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Progressive nonfluent aphasia

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https://www.readbyqxmd.com/read/29695300/a-language-based-sum-score-for-the-course-and-therapeutic-intervention-in-primary-progressive-aphasia
#1
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
#2
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/29559782/repetitive-transcranial-magnetic-stimulation-for-the-treatment-of-cognitive-impairment-in-frontotemporal-dementia-an-open-label-pilot-study
#3
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/29514947/clinical-value-of-neurofilament-and-phospho-tau-tau-ratio-in-the-frontotemporal-dementia-spectrum
#4
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/29504965/amyloid-pet-positivity-in-different-primary-progressive-aphasia-phenotypes
#5
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/29497748/describing-phonological-paraphasias-in-three-variants-of-primary-progressive-aphasia
#6
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/29444966/cerebral-microbleeds-and-csf-alzheimer-biomarkers-in-primary-progressive-aphasias
#7
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...
March 20, 2018: Neurology
https://www.readbyqxmd.com/read/29392464/primary-progressive-aphasia-a-clinical-approach
#8
Charles R Marshall, Chris J D Hardy, Anna Volkmer, Lucy L Russell, Rebecca L Bond, Phillip D Fletcher, Camilla N Clark, Catherine J Mummery, Jonathan M Schott, Martin N Rossor, Nick C Fox, Sebastian J Crutch, Jonathan D Rohrer, Jason D Warren
The primary progressive aphasias are a heterogeneous group of focal 'language-led' dementias that pose substantial challenges for diagnosis and management. Here we present a clinical approach to the progressive aphasias, based on our experience of these disorders and directed at non-specialists. We first outline a framework for assessing language, tailored to the common presentations of progressive aphasia. We then consider the defining features of the canonical progressive nonfluent, semantic and logopenic aphasic syndromes, including 'clinical pearls' that we have found diagnostically useful and neuroanatomical and other key associations of each syndrome...
February 1, 2018: Journal of Neurology
https://www.readbyqxmd.com/read/29201014/impaired-interoceptive-accuracy-in-semantic-variant-primary-progressive-aphasia
#9
Charles R Marshall, Chris J D Hardy, Lucy L Russell, Camilla N Clark, Katrina M Dick, Emilie V Brotherhood, Rebecca L Bond, Catherine J Mummery, Jonathan M Schott, Jonathan D Rohrer, James M Kilner, Jason D Warren
Background: Interoception (the perception of internal bodily sensations) is strongly linked to emotional experience and sensitivity to the emotions of others in healthy subjects. Interoceptive impairment may contribute to the profound socioemotional symptoms that characterize frontotemporal dementia (FTD) syndromes, but remains poorly defined. Methods: Patients representing all major FTD syndromes and healthy age-matched controls performed a heartbeat counting task as a measure of interoceptive accuracy...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/29186630/music-models-aberrant-rule-decoding-and-reward-valuation-in-dementia
#10
Camilla N Clark, Hannah L Golden, Oliver McCallion, Jennifer M Nicholas, Miriam H Cohen, Catherine F Slattery, Ross W Paterson, Phillip D Fletcher, Catherine J Mummery, Jonathan D Rohrer, Sebastian J Crutch, Jason D Warren
Aberrant rule- and reward-based processes underpin abnormalities of socio-emotional behaviour in major dementias. However, these processes remain poorly characterized. Here we used music to probe rule decoding and reward valuation in patients with frontotemporal dementia (FTD) syndromes and Alzheimer's disease (AD) relative to healthy age-matched individuals. We created short melodies that were either harmonically resolved ('finished') or unresolved ('unfinished'); the task was to classify each melody as finished or unfinished (rule processing) and rate its subjective pleasantness (reward valuation)...
February 1, 2018: Social Cognitive and Affective Neuroscience
https://www.readbyqxmd.com/read/29039275/visuospatial-functioning-in-the-primary-progressive-aphasias
#11
Christa L Watson, Katherine Possin, I Elaine Allen, H Isabel Hubbard, Marita Meyer, Ariane E Welch, Gil D Rabinovici, Howard Rosen, Katherine P Rankin, Zachary Miller, Miguel A Santos-Santos, Joel H Kramer, Bruce L Miller, Maria Luisa Gorno-Tempini
OBJECTIVES: The aim of this study was to identify whether the three main primary progressive aphasia (PPA) variants would show differential profiles on measures of visuospatial cognition. We hypothesized that the logopenic variant would have the most difficulty across tasks requiring visuospatial and visual memory abilities. METHODS: PPA patients (n=156), diagnosed using current criteria, and controls were tested on a battery of tests tapping different aspects of visuospatial cognition...
