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Primary progressive aphasia

Nikos Makris, A Zhu, G M Papadimitriou, P Mouradian, I Ng, E Scaccianoce, G Baselli, F Baglio, M E Shenton, Y Rathi, B Dickerson, E Yeterian, M Kubicki
Originally, the middle longitudinal fascicle (MdLF) was defined as a long association fiber tract connecting the superior temporal gyrus and temporal pole with the angular gyrus. More recently its description has been expanded to include all long postrolandic cortico-cortical association connections of the superior temporal gyrus and dorsal temporal pole with the parietal and occipital lobes. Despite its location and size, which makes MdLF one of the most prominent cerebral association fiber tracts, its discovery in humans is recent...
October 6, 2016: Brain Imaging and Behavior
Claire M O'Connor, Lindy Clemson, Emma Flanagan, Cassandra Kaizik, Henry Brodaty, John R Hodges, Olivier Piguet, Eneida Mioshi
BACKGROUND: The contribution of behavioural changes to functional decline is yet to be explored in primary progressive aphasia (PPA). OBJECTIVES: (1) investigate functional changes in two PPA variants [semantic (svPPA) and non-fluent (nfvPPA)], at baseline and after 12 months; (2) investigate baseline differences in behavioural changes between groups, and (3) explore predictors of functional decline after a 12-month period. METHODS: A longitudinal study involving 29 people with PPA (18 svPPA; 11 nfvPPA) seen annually in Sydney/Australia was conducted...
September 30, 2016: Dementia and Geriatric Cognitive Disorders
Mezgebe D Abegaz, Anjum Sayyad
No abstract text is available yet for this article.
September 2016: PM & R: the Journal of Injury, Function, and Rehabilitation
Chiara Cerami, Alessandra Dodich, Lucia Greco, Sandro Iannaccone, Giuseppe Magnani, Alessandra Marcone, Elisabetta Pelagallo, Roberto Santangelo, Stefano F Cappa, Daniela Perani
BACKGROUND AND OBJECTIVE: Primary progressive aphasia (PPA) is a clinical syndrome due to different neurodegenerative conditions in which an accurate early diagnosis needs to be supported by a reliable diagnostic tool at the individual level. In this study, we investigated in PPA the FDG-PET brain metabolic patterns at the single-subject level, in order to assess the case-to-case variability and its relationship with clinical-neuropsychological findings. MATERIAL AND METHODS: 55 patients (i...
September 20, 2016: Journal of Alzheimer's Disease: JAD
Raffaella I Rumiati, Francesco Foroni, Giulio Pergola, Paola Rossi, Maria Caterina Silveri
The study of category specific deficits in brain-damaged patients has been instrumental in explaining how knowledge about different types of objects is organized in the brain. Much of this research focused on testing putative semantic sensory/functional subsystems that could explain the observed dissociations in performance between living things (e.g., animals and fruits/vegetables) and non-living things (e.g., tools). As neuropsychological patterns that did not fit the original living/non-living distinction were observed, an alternative organization of semantic memory in domains constrained by evolutionary pressure was hypothesized...
September 17, 2016: Brain and Cognition
Joanna J Gan, Andrew Lin, Mersal S Samimi, Mario F Mendez
BACKGROUND: Semantic dementia (SD) is a neurodegenerative disorder characterized by loss of semantic knowledge. SD may be associated with somatic symptom disorder due to excessive preoccupation with unidentified somatic sensations. OBJECTIVE: To evaluate the frequency of somatic symptom disorder among patients with SD in comparison to comparably demented patients with Alzheimer׳s disease. METHODS: A retrospective cohort study was conducted using clinical data from a referral-based behavioral neurology program...
August 4, 2016: Psychosomatics
Damith Woods, Schwanagorn Sirirat, Sirirada Pattara-Angkoon, Janja Rattanajan
We report the clinical assessment of J.P., an 86 year-old man with Broca's aphasia complaining of memory problems. Our aim was to objectively investigate his level of cognitive functioning using standardized neuropsychological tests in order to determine the nature of his memory impairment. J.P.'s medical history included left-middle cerebral artery (left-MCA) stroke, high frequency hearing loss, macular degeneration, and a recent hospitalization related to a fall. Results from his neuropsychological testing and from information gathered during the clinical interview with his wife suggested that a deficit in executive functioning might have been the source for some of his perceived memory problems...
