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Progressive supranuclear palsy

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https://www.readbyqxmd.com/read/29054878/pathological-tau-strains-from-human-brains-recapitulate-the-diversity-of-tauopathies-in-non-transgenic-mouse-brain
#1
Sneha Narasimhan, Jing L Guo, Lakshmi Changolkar, Anna Stieber, Jennifer D McBride, Luisa V Silva, Zhuohao He, Bin Zhang, Ronald J Gathagan, John Q Trojanowski, Virginia M Y Lee
Pathological tau aggregates occur in Alzheimer's disease (AD) and other neurodegenerative tauopathies. It is not clearly understood why tauopathies vary greatly in the neuroanatomical and histopathological patterns of tau aggregation, which contribute to clinical heterogeneity in these disorders. Recent studies have shown that tau aggregates may form distinct structural conformations, known as tau strains. Here, we developed a novel model to test the hypothesis that cell-to-cell transmission of different tau strains occurs in non-transgenic (non-Tg) mice, and to investigate whether there are strain-specific differences in the pattern of tau transmission...
October 20, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/29053860/clinicopathological-correlations-in-behavioural-variant-frontotemporal-dementia
#2
David C Perry, Jesse A Brown, Katherine L Possin, Samir Datta, Andrew Trujillo, Anneliese Radke, Anna Karydas, John Kornak, Ana C Sias, Gil D Rabinovici, Maria Luisa Gorno-Tempini, Adam L Boxer, Mary De May, Katherine P Rankin, Virginia E Sturm, Suzee E Lee, Brandy R Matthews, Aimee W Kao, Keith A Vossel, Maria Carmela Tartaglia, Zachary A Miller, Sang Won Seo, Manu Sidhu, Stephanie E Gaus, Alissa L Nana, Jose Norberto S Vargas, Ji-Hye L Hwang, Rik Ossenkoppele, Alainna B Brown, Eric J Huang, Giovanni Coppola, Howard J Rosen, Daniel Geschwind, John Q Trojanowski, Lea T Grinberg, Joel H Kramer, Bruce L Miller, William W Seeley
Accurately predicting the underlying neuropathological diagnosis in patients with behavioural variant frontotemporal dementia (bvFTD) poses a daunting challenge for clinicians but will be critical for the success of disease-modifying therapies. We sought to improve pathological prediction by exploring clinicopathological correlations in a large bvFTD cohort. Among 438 patients in whom bvFTD was either the top or an alternative possible clinical diagnosis, 117 had available autopsy data, including 98 with a primary pathological diagnosis of frontotemporal lobar degeneration (FTLD), 15 with Alzheimer's disease, and four with amyotrophic lateral sclerosis who lacked neurodegenerative disease-related pathology outside of the motor system...
October 6, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/29051824/pure-akinesia-with-gait-freezing-a-clinicopathologic-study
#3
Ahmad Elkouzi, Esther N Bit-Ivan, Rodger J Elble
BACKGROUND: Pure akinesia with gait freezing is a rare syndrome with few autopsied cases. Severe freezing of gait occurs in the absence of bradykinesia and rigidity. Most autopsies have revealed progressive supranuclear palsy. We report the clinical and postmortem findings of two patients with pure akinesia with gait freezing, provide video recordings of these patients, and review the literature describing similar cases. We also discuss bradykinesia, hypokinesia and akinesia in the context of this clinical syndrome...
2017: Journal of Clinical Movement Disorders
https://www.readbyqxmd.com/read/29050386/brain-monoamine-oxidase-b-and-a-in-human-parkinsonian-dopamine-deficiency-disorders
#4
Junchao Tong, Gausiha Rathitharan, Jeffrey H Meyer, Yoshiaki Furukawa, Lee-Cyn Ang, Isabelle Boileau, Mark Guttman, Oleh Hornykiewicz, Stephen J Kish
See Jellinger (doi:10.1093/awx190) for a scientific commentary on this article. The enzyme monoamine oxidases (B and A subtypes, encoded by MAOB and MAOA, respectively) are drug targets in the treatment of Parkinson's disease. Inhibitors of MAOB are used clinically in Parkinson's disease for symptomatic purposes whereas the potential disease-modifying effect of monoamine oxidase inhibitors is debated. As astroglial cells express high levels of MAOB, the enzyme has been proposed as a brain imaging marker of astrogliosis, a cellular process possibly involved in Parkinson's disease pathogenesis as elevation of MAOB in astrocytes might be harmful...
