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"Primary progressive aphasia"

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https://www.readbyqxmd.com/read/29028649/-18f-thk5351-pet-imaging-in-patients-with-semantic-variant-primary-progressive-aphasia
#1
Hyon Lee, Seongho Seo, Sang-Yoon Lee, Hye Jin Jeong, Sung-Ho Woo, Kyoung-Min Lee, Yeong-Bae Lee, Kee Hyung Park, Jae-Hyeok Heo, Cindy W Yoon, Jae Myeong Kang, Jaelim Cho, Nobuyuki Okamura, Shozo Furumoto, Kazuhiko Yanai, Duk L Na, Tatsuo Ido, Victor L Villemagne, Young Noh
BACKGROUND: Semantic variant primary progressive aphasia (svPPA) has been associated with a variety of proteinopathies, mainly transactive response DNA-binding protein, but also with tau and β-amyloid. Recently selective tau tracers for positron emission tomography (PET) have been developed to determine the presence of cerebral tau deposits in vivo. Here, we investigated the topographical distribution of THK5351 in svPPA patients. MATERIALS AND METHODS: Five svPPA patients, 14 Alzheimer's disease patients, and 15 age-matched normal controls underwent [F]-THK5351 PET scans, magnetic resonance imaging, and detailed neuropsychological tests...
October 12, 2017: Alzheimer Disease and Associated Disorders
https://www.readbyqxmd.com/read/28990425/mendelian-forms-of-disease-and-age-at-onset-affect-survival-in-frontotemporal-dementia
#2
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/28986472/flortaucipir-tau-pet-imaging-in-semantic-variant-primary-progressive-aphasia
#3
Sara J Makaretz, Megan Quimby, Jessica Collins, Nikos Makris, Scott McGinnis, Aaron Schultz, Neil Vasdev, Keith A Johnson, Bradford C Dickerson
OBJECTIVE: The semantic variant of primary progressive aphasia (svPPA) is typically associated with frontotemporal lobar degeneration (FTLD) with longTAR DNA-binding protein (TDP)-43-positive neuropil threads and dystrophic neurites (type C), and is only rarely due to a primary tauopathy or Alzheimer's disease. We undertook this study to investigate the localisation and magnitude of the presumed tau Positron Emission Tomography (PET) tracer [(18)F]Flortaucipir (FTP; also known as T807 or AV1451) in patients with svPPA, hypothesising that most patients would not show tracer uptake different from controls...
October 6, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28978658/the-social-and-economic-burden-of-frontotemporal-degeneration
#4
James E Galvin, David H Howard, Sharon S Denny, Susan Dickinson, Nadine Tatton
OBJECTIVE: To quantify the socioeconomic burden of frontotemporal degeneration (FTD) compared to previously published data for Alzheimer disease (AD). METHODS: A 250-item internet survey was administered to primary caregivers of patients with behavioral-variant FTD (bvFTD), primary progressive aphasia, FTD with motor neuron disease, corticobasal syndrome, or progressive supranuclear palsy. The survey included validated scales for disease staging, behavior, activities of daily living, caregiver burden, and health economics, as well as investigator-designed questions to capture patient and caregiver experience with FTD...
October 4, 2017: Neurology
https://www.readbyqxmd.com/read/28975068/longitudinal-white-matter-change-in-frontotemporal-dementia-subtypes-and-sporadic-late-onset-alzheimer-s-disease
#5
Fanny M Elahi, Gabe Marx, Yann Cobigo, Adam M Staffaroni, John Kornak, Duygu Tosun, Adam L Boxer, Joel H Kramer, Bruce L Miller, Howard J Rosen
BACKGROUND: Degradation of white matter microstructure has been demonstrated in frontotemporal lobar degeneration (FTLD) and Alzheimer's disease (AD). In preparation for clinical trials, ongoing studies are investigating the utility of longitudinal brain imaging for quantification of disease progression. To date only one study has examined sample size calculations based on longitudinal changes in white matter integrity in FTLD. OBJECTIVE: To quantify longitudinal changes in white matter microstructural integrity in the three canonical subtypes of frontotemporal dementia (FTD) and AD using diffusion tensor imaging (DTI)...
2017: NeuroImage: Clinical
https://www.readbyqxmd.com/read/28969381/distinct-spatiotemporal-patterns-of-neuronal-functional-connectivity-in-primary-progressive-aphasia-variants
#6
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/28912300/-18-f-av-1451-binding-in-vivo-mirrors-the-expected-distribution-of-tdp-43-pathology-in-the-semantic-variant-of-primary-progressive-aphasia
#7
W R Bevan-Jones, Thomas E Cope, P Simon Jones, Luca Passamonti, Young T Hong, Tim D Fryer, Robert Arnold, Kieren S J Allinson, Jonathan P Coles, Franklin I Aigbirhio, Karalyn Patterson, John T O'Brien, James B Rowe
INTRODUCTION: Semantic dementia, including the semantic variant of primary progressive aphasia (svPPA), is strongly associated with TAR-DNA binding protein 43 (TDP-43) type C pathology. It provides a useful model in which to test the specificity of in vivo binding of the putative tau ligand [(18)F]AV-1451, which is elevated in frontotemporal lobar degeneration tauopathies. METHODS AND RESULTS: Seven patients (five with svPPA and two with 'right' semantic dementia) and 12 healthy controls underwent positron emission tomography brain imaging with [(18)F]AV-1451...
