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Progressive nonfluent aphasia

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https://www.readbyqxmd.com/read/27920291/teaching-neuroimages-nonfluent-variant-primary-progressive-aphasia-a-distinctive-clinico-anatomical-syndrome
#1
Charles R Marshall, Christopher J D Hardy, Martin N Rossor, Jason D Warren
No abstract text is available yet for this article.
December 6, 2016: Neurology
https://www.readbyqxmd.com/read/27858708/cerebrospinal-fluid-biomarkers-as-a-diagnostic-tool-of-the-underlying-pathology-of-primary-progressive-aphasia
#2
George P Paraskevas, Dimitrios Kaselimis, Evie Kourtidou, Vasilios Constantinides, Anastasia Bougea, Costas Potagas, Ioannis Evdokimidis, Elisabeth Kapaki
BACKGROUND: Primary progressive aphasia (PPA) may present with three main clinical variants, namely nonfluent agrammatic (nfaPPA), semantic (sPPA), and logopenic (lPPA) subtypes. Frontotemporal lobar degenerations (FTLD) or Alzheimer's disease (AD) are the most common etiologies. OBJECTIVE: To study the potential of cerebrospinal fluid (CSF) biomarkers for identifying the underlying pathology in patients with PPA. METHODS: CSF levels of total tau protein (τT), amyloid-β peptide (Aβ42), and tau phosphorylated at threonine-181 (τP - 181) were measured by double sandwich, enzyme-linked immunosorbent assay (ELISA) in 43 patients with PPA, 26 patients with AD, and 17 healthy controls...
November 14, 2016: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/27802226/music-perception-in-dementia
#3
Hannah L Golden, Camilla N Clark, Jennifer M Nicholas, Miriam H Cohen, Catherine F Slattery, Ross W Paterson, Alexander J M Foulkes, Jonathan M Schott, Catherine J Mummery, Sebastian J Crutch, Jason D Warren
Despite much recent interest in music and dementia, music perception has not been widely studied across dementia syndromes using an information processing approach. Here we addressed this issue in a cohort of 30 patients representing major dementia syndromes of typical Alzheimer's disease (AD, n = 16), logopenic aphasia (LPA, an Alzheimer variant syndrome; n = 5), and progressive nonfluent aphasia (PNFA; n = 9) in relation to 19 healthy age-matched individuals. We designed a novel neuropsychological battery to assess perception of musical patterns in the dimensions of pitch and temporal information (requiring detection of notes that deviated from the established pattern based on local or global sequence features) and musical scene analysis (requiring detection of a familiar tune within polyphonic harmony)...
October 11, 2016: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/27790240/lack-of-frank-agrammatism-in-the-nonfluent-agrammatic-variant-of-primary-progressive-aphasia
#4
Naida L Graham, Carol Leonard, David F Tang-Wai, Sandra Black, Tiffany W Chow, Chris J M Scott, Alicia A McNeely, Mario Masellis, Elizabeth Rochon
BACKGROUND/AIMS: Frank agrammatism, defined as the omission and/or substitution of grammatical morphemes with associated grammatical errors, is variably reported in patients with nonfluent variant primary progressive aphasia (nfPPA). This study addressed whether frank agrammatism is typical in agrammatic nfPPA patients when this feature is not required for diagnosis. METHOD: We assessed grammatical production in 9 patients who satisfied current diagnostic criteria...
September 2016: Dementia and Geriatric Cognitive Disorders Extra
https://www.readbyqxmd.com/read/27581216/serum-neurofilament-light-chain-protein-is-a-measure-of-disease-intensity-in-frontotemporal-dementia
#5
Jonathan D Rohrer, Ione O C Woollacott, Katrina M Dick, Emilie Brotherhood, Elizabeth Gordon, Alexander Fellows, Jamie Toombs, Ronald Druyeh, M Jorge Cardoso, Sebastien Ourselin, Jennifer M Nicholas, Niklas Norgren, Simon Mead, Ulf Andreasson, Kaj Blennow, Jonathan M Schott, Nick C Fox, Jason D Warren, Henrik Zetterberg
OBJECTIVE: To investigate serum neurofilament light chain (NfL) concentrations in frontotemporal dementia (FTD) and to see whether they are associated with the severity of disease. METHODS: Serum samples were collected from 74 participants (34 with behavioral variant FTD [bvFTD], 3 with FTD and motor neuron disease and 37 with primary progressive aphasia [PPA]) and 28 healthy controls. Twenty-four of the FTD participants carried a pathogenic mutation in C9orf72 (9), microtubule-associated protein tau (MAPT; 11), or progranulin (GRN; 4)...