September 1, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/29050232/progressive-modulation-of-the-human-olfactory-bulb-transcriptome-during-alzheimer%C3%A2-s-disease-evolution-novel-insights-into-the-olfactory-signaling-across-proteinopathies
#5
Mercedes Lachen-Montes, María Victoria Zelaya, Víctor Segura, Joaquín Fernández-Irigoyen, Enrique Santamaría
Alzheimer´s disease (AD) is characterized by progressive dementia, initially presenting olfactory dysfunction. Despite the olfactory bulb (OB) is the first central structure of the olfactory pathway, we lack a complete molecular characterization of the transcriptional events that occurs in this olfactory area during AD progression. To address this gap in knowledge, we have assessed the genome-wide expression in postmortem OBs from subjects with varying degree of AD pathology. A stage-dependent deregulation of specific pathways was observed, revealing transmembrane transport, and neuroinflammation as part of the functional modules that are disrupted across AD grading...
September 19, 2017: Oncotarget
https://www.readbyqxmd.com/read/29042634/a-novel-quantification-driven-proteomic-strategy-identifies-an-endogenous-peptide-of-pleiotrophin-as-a-new-biomarker-of-alzheimer-s-disease
#6
Tobias Skillbäck, Niklas Mattsson, Karl Hansson, Ekaterina Mirgorodskaya, Rahil Dahlén, Wiesje van der Flier, Philip Scheltens, Floor Duits, Oskar Hansson, Charlotte Teunissen, Kaj Blennow, Henrik Zetterberg, Johan Gobom
We present a new, quantification-driven proteomic approach to identifying biomarkers. In contrast to the identification-driven approach, limited in scope to peptides that are identified by database searching in the first step, all MS data are considered to select biomarker candidates. The endopeptidome of cerebrospinal fluid from 40 Alzheimer's disease (AD) patients, 40 subjects with mild cognitive impairment, and 40 controls with subjective cognitive decline was analyzed using multiplex isobaric labeling. Spectral clustering was used to match MS/MS spectra...
October 17, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29037499/neuropsychiatric-and-cognitive-profile-of-early-richardson-s-syndrome-progressive-supranuclear-palsy-parkinsonism-and-parkinson-s-disease
#7
Clelia Pellicano, Francesca Assogna, Nystya Cellupica, Federica Piras, Mariangela Pierantozzi, Alessandro Stefani, Rocco Cerroni, Bruno Mercuri, Carlo Caltagirone, Francesco E Pontieri, Gianfranco Spalletta
INTRODUCTION: The two main variants of Progressive Supranuclear Palsy (PSP), Richardson's syndrome (PSP-RS) and PSP-parkinsonism (PSP-P), share motor and non-motor features with Parkinson's disease (PD) particularly in the early stages. This makes the precocious diagnosis more challenging. We aimed at defining qualitative and quantitative differences of neuropsychiatric and neuropsychological profiles between PSP-P, PSP-RS and PD patients recruited within 24 months after the onset of symptoms, in order to clarify if the identification of peculiar cognitive and psychiatric symptoms is of help for early PSP diagnosis...
