting degeneration of language networks and engagement of compensatory mechanisms. We used magnetoencephalography (MEG) to evaluate oscillatory neural responses in sentence comprehension, in patients with PPA and age-matched controls. Participants viewed sentences containing semantically and syntactically anomalous words that evoke distinct oscillatory responses. For age-matched controls, semantic anomalies elicited left-lateralized 8-30 Hz power decreases distributed along ventral brain regions, whereas syntactic anomalies elicited bilateral power decreases in both ventral and dorsal regions...
2018: NeuroImage: Clinical
https://www.readbyqxmd.com/read/29802865/a-nonverbal-route-to-conceptual-knowledge-involving-the-right-anterior-temporal-lobe
#6
Robert S Hurley, M-Marsel Mesulam, Jaiashre Sridhar, Emily J Rogalski, Cynthia K Thompson
The semantic variant of primary progressive aphasia (PPA-S) is diagnosed based on impaired single-word comprehension, but nonverbal impairments in face and object recognition can also be present, particularly in later disease stages. PPA-S is associated with focal atrophy in the left anterior temporal lobe (ATL), often accompanied by a lesser degree of atrophy in the right ATL. According to a dual-route account, the left ATL is critical for verbal access to conceptual knowledge while nonverbal access to conceptual knowledge depends upon the integrity of right ATL...
May 23, 2018: Neuropsychologia
https://www.readbyqxmd.com/read/29779573/-moon-river-intact-musical-appreciation-and-performance-in-a-man-with-semantic-variant-primary-progressive-aphasia
#7
Patrick A Ho, Theodore A Stern, James K Rustad
No abstract text is available yet for this article.
April 3, 2018: Psychosomatics
https://www.readbyqxmd.com/read/29753915/the-eyes-reveal-uncertainty-about-object-distinctions-in-semantic-variant-primary-progressive-aphasia
#8
Andreia V Faria, David Race, Kevin Kim, Argye E Hillis
At least three distinct variants of primary progressive aphasia (PPA) have been described, but they are difficult to distinguish early in the course, when individuals experience primarily anomia. People with svPPA are often the hardest to care for, because they have impaired comprehension of words and objects and often have negative changes in comportment. We sought to identify an early marker of semantic variant primary progressive aphasia (svPPA) and to enhance the understanding of the semantic deficit in svPPA...
April 3, 2018: Cortex; a Journal Devoted to the Study of the Nervous System and Behavior
https://www.readbyqxmd.com/read/29744573/diagnostic-utility-of-fdg-pet-in-the-differential-diagnosis-between-different-forms-of-primary-progressive-aphasia
#9
Femke Bouwman, Stefania Orini, Federica Gandolfo, Daniele Altomare, Cristina Festari, Federica Agosta, Javier Arbizu, Alexander Drzezga, Peter Nestor, Flavio Nobili, Zuzana Walker, Silvia Morbelli, Marina Boccardi
PURPOSE: A joint effort of the European Association of Nuclear Medicine (EANM) and the European Academy of Neurology (EAN) aims at clinical guidance for the use of FDG-PET in neurodegenerative diseases. This paper addresses the diagnostic utility of FDG-PET over clinical/neuropsychological assessment in the differentiation of the three forms of primary progressive aphasia (PPA). METHODS: Seven panelists were appointed by the EANM and EAN and a literature search was performed by using harmonized PICO (Population, Intervention, Comparison, Outcome) question keywords...
May 9, 2018: European Journal of Nuclear Medicine and Molecular Imaging
https://www.readbyqxmd.com/read/29718131/retraining-speech-production-and-fluency-in-non-fluent-agrammatic-primary-progressive-aphasia
#10
Maya L Henry, H Isabel Hubbard, Stephanie M Grasso, Maria Luisa Mandelli, Stephen M Wilson, Mithra T Sathishkumar, Julius Fridriksson, Wylin Daigle, Adam L Boxer, Bruce L Miller, Maria Luisa Gorno-Tempini
The non-fluent/agrammatic variant of primary progressive aphasia (nfvPPA) presents with a gradual decline in grammar and motor speech resulting from selective degeneration of speech-language regions in the brain. There has been considerable progress in identifying treatment approaches to remediate language deficits in other primary progressive aphasia variants; however, interventions for the core deficits in nfvPPA have yet to be systematically investigated. Further, the neural mechanisms that support behavioural restitution in the context of neurodegeneration are not well understood...