September 27, 2016: Neurology
https://www.readbyqxmd.com/read/27524196/-profiles-of-cognitive-and-language-impairment-of-logopenic-and-non-fluent-variant-of-primary-progressive-aphasia
#6
D Li, L N Zhao, H M Jin, M Zhang, D M Guo, Y Y Yu, L Y Wu, Y Tang, F Y Li, A H Zhou, Y Han, J P Jia
OBJECTIVE: To decipher the cognitive and linguistic feature of logopenic variant primary progressive aphasia (lv-PPA) and nonfluent variant primary progressive aphasia (nfv-PPA) and to explore the extent to which cognitive and language impairment contribute to the dysfunction of activity of daily living(ADL). METHODS: Seven lv-PPA and five nfv-PPA were enrolled in memory clinic of Xuanwu Hospital, Capital Medical University from January 2015 to January 2016 accordig to the international consensus criteria for PPA and its three subtypes...
August 2, 2016: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/27509365/atrophy-and-structural-covariance-of-the-cholinergic-basal-forebrain-in-primary-progressive-aphasia
#7
Stefan Teipel, Theresa Raiser, Lina Riedl, Isabelle Riederer, Matthias L Schroeter, Sandrine Bisenius, Anja Schneider, Johannes Kornhuber, Klaus Fliessbach, Annika Spottke, Michel J Grothe, Johannes Prudlo, Jan Kassubek, Albert Ludolph, Bernhard Landwehrmeyer, Sarah Straub, Markus Otto, Adrian Danek
Primary progressive aphasia (PPA) is characterized by profound destruction of cortical language areas. Anatomical studies suggest an involvement of cholinergic basal forebrain (BF) in PPA syndromes, particularly in the area of the nucleus subputaminalis (NSP). Here we aimed to determine the pattern of atrophy and structural covariance as a proxy of structural connectivity of BF nuclei in PPA variants. We studied 62 prospectively recruited cases with the clinical diagnosis of PPA and 31 healthy older control participants from the cohort study of the German consortium for frontotemporal lobar degeneration (FTLD)...
October 2016: Cortex; a Journal Devoted to the Study of the Nervous System and Behavior
https://www.readbyqxmd.com/read/27439681/frontotemporal-dementia-related-gene-mutations-in-clinical-dementia-patients-from-a-chinese-population
#8
Zhihong Shi, Shuai Liu, Lei Xiang, Ying Wang, Mengyuan Liu, Shuling Liu, Tong Han, Yuying Zhou, Jinhuan Wang, Li Cai, Shuo Gao, Yong Ji
Alzheimer's disease (AD) and frontotemporal dementia (FTD) are two common forms of primary neurodegenerative dementia that show overlapping clinical symptoms. The aim of this study was to perform genetic analyses on GRN, VCP, CHMP2B, FUS, TARDBP, C9orf72 and MAPT genes in Chinese AD and FTD patients. We performed gene sequencing of the GRN, VCP, CHMP2B, FUS, TARDBP, MAPT and C9orf72 genes in 61 clinical AD and 38 FTD Chinese patients. We identified a known mutation of MAPT (p.Pro301Leu, c.902C>T) in four patients from an autosomal dominant FTD family with behavioral variant FTD (bvFTD) and progressive nonfluent aphasia (PNFA) phenotypes, and a novel mutation in MAPT (p...
July 21, 2016: Journal of Human Genetics
https://www.readbyqxmd.com/read/27423618/a-family-with-hereditary-diffuse-leukoencephalopathy-with-spheroids-caused-by-a-novel-c-2442-2t-c-mutation-in-the-csf1r-gene
#9
Ito Kawakami, Eizo Iseki, Koji Kasanuki, Michiko Minegishi, Kiyoshi Sato, Hiroyuki Hino, Katsuhiko Shibuya, Kohshiro Fujisawa, Shinji Higashi, Haruhiko Akiyama, Akiko Furuta, Masashi Takanashi, Yuanzhe Li, Nobutaka Hattori, Yoshio Mitsuyama, Heii Arai
Clinical phenotypes of hereditary diffuse leukoencephalopathy with spheroids (HDLS), a familial progressive neurodegenerative disorder affecting the white matter of the brain, are heterogenous and may include behavioral and personality changes, memory impairment, parkinsonism, seizure, and spasticity. Thus, HDLS is frequently unrecognized and misdiagnosed. Heterozygous mutations located within the kinase domain of the gene encoding the colony-stimulating factor 1 receptor (CSF1R), a cell surface receptor with key roles in development and innate immunity, have been shown in HDLS...