October 6, 2017: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/29029214/sleepless-night-and-day-the-plight-of-progressive-supranuclear-palsy
#8
Christine M Walsh, Leslie Ruoff, Kathleen Walker, Alaisa Emery, Jonathan Varbel, Elissaios Karageorgiou, Phi N Luong, Irida Mance, Hilary W Heuer, Adam L Boxer, Lea T Grinberg, Joel H Kramer, Bruce L Miller, Thomas C Neylan
Objectives: To elucidate the unique sleep and waking characteristics in progressive supranuclear palsy (PSP), a neurodegenerative disease associated with motor deficits and dementia that largely affects the brainstem and thalamic regions. Methods: 20 PSP and 16 healthy older adult controls participated in this study. Participants underwent an overnight polysomnography and multiple sleep latency test (MSLT) the following day. Prior to the MSLT last trial, participants were asked to complete the Stanford Sleepiness Scale...
October 5, 2017: Sleep
https://www.readbyqxmd.com/read/28987182/tauopathies
#9
Gabor G Kovacs
Tauopathies are neurodegenerative disorders characterized by the deposition of abnormal tau protein in the brain. The spectrum of tau pathologies expands beyond the traditionally discussed disease forms like Pick disease, progressive supranuclear palsy, corticobasal degeneration, and argyrophilic grain disease. Emerging entities and pathologies include globular glial tauopathies, primary age-related tauopathy, which includes neurofibrillary tangle dementia, chronic traumatic encephalopathy (CTE), and aging-related tau astrogliopathy...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28987055/the-pattern-of-verbal-visuospatial-and-procedural-learning-in-richardson-variant-of-progressive-supranuclear-palsy-in-comparison-to-parkinson-s-disease
#10
Emilia J Sitek, Dariusz Wieczorek, Agnieszka Konkel, Magda Dąbrowska, Jarosław Sławek
OBJECTIVES: Progressive supranuclear palsy (PSP) is regarded either within spectrum of atypical parkinsonian syndromes or frontotemporal lobar degeneration. We compared the verbal, visuospatial and procedural learning profiles in patients with PSP and Parkinson's disease (PD). Furthermore, the relationship between executive factors (initiation and inhibition) and learning outcomes was analyzed. METHODS: Thirty-three patients with the clinical diagnosis of PSP-Richardson's syndrome (PSP-RS), 39 patients with PD and 29 age -and education -matched controls were administered Mini-Mental State Examination (MMSE), phonemic and semantic fluency tasks, Auditory Verbal Learning Test (AVLT), Visual Learning and Memory Test for Neuropsychological Assessment by Lamberti and Weidlich (Diagnosticum für Cerebralschädigung, DCS), Tower of Toronto (ToT) and two motor sequencing tasks...
August 29, 2017: Psychiatria Polska
https://www.readbyqxmd.com/read/28982612/brain-regional-iron-contents-in-progressive-supranuclear-palsy
#11
Seung Ha Lee, Chul Hyoung Lyoo, Sung Jun Ahn, Juha O Rinne, Myung Sik Lee
INTRODUCTION: To determine motor-related brain regions in which iron contents correlate with the degree of motor deficits of progressive supranuclear palsy (PSP). METHODS: Twenty-four patients with probable PSP and 20 controls were included. Using a 3.0T magnetic resonance imaging scanner, R2* values were measured in the putamen, globus pallidus (GP), substantia nigra (SN), subthalamic nucleus, and dentate nucleus. After adjustment for disease duration and age at examination, correlations between regional brain R2* values and Unified Parkinson Disease Rating Scale (UPDRS) total motor scores or subscores for bradykinesia, rigidity, tremor, or axial motor deficits were investigated...