September 13, 2016: Applied Neuropsychology. Adult
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
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
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
Stephen M Wilson, Andrew T DeMarco, Maya L Henry, Benno Gesierich, Miranda Babiak, Bruce L Miller, Maria Luisa Gorno-Tempini
Syntactic processing deficits are highly variable in individuals with primary progressive aphasia. Damage to left inferior frontal cortex has been associated with syntactic deficits in primary progressive aphasia in a number of structural and functional neuroimaging studies. However, a contrasting picture of a broader syntactic network has emerged from neuropsychological studies in other aphasic cohorts, and functional imaging studies in healthy controls. To reconcile these findings, we used functional magnetic resonance imaging to investigate the functional neuroanatomy of syntactic comprehension in 51 individuals with primary progressive aphasia, composed of all clinical variants and a range of degrees of syntactic processing impairment...
August 23, 2016: Brain: a Journal of Neurology
Marc Teichmann, Constance Lesoil, Juliette Godard, Marine Vernet, Anne Bertrand, Richard Levy, Bruno Dubois, Laurie Lemoine, Dennis Q Truong, Marom Bikson, Aurélie Kas, Antoni Valero-Cabré
OBJECTIVE: Noninvasive brain stimulation in primary progressive aphasia (PPA) is a promising approach. Yet, applied to single cases or insufficiently controlled small-cohort studies, it has not clarified its therapeutic value. We here address the effectiveness of transcranial direct current stimulation (tDCS) on the semantic PPA variant (sv-PPA), applying a rigorous study design to a large, homogeneous sv-PPA cohort. METHODS: Using a double-blind, sham-controlled counterbalanced cross-over design, we applied three tDCS conditions targeting the temporal poles of 12 sv-PPA patients...
August 23, 2016: Annals of Neurology
R A Armstrong
Factors associated with survival were studied in 84 neuropathologically documented cases of the pre-senile dementia frontotemporal dementia lobar degeneration (FTLD) with transactive response (TAR) DNA-binding protein of 43 kDa (TDP-43) proteinopathy (FTLD-TDP). Kaplan-Meier survival analysis estimated mean survival as 7.9 years (range: 1-19 years, SD = 4.64). Familial and sporadic cases exhibited similar survival, including progranulin (GRN) gene mutation cases. No significant differences in survival were associated with sex, disease onset, Braak disease stage, or disease subtype, but higher survival was associated with lower post-mortem brain weight...
2016: Folia Neuropathologica
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
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]
Felix Gervits, Sharon Ash, H Branch Coslett, Katya Rascovsky, Murray Grossman, Roy Hamilton
Primary progressive aphasia (PPA) is a neurodegenerative condition characterized by gradual deterioration of language function. We investigated whether two weeks of daily transcranial direct current stimulation (tDCS) treatment would improve language abilities in six people with a non-fluent form of PPA. tDCS was applied in an unblinded trial at an intensity of 1.5mA for 20min/day over 10days. At the time of stimulation, patients were engaged in narrating one of several children's wordless picture stories. A battery of neuropsychological assessments was administered four times: at baseline, immediately following the 2-week stimulation period, and then 6-weeks and 12-weeks following the end of stimulation...
August 11, 2016: Brain and Language
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
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
Stuart J McCarter, Erik K St Louis, Bradley F Boeve
Sleep disorders appear to be frequent comorbidities in patients with frontotemporal dementia (FTD). Insomnia and excessive daytime sleepiness commonly occur in patients with FTD and significantly contribute to caregiver burden and burnout. Sleep is severely fragmented in FTD patients, likely secondary to behavioral disturbances, other primary sleep disorders such as sleep disordered breathing and restless leg syndrome, and neurodegeneration of nuclei involved in sleep and wakefulness. Treatment of primary sleep disorders may improve excessive daytime sleepiness and sleep quality and may improve daytime cognitive functioning...
September 2016: Current Neurology and Neuroscience Reports
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