August 15, 2016: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/27340847/comparing-longitudinal-behavior-changes-in-the-primary-progressive-aphasias
#10
Tim Van Langenhove, Cristian E Leyton, Olivier Piguet, John R Hodges
BACKGROUND: Differentiating between primary progressive aphasia (PPA) variants based on the profile of language deficits can be difficult in a proportion of patients. Further, little is presently know about the pattern of longitudinal changes in behavior in PPA variants. OBJECTIVE: To determine the presence of behavioral changes in the main variants of PPA: semantic (sv-PPA), nonfluent/agrammatic (nfv-PPA), and logopenic (lv-PPA), and establish the course of these changes over time...
June 18, 2016: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/27311648/analyses-mapt-grn-and-c9orf72-mutations-in-chinese-patients-with-frontotemporal-dementia
#11
Min Tang, Xiaohua Gu, Jingya Wei, Bin Jiao, Lin Zhou, Yafang Zhou, Ling Weng, Xinxiang Yan, Beisha Tang, Jun Xu, Lu Shen
Frontotemporal dementia (FTD) is a clinically heterogeneous neurodegenerative disorder, including behavior behavioral variant FTD (bvFTD), semantic dementia, progressive nonfluent aphasia (PNFA), FTD-parkinsonism, and FTD-motor neuron disease. To date, there are at least 8 causative genes identified in patients with FTD. Among them, variants in the microtubule-associated protein tau (MAPT), GRN, and chromosome 9 open-reading frame 72 (C9orf72) genes are considered the major cause of FTD. To date, no comprehensive analyses of mutations in these 3 genes have been conducted in the Chinese population...
October 2016: Neurobiology of Aging
https://www.readbyqxmd.com/read/27195001/brain-perfusion-in-corticobasal-syndrome-with-progressive-aphasia
#12
Yoshitake Abe, Noriyuki Kimura, Megumi Goto, Yasuhiro Aso, Etsuro Matsubara
BACKGROUND: Brain perfusion may differ between patients with corticobasal syndrome (CBS) with and without aphasia. METHODS: Twenty-six (9 males and 17 females; mean age 76 ± 5.3 years) patients with CBS were enrolled in the study. Brain MRI and single-photon emission computed tomography were performed in all subjects. Language was evaluated using the Standard Language Test of Aphasia. The patients were divided into two subgroups according to the presence or absence of progressive aphasia...
January 2016: Dementia and Geriatric Cognitive Disorders Extra
https://www.readbyqxmd.com/read/27194988/a-dextral-primary-progressive-aphasia-patient-with-right-dominant-hypometabolism-and-tau-accumulation-and-left-dominant-amyloid-accumulation
#13
Young Kyoung Jang, Seongbeom Park, Hee Jin Kim, Hanna Cho, Chul Hyoung Lyoo, Sang Won Seo, Duk L Na
BACKGROUND: Primary progressive aphasia (PPA) is a degenerative disease that presents as progressive decline of language ability with preservation of other cognitive functions in the early stages. Three subtypes of PPA are known: progressive nonfluent aphasia, semantic dementia, and logopenic aphasia (LPA). PATIENTS AND METHODS: We report the case of a 77-year-old patient with PPA whose clinical findings did not correspond to the three subtypes but mainly fit LPA...
January 2016: Case Reports in Neurology
https://www.readbyqxmd.com/read/27111692/features-of-patients-with-nonfluent-agrammatic-primary-progressive-aphasia-with-underlying-progressive-supranuclear-palsy-pathology-or-corticobasal-degeneration
#14
Miguel A Santos-Santos, Maria Luisa Mandelli, Richard J Binney, Jennifer Ogar, Stephen M Wilson, Maya L Henry, H Isabel Hubbard, Minerva Meese, Suneth Attygalle, Lynne Rosenberg, Mikhail Pakvasa, John Q Trojanowski, Lea T Grinberg, Howie Rosen, Adam L Boxer, Bruce L Miller, William W Seeley, Maria Luisa Gorno-Tempini
IMPORTANCE: We provide novel evidence of specific clinical and neuroimaging features that may help for the in vivo prediction of underlying pathology in patients with nonfluent/agrammatic primary progressive aphasia (nfvPPA) and progressive supranuclear palsy (PSP) or corticobasal degeneration (CBD) proved by autopsy. OBJECTIVE: To characterize the neurological, cognitive, and neuroimaging features of patients with nfvPPA-in whom either PSP or CBD was eventually confirmed at autopsy-at initial presentation and at 1-year follow-up...
