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Progressive non-fluent aphasia

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https://www.readbyqxmd.com/read/28209520/deconstructing-empathy-neuroanatomical-dissociations-between-affect-sharing-and-prosocial-motivation-using-a-patient-lesion-model
#1
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 more directly 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 13, 2017: Neuropsychologia
https://www.readbyqxmd.com/read/28133816/typical-and-atypical-pathology-in-primary-progressive-aphasia-variants
#2
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 non-fluent (nfvPPA), 11 logopenic (lvPPA), and 4 mixed PPA. Patterns of grey matter (GM) and white matter (WM) atrophy at presentation were assessed and tested as predictors of pathological diagnosis using support vector machine (SVM) algorithms...
January 30, 2017: Annals of Neurology
https://www.readbyqxmd.com/read/28120406/generalization-and-maintenance-of-treatment-gains-in-primary-progressive-aphasia-ppa-a-systematic-review
#3
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
#4
REVIEW
David Ma 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, behavioural 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
#5
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
#6
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)...
November 4, 2016: 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
#7
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
https://www.readbyqxmd.com/read/27662315/the-role-of-single-subject-brain-metabolic-patterns-in-the-early-differential-diagnosis-of-primary-progressive-aphasias-and-in-prediction-of-progression-to-dementia
#8
Chiara Cerami, Alessandra Dodich, Lucia Greco, Sandro Iannaccone, Giuseppe Magnani, Alessandra Marcone, Elisabetta Pelagallo, Roberto Santangelo, Stefano F Cappa, Daniela Perani
BACKGROUND AND OBJECTIVE: Primary progressive aphasia (PPA) is a clinical syndrome due to different neurodegenerative conditions in which an accurate early diagnosis needs to be supported by a reliable diagnostic tool at the individual level. In this study, we investigated in PPA the FDG-PET brain metabolic patterns at the single-subject level, in order to assess the case-to-case variability and its relationship with clinical-neuropsychological findings. MATERIAL AND METHODS: 55 patients (i...
2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/27589533/primary-progressive-aphasia-in-the-network-of-french-alzheimer-plan-memory-centers
#9
Eloi Magnin, Jean-François Démonet, David Wallon, Julien Dumurgier, Anne-Cécile Troussière, Alain Jager, Emmanuelle Duron, Audrey Gabelle, Vincent de la Sayette, Lisette Volpe-Gillot, Gregory Tio, Sarah Evain, Claire Boutoleau-Bretonnière, Adeline Enderle, François Mouton-Liger, Philippe Robert, Didier Hannequin, Florence Pasquier, Jacques Hugon, Claire Paquet
BACKGROUND: Few demographical data about primary progressive aphasia (PPA) are available, and most knowledge regarding PPA is based on tertiary centers' results. OBJECTIVE: Our aims were to describe demographical characteristics of the PPA population in a large sample of PPA patients from the network of French Alzheimer plan memory centers (Sample 1), and to describe the stratification of cerebrospinal fluid (CSF) biomarkers in two different samples of PPA patients (Samples 2 and 3)...
October 18, 2016: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/27567822/non-fluent-variant-of-primary-progressive-aphasia-due-to-the-novel-grn-g-9543dela-ivs3-2dela-mutation
#10
Sara M G Cioffi, Daniela Galimberti, Federica Barocco, Marco Spallazzi, Chiara Fenoglio, Maria Serpente, Marina Arcaro, Simona Gardini, Elio Scarpini, Paolo Caffarra
Mutations in progranulin gene (GRN) are a common cause of autosomal dominant frontotemporal lobar degeneration syndromes and are associated with a wide phenotypic heterogeneity. The majority of genetic defects in GRN consists of loss-of-function mutations, causing haploinsufficiency, and is associated with extremely low plasma progranulin levels. Herein, we describe a patient who developed language dysfunctions and memory disturbances at 63 years of age. Considering the early onset and the positive family history (sister aged 50 with non-fluent/agrammatic variant of primary progressive aphasia, father with behavioral disturbances in his sixties), a genetic analysis was carried out, showing the presence of a novel mutation [g...
