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"Primary progressive aphasia"

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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/29438114/validity-and-reliability-of-the-frontotemporal-dementia-rating-scale-ftd-frs-for-the-progression-and-staging-of-dementia-in-brazilian-patients
#3
Thaís B Lima-Silva, Valéria S Bahia, Mário A Cecchini, Luciana Cassimiro, Henrique C Guimarães, Leandro B Gambogi, Paulo Caramelli, Márcio Balthazar, Benito Damasceno, Sônia M D Brucki, Leonardo C de Souza, Ricardo Nitrini, Eneida Mioshi, Mônica S Yassuda
INTRODUCTION: Few studies on instruments for staging frontotemporal dementia (FTD) have been conducted. OBJECTIVE: The objective of this study was to analyze the factor structure, internal consistency, reliability, and convergent validity of the Brazilian version of the Frontotemporal Dementia Rating Scale (FTD-FRS). METHODS: A total of 97 individuals aged 40 years and above with >2 years' education took part in the study, 31 patients diagnosed with behavioral variant FTD (bvFTD), 8 patients with primary progressive aphasia, 28 with Alzheimer disease, 8 with mild cognitive impairment, and a control group of 22 healthy subjects...
February 13, 2018: Alzheimer Disease and Associated Disorders
https://www.readbyqxmd.com/read/29429985/posterior-associative-and-cingulate-cortex-involvement-of-brain-single-photon-emission-computed-tomography-spect-imaging-in-semantic-dementia-with-probable-alzheimer-disease-pathology-a-case-report
#4
Yumi Takano, Keiko Kunitoki, Yasuko Tatewaki, Tatsushi Mutoh, Tomoko Totsune, Hideo Shimomura, Manabu Nakagawa, Hiroyuki Arai, Yasuyuki Taki
BACKGROUND Semantic dementia (SD) is a type of primary progressive aphasia with prominent language dysfunction, mostly within the spectrum of frontotemporal lobar degeneration (FTLD). Although there is an overlap in clinical manifestations of SD attributable to FTLD and neuropathologically proven Alzheimer disease (AD), clinical diagnostic clues are not readily available. We present a characteristic finding based on a single-photon emission computed tomography (SPECT)-based regional cerebral blood flow study and its statistical imaging analysis for a rare case of SD with AD-like pathology...
February 12, 2018: American Journal of Case Reports
https://www.readbyqxmd.com/read/29424041/neuropsychological-differentiation-of-progressive-aphasic-disorders
#5
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/29423814/connectivity-based-characterisation-of-subcortical-grey-matter-pathology-in-frontotemporal-dementia-and-als-a-multimodal-neuroimaging-study
#6
Peter Bede, Taha Omer, Eoin Finegan, Rangariroyashe H Chipika, Parameswaran M Iyer, Mark A Doherty, Alice Vajda, Niall Pender, Russell L McLaughlin, Siobhan Hutchinson, Orla Hardiman
Frontotemporal dementia (FTD) phenotypes have distinctive and well-established cortical signatures, but their subcortical grey matter profiles are poorly characterised. The comprehensive characterisation of striatal and thalamic pathology along the ALS-FTD spectrum is particularly timely, as dysfunction of frontostriatal and cortico-thalamic networks contribute to phenotype-defining cognitive, behavioral, and motor deficits. Ten patients with behavioral-variant FTD, 11 patients with non-fluent-variant primary progressive aphasia, 5 patients with semantic-variant primary progressive aphasia, 14 ALS-FTD patients with C9orf72 hexanucleotide expansions, 12 ALS-FTD patients without hexanucleotide repeats, 36 ALS patients without cognitive impairment and 50 healthy controls were included in a prospective neuroimaging study...
February 8, 2018: Brain Imaging and Behavior
https://www.readbyqxmd.com/read/29409157/sentence-composition-ability-in-two-patients-with-non-fluent-agrammatic-variant-primary-progressive-aphasia
#7
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/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/29380657/long-term-maintenance-of-anomia-treatment-effects-in-primary-progressive-aphasia
#9
Aaron M Meyer, Donna C Tippett, R Scott Turner, Rhonda B Friedman
This study examined the maintenance of anomia treatment effects in primary progressive aphasia (PPA). Following baseline testing, a phonological treatment and an orthographic treatment were administered over the course of six months. The treatment stimuli consisted of nouns that were consistently named correctly at baseline (Prophylaxis items) and/or nouns that were consistently named incorrectly at baseline (Remediation items). Naming accuracy was measured at baseline, and it was measured at 1 month, 8 months, and 15 months post-treatment...
