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Progressive non-fluent aphasia

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https://www.readbyqxmd.com/read/28713256/baseline-performance-predicts-tdcs-mediated-improvements-in-language-symptoms-in-primary-progressive-aphasia
#1
Eric M McConathey, Nicole C White, Felix Gervits, Sherry Ash, H Branch Coslett, Murray Grossman, Roy H Hamilton
Primary Progressive Aphasia (PPA) is a neurodegenerative condition characterized by insidious irreversible loss of language abilities. Prior studies suggest that transcranial direct current stimulation (tDCS) directed toward language areas of the brain may help to ameliorate symptoms of PPA. In the present sham-controlled study, we examined whether tDCS could be used to enhance language abilities (e.g., picture naming) in individuals with PPA variants primarily characterized by difficulties with speech production (non-fluent and logopenic)...
2017: Frontiers in Human Neuroscience
https://www.readbyqxmd.com/read/28611820/white-matter-disruption-and-connected-speech-in-non-fluent-and-semantic-variants-of-primary-progressive-aphasia
#2
Karine Marcotte, Naida L Graham, Kathleen C Fraser, Jed A Meltzer, David F Tang-Wai, Tiffany W Chow, Morris Freedman, Carol Leonard, Sandra E Black, Elizabeth Rochon
Differential patterns of white matter disruption have recently been reported in the non-fluent (nfvPPA) and semantic (svPPA) variants of primary progressive aphasia (PPA). No single measure is sufficient to distinguish between the PPA variants, but connected speech allows for the quantification of multiple measures. The aim of the present study was to further investigate the white matter correlates associated with connected speech features in PPA. We examined the relationship between white matter metrics and connected speech deficits using an automated analysis of transcriptions of connected speech and diffusion tensor imaging in language-related tracts...
January 2017: Dementia and Geriatric Cognitive Disorders Extra
https://www.readbyqxmd.com/read/28594853/the-unexpected-co-occurrence-of-grn-and-mapt-p-a152t-in-basque-families-clinical-and-pathological-characteristics
#3
Fermin Moreno, Begoña Indakoetxea, Myriam Barandiaran, María Cristina Caballero, Ana Gorostidi, Francesc Calafell, Alazne Gabilondo, Mikel Tainta, Miren Zulaica, José F Martí Massó, Adolfo López de Munain, Pascual Sánchez-Juan, Suzee E Lee
BACKGROUND: The co-occurrence of the c.709-1G>A GRN mutation and the p.A152T MAPT variant has been identified in 18 Basque families affected by frontotemporal dementia (FTD). We aimed to investigate the influence of the p.A152T MAPT variant on the clinical and neuropathological features of these Basque GRN families. METHODS AND FINDINGS: We compared clinical characteristics of 14 patients who carried the c.709-1G>A GRN mutation (GRN+/A152T-) with 21 patients who carried both the c...
2017: PloS One
https://www.readbyqxmd.com/read/28579951/communicative-pragmatic-assessment-is-sensitive-and-time-effective-in-measuring-the-outcome-of-aphasia-therapy
#4
Benjamin Stahl, Bettina Mohr, Felix R Dreyer, Guglielmo Lucchese, Friedemann Pulvermüller
A range of methods in clinical research aim to assess treatment-induced progress in aphasia therapy. Here, we used a crossover randomized controlled design to compare the suitability of utterance-centered and dialogue-sensitive outcome measures in speech-language testing. Fourteen individuals with post-stroke chronic non-fluent aphasia each received two types of intensive training in counterbalanced order: conventional confrontation naming, and communicative-pragmatic speech-language therapy (Intensive Language-Action Therapy, an expanded version of Constraint-Induced Aphasia Therapy)...