September 28, 2017: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/28980714/-18-f-flortaucipir-tau-pet-distinguishes-established-progressive-supranuclear-palsy-from-controls-and-parkinson-s-disease-a-multicenter-study
#12
Daniel R Schonhaut, Corey T McMillan, Salvatore Spina, Bradford C Dickerson, Andrew Siderowf, Michael D Devous, Richard Tsai, Joseph Winer, David S Russell, Irene Litvan, Erik D Roberson, William W Seeley, Lea T Grinberg, Joel H Kramer, Bruce L Miller, Peter Pressman, Ilya Nasrallah, Suzanne L Baker, Stephen N Gomperts, Keith A Johnson, Murray Grossman, William J Jagust, Adam L Boxer, Gil D Rabinovici
OBJECTIVE: (18) F-flortaucipir (formerly (18) F-AV1451 or (18) F-T807) binds to neurofibrillary tangles in Alzheimer's disease (AD), but tissue studies assessing binding to tau aggregates in progressive supranuclear palsy (PSP) have yielded mixed results. We compared in vivo (18) F-flortaucipir uptake in patients meeting clinical research criteria for PSP (N=33) to normal controls (N=46) and patients meeting criteria for Parkinson's disease (PD, N=26). METHODS: Participants underwent MRI and positron emission tomography for amyloid-β ((11) C-PiB or (18) F-florbetapir) and tau ((18) F-flortaucipir)...
October 5, 2017: Annals of Neurology
https://www.readbyqxmd.com/read/28978658/the-social-and-economic-burden-of-frontotemporal-degeneration
#13
James E Galvin, David H Howard, Sharon S Denny, Susan Dickinson, Nadine Tatton
OBJECTIVE: To quantify the socioeconomic burden of frontotemporal degeneration (FTD) compared to previously published data for Alzheimer disease (AD). METHODS: A 250-item internet survey was administered to primary caregivers of patients with behavioral-variant FTD (bvFTD), primary progressive aphasia, FTD with motor neuron disease, corticobasal syndrome, or progressive supranuclear palsy. The survey included validated scales for disease staging, behavior, activities of daily living, caregiver burden, and health economics, as well as investigator-designed questions to capture patient and caregiver experience with FTD...
October 4, 2017: Neurology
https://www.readbyqxmd.com/read/28974731/alternative-promoter-usage-generates-novel-shorter-mapt-mrna-transcripts-in-alzheimer-s-disease-and-progressive-supranuclear-palsy-brains
#14
Vincent Huin, Luc Buée, Hélène Behal, Julien Labreuche, Bernard Sablonnière, Claire-Marie Dhaenens
Alternative promoter usage is an important mechanism for transcriptome diversity and the regulation of gene expression. Indeed, this alternative usage may influence tissue/subcellular specificity, protein translation and function of the proteins. The existence of an alternative promoter for MAPT gene was considered for a long time to explain differential tissue specificity and differential response to transcription and growth factors between mRNA transcripts. The alternative promoter usage could explain partly the different tau proteins expression patterns observed in tauopathies...
October 3, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28972305/mass-spectrometric-analysis-of-cerebrospinal-fluid-ubiquitin-in-alzheimer-s-disease-and-parkinsonian-disorders
#15
Simon Sjödin, Oskar Hansson, Annika Öhrfelt, Gunnar Brinkmalm, Henrik Zetterberg, Ann Brinkmalm, Kaj Blennow
PURPOSE: Dysfunctional proteostasis, with decreased protein degradation and an accumulation of ubiquitin into aggregated protein inclusions, is a feature of neurodegenerative diseases. Identifying new potential biomarkers in cerebrospinal fluid (CSF) reflecting this process could contribute important information on pathophysiology. EXPERIMENTAL DESIGN: We developed a method combining solid-phase extraction and parallel reaction monitoring mass spectrometry and monitored the concentration of ubiquitin in CSF from subjects with Alzheimer's disease (AD), Parkinson's disease (PD) and progressive supranuclear palsy (PSP)...
October 3, 2017: Proteomics. Clinical Applications
https://www.readbyqxmd.com/read/28971440/probing-conformational-dynamics-of-tau-protein-by-hydrogen-deuterium-exchange-mass-spectrometry
#16
Richard Y-C Huang, Roxana E Iacob, Sethu Sankaranarayanan, Ling Yang, Michael Ahlijanian, Li Tao, Adrienne A Tymiak, Guodong Chen
Fibrillization of the microtubule-associated protein tau has been recognized as one of the signature pathologies of the nervous system in Alzheimer's disease, progressive supranuclear palsy, and other tauopathies. The conformational transition of tau in the fibrillization process, tau monomer to soluble aggregates to fibrils in particular, remains unclear. Here we report on the use of hydrogen/deuterium exchange mass spectrometry (HDX-MS) in combination with other biochemical approaches, including Thioflavin S fluorescence measurements, enzyme-linked immunosorbent assay (ELISA), and Western blotting to understand the heparin-induced tau's fibrillization...
