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Primary progressive aphasia

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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
#1
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
#2
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
#3
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...
August 10, 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
#4
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
#5
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
#6
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
#7
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
https://www.readbyqxmd.com/read/28662598/comparing-the-effects-of-clinician-and-caregiver-administered-lexical-retrieval-training-for-progressive-anomia
#8
Stephanie M Grasso, Kaleigh M Shuster, Maya L Henry
There is a growing body of literature indicating that lexical retrieval training can result in improved naming ability in individuals with neurodegenerative disease. Traditionally, treatment is administered by a speech-language pathologist, with little involvement of caregivers or carry-over of practice into the home. This study examined the effects of a lexical retrieval training programme that was implemented first by a clinician and, subsequently, by a trained caregiver. Two dyads, each consisting of one individual with anomia caused by neurodegenerative disease (one with mild cognitive impairment and one with logopenic primary progressive aphasia) and their caregiver, participated in the study...
June 30, 2017: Neuropsychological Rehabilitation
https://www.readbyqxmd.com/read/28659753/neural-correlates-of-verbal-episodic-memory-and-lexical-retrieval-in-logopenic-variant-primary-progressive-aphasia
#9
Khaing T Win, John Pluta, Paul Yushkevich, David J Irwin, Corey T McMillan, Katya Rascovsky, David Wolk, Murray Grossman
Objective: Logopenic variant primary progressive aphasia (lvPPA) is commonly associated with Alzheimer's disease (AD) pathology. But lvPPA patients display different cognitive and anatomical profile from the common clinical AD patients, whose verbal episodic memory is primarily affected. Reports of verbal episodic memory difficulty in lvPPA are inconsistent, and we hypothesized that their lexical retrieval impairment contributes to verbal episodic memory performance and is associated with left middle temporal gyrus atrophy...
2017: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/28624827/qualitative-assessment-of-verbal-fluency-performance-in-frontotemporal-dementia
#10
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/28611820/white-matter-disruption-and-connected-speech-in-non-fluent-and-semantic-variants-of-primary-progressive-aphasia
#11
Karine Marcotte, Naida L Graham, Kathleen C Fraser, Jed A Meltzer, David F Tang-Wai, Tiffany W Chow, Morris Freedman, Carol Leonard, Sandra E Black, Elizabeth Rochon
Differential patterns of white matter disruption have recently been reported in the non-fluent (nfvPPA) and semantic (svPPA) variants of primary progressive aphasia (PPA). No single measure is sufficient to distinguish between the PPA variants, but connected speech allows for the quantification of multiple measures. The aim of the present study was to further investigate the white matter correlates associated with connected speech features in PPA. We examined the relationship between white matter metrics and connected speech deficits using an automated analysis of transcriptions of connected speech and diffusion tensor imaging in language-related tracts...
January 2017: Dementia and Geriatric Cognitive Disorders Extra
https://www.readbyqxmd.com/read/28594853/the-unexpected-co-occurrence-of-grn-and-mapt-p-a152t-in-basque-families-clinical-and-pathological-characteristics
#12
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/28593829/communication-behaviors-associated-with-successful-conversation-in-semantic-variant-primary-progressive-aphasia
#13
Cathleen Taylor-Rubin, Karen Croot, Emma Power, Sharon A Savage, John R Hodges, Leanne Togher
BACKGROUND: Primary progressive aphasia (PPA) affects a range of language and cognitive domains that impact on conversation. Little is known about conversation breakdown in the semantic variant of PPA (svPPA, also known as semantic dementia). This study investigates conversation of people with svPPA. METHODS: Dyadic conversations about everyday activities between seven individuals with svPPA and their partners, and seven control pairs were video recorded and transcribed...
June 8, 2017: International Psychogeriatrics
https://www.readbyqxmd.com/read/28592456/csf-sapp%C3%AE-ykl-40-and-neurofilament-light-in-frontotemporal-lobar-degeneration
#14
Daniel Alcolea, Eduard Vilaplana, Marc Suárez-Calvet, Ignacio Illán-Gala, Rafael Blesa, Jordi Clarimón, Albert Lladó, Raquel Sánchez-Valle, José L Molinuevo, Guillermo García-Ribas, Yaroslau Compta, María José Martí, Gerard Piñol-Ripoll, Guillermo Amer-Ferrer, Aina Noguera, Ana García-Martín, Juan Fortea, Alberto Lleó
OBJECTIVE: To analyze the clinical utility of 3 CSF biomarkers and their structural imaging correlates in a large cohort of patients with different dementia and parkinsonian syndromes within the spectrum of frontotemporal lobar degeneration (FTLD). METHODS: We analyzed 3 CSF biomarkers (YKL-40, soluble β fragment of amyloid precursor protein [sAPPβ], neurofilament light [NfL]) and core Alzheimer disease (AD) biomarkers (β-amyloid1-42, total tau, phosphorylated tau) in patients with FTLD-related clinical syndromes (n = 159): behavioral variant of frontotemporal dementia (n = 68), nonfluent (n = 23) and semantic (n = 19) variants of primary progressive aphasia, progressive supranuclear palsy (n = 28), and corticobasal syndrome (n = 21)...