2017: Frontiers in Human Neuroscience
https://www.readbyqxmd.com/read/28562080/neuroimaging-patterns-along-the-als-ftd-spectrum-a-multiparametric-imaging-study
#5
Taha Omer, Eoin Finegan, Siobhan Hutchinson, Mark Doherty, Alice Vajda, Russell L McLaughlin, Niall Pender, Orla Hardiman, Peter Bede
Frontotemporal dementia is associated with considerable clinical, genetic and pathological heterogeneity. The objective of this study is to characterise the imaging signatures of the main FTD phenotypes along the ALS-FTD spectrum. A total of 100 participants underwent comprehensive multimodal neuroimaging, genetic testing and neuropsychological evaluation. Seven patients with behavioural variant FTD (bvFTD), 11 patients with non-fluent-variant primary progressive aphasia (nfvPPA), two patients with sematic-variant primary progressive aphasia(svPPA), 10 patients with amyotrophic lateral sclerosis and FTD carrying the C9orf72 hexanucleotide repeat (C9 + ALS-FTD), 10 patients with ALS-FTD without hexanucleotide repeats (C9-ALS-FTD), 20 ALS patients without behavioural or cognitive deficits (ALSnci) and 40 healthy controls (HC) were included in a prospective quantitative neuroimaging study...
May 31, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28510312/the-midbrain-to-pons-ratio-distinguishes-progressive-supranuclear-palsy-from-non-fluent-primary-progressive-aphasias
#6
M Silsby, R Y Tweedie-Cullen, C R Murray, G M Halliday, J R Hodges, J R Burrell
BACKGROUND AND PURPOSE: To determine the clinical utility of the midbrain-to-pons (M/P) ratio as a clinical biomarker of progressive supranuclear palsy (PSP) in patients with non-fluent primary progressive aphasia syndromes. METHODS: Patients with PSP, progressive non-fluent aphasia (PNFA) and logopenic progressive aphasia (LPA) were recruited. Patients were diagnosed clinically, but pathological confirmation was available in a proportion of patients. Midbrain and pons areas were measured using Osirix Lite, a free DICOM viewer...
July 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28462591/a-case-of-trem2-mutation-presenting-with-features-of-progressive-non-fluent-aphasia-and-without-bone-involvement
#7
Kok Yoon Chee, Frank Gaillard, Dennis Velakoulis, Chong Lip Ang, Loi Khim Chin, Roziana Ariffin
No abstract text is available yet for this article.
April 1, 2017: Australian and New Zealand Journal of Psychiatry
https://www.readbyqxmd.com/read/28387812/gene-based-association-studies-report-genetic-links-for-clinical-subtypes-of-frontotemporal-dementia
#8
Aniket Mishra, Raffaele Ferrari, Peter Heutink, John Hardy, Yolande Pijnenburg, Danielle Posthuma
Genome-wide association studies in frontotemporal dementia showed limited success in identifying associated loci. This is possibly due to small sample size, allelic heterogeneity, small effect sizes of single genetic variants, and the necessity to statistically correct for testing millions of genetic variants. To overcome these issues, we performed gene-based association studies on 3348 clinically identified frontotemporal dementia cases and 9390 controls (discovery, replication and joint-cohort analyses). We report association of APOE and TOMM40 with behavioural variant frontotemporal dementia, and ARHGAP35 and SERPINA1 with progressive non-fluent aphasia...
April 5, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/28387671/predicting-development-of-amyotrophic-lateral-sclerosis-in-frontotemporal-dementia
#9
Tim Van Langenhove, Olivier Piguet, James R Burrell, Cristian Leyton, David Foxe, Melissa Abela, Lauren Bartley, Woojin S Kim, Eve Jary, Yue Huang, Carol Dobson-Stone, John B Kwok, Glenda M Halliday, John R Hodges
BACKGROUND: A proportion of patients with frontotemporal dementia (FTD) also develop amyotrophic lateral sclerosis (ALS). OBJECTIVE: We aimed to establish the risk of developing ALS in patients presenting with FTD and to identify the relevant clinical variables associated with progression from FTD to FTD-ALS. METHODS: Of 218 consecutive patients with FTD, 10.1% had a dual FTD-ALS diagnosis at presentation. The remaining 152 FTD patients with follow-up of at least 12 months were included in the present study...