June 1, 2016: JAMA Neurology
https://www.readbyqxmd.com/read/27103514/syntactic-comprehension-deficits-across-the-ftd-als-continuum
#15
Jody Kamminga, Felicity V C Leslie, Sharpley Hsieh, Jashelle Caga, Eneida Mioshi, Michael Hornberger, Kirrie J Ballard, Matthew C Kiernan, John R Hodges, James R Burrell
To establish the frequency, severity, relationship to bulbar symptoms, and neural correlates of syntactic comprehension deficits across the frontotemporal dementia-amyotrophic lateral sclerosis (FTD-ALS) disease spectrum. In total, 85 participants were included in the study; 20 amyotrophic lateral sclerosis (ALS), 15 FTD-ALS, 27 progressive nonfluent aphasia (PNFA), and 23 controls. Syntactic comprehension was evaluated in ALS, FTD-ALS, PNFA, and controls using the Test for Reception of Grammar. Voxel-based morphometry examined neuroanatomical correlates of performance...
May 2016: Neurobiology of Aging
https://www.readbyqxmd.com/read/27097664/classification-of-the-primary-progressive-aphasias-principles-and-review-of-progress-since-2011
#16
REVIEW
Rik Vandenberghe
Highly influential recommendations published in 2011 for the classification of the primary progressive aphasias (PPA) distinguished three subtypes: the semantic variant, the nonfluent/agrammatic variant, and the logopenic variant. We review empirical evidence published after 2011 that bears relevance to the validity of the recommended classification scheme. The studies that we review principally rely on monocentric, memory clinic-based consecutive series of PPA patients. We review whether a data-driven analysis of neurolinguistic test scores confirms the subtyping that was based on expert consensus, whether the 2011 subtyping covers the diversity of PPA in a comprehensive manner, and whether the proposed subgroups differ along dimensions that are not explicitly part of the defining criteria, such as diffusion tractography...
April 21, 2016: Alzheimer's Research & Therapy
https://www.readbyqxmd.com/read/27087732/telerehabilitation-of-anomia-in-primary-progressive-aphasia
#17
Aaron M Meyer, Heidi R Getz, David M Brennan, Tang M Hu, Rhonda B Friedman
BACKGROUND: The efficacy of telerehabilitation-based treatment for anomia has been demonstrated in post-stroke aphasia, but the efficacy of this method of anomia treatment delivery has not been established within the context of degenerative illness. AIMS: The current study evaluated the feasibility and efficacy of a telerehabilitation-based approach to anomia treatment within the three subtypes of primary progressive aphasia (PPA). METHODS & PROCEDURES: Each of the three telerehabilitation participants represented a distinct subtype of PPA...
April 1, 2016: Aphasiology
https://www.readbyqxmd.com/read/27082848/grn-and-mapt-mutations-in-2-frontotemporal-dementia-research-centers-in-brazil
#18
Leonel T Takada, Valeria S Bahia, Henrique C Guimarães, Thais V M M Costa, Thiago C Vale, Roberta D Rodriguez, Fabio H G Porto, João C B Machado, Rogério G Beato, Karolina G Cesar, Jerusa Smid, Camila F Nascimento, Lea T Grinberg, Sonia M D Brucki, Jessica R Maximino, Sarah T Camargos, Gerson Chadi, Paulo Caramelli, Ricardo Nitrini
BACKGROUND: Mutations in GRN (progranulin) and MAPT (microtubule-associated protein tau) are among the most frequent causes of monogenic frontotemporal dementia (FTD), but data on the frequency of these mutations in regions such as Latin America are still lacking. OBJECTIVE: We aimed to investigate the frequencies of GRN and MAPT mutations in FTD cohorts from 2 Brazilian dementia research centers, the University of Sao Paulo and the Federal University of Minas Gerais medical schools...
October 2016: Alzheimer Disease and Associated Disorders
https://www.readbyqxmd.com/read/27042904/frontotemporal-dementias
#19
Elizabeth C Finger
PURPOSE OF REVIEW: This article reviews the common behavioral and cognitive features of frontotemporal dementia (FTD) and related disorders as well as the distinguishing clinical, genetic, and pathologic features of the most common subtypes. RECENT FINDINGS: Advances in clinical phenotyping, genetics, and biomarkers have enabled improved predictions of the specific underlying molecular pathology associated with different presentations of FTD. Evaluation of large international cohorts has led to recent refinements in diagnostic criteria for several of the FTD subtypes...
April 2016: Continuum: Lifelong Learning in Neurology
https://www.readbyqxmd.com/read/27025090/-pick-s-disease
#20
Kazuki Yamamoto, Tomomi Ogihara
Pick's disease is a type of frontotemporal lobar degeneration(FTLD) with circumscribed atrophy in the frontotemporal lobe. The terminology for Pick's disease has evolved over time. Pick's disease was a term formerly used to define a disorder with symptoms caused by frontal and temporal lobe dysfunction. Therefore, the diagnosis was previously based on clinical features and the distribution of brain atrophy. Pick's disease is currently defined by the presence of tau-positive Pick bodies, and thus can be diagnosed only pathologically...
March 2016: Nihon Rinsho. Japanese Journal of Clinical Medicine
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