September 14, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28879086/emotion-detection-deficits-and-changes-in-personality-traits-linked-to-loss-of-white-matter-integrity-in-primary-progressive-aphasia
#8
Namita Multani, Sebastiano Galantucci, Stephen M Wilson, Tal Shany-Ur, Pardis Poorzand, Matthew E Growdon, Jung Yun Jang, Joel H Kramer, Bruce L Miller, Katherine P Rankin, Maria Luisa Gorno-Tempini, Maria Carmela Tartaglia
Non-cognitive features including personality changes are increasingly recognized in the three PPA variants (semantic-svPPA, non fluent-nfvPPA, and logopenic-lvPPA). However, differences in emotion processing among the PPA variants and its association with white matter tracts are unknown. We compared emotion detection across the three PPA variants and healthy controls (HC), and related them to white matter tract integrity and cortical degeneration. Personality traits in the PPA group were also examined in relation to white matter tracts...
2017: NeuroImage: Clinical
https://www.readbyqxmd.com/read/28872040/structural-magnetic-resonance-imaging-in-frontotemporal-lobar-dementia
#9
Anne Bertrand, Sebastian Stroër, Isabelle Le Ber, Marc Teichmann, Didier Dormont
Frontotemporal lobar dementia (FTLD) is a heterogeneous group of neurodegenerative diseases. FTLD encompass: 1) behavioral forms, sometimes associated with amyotrophic lateral sclerosis; 2) linguistic forms (semantic and non-fluent primary progressive aphasia); 3) atypical parkinsonian syndromes (progressive supranuclear palsy and corticobasal syndrome). Standard brain MRI allows for strengthening the clinical suspicion of FTLD, by showing a pattern of atrophy in relation with the patient's clinical symptoms: frontotemporal anterior atrophy in behavioral forms; temporopolar or inferior left frontal atrophy in the linguistic forms; mesencephalic or corticosubcortical hemispheric atrophy in forms with atypical pakinsonism...
September 1, 2017: Gériatrie et Psychologie Neuropsychiatrie du Vieillissement
https://www.readbyqxmd.com/read/28867555/lexical-access-in-semantic-variant-ppa-evidence-for-a-post-semantic-contribution-to-naming-deficits
#10
Stephen M Wilson, Charlotte Dehollain, Sophie Ferrieux, Laura E H Christensen, Marc Teichmann
The most salient clinical symptom of semantic variant primary progressive aphasia (PPA) is a profound and pervasive anomia. These patients' naming impairments have been shown to reflect in large part a domain-general deterioration of conceptual knowledge that impacts both linguistic and non-linguistic processing. However, it is possible that post-semantic stages of lexical access may also contribute to naming deficits. To clarify the stages at which lexical access breaks down in semantic variant PPA, eleven French-speaking patients were asked to name objects, and were then queried for semantic, lexical-syntactic, and word form information pertaining to the items they could not name...
September 1, 2017: Neuropsychologia
https://www.readbyqxmd.com/read/28853339/tdq-60-a-color-picture-naming-test-for-adults-and-elderly-people-validation-and-normalization-data
#11
Joël Macoir, Catherine Beaudoin, Josée Bluteau, Olivier Potvin, Maximiliano A Wilson
Word-finding difficulties are usually assessed with picture-naming tests. In this article, we present the TDQ-60, a new test designed to assess acquired lexical access deficits, taking into account semantics and psycholinguistic variables. The article includes three studies. Study 1 describes the development phase of the TDQ-60.  In study 2, healthy control participants and individuals with a diagnosis of the semantic variant of primary progressive aphasia were assessed to establish the convergent and discriminant validity of the TDQ-60...
August 30, 2017: Neuropsychology, Development, and Cognition. Section B, Aging, Neuropsychology and Cognition
https://www.readbyqxmd.com/read/28843341/artificial-grammar-learning-in-vascular-and-progressive-non-fluent-aphasias
#12
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/28838910/assessment-of-iron-deposition-in-the-brain-in-frontotemporal-dementia-and-its-correlation-with-behavioral-traits
#13
R Sheelakumari, C Kesavadas, T Varghese, R M Sreedharan, B Thomas, J Verghese, P S Mathuranath
BACKGROUND AND PURPOSE: Brain iron deposition has been implicated as a major culprit in the pathophysiology of neurodegeneration. However, the quantitative assessment of iron in behavioral variant frontotemporal dementia and primary progressive aphasia brains has not been performed, to our knowledge. The aim of our study was to investigate the characteristic iron levels in the frontotemporal dementia subtypes using susceptibility-weighted imaging and report its association with behavioral profiles...