April 30, 2018: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/29710718/the-progressive-acalculia-presentation-of-parietal-variant-alzheimer-s-disease
#11
Mario F Mendez, Negar Moheb, Randy E Desarzant, Edmond H Teng
BACKGROUND: Many patients with early-onset Alzheimer's disease (EOAD; age of onset <65 years) have non-amnestic presentations involving language (logopenic primary progressive aphasia, lvPPA), visuospatial abilities (posterior cortical atrophy, PCA), and even asymmetric symptoms consistent with corticobasal syndrome (CBS). An inferior parietal lobule variant of EOAD commonly presents with progressive difficulty with calculations. METHODS: We reviewed 276 EOAD patients for presentations with predominant acalculia...
April 28, 2018: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/29695300/a-language-based-sum-score-for-the-course-and-therapeutic-intervention-in-primary-progressive-aphasia
#12
Elisa Semler, Sarah Anderl-Straub, Ingo Uttner, Janine Diehl-Schmid, Adrian Danek, Beate Einsiedler, Klaus Fassbender, Klaus Fliessbach, Hans-Jürgen Huppertz, Holger Jahn, Johannes Kornhuber, Bernhard Landwehrmeyer, Martin Lauer, Rainer Muche, Johannes Prudlo, Anja Schneider, Matthias L Schroeter, Albert C Ludolph, Markus Otto
BACKGROUND: With upcoming therapeutic interventions for patients with primary progressive aphasia (PPA), instruments for the follow-up of patients are needed to describe disease progression and to evaluate potential therapeutic effects. So far, volumetric brain changes have been proposed as clinical endpoints in the literature, but cognitive scores are still lacking. This study followed disease progression predominantly in language-based performance within 1 year and defined a PPA sum score which can be used in therapeutic interventions...
April 25, 2018: Alzheimer's Research & Therapy
https://www.readbyqxmd.com/read/29682238/rate-and-rhythm-control-strategies-for-apraxia-of-speech-in-nonfluent-primary-progressive-aphasia
#13
Bárbara Costa Beber, Monalise Costa Batista Berbert, Ruth Siqueira Grawer, Maria Cristina de Almeida Freitas Cardoso
The nonfluent/agrammatic variant of primary progressive aphasia is characterized by apraxia of speech and agrammatism. Apraxia of speech limits patients' communication due to slow speaking rate, sound substitutions, articulatory groping, false starts and restarts, segmentation of syllables, and increased difficulty with increasing utterance length. Speech and language therapy is known to benefit individuals with apraxia of speech due to stroke, but little is known about its effects in primary progressive aphasia...
January 2018: Dementia & Neuropsychologia
https://www.readbyqxmd.com/read/29679378/prominent-microglial-activation-in-cortical-white-matter-is-selectively-associated-with-cortical-atrophy-in-primary-progressive-aphasia
#14
D T Ohm, G Kim, T Gefen, A Rademaker, S Weintraub, E H Bigio, M-M Mesulam, E Rogalski, C 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 remain unknown...
April 21, 2018: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/29665116/atrophy-and-microglial-distribution-in-primary-progressive-aphasia-with-transactive-response-dna-binding-protein-43-kda
#15
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 pathological diagnoses of frontotemporal lobar degeneration with transactive response DNA-binding protein-43 (TDP-43) inclusions 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 five 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
#16
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...
June 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
#17
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...
May 1, 2018: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/29569990/molecular-neuroimaging-in-primary-progressive-aphasia-with-predominant-agraphia
#18
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...
April 2018: Neurocase
https://www.readbyqxmd.com/read/29561315/a-degenerative-form-of-mixed-transcortical-aphasia
#19
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
#20
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
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