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Primary progressive aphasia

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https://www.readbyqxmd.com/read/28323683/genetic-alzheimer-disease-and-sporadic-dementia-with-lewy-bodies-a-comorbidity-presenting-as-primary-progressive-aphasia
#1
Tereza Picková, Radoslav Matěj, Ondrej Bezdicek, Jiří Keller, Julie van der Zee, Christine Van Broeckhoven, Zsolt Cséfalvay, Robert Rusina
We report a 44-year-old woman, with a family history of early-onset dementia, presenting with primary progressive aphasia. This clinically variable syndrome has multiple underlying pathologies, and correlations between clinical manifestations and postmortem neuropathologic findings are controversial. Our patient suffered worsening language impairment with major word-finding difficulties but preserved comprehension. She also developed episodic memory impairment. Her condition progressed to dementia with behavioral changes...
March 2017: Cognitive and Behavioral Neurology: Official Journal of the Society for Behavioral and Cognitive Neurology
https://www.readbyqxmd.com/read/28304311/frontotemporal-dementia-due-to-the-novel-grn-arg161glyfsx36-mutation
#2
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...
March 18, 2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28304308/violation-of-laws-in-frontotemporal-dementia-a-multicenter-study-in-japan
#3
Shunichiro Shinagawa, Kazue Shigenobu, Kenji Tagai, Ryuji Fukuhara, Naoto Kamimura, Takaaki Mori, Kenji Yoshiyama, Hiroaki Kazui, Kazuhiko Nakayama, Manabu Ikeda
Although violations of laws, such as shoplifting, are considered to be common in frontotemporal dementia (FTD) patients, there have been few studies on this subject and the frequencies and types of such violations have not been clarified. The objective of this study was to conduct a retrospective investigation of FTD patients in the psychiatry departments of multiple institutions to determine the types and frequencies of any law violations and compare them with those of AD patients. All patients were examined between January 2011 and December 2015 at the specialized dementia outpatient clinics of 10 facilities (5 psychiatry departments of university hospitals, 5 psychiatric hospitals)...
March 18, 2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28304307/logopenic-aphasia-due-to-a-strategic-stroke-new-evidence-from-a-single-case
#4
Javier Riancho, Ana Pozueta, Miguel Santos, Carmen Lage, José M Carril, Ignacio Banzo, Isabel Martínez-Rodriguez, Marilu Gorno-Tempini, Pascual Sánchez-Juan
Among primary progressive aphasias (PPAs), logopenic variant PPA (lv-PPA) is usually related to Alzheimer's disease. Although it has been widely clinically and pathologically evaluated, the topography in LPA is still controversial. We report a patient presenting with a logopenic syndrome due to a strategic lesion located in the superior and middle temporal gyrus and compare our findings with those of a PiB-PET positive lv-PPA patient matched by age, gender, and education. We consider that our study provides new anatomical clues to better understand the cognitive mechanisms underlying the logopenic syndrome...
March 10, 2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28289749/motor-speech-phenotypes-of-frontotemporal-dementia-primary-progressive-aphasia-and-progressive-apraxia-of-speech
#5
Matthew L Poole, Amy Brodtmann, David Darby, Adam P Vogel
Purpose: Our purpose was to create a comprehensive review of speech impairment in frontotemporal dementia (FTD), primary progressive aphasia (PPA), and progressive apraxia of speech in order to identify the most effective measures for diagnosis and monitoring, and to elucidate associations between speech and neuroimaging. Method: Speech and neuroimaging data described in studies of FTD and PPA were systematically reviewed. A meta-analysis was conducted for speech measures that were used consistently in multiple studies...
March 13, 2017: Journal of Speech, Language, and Hearing Research: JSLHR
https://www.readbyqxmd.com/read/28288010/functional-connectivity-is-reduced-in-early-stage-primary-progressive-aphasia-when-atrophy-is-not-prominent
#6
Borna Bonakdarpour, Emily J Rogalski, Allan Wang, Jaiashre Sridhar, M M Mesulam, Robert S Hurley
Primary progressive aphasia (PPA) is a clinical syndrome of language decline caused by neurodegenerative pathology. Although language impairments in PPA are typically localized via the morphometric assessment of atrophy, functional changes may accompany or even precede detectable structural alterations, in which case resting state functional connectivity (RSFC) could provide an alternative approach. The goal of this study was to determine whether language network RSFC is reduced in early-stage PPA when atrophy is not prominent...
