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Agrammatic variant

Eloi Magnin, Jean-François Démonet, David Wallon, Julien Dumurgier, Anne-Cécile Troussière, Alain Jager, Emmanuelle Duron, Audrey Gabelle, Vincent de la Sayette, Lisette Volpe-Gillot, Gregory Tio, Sarah Evain, Claire Boutoleau-Bretonnière, Adeline Enderle, François Mouton-Liger, Philippe Robert, Didier Hannequin, Florence Pasquier, Jacques Hugon, Claire Paquet
BACKGROUND: Few demographical data about primary progressive aphasia (PPA) are available, and most knowledge regarding PPA is based on tertiary centers' results. OBJECTIVE: Our aims were to describe demographical characteristics of the PPA population in a large sample of PPA patients from the network of French Alzheimer plan memory centers (Sample 1), and to describe the stratification of cerebrospinal fluid (CSF) biomarkers in two different samples of PPA patients (Samples 2 and 3)...
October 18, 2016: Journal of Alzheimer's Disease: JAD
Sara M G Cioffi, Daniela Galimberti, Federica Barocco, Marco Spallazzi, Chiara Fenoglio, Maria Serpente, Marina Arcaro, Simona Gardini, Elio Scarpini, Paolo Caffarra
Mutations in progranulin gene (GRN) are a common cause of autosomal dominant frontotemporal lobar degeneration syndromes and are associated with a wide phenotypic heterogeneity. The majority of genetic defects in GRN consists of loss-of-function mutations, causing haploinsufficiency, and is associated with extremely low plasma progranulin levels. Herein, we describe a patient who developed language dysfunctions and memory disturbances at 63 years of age. Considering the early onset and the positive family history (sister aged 50 with non-fluent/agrammatic variant of primary progressive aphasia, father with behavioral disturbances in his sixties), a genetic analysis was carried out, showing the presence of a novel mutation [g...
September 6, 2016: Journal of Alzheimer's Disease: JAD
Emily Rogalski, Jaiashre Sridhar, Benjamin Rader, Adam Martersteck, Kewei Chen, Derin Cobia, Cynthia K Thompson, Sandra Weintraub, Eileen H Bigio, M-Marsel Mesulam
OBJECTIVE: To identify features of primary progressive aphasia (PPA) associated with Alzheimer disease (AD) neuropathology. A related objective was to determine whether logopenic PPA is a clinical marker for AD. METHODS: A total of 139 prospectively enrolled participants with a root diagnosis of PPA constituted the reference set. Those with autopsy or biomarker evidence of AD, and who had been evaluated at mild disease stages (Aphasia Quotient ≥85), were included (n = 19)...
September 27, 2016: Neurology
Pawel Tacik, Michael A DeTure, Yari Carlomagno, Wen-Lang Lin, Melissa E Murray, Matthew C Baker, Keith A Josephs, Bradley F Boeve, Zbigniew K Wszolek, Neill R Graff-Radford, Joseph E Parisi, Leonard Petrucelli, Rosa Rademakers, Richard S Isaacson, Kenneth M Heilman, Ronald C Petersen, Dennis W Dickson, Naomi Kouri
Mutations in microtubule-associated protein tau gene (MAPT) cause frontotemporal dementia with parkinsonism linked to chromosome 17 (FTDP-17). Here, we describe a patient with FTDP-17 and a novel missense mutation in exon 13 of MAPT, p.E372G. We compare clinicopathologic features of this patient to two previously unreported patients with another exon 13 mutation, p.G389R. The patient with the p.E372G mutation was a 40-year-old man with behavioral variant frontotemporal dementia (bvFTD), who subsequently developed agrammatic speech and parkinsonism...
October 5, 2016: Brain Pathology
Maria Luisa Mandelli, Eduard Vilaplana, Jesse A Brown, H Isabel Hubbard, Richard J Binney, Suneth Attygalle, Miguel A Santos-Santos, Zachary A Miller, Mikhail Pakvasa, Maya L Henry, Howard J Rosen, Roland G Henry, Gil D Rabinovici, Bruce L Miller, William W Seeley, Maria Luisa Gorno-Tempini
Neurodegeneration has been hypothesized to follow predetermined large-scale networks through the trans-synaptic spread of toxic proteins from a syndrome-specific epicentre. To date, no longitudinal neuroimaging study has tested this hypothesis in vivo in frontotemporal dementia spectrum disorders. The aim of this study was to demonstrate that longitudinal progression of atrophy in non-fluent/agrammatic variant primary progressive aphasia spreads over time from a syndrome-specific epicentre to additional regions, based on their connectivity to the epicentre in healthy control subjects...