March 2018: Journal of the International Neuropsychological Society: JINS
https://www.readbyqxmd.com/read/28906375/correlations-between-clinical-characteristics-and-neuroimaging-in-chinese-patients-with-subtypes-of-frontotemporal-lobe-degeneration
#12
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/28813486/differentiating-between-subtypes-of-primary-progressive-aphasia-and-mild-cognitive-impairment-on-a-modified-version-of-the-frontal-behavioral-inventory
#13
Donna C Tippett, Carol B Thompson, Cornelia Demsky, Rajani Sebastian, Amy Wright, Argye E Hillis
Behavioral assessment has been investigated in frontotemporal lobar degeneration and Alzheimer's disease, but has not been explored extensively in subtypes of primary progressive aphasia (PPA). We explored the ability of a modified version of the Frontal Behavioral Inventory (FBI-mod) to discriminate between patients with distinct subtypes of PPA and patients with mild cognitive impairment (MCI). We hypothesized that individuals with nonfluent agrammatic PPA (nfaPPA) would have higher negative behavior scores than other groups and that individuals with semantic variant PPA (svPPA) would have higher disinhibition scores than other groups...
2017: PloS One
https://www.readbyqxmd.com/read/28803212/data-driven-classification-of-patients-with-primary-progressive-aphasia
#14
Paul Hoffman, Seyed Ahmad Sajjadi, Karalyn Patterson, Peter J Nestor
Current diagnostic criteria classify primary progressive aphasia into three variants-semantic (sv), nonfluent (nfv) and logopenic (lv) PPA-though the adequacy of this scheme is debated. This study took a data-driven approach, applying k-means clustering to data from 43 PPA patients. The algorithm grouped patients based on similarities in language, semantic and non-linguistic cognitive scores. The optimum solution consisted of three groups. One group, almost exclusively those diagnosed as svPPA, displayed a selective semantic impairment...
November 2017: Brain and Language
https://www.readbyqxmd.com/read/28624827/qualitative-assessment-of-verbal-fluency-performance-in-frontotemporal-dementia
#15
Esther van den Berg, Lize C Jiskoot, Mariëlle J H Grosveld, John C van Swieten, Janne M Papma
BACKGROUND/AIMS: Verbal fluency is impaired in patients with frontotemporal dementia (FTD) and primary progressive aphasia (PPA). This study explored qualitative differences in verbal fluency (clustering of words, switching between strategies) between FTD and PPA variants. METHODS: Twenty-nine patients with behavioral variant FTD (bvFTD) and 50 with PPA (13 nonfluent/agrammatic, 14 semantic, and 23 logopenic) performed a semantic and letter fluency task. Clustering (number of multiword strings) and switching (number of transitions between clustered and nonclustered words) were recorded by two independent raters...
2017: Dementia and Geriatric Cognitive Disorders
https://www.readbyqxmd.com/read/28592456/csf-sapp%C3%AE-ykl-40-and-neurofilament-light-in-frontotemporal-lobar-degeneration
#16
Daniel Alcolea, Eduard Vilaplana, Marc Suárez-Calvet, Ignacio Illán-Gala, Rafael Blesa, Jordi Clarimón, Albert Lladó, Raquel Sánchez-Valle, José L Molinuevo, Guillermo García-Ribas, Yaroslau Compta, María José Martí, Gerard Piñol-Ripoll, Guillermo Amer-Ferrer, Aina Noguera, Ana García-Martín, Juan Fortea, Alberto Lleó
OBJECTIVE: To analyze the clinical utility of 3 CSF biomarkers and their structural imaging correlates in a large cohort of patients with different dementia and parkinsonian syndromes within the spectrum of frontotemporal lobar degeneration (FTLD). METHODS: We analyzed 3 CSF biomarkers (YKL-40, soluble β fragment of amyloid precursor protein [sAPPβ], neurofilament light [NfL]) and core Alzheimer disease (AD) biomarkers (β-amyloid1-42, total tau, phosphorylated tau) in patients with FTLD-related clinical syndromes (n = 159): behavioral variant of frontotemporal dementia (n = 68), nonfluent (n = 23) and semantic (n = 19) variants of primary progressive aphasia, progressive supranuclear palsy (n = 28), and corticobasal syndrome (n = 21)...