2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28386688/beyond-the-midbrain-atrophy-wide-spectrum-of-structural-mri-finding-in-cases-of-pathologically-proven-progressive-supranuclear-palsy
#10
REVIEW
Keita Sakurai, Aya M Tokumaru, Keigo Shimoji, Shigeo Murayama, Kazutomi Kanemaru, Satoru Morimoto, Ikuko Aiba, Motoo Nakagawa, Yoshiyuki Ozawa, Masashi Shimohira, Noriyuki Matsukawa, Yoshio Hashizume, Yuta Shibamoto
PURPOSE: Recently, it has been recognized that pathologically proven progressive supranuclear palsy (PSP) cases are classified into various clinical subtypes with non-uniform symptoms and imaging findings. This article reviews essential imaging findings, general information, and advanced magnetic resonance imaging (MRI) techniques for PSP and presents these MRI findings of pathologically proven typical and atypical PSP cases for educational purposes. METHODS: With the review of literatures, notably including atypical pathologically proven PSP cases, MRI and clinical information of 15 pathologically proven typical and atypical PSP cases were retrospectively evaluated...
May 2017: Neuroradiology
https://www.readbyqxmd.com/read/28373956/a-neural-network-underlying-intentional-emotional-facial-expression-in-neurodegenerative-disease
#11
Kelly A Gola, Tal Shany-Ur, Peter Pressman, Isa Sulman, Eduardo Galeana, Hillary Paulsen, Lauren Nguyen, Teresa Wu, Babu Adhimoolam, Pardis Poorzand, Bruce L Miller, Katherine P Rankin
Intentional facial expression of emotion is critical to healthy social interactions. Patients with neurodegenerative disease, particularly those with right temporal or prefrontal atrophy, show dramatic socioemotional impairment. This was an exploratory study examining the neural and behavioral correlates of intentional facial expression of emotion in neurodegenerative disease patients and healthy controls. One hundred and thirty three participants (45 Alzheimer's disease, 16 behavioral variant frontotemporal dementia, 8 non-fluent primary progressive aphasia, 10 progressive supranuclear palsy, 11 right-temporal frontotemporal dementia, 9 semantic variant primary progressive aphasia patients and 34 healthy controls) were video recorded while imitating static images of emotional faces and producing emotional expressions based on verbal command; the accuracy of their expression was rated by blinded raters...
2017: NeuroImage: Clinical
https://www.readbyqxmd.com/read/28326689/behavioural-and-neuropsychiatric-disturbance-in-three-clinical-subtypes-of-frontotemporal-dementia-a-clinical-research-center-for-dementia-of-south-korea-ftd-study
#12
Moon Ho Park, Eun-Joo Kim, Kyung Won Park, Jae Cheol Kwon, Bon D Ku, Seol-Heui Han, SangYun Kim, Dong Won Yang, Duk L Na, Seong Hye Choi
OBJECTIVES: To characterise the behavioural and neuropsychiatric disturbances of patients with three clinical subtypes of frontotemporal dementia (FTD): behavioural variant FTD (bvFTD), semantic dementia (SD) and progressive non-fluent aphasia (PNFA). METHODS: Consecutive series of 66 patients with bvFTD, 58 patients with SD and 21 patients with PNFA were compared using the Frontal Behavioural Inventory (FBI) and the Neuropsychiatric Inventory (NPI). RESULTS: Patients with bvFTD had more behavioural and neuropsychiatric disturbances than patients with PNFA based on the total scores of FBI and NPI...