March 10, 2017: Alzheimer Disease and Associated Disorders
https://www.readbyqxmd.com/read/28263214/regional-cerebral-blood-flow-spect-and-mri-in-logopenic-variant-primary-progressive-aphasia
#7
Aristeidis H Katsanos, Spyridon Tsiouris, Chrissa Sioka, Anastasia Zikou, Andreas Fotopoulos
We report a 61-year-old woman who presented because of gradually progressive speech disorders. MRI scan revealed left temporal lobe atrophy with ex vacuo dilatation of the temporal horn and atrophy of the left inferior frontal gyrus and the left inferior parietal lobe. These findings in brain perfusion SPECT corresponded to a generalized hypoperfusion of the left hemisphere, particularly prominent on the rear parietal cortex and the temporal lobe. After taking into consideration the clinical presentation and imaging findings on MRI and SPECT, the diagnosis of the logopenic variant of primary progressive aphasia was finally reached...
March 3, 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28241163/association-of-in-vivo-18f-av-1451-tau-pet-imaging-results-with-cortical-atrophy-and-symptoms-in-typical-and-atypical-alzheimer-disease
#8
Chenjie Xia, Sara J Makaretz, Christina Caso, Scott McGinnis, Stephen N Gomperts, Jorge Sepulcre, Teresa Gomez-Isla, Bradley T Hyman, Aaron Schultz, Neil Vasdev, Keith A Johnson, Bradford C Dickerson
Importance: Previous postmortem studies have long demonstrated that neurofibrillary tangles made of hyperphosphorylated tau proteins are closely associated with Alzheimer disease clinical phenotype and neurodegeneration pattern. Validating these associations in vivo will lead to new diagnostic tools for Alzheimer disease and better understanding of its neurobiology. Objective: To examine whether topographical distribution and severity of hyperphosphorylated tau pathologic findings measured by fluorine 18-labeled AV-1451 ([18F]AV-1451) positron emission tomographic (PET) imaging are linked with clinical phenotype and cortical atrophy in patients with Alzheimer disease...
February 20, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28235777/observing-conversational-laughter-in-frontotemporal-dementia
#9
Peter S Pressman, Michaela Simpson, Kelly Gola, Suzanne M Shdo, Edoardo G Spinelli, Bruce L Miller, Maria Luisa Gorno-Tempini, Katherine Rankin, Robert W Levenson
BACKGROUND: We performed an observational study of laughter during seminaturalistic conversations between patients with dementia and familial caregivers. Patients were diagnosed with (1) behavioural variant frontotemporal dementia (bvFTD), (2) right temporal variant frontotemporal dementia (rtFTD), (3) semantic variant of primary progressive aphasia (svPPA), (4) non-fluent variant primary progressive aphasia (nfvPPA) or (5) early onset Alzheimer's disease (eoAD). We hypothesised that those with bvFTD would laugh less in response to their own speech than other dementia groups or controls, while those with rtFTD would laugh less regardless of who was speaking...
February 24, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28229040/predicting-primary-progressive-aphasias-with-support-vector-machine-approaches-in-structural-mri-data
#10
Sandrine Bisenius, Karsten Mueller, Janine Diehl-Schmid, Klaus Fassbender, Timo Grimmer, Frank Jessen, Jan Kassubek, Johannes Kornhuber, Bernhard Landwehrmeyer, Albert Ludolph, Anja Schneider, Sarah Anderl-Straub, Katharina Stuke, Adrian Danek, Markus Otto, Matthias L Schroeter
Primary progressive aphasia (PPA) encompasses the three subtypes nonfluent/agrammatic variant PPA, semantic variant PPA, and the logopenic variant PPA, which are characterized by distinct patterns of language difficulties and regional brain atrophy. To validate the potential of structural magnetic resonance imaging data for early individual diagnosis, we used support vector machine classification on grey matter density maps obtained by voxel-based morphometry analysis to discriminate PPA subtypes (44 patients: 16 nonfluent/agrammatic variant PPA, 17 semantic variant PPA, 11 logopenic variant PPA) from 20 healthy controls (matched for sample size, age, and gender) in the cohort of the multi-center study of the German consortium for frontotemporal lobar degeneration...
2017: NeuroImage: Clinical
https://www.readbyqxmd.com/read/28222300/free-and-cued-selective-reminding-test-accuracy-for-the-differential-diagnosis-of-alzheimer-s-and-neurodegenerative-diseases-a-large-scale-biomarker-characterized-monocenter-cohort-study-clinad
#11
Marc Teichmann, Stéphane Epelbaum, Dalila Samri, Marcel Levy Nogueira, Agnès Michon, Harald Hampel, Foudil Lamari, Bruno Dubois
INTRODUCTION: The International Working Group recommended the Free and Cued Selective Reminding Test (FCSRT) as a sensitive detector of the amnesic syndrome of the hippocampal type in typical Alzheimer's disease (AD). But does it differentiate AD from other neurodegenerative diseases? METHODS: We assessed the FCSRT and cerebrospinal fluid (CSF) AD biomarkers in 992 cases. Experts, blinded to biomarker data, attributed in 650 cases a diagnosis of typical AD, frontotemporal dementia, posterior cortical atrophy, Lewy body disease, progressive supranuclear palsy, corticobasal syndrome, primary progressive aphasias, "subjective cognitive decline," or depression...
