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https://www.readbyqxmd.com/read/28624827/qualitative-assessment-of-verbal-fluency-performance-in-frontotemporal-dementia
#1
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...
June 17, 2017: Dementia and Geriatric Cognitive Disorders
https://www.readbyqxmd.com/read/28596248/examining-the-language-and-behavioural-profile-in-ftd-and-als-ftd
#2
Jennifer A Saxon, Jennifer C Thompson, Matthew Jones, Jennifer M Harris, Anna Mt Richardson, Tobias Langheinrich, David Neary, David Ma Mann, Julie S Snowden
BACKGROUND: A proportion of patients with behavioural variant frontotemporal dementia (bvFTD) develop amyotrophic lateral sclerosis (ALS). It is currently unknown whether the behavioural and cognitive syndrome in bvFTD with ALS (ALS-FTD) is indistinguishable from that of bvFTD alone. METHODS: A retrospective cohort of 241 patients with clinical diagnoses of bvFTD (n=185) or ALS-FTD (n=56) was examined with respect to behavioural, cognitive and neuropsychiatric symptoms...
June 8, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
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/28579383/clinical-and-biological-phenotypes-of-frontotemporal-dementia-perspectives-for-disease-modifying-therapies
#4
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/28549937/is-learning-episodic-memory-distinct-cognitive-and-neuroanatomic-correlates-of-immediate-recall-during-learning-trials-in-neurologically-normal-aging-and-neurodegenerative-cohorts
#5
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...
May 23, 2017: Neuropsychologia
https://www.readbyqxmd.com/read/28532703/reading-difficulties-in-primary-progressive-aphasia-in-a-regular-language-speaking-cohort-of-patients
#6
Jordi A Matías-Guiu, Fernando Cuetos, María Nieves Cabrera-Martín, María Valles-Salgado, Teresa Moreno-Ramos, José Luis Carreras, Jorge Matías-Guiu
Reading impairment is an important feature in Primary Progressive Aphasia (PPA). The Spanish orthography entails completely regular spelling to sound correspondences, so reading disorders may be different to English. In the current study, reading, phonological and semantic abilities of 35 patients with the three variants of PPA, and 13 healthy volunteers were assessed. Brain metabolism was concomitantly obtained from each participant using (18)F-fluorodeoxyglucose positron emission tomography imaging. Two main patterns of impairment were identified: difficulties in nonwords reading with preservation of exception words in agrammatic and logopenic aphasia, and the inverse pattern in semantic dementia...
May 19, 2017: Neuropsychologia
https://www.readbyqxmd.com/read/28410663/frontotemporal-dementia
#7
REVIEW
Nicholas T Olney, Salvatore Spina, Bruce L Miller
Frontotemporal dementia (FTD) is a heterogeneous disorder with distinct clinical phenotypes associated with multiple neuropathologic entities. Presently, the term FTD encompasses clinical disorders that include changes in behavior, language, executive control, and often motor symptoms. The core FTD spectrum disorders include behavioral variant FTD, nonfluent/agrammatic variant primary progressive aphasia, and semantic variant PPA. Related FTD disorders include frontotemporal dementia with motor neuron disease, progressive supranuclear palsy syndrome, and corticobasal syndrome...
May 2017: Neurologic Clinics
https://www.readbyqxmd.com/read/28355946/dysexecutive-symptoms-in-primary-progressive-aphasia-beyond-diagnostic-criteria
#8
Joël Macoir, Monica Lavoie, Robert Laforce, Simona M Brambati, Maximiliano A Wilson
Primary progressive aphasia (PPA) is a heterogeneous neurodegenerative condition in which the most prominent clinical feature is language difficulties. Other cognitive domains have been described to remain unaffected at the early stages of the disease and, therefore, excluded from diagnostic criteria. However, we show in this article that executive function (EF) disorders may be present in the 3 variants (nonfluent/agrammatic, logopenic, and semantic) of PPA. We also illustrate changes in language and EF by means of a 3-year behavioral and neuroimaging longitudinal study of a patient suffering from the semantic variant of PPA...
May 2017: Journal of Geriatric Psychiatry and Neurology
https://www.readbyqxmd.com/read/28304311/frontotemporal-dementia-due-to-the-novel-grn-arg161glyfsx36-mutation
#9
Stefano Gazzina, Silvana Archetti, Antonella Alberici, Elisa Bonomi, Maura Cosseddu, Diego Di Lorenzo, Alessandro Padovani, Barbara Borroni
Progranulin is a multifunctional growth factor mainly expressed in neurons and microglia. Loss-of-function mutations in the Granulin (GRN) gene are causative of frontotemporal dementia with TAR DNA-binding protein-43 inclusions. We reported the case of a 51-year-old male patient affected by sporadic agrammatic variant of primary progressive aphasia, in whom we identified a novel heterozygous deletion in the exon 6 (g.10338_39delAG, p.Arg161GlyfsX36). Plasma progranulin levels were significantly reduced and in silico analysis predicted a premature termination codon...