March 2017: Australasian Journal on Ageing
https://www.readbyqxmd.com/read/28235777/observing-conversational-laughter-in-frontotemporal-dementia
#13
Peter S Pressman, Michaela Simpson, Kelly Gola, Suzanne M Shdo, Edoardo G Spinelli, Bruce L Miller, Maria Luisa Gorno-Tempini, Katherine Rankin, Robert W Levenson
BACKGROUND: We performed an observational study of laughter during seminaturalistic conversations between patients with dementia and familial caregivers. Patients were diagnosed with (1) behavioural variant frontotemporal dementia (bvFTD), (2) right temporal variant frontotemporal dementia (rtFTD), (3) semantic variant of primary progressive aphasia (svPPA), (4) non-fluent variant primary progressive aphasia (nfvPPA) or (5) early onset Alzheimer's disease (eoAD). We hypothesised that those with bvFTD would laugh less in response to their own speech than other dementia groups or controls, while those with rtFTD would laugh less regardless of who was speaking...
May 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28209520/deconstructing-empathy-neuroanatomical-dissociations-between-affect-sharing-and-prosocial-motivation-using-a-patient-lesion-model
#14
Suzanne M Shdo, Kamalini G Ranasinghe, Kelly A Gola, Clinton J Mielke, Paul V Sukhanov, Bruce L Miller, Katherine P Rankin
Affect sharing and prosocial motivation are integral parts of empathy that are conceptually and mechanistically distinct. We used a neurodegenerative disease (NDG) lesion model to examine the neural correlates of these two aspects of real-world empathic responding. The study enrolled 275 participants, including 44 healthy older controls and 231 patients diagnosed with one of five neurodegenerative diseases (75 Alzheimer's disease, 58 behavioral variant frontotemporal dementia (bvFTD), 42 semantic variant primary progressive aphasia (svPPA), 28 progressive supranuclear palsy, and 28 non-fluent variant (nfvPPA)...
February 14, 2017: Neuropsychologia
https://www.readbyqxmd.com/read/28133816/typical-and-atypical-pathology-in-primary-progressive-aphasia-variants
#15
Edoardo G Spinelli, Maria Luisa Mandelli, Zachary A Miller, Miguel A Santos-Santos, Stephen M Wilson, Federica Agosta, Lea T Grinberg, Eric J Huang, John Q Trojanowski, Marita Meyer, Maya L Henry, Giancarlo Comi, Gil Rabinovici, Howard J Rosen, Massimo Filippi, Bruce L Miller, William W Seeley, Maria Luisa Gorno-Tempini
OBJECTIVE: To characterize in vivo signatures of pathological diagnosis in a large cohort of patients with primary progressive aphasia (PPA) variants defined by current diagnostic classification. METHODS: Extensive clinical, cognitive, neuroimaging, and neuropathological data were collected from 69 patients with sporadic PPA, divided into 29 semantic (svPPA), 25 nonfluent (nfvPPA), 11 logopenic (lvPPA), and 4 mixed PPA. Patterns of gray matter (GM) and white matter (WM) atrophy at presentation were assessed and tested as predictors of pathological diagnosis using support vector machine (SVM) algorithms...
March 2017: Annals of Neurology
https://www.readbyqxmd.com/read/28120406/generalization-and-maintenance-of-treatment-gains-in-primary-progressive-aphasia-ppa-a-systematic-review
#16
REVIEW
Inês Cadório, Marisa Lousada, Paula Martins, Daniela Figueiredo
BACKGROUND: Cognitive-linguistic treatments and interventions targeting communication have been developed within the context of primary progressive aphasia (PPA), however knowledge about the scope of generalization and maintenance of therapy gains considering PPA subtypes remains scarce and awaits systematic investigation. AIMS: To analyse the effects of semantic therapy on generalization and maintenance of treatment outcomes in individuals with PPA, considering its different subtypes...