February 21, 2017: Alzheimer's & Dementia: the Journal of the Alzheimer's Association
https://www.readbyqxmd.com/read/28209520/deconstructing-empathy-neuroanatomical-dissociations-between-affect-sharing-and-prosocial-motivation-using-a-patient-lesion-model
#12
Suzanne M Shdo, Kamalini G Ranasinghe, Kelly A Gola, Clinton J Mielke, Paul V Sukhanov, Bruce L Miller, Katherine P Rankin
Affect sharing and prosocial motivation are integral parts of empathy that are conceptually and mechanistically distinct. We used a neurodegenerative disease (NDG) lesion model to examine the neural correlates of these two aspects of real-world empathic responding. The study enrolled 275 participants, including 44 healthy older controls and 231 patients diagnosed with one of five neurodegenerative diseases (75 Alzheimer's disease, 58 behavioral variant frontotemporal dementia (bvFTD), 42 semantic variant primary progressive aphasia (svPPA), 28 progressive supranuclear palsy, and 28 non-fluent variant (nfvPPA)...
February 14, 2017: Neuropsychologia
https://www.readbyqxmd.com/read/28196768/cerebrospinal-fluid-markers-detect-alzheimer-s-disease-in-nonamnestic-dementia
#13
Carly Oboudiyat, Tamar Gefen, Eleni Varelas, Sandra Weintraub, Emily Rogalski, Eileen H Bigio, M-Marsel Mesulam
INTRODUCTION: The accuracy of cerebrospinal fluid (CSF) biomarkers for detecting Alzheimer's disease (AD) pathology has not been fully validated in autopsied nonamnestic dementias. METHODS: We retrospectively evaluated CSF β-amyloid 1-42, phosphorylated-tau, and amyloid-tau index as predictors of Alzheimer pathology in patients with primary progressive aphasia, frontotemporal dementia, and progressive supranuclear palsy. RESULTS: Nineteen nonamnestic autopsied cases with relevant CSF values were included...
February 11, 2017: Alzheimer's & Dementia: the Journal of the Alzheimer's Association
https://www.readbyqxmd.com/read/28187331/temporal-acoustic-measures-distinguish-primary-progressive-apraxia-of-speech-from-primary-progressive-aphasia
#14
Joseph R Duffy, Holly Hanley, Rene Utianski, Heather Clark, Edythe Strand, Keith A Josephs, Jennifer L Whitwell
The purpose of this study was to determine if acoustic measures of duration and syllable rate during word and sentence repetition, and a measure of within-word lexical stress, distinguish speakers with primary progressive apraxia of speech (PPAOS) from nonapraxic speakers with the agrammatic or logopenic variants of primary progressive aphasia (PPA), and control speakers. Results revealed that the PPAOS group had longer durations and reduced rate of syllable production for most words and sentences, and the measure of lexical stress...
February 7, 2017: Brain and Language
https://www.readbyqxmd.com/read/28179468/neurofilament-as-a-blood-marker-for-diagnosis-and-monitoring-of-primary-progressive-aphasias
#15
Petra Steinacker, Elisa Semler, Sarah Anderl-Straub, Janine Diehl-Schmid, Matthias L Schroeter, Ingo Uttner, Hans Foerstl, Bernhard Landwehrmeyer, Christine A F von Arnim, Jan Kassubek, Patrick Oeckl, Hans-Jürgen Huppertz, Klaus Fassbender, Klaus Fliessbach, Johannes Prudlo, Carola Roßmeier, Johannes Kornhuber, Anja Schneider, Alexander E Volk, Martin Lauer, Adrian Danek, Albert C Ludolph, Markus Otto
OBJECTIVE: To assess the utility of serum neurofilament for diagnosis and monitoring of primary progressive aphasia (PPA) variants. METHODS: We investigated neurofilament light chain (NF-L) levels in blood of 99 patients with PPA (40 with nonfluent variant PPA [nfvPPA], 38 with semantic variant PPA [svPPA], 21 with logopenic variant PPA [lvPPA]) and compared diagnostic performance with that reached by CSF NF-L, phosphorylated neurofilament heavy chain (pNF-H), β-amyloid (Aβ1-42), tau, and phosphorylated tau...