January 30, 2018: Neuropsychological Rehabilitation
https://www.readbyqxmd.com/read/29357796/autoimmunity-and-frontotemporal-dementia
#10
Antonella Alberici, Viviana Cristillo, Stefano Gazzina, Alberto Benussi, Alessandro Padovani, Barbara Borroni
Frontotemporal Dementia (FTD) is a neurodegenerative disorder which asymmetrically affects the frontotemporal lobes; it is characterized by behavioural abnormalities, language impairment, and deficits of executive functions. Behavioural variant FTD (bvFTD) and Primary Progressive Aphasias (PPAs) represent the most common phenotypes. The identification of mutations responsible for autosomal dominant inherited disorder, namely Microtubule Associated Protein Tau (MAPT), Granulin (GRN) and chromosome 9 open reading frame 72 (C9orf72) mutations, contributed to elucidate the molecular pathways involved in brain depositions of either Tau or TAR DNA-binding protein 43 (TDP43) inclusions...
January 18, 2018: Current Alzheimer Research
https://www.readbyqxmd.com/read/29305438/asymmetric-tdp-pathology-in-primary-progressive-aphasia-with-right-hemisphere-language-dominance
#11
Garam Kim, Shahrooz Vahedi, Tamar Gefen, Sandra Weintraub, Eileen H Bigio, Marek-Marsel Mesulam, Changiz Geula
OBJECTIVE: To quantitatively examine the regional densities and hemispheric distribution of the 43-kDa transactive response DNA-binding protein (TDP-43) inclusions, neurons, and activated microglia in a left-handed patient with right hemisphere language dominance and logopenic-variant primary progressive aphasia (PPA). METHODS: Phosphorylated TDP-43 inclusions, neurons, and activated microglia were visualized with immunohistochemical and histologic methods. Markers were quantified bilaterally with unbiased stereology in language- and memory-related cortical regions...
January 5, 2018: Neurology
https://www.readbyqxmd.com/read/29241649/the-structure-of-the-mental-lexicon-what-primary-progressive-aphasias-reveal
#12
Clara Sanches, Alexandre Routier, Olivier Colliot, Marc Teichmann
Like recursive syntax, a structured mental lexicon is specific to the human species but its internal organization remains unclear. It is thought to contain information about the semantic, syntactic (e.g., gender) and formal (orthographic/phonological) features of a word. Previous studies suggested that these three components might be separated at the behavioral level and that they might be implemented by temporal cortices. However, the available investigations are based on case reports or small-cohort studies with patients demonstrating post-stroke aphasia, and they did not contrast the three lexical components in a directly comparable way...
December 11, 2017: Neuropsychologia
https://www.readbyqxmd.com/read/29228180/longitudinal-structural-and-molecular-neuroimaging-in-agrammatic-primary-progressive-aphasia
#13
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/29223682/neocortical-origin-and-progression-of-gray-matter-atrophy-in-nonamnestic-alzheimer-s-disease
#14
Jeffrey S Phillips, Fulvio Da Re, Laynie Dratch, Sharon X Xie, David J Irwin, Corey T McMillan, Sanjeev N Vaishnavi, Carlo Ferrarese, Edward B Lee, Leslie M Shaw, John Q Trojanowski, David A Wolk, Murray Grossman
Amnestic Alzheimer's disease (AD) is characterized by early atrophy of the hippocampus and medial temporal lobes before spreading to the neocortex. In contrast, nonamnestic Alzheimer's patients have relative sparing of the hippocampus, but the pattern in which the disease spreads is unclear. We examined spreading disease in nonamnestic AD using a novel magnetic resonance imaging-based analysis adapted from pathologic staging studies, applied here to cross-sectional imaging data. We selected 240 T1-weighted scans from 129 patients with pathology confirmed by autopsy or cerebrospinal fluid, and atrophy maps were computed relative to 238 scans from 115 elderly controls...