July 11, 2017: Neurology
https://www.readbyqxmd.com/read/28579383/clinical-and-biological-phenotypes-of-frontotemporal-dementia-perspectives-for-disease-modifying-therapies
#17
S Gazzina, M A Manes, A Padovani, B Borroni
Frontotemporal Dementia (FTD) is a progressive neurodegenerative condition which encompasses a group of clinically, neuropathologically and genetically heterogeneous disorders characterized by selective involvement of the frontal and temporal lobes. FTD is characterized by changes in behaviour and personality, frontal executive deficits and language dysfunction. Different phenotypes have been defined on the basis of presenting clinical symptoms, behavioural variants of FTD (bvFTD) and primary progressive aphasia (PPA), which includes nonfluent/agrammatic variant PPA (avPPA) and semantic variant PPA (svPPA)...
June 1, 2017: European Journal of Pharmacology
https://www.readbyqxmd.com/read/28571652/functional-neuroanatomy-of-speech-signal-decoding-in-primary-progressive-aphasias
#18
Chris J D Hardy, Jennifer L Agustus, Charles R Marshall, Camilla N Clark, Lucy L Russell, Emilie V Brotherhood, Rebecca L Bond, Cassidy M Fiford, Sasha Ondobaka, David L Thomas, Sebastian J Crutch, Jonathan D Rohrer, Jason D Warren
The pathophysiology of primary progressive aphasias remains poorly understood. Here, we addressed this issue using activation fMRI in a cohort of 27 patients with primary progressive aphasia (nonfluent, semantic, and logopenic variants) versus 15 healthy controls. Participants listened passively to sequences of spoken syllables in which we manipulated 3-key auditory speech signal characteristics: temporal regularity, phonemic spectral structure, and pitch sequence entropy. Relative to healthy controls, nonfluent variant patients showed reduced activation of medial Heschl's gyrus in response to any auditory stimulation and reduced activation of anterior cingulate to temporal irregularity...
August 2017: Neurobiology of Aging
https://www.readbyqxmd.com/read/28549937/is-learning-episodic-memory-distinct-cognitive-and-neuroanatomic-correlates-of-immediate-recall-during-learning-trials-in-neurologically-normal-aging-and-neurodegenerative-cohorts
#19
K B Casaletto, G Marx, S Dutt, J Neuhaus, R Saloner, L Kritikos, B Miller, J H Kramer
Although commonly interpreted as a marker of episodic memory during neuropsychological exams, relatively little is known regarding the neurobehavior of "total learning" immediate recall scores. Medial temporal lobes are clearly associated with delayed recall performances, yet immediate recall may necessitate networks beyond traditional episodic memory. We aimed to operationalize cognitive and neuroanatomic correlates of total immediate recall in several aging syndromes. Demographically-matched neurologically normal adults (n=91), individuals with Alzheimer's disease (n=566), logopenic variant primary progressive aphasia (PPA) (n=34), behavioral variant frontotemporal dementia (n=97), semantic variant PPA (n=71), or nonfluent/agrammatic variant PPA (n=39) completed a neurocognitive battery, including the CVLT-Short Form trials 1-4 Total Immediate Recall; a majority subset also completed a brain MRI...
July 28, 2017: Neuropsychologia
https://www.readbyqxmd.com/read/28434708/dual-phase-18-f-florbetapir-positron-emission-tomography-in-patients-with-primary-progressive-aphasia-alzheimer-s-disease-and-healthy-controls-a-preliminary-study
#20
Hung-Chou Kuo, Ing-Tsung Hsiao, Chia-Ju Hsieh, Chu-Yun Huang, Kuo-Lun Huang, Yau-Yau Wai, Wen-Li Chuang, Mei-Ping Kung, Yi-Chuan Chu, Tzu-Chen Yen, Kun-Ju Lin, Chin-Chang Huang
BACKGROUND/PURPOSE: To determine whether dual-phase18 F-florbetapir positron emission tomography imaging with perfusion-like and amyloid deposition information can distinguish among primary progressive aphasia (PPA), Alzheimer's disease (AD), and healthy controls (HCs). METHODS: Patients diagnosed with PPA, including four semantic dementia (SD) and two progressive nonfluent aphasia (PNFA), as well as one logopenic variant (LV) of PPA, were studied. All PPA patients, and age-/sex-matched patients with probable AD (n=8) and HCs (n=8) were subjected to dual-phase18 F-florbetapir imaging...
December 2017: Journal of the Formosan Medical Association, Taiwan Yi Zhi
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