March 7, 2017: Neurology
https://www.readbyqxmd.com/read/28153380/grn-deletion-in-familial-frontotemporal-dementia-showing-association-with-clinical-variability-in-3-familial-cases
#16
Graziella Milan, Sabrina Napoletano, Sabina Pappatà, Maria Teresa Gentile, Luca Colucci-D'Amato, Gennaro Della Rocca, Anna Maciag, Carmen Palermo Rossetti, Laura Fucci, Annibale Puca, Dario Grossi, Alfredo Postiglione, Emilia Vitale
Progranulin (GRN) gene mutations have been genetically associated with frontotemporal dementia (FTD) and are present in about 23% of patients with familial FTD. However, the neurobiology of this secreted glycoprotein remains unclear. Here, we report the identification of 3 pedigrees of Southern Italian extraction in whom FTD segregates with autosomal dominant inheritance patterns. We present evidence that all the available patients in these 3 familial cases are carrying the rare GRN gene exon 6 deletion g10325_10331delCTGCTGT (relative to nt 1 inNG_007886...
January 9, 2017: Neurobiology of Aging
https://www.readbyqxmd.com/read/28133816/typical-and-atypical-pathology-in-primary-progressive-aphasia-variants
#17
Edoardo G Spinelli, Maria Luisa Mandelli, Zachary A Miller, Miguel A Santos-Santos, Stephen M Wilson, Federica Agosta, Lea T Grinberg, Eric J Huang, John Q Trojanowski, Marita Meyer, Maya L Henry, Giancarlo Comi, Gil Rabinovici, Howard J Rosen, Massimo Filippi, Bruce L Miller, William W Seeley, Maria Luisa Gorno-Tempini
OBJECTIVE: To characterize in vivo signatures of pathological diagnosis in a large cohort of patients with primary progressive aphasia (PPA) variants defined by current diagnostic classification. METHODS: Extensive clinical, cognitive, neuroimaging, and neuropathological data were collected from 69 patients with sporadic PPA, divided into 29 semantic (svPPA), 25 non-fluent (nfvPPA), 11 logopenic (lvPPA), and 4 mixed PPA. Patterns of grey matter (GM) and white matter (WM) atrophy at presentation were assessed and tested as predictors of pathological diagnosis using support vector machine (SVM) algorithms...
January 30, 2017: Annals of Neurology
https://www.readbyqxmd.com/read/28120406/generalization-and-maintenance-of-treatment-gains-in-primary-progressive-aphasia-ppa-a-systematic-review
#18
REVIEW
Inês Cadório, Marisa Lousada, Paula Martins, Daniela Figueiredo
BACKGROUND: Cognitive-linguistic treatments and interventions targeting communication have been developed within the context of primary progressive aphasia (PPA), however knowledge about the scope of generalization and maintenance of therapy gains considering PPA subtypes remains scarce and awaits systematic investigation. AIMS: To analyse the effects of semantic therapy on generalization and maintenance of treatment outcomes in individuals with PPA, considering its different subtypes...
January 24, 2017: International Journal of Language & Communication Disorders
https://www.readbyqxmd.com/read/28106561/the-modified-frontal-behavioral-inventory-fbi-mod-for-patients-with-frontotemporal-lobar-degeneration-alzheimer-s-disease-and-mild-cognitive-impairment
#19
Noora-Maria Suhonen, Ilona Hallikainen, Tuomo Hänninen, Jari Jokelainen, Johanna Krüger, Anette Hall, Maria Pikkarainen, Hilkka Soininen, Anne M Remes
While behavioral symptoms are both early and prevalent features of behavioral variant frontotemporal dementia (bvFTD), they can be present in other types of dementia as well, including Alzheimer's disease (AD) and even mild cognitive impairment (MCI). The Frontal Behavioral Inventory (FBI) was specifically developed to capture the behavioral and personality changes in bvFTD; it has also been modified into a self-administered caregiver questionnaire (FBI-mod). We examined the utility of the FBI-mod in differentiating bvFTD (n = 26), primary progressive aphasia (PPA) (n = 7), AD (n = 53), and MCI (n = 50) patients, and investigated how the FBI-mod may be associated with neuropsychological measures...
2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28103593/executive-dysfunction-and-behavioral-symptoms-are-associated-with-deficits-in-instrumental-activities-of-daily-living-in-frontotemporal-dementia
#20
Negar Moheb, Mario F Mendez, Sarah A Kremen, Edmond Teng
BACKGROUND: Deficits in instrumental activities of daily living (ADLs) may be more prominent in behavioral variant frontotemporal dementia (bvFTD) than in nonfluent/agrammatic variant primary progressive aphasia (nfvPPA) or semantic variant primary progressive aphasia (svPPA). It is uncertain whether frontotemporal dementia (FTD) subgroups exhibit different patterns and/or predictors of functional impairment. METHODS: We examined data from participants diagnosed with bvFTD (n = 607), svPPA (n = 132), and nfvPPA (n = 155) who were included in the National Alzheimer's Coordinating Center (NACC) Uniform Data Set (UDS) and assessed with the Functional Activities Questionnaire (FAQ)...
2017: Dementia and Geriatric Cognitive Disorders
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