September 6, 2016: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/27524196/-profiles-of-cognitive-and-language-impairment-of-logopenic-and-non-fluent-variant-of-primary-progressive-aphasia
#11
D Li, L N Zhao, H M Jin, M Zhang, D M Guo, Y Y Yu, L Y Wu, Y Tang, F Y Li, A H Zhou, Y Han, J P Jia
OBJECTIVE: To decipher the cognitive and linguistic feature of logopenic variant primary progressive aphasia (lv-PPA) and nonfluent variant primary progressive aphasia (nfv-PPA) and to explore the extent to which cognitive and language impairment contribute to the dysfunction of activity of daily living(ADL). METHODS: Seven lv-PPA and five nfv-PPA were enrolled in memory clinic of Xuanwu Hospital, Capital Medical University from January 2015 to January 2016 accordig to the international consensus criteria for PPA and its three subtypes...
August 2, 2016: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/27522537/transcranial-direct-current-stimulation-for-the-treatment-of-primary-progressive-aphasia-an-open-label-pilot-study
#12
Felix Gervits, Sharon Ash, H Branch Coslett, Katya Rascovsky, Murray Grossman, Roy Hamilton
Primary progressive aphasia (PPA) is a neurodegenerative condition characterized by gradual deterioration of language function. We investigated whether two weeks of daily transcranial direct current stimulation (tDCS) treatment would improve language abilities in six people with a non-fluent form of PPA. tDCS was applied in an unblinded trial at an intensity of 1.5mA for 20min/day over 10days. At the time of stimulation, patients were engaged in narrating one of several children's wordless picture stories. A battery of neuropsychological assessments was administered four times: at baseline, immediately following the 2-week stimulation period, and then 6-weeks and 12-weeks following the end of stimulation...
November 2016: Brain and Language
https://www.readbyqxmd.com/read/27497488/healthy-brain-connectivity-predicts-atrophy-progression-in-non-fluent-variant-of-primary-progressive-aphasia
#13
Maria Luisa Mandelli, Eduard Vilaplana, Jesse A Brown, H Isabel Hubbard, Richard J Binney, Suneth Attygalle, Miguel A Santos-Santos, Zachary A Miller, Mikhail Pakvasa, Maya L Henry, Howard J Rosen, Roland G Henry, Gil D Rabinovici, Bruce L Miller, William W Seeley, Maria Luisa Gorno-Tempini
Neurodegeneration has been hypothesized to follow predetermined large-scale networks through the trans-synaptic spread of toxic proteins from a syndrome-specific epicentre. To date, no longitudinal neuroimaging study has tested this hypothesis in vivo in frontotemporal dementia spectrum disorders. The aim of this study was to demonstrate that longitudinal progression of atrophy in non-fluent/agrammatic variant primary progressive aphasia spreads over time from a syndrome-specific epicentre to additional regions, based on their connectivity to the epicentre in healthy control subjects...
October 2016: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/27350781/co-occurrence-of-language-and-behavioural-change-in-frontotemporal-lobar-degeneration
#14
Jennifer M Harris, Matthew Jones, Claire Gall, Anna M T Richardson, David Neary, Daniel du Plessis, Piyali Pal, David M A Mann, Julie S Snowden, Jennifer C Thompson
BACKGROUND/OBJECTIVES: We aimed to evaluate the co-occurrence of language and behavioural impairment in patients with frontotemporal lobar degeneration (FTLD) spectrum pathology. METHODS: Eighty-one dementia patients with pathological confirmation of FTLD were identified. Anonymized clinical records from patients' first assessment were rated for language and behavioural features from frontotemporal dementia consensus criteria, primary progressive aphasia (PPA) criteria and 1998 FTLD criteria...
May 2016: Dementia and Geriatric Cognitive Disorders Extra
https://www.readbyqxmd.com/read/27341394/wh-questions-and-passive-sentences-in-non-fluent-variant-ppa-and-semantic-variant-ppa-longitudinal-findings-of-an-anagram-production-task
#15
Jennifer Cupit, Naida L Graham, Carol Leonard, David Tang-Wai, Sandra E Black, Elizabeth Rochon
Using an anagram task, we investigated longitudinal syntactic production by individuals with semantic variant primary progressive aphasia (svPPA) and non-fluent variant PPA (nfvPPA), compared to controls. The accuracy of the production of active and passive, and reversible and non-reversible sentences, as well as of wh- questions was compared across the three groups. Results showed a different pattern of syntactic impairment across the two patient groups. The nfvPPA group showed difficulty with wh- questions at Time 1 and impairment with the passive structure approximately one year later, at Time 2...
