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https://www.readbyqxmd.com/read/29451323/-18f-av-1451-tau-pet-and-primary-progressive-aphasia
#1
Keith A Josephs, Peter R Martin, Hugo Botha, Christopher G Schwarz, Joseph R Duffy, Heather M Clark, Mary M Machulda, Jonathan Graff-Radford, Stephen D Weigand, Matthew L Senjem, Rene L Utianski, Daniel A Drubach, Bradley F Boeve, David T Jones, David S Knopman, Ronald C Petersen, Clifford R Jack, Val J Lowe, Jennifer L Whitwell
OBJECTIVES: To assess [ 18 F]AV-1451 tau-PET uptake patterns across the primary progressive aphasia (PPA) variants (logopenic, semantic and agrammatic), examine regional uptake patterns of [ 18 F]AV-1451 independent of clinical diagnosis, and compare the diagnostic utility of [ 18 F]AV-1451, [ 18 F]-fluorodeoxygluclose (FDG)-PET and MRI to differentiate the PPA variants. METHODS: We performed statistical parametric mapping of [ 18 F]AV-1451 across 40 PPA patients (logopenic-PPA=14, semantic-PPA=13 and agrammatic-PPA=13) compared to 80 cognitively normal, PiB-negative controls, age and gender matched 2:1...
February 16, 2018: Annals of Neurology
https://www.readbyqxmd.com/read/29444966/cerebral-microbleeds-and-csf-alzheimer-biomarkers-in-primary-progressive-aphasias
#2
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...
February 14, 2018: Neurology
https://www.readbyqxmd.com/read/29424041/neuropsychological-differentiation-of-progressive-aphasic-disorders
#3
Jennifer M Harris, Jennifer A Saxon, Matthew Jones, Julie S Snowden, Jennifer C Thompson
The differentiation of subtypes of primary progressive aphasia (PPA) remains challenging. We aimed to identify optimum neuropsychological measures for characterizing PPA, to examine the relationship between behavioural change and subtypes of PPA and to determine whether characteristic profiles of language, working memory, and behavioural changes occur in PPA. Forty-seven patients with PPA and multi-domain Alzheimer's disease (AD) together with 19 age-matched controls underwent a large battery of working memory and language tests...
February 8, 2018: Journal of Neuropsychology
https://www.readbyqxmd.com/read/29409157/sentence-composition-ability-in-two-patients-with-non-fluent-agrammatic-variant-primary-progressive-aphasia
#4
Hiroyuki Watanabe, Minoru Matsuda, Shoko Ota, Toru Baba, Osamu Iizuka, Etsuro Mori
Agrammatism is one of the core clinical features of non-fluent/agrammatic variant primary progressive aphasia, and it has traditionally been considered the hallmark of non-fluent aphasia in Western countries. However, agrammatic speech may remain undetected in Japanese patients because of the agglutinative structure of the language and high flexibility in word order. In the present study, we aimed to analyze agrammatism in the speech production of Japanese patients with aphasia due to neurodegenerative disease using an anagram test generated by our laboratory...
February 6, 2018: Psychogeriatrics: the Official Journal of the Japanese Psychogeriatric Society
https://www.readbyqxmd.com/read/29309493/rates-of-amyloid-imaging-positivity-in-patients-with-primary-progressive-aphasia
#5
Miguel A Santos-Santos, Gil D Rabinovici, Leonardo Iaccarino, Nagehan Ayakta, Gautam Tammewar, Iryna Lobach, Maya L Henry, Isabel Hubbard, Maria Luisa Mandelli, Edoardo Spinelli, Zachary A Miller, Peter S Pressman, James P O'Neil, Pia Ghosh, Andreas Lazaris, Marita Meyer, Christa Watson, Soo Jin Yoon, Howard J Rosen, Lea Grinberg, William W Seeley, Bruce L Miller, William J Jagust, Maria Luisa Gorno-Tempini
Importance: The ability to predict the pathology underlying different neurodegenerative syndromes is of critical importance owing to the advent of molecule-specific therapies. Objective: To determine the rates of positron emission tomography (PET) amyloid positivity in the main clinical variants of primary progressive aphasia (PPA). Design, Setting, and Participants: This prospective clinical-pathologic case series was conducted at a tertiary research clinic specialized in cognitive disorders...