August 24, 2017: AJNR. American Journal of Neuroradiology
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
#14
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
#15
REVIEW
Angela B Deutschländer, Owen A Ross, Dennis W Dickson, Zbigniew 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 we review clinical, imaging, neuropathologic and genetic features of multiple system atrophy (MSA), progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), and frontotemporal lobar degeneration (FTLD). The terms CBD and FTLD refer to pathologically confirmed cases of corticobasal syndrome (CBS) and frontotemporal dementia (FTD)...
August 12, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28803212/data-driven-classification-of-patients-with-primary-progressive-aphasia
#16
Paul Hoffman, Seyed Ahmad Sajjadi, Karalyn Patterson, Peter J Nestor
Current diagnostic criteria classify primary progressive aphasia into three variants-semantic (sv), nonfluent (nfv) and logopenic (lv) PPA-though the adequacy of this scheme is debated. This study took a data-driven approach, applying k-means clustering to data from 43 PPA patients. The algorithm grouped patients based on similarities in language, semantic and non-linguistic cognitive scores. The optimum solution consisted of three groups. One group, almost exclusively those diagnosed as svPPA, displayed a selective semantic impairment...
November 2017: Brain and Language
https://www.readbyqxmd.com/read/28757671/slowed-articulation-rate-is-a-sensitive-diagnostic-marker-for-identifying-non-fluent-primary-progressive-aphasia
#17
Claire Cordella, Bradford C Dickerson, Megan Quimby, Yana Yunusova, Jordan R Green
BACKGROUND: Primary progressive aphasia (PPA) is a neurodegenerative aphasic syndrome with three distinct clinical variants: non-fluent (nfvPPA), logopenic (lvPPA), and semantic (svPPA). Speech (non-) fluency is a key diagnostic marker used to aid identification of the clinical variants, and researchers have been actively developing diagnostic tools to assess speech fluency. Current approaches reveal coarse differences in fluency between subgroups, but often fail to clearly differentiate nfvPPA from the variably fluent lvPPA...
2017: Aphasiology
https://www.readbyqxmd.com/read/28750682/behavioural-and-neuroanatomical-correlates-of-auditory-speech-analysis-in-primary-progressive-aphasias
#18
Chris J D Hardy, Jennifer L Agustus, Charles R Marshall, Camilla N Clark, Lucy L Russell, Rebecca L Bond, Emilie V Brotherhood, David L Thomas, Sebastian J Crutch, Jonathan D Rohrer, Jason D Warren
BACKGROUND: Non-verbal auditory impairment is increasingly recognised in the primary progressive aphasias (PPAs) but its relationship to speech processing and brain substrates has not been defined. Here we addressed these issues in patients representing the non-fluent variant (nfvPPA) and semantic variant (svPPA) syndromes of PPA. METHODS: We studied 19 patients with PPA in relation to 19 healthy older individuals. We manipulated three key auditory parameters-temporal regularity, phonemic spectral structure and prosodic predictability (an index of fundamental information content, or entropy)-in sequences of spoken syllables...
July 27, 2017: Alzheimer's Research & Therapy
https://www.readbyqxmd.com/read/28747523/-18-f-flortaucipir-pet-mri-correlations-in-non-amnestic-and-amnestic-variants-of-alzheimer-disease
#19
Ilya M Nasrallah, Yin Jie Chen, Meng-Kang Hsieh, Jeffrey S Philips, Kylie Ternes, Grace Stockbower, Yvette Sheline, Corey T McMillan, Murray Grossman, David A Wolk
Non-amnestic Alzheimer disease (AD) variants, including posterior cortical atrophy (PCA) and logopenic variant of primary progressive aphasia (lvPPA), differ in distributions of tau aggregates and neurodegeneration compared to amnestic AD. We evaluated whether (18)F-flortaucipir (also called (18)F-AV-1451) Positron Emission Tomography (PET), targeting tau aggregates, detects these differences and compared this to magnetic resonance imaging (MRI) measures of gray matter (GM) atrophy. Methods: 5 PCA, 4 lvPPA, 6 age-matched AD, and 6 control subjects underwent (18)F-flortaucipir PET and MRI...
July 26, 2017: Journal of Nuclear Medicine: Official Publication, Society of Nuclear Medicine
https://www.readbyqxmd.com/read/28713256/baseline-performance-predicts-tdcs-mediated-improvements-in-language-symptoms-in-primary-progressive-aphasia
#20
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
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