July 2016: Cognitive Neuropsychology
https://www.readbyqxmd.com/read/27186717/neurochemical-biomarkers-in-the-diagnosis-of-frontotemporal-lobar-degeneration-an-update
#16
REVIEW
Patrick Oeckl, Petra Steinacker, Emily Feneberg, Markus Otto
Frontotemporal lobar degeneration (FTLD) is a spectrum of rare neurodegenerative diseases with overlapping symptoms and neuropathology. It includes the behavioral variant of frontotemporal dementia (bvFTD), the semantic and non-fluent variant of primary progressive aphasia (svPPA and nfvPPA), FTD with motor neuron disease (FTD-MND), progressive supranuclear palsy, and corticobasal syndrome. The diagnosis of the FTLD spectrum of diseases is based on clinical symptoms which hampers the differentiation of the diseases among each other and with other disorders that show a similar clinical appearance resulting in a high rate of misdiagnoses...
August 2016: Journal of Neurochemistry
https://www.readbyqxmd.com/read/27060523/core-auditory-processing-deficits-in-primary-progressive-aphasia
#17
Manon Grube, Rose Bruffaerts, Jolien Schaeverbeke, Veerle Neyens, An-Sofie De Weer, Alexandra Seghers, Bruno Bergmans, Eva Dries, Timothy D Griffiths, Rik Vandenberghe
The extent to which non-linguistic auditory processing deficits may contribute to the phenomenology of primary progressive aphasia is not established. Using non-linguistic stimuli devoid of meaning we assessed three key domains of auditory processing (pitch, timing and timbre) in a consecutive series of 18 patients with primary progressive aphasia (eight with semantic variant, six with non-fluent/agrammatic variant, and four with logopenic variant), as well as 28 age-matched healthy controls. We further examined whether performance on the psychoacoustic tasks in the three domains related to the patients' speech and language and neuropsychological profile...
June 2016: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/27025087/-new-entities-and-the-criteria-of-ftld
#18
Mika Otsuki
This article provides an overview of the historical entity hitherto, the pivotal clinical symptoms of FTLD (frontotemporal lobar degeneration), and an introduction for the revised criteria for bvFTD (behavioral variant frontotemporal dementia): FTDC (International Behavioral Variant FTD Criteria Consortium) presented by Rascovsky et al(2011), and the classification criteria for PPA(primary progressive aphasia) heralded by Gorno-Tempini et al (2011). According to the former criteria, bvFTD can be diagnosed by the clinical symptoms as having possible bvFTD or probable bvFTD, and the pathological findings could lead definite bvFTD...
March 2016: Nihon Rinsho. Japanese Journal of Clinical Medicine
https://www.readbyqxmd.com/read/26882509/the-effects-of-behavioral-and-psychological-symptoms-on-caregiver-burden-in-frontotemporal-dementia-lewy-body-dementia-and-alzheimer-s-disease-clinical-experience-in-china
#19
Shuling Liu, Yi Jin, Zhihong Shi, Ya Ruth Huo, Yalin Guan, Mengyuan Liu, Shuai Liu, Yong Ji
BACKGROUND AND AIMS: Caregivers of individuals with neurodegenerative diseases, including frontotemporal dementia (FTD), Lewy body dementia (DLB), and Alzheimer's disease (AD), experience high levels of psychological and physical stress, likely due to behavioral and psychological symptoms of dementia (BPSD). This study is the first to simultaneously evaluate the effects of BPSD on caregiver burden in these three types of dementia. METHOD: A total of 214 dementia patients, including probable FTD (n = 82), DLB (n = 22), and AD (n = 110), as well as their primary caregivers, were assessed using psychological inventories and cognitive evaluation...
February 16, 2016: Aging & Mental Health
https://www.readbyqxmd.com/read/26854827/survival-in-frontotemporal-dementia-phenotypes-a-meta-analysis
#20
REVIEW
Kalyani Kansal, Manisha Mareddy, Kelly L Sloane, Alexa A Minc, Peter V Rabins, John B McGready, Chiadi U Onyike
BACKGROUND: Survival in frontotemporal dementia (FTD) is not well understood. We conducted a mixed effects meta-analysis of survival in FTD to examine phenotype differences and contributory factors. METHODS: The PubMed, Medline, EMBASE, CINAHL, PsycINFO and Cochrane databases were searched for studies describing survival or natural history of behavioral variant FTD (bvFTD), progressive non-fluent aphasia (PNFA), semantic dementia (SD), FTD with amyotrophic lateral sclerosis (FTD-ALS), progressive supranuclear palsy and corticobasal degeneration...
2016: Dementia and Geriatric Cognitive Disorders
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