January 24, 2017: International Journal of Language & Communication Disorders
https://www.readbyqxmd.com/read/28100023/frontotemporal-lobar-degeneration-pathogenesis-pathology-and-pathways-to-phenotype
#17
REVIEW
David M A Mann, Julie S Snowden
Frontotemporal Lobar Degeneration (FTLD) is a clinically, pathologically and genetically heterogeneous group of disorders that affect principally the frontal and temporal lobes of the brain. There are three major associated clinical syndromes, behavioral variant frontotemporal dementia (bvFTD), semantic dementia (SD) and progressive non-fluent aphasia (PNFA); three principal histologies, involving tau, TDP-43 and FUS proteins; and mutations in three major genes, MAPT, GRN and C9orf72, along with several other less common gene mutations...
January 18, 2017: Brain Pathology
https://www.readbyqxmd.com/read/27929804/familial-creutzfeldt-jakob-disease-case-report-and-role-of-genetic-counseling-in-post-mortem-testing
#18
Kristin Clift, Kimberly Guthrie, Eric W Klee, Nicole Boczek, Margot Cousin, Patrick Blackburn, Paldeep Atwal
Here we present a case of an asymptomatic 53-year-old woman who sought genetic testing for Familial Creutzfeldt-Jakob Disease (fCJD) after learning that her mother had fCJD. The patient's mother had a sudden onset of memory problems and rapidly deteriorating mental faculties in her late 70s, which led to difficulties ambulating, progressive non-fluent aphasia, dysphagia and death within ∼1 y of symptom onset. The cause of death was reported as "rapid onset dementia." The patient's family, unhappy with the vague diagnosis, researched prion disorders online and aggressively pursued causation and submitted frozen brain tissue from the mother to the National Prion Disease Surveillance Center, where testing revealed a previously described 5-octapeptide repeat insertion (5-OPRI) in the prion protein gene (PRNP) that is known to cause fCJD...
November 2016: Prion
https://www.readbyqxmd.com/read/27815682/amyloid-pet-in-primary-progressive-aphasia-case-series-and-systematic-review-of-the-literature
#19
REVIEW
Alberto Villarejo-Galende, Sara Llamas-Velasco, Adolfo Gómez-Grande, Verónica Puertas-Martín, Israel Contador, Pilar Sarandeses, Marta González-Sánchez, Rocío Trincado, Patrick Pilkington, Sebastián Ruiz-Solis, David A Pérez-Martínez, Alejandro Herrero-San Martín
Primary progressive aphasia (PPA) is considered a heterogeneous syndrome, with different clinical subtypes and neuropathological causes. Novel PET biomarkers may help to predict the underlying neuropathology, but many aspects remain unclear. We studied the relationship between amyloid PET and PPA variant in a clinical series of PPA patients. A systematic review of the literature was performed. Patients with PPA were assessed over a 2-year period and classified based on language testing and the International Consensus Criteria as non-fluent/agrammatic (nfvPPA), semantic (svPPA), logopenic variant (lvPPA) or as unclassifiable (ucPPA)...
January 2017: Journal of Neurology
https://www.readbyqxmd.com/read/27684067/the-relationship-between-behavioural-changes-cognitive-symptoms-and-functional-disability-in-primary-progressive-aphasia-a-longitudinal-study
#20
Claire M O'Connor, Lindy Clemson, Emma Flanagan, Cassandra Kaizik, Henry Brodaty, John R Hodges, Olivier Piguet, Eneida Mioshi
BACKGROUND: The contribution of behavioural changes to functional decline is yet to be explored in primary progressive aphasia (PPA). OBJECTIVES: (1) investigate functional changes in two PPA variants [semantic (svPPA) and non-fluent (nfvPPA)], at baseline and after 12 months; (2) investigate baseline differences in behavioural changes between groups, and (3) explore predictors of functional decline after a 12-month period. METHODS: A longitudinal study involving 29 people with PPA (18 svPPA; 11 nfvPPA) seen annually in Sydney/Australia was conducted...
2016: Dementia and Geriatric Cognitive Disorders
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