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Progressive aphasia

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https://www.readbyqxmd.com/read/28645277/dignity-in-people-with-frontotemporal-dementia-and-similar-disorders-a-qualitative-study-of-the-perspective-of-family-caregivers
#1
Mette Sagbakken, Dagfinn Nåden, Ingun Ulstein, Kari Kvaal, Birgitta Langhammer, May-Karin Rognstad
BACKGROUND: Frontotemporal dementia (FTD) constitutes on average 10-15% of dementia in younger persons (≤65 years old), but can also affect older people. These patients demonstrate a decline in social conduct, and/or language aphasias, apathy, and loss of insight that is gradual and progressive. Preservation of dignity seems to be highly relevant both before and after admission to different types of institutionalized care, but the research is scant. From the perspective of close relatives, this study aims to develop knowledge related to dignified or undignified care of patients with FTD and similar conditions...
June 23, 2017: BMC Health Services Research
https://www.readbyqxmd.com/read/28626543/cerebral-stroke-in-a-teenage-girl-with-paroxysmal-nocturnal-hemoglobinuria
#2
Francesco Gervasi, Lucia D'Amelio, Antonino Trizzino, Fabrizia Ferraro, Delia Russo, Giuseppe Santangelo, Francesca Cardella, Angela Trizzino, Floriana Di Marco, Piero Farruggia
We report a case of paroxysmal nocturnal hemoglobinuria (PNH) in a 14 year-old girl presenting a cerebral arterial thrombosis. The initial diagnosis was carential anemia due to menarche following identification of slight macrocytic anemia, leucopenia and mild thrombocytopenia at routine blood analysis. The child was eventually referred to a children's hospital after the onset of progressive fatigue, anorexia and paleness. Severe anemia (hemoglobin 6 g/dL) with negative Coombs test, mild leucopenia (white blood cells 4...
June 1, 2017: Hematology Reports
https://www.readbyqxmd.com/read/28625346/the-role-of-the-motor-system-in-action-naming-in-patients-with-neurodegenerative-extrapyramidal-syndromes
#3
REVIEW
Maria Cotelli, Rosa Manenti, Michela Brambilla, Barbara Borroni
Previous studies of patients with brain damage have suggested a close relationship between aphasia and movement disorders. Neurodegenerative extrapyramidal syndromes associated with cognitive impairment provide an interesting model for studying the neural substrates of cognitive and motor symptoms. In this review, we focused on studies investigating language production abilities in patients with Parkinson's disease (PD), Corticobasal Syndrome (CBS) and Progressive Supranuclear Palsy (PSP). According to some reports, these patients exhibit a reduction in performance in both action and object naming or verb production compared to healthy individuals...
May 26, 2017: Cortex; a Journal Devoted to the Study of the Nervous System and Behavior
https://www.readbyqxmd.com/read/28624827/qualitative-assessment-of-verbal-fluency-performance-in-frontotemporal-dementia
#4
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
#5
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/28598840/neuropsychological-symptoms-in-sporadic-creutzfeldt-jakob-disease-patients-in-germany
#6
Anna Krasnianski, Geeske T Bohling, Uta Heinemann, Daniela Varges, Bettina Meissner, Walter J Schulz-Schaeffer, Andreas Reif, Inga Zerr
BACKGROUND: The polymorphism at codon 129 of the prion protein gene (PRNP) and the PrPSc types 1 and 2 belong to a molecular classification of sporadic Creutzfeldt-Jakob disease (sCJD) that correlates well with the clinical and neuropathological phenotype of sCJD. OBJECTIVE: The aim of the study was to perform the first detailed evaluation of neuropsychological deficits in a large group of definite sCJD patients with known molecular subtype. METHODS: We analyzed neuropsychological symptoms in a cohort of 248 sCJD patients with known M129 V polymorphism of PRNP and prion protein type...