July 11, 2017: Neurology
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/28578483/sand-a-screening-for-aphasia-in-neurodegeneration-development-and-normative-data
#16
Eleonora Catricalà, Elena Gobbi, Petronilla Battista, Antonio Miozzo, Cristina Polito, Veronica Boschi, Valentina Esposito, Sofia Cuoco, Paolo Barone, Sandro Sorbi, Stefano F Cappa, Peter Garrard
Language assessment has a critical role in the clinical diagnosis of neurodegenerative diseases, in particular, in the case of Primary Progressive Aphasia (PPA). The current diagnostic criteria (Gorno-Tempini et al., 2011) identify three main variants on the basis of clinical features and patterns of brain atrophy. Widely accepted tools to diagnose, clinically classify, and follow up the heterogeneous language profiles of PPA are still lacking. In this study, we develop a screening battery, composed of nine tests (picture naming, word and sentence comprehension, word and sentence repetition, reading, semantic association, writing and picture description), following the recommendations of current diagnostic guidelines and taking into account recent research on the topic...
August 2017: Neurological Sciences
https://www.readbyqxmd.com/read/28571652/functional-neuroanatomy-of-speech-signal-decoding-in-primary-progressive-aphasias
#17
Chris J D Hardy, Jennifer L Agustus, Charles R Marshall, Camilla N Clark, Lucy L Russell, Emilie V Brotherhood, Rebecca L Bond, Cassidy M Fiford, Sasha Ondobaka, David L Thomas, Sebastian J Crutch, Jonathan D Rohrer, Jason D Warren
The pathophysiology of primary progressive aphasias remains poorly understood. Here, we addressed this issue using activation fMRI in a cohort of 27 patients with primary progressive aphasia (nonfluent, semantic, and logopenic variants) versus 15 healthy controls. Participants listened passively to sequences of spoken syllables in which we manipulated 3-key auditory speech signal characteristics: temporal regularity, phonemic spectral structure, and pitch sequence entropy. Relative to healthy controls, nonfluent variant patients showed reduced activation of medial Heschl's gyrus in response to any auditory stimulation and reduced activation of anterior cingulate to temporal irregularity...
August 2017: Neurobiology of Aging
https://www.readbyqxmd.com/read/28562080/neuroimaging-patterns-along-the-als-ftd-spectrum-a-multiparametric-imaging-study
#18
Taha Omer, Eoin Finegan, Siobhan Hutchinson, Mark Doherty, Alice Vajda, Russell L McLaughlin, Niall Pender, Orla Hardiman, Peter Bede
Frontotemporal dementia is associated with considerable clinical, genetic and pathological heterogeneity. The objective of this study is to characterise the imaging signatures of the main FTD phenotypes along the ALS-FTD spectrum. A total of 100 participants underwent comprehensive multimodal neuroimaging, genetic testing and neuropsychological evaluation. Seven patients with behavioural variant FTD (bvFTD), 11 patients with non-fluent-variant primary progressive aphasia (nfvPPA), two patients with sematic-variant primary progressive aphasia(svPPA), 10 patients with amyotrophic lateral sclerosis and FTD carrying the C9orf72 hexanucleotide repeat (C9 + ALS-FTD), 10 patients with ALS-FTD without hexanucleotide repeats (C9-ALS-FTD), 20 ALS patients without behavioural or cognitive deficits (ALSnci) and 40 healthy controls (HC) were included in a prospective quantitative neuroimaging study...
May 31, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28559805/semantic-feature-training-in-combination-with-transcranial-direct-current-stimulation-tdcs-for-progressive-anomia
#19
Jinyi Hung, Ashley Bauer, Murray Grossman, Roy H Hamilton, H B Coslett, Jamie Reilly
We examined the effectiveness of a 2-week regimen of a semantic feature training in combination with transcranial direct current stimulation (tDCS) for progressive naming impairment associated with primary progressive aphasia (N = 4) or early onset Alzheimer's Disease (N = 1). Patients received a 2-week regimen (10 sessions) of anodal tDCS delivered over the left temporoparietal cortex while completing a language therapy that consisted of repeated naming and semantic feature generation. Therapy targets consisted of familiar people, household items, clothes, foods, places, hygiene implements, and activities...
2017: Frontiers in Human Neuroscience
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
#20
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
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