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Progressive aphasia

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https://www.readbyqxmd.com/read/28100023/frontotemporal-lobar-degeneration-pathogenesis-pathology-and-pathways-to-phenotype
#1
REVIEW
David Ma Mann, Julie S Snowden
Frontotemporal Lobar Degeneration (FTLD) is a clinically, pathologically and genetically heterogeneous group of disorders that affect principally the frontal and temporal lobes of the brain. There are three major associated clinical syndromes, behavioural variant frontotemporal dementia (bvFTD), semantic dementia (SD) and progressive non-fluent aphasia (PNFA); three principal histologies, involving tau, TDP-43 and FUS proteins; and mutations in three major genes, MAPT, GRN and C9orf72, along with several other less common gene mutations...
January 18, 2017: Brain Pathology
https://www.readbyqxmd.com/read/28040670/focal-temporal-pole-atrophy-and-network-degeneration-in-semantic-variant-primary-progressive-aphasia
#2
Jessica A Collins, Victor Montal, Daisy Hochberg, Megan Quimby, Maria Luisa Mandelli, Nikos Makris, William W Seeley, Maria Luisa Gorno-Tempini, Bradford C Dickerson
A wealth of neuroimaging research has associated semantic variant primary progressive aphasia with distributed cortical atrophy that is most prominent in the left anterior temporal cortex; however, there is little consensus regarding which region within the anterior temporal cortex is most prominently damaged, which may indicate the putative origin of neurodegeneration. In this study, we localized the most prominent and consistent region of atrophy in semantic variant primary progressive aphasia using cortical thickness analysis in two independent patient samples (n = 16 and 28, respectively) relative to age-matched controls (n = 30)...
December 31, 2016: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/28018854/cholinergic-depletion-and-basal-forebrain-volume-in-primary-progressive-aphasia
#3
Jolien Schaeverbeke, Charlotte Evenepoel, Rose Bruffaerts, Koen Van Laere, Guy Bormans, Eva Dries, Thomas Tousseyn, Natalie Nelissen, Ronald Peeters, Mathieu Vandenbulcke, Patrick Dupont, Rik Vandenberghe
Primary progressive aphasia (PPA) is a heterogeneous syndrome with various neuropathological causes for which no medical treatment with proven efficacy exists. Basal forebrain (BF) volume loss has been reported in PPA but its relation to cholinergic depletion is still unclear. The primary objective of this study was to investigate whether cholinergic alterations occur in PPA variants and how this relates to BF volume loss. An academic memory clinic based consecutive series of 11 PPA patients (five with the semantic variant (SV), four with the logopenic variant (LV) and two with the nonfluent variant (NFV)) participated in this cross-sectional in vivo PET imaging study together with 10 healthy control subjects...
2017: NeuroImage: Clinical
https://www.readbyqxmd.com/read/28017247/clinical-characteristics-of-parkinsonism-in-frontotemporal-dementia-according-to-subtypes
#4
Hee Kyung Park, Kee Hyung Park, Bora Yoon, Jae-Hong Lee, Seong Hye Choi, Jee H Joung, Soo Jin Yoon, Byeong C Kim, Seung Hyun Kim, Eun-Joo Kim, Duk L Na, Kyung Won Park
BACKGROUND: We investigated the prevalence of parkinsonism in frontotemporal dementia (FTD) subtypes and the cognitive and behavioral differences between FTD with and without parkinsonism in a well-structured, prospective cohort. METHODS: One hundred and ninety-one FTD patients were enrolled and all patients underwent comprehensive neurological evaluations, neuropsychological tests, and the Unified Parkinson's Disease Rating Scale. RESULTS: The prevalence of parkinsonism was 38...
January 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28009766/urgent-bypass-surgery-following-failed-endovascular-treatment-in-acute-symptomatic-stroke-patient-with-mca-occlusion
#5
Chang Yeob Lee, Chang Hyun Kim, Chang-Young Lee, Sung-Il Sohn, Jeong-Ho Hong
INTRODUCTION: Although the benefits of extracranial-intracranial bypass surgery remain controversial, there is some surgical rationale for the augmentation of cerebral blood flow in cases of acute ischemic stroke with hemodynamic instability. CASE REPORT: We report a case of a 62-year-old woman who suddenly developed right hemiplegia and global aphasia. Initial magnetic resonance imaging and magnetic resonance angiography revealed a small acute ischemic lesion in left parietal lobe with occlusion at the left middle cerebral artery...