August 6, 2016: Brain: a Journal of Neurology
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
Maria Cotelli, Rosa Manenti, Donata Paternicò, Maura Cosseddu, Michela Brambilla, Michela Petesi, Enrico Premi, Roberto Gasparotti, Orazio Zanetti, Alessandro Padovani, Barbara Borroni
Agrammatic variant primary progressive aphasia is a neurodegenerative disorder specifically characterized by language deficits. A recent study has demonstrated a beneficial effect of transcranial direct current stimulation (tDCS) in combination with language training on naming accuracy in these patients. The aim of the study was to evaluate whether the improvement of naming accuracy after tDCS during language training was related to regional grey matter (GM) density. Eighteen avPPA patients underwent a brain magnetic resonance imaging before receiving a treatment that consisted of tDCS over the left dorsolateral prefrontal cortex during individualized language training (10 daily therapy sessions, 5 days per week from Monday to Friday)...
September 2016: Brain Topography
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...
2016: Alzheimer's Research & Therapy
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
Manon Grube, Rose Bruffaerts, Jolien Schaeverbeke, Veerle Neyens, An-Sofie De Weer, Alexandra Seghers, Bruno Bergmans, Eva Dries, Timothy D Griffiths, Rik Vandenberghe
The extent to which non-linguistic auditory processing deficits may contribute to the phenomenology of primary progressive aphasia is not established. Using non-linguistic stimuli devoid of meaning we assessed three key domains of auditory processing (pitch, timing and timbre) in a consecutive series of 18 patients with primary progressive aphasia (eight with semantic variant, six with non-fluent/agrammatic variant, and four with logopenic variant), as well as 28 age-matched healthy controls. We further examined whether performance on the psychoacoustic tasks in the three domains related to the patients' speech and language and neuropsychological profile...
June 2016: Brain: a Journal of Neurology
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
Mika Otsuki
This article provides an overview of the historical entity hitherto, the pivotal clinical symptoms of FTLD (frontotemporal lobar degeneration), and an introduction for the revised criteria for bvFTD (behavioral variant frontotemporal dementia): FTDC (International Behavioral Variant FTD Criteria Consortium) presented by Rascovsky et al(2011), and the classification criteria for PPA(primary progressive aphasia) heralded by Gorno-Tempini et al (2011). According to the former criteria, bvFTD can be diagnosed by the clinical symptoms as having possible bvFTD or probable bvFTD, and the pathological findings could lead definite bvFTD...
March 2016: Nihon Rinsho. Japanese Journal of Clinical Medicine
Jolanta Góral-Półrola, Paweł Półrola, Natalia Mirska, Andrzej Mirski, Izabela Herman-Sucharska, Maria Pąchalska
INTRODUCTION: The paper presents an example of the successful administration of the Augmentative and Alternative Communication (AAC) system. Such an approach is of particular significance in cases of patients with speech and language deterioration, which is observed in a nonfluent/agrammatic variant of primary progressive aphasia (PPA-G). Regaining the ability to communicate with others proves to be very important for the patients' self-esteem and enables them to restore previously broken social bonds...
2016: Annals of Agricultural and Environmental Medicine: AAEM
Raffaella Migliaccio, Claire Boutet, Romain Valabregue, Sophie Ferrieux, Marie Nogues, Stéphane Lehéricy, Didier Dormont, Richard Levy, Bruno Dubois, Marc Teichmann
OBJECTIVE: Word finding depends on the processing of semantic and lexical information, and it involves an intermediate level for mapping semantic-to-lexical information which also subserves lexical-to-semantic mapping during word comprehension. However, the brain regions implementing these components are still controversial and have not been clarified via a comprehensive lesion model encompassing the whole range of language-related cortices. Primary progressive aphasia (PPA), for which anomia is thought to be the most common sign, provides such a model, but the exploration of cortical areas impacting naming in its three main variants and the underlying processing mechanisms is still lacking...
