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Primary progressive aphasia

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https://www.readbyqxmd.com/read/29779573/-moon-river-intact-musical-appreciation-and-performance-in-a-man-with-semantic-variant-primary-progressive-aphasia
#1
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
#2
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
#3
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/29744128/fatal-case-of-amoebic-encephalitis-masquerading-as-herpes
#4
Rikesh Baral, Binit Vaidya
Primary amoebic meningoencephalitis (PAM) is a rare, fulminating, hemorrhagic infection of the brain caused by Naegleria fowleri , a thermophilic, free-living amoeba. A 74-year male presented with sudden severe global headache and fever with features of anomic aphasia. Magnetic resonance imaging (MRI) suggested herpes encephalitis and acyclovir (IV) was started but the patient developed altered sensorium, agitation and progressive weakness of lower limbs with gradual truncal weakness. Repeat MRI showed increase in lesion size and edema with confluent blood areas...
May 2018: Oxford Medical Case Reports
https://www.readbyqxmd.com/read/29718131/retraining-speech-production-and-fluency-in-non-fluent-agrammatic-primary-progressive-aphasia
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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