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Progressive aphasia

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https://www.readbyqxmd.com/read/29781369/contralateral-extensive-cerebral-hemorrhagic-venous-infarction-caused-by-retrograde-venous-reflux-into-the-opposite-basal-vein-of-rosenthal-in-posttraumatic-carotid-cavernous-fistula-a-case-report-and-literature-review
#1
Prasert Iampreechakul, Adisak Tanpun, Punjama Lertbusayanukul, Somkiet Siriwimonmas
We describe a patient with traumatic carotid-cavernous fistula (CCF), subsequently developing contralateral extensive hemorrhagic venous infarction from retrograde venous reflux into the opposite basal vein of Rosenthal. A 54-year-old woman was involved in a motor vehicle accident and sustained severe traumatic brain injury. Two months later, she developed bilateral proptosis and audible bruit. Magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) of the brain demonstrated the right direct CCF...
January 1, 2018: Interventional Neuroradiology
https://www.readbyqxmd.com/read/29779573/-moon-river-intact-musical-appreciation-and-performance-in-a-man-with-semantic-variant-primary-progressive-aphasia
#2
Patrick A Ho, Theodore A Stern, James K Rustad
No abstract text is available yet for this article.
April 3, 2018: Psychosomatics
https://www.readbyqxmd.com/read/29760288/-neuropathologic-subtypes-of-frontotemporal-lobar-degeneration
#3
Mari Tada, Akiyoshi Kakita
Frontotemporal lobar degeneration (FTLD) is a heterogeneous disease entity encompassing a wide variety of histopathological features and genetic backgrounds. The last two decades have seen the discovery of causative genes and the identification of relevant proteins. The current histopathological classification is based on the major types of protein deposition in the brain, and most FTLD cases can be placed into one of three pathological subgroups: FTLD-tau, FTLD-TDP, and FTLD-FUS. Further sub-classification within each subgroup is based on the morphology of neuronal and glial inclusions and lesion distribution...
May 2018: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/29753915/the-eyes-reveal-uncertainty-about-object-distinctions-in-semantic-variant-primary-progressive-aphasia
#4
Andreia V Faria, David Race, Kevin Kim, Argye E Hillis
At least three distinct variants of primary progressive aphasia (PPA) have been described, but they are difficult to distinguish early in the course, when individuals experience primarily anomia. People with svPPA are often the hardest to care for, because they have impaired comprehension of words and objects and often have negative changes in comportment. We sought to identify an early marker of semantic variant primary progressive aphasia (svPPA) and to enhance the understanding of the semantic deficit in svPPA...
April 3, 2018: Cortex; a Journal Devoted to the Study of the Nervous System and Behavior
https://www.readbyqxmd.com/read/29744573/diagnostic-utility-of-fdg-pet-in-the-differential-diagnosis-between-different-forms-of-primary-progressive-aphasia
#5
Femke Bouwman, Stefania Orini, Federica Gandolfo, Daniele Altomare, Cristina Festari, Federica Agosta, Javier Arbizu, Alexander Drzezga, Peter Nestor, Flavio Nobili, Zuzana Walker, Silvia Morbelli, Marina Boccardi
PURPOSE: A joint effort of the European Association of Nuclear Medicine (EANM) and the European Academy of Neurology (EAN) aims at clinical guidance for the use of FDG-PET in neurodegenerative diseases. This paper addresses the diagnostic utility of FDG-PET over clinical/neuropsychological assessment in the differentiation of the three forms of primary progressive aphasia (PPA). METHODS: Seven panelists were appointed by the EANM and EAN and a literature search was performed by using harmonized PICO (Population, Intervention, Comparison, Outcome) question keywords...
May 9, 2018: European Journal of Nuclear Medicine and Molecular Imaging
https://www.readbyqxmd.com/read/29744128/fatal-case-of-amoebic-encephalitis-masquerading-as-herpes
#6
Rikesh Baral, Binit Vaidya
Primary amoebic meningoencephalitis (PAM) is a rare, fulminating, hemorrhagic infection of the brain caused by Naegleria fowleri , a thermophilic, free-living amoeba. A 74-year male presented with sudden severe global headache and fever with features of anomic aphasia. Magnetic resonance imaging (MRI) suggested herpes encephalitis and acyclovir (IV) was started but the patient developed altered sensorium, agitation and progressive weakness of lower limbs with gradual truncal weakness. Repeat MRI showed increase in lesion size and edema with confluent blood areas...
