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Progressive aphasia

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https://www.readbyqxmd.com/read/27929804/familial-creutzfeldt-jakob-disease-case-report-and-role-of-genetic-counseling-in-post-mortem-testing
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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/27849128/when-the-wedding-march-becomes-sad-semantic-memory-impairment-for-music-in-the-semantic-variant-of-primary-progressive-aphasia
#10
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...
November 16, 2016: Neurocase
https://www.readbyqxmd.com/read/27847684/longitudinal-change-in-everyday-function-and-behavioral-symptoms-in-frontotemporal-dementia
#11
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
#12
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
https://www.readbyqxmd.com/read/27815682/amyloid-pet-in-primary-progressive-aphasia-case-series-and-systematic-review-of-the-literature
#13
Alberto Villarejo-Galende, Sara Llamas-Velasco, Adolfo Gómez-Grande, Verónica Puertas-Martín, Israel Contador, Pilar Sarandeses, Marta González-Sánchez, Rocío Trincado, Patrick Pilkington, Sebastián Ruiz-Solis, David A Pérez-Martínez, Alejandro Herrero-San Martín
Primary progressive aphasia (PPA) is considered a heterogeneous syndrome, with different clinical subtypes and neuropathological causes. Novel PET biomarkers may help to predict the underlying neuropathology, but many aspects remain unclear. We studied the relationship between amyloid PET and PPA variant in a clinical series of PPA patients. A systematic review of the literature was performed. Patients with PPA were assessed over a 2-year period and classified based on language testing and the International Consensus Criteria as non-fluent/agrammatic (nfvPPA), semantic (svPPA), logopenic variant (lvPPA) or as unclassifiable (ucPPA)...
November 4, 2016: Journal of Neurology
https://www.readbyqxmd.com/read/27803826/a-corticobasal-syndrome-variant-of-familial-creutzfeldt-jakob-disease-with-stroke-like-onset
#14
Ján Necpál, Martin Stelzer, Silvia Koščová, Michal Patarák
Creutzfeldt-Jakob disease (CJD) is an untreatable rare human prion disease characterized by rapidly progressive dementia along with various neurological features, including myoclonus and sometimes other movement disorders. The clinical course is typically insidious and rapid, leading to an early death. In general, the most common form is sporadic CJD; however, Slovakia is typical for a high percentage of genetic cases. We present an unusual case report of a 65-year-old man with a sudden, stroke-like onset of motor aphasia with right-sided levodopa unresponsive parkinsonism, alien hand, and other characteristic features of corticobasal syndrome (CBS), with rapid deterioration and death on the 32nd day of the disease...
2016: Case Reports in Neurological Medicine
https://www.readbyqxmd.com/read/27802240/bipolar-disorder-and-frontotemporal-dementia-an-intriguing-association
#15
Apostolos Papazacharias, Madia Lozupone, Maria Rosaria Barulli, Rosa Capozzo, Bruno P Imbimbo, Federica Veneziani, Roberto De Blasi, Marcello Nardini, Davide Seripa, Francesco Panza, Giancarlo Logroscino
Bipolar disorder (BD) could represent a prodromal state of frontotemporal dementia (FTD). Two patients affected by lifelong BD with a progressive decline of cognitive functions, behavioral, and neurological signs, reached the early diagnosis of FTD before the age of 60. They were diagnosed as affected by primary progressive aphasia and FTD with parkinsonism, respectively. A diagnosis of FTD should therefore be taken into account, in case of unexpected cognitive and behavioral decline in patients with a long history of BD...
October 11, 2016: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/27802232/varying-degrees-of-temporoparietal-hypometabolism-on-fdg-pet-reveal-amyloid-positive-logopenic-primary-progressive-aphasia-is-not-a%C3%A2-homogeneous-clinical-entity
#16
Kamini Krishnan, Mary M Machulda, Jennifer L Whitwell, Alissa M Butts, Joseph R Duffy, Edythe A Strand, Matthew L Senjem, Anthony J Spychalla, Clifford R Jack, Val J Lowe, Keith A Josephs
BACKGROUND: The logopenic variant of primary progressive aphasia (lvPPA) manifests due to a breakdown of the language network with prominent hypometabolism of the left temporoparietal region. LvPPA is strongly associated with amyloid deposition, yet there is question as to whether it is a homogeneous clinical entity. OBJECTIVE: This study investigated whether differences in temporoparietal metabolic patterns on 18F fludeoxyglucose positron emission tomography (FDG-PET) could elucidate brain regions preferentially affected in lvPPA...
October 12, 2016: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/27802226/music-perception-in-dementia
#17
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
#18
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/27787806/acyclovir-resistance-in-herpes-simplex-virus-type-i-encephalitis-a-case-report
#19
M Bergmann, R Beer, M Kofler, R Helbok, B Pfausler, E Schmutzhard
Acyclovir resistance is rarely seen in herpes simplex virus (HSV) type I encephalitis. Prevalence rates vary between 0.5 % in immunocompetent patients (Christophers et al. 1998; Fife et al. 1994) and 3.5-10 % in immunocompromised patients (Stranska et al. 2005). We report a 45-year-old, immunocompetent (negative HIV antigen/antibody testing), female patient, without previous illness who developed-after a febrile prodromal stage-aphasia and psychomotor slowing. Cerebral magnetic resonance imaging (cMRI) showed right temporal and insular T2-hyperintense lesions with spreading to the contralateral temporal lobe...
October 27, 2016: Journal of Neurovirology
https://www.readbyqxmd.com/read/27784208/early-and-mild-phases-of-primary-progressive-aphasia-a-case-series
#20
Jessica Powell, James Lendrum, Rosalind Huff, Christine Belden, Marwan Noel Sabbagh
BACKGROUND: The early and mild phases of various neurodegenerative diseases, sometimes described as mild cognitive impairment (MCI), has been characterized as the transitional state between normal cognition and dementia. It is described as having cognitive decline not severe enough to cause functional impairment. MCI has been divided into amnestic and non-amnestic subtypes with the amnestic subtype most commonly progressing to AD. The MCI phase of other dementias such as DLB has also been described...
October 24, 2016: Current Aging Science
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