2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28288010/functional-connectivity-is-reduced-in-early-stage-primary-progressive-aphasia-when-atrophy-is-not-prominent
#10
Borna Bonakdarpour, Emily J Rogalski, Allan Wang, Jaiashre Sridhar, M M Mesulam, Robert S Hurley
Primary progressive aphasia (PPA) is a clinical syndrome of language decline caused by neurodegenerative pathology. Although language impairments in PPA are typically localized via the morphometric assessment of atrophy, functional changes may accompany or even precede detectable structural alterations, in which case resting state functional connectivity (RSFC) could provide an alternative approach. The goal of this study was to determine whether language network RSFC is reduced in early-stage PPA when atrophy is not prominent...
April 2017: Alzheimer Disease and Associated Disorders
https://www.readbyqxmd.com/read/28229040/predicting-primary-progressive-aphasias-with-support-vector-machine-approaches-in-structural-mri-data
#11
Sandrine Bisenius, Karsten Mueller, Janine Diehl-Schmid, Klaus Fassbender, Timo Grimmer, Frank Jessen, Jan Kassubek, Johannes Kornhuber, Bernhard Landwehrmeyer, Albert Ludolph, Anja Schneider, Sarah Anderl-Straub, Katharina Stuke, Adrian Danek, Markus Otto, Matthias L Schroeter
Primary progressive aphasia (PPA) encompasses the three subtypes nonfluent/agrammatic variant PPA, semantic variant PPA, and the logopenic variant PPA, which are characterized by distinct patterns of language difficulties and regional brain atrophy. To validate the potential of structural magnetic resonance imaging data for early individual diagnosis, we used support vector machine classification on grey matter density maps obtained by voxel-based morphometry analysis to discriminate PPA subtypes (44 patients: 16 nonfluent/agrammatic variant PPA, 17 semantic variant PPA, 11 logopenic variant PPA) from 20 healthy controls (matched for sample size, age, and gender) in the cohort of the multi-center study of the German consortium for frontotemporal lobar degeneration...
2017: NeuroImage: Clinical
https://www.readbyqxmd.com/read/28187331/temporal-acoustic-measures-distinguish-primary-progressive-apraxia-of-speech-from-primary-progressive-aphasia
#12
Joseph R Duffy, Holly Hanley, Rene Utianski, Heather Clark, Edythe Strand, Keith A Josephs, Jennifer L Whitwell
The purpose of this study was to determine if acoustic measures of duration and syllable rate during word and sentence repetition, and a measure of within-word lexical stress, distinguish speakers with primary progressive apraxia of speech (PPAOS) from nonapraxic speakers with the agrammatic or logopenic variants of primary progressive aphasia (PPA), and control speakers. Results revealed that the PPAOS group had longer durations and reduced rate of syllable production for most words and sentences, and the measure of lexical stress...
May 2017: Brain and Language
https://www.readbyqxmd.com/read/28179468/neurofilament-as-a-blood-marker-for-diagnosis-and-monitoring-of-primary-progressive-aphasias
#13
Petra Steinacker, Elisa Semler, Sarah Anderl-Straub, Janine Diehl-Schmid, Matthias L Schroeter, Ingo Uttner, Hans Foerstl, Bernhard Landwehrmeyer, Christine A F von Arnim, Jan Kassubek, Patrick Oeckl, Hans-Jürgen Huppertz, Klaus Fassbender, Klaus Fliessbach, Johannes Prudlo, Carola Roßmeier, Johannes Kornhuber, Anja Schneider, Alexander E Volk, Martin Lauer, Adrian Danek, Albert C Ludolph, Markus Otto
OBJECTIVE: To assess the utility of serum neurofilament for diagnosis and monitoring of primary progressive aphasia (PPA) variants. METHODS: We investigated neurofilament light chain (NF-L) levels in blood of 99 patients with PPA (40 with nonfluent variant PPA [nfvPPA], 38 with semantic variant PPA [svPPA], 21 with logopenic variant PPA [lvPPA]) and compared diagnostic performance with that reached by CSF NF-L, phosphorylated neurofilament heavy chain (pNF-H), β-amyloid (Aβ1-42), tau, and phosphorylated tau...