January 2017: Neurologist
https://www.readbyqxmd.com/read/27999880/-pathomechanisms-and-clinical-aspects-of-frontotemporal-lobar-degeneration
#6
REVIEW
K Bürger, T Arzberger, J Stephan, J Levin, D Edbauer
BACKGROUND: Frontotemporal lobar degeneration (FTLD) includes a spectrum of heterogeneous clinical and neuropathological diseases. In a strict sense this includes the behavioral variant of frontotemporal dementia (bvFTD) and primary progressive aphasia (PPA) and both variants can be associated with amyotrophic lateral sclerosis (FTD-ALS). In a broader sense FTLD also includes progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS). In recent years the strong genetic component of FTLD has become increasingly clear...
December 20, 2016: Der Nervenarzt
https://www.readbyqxmd.com/read/27939367/naming-unique-entities-in-the-semantic-variant-of-primary-progressive-aphasia-and-alzheimer-s-disease-towards-a-better-understanding-of-the-semantic-impairment
#7
M Montembeault, S M Brambati, S Joubert, M Boukadi, M Chapleau, R Jr Laforce, M A Wilson, J Macoir, I Rouleau
While the semantic variant of primary progressive aphasia (svPPA) is characterized by a predominant semantic memory impairment, episodic memory impairments are the clinical hallmark of Alzheimer's disease (AD). However, AD patients also present with semantic deficits, which are more severe for semantically unique entities (e.g. a famous person) than for common concepts (e.g. a beaver). Previous studies in these patient populations have largely focused on famous-person naming. Therefore, we aimed to evaluate if these impairments also extend to other semantically unique entities such as famous places and famous logos...
December 7, 2016: Neuropsychologia
https://www.readbyqxmd.com/read/27934843/successful-treatment-of-primary-cerebral-mucormycosis-role-of-microbiologist
#8
K K Benachinmardi, P Rajalakshmi, H B Veenakumari, R D Bharath, V Vikas, A Mahadevan, S Nagarathna
Fungal brain abscess is rare with a rapidly progressive disease with fulminant course and invariably fatal outcome, unless diagnosed early and treated rapidly. We report a 56-year-old woman diagnosed to have fungal abscess who recovered completely following amphotericin B treatment. She presented with weakness of the right hand, deviation of mouth to left and aphasia for 2 days. Computed tomography of the brain revealed a left frontal capsuloganglionic hypodense lesion. Stereotactic biopsy was performed, and microbiological confirmation of non-septate fungal hyphae from pus from aspirate within 2 h helped initiate timely and appropriate treatment leading to cure...
October 2016: Indian Journal of Medical Microbiology
https://www.readbyqxmd.com/read/27929804/familial-creutzfeldt-jakob-disease-case-report-and-role-of-genetic-counseling-in-post-mortem-testing
#9
Kristin Clift, Kimberly Guthrie, Eric W Klee, Nicole Boczek, Margot Cousin, Patrick Blackburn, Paldeep Atwal
Here we present a case of an asymptomatic 53-year-old woman who sought genetic testing for Familial Creutzfeldt-Jakob Disease (fCJD) after learning that her mother had fCJD. The patient's mother had a sudden onset of memory problems and rapidly deteriorating mental faculties in her late 70s, which led to difficulties ambulating, progressive non-fluent aphasia, dysphagia and death within ∼1 y of symptom onset. The cause of death was reported as "rapid onset dementia." The patient's family, unhappy with the vague diagnosis, researched prion disorders online and aggressively pursued causation and submitted frozen brain tissue from the mother to the National Prion Disease Surveillance Center, where testing revealed a previously described 5-octapeptide repeat insertion (5-OPRI) in the prion protein gene (PRNP) that is known to cause fCJD...