May 2018: Oxford Medical Case Reports
https://www.readbyqxmd.com/read/29725611/fingolimod-associated-pml-with-mild-iris-in-ms-a-clinicopathologic-study
#7
Shuhei Nishiyama, Tatsuro Misu, Yukiko Shishido-Hara, Kazuo Nakamichi, Masayuki Saijo, Yoshiki Takai, Kentarou Takei, Naoki Yamamoto, Hiroshi Kuroda, Ryuta Saito, Mika Watanabe, Teiji Tominaga, Ichiro Nakashima, Kazuo Fujihara, Masashi Aoki
Objective: To clarify the clinical, neuropathologic, and virologic characteristics of progressive multifocal leukoencephalopathy (PML) and its immune reconstitution inflammatory syndrome (IRIS) in a patient with fingolimod-treated MS. Methods: A case study. Results: A 34-year-old patient with MS using fingolimod for 4 years had a gradual progression of right hemiparesis and aphasia with a new subcortical white matter lesion in the precentral gyrus by initial MRI...
January 2018: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/29718131/retraining-speech-production-and-fluency-in-non-fluent-agrammatic-primary-progressive-aphasia
#8
Maya L Henry, H Isabel Hubbard, Stephanie M Grasso, Maria Luisa Mandelli, Stephen M Wilson, Mithra T Sathishkumar, Julius Fridriksson, Wylin Daigle, Adam L Boxer, Bruce L Miller, Maria Luisa Gorno-Tempini
The non-fluent/agrammatic variant of primary progressive aphasia (nfvPPA) presents with a gradual decline in grammar and motor speech resulting from selective degeneration of speech-language regions in the brain. There has been considerable progress in identifying treatment approaches to remediate language deficits in other primary progressive aphasia variants; however, interventions for the core deficits in nfvPPA have yet to be systematically investigated. Further, the neural mechanisms that support behavioural restitution in the context of neurodegeneration are not well understood...
April 30, 2018: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/29710718/the-progressive-acalculia-presentation-of-parietal-variant-alzheimer-s-disease
#9
Mario F Mendez, Negar Moheb, Randy E Desarzant, Edmond H Teng
BACKGROUND: Many patients with early-onset Alzheimer's disease (EOAD; age of onset <65 years) have non-amnestic presentations involving language (logopenic primary progressive aphasia, lvPPA), visuospatial abilities (posterior cortical atrophy, PCA), and even asymmetric symptoms consistent with corticobasal syndrome (CBS). An inferior parietal lobule variant of EOAD commonly presents with progressive difficulty with calculations. METHODS: We reviewed 276 EOAD patients for presentations with predominant acalculia...
April 28, 2018: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/29695300/a-language-based-sum-score-for-the-course-and-therapeutic-intervention-in-primary-progressive-aphasia
#10
Elisa Semler, Sarah Anderl-Straub, Ingo Uttner, Janine Diehl-Schmid, Adrian Danek, Beate Einsiedler, Klaus Fassbender, Klaus Fliessbach, Hans-Jürgen Huppertz, Holger Jahn, Johannes Kornhuber, Bernhard Landwehrmeyer, Martin Lauer, Rainer Muche, Johannes Prudlo, Anja Schneider, Matthias L Schroeter, Albert C Ludolph, Markus Otto
BACKGROUND: With upcoming therapeutic interventions for patients with primary progressive aphasia (PPA), instruments for the follow-up of patients are needed to describe disease progression and to evaluate potential therapeutic effects. So far, volumetric brain changes have been proposed as clinical endpoints in the literature, but cognitive scores are still lacking. This study followed disease progression predominantly in language-based performance within 1 year and defined a PPA sum score which can be used in therapeutic interventions...