March 7, 2017: Neurology
https://www.readbyqxmd.com/read/28153380/grn-deletion-in-familial-frontotemporal-dementia-showing-association-with-clinical-variability-in-3-familial-cases
#14
Graziella Milan, Sabrina Napoletano, Sabina Pappatà, Maria Teresa Gentile, Luca Colucci-D'Amato, Gennaro Della Rocca, Anna Maciag, Carmen Palermo Rossetti, Laura Fucci, Annibale Puca, Dario Grossi, Alfredo Postiglione, Emilia Vitale
Progranulin (GRN) gene mutations have been genetically associated with frontotemporal dementia (FTD) and are present in about 23% of patients with familial FTD. However, the neurobiology of this secreted glycoprotein remains unclear. Here, we report the identification of 3 pedigrees of Southern Italian extraction in whom FTD segregates with autosomal dominant inheritance patterns. We present evidence that all the available patients in these 3 familial cases are carrying the rare GRN gene exon 6 deletion g10325_10331delCTGCTGT (relative to nt 1 inNG_007886...
May 2017: Neurobiology of Aging
https://www.readbyqxmd.com/read/28103593/executive-dysfunction-and-behavioral-symptoms-are-associated-with-deficits-in-instrumental-activities-of-daily-living-in-frontotemporal-dementia
#15
Negar Moheb, Mario F Mendez, Sarah A Kremen, Edmond Teng
BACKGROUND: Deficits in instrumental activities of daily living (ADLs) may be more prominent in behavioral variant frontotemporal dementia (bvFTD) than in nonfluent/agrammatic variant primary progressive aphasia (nfvPPA) or semantic variant primary progressive aphasia (svPPA). It is uncertain whether frontotemporal dementia (FTD) subgroups exhibit different patterns and/or predictors of functional impairment. METHODS: We examined data from participants diagnosed with bvFTD (n = 607), svPPA (n = 132), and nfvPPA (n = 155) who were included in the National Alzheimer's Coordinating Center (NACC) Uniform Data Set (UDS) and assessed with the Functional Activities Questionnaire (FAQ)...
2017: Dementia and Geriatric Cognitive Disorders
https://www.readbyqxmd.com/read/27858708/cerebrospinal-fluid-biomarkers-as-a-diagnostic-tool-of-the-underlying-pathology-of-primary-progressive-aphasia
#16
George P Paraskevas, Dimitrios Kasselimis, Evie Kourtidou, Vasilios Constantinides, Anastasia Bougea, Costas Potagas, Ioannis Evdokimidis, Elisabeth Kapaki
BACKGROUND: Primary progressive aphasia (PPA) may present with three main clinical variants, namely nonfluent agrammatic (nfaPPA), semantic (sPPA), and logopenic (lPPA) subtypes. Frontotemporal lobar degenerations (FTLD) or Alzheimer's disease (AD) are the most common etiologies. OBJECTIVE: To study the potential of cerebrospinal fluid (CSF) biomarkers for identifying the underlying pathology in patients with PPA. METHODS: CSF levels of total tau protein (τT), amyloid-β peptide (Aβ42), and tau phosphorylated at threonine-181 (τP - 181) were measured by double sandwich, enzyme-linked immunosorbent assay (ELISA) in 43 patients with PPA, 26 patients with AD, and 17 healthy controls...
2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/27815682/amyloid-pet-in-primary-progressive-aphasia-case-series-and-systematic-review-of-the-literature
#17
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/27790240/lack-of-frank-agrammatism-in-the-nonfluent-agrammatic-variant-of-primary-progressive-aphasia
#18
Naida L Graham, Carol Leonard, David F Tang-Wai, Sandra Black, Tiffany W Chow, Chris J M Scott, Alicia A McNeely, Mario Masellis, Elizabeth Rochon
BACKGROUND/AIMS: Frank agrammatism, defined as the omission and/or substitution of grammatical morphemes with associated grammatical errors, is variably reported in patients with nonfluent variant primary progressive aphasia (nfPPA). This study addressed whether frank agrammatism is typical in agrammatic nfPPA patients when this feature is not required for diagnosis. METHOD: We assessed grammatical production in 9 patients who satisfied current diagnostic criteria...
September 2016: Dementia and Geriatric Cognitive Disorders Extra
https://www.readbyqxmd.com/read/27589533/primary-progressive-aphasia-in-the-network-of-french-alzheimer-plan-memory-centers
#19
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
#20
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
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