November 2016: Prion
https://www.readbyqxmd.com/read/27922867/the-value-of-99mtc-ecd-spect-with-statistical-image-analysis-on-enhancing-the-early-diagnosis-of-primary-progressive-aphasia
#10
Cheng-Yu Wei, Pai-Yi Chiu, Po-Nien Hou, Hiroshi Matsuda, Guang-Uei Hung
A 64-year-old woman with poor short-term memory was first suspected as early Alzheimer disease. Tc ECD brain SPECT was arranged for differential diagnosis. A small area of mild hypoperfusion was noted in the left temporal lobe on conventional display. Further statistical analysis of SPECT with an easy Z-score imaging system showed large areas of distinct hypoperfusion in left precentral and perisylvian cortical areas, compatible with typical pictures of nonfluent variant primary progressive aphasia (PPA), but no involvement in areas characteristic for Alzheimer disease...
December 3, 2016: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/27920291/teaching-neuroimages-nonfluent-variant-primary-progressive-aphasia-a-distinctive-clinico-anatomical-syndrome
#11
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/27899886/relearning-and-retaining-personally-relevant-words-using-computer-based-flashcard-software-in-primary-progressive-aphasia
#12
William S Evans, Megan Quimby, Michael Walsh Dickey, Bradford C Dickerson
Although anomia treatments have often focused on training small sets of words in the hopes of promoting generalization to untrained items, an alternative is to directly train a larger set of words more efficiently. The current case study reports on a novel treatment for a patient with semantic variant Primary Progressive Aphasia (svPPA), in which the patient was taught to make and practice flashcards for personally-relevant words using an open-source computer program (Anki). Results show that the patient was able to relearn and retain a large subset of her studied words for up to 20 months, the full duration of the study period...
2016: Frontiers in Human Neuroscience
https://www.readbyqxmd.com/read/27891726/everyday-conversation-in-dementia-a-review-of-the-literature-to-inform-research-and-practice
#13
REVIEW
Jacqueline Kindell, John Keady, Karen Sage, Ray Wilkinson
BACKGROUND: There has been increasing interest in dementia care in recent years, including how practitioners, service providers and society in general can help individuals to live well with the condition. An important aspect to this is provision of advice to ensure conversation partners effectively support the person with dementia in conversation. AIMS: To provide a descriptive review of the literature examining everyday conversation in dementia in order to inform practice and research...
November 27, 2016: International Journal of Language & Communication Disorders
https://www.readbyqxmd.com/read/27875715/common-and-divergent-neural-correlates-of-anomia-in-amnestic-and-logopenic-presentations-of-alzheimer-s-disease
#14
Cristian E Leyton, John R Hodges, Olivier Piguet, Kirrie J Ballard
The majority of logopenic variant primary progressive aphasia (lv-PPA) cases harbour Alzheimer pathology, suggesting that lv-PPA constitutes an atypical presentation of Alzheimer's disease (AD). However, even if caused by Alzheimer pathology, the clinical manifestations of lv-PPA differ from those observed in the typical or amnestic AD presentation: in lv-PPA, aphasia is the main feature while amnestic AD is characterised by impaired episodic memory. Anomia or impaired naming, however, is present in both AD presentations...
November 5, 2016: Cortex; a Journal Devoted to the Study of the Nervous System and Behavior
https://www.readbyqxmd.com/read/27865707/a-comprehensive-database-of-published-tdcs-clinical-trials-2005-2016
#15
REVIEW
Jean-Pascal Lefaucheur
Transcranial direct current stimulation (tDCS) is a technique of noninvasive cortical stimulation allowing significant modification of brain functions. Clinical application of this technique was reported for the first time in March 2005. This paper presents a detailed list of the 340 articles (excluding single case reports) which have assessed the clinical effect of tDCS in patients, at least when delivered to cortical targets. The reviewed conditions were: pain syndromes, Parkinson's disease, dystonia, cerebral palsy, post-stroke limb motor impairment, post-stroke neglect, post-stroke dysphagia, post-stroke aphasia, primary progressive aphasia, multiple sclerosis, epilepsy, consciousness disorders, Alzheimer's disease and other types of dementia, tinnitus, depression, auditory hallucinations and negative symptoms of schizophrenia, addiction and craving, autism, and attention disorders...