October 2, 2017: Journal of the American Society for Mass Spectrometry
https://www.readbyqxmd.com/read/28961692/the-neuropsychology-broadly-conceived-of-multiple-system-atrophy-progressive-supranuclear-palsy-and-corticobasal-degeneration
#17
Adam Gerstenecker
Objective: To review the cognitive and behavioral features of the different atypical parkinsonian syndromes in which motor symptoms dominate early clinical symptomology: multiple systems atrophy (MSA), progressive supranuclear palsy (PSP), and corticobasal degeneration (CBD). The impact of cognitive and behavioral deficits on quality of life, associations between neuropsychological and neuropsychiatric findings and brain imaging, and cognitive and behavioral symptom management are also discussed...
September 15, 2017: Archives of Clinical Neuropsychology: the Official Journal of the National Academy of Neuropsychologists
https://www.readbyqxmd.com/read/28951832/advancing-functional-dysconnectivity-and-atrophy-in-progressive-supranuclear-palsy
#18
Jesse A Brown, Alice Y Hua, Andrew Trujllo, Suneth Attygalle, Richard J Binney, Salvatore Spina, Suzee E Lee, Joel H Kramer, Bruce L Miller, Howard J Rosen, Adam L Boxer, William W Seeley
Progressive supranuclear palsy syndrome (PSP-S) results from neurodegeneration within a network of brainstem, subcortical, frontal and parietal cortical brain regions. It is unclear how network dysfunction progresses and relates to longitudinal atrophy and clinical decline. In this study, we evaluated patients with PSP-S (n = 12) and healthy control subjects (n = 20) at baseline and 6 months later. Subjects underwent structural MRI and task-free functional MRI (tf-fMRI) scans and clinical evaluations at both time points...
2017: NeuroImage: Clinical
https://www.readbyqxmd.com/read/28944256/depressive-symptoms-are-frequent-in-atypical-parkinsonian-disorders
#19
Leonardo Almeida, Bilal Ahmed, Roger Walz, Sol De Jesus, Addie Patterson, Daniel Martinez-Ramirez, David Vaillancourt, Dawn Bowers, Herbert Ward, Michael S Okun, Nikolaus R McFarland
OBJECTIVE: To compare the incidence and prevalence of depressive symptoms in atypical parkinsonian (APD) syndromes versus Parkinson disease (PD). METHODS: In a large retrospective patient cohort we analyzed the incidence and prevalence of depressive symptoms using the Beck Depression Inventory (BDI) and evaluated subjects longitudinally on subsequent visits. For individuals who followed in subsequent visits we calculated incidence rates in person-years as a measure of incidence...
March 2017: Movement Disorders Clinical Practice
https://www.readbyqxmd.com/read/28943542/a-card-type-odor-identification-test-for-japanese-patients-with-parkinson-s-disease-and-related-disorders
#20
Yuji Watanabe, Keisuke Suzuki, Tomoyuki Miyamoto, Masayuki Miyamoto, Ayaka Numao, Hiroaki Fujita, Tomoyuki Uchiyama, Taro Kadowaki, Takeo Matsubara, Koichi Hirata
Objective The characteristics of olfactory impairment in Parkinson's disease (PD), multiple system atrophy (MSA) and progressive supranuclear palsy (PSP) have not been determined in detail. We assessed the olfactory function among PD, MSA and PSP patients. Methods A card-type odor identification test, Open Essence (OE, Wako, Japan), which consists of 12 different odorants familiar to Japanese subjects, was administered to 98 PD patients, 32 MSA patients, 17 PSP patients and 96 control subjects ≥50 years of age...
September 25, 2017: Internal Medicine
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