January 8, 2018: JAMA Neurology
https://www.readbyqxmd.com/read/29228180/longitudinal-structural-and-molecular-neuroimaging-in-agrammatic-primary-progressive-aphasia
#6
Katerina A Tetzloff, Joseph R Duffy, Heather M Clark, Edythe A Strand, Mary M Machulda, Christopher G Schwarz, Matthew L Senjem, Robert I Reid, Anthony J Spychalla, Nirubol Tosakulwong, Val J Lowe, Clifford R Jack, Keith A Josephs, Jennifer L Whitwell
The agrammatic variant of primary progressive aphasia affects normal grammatical language production, often occurs with apraxia of speech, and is associated with left frontal abnormalities on cross-sectional neuroimaging studies. We aimed to perform a detailed assessment of longitudinal change on structural and molecular neuroimaging to provide a complete picture of neurodegeneration in these patients, and to determine how patterns of progression compare to patients with isolated apraxia of speech (primary progressive apraxia of speech)...
December 8, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/28990425/mendelian-forms-of-disease-and-age-at-onset-affect-survival-in-frontotemporal-dementia
#7
Maura Cosseddu, Alberto Benussi, Stefano Gazzina, Rosanna Turrone, Silvana Archetti, Elisa Bonomi, Giorgio Biasiotto, Isabella Zanella, Raffaele Ferrari, Maria S Cotelli, Antonella Alberici, Alessandro Padovani, Barbara Borroni
OBJECTIVE: Frontotemporal dementia (FTD) is a common cause of young onset dementia. Very few reports on disease duration are currently available and predictors of survival are still undefined. The aim of the present study was to assess the natural history of FTD and to define predictors of survival. METHODS: Four hundred amd eleven FTD patients, including 294 with behavioural variant FTD, 77 with agrammatic variant primary progressive aphasia (PPA) and 40 with semantic variant PPA, were consecutively enrolled and demographic and clinical variables carefully recorded...
October 8, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28969381/distinct-spatiotemporal-patterns-of-neuronal-functional-connectivity-in-primary-progressive-aphasia-variants
#8
Kamalini G Ranasinghe, Leighton B Hinkley, Alexander J Beagle, Danielle Mizuiri, Susanne M Honma, Ariane E Welch, Isabel Hubbard, Maria Luisa Mandelli, Zachary A Miller, Coleman Garrett, Alice La, Adam L Boxer, John F Houde, Bruce L Miller, Keith A Vossel, Maria Luisa Gorno-Tempini, Srikantan S Nagarajan
Primary progressive aphasia is a syndrome characterized by progressive loss of language abilities with three main phenotypic clinical presentations, including logopenic, non-fluent/agrammatic, and semantic variants. Previous imaging studies have shown unique anatomic impacts within language networks in each variant. However, direct measures of spontaneous neuronal activity and functional integrity of these impacted neural networks in primary progressive aphasia are lacking. The aim of this study was to characterize the spatial and temporal patterns of resting state neuronal synchronizations in primary progressive aphasia syndromes...
October 1, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/28843341/artificial-grammar-learning-in-vascular-and-progressive-non-fluent-aphasias
#9
Thomas E Cope, Benjamin Wilson, Holly Robson, Rebecca Drinkall, Lauren Dean, Manon Grube, P Simon Jones, Karalyn Patterson, Timothy D Griffiths, James B Rowe, Christopher I Petkov
Patients with non-fluent aphasias display impairments of expressive and receptive grammar. This has been attributed to deficits in processing configurational and hierarchical sequencing relationships. This hypothesis had not been formally tested. It was also controversial whether impairments are specific to language, or reflect domain general deficits in processing structured auditory sequences. Here we used an artificial grammar learning paradigm to compare the abilities of controls to participants with agrammatic aphasia of two different aetiologies: stroke and frontotemporal dementia...
September 2017: Neuropsychologia
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
#10
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/28803444/atypical-parkinsonian-syndromes-a-general-neurologist-s-perspective
#11
REVIEW
A B Deutschländer, O A Ross, D W Dickson, Z K Wszolek
The differential diagnosis of atypical parkinsonian syndromes is challenging. These severe and often rapidly progressive neurodegenerative disorders are clinically heterogeneous and show significant phenotypic overlap. Here, clinical, imaging, neuropathological and genetic features of multiple system atrophy, progressive supranuclear palsy, corticobasal degeneration and frontotemporal lobar degeneration (FTLD) are reviewed. The terms corticobasal degeneration and FTLD refer to pathologically confirmed cases of corticobasal syndrome and frontotemporal dementia (FTD)...
January 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28624827/qualitative-assessment-of-verbal-fluency-performance-in-frontotemporal-dementia
#12
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/28596248/examining-the-language-and-behavioural-profile-in-ftd-and-als-ftd
#13
COMPARATIVE STUDY
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...
August 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
#14
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
#15
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
#16
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/28532703/reading-difficulties-in-primary-progressive-aphasia-in-a-regular-language-speaking-cohort-of-patients
#17
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...
July 1, 2017: Neuropsychologia
https://www.readbyqxmd.com/read/28410663/frontotemporal-dementia
#18
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
#19
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
#20
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
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