December 2016: Neurophysiologie Clinique, Clinical Neurophysiology
https://www.readbyqxmd.com/read/27859539/clinicopathologic-heterogeneity-in-ftdp-17-due-to-mapt-p-p301l-mutation-including-a-patient-with-globular-glial-tauopathy
#16
Pawel Tacik, Monica Sanchez-Contreras, Michael DeTure, Melissa E Murray, Rosa Rademakers, Owen A Ross, Zbigniew K Wszolek, Joseph E Parisi, David S Knopman, Ronald C Petersen, Dennis W Dickson
AIM: The p.P301L mutation in microtubule-associated protein tau (MAPT) is a common cause of frontotemporal dementia and Parkinsonism linked to chromosome 17 (FTDP-17). We compare clinicopathologic features of five unrelated and three related (brother, sister and cousin) patients with FTDP-17 due to p.P301L mutation. METHODS: Genealogical, clinical, neuropathologic, and genetic data were reviewed from eight individuals RESULTS: The series consisted of five men and three women with an average age of death of 58 years (52-65 years) and average disease duration of 9 years (3-14 years)...
November 8, 2016: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/27858708/cerebrospinal-fluid-biomarkers-as-a-diagnostic-tool-of-the-underlying-pathology-of-primary-progressive-aphasia
#17
George P Paraskevas, Dimitrios Kasselimis, 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...
2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/27849128/when-the-wedding-march-becomes-sad-semantic-memory-impairment-for-music-in-the-semantic-variant-of-primary-progressive-aphasia
#18
Joël Macoir, Sarah Berubé-Lalancette, Maximiliano A Wilson, Robert Laforce, Carol Hudon, Pierre Gravel, Olivier Potvin, Simon Duchesne, Laura Monetta
Music can induce particular emotions and activate semantic knowledge. In the semantic variant of primary progressive aphasia (svPPA), semantic memory is impaired as a result of anterior temporal lobe (ATL) atrophy. Semantics is responsible for the encoding and retrieval of factual knowledge about music, including associative and emotional attributes. In the present study, we report the performance of two individuals with svPPA in three experiments. NG with bilateral ATL atrophy and ND with atrophy largely restricted to the left ATL...
December 2016: Neurocase
https://www.readbyqxmd.com/read/27847684/longitudinal-change-in-everyday-function-and-behavioral-symptoms-in-frontotemporal-dementia
#19
Claire M O'Connor, Lindy Clemson, Michael Hornberger, Cristian E Leyton, John R Hodges, Olivier Piguet, Eneida Mioshi
BACKGROUND: The relationship between behavioral changes and functional decline in frontotemporal dementia (FTD) is not well understood. METHODS: Thirty-nine patients (21 behavioral variant FTD [bvFTD], 18 semantic variant primary progressive aphasia [svPPA]) were followed up longitudinally (2-4 years follow-up). Functional (Disability Assessment for Dementia) and behavioral (Cambridge Behavioural Inventory Revised) assessments were included for between-group (pairwise comparisons, mixed model analysis) and within-group analyses (bivariate correlations)...
October 2016: Neurology. Clinical Practice
https://www.readbyqxmd.com/read/27844039/increased-prevalence-of-autoimmune-disease-within-c9-and-ftd-mnd-cohorts-completing-the-picture
#20
Zachary A Miller, Virginia E Sturm, Gamze Balci Camsari, Anna Karydas, Jennifer S Yokoyama, Lea T Grinberg, Adam L Boxer, Howard J Rosen, Katherine P Rankin, Maria Luisa Gorno-Tempini, Giovanni Coppola, Daniel H Geschwind, Rosa Rademakers, William W Seeley, Neill R Graff-Radford, Bruce L Miller
OBJECTIVE: To determine the prevalence of autoimmune disease in symptomatic C9ORF72 (C9) mutation carriers and frontotemporal dementia with motor neuron disease (FTD/MND) cohorts. METHODS: In this case-control study, we reviewed the clinical histories of 66 patients with FTD/MND and 57 symptomatic C9 carriers (24 overlapping cases), a total of 99 charts, for history of autoimmune disease. The prevalence of autoimmune disease in C9 and FTD/MND cohorts was determined by χ(2) and Fisher exact comparisons between the combined C9 and FTD/MND group with normal control, Alzheimer disease, and progressive supranuclear palsy cohorts, as well as comparisons within C9 and FTD/MND cohorts...
December 2016: Neurology® Neuroimmunology & Neuroinflammation
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