2016: PloS One
Siddharth Ramanan, Emma Flanagan, Cristian E Leyton, Victor L Villemagne, Christopher C Rowe, John R Hodges, Michael Hornberger
Diagnostic distinction of primary progressive aphasias (PPA) remains challenging, in particular for the logopenic (lvPPA) and nonfluent/agrammatic (naPPA) variants. Recent findings highlight that episodic memory deficits appear to discriminate these PPA variants from each other, as only lvPPA perform poorly on these tasks while having underlying amyloid pathology similar to that seen in amnestic dementias like Alzheimer's disease (AD). Most memory tests are, however, language based and thus potentially confounded by the prevalent language deficits in PPA...
2016: Journal of Alzheimer's Disease: JAD
Sheilla de Medeiros Correia Marin, Paulo Henrique Ferreira Bertolucci, Luis Fabiano Marin, Fabricio Ferreira de Oliveira, Jose Roberto Wajman, Valéria Santoro Bahia, Letícia Lessa Mansur
BACKGROUND: Few studies have described characteristics of swallowing in primary progressive aphasia (PPA) and its variants. OBJECTIVE: To describe and characterize swallowing and eating behaviors of patients with PPA, as well as their correlates with neuropsychiatric symptoms and patterns of communication. METHODS: We studied 16 patients with PPA and 16 their caregivers. PPA was subdivided in agrammatic variant (PPA-G), semantic variant (PPA-S) and logopenic variant (PPA-L)...
2016: NeuroRehabilitation
Takayoshi Shimohata, Ikuko Aiba, Masatoyo Nishizawa
Experts use the term corticobasal syndrome (CBS) for patients with a clinical diagnosis of corticobasal degeneration (CBD), and reserve CBD for those whose conditions have been diagnosed on the basis of neuropathological analyses. Several studies demonstrated that patients with CBD may also present with progressive supranuclear syndrome (PSPS), aphasia, Alzheimer disease-like dementia or behavioral change, suggesting that CBS is merely one of the presenting phenotypes of CBD. Although previous CBD diagnostic criteria reflected only CBS, the international consortium proposed new diagnostic criteria for CBD in 2013 (Armstrong's criteria)...
2016: Rinshō Shinkeigaku, Clinical Neurology
Cristian E Leyton, Anna K Britton, John R Hodges, Glenda M Halliday, Jillian J Kril
Primary progressive aphasia (PPA) comprises a heterogeneous group of neurodegenerative conditions that can be classified in three cliniconeuroanatomic syndromes. Limited information exists, however, about patterns of neuropathologic spreading and microscopic changes underpinning each syndrome. We performed an analysis of a longitudinal in vivo cohort and a postmortem PPA cohort to investigate neurodegeneration over time and to quantify microscopic changes in key language brain areas. The longitudinal analyses demonstrated distinctive patterns of topological extension of brain atrophy...
February 2016: Neurobiology of Aging
Maria Luisa Mandelli, Paolo Vitali, Miguel Santos, Maya Henry, Kelly Gola, Lynne Rosenberg, Nina Dronkers, Bruce Miller, William W Seeley, Maria Luisa Gorno-Tempini
The non-fluent/agrammatic variant of primary progressive aphasia (nfvPPA) and the behavioral variant frontotemporal dementia (bvFTD) are focal neurodegenerative disorders belonging to the FTD-spectrum clinical syndromes. NfvPPA is characterized by effortful speech and/or agrammatism and left frontal atrophy, while bvFTD is characterized by social-emotional dysfunction often accompanied by right-lateralized frontal damage. Despite their contrasting clinical presentations, both disorders show prominent left anterior insula atrophy...
January 2016: Cortex; a Journal Devoted to the Study of the Nervous System and Behavior
Maya L Henry, Stephen M Wilson, Miranda C Babiak, Maria Luisa Mandelli, Pelagie M Beeson, Zachary A Miller, Maria Luisa Gorno-Tempini
Individuals with primary progressive aphasia (PPA) show selective breakdown in regions within the proposed dorsal (articulatory-phonological) and ventral (lexical-semantic) pathways involved in language processing. Phonological STM impairment, which has been attributed to selective damage to dorsal pathway structures, is considered to be a distinctive feature of the logopenic variant of PPA. By contrast, phonological abilities are considered to be relatively spared in the semantic variant and are largely unexplored in the nonfluent/agrammatic variant...
February 2016: Journal of Cognitive Neuroscience
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