November 21, 2017: Neurobiology of Aging
https://www.readbyqxmd.com/read/29223183/conversion-between-addenbrooke-s-cognitive-examination-iii-and-mini-mental-state-examination
#15
Jordi A Matías-Guiu, Vanesa Pytel, Ana Cortés-Martínez, María Valles-Salgado, Teresa Rognoni, Teresa Moreno-Ramos, Jorge Matías-Guiu
BACKGROUND: We aim to provide a conversion between Addenbrooke's Cognitive Examination III (ACE-III) and Mini-Mental State Examination (MMSE) scores, to predict the MMSE result based on ACE-III, thus avoiding the need for both tests, and improving their comparability. METHODS: Equipercentile equating method was used to elaborate a conversion table using a group of 400 participants comprising healthy controls and Alzheimer's disease (AD) patients. Then, reliability was assessed in a group of 100 healthy controls and patients with AD, 52 with primary progressive aphasia and 22 with behavioral variant frontotemporal dementia...
December 10, 2017: International Psychogeriatrics
https://www.readbyqxmd.com/read/29213499/an-interdisciplinary-approach-aiding-the-diagnosis-of-primary-progressive-aphasia-a-case-report
#16
Nadia Shigaeff, Mayra Zanetti, Sibelle de Almeida Tierno, Ana Beatriz Galhardi Di Tommaso, Thais Cristina Marques, Fábio Gazelato de Mello Franco
Frontotemporal dementia (FTD) is one of the most common causes of early-onset dementia with primary progressive aphasia (PPA) being the second-most-frequent form of this degenerative disease. Despite the similarity with progressive dementia (especially in early stages of Alzheimer´s disease), three types of PPA can be differentiated: semantic, agrammatic and logopenic (subtype discussed in this study). To date, no medications have been shown to improve or stabilize cognitive deficits in patients with PPA. We report the case of a 62-year-old woman with difficulty naming objects and planning...
January 2017: Dementia & Neuropsychologia
https://www.readbyqxmd.com/read/29201014/impaired-interoceptive-accuracy-in-semantic-variant-primary-progressive-aphasia
#17
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/29178408/spousal-recollections-of-early-signs-of-primary-progressive-aphasia
#18
Margaret Pozzebon, Jacinta Douglas, David Ames
BACKGROUND: Although primary progressive aphasia (PPA) is characterized by progressive loss of language and communication skills, knowledge about the earliest emerging signs announcing the onset of this condition is limited. AIMS: To explore spousal recollections regarding the earliest signs of PPA and to compare the nature of the earliest perceived symptoms across the three PPA variants. METHODS & PROCEDURES: In-depth interviews focusing on the earliest signs of illness onset were conducted with 13 spouses whose partners were diagnosed with PPA...
November 26, 2017: International Journal of Language & Communication Disorders
https://www.readbyqxmd.com/read/29149795/development-of-a-psycho-educational-support-program-for-individuals-with-primary-progressive-aphasia-and-their-care-partners
#19
Darby J Morhardt, Mary C O'Hara, Kristine Zachrich, Christina Wieneke, Emily J Rogalski
Primary progressive aphasia is a language-based dementia that initially spares other cognitive domains; however, aphasia interferes with many life roles such as work and interpersonal relationships. Psycho-educational programs, such as support groups have been shown to be effective for persons with Alzheimer's dementia; however, little is known regarding their effectiveness for persons with primary progressive aphasia. This paper describes the development of a program that offers support, education and activities for persons with primary progressive aphasia and their care-partners and its feasibility...
January 1, 2017: Dementia
https://www.readbyqxmd.com/read/29117794/cognitive-telerehabilitation-in-mild-cognitive-impairment-alzheimer-s-disease-and-frontotemporal-dementia-a-systematic-review
#20
Maria Cotelli, Rosa Manenti, Michela Brambilla, Elena Gobbi, Clarissa Ferrari, Giuliano Binetti, Stefano F Cappa
Introduction Given the limited effectiveness of pharmacological treatments, non-pharmacological interventions in neurodegenerative diseases have gained increasing attention in recent years and telerehabilitation has been proposed as a cognitive rehabilitation strategy. The purpose of this systematic review is to examine the evidence for the efficacy of cognitive telerehabilitation interventions compared with face-to-face rehabilitation in patients with mild cognitive impairment, Alzheimer's disease and frontotemporal dementia...
January 1, 2017: Journal of Telemedicine and Telecare
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