June 8, 2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28594853/the-unexpected-co-occurrence-of-grn-and-mapt-p-a152t-in-basque-families-clinical-and-pathological-characteristics
#7
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
#8
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
#9
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)...
June 7, 2017: Neurology
https://www.readbyqxmd.com/read/28579951/communicative-pragmatic-assessment-is-sensitive-and-time-effective-in-measuring-the-outcome-of-aphasia-therapy
#10
Benjamin Stahl, Bettina Mohr, Felix R Dreyer, Guglielmo Lucchese, Friedemann Pulvermüller
A range of methods in clinical research aim to assess treatment-induced progress in aphasia therapy. Here, we used a crossover randomized controlled design to compare the suitability of utterance-centered and dialogue-sensitive outcome measures in speech-language testing. Fourteen individuals with post-stroke chronic non-fluent aphasia each received two types of intensive training in counterbalanced order: conventional confrontation naming, and communicative-pragmatic speech-language therapy (Intensive Language-Action Therapy, an expanded version of Constraint-Induced Aphasia Therapy)...
2017: Frontiers in Human Neuroscience
https://www.readbyqxmd.com/read/28579383/clinical-and-biological-phenotypes-of-frontotemporal-dementia-perspectives-for-disease-modifying-therapies
#11
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
#12
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...
June 4, 2017: Neurological Sciences
https://www.readbyqxmd.com/read/28571652/functional-neuroanatomy-of-speech-signal-decoding-in-primary-progressive-aphasias
#13
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...
May 10, 2017: Neurobiology of Aging
https://www.readbyqxmd.com/read/28562080/neuroimaging-patterns-along-the-als-ftd-spectrum-a-multiparametric-imaging-study
#14
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
#15
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/28551838/5-ala-fluorescence-in-intraparenchymal-endodermal-cysts
#16
José Pedro Lavrador, Christian Brogna, Francesco Vergani, Fay Greenway, Miren Aizpurua, Ranj Bhangoo
In recent years, new indications have been suggested for 5-ALA, particularly for cystic lesions. We report the use of 5-ALA fluorescence in an intraparenchymal supratentorial endodermal cyst of a 52-year-old female presenting with headache, progressive right side hemiparesis and anomic aphasia. She underwent an image-guided 5-ALA-assisted left minicraniotomy for fenestration of the cystic lesion into the ventricular system. The capsule of the cyst was noted to fluoresce with 5-ALA. She recovered from the previous deficits and the cyst decreased in size...
May 27, 2017: Acta Neurochirurgica
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
#17
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/28541543/validation-of-the%C3%A2-d%C3%A3-pistage-cognitif-de-qu%C3%A3-bec-a-new-cognitive-screening-tool-for-atypical-dementias
#18
Robert Laforce, Leila Sellami, David Bergeron, Audrey Paradis, Louis Verret, Marie-Pierre Fortin, Michèle Houde, Martin Roy, Stéphane Poulin, Joël Macoir, Carol Hudon, Rémi W Bouchard
Objective: This study aimed to validate and provide normative data for the Dépistage Cognitif de Québec (DCQ; www.dcqtest.org), a new cognitive screening tool for atypical dementias. Method: The DCQ was developed by expert behavioral neurologists and clinical neuropsychologists based on updated criteria for Alzheimer's disease, primary progressive aphasia, and behavioral variant frontotemporal dementia. It targets five relevant domains: Memory, Visuospatial, Executive, Language, and Behavior...
May 25, 2017: Archives of Clinical Neuropsychology: the Official Journal of the National Academy of Neuropsychologists
https://www.readbyqxmd.com/read/28532703/reading-difficulties-in-primary-progressive-aphasia-in-a-regular-language-speaking-cohort-of-patients
#19
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/28515274/beyond-clinical-syndromes-in-primary-progressive-aphasia-seeking-etiologic-diagnoses
#20
EDITORIAL
David S Knopman, Peter J Nestor
No abstract text is available yet for this article.
May 17, 2017: Neurology
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