April 25, 2018: Alzheimer's Research & Therapy
https://www.readbyqxmd.com/read/29689406/hyperperfusion-syndrome-after-trapping-with-high-flow-bypass-for-a-giant-paraclinoid-internal-carotid-artery-aneurysm
#11
Masanao Mohri, Toshiya Ichinose, Naoyuki Uchiyama, Kouichi Misaki, Iku Nambu, Yasushi Takabatake, Mitsutoshi Nakada
BACKGROUND: Hyperperfusion syndrome associated with aneurysm surgery is rare. The occurrence of the syndrome after trapping with high-flow bypass has not been described previously. Herein, we present a case of the syndrome occurring after trapping with high-flow bypass of an unruptured giant paraclinoid internal carotid artery (ICA) aneurysm. CASE DESCRIPTION: The patient was a 68-year-old woman with progressive loss of vision in her left eye. After a diagnosis of a left giant ICA aneurysm, she underwent successful trapping with high-flow bypass...
April 21, 2018: World Neurosurgery
https://www.readbyqxmd.com/read/29682238/rate-and-rhythm-control-strategies-for-apraxia-of-speech-in-nonfluent-primary-progressive-aphasia
#12
Bárbara Costa Beber, Monalise Costa Batista Berbert, Ruth Siqueira Grawer, Maria Cristina de Almeida Freitas Cardoso
The nonfluent/agrammatic variant of primary progressive aphasia is characterized by apraxia of speech and agrammatism. Apraxia of speech limits patients' communication due to slow speaking rate, sound substitutions, articulatory groping, false starts and restarts, segmentation of syllables, and increased difficulty with increasing utterance length. Speech and language therapy is known to benefit individuals with apraxia of speech due to stroke, but little is known about its effects in primary progressive aphasia...
January 2018: Dementia & Neuropsychologia
https://www.readbyqxmd.com/read/29679378/prominent-microglial-activation-in-cortical-white-matter-is-selectively-associated-with-cortical-atrophy-in-primary-progressive-aphasia
#13
Daniel T Ohm, Garam Kim, Tamar Gefen, Alfred Rademaker, Sandra Weintraub, Eileen Bigio, M-Marsel Mesulam, Emily Rogalski, Changiz Geula
AIMS: Primary progressive aphasia (PPA) is a clinical syndrome characterized by selective language impairments associated with focal cortical atrophy favouring the language dominant hemisphere. PPA is associated with Alzheimer's disease (AD), frontotemporal lobar degeneration (FTLD), and significant accumulation of activated microglia. Activated microglia can initiate an inflammatory cascade that may contribute to neurodegeneration, but their quantitative distribution in cortical white matter and their relationship with cortical atrophy are unknown...
April 21, 2018: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/29666711/creutzfeldt-jakob-disease-presenting-as-expressive-aphasia-and-nonconvulsive-status-epilepticus
#14
Hafiz B Mahboob, Kazi H Kaokaf, Jeremy M Gonda
Creutzfeldt-Jakob disease (CJD), the most common form of human prion diseases, is a fatal condition with a mortality rate reaching 85% within one year of clinical presentation. CJD is characterized by rapidly progressive neurological deterioration in combination with typical electroencephalography (EEG) and magnetic resonance imaging (MRI) findings and positive cerebrospinal spinal fluid (CSF) analysis for 14-3-3 proteins. Unfortunately, CJD can have atypical clinical and radiological presentation in approximately 10% of cases, thus making the diagnosis often challenging...
2018: Case Reports in Critical Care
https://www.readbyqxmd.com/read/29665116/atrophy-and-microglial-distribution-in-primary-progressive-aphasia-with-tdp-43
#15
Garam Kim, Kabriya Bolbolan, Tamar Gefen, Sandra Weintraub, Eileen H Bigio, Emily Rogalski, Marek-Marsel Mesulam, Changiz Geula
OBJECTIVE: To quantitatively determine the density and distribution of activated microglia across cortical regions and hemispheres in the brains of primary progressive aphasia (PPA) participants with pathologic diagnoses of FTLD-TDP, and to examine the relationships between microglial densities, patterns of focal atrophy, TDP-43 inclusions, and clinical phenotype. METHODS: Activated microglia and TDP-43 inclusions were visualized in whole-hemisphere brain sections using immunohistochemical methods from 5 participants with PPA-TDP...
