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Progressive aphasia

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https://www.readbyqxmd.com/read/28326689/behavioural-and-neuropsychiatric-disturbance-in-three-clinical-subtypes-of-frontotemporal-dementia-a-clinical-research-center-for-dementia-of-south-korea-ftd-study
#1
Moon Ho Park, Eun-Joo Kim, Kyung Won Park, Jae Cheol Kwon, Bon D Ku, Seol-Heui Han, SangYun Kim, Dong Won Yang, Duk L Na, Seong Hye Choi
OBJECTIVES: To characterise the behavioural and neuropsychiatric disturbances of patients with three clinical subtypes of frontotemporal dementia (FTD): behavioural variant FTD (bvFTD), semantic dementia (SD) and progressive non-fluent aphasia (PNFA). METHODS: Consecutive series of 66 patients with bvFTD, 58 patients with SD and 21 patients with PNFA were compared using the Frontal Behavioural Inventory (FBI) and the Neuropsychiatric Inventory (NPI). RESULTS: Patients with bvFTD had more behavioural and neuropsychiatric disturbances than patients with PNFA based on the total scores of FBI and NPI...
March 2017: Australasian Journal on Ageing
https://www.readbyqxmd.com/read/28323683/genetic-alzheimer-disease-and-sporadic-dementia-with-lewy-bodies-a-comorbidity-presenting-as-primary-progressive-aphasia
#2
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/28322905/bilingualism-delays-the-onset-of-behavioral-but-not-aphasic-forms-of-frontotemporal-dementia
#3
Suvarna Alladi, Thomas H Bak, Mekala Shailaja, Divyaraj Gollahalli, Amuya Rajan, Bapiraju Surampudi, Michael Hornberger, Vasanta Duggirala, Jaydip Ray Chaudhuri, Subhash Kaul
Bilingualism has been found to delay onset of dementia and this has been attributed to an advantage in executive control in bilinguals. However, the relationship between bilingualism and cognition is complex, with costs as well as benefits to language functions. To further explore the cognitive consequences of bilingualism, the study used Frontotemporal dementia (FTD) syndromes, to examine whether bilingualism modifies the age at onset of behavioral and language variants of Frontotemporal dementia (FTD) differently...
March 18, 2017: Neuropsychologia
https://www.readbyqxmd.com/read/28322482/progressive-multifocal-leukoencephalopathy-associated-with-fumaric-acid-esters-treatment-in-psoriasis-patients
#4
Deepak M W Balak, Enes Hajdarbegovic, Wichor M Bramer, Martino H A Neumann, H Bing Thio
BACKGROUND: Fumaric acid esters (FAEs) are a systemic treatment for psoriasis considered to have a favorable long-term safety profile without an increased risk for immunosuppression. However, progressive multifocal leukoencephalopathy (PML), a rare, opportunistic viral infection of the central nervous system, has been linked anecdotally to FAE treatment. OBJECTIVE: to assess clinical features and outcomes of FAE-associated PML cases. METHODS: Systematic literature search in multiple databases up to February, 25(th) 2016 for reports of PML in psoriasis patients treated with FAEs...
March 21, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28321196/connected-speech-in-neurodegenerative-language-disorders-a-review
#5
REVIEW
Veronica Boschi, Eleonora Catricalà, Monica Consonni, Cristiano Chesi, Andrea Moro, Stefano F Cappa
Language assessment has a crucial role in the clinical diagnosis of several neurodegenerative diseases. The analysis of extended speech production is a precious source of information encompassing the phonetic, phonological, lexico-semantic, morpho-syntactic, and pragmatic levels of language organization. The knowledge about the distinctive linguistic variables identifying language deficits associated to different neurodegenerative diseases has progressively improved in the last years. However, the heterogeneity of such variables and of the way they are measured and classified limits any generalization and makes the comparison among studies difficult...
2017: Frontiers in Psychology
https://www.readbyqxmd.com/read/28304311/frontotemporal-dementia-due-to-the-novel-grn-arg161glyfsx36-mutation
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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/28201834/short-term-memory-and-aphasia-from-theory-to-treatment
#17
Irene Minkina, Samantha Rosenberg, Michelene Kalinyak-Fliszar, Nadine Martin
This article reviews existing research on the interactions between verbal short-term memory and language processing impairments in aphasia. Theoretical models of short-term memory are reviewed, starting with a model assuming a separation between short-term memory and language, and progressing to models that view verbal short-term memory as a cognitive requirement of language processing. The review highlights a verbal short-term memory model derived from an interactive activation model of word retrieval. This model holds that verbal short-term memory encompasses the temporary activation of linguistic knowledge (e...
February 2017: Seminars in Speech and Language
https://www.readbyqxmd.com/read/28196768/cerebrospinal-fluid-markers-detect-alzheimer-s-disease-in-nonamnestic-dementia
#18
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/28196556/approach-to-atypical-alzheimer-s-disease-and-case-studies-of-the-major-subtypes
#19
Bradford C Dickerson, Scott M McGinnis, Chenjie Xia, Bruce H Price, Alireza Atri, Melissa E Murray, Mario F Mendez, David A Wolk
Alzheimer's disease (AD) has long been recognized as a heterogeneous illness, with a common clinical presentation of progressive amnesia and less common "atypical" clinical presentations, including syndromes dominated by visual, aphasic, "frontal," or apraxic symptoms. Our knowledge of atypical clinical phenotypes of AD comes from clinicopathologic studies, but with the growing use of in vivo molecular biomarkers of amyloid and tau pathology, we are beginning to recognize that these syndromes may not be as rare as once thought...
February 15, 2017: CNS Spectrums
https://www.readbyqxmd.com/read/28191623/the-ivy-sign-on-fluid-attenuated-inversion-recovery-images-related-to-single-photon-emission-computed-tomography-cerebral-blood-flow-in-moyamoya-disease-a-case-report
#20
Fumihiro Matano, Yasuo Murai, Asami Kubota, Takayuki Mizunari, Shiro Kobayashi, Akio Morita
BACKGROUND: Moyamoya disease is an idiopathic progressive cerebrovascular steno-occlusive disorder characterized by the formation of numerous collaterals called moyamoya vessels. Accurate evaluation of vascular status and CBF is needed for prompt treatment to prevent ischemic and/or hemorrhagic events. The pathogenesis of the ivy sign on fluid attenuated inversion recovery (FLAIR) images of moyamoya disease patients is unclear. We report a moyamoya disease case wherein the ivy sign changed in relation to SPECT-measured CBF during progression and following treatment...
January 17, 2017: Turkish Neurosurgery
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