April 17, 2018: Annals of Neurology
https://www.readbyqxmd.com/read/29656245/individual-differences-in-socioemotional-sensitivity-are-an-index-of-salience-network-function
#16
Gianina Toller, Jesse Brown, Marc Sollberger, Suzanne M Shdo, Laura Bouvet, Paul Sukhanov, William W Seeley, Bruce L Miller, Katherine P Rankin
Connectivity in intrinsically connected networks (ICNs) may predict individual differences in cognition and behavior. The drastic alterations in socioemotional awareness of patients with behavioral variant frontotemporal dementia (bvFTD) are presumed to arise from changes in one such ICN, the salience network (SN). We examined how individual differences in SN connectivity are reflected in overt social behavior in healthy individuals and patients, both to provide neuroscientific insight into this key brain-behavior relationship, and to provide a practical tool to diagnose patients with early bvFTD...
February 27, 2018: Cortex; a Journal Devoted to the Study of the Nervous System and Behavior
https://www.readbyqxmd.com/read/29618124/microsurgical-resection-of-a-large-intraventricular-trigonal-tumor-3-dimensional-operative-video
#17
Chun-Yu Cheng, Rakshith Shetty, Laligam N Sekhar
A 62-yr-old woman presented with incidentally detected left trigonal mass by magnetic resonance imaging (MRI) performed during workup for left-sided hearing loss and vertigo of 5-yr duration. Due to persistent dizziness, headache, and progressive enlargement of the tumor in follow-up scans, operation was planned. Because the tumor extended superiorly, a superior parietal lobule approach was selected.She underwent a left parietal craniotomy. A strip electrode was used to localize the motor and sensory regions, and neuronavigation was used to confirm the entry site...
March 30, 2018: Operative Neurosurgery (Hagerstown, Md.)
https://www.readbyqxmd.com/read/29614676/regional-distribution-asymmetry-and-clinical-correlates-of-tau-uptake-on-18f-av-1451-pet-in-atypical-alzheimer-s-disease
#18
Katerina A Tetzloff, Jonathan Graff-Radford, Peter R Martin, Nirubol Tosakulwong, Mary M Machulda, Joseph R Duffy, Heather M Clark, Matthew L Senjem, Christopher G Schwarz, Anthony J Spychalla, Daniel A Drubach, Clifford R Jack, Val J Lowe, Keith A Josephs, Jennifer L Whitwell
BACKGROUND: Despite common pathology, Alzheimer's disease (AD) can have multiple clinical presentations which pathological studies suggest result from differences in the regional distribution of tau pathology. Positron emission tomography (PET) ligands are now available that can detect tau proteins in vivo and hence can be used to investigate the biological mechanisms underlying atypical AD. OBJECTIVE: To assess regional patterns of tau uptake on PET imaging in two atypical AD variants, posterior cortical atrophy (PCA) and logopenic progressive aphasia (lvPPA)...
2018: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/29584904/combined-pathologies-in-ftld-tdp-types-a-and-c
#19
Tamar Gefen, Saman S Ahmadian, Qinwen Mao, Garam Kim, Mustafa Seckin, Borna Bonakdarpour, Eliana Marisa Ramos, Giovanni Coppola, Rosa Rademakers, Emily Rogalski, Alfred Rademaker, Sandra Weintraub, M-Marsel Mesulam, Changiz Geula, Eileen H Bigio
This study investigated the presence of combined pathologies in a large cohort of autopsies that show a primary pathologic diagnosis of phosphorylated 43-kDa TAR DNA-binding protein (FTLD-TDP), the majority of which portrayed clinical phenotypes consistent with primary progressive aphasia or behavioral variant frontotemporal dementia (bvFTD). Thirty-eight cases with FTLD-TDP (30 type-A and 8 type-C) were identified to determine characteristic differences between cases with and without combined pathologies. Findings indicated that combined pathologies co-occur with FTLD-TDP type-A at a high frequency (50%)-greater than when compared to FTLD-TDP type-C cases (12...
March 23, 2018: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/29569990/molecular-neuroimaging-in-primary-progressive-aphasia-with-predominant-agraphia
#20
Rene L Utianski, Joseph R Duffy, Rodolfo Savica, Jennifer L Whitwell, Mary M Machulda, Keith A Josephs
A 62-year-old male presented with progressive isolated writing and spelling difficulties. Neurological, neuropsychological, speech, and language evaluations identified only minimal additional abnormalities. The presenting characteristics did not meet criteria for any particular variant of primary progressive aphasia; his clinical presentation is best described as primary progressive aphasia, with a predominant, almost pure agraphia. Brain MRI showed asymmetric, bilateral parenchymal volume loss, with left hippocampal atrophy...
March 23, 2018: Neurocase
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